home *** CD-ROM | disk | FTP | other *** search
Unknown | 1992-06-06 | 495.3 KB |
open in:
MacOS 8.1
|
Win98
|
DOS
view JSON data
|
view as text
This file was not able to be converted.
This format is not currently supported by dexvert.
Confidence | Program | Detection | Match Type | Support
|
---|
66%
| dexvert
| OS/2 Installation Package (archive/os2InstallPackage)
| ext
| Supported |
1%
| dexvert
| BeOS Installation Package (archive/beOSInstallPackage)
| ext
| Unsupported |
1%
| dexvert
| Newton Package (archive/newtonPackage)
| ext
| Unsupported |
1%
| dexvert
| Independence War 2 mod Package (other/independenceWar2ModPackage)
| ext
| Unsupported |
100%
| file
| data
| default
|
|
hex view+--------+-------------------------+-------------------------+--------+--------+
|00000000| 1a 44 50 31 2e 31 00 00 | 00 00 00 00 00 05 64 00 |.DP1.1..|......d.|
|00000010| 00 00 16 00 00 00 30 30 | 00 00 00 01 9c 05 00 00 |......00|........|
|00000020| 76 19 00 00 30 31 00 00 | 00 01 12 1f 00 00 7d 15 |v...01..|......}.|
|00000030| 00 00 30 32 00 00 00 01 | 8f 34 00 00 fb 15 00 00 |..02....|.4......|
|00000040| 30 33 00 00 00 01 8a 4a | 00 00 4f 1a 00 00 30 34 |03.....J|..O...04|
|00000050| 00 00 00 01 d9 64 00 00 | 74 14 00 00 30 35 00 00 |.....d..|t...05..|
|00000060| 00 01 4d 79 00 00 c2 09 | 00 00 30 36 00 00 00 01 |..My....|..06....|
|00000070| 0f 83 00 00 00 0f 00 00 | 30 37 00 00 00 01 0f 92 |........|07......|
|00000080| 00 00 0e 20 00 00 30 38 | 00 00 00 01 1d b2 00 00 |... ..08|........|
|00000090| 5d 09 00 00 30 39 00 00 | 00 01 7a bb 00 00 31 18 |]...09..|..z...1.|
|000000a0| 00 00 31 30 00 00 00 01 | ab d3 00 00 3d 0b 00 00 |..10....|....=...|
|000000b0| 31 31 00 00 00 01 e8 de | 00 00 ec 15 00 00 31 32 |11......|......12|
|000000c0| 00 00 00 01 d4 f4 00 00 | 10 17 00 00 31 33 00 00 |........|....13..|
|000000d0| 00 01 e4 0b 01 00 40 1b | 00 00 31 34 00 00 00 01 |......@.|..14....|
|000000e0| 24 27 01 00 42 13 00 00 | 31 35 00 00 00 01 66 3a |$'..B...|15....f:|
|000000f0| 01 00 5d 10 00 00 31 36 | 00 00 00 01 c3 4a 01 00 |..]...16|.....J..|
|00000100| 67 13 00 00 31 37 00 00 | 00 01 2a 5e 01 00 67 12 |g...17..|..*^..g.|
|00000110| 00 00 31 38 00 00 00 01 | 91 70 01 00 45 17 00 00 |..18....|.p..E...|
|00000120| 31 39 00 00 00 01 d6 87 | 01 00 d9 11 00 00 32 30 |19......|......20|
|00000130| 00 00 00 01 af 99 01 00 | eb 16 00 00 32 31 00 00 |........|....21..|
|00000140| 00 01 9a b0 01 00 23 13 | 00 00 32 32 00 00 00 01 |......#.|..22....|
|00000150| bd c3 01 00 10 1c 00 00 | 32 33 00 00 00 01 cd df |........|23......|
|00000160| 01 00 92 15 00 00 32 34 | 00 00 00 01 5f f5 01 00 |......24|...._...|
|00000170| 7c 13 00 00 32 35 00 00 | 00 01 db 08 02 00 d3 0e ||...25..|........|
|00000180| 00 00 32 36 00 00 00 01 | ae 17 02 00 21 15 00 00 |..26....|....!...|
|00000190| 32 37 00 00 00 01 cf 2c | 02 00 bc 11 00 00 32 38 |27.....,|......28|
|000001a0| 00 00 00 01 8b 3e 02 00 | fa 11 00 00 32 39 00 00 |.....>..|....29..|
|000001b0| 00 01 85 50 02 00 61 1a | 00 00 33 30 00 00 00 01 |...P..a.|..30....|
|000001c0| e6 6a 02 00 e4 15 00 00 | 33 31 00 00 00 01 ca 80 |.j......|31......|
|000001d0| 02 00 1f 0d 00 00 33 32 | 00 00 00 01 e9 8d 02 00 |......32|........|
|000001e0| 9c 12 00 00 33 33 00 00 | 00 01 85 a0 02 00 b1 19 |....33..|........|
|000001f0| 00 00 33 34 00 00 00 01 | 36 ba 02 00 ed 0b 00 00 |..34....|6.......|
|00000200| 33 35 00 00 00 01 23 c6 | 02 00 da 18 00 00 33 36 |35....#.|......36|
|00000210| 00 00 00 01 fd de 02 00 | 6f 11 00 00 33 37 00 00 |........|o...37..|
|00000220| 00 01 6c f0 02 00 7f 18 | 00 00 33 38 00 00 00 01 |..l.....|..38....|
|00000230| eb 08 03 00 eb 19 00 00 | 33 39 00 00 00 01 d6 22 |........|39....."|
|00000240| 03 00 bf 18 00 00 34 30 | 00 00 00 01 95 3b 03 00 |......40|.....;..|
|00000250| 96 16 00 00 34 31 00 00 | 00 01 2b 52 03 00 41 09 |....41..|..+R..A.|
|00000260| 00 00 34 32 00 00 00 01 | 6c 5b 03 00 99 11 00 00 |..42....|l[......|
|00000270| 34 33 00 00 00 01 05 6d | 03 00 ab 17 00 00 34 34 |43.....m|......44|
|00000280| 00 00 00 01 b0 84 03 00 | 4a 13 00 00 34 35 00 00 |........|J...45..|
|00000290| 00 01 fa 97 03 00 2a 15 | 00 00 34 36 00 00 00 01 |......*.|..46....|
|000002a0| 24 ad 03 00 0d 13 00 00 | 34 37 00 00 00 01 31 c0 |$.......|47....1.|
|000002b0| 03 00 51 0c 00 00 34 38 | 00 00 00 01 82 cc 03 00 |..Q...48|........|
|000002c0| e8 1d 00 00 34 39 00 00 | 00 01 6a ea 03 00 44 15 |....49..|..j...D.|
|000002d0| 00 00 35 30 00 00 00 01 | ae ff 03 00 14 18 00 00 |..50....|........|
|000002e0| 35 31 00 00 00 01 c2 17 | 04 00 74 12 00 00 35 32 |51......|..t...52|
|000002f0| 00 00 00 01 36 2a 04 00 | 69 11 00 00 35 33 00 00 |....6*..|i...53..|
|00000300| 00 01 9f 3b 04 00 9f 13 | 00 00 35 34 00 00 00 01 |...;....|..54....|
|00000310| 3e 4f 04 00 e0 0f 00 00 | 35 35 00 00 00 01 1e 5f |>O......|55....._|
|00000320| 04 00 b3 16 00 00 35 36 | 00 00 00 01 d1 75 04 00 |......56|.....u..|
|00000330| b1 0f 00 00 35 37 00 00 | 00 01 82 85 04 00 40 12 |....57..|......@.|
|00000340| 00 00 35 38 00 00 00 01 | c2 97 04 00 03 0c 00 00 |..58....|........|
|00000350| 35 39 00 00 00 01 c5 a3 | 04 00 71 17 00 00 36 30 |59......|..q...60|
|00000360| 00 00 00 01 36 bb 04 00 | 80 12 00 00 36 31 00 00 |....6...|....61..|
|00000370| 00 01 b6 cd 04 00 5c 19 | 00 00 36 32 00 00 00 01 |......\.|..62....|
|00000380| 12 e7 04 00 17 17 00 00 | 36 33 00 00 00 01 29 fe |........|63....).|
|00000390| 04 00 88 09 00 00 36 34 | 00 00 00 01 b1 07 05 00 |......64|........|
|000003a0| 8b 15 00 00 36 35 00 00 | 00 01 3c 1d 05 00 75 09 |....65..|..<...u.|
|000003b0| 00 00 36 36 00 00 00 01 | b1 26 05 00 fe 1f 00 00 |..66....|.&......|
|000003c0| 36 37 00 00 00 01 af 46 | 05 00 2a 15 00 00 36 38 |67.....F|..*...68|
|000003d0| 00 00 00 01 d9 5b 05 00 | 7f 16 00 00 36 39 00 00 |.....[..|....69..|
|000003e0| 00 01 58 72 05 00 c7 11 | 00 00 37 30 00 00 00 01 |..Xr....|..70....|
|000003f0| 1f 84 05 00 62 13 00 00 | 37 31 00 00 00 01 81 97 |....b...|71......|
|00000400| 05 00 12 17 00 00 37 32 | 00 00 00 01 93 ae 05 00 |......72|........|
|00000410| a0 0b 00 00 37 33 00 00 | 00 01 33 ba 05 00 29 17 |....73..|..3...).|
|00000420| 00 00 37 34 00 00 00 01 | 5c d1 05 00 e1 09 00 00 |..74....|\.......|
|00000430| 37 35 00 00 00 01 3d db | 05 00 82 08 00 00 37 36 |75....=.|......76|
|00000440| 00 00 00 01 bf e3 05 00 | 18 15 00 00 37 37 00 00 |........|....77..|
|00000450| 00 01 d7 f8 05 00 2c 12 | 00 00 37 38 00 00 00 01 |......,.|..78....|
|00000460| 03 0b 06 00 7d 19 00 00 | 37 39 00 00 00 01 80 24 |....}...|79.....$|
|00000470| 06 00 59 0b 00 00 38 30 | 00 00 00 01 d9 2f 06 00 |..Y...80|...../..|
|00000480| 37 15 00 00 38 31 00 00 | 00 01 10 45 06 00 9a 16 |7...81..|...E....|
|00000490| 00 00 38 32 00 00 00 01 | aa 5b 06 00 c9 11 00 00 |..82....|.[......|
|000004a0| 38 33 00 00 00 01 73 6d | 06 00 cf 15 00 00 38 34 |83....sm|......84|
|000004b0| 00 00 00 01 42 83 06 00 | 53 16 00 00 38 35 00 00 |....B...|S...85..|
|000004c0| 00 01 95 99 06 00 05 11 | 00 00 38 36 00 00 00 01 |........|..86....|
|000004d0| 9a aa 06 00 dd 09 00 00 | 38 37 00 00 00 01 77 b4 |........|87....w.|
|000004e0| 06 00 0e 18 00 00 38 38 | 00 00 00 01 85 cc 06 00 |......88|........|
|000004f0| ab 1a 00 00 38 39 00 00 | 00 01 30 e7 06 00 12 10 |....89..|..0.....|
|00000500| 00 00 39 30 00 00 00 01 | 42 f7 06 00 35 11 00 00 |..90....|B...5...|
|00000510| 39 31 00 00 00 01 77 08 | 07 00 40 11 00 00 39 32 |91....w.|..@...92|
|00000520| 00 00 00 01 b7 19 07 00 | c1 21 00 00 39 33 00 00 |........|.!..93..|
|00000530| 00 01 78 3b 07 00 29 08 | 00 00 39 34 00 00 00 01 |..x;..).|..94....|
|00000540| a1 43 07 00 c2 17 00 00 | 39 35 00 00 00 01 63 5b |.C......|95....c[|
|00000550| 07 00 4f 18 00 00 39 36 | 00 00 00 01 b2 73 07 00 |..O...96|.....s..|
|00000560| f0 17 00 00 39 37 00 00 | 00 01 a2 8b 07 00 a7 0a |....97..|........|
|00000570| 00 00 39 38 00 00 00 01 | 49 96 07 00 d4 12 00 00 |..98....|I.......|
|00000580| 39 39 00 00 00 01 1d a9 | 07 00 2f 14 00 00 00 00 |99......|../.....|
|00000590| 00 00 00 00 4c bd 07 00 | 00 00 00 00 24 55 6e 69 |....L...|....$Uni|
|000005a0| 71 75 65 5f 49 44 7b 59 | 52 42 31 32 30 30 30 7d |que_ID{Y|RB12000}|
|000005b0| 0d 0a 24 54 69 74 6c 65 | 7b 50 72 65 64 69 63 74 |..$Title|{Predict|
|000005c0| 69 6e 67 20 74 68 65 20 | 52 65 73 70 6f 6e 73 65 |ing the |Response|
|000005d0| 20 74 6f 20 43 79 74 6f | 74 6f 78 69 63 20 54 68 | to Cyto|toxic Th|
|000005e0| 65 72 61 70 79 20 66 6f | 72 20 43 68 69 6c 64 68 |erapy fo|r Childh|
|000005f0| 6f 6f 64 20 4e 65 70 68 | 72 6f 74 69 63 20 53 79 |ood Neph|rotic Sy|
|00000600| 6e 64 72 6f 6d 65 3a 20 | 20 53 75 70 65 72 69 6f |ndrome: | Superio|
|00000610| 72 69 74 79 20 6f 66 20 | 52 65 73 70 6f 6e 73 65 |rity of |Response|
|00000620| 20 74 6f 20 43 6f 72 74 | 69 63 6f 73 74 65 72 6f | to Cort|icostero|
|00000630| 69 64 20 54 68 65 72 61 | 70 79 20 4f 76 65 72 20 |id Thera|py Over |
|00000640| 48 69 73 74 6f 70 61 74 | 68 6f 6c 6f 67 69 63 20 |Histopat|hologic |
|00000650| 50 61 74 74 65 72 6e 73 | 7d 0d 0a 24 41 75 74 68 |Patterns|}..$Auth|
|00000660| 6f 72 7b 53 63 68 75 6c | 6d 61 6e 2c 20 53 4c 0d |or{Schul|man, SL.|
|00000670| 0a 4b 61 69 73 65 72 2c | 20 42 41 0d 0a 50 6f 6c |.Kaiser,| BA..Pol|
|00000680| 69 6e 73 6b 79 2c 20 4d | 53 0d 0a 53 72 69 6e 69 |insky, M|S..Srini|
|00000690| 76 61 73 61 6e 2c 20 52 | 0d 0a 42 61 6c 75 61 72 |vasan, R|..Baluar|
|000006a0| 74 65 2c 20 48 4a 7d 0d | 0a 24 41 66 66 69 6c 69 |te, HJ}.|.$Affili|
|000006b0| 61 74 69 6f 6e 7b 53 74 | 20 43 68 72 69 73 74 6f |ation{St| Christo|
|000006c0| 70 68 65 72 27 73 20 48 | 6f 73 70 20 66 6f 72 20 |pher's H|osp for |
|000006d0| 43 68 69 6c 64 72 65 6e | 2c 20 50 68 69 6c 61 64 |Children|, Philad|
|000006e0| 65 6c 70 68 69 61 3b 20 | 54 65 6d 70 6c 65 20 55 |elphia; |Temple U|
|000006f0| 6e 69 76 7d 0d 0a 24 4a | 6f 75 72 6e 61 6c 7b 4a |niv}..$J|ournal{J|
|00000700| 20 50 65 64 69 61 74 72 | 7d 0d 0a 24 56 6f 6c 75 | Pediatr|}..$Volu|
|00000710| 6d 65 7b 31 31 33 7d 0d | 0a 24 50 61 67 65 7b 39 |me{113}.|.$Page{9|
|00000720| 39 36 2d 31 30 30 31 7d | 0d 0a 24 44 61 74 65 7b |96-1001}|..$Date{|
|00000730| 44 65 63 65 6d 62 65 72 | 20 31 39 38 38 7d 0d 0a |December| 1988}..|
|00000740| 24 59 42 56 6f 6c 7b 31 | 39 39 30 20 59 65 61 72 |$YBVol{1|990 Year|
|00000750| 20 42 6f 6f 6b 20 6f 66 | 20 50 65 64 69 61 74 72 | Book of| Pediatr|
|00000760| 69 63 73 7d 0d 0a 24 43 | 68 61 70 74 65 72 7b 31 |ics}..$C|hapter{1|
|00000770| 30 2d 31 30 7d 0d 0a 24 | 59 42 45 64 69 74 6f 72 |0-10}..$|YBEditor|
|00000780| 7b 44 72 73 2e 20 4f 73 | 6b 69 20 61 6e 64 20 53 |{Drs. Os|ki and S|
|00000790| 74 6f 63 6b 6d 61 6e 7d | 0d 0a 24 54 61 62 6c 65 |tockman}|..$Table|
|000007a0| 7b 7d 0d 0a 24 43 6f 6d | 6d 65 6e 74 61 74 6f 72 |{}..$Com|mentator|
|000007b0| 7b 54 2e 52 2e 20 57 65 | 6c 63 68 2c 20 4d 2e 44 |{T.R. We|lch, M.D|
|000007c0| 2e 7d 0d 0a 0d 0a 43 6f | 70 79 72 69 67 68 74 20 |.}....Co|pyright |
|000007d0| 28 63 29 20 59 65 61 72 | 62 6f 6f 6b 20 4d 65 64 |(c) Year|book Med|
|000007e0| 69 63 61 6c 20 50 75 62 | 6c 69 73 68 65 72 73 2c |ical Pub|lishers,|
|000007f0| 20 49 6e 63 2e 2c 20 31 | 39 39 30 0d 0a 0d 0a 31 | Inc., 1|990....1|
|00000800| 39 39 30 20 59 65 61 72 | 20 42 6f 6f 6b 20 6f 66 |990 Year| Book of|
|00000810| 20 50 65 64 69 61 74 72 | 69 63 73 20 28 52 29 0d | Pediatr|ics (R).|
|00000820| 0a 0d 0a 43 68 61 70 74 | 65 72 20 31 30 2d 2d 54 |...Chapt|er 10--T|
|00000830| 68 65 20 47 65 6e 69 74 | 6f 75 72 69 6e 61 72 79 |he Genit|ourinary|
|00000840| 20 54 72 61 63 74 0d 0a | 0d 0a 2d 2d 2d 2d 2d 2d | Tract..|..------|
|00000850| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00000860| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00000870| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00000880| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00000890| 2d 2d 2d 2d 2d 2d 2d 2d | 0d 0a 50 72 65 64 69 63 |--------|..Predic|
|000008a0| 74 69 6e 67 20 74 68 65 | 20 52 65 73 70 6f 6e 73 |ting the| Respons|
|000008b0| 65 20 74 6f 20 43 79 74 | 6f 74 6f 78 69 63 20 54 |e to Cyt|otoxic T|
|000008c0| 68 65 72 61 70 79 20 66 | 6f 72 20 43 68 69 6c 64 |herapy f|or Child|
|000008d0| 68 6f 6f 64 20 4e 65 70 | 68 72 6f 74 69 63 20 53 |hood Nep|hrotic S|
|000008e0| 79 6e 64 72 6f 6d 65 3a | 0d 0a 53 75 70 65 72 69 |yndrome:|..Superi|
|000008f0| 6f 72 69 74 79 20 6f 66 | 20 52 65 73 70 6f 6e 73 |ority of| Respons|
|00000900| 65 20 74 6f 20 43 6f 72 | 74 69 63 6f 73 74 65 72 |e to Cor|ticoster|
|00000910| 6f 69 64 20 54 68 65 72 | 61 70 79 20 4f 76 65 72 |oid Ther|apy Over|
|00000920| 20 48 69 73 74 6f 70 61 | 74 68 6f 6c 6f 67 69 63 | Histopa|thologic|
|00000930| 0d 0a 50 61 74 74 65 72 | 6e 73 0d 0a 0d 0a 53 63 |..Patter|ns....Sc|
|00000940| 68 75 6c 6d 61 6e 20 53 | 4c 2c 20 4b 61 69 73 65 |hulman S|L, Kaise|
|00000950| 72 20 42 41 2c 20 50 6f | 6c 69 6e 73 6b 79 20 4d |r BA, Po|linsky M|
|00000960| 53 2c 20 53 72 69 6e 69 | 76 61 73 61 6e 20 52 2c |S, Srini|vasan R,|
|00000970| 20 42 61 6c 75 61 72 74 | 65 20 48 4a 0d 0a 28 53 | Baluart|e HJ..(S|
|00000980| 74 20 43 68 72 69 73 74 | 6f 70 68 65 72 27 73 20 |t Christ|opher's |
|00000990| 48 6f 73 70 20 66 6f 72 | 20 43 68 69 6c 64 72 65 |Hosp for| Childre|
|000009a0| 6e 2c 20 50 68 69 6c 61 | 64 65 6c 70 68 69 61 3b |n, Phila|delphia;|
|000009b0| 20 54 65 6d 70 6c 65 20 | 55 6e 69 76 29 0d 0a 4a | Temple |Univ)..J|
|000009c0| 20 50 65 64 69 61 74 72 | 20 31 31 33 3a 39 39 36 | Pediatr| 113:996|
|000009d0| 2d 31 30 30 31 2c 20 44 | 65 63 65 6d 62 65 72 20 |-1001, D|ecember |
|000009e0| 31 39 38 38 0d 0a 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |1988..--|--------|
|000009f0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00000a00| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00000a10| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00000a20| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00000a30| 2d 2d 2d 2d 0d 0a 0d 0a | 41 62 73 74 72 61 63 74 |----....|Abstract|
|00000a40| 0d 0a 0d 0a 20 20 20 20 | 20 43 6f 6e 74 72 6f 76 |.... | Controv|
|00000a50| 65 72 73 79 20 65 78 69 | 73 74 73 20 72 65 67 61 |ersy exi|sts rega|
|00000a60| 72 64 69 6e 67 20 74 68 | 65 20 76 61 6c 75 65 20 |rding th|e value |
|00000a70| 6f 66 20 72 65 6e 61 6c | 20 62 69 6f 70 73 79 20 |of renal| biopsy |
|00000a80| 62 65 66 6f 72 65 20 69 | 6e 69 74 69 61 74 69 6e |before i|nitiatin|
|00000a90| 67 0d 0a 63 79 74 6f 74 | 6f 78 69 63 20 64 72 75 |g..cytot|oxic dru|
|00000aa0| 67 20 74 68 65 72 61 70 | 79 20 69 6e 20 61 20 63 |g therap|y in a c|
|00000ab0| 68 69 6c 64 20 77 69 74 | 68 20 73 74 65 72 6f 69 |hild wit|h steroi|
|00000ac0| 64 2d 73 65 6e 73 69 74 | 69 76 65 20 62 75 74 20 |d-sensit|ive but |
|00000ad0| 66 72 65 71 75 65 6e 74 | 6c 79 0d 0a 72 65 6c 61 |frequent|ly..rela|
|00000ae0| 70 73 69 6e 67 20 6e 65 | 70 68 72 6f 74 69 63 20 |psing ne|phrotic |
|00000af0| 73 79 6e 64 72 6f 6d 65 | 20 6f 72 20 77 69 74 68 |syndrome| or with|
|00000b00| 20 73 74 65 72 6f 69 64 | 2d 64 65 70 65 6e 64 65 | steroid|-depende|
|00000b10| 6e 74 20 6e 65 70 68 72 | 6f 74 69 63 20 73 79 6e |nt nephr|otic syn|
|00000b20| 64 72 6f 6d 65 2e 20 20 | 49 6e 0d 0a 61 20 72 65 |drome. |In..a re|
|00000b30| 74 72 6f 73 70 65 63 74 | 69 76 65 20 73 74 75 64 |trospect|ive stud|
|00000b40| 79 2c 20 31 31 39 20 62 | 69 6f 70 73 79 20 73 70 |y, 119 b|iopsy sp|
|00000b50| 65 63 69 6d 65 6e 73 20 | 66 72 6f 6d 20 39 32 20 |ecimens |from 92 |
|00000b60| 63 68 69 6c 64 72 65 6e | 20 61 67 65 64 20 31 2d |children| aged 1-|
|00000b70| 31 36 20 79 65 61 72 73 | 0d 0a 77 65 72 65 20 65 |16 years|..were e|
|00000b80| 78 61 6d 69 6e 65 64 20 | 74 6f 20 64 65 74 65 72 |xamined |to deter|
|00000b90| 6d 69 6e 65 20 74 68 65 | 20 75 73 65 66 75 6c 6e |mine the| usefuln|
|00000ba0| 65 73 73 20 6f 66 20 74 | 68 65 20 73 74 65 72 6f |ess of t|he stero|
|00000bb0| 69 64 20 72 65 73 70 6f | 6e 73 65 20 69 6e 0d 0a |id respo|nse in..|
|00000bc0| 63 6c 61 73 73 69 66 79 | 69 6e 67 20 63 68 69 6c |classify|ing chil|
|00000bd0| 64 68 6f 6f 64 20 6e 65 | 70 68 72 6f 74 69 63 20 |dhood ne|phrotic |
|00000be0| 73 79 6e 64 72 6f 6d 65 | 2e 20 20 53 74 65 72 6f |syndrome|. Stero|
|00000bf0| 69 64 20 72 65 73 70 6f | 6e 73 65 73 2c 20 63 6c |id respo|nses, cl|
|00000c00| 61 73 73 69 66 69 65 64 | 20 61 73 0d 0a 73 74 65 |assified| as..ste|
|00000c10| 72 6f 69 64 20 72 65 73 | 69 73 74 61 6e 74 2c 20 |roid res|istant, |
|00000c20| 73 74 65 72 6f 69 64 20 | 64 65 70 65 6e 64 65 6e |steroid |dependen|
|00000c30| 74 2c 20 61 6e 64 20 66 | 72 65 71 75 65 6e 74 20 |t, and f|requent |
|00000c40| 72 65 6c 61 70 73 65 72 | 20 61 73 20 64 65 66 69 |relapser| as defi|
|00000c50| 6e 65 64 20 62 79 20 74 | 68 65 0d 0a 49 6e 74 65 |ned by t|he..Inte|
|00000c60| 72 6e 61 74 69 6f 6e 61 | 6c 20 53 74 75 64 79 20 |rnationa|l Study |
|00000c70| 6f 66 20 4b 69 64 6e 65 | 79 20 44 69 73 65 61 73 |of Kidne|y Diseas|
|00000c80| 65 20 69 6e 20 43 68 69 | 6c 64 72 65 6e 2c 20 77 |e in Chi|ldren, w|
|00000c90| 65 72 65 20 63 6f 72 72 | 65 6c 61 74 65 64 20 77 |ere corr|elated w|
|00000ca0| 69 74 68 20 74 68 65 0d | 0a 68 69 73 74 6f 6c 6f |ith the.|.histolo|
|00000cb0| 67 69 63 20 64 69 61 67 | 6e 6f 73 69 73 2e 20 20 |gic diag|nosis. |
|00000cc0| 54 68 65 20 6d 65 61 6e | 20 66 6f 6c 6c 6f 77 2d |The mean| follow-|
|00000cd0| 75 70 20 77 61 73 20 37 | 2e 32 20 79 65 61 72 73 |up was 7|.2 years|
|00000ce0| 2e 0d 0a 20 20 20 20 20 | 42 69 6f 70 73 79 20 73 |... |Biopsy s|
|00000cf0| 70 65 63 69 6d 65 6e 73 | 20 73 68 6f 77 65 64 20 |pecimens| showed |
|00000d00| 66 6f 63 61 6c 20 67 6c | 6f 6d 65 72 75 6c 6f 73 |focal gl|omerulos|
|00000d10| 63 6c 65 72 6f 73 69 73 | 20 69 6e 20 33 39 20 63 |clerosis| in 39 c|
|00000d20| 68 69 6c 64 72 65 6e 2c | 20 6c 69 70 6f 69 64 0d |hildren,| lipoid.|
|00000d30| 0a 6e 65 70 68 72 6f 73 | 69 73 20 69 6e 20 32 38 |.nephros|is in 28|
|00000d40| 2c 20 61 6e 64 20 71 75 | 65 73 74 69 6f 6e 61 62 |, and qu|estionab|
|00000d50| 6c 65 20 66 69 6e 64 69 | 6e 67 73 20 69 6e 20 32 |le findi|ngs in 2|
|00000d60| 35 2e 20 20 54 68 65 72 | 65 20 77 61 73 20 73 74 |5. Ther|e was st|
|00000d70| 72 6f 6e 67 20 61 67 72 | 65 65 6d 65 6e 74 0d 0a |rong agr|eement..|
|00000d80| 62 65 74 77 65 65 6e 20 | 63 68 69 6c 64 72 65 6e |between |children|
|00000d90| 20 77 68 6f 73 65 20 66 | 6f 63 61 6c 20 67 6c 6f | whose f|ocal glo|
|00000da0| 6d 65 72 75 6c 6f 73 63 | 6c 65 72 6f 73 69 73 20 |merulosc|lerosis |
|00000db0| 77 61 73 20 73 74 65 72 | 6f 69 64 20 72 65 73 69 |was ster|oid resi|
|00000dc0| 73 74 61 6e 74 20 61 6e | 64 0d 0a 74 68 6f 73 65 |stant an|d..those|
|00000dd0| 20 77 68 6f 73 65 20 6c | 69 70 6f 69 64 20 6e 65 | whose l|ipoid ne|
|00000de0| 70 68 72 6f 73 69 73 20 | 72 65 73 75 6c 74 65 64 |phrosis |resulted|
|00000df0| 20 69 6e 20 66 72 65 71 | 75 65 6e 74 20 72 65 6c | in freq|uent rel|
|00000e00| 61 70 73 65 73 2e 20 20 | 44 75 72 61 74 69 6f 6e |apses. |Duration|
|00000e10| 20 6f 66 0d 0a 72 65 6d | 69 73 73 69 6f 6e 73 20 | of..rem|issions |
|00000e20| 61 66 74 65 72 20 74 68 | 65 72 61 70 79 20 77 69 |after th|erapy wi|
|00000e30| 74 68 20 63 68 6c 6f 72 | 61 6d 62 75 63 69 6c 20 |th chlor|ambucil |
|00000e40| 6f 72 20 63 79 63 6c 6f | 70 68 6f 73 70 68 61 6d |or cyclo|phospham|
|00000e50| 69 64 65 20 77 61 73 20 | 64 65 74 65 72 6d 69 6e |ide was |determin|
|00000e60| 65 64 0d 0a 69 6e 20 38 | 34 20 63 68 69 6c 64 72 |ed..in 8|4 childr|
|00000e70| 65 6e 2e 20 20 49 6e 20 | 70 61 74 69 65 6e 74 73 |en. In |patients|
|00000e80| 20 77 69 74 68 20 66 6f | 63 61 6c 20 67 6c 6f 6d | with fo|cal glom|
|00000e90| 65 72 75 6c 6f 73 63 6c | 65 72 6f 73 69 73 2c 20 |eruloscl|erosis, |
|00000ea0| 74 68 65 20 64 75 72 61 | 74 69 6f 6e 20 6f 66 0d |the dura|tion of.|
|00000eb0| 0a 72 65 6d 69 73 73 69 | 6f 6e 20 61 66 74 65 72 |.remissi|on after|
|00000ec0| 20 63 79 74 6f 74 6f 78 | 69 63 20 74 68 65 72 61 | cytotox|ic thera|
|00000ed0| 70 79 20 77 61 73 20 73 | 69 67 6e 69 66 69 63 61 |py was s|ignifica|
|00000ee0| 6e 74 6c 79 20 73 68 6f | 72 74 65 72 20 61 73 20 |ntly sho|rter as |
|00000ef0| 63 6f 6d 70 61 72 65 64 | 20 77 69 74 68 0d 0a 74 |compared| with..t|
|00000f00| 68 6f 73 65 20 77 68 6f | 20 68 61 64 20 6c 69 70 |hose who| had lip|
|00000f10| 6f 69 64 20 6e 65 70 68 | 72 6f 73 69 73 2e 20 20 |oid neph|rosis. |
|00000f20| 54 68 69 73 20 64 69 66 | 66 65 72 65 6e 63 65 20 |This dif|ference |
|00000f30| 62 65 63 61 6d 65 20 6d | 6f 72 65 20 73 69 67 6e |became m|ore sign|
|00000f40| 69 66 69 63 61 6e 74 0d | 0a 62 65 74 77 65 65 6e |ificant.|.between|
|00000f50| 20 63 68 69 6c 64 72 65 | 6e 20 77 68 6f 20 77 65 | childre|n who we|
|00000f60| 72 65 20 73 74 65 72 6f | 69 64 20 72 65 73 69 73 |re stero|id resis|
|00000f70| 74 61 6e 74 20 61 73 20 | 6f 70 70 6f 73 65 64 20 |tant as |opposed |
|00000f80| 74 6f 20 73 74 65 72 6f | 69 64 2d 64 65 70 65 6e |to stero|id-depen|
|00000f90| 64 65 6e 74 0d 0a 61 6e | 64 20 66 72 65 71 75 65 |dent..an|d freque|
|00000fa0| 6e 74 6c 79 20 72 65 6c | 61 70 73 69 6e 67 20 70 |ntly rel|apsing p|
|00000fb0| 61 74 69 65 6e 74 73 2e | 20 20 49 6e 20 73 74 65 |atients.| In ste|
|00000fc0| 72 6f 69 64 2d 72 65 73 | 69 73 74 61 6e 74 20 70 |roid-res|istant p|
|00000fd0| 61 74 69 65 6e 74 73 2c | 20 66 6f 63 61 6c 0d 0a |atients,| focal..|
|00000fe0| 67 6c 6f 6d 65 72 75 6c | 6f 73 63 6c 65 72 6f 73 |glomerul|oscleros|
|00000ff0| 69 73 20 68 61 64 20 61 | 20 73 69 67 6e 69 66 69 |is had a| signifi|
|00001000| 63 61 6e 74 6c 79 20 73 | 68 6f 72 74 65 72 20 64 |cantly s|horter d|
|00001010| 75 72 61 74 69 6f 6e 20 | 6f 66 20 72 65 6d 69 73 |uration |of remis|
|00001020| 73 69 6f 6e 20 74 68 61 | 6e 20 69 6e 0d 0a 70 61 |sion tha|n in..pa|
|00001030| 74 69 65 6e 74 73 20 77 | 69 74 68 20 6f 74 68 65 |tients w|ith othe|
|00001040| 72 20 68 69 73 74 6f 6c | 6f 67 69 63 20 63 68 61 |r histol|ogic cha|
|00001050| 6e 67 65 73 2e 0d 0a 20 | 20 20 20 20 54 68 65 20 |nges... | The |
|00001060| 62 65 6e 65 66 69 63 69 | 61 6c 20 65 66 66 65 63 |benefici|al effec|
|00001070| 74 20 6f 66 20 63 79 74 | 6f 74 6f 78 69 63 20 74 |t of cyt|otoxic t|
|00001080| 68 65 72 61 70 79 20 69 | 6e 20 63 68 69 6c 64 68 |herapy i|n childh|
|00001090| 6f 6f 64 20 6e 65 70 68 | 72 6f 74 69 63 0d 0a 73 |ood neph|rotic..s|
|000010a0| 79 6e 64 72 6f 6d 65 20 | 63 6f 72 72 65 6c 61 74 |yndrome |correlat|
|000010b0| 65 73 20 77 69 74 68 20 | 74 68 65 20 70 61 74 74 |es with |the patt|
|000010c0| 65 72 6e 20 6f 66 20 72 | 65 73 70 6f 6e 73 65 20 |ern of r|esponse |
|000010d0| 74 6f 20 73 74 65 72 6f | 69 64 20 74 68 65 72 61 |to stero|id thera|
|000010e0| 70 79 20 72 61 74 68 65 | 72 0d 0a 74 68 61 6e 20 |py rathe|r..than |
|000010f0| 68 69 73 74 6f 6c 6f 67 | 69 63 20 61 62 6e 6f 72 |histolog|ic abnor|
|00001100| 6d 61 6c 69 74 79 2e 20 | 20 50 61 74 69 65 6e 74 |mality. | Patient|
|00001110| 73 20 77 69 74 68 20 73 | 74 65 72 6f 69 64 2d 73 |s with s|teroid-s|
|00001120| 65 6e 73 69 74 69 76 65 | 20 6e 65 70 68 72 6f 73 |ensitive| nephros|
|00001130| 69 73 20 6e 65 65 64 0d | 0a 6e 6f 74 20 75 6e 64 |is need.|.not und|
|00001140| 65 72 67 6f 20 72 65 6e | 61 6c 20 62 69 6f 70 73 |ergo ren|al biops|
|00001150| 79 20 62 65 66 6f 72 65 | 20 72 65 63 65 69 76 69 |y before| receivi|
|00001160| 6e 67 20 63 79 74 6f 74 | 6f 78 69 63 20 74 68 65 |ng cytot|oxic the|
|00001170| 72 61 70 79 2c 20 77 68 | 65 72 65 61 73 0d 0a 73 |rapy, wh|ereas..s|
|00001180| 74 65 72 6f 69 64 2d 72 | 65 73 69 73 74 61 6e 74 |teroid-r|esistant|
|00001190| 20 70 61 74 69 65 6e 74 | 73 20 63 61 6e 20 62 65 | patient|s can be|
|000011a0| 6e 65 66 69 74 20 66 72 | 6f 6d 20 61 20 72 65 6e |nefit fr|om a ren|
|000011b0| 61 6c 20 62 69 6f 70 73 | 79 20 62 65 63 61 75 73 |al biops|y becaus|
|000011c0| 65 20 74 68 65 0d 0a 66 | 69 6e 64 69 6e 67 73 20 |e the..f|indings |
|000011d0| 74 65 6e 64 20 74 6f 20 | 70 72 65 64 69 63 74 20 |tend to |predict |
|000011e0| 74 68 65 20 6f 75 74 63 | 6f 6d 65 2e 20 20 50 61 |the outc|ome. Pa|
|000011f0| 74 69 65 6e 74 73 20 77 | 69 74 68 20 63 6f 6d 62 |tients w|ith comb|
|00001200| 69 6e 65 64 20 73 74 65 | 72 6f 69 64 0d 0a 72 65 |ined ste|roid..re|
|00001210| 73 69 73 74 61 6e 63 65 | 20 61 6e 64 20 66 6f 63 |sistance| and foc|
|00001220| 61 6c 20 67 6c 6f 6d 65 | 72 75 6c 6f 73 63 6c 65 |al glome|ruloscle|
|00001230| 72 6f 73 69 73 20 72 65 | 73 70 6f 6e 64 20 70 6f |rosis re|spond po|
|00001240| 6f 72 6c 79 20 74 6f 20 | 63 79 74 6f 74 6f 78 69 |orly to |cytotoxi|
|00001250| 63 20 74 68 65 72 61 70 | 79 2e 0d 0a 0d 0a 50 6f |c therap|y.....Po|
|00001260| 73 74 2d 43 6f 6d 6d 65 | 6e 74 0d 0a 0d 0a 20 20 |st-Comme|nt.... |
|00001270| 20 20 20 44 72 2e 20 54 | 68 6f 6d 61 73 20 52 2e | Dr. T|homas R.|
|00001280| 20 57 65 6c 63 68 2c 20 | 44 69 72 65 63 74 6f 72 | Welch, |Director|
|00001290| 20 6f 66 20 74 68 65 20 | 44 69 76 69 73 69 6f 6e | of the |Division|
|000012a0| 20 6f 66 20 4e 65 70 68 | 72 6f 6c 6f 67 79 2c 20 | of Neph|rology, |
|000012b0| 43 68 69 6c 64 72 65 6e | 27 73 0d 0a 48 6f 73 70 |Children|'s..Hosp|
|000012c0| 69 74 61 6c 20 4d 65 64 | 69 63 61 6c 20 43 65 6e |ital Med|ical Cen|
|000012d0| 74 65 72 2c 20 61 6e 64 | 20 41 73 73 6f 63 69 61 |ter, and| Associa|
|000012e0| 74 65 20 50 72 6f 66 65 | 73 73 6f 72 20 6f 66 20 |te Profe|ssor of |
|000012f0| 50 65 64 69 61 74 72 69 | 63 73 2c 20 55 6e 69 76 |Pediatri|cs, Univ|
|00001300| 65 72 73 69 74 79 20 6f | 66 0d 0a 43 69 6e 63 69 |ersity o|f..Cinci|
|00001310| 6e 6e 61 74 69 2c 20 63 | 6f 6d 6d 65 6e 74 73 3a |nnati, c|omments:|
|00001320| 0d 0a 0d 0a 50 6f 73 74 | 2d 43 6f 6d 6d 65 6e 74 |....Post|-Comment|
|00001330| 0d 0a 0d 0a 20 20 20 20 | 20 54 68 65 20 61 64 76 |.... | The adv|
|00001340| 65 6e 74 20 6f 66 20 63 | 6f 72 74 69 63 6f 73 74 |ent of c|orticost|
|00001350| 65 72 6f 69 64 20 74 68 | 65 72 61 70 79 20 66 6f |eroid th|erapy fo|
|00001360| 72 20 69 64 69 6f 70 61 | 74 68 69 63 20 6e 65 70 |r idiopa|thic nep|
|00001370| 68 72 6f 74 69 63 20 73 | 79 6e 64 72 6f 6d 65 20 |hrotic s|yndrome |
|00001380| 69 6e 0d 0a 74 68 65 20 | 31 39 35 30 73 20 77 61 |in..the |1950s wa|
|00001390| 73 20 61 20 67 72 65 61 | 74 20 74 68 65 72 61 70 |s a grea|t therap|
|000013a0| 65 75 74 69 63 20 61 64 | 76 61 6e 63 65 20 69 6e |eutic ad|vance in|
|000013b0| 20 6e 65 70 68 72 6f 6c | 6f 67 79 2c 20 61 20 73 | nephrol|ogy, a s|
|000013c0| 75 62 73 70 65 63 69 61 | 6c 74 79 20 6e 6f 74 0d |ubspecia|lty not.|
|000013d0| 0a 62 6c 65 73 73 65 64 | 20 77 69 74 68 20 6d 61 |.blessed| with ma|
|000013e0| 6e 79 20 73 75 63 68 20 | 61 64 76 61 6e 63 65 73 |ny such |advances|
|000013f0| 2e 20 20 4d 6f 72 65 20 | 74 68 61 6e 20 37 35 25 |. More |than 75%|
|00001400| 20 6f 66 20 63 68 69 6c | 64 72 65 6e 20 77 69 74 | of chil|dren wit|
|00001410| 68 20 61 20 6e 65 70 68 | 72 6f 74 69 63 0d 0a 73 |h a neph|rotic..s|
|00001420| 79 6e 64 72 6f 6d 65 20 | 6e 6f 74 20 61 73 73 6f |yndrome |not asso|
|00001430| 63 69 61 74 65 64 20 77 | 69 74 68 20 73 79 73 74 |ciated w|ith syst|
|00001440| 65 6d 69 63 20 64 69 73 | 65 61 73 65 20 6e 6f 77 |emic dis|ease now|
|00001450| 20 63 61 6e 20 62 65 20 | 65 78 70 65 63 74 65 64 | can be |expected|
|00001460| 20 74 6f 20 67 6f 20 69 | 6e 74 6f 0d 0a 72 65 6d | to go i|nto..rem|
|00001470| 69 73 73 69 6f 6e 20 64 | 75 72 69 6e 67 20 61 20 |ission d|uring a |
|00001480| 63 6f 75 72 73 65 20 6f | 66 20 6f 72 61 6c 20 73 |course o|f oral s|
|00001490| 74 65 72 6f 69 64 73 2e | 20 20 53 75 63 68 20 74 |teroids.| Such t|
|000014a0| 68 65 72 61 70 79 20 69 | 73 20 67 65 6e 65 72 61 |herapy i|s genera|
|000014b0| 6c 6c 79 20 67 69 76 65 | 6e 0d 0a 69 6e 20 61 20 |lly give|n..in a |
|000014c0| 64 61 69 6c 79 20 72 65 | 67 69 6d 65 6e 20 75 6e |daily re|gimen un|
|000014d0| 74 69 6c 20 70 72 6f 74 | 65 69 6e 75 72 69 61 20 |til prot|einuria |
|000014e0| 61 62 61 74 65 73 2c 20 | 61 6e 64 20 74 68 65 6e |abates, |and then|
|000014f0| 20 69 73 20 63 6f 6e 74 | 69 6e 75 65 64 20 69 6e | is cont|inued in|
|00001500| 0d 0a 61 6c 74 65 72 6e | 61 74 65 2d 64 61 79 20 |..altern|ate-day |
|00001510| 66 61 73 68 69 6f 6e 20 | 66 6f 72 20 61 20 76 61 |fashion |for a va|
|00001520| 72 69 61 62 6c 65 20 70 | 65 72 69 6f 64 2e 0d 0a |riable p|eriod...|
|00001530| 20 20 20 20 20 46 6f 72 | 20 6d 61 6e 79 20 63 68 | For| many ch|
|00001540| 69 6c 64 72 65 6e 2c 20 | 68 6f 77 65 76 65 72 2c |ildren, |however,|
|00001550| 20 70 72 65 64 6e 69 73 | 6f 6e 65 20 69 73 20 6e | prednis|one is n|
|00001560| 6f 74 20 74 68 65 20 63 | 6f 6d 70 6c 65 74 65 20 |ot the c|omplete |
|00001570| 73 6f 6c 75 74 69 6f 6e | 2e 0d 0a 53 6f 6d 65 20 |solution|...Some |
|00001580| 28 22 66 72 65 71 75 65 | 6e 74 20 72 65 6c 61 70 |("freque|nt relap|
|00001590| 73 65 72 73 22 29 20 68 | 61 76 65 20 63 6f 6d 70 |sers") h|ave comp|
|000015a0| 6c 65 74 65 20 72 65 6d | 69 73 73 69 6f 6e 20 6f |lete rem|ission o|
|000015b0| 66 20 74 68 65 69 72 20 | 64 69 73 65 61 73 65 20 |f their |disease |
|000015c0| 61 66 74 65 72 20 61 0d | 0a 63 6f 75 72 73 65 20 |after a.|.course |
|000015d0| 6f 66 20 73 74 65 72 6f | 69 64 73 20 62 75 74 20 |of stero|ids but |
|000015e0| 68 61 76 65 20 34 20 6f | 72 20 6d 6f 72 65 20 72 |have 4 o|r more r|
|000015f0| 65 6c 61 70 73 65 73 20 | 69 6e 20 61 20 79 65 61 |elapses |in a yea|
|00001600| 72 2e 20 20 41 6e 6f 74 | 68 65 72 20 67 72 6f 75 |r. Anot|her grou|
|00001610| 70 0d 0a 28 22 73 74 65 | 72 6f 69 64 20 64 65 70 |p..("ste|roid dep|
|00001620| 65 6e 64 65 6e 74 22 29 | 20 72 65 73 70 6f 6e 64 |endent")| respond|
|00001630| 73 20 74 6f 20 64 61 69 | 6c 79 20 63 6f 72 74 69 |s to dai|ly corti|
|00001640| 63 6f 73 74 65 72 6f 69 | 64 73 20 62 75 74 20 72 |costeroi|ds but r|
|00001650| 65 6c 61 70 73 65 73 20 | 64 75 72 69 6e 67 20 6f |elapses |during o|
|00001660| 72 0d 0a 6a 75 73 74 20 | 61 66 74 65 72 20 64 69 |r..just |after di|
|00001670| 73 63 6f 6e 74 69 6e 75 | 61 74 69 6f 6e 20 6f 66 |scontinu|ation of|
|00001680| 20 61 6c 74 65 72 6e 61 | 74 65 2d 64 61 79 20 74 | alterna|te-day t|
|00001690| 68 65 72 61 70 79 2e 20 | 20 46 69 6e 61 6c 6c 79 |herapy. | Finally|
|000016a0| 2c 20 61 20 76 65 72 79 | 0d 0a 74 72 6f 75 62 6c |, a very|..troubl|
|000016b0| 65 73 6f 6d 65 20 67 72 | 6f 75 70 20 28 22 73 74 |esome gr|oup ("st|
|000016c0| 65 72 6f 69 64 20 72 65 | 73 69 73 74 61 6e 74 22 |eroid re|sistant"|
|000016d0| 29 20 63 6f 6e 74 69 6e | 75 65 73 20 74 6f 20 68 |) contin|ues to h|
|000016e0| 61 76 65 20 6e 65 70 68 | 72 6f 74 69 63 0d 0a 70 |ave neph|rotic..p|
|000016f0| 72 6f 74 65 69 6e 75 72 | 69 61 20 64 65 73 70 69 |roteinur|ia despi|
|00001700| 74 65 20 32 20 6d 6f 6e | 74 68 73 20 6f 66 20 74 |te 2 mon|ths of t|
|00001710| 72 65 61 74 6d 65 6e 74 | 2e 0d 0a 20 20 20 20 20 |reatment|... |
|00001720| 46 6f 72 20 6d 61 6e 79 | 20 63 68 69 6c 64 72 65 |For many| childre|
|00001730| 6e 20 69 6e 20 74 68 65 | 73 65 20 67 72 6f 75 70 |n in the|se group|
|00001740| 73 20 74 68 65 20 70 72 | 6f 62 6c 65 6d 73 20 6f |s the pr|oblems o|
|00001750| 66 20 6e 65 70 68 72 6f | 74 69 63 20 73 79 6e 64 |f nephro|tic synd|
|00001760| 72 6f 6d 65 20 61 72 65 | 0d 0a 63 6f 6d 70 6f 75 |rome are|..compou|
|00001770| 6e 64 65 64 20 62 79 20 | 74 68 6f 73 65 20 6f 66 |nded by |those of|
|00001780| 20 74 72 65 61 74 6d 65 | 6e 74 3a 20 20 68 79 70 | treatme|nt: hyp|
|00001790| 65 72 74 65 6e 73 69 6f | 6e 2c 20 62 6f 6e 65 20 |ertensio|n, bone |
|000017a0| 6e 65 63 72 6f 73 69 73 | 2c 20 67 72 6f 77 74 68 |necrosis|, growth|
|000017b0| 0d 0a 72 65 74 61 72 64 | 61 74 69 6f 6e 2c 20 63 |..retard|ation, c|
|000017c0| 61 74 61 72 61 63 74 73 | 2c 20 61 6e 64 20 63 75 |ataracts|, and cu|
|000017d0| 73 68 69 6e 67 6f 69 64 | 20 66 61 63 69 65 73 2e |shingoid| facies.|
|000017e0| 20 20 54 68 65 73 65 20 | 63 68 69 6c 64 72 65 6e | These |children|
|000017f0| 20 62 65 63 6f 6d 65 0d | 0a 63 61 6e 64 69 64 61 | become.|.candida|
|00001800| 74 65 73 20 66 6f 72 20 | 61 6c 74 65 72 6e 61 74 |tes for |alternat|
|00001810| 69 76 65 20 74 68 65 72 | 61 70 79 2e 0d 0a 20 20 |ive ther|apy... |
|00001820| 20 20 20 46 6f 72 20 74 | 68 65 20 6d 6f 73 74 20 | For t|he most |
|00001830| 70 61 72 74 2c 20 22 61 | 6c 74 65 72 6e 61 74 69 |part, "a|lternati|
|00001840| 76 65 20 74 68 65 72 61 | 70 79 22 20 6d 65 61 6e |ve thera|py" mean|
|00001850| 73 20 74 68 65 20 75 73 | 65 20 6f 66 20 61 6c 6b |s the us|e of alk|
|00001860| 79 6c 61 74 69 6e 67 0d | 0a 61 67 65 6e 74 73 2c |ylating.|.agents,|
|00001870| 20 67 65 6e 65 72 61 6c | 6c 79 20 63 79 63 6c 6f | general|ly cyclo|
|00001880| 70 68 6f 73 70 68 61 6d | 69 64 65 20 6f 72 20 63 |phospham|ide or c|
|00001890| 68 6c 6f 72 61 6d 62 75 | 63 69 6c 2e 20 20 54 68 |hlorambu|cil. Th|
|000018a0| 65 73 65 20 64 72 75 67 | 73 20 63 61 72 72 79 0d |ese drug|s carry.|
|000018b0| 0a 73 75 62 73 74 61 6e | 74 69 61 6c 20 74 6f 78 |.substan|tial tox|
|000018c0| 69 63 69 74 79 20 6f 66 | 20 74 68 65 69 72 20 6f |icity of| their o|
|000018d0| 77 6e 3a 20 20 74 68 65 | 20 72 69 73 6b 20 6f 66 |wn: the| risk of|
|000018e0| 20 70 72 6f 66 6f 75 6e | 64 20 6e 65 75 74 72 6f | profoun|d neutro|
|000018f0| 70 65 6e 69 61 2c 0d 0a | 73 74 65 72 69 6c 69 74 |penia,..|sterilit|
|00001900| 79 2c 20 61 6e 64 20 6d | 61 6c 69 67 6e 61 6e 63 |y, and m|alignanc|
|00001910| 69 65 73 2e 20 20 4e 6f | 74 20 61 6c 6c 20 63 68 |ies. No|t all ch|
|00001920| 69 6c 64 72 65 6e 20 77 | 69 74 68 20 6e 65 70 68 |ildren w|ith neph|
|00001930| 72 6f 74 69 63 20 73 79 | 6e 64 72 6f 6d 65 20 69 |rotic sy|ndrome i|
|00001940| 6d 70 72 6f 76 65 0d 0a | 77 69 74 68 20 74 68 69 |mprove..|with thi|
|00001950| 73 20 74 68 65 72 61 70 | 79 2c 20 68 6f 77 65 76 |s therap|y, howev|
|00001960| 65 72 2e 20 20 49 74 20 | 77 6f 75 6c 64 20 62 65 |er. It |would be|
|00001970| 20 69 64 65 61 6c 2c 20 | 74 68 65 72 65 66 6f 72 | ideal, |therefor|
|00001980| 65 2c 20 69 66 20 70 6f | 74 65 6e 74 69 61 6c 0d |e, if po|tential.|
|00001990| 0a 6e 6f 6e 72 65 73 70 | 6f 6e 64 65 72 73 20 63 |.nonresp|onders c|
|000019a0| 6f 75 6c 64 20 62 65 20 | 69 64 65 6e 74 69 66 69 |ould be |identifi|
|000019b0| 65 64 20 73 6f 20 74 68 | 61 74 20 74 68 65 79 20 |ed so th|at they |
|000019c0| 63 6f 75 6c 64 20 62 65 | 20 73 70 61 72 65 64 20 |could be| spared |
|000019d0| 74 68 65 20 61 64 64 69 | 74 69 6f 6e 61 6c 0d 0a |the addi|tional..|
|000019e0| 6d 6f 72 62 69 64 69 74 | 79 20 6f 66 20 63 79 74 |morbidit|y of cyt|
|000019f0| 6f 74 6f 78 69 63 20 64 | 72 75 67 73 2e 0d 0a 20 |otoxic d|rugs... |
|00001a00| 20 20 20 20 49 6e 20 6d | 61 6e 79 20 63 65 6e 74 | In m|any cent|
|00001a10| 65 72 73 20 63 61 6e 64 | 69 64 61 74 65 73 20 66 |ers cand|idates f|
|00001a20| 6f 72 20 63 79 74 6f 74 | 6f 78 69 63 20 64 72 75 |or cytot|oxic dru|
|00001a30| 67 73 20 75 6e 64 65 72 | 67 6f 20 72 65 6e 61 6c |gs under|go renal|
|00001a40| 20 62 69 6f 70 73 79 2e | 20 20 54 68 65 0d 0a 62 | biopsy.| The..b|
|00001a50| 61 73 69 73 20 66 6f 72 | 20 74 68 69 73 20 61 70 |asis for| this ap|
|00001a60| 70 72 6f 61 63 68 20 69 | 73 20 74 68 65 20 62 65 |proach i|s the be|
|00001a70| 6c 69 65 66 20 74 68 61 | 74 20 72 65 6e 61 6c 20 |lief tha|t renal |
|00001a80| 68 69 73 74 6f 6c 6f 67 | 79 20 69 73 20 70 72 65 |histolog|y is pre|
|00001a90| 64 69 63 74 69 76 65 20 | 6f 66 0d 0a 74 68 65 20 |dictive |of..the |
|00001aa0| 72 65 73 70 6f 6e 73 65 | 20 74 6f 20 61 6c 6b 79 |response| to alky|
|00001ab0| 6c 61 74 69 6e 67 20 61 | 67 65 6e 74 20 74 68 65 |lating a|gent the|
|00001ac0| 72 61 70 79 2e 20 20 54 | 68 69 73 20 73 74 75 64 |rapy. T|his stud|
|00001ad0| 79 20 62 79 20 53 63 68 | 75 6c 6d 61 6e 20 61 6e |y by Sch|ulman an|
|00001ae0| 64 0d 0a 61 73 73 6f 63 | 69 61 74 65 73 2c 20 68 |d..assoc|iates, h|
|00001af0| 6f 77 65 76 65 72 2c 20 | 72 65 6d 69 6e 64 73 20 |owever, |reminds |
|00001b00| 75 73 20 74 68 61 74 20 | 74 68 65 20 63 6f 72 72 |us that |the corr|
|00001b10| 65 6c 61 74 69 6f 6e 20 | 62 65 74 77 65 65 6e 20 |elation |between |
|00001b20| 67 6c 6f 6d 65 72 75 6c | 61 72 0d 0a 6d 6f 72 70 |glomerul|ar..morp|
|00001b30| 68 6f 6c 6f 67 79 20 61 | 6e 64 20 63 6c 69 6e 69 |hology a|nd clini|
|00001b40| 63 61 6c 20 63 6f 75 72 | 73 65 73 20 69 73 20 69 |cal cour|ses is i|
|00001b50| 6d 70 65 72 66 65 63 74 | 2e 20 20 43 68 69 6c 64 |mperfect|. Child|
|00001b60| 72 65 6e 20 77 69 74 68 | 20 61 20 6e 65 70 68 72 |ren with| a nephr|
|00001b70| 6f 74 69 63 0d 0a 73 79 | 6e 64 72 6f 6d 65 20 73 |otic..sy|ndrome s|
|00001b80| 65 6e 73 69 74 69 76 65 | 20 74 6f 20 63 6f 72 74 |ensitive| to cort|
|00001b90| 69 63 6f 73 74 65 72 6f | 69 64 73 20 68 61 76 65 |icostero|ids have|
|00001ba0| 20 61 20 67 6f 6f 64 20 | 63 68 61 6e 63 65 20 6f | a good |chance o|
|00001bb0| 66 20 73 75 73 74 61 69 | 6e 69 6e 67 20 61 0d 0a |f sustai|ning a..|
|00001bc0| 70 72 6f 6c 6f 6e 67 65 | 64 20 70 72 65 64 6e 69 |prolonge|d predni|
|00001bd0| 73 6f 6e 65 2d 66 72 65 | 65 20 72 65 6d 69 73 73 |sone-fre|e remiss|
|00001be0| 69 6f 6e 20 77 69 74 68 | 20 63 79 74 6f 74 6f 78 |ion with| cytotox|
|00001bf0| 69 63 20 74 68 65 72 61 | 70 79 2c 20 72 65 67 61 |ic thera|py, rega|
|00001c00| 72 64 6c 65 73 73 20 6f | 66 0d 0a 68 69 73 74 6f |rdless o|f..histo|
|00001c10| 6c 6f 67 79 2e 0d 0a 20 | 20 20 20 20 41 20 6d 61 |logy... | A ma|
|00001c20| 6a 6f 72 20 72 65 6d 61 | 69 6e 69 6e 67 20 74 68 |jor rema|ining th|
|00001c30| 65 72 61 70 65 75 74 69 | 63 20 70 72 6f 62 6c 65 |erapeuti|c proble|
|00001c40| 6d 20 69 73 20 74 68 65 | 20 61 70 70 72 6f 61 63 |m is the| approac|
|00001c50| 68 20 74 6f 20 63 68 69 | 6c 64 72 65 6e 20 77 69 |h to chi|ldren wi|
|00001c60| 74 68 0d 0a 73 74 65 72 | 6f 69 64 2d 72 65 73 69 |th..ster|oid-resi|
|00001c70| 73 74 61 6e 74 20 6e 65 | 70 68 72 6f 74 69 63 20 |stant ne|phrotic |
|00001c80| 73 79 6e 64 72 6f 6d 65 | 2c 20 6d 6f 73 74 20 6f |syndrome|, most o|
|00001c90| 66 20 77 68 6f 6d 20 68 | 61 76 65 20 66 6f 63 61 |f whom h|ave foca|
|00001ca0| 6c 20 73 65 67 6d 65 6e | 74 61 6c 0d 0a 67 6c 6f |l segmen|tal..glo|
|00001cb0| 6d 65 72 75 6c 6f 73 63 | 6c 65 72 6f 73 69 73 2e |merulosc|lerosis.|
|00001cc0| 20 20 4d 6f 73 74 20 6f | 66 20 74 68 65 73 65 20 | Most o|f these |
|00001cd0| 63 68 69 6c 64 72 65 6e | 20 68 61 76 65 20 70 65 |children| have pe|
|00001ce0| 72 73 69 73 74 65 6e 74 | 20 6e 65 70 68 72 6f 73 |rsistent| nephros|
|00001cf0| 69 73 20 61 6e 64 0d 0a | 70 72 6f 67 72 65 73 73 |is and..|progress|
|00001d00| 20 74 6f 20 65 6e 64 2d | 73 74 61 67 65 20 72 65 | to end-|stage re|
|00001d10| 6e 61 6c 20 64 69 73 65 | 61 73 65 2e 20 20 54 68 |nal dise|ase. Th|
|00001d20| 65 72 65 20 69 73 20 61 | 73 20 79 65 74 20 6e 6f |ere is a|s yet no|
|00001d30| 20 70 72 6f 76 65 6e 20 | 74 72 65 61 74 6d 65 6e | proven |treatmen|
|00001d40| 74 20 66 6f 72 0d 0a 73 | 75 63 68 20 70 61 74 69 |t for..s|uch pati|
|00001d50| 65 6e 74 73 2e 20 20 41 | 20 72 65 70 6f 72 74 20 |ents. A| report |
|00001d60| 62 79 20 54 65 6a 61 6e | 69 20 61 6e 64 20 61 73 |by Tejan|i and as|
|00001d70| 73 6f 63 69 61 74 65 73 | 20 28 31 29 20 73 75 67 |sociates| (1) sug|
|00001d80| 67 65 73 74 73 20 61 20 | 72 6f 6c 65 20 66 6f 72 |gests a |role for|
|00001d90| 0d 0a 63 79 63 6c 6f 73 | 70 6f 72 69 6e 65 20 69 |..cyclos|porine i|
|00001da0| 6e 20 74 68 65 73 65 20 | 63 68 69 6c 64 72 65 6e |n these |children|
|00001db0| 2c 20 77 68 65 72 65 61 | 73 20 47 72 69 73 77 6f |, wherea|s Griswo|
|00001dc0| 6c 64 20 61 6e 64 20 63 | 6f 2d 77 6f 72 6b 65 72 |ld and c|o-worker|
|00001dd0| 73 20 28 32 29 20 72 65 | 70 6f 72 74 0d 0a 73 75 |s (2) re|port..su|
|00001de0| 63 63 65 73 73 20 77 69 | 74 68 20 70 75 6c 73 65 |ccess wi|th pulse|
|00001df0| 20 6d 65 74 68 79 6c 70 | 72 65 64 6e 69 73 6f 6c | methylp|rednisol|
|00001e00| 6f 6e 65 2e 20 20 4e 6f | 6e 65 20 6f 66 20 74 68 |one. No|ne of th|
|00001e10| 65 73 65 20 74 68 65 72 | 61 70 69 65 73 20 63 61 |ese ther|apies ca|
|00001e20| 6e 20 61 73 20 79 65 74 | 20 62 65 0d 0a 63 6f 6e |n as yet| be..con|
|00001e30| 73 69 64 65 72 65 64 20 | 73 74 61 6e 64 61 72 64 |sidered |standard|
|00001e40| 2e 20 20 57 65 20 61 6e | 78 69 6f 75 73 6c 79 20 |. We an|xiously |
|00001e50| 61 77 61 69 74 20 66 75 | 72 74 68 65 72 20 64 65 |await fu|rther de|
|00001e60| 76 65 6c 6f 70 6d 65 6e | 74 73 20 69 6e 20 74 68 |velopmen|ts in th|
|00001e70| 69 73 0d 0a 69 6d 70 6f | 72 74 61 6e 74 20 61 72 |is..impo|rtant ar|
|00001e80| 65 61 2e 20 20 49 6e 20 | 74 68 65 20 6d 65 61 6e |ea. In |the mean|
|00001e90| 74 69 6d 65 2c 20 64 6f | 6e 27 74 20 66 6f 72 67 |time, do|n't forg|
|00001ea0| 65 74 20 74 68 61 74 20 | 68 65 61 64 2d 6f 75 74 |et that |head-out|
|00001eb0| 20 77 61 74 65 72 20 69 | 6d 6d 65 72 73 69 6f 6e | water i|mmersion|
|00001ec0| 0d 0a 69 73 20 61 6e 20 | 65 66 66 65 63 74 69 76 |..is an |effectiv|
|00001ed0| 65 20 64 69 75 72 65 74 | 69 63 20 6d 61 6e 65 75 |e diuret|ic maneu|
|00001ee0| 76 65 72 20 69 6e 20 6e | 65 70 68 72 6f 74 69 63 |ver in n|ephrotic|
|00001ef0| 20 73 79 6e 64 72 6f 6d | 65 20 28 33 29 21 2d 2d | syndrom|e (3)!--|
|00001f00| 54 2e 52 2e 20 57 65 6c | 63 68 2c 20 4d 2e 44 2e |T.R. Wel|ch, M.D.|
|00001f10| 0d 0a 24 55 6e 69 71 75 | 65 5f 49 44 7b 59 52 42 |..$Uniqu|e_ID{YRB|
|00001f20| 31 32 30 30 31 7d 0d 0a | 24 54 69 74 6c 65 7b 4c |12001}..|$Title{L|
|00001f30| 69 6e 6b 61 67 65 20 48 | 65 74 65 72 6f 67 65 6e |inkage H|eterogen|
|00001f40| 65 69 74 79 20 6f 66 20 | 41 75 74 6f 73 6f 6d 61 |eity of |Autosoma|
|00001f50| 6c 20 44 6f 6d 69 6e 61 | 6e 74 20 50 6f 6c 79 63 |l Domina|nt Polyc|
|00001f60| 79 73 74 69 63 20 4b 69 | 64 6e 65 79 20 44 69 73 |ystic Ki|dney Dis|
|00001f70| 65 61 73 65 7d 0d 0a 24 | 41 75 74 68 6f 72 7b 4b |ease}..$|Author{K|
|00001f80| 69 6d 62 65 72 6c 69 6e | 67 2c 20 57 4a 0d 0a 46 |imberlin|g, WJ..F|
|00001f90| 61 69 6e 2c 20 50 52 0d | 0a 4b 65 6e 79 6f 6e 2c |ain, PR.|.Kenyon,|
|00001fa0| 20 4a 42 0d 0a 47 6f 6c | 64 67 61 72 2c 20 44 0d | JB..Gol|dgar, D.|
|00001fb0| 0a 53 75 6a 61 6e 73 6b | 79 2c 20 45 0d 0a 47 61 |.Sujansk|y, E..Ga|
|00001fc0| 62 6f 77 2c 20 50 41 7d | 0d 0a 24 41 66 66 69 6c |bow, PA}|..$Affil|
|00001fd0| 69 61 74 69 6f 6e 7b 43 | 72 65 69 67 68 74 6f 6e |iation{C|reighton|
|00001fe0| 20 55 6e 69 76 2c 20 4f | 6d 61 68 61 3b 20 55 6e | Univ, O|maha; Un|
|00001ff0| 69 76 20 6f 66 20 55 74 | 61 68 3b 20 55 6e 69 76 |iv of Ut|ah; Univ|
|00002000| 20 6f 66 20 43 6f 6c 6f | 72 61 64 6f 7d 0d 0a 24 | of Colo|rado}..$|
|00002010| 4a 6f 75 72 6e 61 6c 7b | 4e 20 45 6e 67 6c 20 4a |Journal{|N Engl J|
|00002020| 20 4d 65 64 7d 0d 0a 24 | 56 6f 6c 75 6d 65 7b 33 | Med}..$|Volume{3|
|00002030| 31 39 7d 0d 0a 24 50 61 | 67 65 7b 39 31 33 2d 39 |19}..$Pa|ge{913-9|
|00002040| 31 38 7d 0d 0a 24 44 61 | 74 65 7b 4f 63 74 20 36 |18}..$Da|te{Oct 6|
|00002050| 2c 20 31 39 38 38 7d 0d | 0a 24 59 42 56 6f 6c 7b |, 1988}.|.$YBVol{|
|00002060| 31 39 39 30 20 59 65 61 | 72 20 42 6f 6f 6b 20 6f |1990 Yea|r Book o|
|00002070| 66 20 50 65 64 69 61 74 | 72 69 63 73 7d 0d 0a 24 |f Pediat|rics}..$|
|00002080| 43 68 61 70 74 65 72 7b | 31 30 2d 31 31 7d 0d 0a |Chapter{|10-11}..|
|00002090| 24 59 42 45 64 69 74 6f | 72 7b 44 72 73 2e 20 4f |$YBEdito|r{Drs. O|
|000020a0| 73 6b 69 20 61 6e 64 20 | 53 74 6f 63 6b 6d 61 6e |ski and |Stockman|
|000020b0| 7d 0d 0a 24 54 61 62 6c | 65 7b 7d 0d 0a 24 43 6f |}..$Tabl|e{}..$Co|
|000020c0| 6d 6d 65 6e 74 61 74 6f | 72 7b 4a 2e 41 2e 20 53 |mmentato|r{J.A. S|
|000020d0| 74 6f 63 6b 6d 61 6e 2c | 20 49 49 49 2c 20 4d 2e |tockman,| III, M.|
|000020e0| 44 2e 7d 0d 0a 0d 0a 43 | 6f 70 79 72 69 67 68 74 |D.}....C|opyright|
|000020f0| 20 28 63 29 20 59 65 61 | 72 62 6f 6f 6b 20 4d 65 | (c) Yea|rbook Me|
|00002100| 64 69 63 61 6c 20 50 75 | 62 6c 69 73 68 65 72 73 |dical Pu|blishers|
|00002110| 2c 20 49 6e 63 2e 2c 20 | 31 39 39 30 0d 0a 0d 0a |, Inc., |1990....|
|00002120| 31 39 39 30 20 59 65 61 | 72 20 42 6f 6f 6b 20 6f |1990 Yea|r Book o|
|00002130| 66 20 50 65 64 69 61 74 | 72 69 63 73 20 28 52 29 |f Pediat|rics (R)|
|00002140| 0d 0a 0d 0a 43 68 61 70 | 74 65 72 20 31 30 2d 2d |....Chap|ter 10--|
|00002150| 54 68 65 20 47 65 6e 69 | 74 6f 75 72 69 6e 61 72 |The Geni|tourinar|
|00002160| 79 20 54 72 61 63 74 0d | 0a 0d 0a 2d 2d 2d 2d 2d |y Tract.|...-----|
|00002170| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00002180| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00002190| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|000021a0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|000021b0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 0d 0a 4c 69 6e 6b 61 |--------|-..Linka|
|000021c0| 67 65 20 48 65 74 65 72 | 6f 67 65 6e 65 69 74 79 |ge Heter|ogeneity|
|000021d0| 20 6f 66 20 41 75 74 6f | 73 6f 6d 61 6c 20 44 6f | of Auto|somal Do|
|000021e0| 6d 69 6e 61 6e 74 20 50 | 6f 6c 79 63 79 73 74 69 |minant P|olycysti|
|000021f0| 63 20 4b 69 64 6e 65 79 | 20 44 69 73 65 61 73 65 |c Kidney| Disease|
|00002200| 0d 0a 0d 0a 4b 69 6d 62 | 65 72 6c 69 6e 67 20 57 |....Kimb|erling W|
|00002210| 4a 2c 20 46 61 69 6e 20 | 50 52 2c 20 4b 65 6e 79 |J, Fain |PR, Keny|
|00002220| 6f 6e 20 4a 42 2c 20 47 | 6f 6c 64 67 61 72 20 44 |on JB, G|oldgar D|
|00002230| 2c 20 53 75 6a 61 6e 73 | 6b 79 20 45 2c 20 47 61 |, Sujans|ky E, Ga|
|00002240| 62 6f 77 20 50 41 0d 0a | 28 43 72 65 69 67 68 74 |bow PA..|(Creight|
|00002250| 6f 6e 20 55 6e 69 76 2c | 20 4f 6d 61 68 61 3b 20 |on Univ,| Omaha; |
|00002260| 55 6e 69 76 20 6f 66 20 | 55 74 61 68 3b 20 55 6e |Univ of |Utah; Un|
|00002270| 69 76 20 6f 66 20 43 6f | 6c 6f 72 61 64 6f 29 0d |iv of Co|lorado).|
|00002280| 0a 4e 20 45 6e 67 6c 20 | 4a 20 4d 65 64 20 33 31 |.N Engl |J Med 31|
|00002290| 39 3a 39 31 33 2d 39 31 | 38 2c 20 4f 63 74 20 36 |9:913-91|8, Oct 6|
|000022a0| 2c 20 31 39 38 38 0d 0a | 2d 2d 2d 2d 2d 2d 2d 2d |, 1988..|--------|
|000022b0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|000022c0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|000022d0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|000022e0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|000022f0| 2d 2d 2d 2d 2d 2d 0d 0a | 0d 0a 41 62 73 74 72 61 |------..|..Abstra|
|00002300| 63 74 0d 0a 0d 0a 20 20 | 20 20 20 41 75 74 6f 73 |ct.... | Autos|
|00002310| 6f 6d 61 6c 20 64 6f 6d | 69 6e 61 6e 74 20 70 6f |omal dom|inant po|
|00002320| 6c 79 63 79 73 74 69 63 | 20 6b 69 64 6e 65 79 20 |lycystic| kidney |
|00002330| 64 69 73 65 61 73 65 20 | 69 73 20 61 20 63 6f 6d |disease |is a com|
|00002340| 6d 6f 6e 20 68 65 72 65 | 64 69 74 61 72 79 0d 0a |mon here|ditary..|
|00002350| 64 69 73 65 61 73 65 20 | 74 68 61 74 20 61 63 63 |disease |that acc|
|00002360| 6f 75 6e 74 73 20 66 6f | 72 20 61 62 6f 75 74 20 |ounts fo|r about |
|00002370| 31 32 25 20 6f 66 20 61 | 6c 6c 20 70 61 74 69 65 |12% of a|ll patie|
|00002380| 6e 74 73 20 77 69 74 68 | 20 65 6e 64 2d 73 74 61 |nts with| end-sta|
|00002390| 67 65 20 72 65 6e 61 6c | 0d 0a 64 69 73 65 61 73 |ge renal|..diseas|
|000023a0| 65 20 69 6e 20 74 68 65 | 20 55 6e 69 74 65 64 20 |e in the| United |
|000023b0| 53 74 61 74 65 73 2e 20 | 20 49 74 20 69 73 20 63 |States. | It is c|
|000023c0| 68 61 72 61 63 74 65 72 | 69 7a 65 64 20 62 79 20 |haracter|ized by |
|000023d0| 6d 75 6c 74 69 70 6c 65 | 20 72 65 6e 61 6c 20 63 |multiple| renal c|
|000023e0| 79 73 74 73 0d 0a 74 68 | 61 74 20 69 6e 63 72 65 |ysts..th|at incre|
|000023f0| 61 73 65 20 69 6e 20 6e | 75 6d 62 65 72 20 61 6e |ase in n|umber an|
|00002400| 64 20 73 69 7a 65 20 77 | 69 74 68 20 61 67 65 2e |d size w|ith age.|
|00002410| 20 20 50 6f 6c 79 63 79 | 73 74 69 63 20 6b 69 64 | Polycy|stic kid|
|00002420| 6e 65 79 20 64 69 73 65 | 61 73 65 20 69 73 0d 0a |ney dise|ase is..|
|00002430| 61 63 74 75 61 6c 6c 79 | 20 61 20 6d 75 6c 74 69 |actually| a multi|
|00002440| 73 79 73 74 65 6d 20 64 | 69 73 6f 72 64 65 72 20 |system d|isorder |
|00002450| 77 69 74 68 20 63 79 73 | 74 20 66 6f 72 6d 61 74 |with cys|t format|
|00002460| 69 6f 6e 20 69 6e 20 74 | 68 65 20 6c 69 76 65 72 |ion in t|he liver|
|00002470| 2c 20 70 61 6e 63 72 65 | 61 73 2c 0d 0a 73 70 6c |, pancre|as,..spl|
|00002480| 65 65 6e 2c 20 6f 76 61 | 72 69 65 73 2c 20 61 6e |een, ova|ries, an|
|00002490| 64 20 74 65 73 74 65 73 | 2e 20 20 49 74 20 69 73 |d testes|. It is|
|000024a0| 20 6c 69 6e 6b 65 64 20 | 74 6f 20 32 20 6d 61 72 | linked |to 2 mar|
|000024b0| 6b 65 72 73 20 6f 6e 20 | 74 68 65 20 73 68 6f 72 |kers on |the shor|
|000024c0| 74 20 61 72 6d 20 6f 66 | 0d 0a 63 68 72 6f 6d 6f |t arm of|..chromo|
|000024d0| 73 6f 6d 65 20 31 36 2c | 20 6e 61 6d 65 6c 79 2c |some 16,| namely,|
|000024e0| 20 74 68 65 20 61 6c 70 | 68 61 2d 68 65 6d 6f 67 | the alp|ha-hemog|
|000024f0| 6c 6f 62 69 6e 20 67 65 | 6e 65 20 63 6f 6d 70 6c |lobin ge|ne compl|
|00002500| 65 78 20 61 6e 64 20 70 | 68 6f 73 70 68 6f 67 6c |ex and p|hosphogl|
|00002510| 79 63 65 72 61 74 65 0d | 0a 6b 69 6e 61 73 65 2e |ycerate.|.kinase.|
|00002520| 20 20 41 20 35 2d 67 65 | 6e 65 72 61 74 69 6f 6e | A 5-ge|neration|
|00002530| 20 6b 69 6e 64 72 65 64 | 20 6f 66 20 64 65 73 63 | kindred| of desc|
|00002540| 65 6e 64 61 6e 74 73 20 | 6f 66 20 53 69 63 69 6c |endants |of Sicil|
|00002550| 69 61 6e 20 69 6d 6d 69 | 67 72 61 6e 74 73 20 77 |ian immi|grants w|
|00002560| 61 73 0d 0a 6f 62 73 65 | 72 76 65 64 20 69 6e 20 |as..obse|rved in |
|00002570| 77 68 6f 6d 20 74 68 65 | 20 64 69 73 65 61 73 65 |whom the| disease|
|00002580| 20 77 61 73 20 70 72 65 | 73 65 6e 74 20 77 69 74 | was pre|sent wit|
|00002590| 68 6f 75 74 20 6c 69 6e | 6b 61 67 65 20 74 6f 20 |hout lin|kage to |
|000025a0| 74 68 65 0d 0a 61 6c 70 | 68 61 2d 68 65 6d 6f 67 |the..alp|ha-hemog|
|000025b0| 6c 6f 62 69 6e 20 63 6f | 6d 70 6c 65 78 2e 0d 0a |lobin co|mplex...|
|000025c0| 20 20 20 20 20 54 68 65 | 20 66 61 6d 69 6c 79 20 | The| family |
|000025d0| 77 61 73 20 61 20 6c 61 | 72 67 65 20 6b 69 6e 64 |was a la|rge kind|
|000025e0| 72 65 64 20 6f 66 20 6d | 6f 72 65 20 74 68 61 6e |red of m|ore than|
|000025f0| 20 32 35 30 20 6d 65 6d | 62 65 72 73 2e 20 20 4d | 250 mem|bers. M|
|00002600| 6f 73 74 20 6c 69 76 69 | 6e 67 0d 0a 66 61 6d 69 |ost livi|ng..fami|
|00002610| 6c 79 20 6d 65 6d 62 65 | 72 73 20 75 6e 64 65 72 |ly membe|rs under|
|00002620| 77 65 6e 74 20 61 62 64 | 6f 6d 69 6e 61 6c 20 75 |went abd|ominal u|
|00002630| 6c 74 72 61 73 6f 6e 6f | 67 72 61 70 68 79 20 61 |ltrasono|graphy a|
|00002640| 6e 64 20 61 20 63 6f 6d | 70 6c 65 74 65 20 63 6c |nd a com|plete cl|
|00002650| 69 6e 69 63 61 6c 0d 0a | 65 76 61 6c 75 61 74 69 |inical..|evaluati|
|00002660| 6f 6e 20 74 68 61 74 20 | 69 6e 63 6c 75 64 65 64 |on that |included|
|00002670| 20 68 69 73 74 6f 72 79 | 2c 20 70 68 79 73 69 63 | history|, physic|
|00002680| 61 6c 20 65 78 61 6d 69 | 6e 61 74 69 6f 6e 2c 20 |al exami|nation, |
|00002690| 61 6e 64 20 72 6f 75 74 | 69 6e 65 20 6c 61 62 6f |and rout|ine labo|
|000026a0| 72 61 74 6f 72 79 0d 0a | 74 65 73 74 69 6e 67 20 |ratory..|testing |
|000026b0| 6f 66 20 72 65 6e 61 6c | 20 61 6e 64 20 6c 69 76 |of renal| and liv|
|000026c0| 65 72 20 66 75 6e 63 74 | 69 6f 6e 2e 20 20 46 6f |er funct|ion. Fo|
|000026d0| 72 20 74 68 65 20 44 4e | 41 20 6c 69 6e 6b 61 67 |r the DN|A linkag|
|000026e0| 65 20 73 74 75 64 69 65 | 73 2c 20 62 6c 6f 6f 64 |e studie|s, blood|
|000026f0| 0d 0a 73 61 6d 70 6c 65 | 73 20 77 65 72 65 20 6f |..sample|s were o|
|00002700| 62 74 61 69 6e 65 64 20 | 66 72 6f 6d 20 31 36 33 |btained |from 163|
|00002710| 20 66 61 6d 69 6c 79 20 | 6d 65 6d 62 65 72 73 2e | family |members.|
|00002720| 0d 0a 20 20 20 20 20 54 | 68 65 20 72 65 73 75 6c |.. T|he resul|
|00002730| 74 73 20 6f 66 20 44 4e | 41 20 70 72 6f 62 69 6e |ts of DN|A probin|
|00002740| 67 20 73 68 6f 77 65 64 | 20 74 68 61 74 20 37 31 |g showed| that 71|
|00002750| 20 6f 66 20 74 68 65 20 | 31 36 33 20 65 78 61 6d | of the |163 exam|
|00002760| 69 6e 65 64 20 66 61 6d | 69 6c 79 0d 0a 6d 65 6d |ined fam|ily..mem|
|00002770| 62 65 72 73 20 77 65 72 | 65 20 65 69 74 68 65 72 |bers wer|e either|
|00002780| 20 61 66 66 65 63 74 65 | 64 20 62 79 20 6f 72 20 | affecte|d by or |
|00002790| 61 74 20 72 69 73 6b 20 | 66 6f 72 20 61 75 74 6f |at risk |for auto|
|000027a0| 73 6f 6d 61 6c 20 64 6f | 6d 69 6e 61 6e 74 20 70 |somal do|minant p|
|000027b0| 6f 6c 79 63 79 73 74 69 | 63 0d 0a 6b 69 64 6e 65 |olycysti|c..kidne|
|000027c0| 79 20 64 69 73 65 61 73 | 65 2e 20 20 41 6c 6c 20 |y diseas|e. All |
|000027d0| 64 69 61 67 6e 6f 73 65 | 73 20 77 65 72 65 20 63 |diagnose|s were c|
|000027e0| 6f 6e 66 69 72 6d 65 64 | 20 62 79 20 75 6c 74 72 |onfirmed| by ultr|
|000027f0| 61 73 6f 75 6e 64 20 65 | 78 61 6d 69 6e 61 74 69 |asound e|xaminati|
|00002800| 6f 6e 2e 20 20 54 68 65 | 0d 0a 66 72 65 71 75 65 |on. The|..freque|
|00002810| 6e 63 79 20 6f 66 20 72 | 65 63 6f 6d 62 69 6e 61 |ncy of r|ecombina|
|00002820| 74 69 6f 6e 20 62 65 74 | 77 65 65 6e 20 74 68 65 |tion bet|ween the|
|00002830| 20 61 6c 70 68 61 2d 68 | 65 6d 6f 67 6c 6f 62 69 | alpha-h|emoglobi|
|00002840| 6e 20 63 6f 6d 70 6c 65 | 78 20 61 6e 64 20 74 68 |n comple|x and th|
|00002850| 65 20 72 65 67 69 6f 6e | 0d 0a 73 68 6f 77 6e 20 |e region|..shown |
|00002860| 69 6e 20 70 72 65 76 69 | 6f 75 73 20 73 74 75 64 |in previ|ous stud|
|00002870| 69 65 73 20 74 6f 20 63 | 6f 6e 74 61 69 6e 20 74 |ies to c|ontain t|
|00002880| 68 65 20 6d 75 74 61 74 | 69 6f 6e 20 74 68 61 74 |he mutat|ion that|
|00002890| 20 63 61 75 73 65 73 20 | 70 6f 6c 79 63 79 73 74 | causes |polycyst|
|000028a0| 69 63 0d 0a 6b 69 64 6e | 65 79 20 64 69 73 65 61 |ic..kidn|ey disea|
|000028b0| 73 65 20 65 78 63 65 65 | 64 65 64 20 32 34 25 2c |se excee|ded 24%,|
|000028c0| 20 69 6e 64 69 63 61 74 | 69 6e 67 20 61 20 6d 75 | indicat|ing a mu|
|000028d0| 74 61 74 69 6f 6e 20 61 | 74 20 61 20 64 69 66 66 |tation a|t a diff|
|000028e0| 65 72 65 6e 74 20 6c 6f | 63 75 73 2e 0d 0a 48 6f |erent lo|cus...Ho|
|000028f0| 77 65 76 65 72 2c 20 74 | 68 65 20 63 6c 69 6e 69 |wever, t|he clini|
|00002900| 63 61 6c 20 66 69 6e 64 | 69 6e 67 73 20 69 6e 20 |cal find|ings in |
|00002910| 74 68 69 73 20 66 61 6d | 69 6c 79 20 77 65 72 65 |this fam|ily were|
|00002920| 20 69 6e 64 69 73 74 69 | 6e 67 75 69 73 68 61 62 | indisti|nguishab|
|00002930| 6c 65 20 66 72 6f 6d 0d | 0a 74 68 6f 73 65 20 69 |le from.|.those i|
|00002940| 6e 20 6f 74 68 65 72 20 | 6b 69 6e 64 72 65 64 73 |n other |kindreds|
|00002950| 20 77 69 74 68 20 70 6f | 6c 79 63 79 73 74 69 63 | with po|lycystic|
|00002960| 20 6b 69 64 6e 65 79 20 | 64 69 73 65 61 73 65 2e | kidney |disease.|
|00002970| 0d 0a 20 20 20 20 20 54 | 68 65 72 65 20 6d 61 79 |.. T|here may|
|00002980| 20 62 65 20 61 20 73 65 | 63 6f 6e 64 20 67 65 6e | be a se|cond gen|
|00002990| 65 20 66 6f 72 20 61 75 | 74 6f 73 6f 6d 61 6c 20 |e for au|tosomal |
|000029a0| 64 6f 6d 69 6e 61 6e 74 | 20 70 6f 6c 79 63 79 73 |dominant| polycys|
|000029b0| 74 69 63 20 6b 69 64 6e | 65 79 0d 0a 64 69 73 65 |tic kidn|ey..dise|
|000029c0| 61 73 65 2e 20 20 48 65 | 74 65 72 6f 67 65 6e 65 |ase. He|terogene|
|000029d0| 69 74 79 20 6f 66 20 70 | 6f 6c 79 63 79 73 74 69 |ity of p|olycysti|
|000029e0| 63 20 6b 69 64 6e 65 79 | 20 64 69 73 65 61 73 65 |c kidney| disease|
|000029f0| 20 63 6f 75 6c 64 20 70 | 6f 73 65 20 70 72 6f 62 | could p|ose prob|
|00002a00| 6c 65 6d 73 20 66 6f 72 | 0d 0a 72 65 73 65 61 72 |lems for|..resear|
|00002a10| 63 68 65 72 73 20 61 6e | 64 20 63 6c 69 6e 69 63 |chers an|d clinic|
|00002a20| 69 61 6e 73 20 69 6e 20 | 74 68 61 74 20 74 68 65 |ians in |that the|
|00002a30| 20 61 6e 74 65 6e 61 74 | 61 6c 20 61 6e 64 20 70 | antenat|al and p|
|00002a40| 72 65 73 79 6d 70 74 6f | 6d 61 74 69 63 20 64 69 |resympto|matic di|
|00002a50| 61 67 6e 6f 73 69 73 0d | 0a 6d 75 73 74 20 6e 6f |agnosis.|.must no|
|00002a60| 77 20 62 65 20 61 70 70 | 72 6f 61 63 68 65 64 20 |w be app|roached |
|00002a70| 77 69 74 68 20 67 72 65 | 61 74 65 72 20 63 61 75 |with gre|ater cau|
|00002a80| 74 69 6f 6e 2e 0d 0a 0d | 0a 50 6f 73 74 2d 43 6f |tion....|.Post-Co|
|00002a90| 6d 6d 65 6e 74 0d 0a 0d | 0a 20 20 20 20 20 4a 75 |mment...|. Ju|
|00002aa0| 73 74 20 77 68 65 6e 20 | 49 20 74 68 6f 75 67 68 |st when |I though|
|00002ab0| 74 20 49 20 68 61 64 20 | 66 69 6e 61 6c 6c 79 20 |t I had |finally |
|00002ac0| 67 6f 74 74 65 6e 20 6f | 6e 65 20 67 65 6e 65 74 |gotten o|ne genet|
|00002ad0| 69 63 20 73 74 6f 72 79 | 20 73 74 72 61 69 67 68 |ic story| straigh|
|00002ae0| 74 2c 20 74 68 65 0d 0a | 6f 6e 65 20 64 65 61 6c |t, the..|one deal|
|00002af0| 69 6e 67 20 77 69 74 68 | 20 70 6f 6c 79 63 79 73 |ing with| polycys|
|00002b00| 74 69 63 20 6b 69 64 6e | 65 79 20 64 69 73 65 61 |tic kidn|ey disea|
|00002b10| 73 65 2c 20 74 68 65 20 | 61 62 6f 76 65 20 72 65 |se, the |above re|
|00002b20| 70 6f 72 74 20 61 70 70 | 65 61 72 65 64 20 61 6e |port app|eared an|
|00002b30| 64 0d 0a 70 75 6c 6c 65 | 64 20 74 68 65 20 72 75 |d..pulle|d the ru|
|00002b40| 67 20 6f 75 74 20 66 72 | 6f 6d 20 75 6e 64 65 72 |g out fr|om under|
|00002b50| 20 6d 79 20 6b 6e 6f 77 | 6c 65 64 67 65 20 62 61 | my know|ledge ba|
|00002b60| 73 65 2e 20 20 54 68 69 | 73 20 6c 65 61 76 65 73 |se. Thi|s leaves|
|00002b70| 20 6f 6e 65 20 77 69 74 | 68 20 61 0d 0a 74 65 72 | one wit|h a..ter|
|00002b80| 72 69 62 6c 65 20 66 65 | 65 6c 69 6e 67 20 6f 66 |rible fe|eling of|
|00002b90| 20 64 69 73 61 70 70 6f | 69 6e 74 6d 65 6e 74 20 | disappo|intment |
|00002ba0| 61 6e 64 20 61 20 72 65 | 63 6f 67 6e 69 74 69 6f |and a re|cognitio|
|00002bb0| 6e 20 74 68 61 74 20 61 | 20 77 69 73 65 20 6f 6c |n that a| wise ol|
|00002bc0| 64 20 70 68 79 73 69 63 | 73 0d 0a 70 72 6f 66 65 |d physic|s..profe|
|00002bd0| 73 73 6f 72 20 6f 66 20 | 6d 69 6e 65 20 69 6e 20 |ssor of |mine in |
|00002be0| 63 6f 6c 6c 65 67 65 20 | 77 61 73 20 70 72 6f 62 |college |was prob|
|00002bf0| 61 62 6c 79 20 63 6f 72 | 72 65 63 74 20 77 68 65 |ably cor|rect whe|
|00002c00| 6e 20 68 65 20 73 61 69 | 64 2c 20 22 54 68 65 72 |n he sai|d, "Ther|
|00002c10| 65 20 69 73 20 6e 6f 0d | 0a 67 72 61 76 69 74 79 |e is no.|.gravity|
|00002c20| 3b 20 74 68 65 20 65 61 | 72 74 68 20 73 69 6d 70 |; the ea|rth simp|
|00002c30| 6c 79 20 73 75 63 6b 73 | 2e 22 0d 0a 20 20 20 20 |ly sucks|.".. |
|00002c40| 20 54 68 65 20 61 75 74 | 68 6f 72 73 20 6f 66 20 | The aut|hors of |
|00002c50| 74 68 69 73 20 73 74 75 | 64 79 20 73 70 65 63 75 |this stu|dy specu|
|00002c60| 6c 61 74 65 64 20 74 68 | 61 74 20 69 6e 20 74 68 |lated th|at in th|
|00002c70| 69 73 20 68 75 67 65 20 | 6b 69 6e 64 72 65 64 20 |is huge |kindred |
|00002c80| 6f 66 20 32 35 30 0d 0a | 66 61 6d 69 6c 79 20 6d |of 250..|family m|
|00002c90| 65 6d 62 65 72 73 2c 20 | 61 20 73 65 63 6f 6e 64 |embers, |a second|
|00002ca0| 20 67 65 6e 65 20 61 62 | 6e 6f 72 6d 61 6c 69 74 | gene ab|normalit|
|00002cb0| 79 20 77 61 73 20 70 72 | 6f 62 61 62 6c 79 20 6e |y was pr|obably n|
|00002cc0| 65 63 65 73 73 61 72 79 | 20 74 6f 20 65 78 70 6c |ecessary| to expl|
|00002cd0| 61 69 6e 0d 0a 74 68 65 | 20 66 61 6d 69 6c 69 61 |ain..the| familia|
|00002ce0| 6c 20 70 6f 6c 79 63 79 | 73 74 69 63 20 6b 69 64 |l polycy|stic kid|
|00002cf0| 6e 65 79 20 64 69 73 65 | 61 73 65 2e 20 20 54 68 |ney dise|ase. Th|
|00002d00| 69 73 20 77 61 73 20 61 | 66 74 65 72 20 6a 75 73 |is was a|fter jus|
|00002d10| 74 20 61 62 6f 75 74 20 | 65 76 65 72 79 62 6f 64 |t about |everybod|
|00002d20| 79 0d 0a 68 61 64 20 73 | 65 74 74 6c 65 64 20 64 |y..had s|ettled d|
|00002d30| 6f 77 6e 20 77 69 74 68 | 20 74 68 65 20 66 61 63 |own with| the fac|
|00002d40| 74 20 74 68 61 74 20 70 | 6f 6c 79 63 79 73 74 69 |t that p|olycysti|
|00002d50| 63 20 6b 69 64 6e 65 79 | 73 20 77 65 72 65 20 63 |c kidney|s were c|
|00002d60| 61 75 73 65 64 20 62 79 | 20 61 20 73 69 6e 67 6c |aused by| a singl|
|00002d70| 65 0d 0a 67 65 6e 65 20 | 61 62 6e 6f 72 6d 61 6c |e..gene |abnormal|
|00002d80| 69 74 79 20 6f 6e 20 63 | 68 72 6f 6d 6f 73 6f 6d |ity on c|hromosom|
|00002d90| 65 20 31 36 2e 20 20 49 | 6e 64 65 65 64 2c 20 69 |e 16. I|ndeed, i|
|00002da0| 6e 76 65 73 74 69 67 61 | 74 6f 72 73 20 66 72 6f |nvestiga|tors fro|
|00002db0| 6d 20 49 74 61 6c 79 2c | 20 45 6e 67 6c 61 6e 64 |m Italy,| England|
|00002dc0| 2c 0d 0a 61 6e 64 20 4d | 61 73 73 61 63 68 75 73 |,..and M|assachus|
|00002dd0| 65 74 74 73 20 68 61 76 | 65 20 64 65 73 63 72 69 |etts hav|e descri|
|00002de0| 62 65 64 20 61 20 73 65 | 63 6f 6e 64 20 67 65 6e |bed a se|cond gen|
|00002df0| 65 74 69 63 20 6c 6f 63 | 75 73 20 72 65 6c 61 74 |etic loc|us relat|
|00002e00| 65 64 20 74 6f 20 61 75 | 74 6f 73 6f 6d 61 6c 0d |ed to au|tosomal.|
|00002e10| 0a 64 6f 6d 69 6e 61 6e | 74 20 70 6f 6c 79 63 79 |.dominan|t polycy|
|00002e20| 73 74 69 63 20 64 69 73 | 65 61 73 65 20 28 31 29 |stic dis|ease (1)|
|00002e30| 2e 20 20 53 6f 20 77 68 | 61 74 2c 20 79 6f 75 20 |. So wh|at, you |
|00002e40| 73 61 79 3f 20 20 57 65 | 6c 6c 20 64 6f 6e 27 74 |say? We|ll don't|
|00002e50| 20 62 65 20 63 61 76 61 | 6c 69 65 72 0d 0a 61 62 | be cava|lier..ab|
|00002e60| 6f 75 74 20 74 68 69 73 | 20 64 69 73 6f 72 64 65 |out this| disorde|
|00002e70| 72 2c 20 62 65 63 61 75 | 73 65 20 74 68 65 20 6d |r, becau|se the m|
|00002e80| 6f 72 65 20 77 65 20 6c | 65 61 72 6e 20 61 62 6f |ore we l|earn abo|
|00002e90| 75 74 20 69 74 2c 20 74 | 68 65 20 6d 6f 72 65 20 |ut it, t|he more |
|00002ea0| 74 68 65 20 66 65 61 72 | 20 6f 66 0d 0a 47 6f 64 |the fear| of..God|
|00002eb0| 20 73 68 6f 75 6c 64 20 | 62 65 20 70 75 74 20 69 | should |be put i|
|00002ec0| 6e 74 6f 20 75 73 2e 0d | 0a 20 20 20 20 20 54 68 |nto us..|. Th|
|00002ed0| 65 20 50 6f 6c 79 63 79 | 73 74 69 63 20 4b 69 64 |e Polycy|stic Kid|
|00002ee0| 6e 65 79 20 52 65 73 65 | 61 72 63 68 20 46 6f 75 |ney Rese|arch Fou|
|00002ef0| 6e 64 61 74 69 6f 6e 20 | 65 73 74 69 6d 61 74 65 |ndation |estimate|
|00002f00| 73 20 74 68 61 74 20 62 | 65 74 77 65 65 6e 20 34 |s that b|etween 4|
|00002f10| 30 30 2c 30 30 30 0d 0a | 61 6e 64 20 36 30 30 2c |00,000..|and 600,|
|00002f20| 30 30 30 20 41 6d 65 72 | 69 63 61 6e 73 20 61 6e |000 Amer|icans an|
|00002f30| 64 20 6d 6f 72 65 20 74 | 68 61 6e 20 35 20 6d 69 |d more t|han 5 mi|
|00002f40| 6c 6c 69 6f 6e 20 70 65 | 6f 70 6c 65 20 77 6f 72 |llion pe|ople wor|
|00002f50| 6c 64 77 69 64 65 20 68 | 61 76 65 20 74 68 65 20 |ldwide h|ave the |
|00002f60| 67 65 6e 65 0d 0a 64 65 | 66 65 63 74 20 66 6f 72 |gene..de|fect for|
|00002f70| 20 74 68 69 73 20 61 75 | 74 6f 73 6f 6d 61 6c 20 | this au|tosomal |
|00002f80| 64 69 73 6f 72 64 65 72 | 2e 20 20 44 65 73 70 69 |disorder|. Despi|
|00002f90| 74 65 20 69 74 73 20 68 | 69 67 68 20 70 72 6f 66 |te its h|igh prof|
|00002fa0| 69 6c 65 20 61 6d 6f 6e | 67 20 72 65 6e 61 6c 0d |ile amon|g renal.|
|00002fb0| 0a 64 69 73 6f 72 64 65 | 72 73 20 74 68 61 74 20 |.disorde|rs that |
|00002fc0| 63 61 75 73 65 20 6b 69 | 64 6e 65 79 20 66 61 69 |cause ki|dney fai|
|00002fd0| 6c 75 72 65 2c 20 72 65 | 6c 61 74 69 76 65 6c 79 |lure, re|latively|
|00002fe0| 20 6c 69 74 74 6c 65 20 | 69 73 20 6b 6e 6f 77 6e | little |is known|
|00002ff0| 20 61 62 6f 75 74 20 74 | 68 65 0d 0a 65 70 69 64 | about t|he..epid|
|00003000| 65 6d 69 6f 6c 6f 67 79 | 2c 20 6e 61 74 75 72 61 |emiology|, natura|
|00003010| 6c 20 68 69 73 74 6f 72 | 79 2c 20 6f 72 20 70 61 |l histor|y, or pa|
|00003020| 74 68 6f 67 65 6e 65 73 | 69 73 20 6f 66 20 70 6f |thogenes|is of po|
|00003030| 6c 79 63 79 73 74 69 63 | 20 6b 69 64 6e 65 79 73 |lycystic| kidneys|
|00003040| 2e 20 20 57 65 20 64 6f | 0d 0a 6b 6e 6f 77 20 74 |. We do|..know t|
|00003050| 68 61 74 20 6f 74 68 65 | 72 20 6f 72 67 61 6e 73 |hat othe|r organs|
|00003060| 20 61 72 65 20 63 6f 6d | 6d 6f 6e 6c 79 20 69 6e | are com|monly in|
|00003070| 76 6f 6c 76 65 64 20 28 | 34 30 25 20 74 6f 20 39 |volved (|40% to 9|
|00003080| 30 25 20 70 61 74 69 65 | 6e 74 73 20 68 61 76 65 |0% patie|nts have|
|00003090| 20 63 79 73 74 73 0d 0a | 69 6e 20 74 68 65 20 6c | cysts..|in the l|
|000030a0| 69 76 65 72 20 61 6e 64 | 20 35 25 20 74 6f 20 31 |iver and| 5% to 1|
|000030b0| 30 25 20 68 61 76 65 20 | 70 61 6e 63 72 65 61 74 |0% have |pancreat|
|000030c0| 69 63 20 63 79 73 74 73 | 29 2e 20 20 43 65 72 65 |ic cysts|). Cere|
|000030d0| 62 72 61 6c 20 61 6e 65 | 75 72 79 73 6d 73 20 61 |bral ane|urysms a|
|000030e0| 72 65 0d 0a 73 65 65 6e | 20 69 6e 20 61 73 20 6d |re..seen| in as m|
|000030f0| 61 6e 79 20 61 73 20 61 | 20 74 68 69 72 64 20 6f |any as a| third o|
|00003100| 66 20 70 61 74 69 65 6e | 74 73 2e 20 20 54 68 61 |f patien|ts. Tha|
|00003110| 74 27 73 20 61 20 6c 6f | 74 20 6f 66 20 22 73 6f |t's a lo|t of "so|
|00003120| 20 77 68 61 74 73 2e 22 | 20 20 54 68 65 20 67 65 | whats."| The ge|
|00003130| 6e 65 0d 0a 61 62 6e 6f | 72 6d 61 6c 69 74 79 20 |ne..abno|rmality |
|00003140| 77 61 73 20 64 65 73 63 | 72 69 62 65 64 20 6a 75 |was desc|ribed ju|
|00003150| 73 74 20 35 20 79 65 61 | 72 73 20 61 67 6f 20 61 |st 5 yea|rs ago a|
|00003160| 6e 64 20 74 68 65 20 72 | 75 73 68 20 69 73 20 6f |nd the r|ush is o|
|00003170| 6e 20 6e 6f 77 20 74 6f | 20 69 73 6f 6c 61 74 65 |n now to| isolate|
|00003180| 0d 0a 61 6e 64 20 63 6c | 6f 6e 65 20 74 68 61 74 |..and cl|one that|
|00003190| 20 67 65 6e 65 2e 20 20 | 54 68 65 20 72 65 70 6f | gene. |The repo|
|000031a0| 72 74 20 62 79 20 4b 69 | 6d 62 65 72 6c 69 6e 67 |rt by Ki|mberling|
|000031b0| 20 65 74 20 61 6c 2e 20 | 61 62 73 74 72 61 63 74 | et al. |abstract|
|000031c0| 65 64 20 61 62 6f 76 65 | 0d 0a 63 6f 6d 70 6c 69 |ed above|..compli|
|000031d0| 63 61 74 65 73 20 6d 61 | 74 74 65 72 73 20 73 6f |cates ma|tters so|
|000031e0| 6d 65 77 68 61 74 20 62 | 79 20 69 6e 64 69 63 61 |mewhat b|y indica|
|000031f0| 74 69 6e 67 20 74 68 65 | 20 70 72 65 73 65 6e 63 |ting the| presenc|
|00003200| 65 20 6f 66 20 61 20 67 | 65 6e 65 20 6c 6f 63 75 |e of a g|ene locu|
|00003210| 73 20 6f 74 68 65 72 0d | 0a 74 68 61 6e 20 74 68 |s other.|.than th|
|00003220| 61 74 20 61 6c 72 65 61 | 64 79 20 64 65 73 63 72 |at alrea|dy descr|
|00003230| 69 62 65 64 2e 20 20 47 | 72 61 6e 74 68 61 6d 20 |ibed. G|rantham |
|00003240| 28 32 29 20 6e 6f 74 65 | 73 20 74 68 61 74 20 74 |(2) note|s that t|
|00003250| 68 65 20 66 69 6e 64 69 | 6e 67 20 6f 66 20 6d 6f |he findi|ng of mo|
|00003260| 72 65 20 74 68 61 6e 0d | 0a 61 20 73 69 6e 67 6c |re than.|.a singl|
|00003270| 65 20 61 62 6e 6f 72 6d | 61 6c 20 67 65 6e 65 20 |e abnorm|al gene |
|00003280| 74 68 61 74 20 6d 61 79 | 20 63 61 75 73 65 20 74 |that may| cause t|
|00003290| 68 65 20 64 69 73 65 61 | 73 65 20 73 68 6f 75 6c |he disea|se shoul|
|000032a0| 64 20 63 6f 6d 65 20 61 | 73 20 6e 6f 20 73 75 72 |d come a|s no sur|
|000032b0| 70 72 69 73 65 0d 0a 74 | 6f 20 74 68 65 20 69 6e |prise..t|o the in|
|000032c0| 76 65 73 74 69 67 61 74 | 6f 72 73 20 77 68 6f 20 |vestigat|ors who |
|000032d0| 68 61 76 65 20 73 74 72 | 75 67 67 6c 65 64 20 73 |have str|uggled s|
|000032e0| 6f 20 6c 6f 6e 67 20 74 | 6f 20 75 6e 64 65 72 73 |o long t|o unders|
|000032f0| 74 61 6e 64 20 74 68 65 | 20 70 61 74 68 6f 67 65 |tand the| pathoge|
|00003300| 6e 65 73 69 73 0d 0a 6f | 66 20 72 65 6e 61 6c 20 |nesis..o|f renal |
|00003310| 63 79 73 74 73 2e 20 20 | 49 6e 20 66 61 63 74 2c |cysts. |In fact,|
|00003320| 20 74 68 65 72 65 20 69 | 73 20 61 20 72 65 63 65 | there i|s a rece|
|00003330| 73 73 69 76 65 20 66 6f | 72 6d 20 6f 66 20 70 6f |ssive fo|rm of po|
|00003340| 6c 79 63 79 73 74 69 63 | 20 6b 69 64 6e 65 79 0d |lycystic| kidney.|
|00003350| 0a 64 69 73 65 61 73 65 | 20 74 68 61 74 20 69 73 |.disease| that is|
|00003360| 6e 27 74 20 72 65 6c 61 | 74 65 64 20 74 6f 20 74 |n't rela|ted to t|
|00003370| 68 65 20 63 68 72 6f 6d | 6f 73 6f 6d 65 20 31 36 |he chrom|osome 16|
|00003380| 20 64 65 66 65 63 74 20 | 61 74 20 61 6c 6c 2e 0d | defect |at all..|
|00003390| 0a 20 20 20 20 20 49 74 | 27 73 20 64 69 66 66 69 |. It|'s diffi|
|000033a0| 63 75 6c 74 20 74 6f 20 | 66 6f 6c 6c 6f 77 20 74 |cult to |follow t|
|000033b0| 68 65 20 63 68 61 6e 67 | 69 6e 67 20 73 63 65 6e |he chang|ing scen|
|000033c0| 65 20 6f 66 20 67 65 6e | 65 74 69 63 73 20 74 68 |e of gen|etics th|
|000033d0| 65 73 65 20 64 61 79 73 | 20 61 6e 64 0d 0a 68 61 |ese days| and..ha|
|000033e0| 76 65 20 63 6f 6e 66 69 | 64 65 6e 63 65 20 69 6e |ve confi|dence in|
|000033f0| 20 74 68 65 20 66 61 63 | 74 20 74 68 61 74 20 77 | the fac|t that w|
|00003400| 68 61 74 20 79 6f 75 20 | 72 65 61 64 20 77 69 6c |hat you |read wil|
|00003410| 6c 20 6e 65 76 65 72 20 | 63 68 61 6e 67 65 2e 20 |l never |change. |
|00003420| 20 45 76 65 72 79 62 6f | 64 79 0d 0a 73 68 6f 75 | Everybo|dy..shou|
|00003430| 6c 64 20 66 69 72 6d 6c | 79 20 62 65 6c 69 65 76 |ld firml|y believ|
|00003440| 65 20 69 6e 20 73 6f 6d | 65 74 68 69 6e 67 3b 20 |e in som|ething; |
|00003450| 49 20 62 65 6c 69 65 76 | 65 20 49 27 6c 6c 20 68 |I believ|e I'll h|
|00003460| 61 76 65 20 61 6e 6f 74 | 68 65 72 20 64 72 69 6e |ave anot|her drin|
|00003470| 6b 2e 2d 2d 4a 2e 41 2e | 0d 0a 53 74 6f 63 6b 6d |k.--J.A.|..Stockm|
|00003480| 61 6e 2c 20 49 49 49 2c | 20 4d 2e 44 2e 0d 0a 24 |an, III,| M.D...$|
|00003490| 55 6e 69 71 75 65 5f 49 | 44 7b 59 52 42 31 32 30 |Unique_I|D{YRB120|
|000034a0| 30 32 7d 0d 0a 24 54 69 | 74 6c 65 7b 4c 61 62 6f |02}..$Ti|tle{Labo|
|000034b0| 72 61 74 6f 72 79 20 61 | 6e 64 20 43 6c 69 6e 69 |ratory a|nd Clini|
|000034c0| 63 61 6c 20 56 61 72 69 | 61 62 6c 65 73 20 74 6f |cal Vari|ables to|
|000034d0| 20 50 72 65 64 69 63 74 | 20 4f 75 74 63 6f 6d 65 | Predict| Outcome|
|000034e0| 20 69 6e 20 48 65 6d 6f | 6c 79 74 69 63 2d 55 72 | in Hemo|lytic-Ur|
|000034f0| 65 6d 69 63 20 53 79 6e | 64 72 6f 6d 65 7d 0d 0a |emic Syn|drome}..|
|00003500| 24 41 75 74 68 6f 72 7b | 48 61 76 65 6e 73 2c 20 |$Author{|Havens, |
|00003510| 50 4c 0d 0a 4f 27 52 6f | 75 72 6b 65 2c 20 50 50 |PL..O'Ro|urke, PP|
|00003520| 0d 0a 48 61 68 6e 2c 20 | 4a 0d 0a 48 69 67 67 69 |..Hahn, |J..Higgi|
|00003530| 6e 73 2c 20 4a 0d 0a 57 | 61 6c 6b 65 72 2c 20 41 |ns, J..W|alker, A|
|00003540| 4d 7d 0d 0a 24 41 66 66 | 69 6c 69 61 74 69 6f 6e |M}..$Aff|iliation|
|00003550| 7b 43 68 69 6c 64 72 65 | 6e 27 73 20 48 6f 73 70 |{Childre|n's Hosp|
|00003560| 2c 20 42 6f 73 74 6f 6e | 3b 20 48 61 72 76 61 72 |, Boston|; Harvar|
|00003570| 64 20 53 63 68 6f 6f 6c | 20 6f 66 20 50 75 62 6c |d School| of Publ|
|00003580| 69 63 20 48 65 61 6c 74 | 68 2c 20 42 6f 73 74 6f |ic Healt|h, Bosto|
|00003590| 6e 7d 0d 0a 24 4a 6f 75 | 72 6e 61 6c 7b 41 6d 20 |n}..$Jou|rnal{Am |
|000035a0| 4a 20 44 69 73 20 43 68 | 69 6c 64 7d 0d 0a 24 56 |J Dis Ch|ild}..$V|
|000035b0| 6f 6c 75 6d 65 7b 31 34 | 32 7d 0d 0a 24 50 61 67 |olume{14|2}..$Pag|
|000035c0| 65 7b 39 36 31 2d 39 36 | 34 7d 0d 0a 24 44 61 74 |e{961-96|4}..$Dat|
|000035d0| 65 7b 53 65 70 74 65 6d | 62 65 72 20 31 39 38 38 |e{Septem|ber 1988|
|000035e0| 7d 0d 0a 24 59 42 56 6f | 6c 7b 31 39 39 30 20 59 |}..$YBVo|l{1990 Y|
|000035f0| 65 61 72 20 42 6f 6f 6b | 20 6f 66 20 50 65 64 69 |ear Book| of Pedi|
|00003600| 61 74 72 69 63 73 7d 0d | 0a 24 43 68 61 70 74 65 |atrics}.|.$Chapte|
|00003610| 72 7b 31 30 2d 31 32 7d | 0d 0a 24 59 42 45 64 69 |r{10-12}|..$YBEdi|
|00003620| 74 6f 72 7b 44 72 73 2e | 20 4f 73 6b 69 20 61 6e |tor{Drs.| Oski an|
|00003630| 64 20 53 74 6f 63 6b 6d | 61 6e 7d 0d 0a 24 54 61 |d Stockm|an}..$Ta|
|00003640| 62 6c 65 7b 54 61 62 6c | 65 20 4f 6d 69 74 74 65 |ble{Tabl|e Omitte|
|00003650| 64 7d 0d 0a 24 43 6f 6d | 6d 65 6e 74 61 74 6f 72 |d}..$Com|mentator|
|00003660| 7b 4a 2e 41 2e 20 53 74 | 6f 63 6b 6d 61 6e 2c 20 |{J.A. St|ockman, |
|00003670| 49 49 49 2c 20 4d 2e 44 | 2e 7d 0d 0a 0d 0a 43 6f |III, M.D|.}....Co|
|00003680| 70 79 72 69 67 68 74 20 | 28 63 29 20 59 65 61 72 |pyright |(c) Year|
|00003690| 62 6f 6f 6b 20 4d 65 64 | 69 63 61 6c 20 50 75 62 |book Med|ical Pub|
|000036a0| 6c 69 73 68 65 72 73 2c | 20 49 6e 63 2e 2c 20 31 |lishers,| Inc., 1|
|000036b0| 39 39 30 0d 0a 0d 0a 31 | 39 39 30 20 59 65 61 72 |990....1|990 Year|
|000036c0| 20 42 6f 6f 6b 20 6f 66 | 20 50 65 64 69 61 74 72 | Book of| Pediatr|
|000036d0| 69 63 73 20 28 52 29 0d | 0a 0d 0a 43 68 61 70 74 |ics (R).|...Chapt|
|000036e0| 65 72 20 31 30 2d 2d 54 | 68 65 20 47 65 6e 69 74 |er 10--T|he Genit|
|000036f0| 6f 75 72 69 6e 61 72 79 | 20 54 72 61 63 74 0d 0a |ourinary| Tract..|
|00003700| 0d 0a 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |..------|--------|
|00003710| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003720| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003730| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003740| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003750| 0d 0a 4c 61 62 6f 72 61 | 74 6f 72 79 20 61 6e 64 |..Labora|tory and|
|00003760| 20 43 6c 69 6e 69 63 61 | 6c 20 56 61 72 69 61 62 | Clinica|l Variab|
|00003770| 6c 65 73 20 74 6f 20 50 | 72 65 64 69 63 74 20 4f |les to P|redict O|
|00003780| 75 74 63 6f 6d 65 20 69 | 6e 20 48 65 6d 6f 6c 79 |utcome i|n Hemoly|
|00003790| 74 69 63 2d 55 72 65 6d | 69 63 0d 0a 53 79 6e 64 |tic-Urem|ic..Synd|
|000037a0| 72 6f 6d 65 0d 0a 0d 0a | 48 61 76 65 6e 73 20 50 |rome....|Havens P|
|000037b0| 4c 2c 20 4f 27 52 6f 75 | 72 6b 65 20 50 50 2c 20 |L, O'Rou|rke PP, |
|000037c0| 48 61 68 6e 20 4a 2c 20 | 48 69 67 67 69 6e 73 20 |Hahn J, |Higgins |
|000037d0| 4a 2c 20 57 61 6c 6b 65 | 72 20 41 4d 0d 0a 28 43 |J, Walke|r AM..(C|
|000037e0| 68 69 6c 64 72 65 6e 27 | 73 20 48 6f 73 70 2c 20 |hildren'|s Hosp, |
|000037f0| 42 6f 73 74 6f 6e 3b 20 | 48 61 72 76 61 72 64 20 |Boston; |Harvard |
|00003800| 53 63 68 6f 6f 6c 20 6f | 66 20 50 75 62 6c 69 63 |School o|f Public|
|00003810| 20 48 65 61 6c 74 68 2c | 20 42 6f 73 74 6f 6e 29 | Health,| Boston)|
|00003820| 0d 0a 41 6d 20 4a 20 44 | 69 73 20 43 68 69 6c 64 |..Am J D|is Child|
|00003830| 20 31 34 32 3a 39 36 31 | 2d 39 36 34 2c 20 53 65 | 142:961|-964, Se|
|00003840| 70 74 65 6d 62 65 72 20 | 31 39 38 38 0d 0a 2d 2d |ptember |1988..--|
|00003850| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003860| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003870| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003880| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003890| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 0d 0a 0d 0a |--------|----....|
|000038a0| 41 62 73 74 72 61 63 74 | 0d 0a 0d 0a 20 20 20 20 |Abstract|.... |
|000038b0| 20 49 6d 70 72 6f 76 65 | 6d 65 6e 74 73 20 69 6e | Improve|ments in|
|000038c0| 20 64 69 61 6c 79 73 69 | 73 20 61 6e 64 20 67 65 | dialysi|s and ge|
|000038d0| 6e 65 72 61 6c 20 70 61 | 74 69 65 6e 74 20 63 61 |neral pa|tient ca|
|000038e0| 72 65 20 68 61 76 65 20 | 72 65 64 75 63 65 64 20 |re have |reduced |
|000038f0| 74 68 65 0d 0a 6d 6f 72 | 74 61 6c 69 74 79 20 72 |the..mor|tality r|
|00003900| 61 74 65 20 69 6e 20 70 | 61 74 69 65 6e 74 73 20 |ate in p|atients |
|00003910| 77 69 74 68 20 68 65 6d | 6f 6c 79 74 69 63 2d 75 |with hem|olytic-u|
|00003920| 72 65 6d 69 63 20 73 79 | 6e 64 72 6f 6d 65 20 28 |remic sy|ndrome (|
|00003930| 48 55 53 29 2c 20 62 75 | 74 0d 0a 73 69 67 6e 69 |HUS), bu|t..signi|
|00003940| 66 69 63 61 6e 74 20 6d | 6f 72 62 69 64 69 74 79 |ficant m|orbidity|
|00003950| 20 73 74 69 6c 6c 20 6f | 63 63 75 72 73 20 69 6e | still o|ccurs in|
|00003960| 20 31 30 25 20 74 6f 20 | 32 30 25 20 6f 66 20 70 | 10% to |20% of p|
|00003970| 61 74 69 65 6e 74 73 20 | 77 69 74 68 20 63 68 72 |atients |with chr|
|00003980| 6f 6e 69 63 0d 0a 72 65 | 6e 61 6c 20 66 61 69 6c |onic..re|nal fail|
|00003990| 75 72 65 20 28 43 52 46 | 29 2c 20 68 79 70 65 72 |ure (CRF|), hyper|
|000039a0| 74 65 6e 73 69 6f 6e 2c | 20 6f 72 20 73 65 76 65 |tension,| or seve|
|000039b0| 72 65 20 6e 65 75 72 6f | 6c 6f 67 69 63 20 73 65 |re neuro|logic se|
|000039c0| 71 75 65 6c 61 65 2e 20 | 20 41 0d 0a 70 72 6f 67 |quelae. | A..prog|
|000039d0| 6e 6f 73 74 69 63 20 67 | 75 69 64 65 20 77 61 73 |nostic g|uide was|
|000039e0| 20 64 65 76 65 6c 6f 70 | 65 64 2c 20 75 73 69 6e | develop|ed, usin|
|000039f0| 67 20 6f 62 6a 65 63 74 | 69 76 65 20 63 72 69 74 |g object|ive crit|
|00003a00| 65 72 69 61 20 61 76 61 | 69 6c 61 62 6c 65 20 77 |eria ava|ilable w|
|00003a10| 69 74 68 69 6e 20 74 68 | 65 0d 0a 66 69 72 73 74 |ithin th|e..first|
|00003a20| 20 34 38 20 68 6f 75 72 | 73 20 6f 66 20 68 6f 73 | 48 hour|s of hos|
|00003a30| 70 69 74 61 6c 69 7a 61 | 74 69 6f 6e 2c 20 74 68 |pitaliza|tion, th|
|00003a40| 61 74 20 63 6f 75 6c 64 | 20 69 64 65 6e 74 69 66 |at could| identif|
|00003a50| 79 20 70 61 74 69 65 6e | 74 73 20 77 69 74 68 20 |y patien|ts with |
|00003a60| 48 55 53 20 61 74 0d 0a | 68 69 67 68 20 72 69 73 |HUS at..|high ris|
|00003a70| 6b 20 6f 66 20 64 65 61 | 74 68 2c 20 43 52 46 2c |k of dea|th, CRF,|
|00003a80| 20 6f 72 20 73 65 72 69 | 6f 75 73 20 6e 65 75 72 | or seri|ous neur|
|00003a90| 6f 6c 6f 67 69 63 20 73 | 65 71 75 65 6c 61 65 2e |ologic s|equelae.|
|00003aa0| 0d 0a 20 20 20 20 20 54 | 77 6f 20 6f 75 74 63 6f |.. T|wo outco|
|00003ab0| 6d 65 20 67 72 6f 75 70 | 73 2c 20 62 61 73 65 64 |me group|s, based|
|00003ac0| 20 6f 6e 20 63 6f 6e 64 | 69 74 69 6f 6e 20 61 74 | on cond|ition at|
|00003ad0| 20 68 6f 73 70 69 74 61 | 6c 20 64 69 73 63 68 61 | hospita|l discha|
|00003ae0| 72 67 65 2c 20 77 65 72 | 65 0d 0a 64 65 66 69 6e |rge, wer|e..defin|
|00003af0| 65 64 20 61 6d 6f 6e 67 | 20 74 68 65 20 37 38 20 |ed among| the 78 |
|00003b00| 70 61 74 69 65 6e 74 73 | 20 77 69 74 68 20 48 55 |patients| with HU|
|00003b10| 53 2e 20 20 54 68 65 20 | 22 67 6f 6f 64 20 6f 75 |S. The |"good ou|
|00003b20| 74 63 6f 6d 65 22 20 67 | 72 6f 75 70 20 63 6f 6e |tcome" g|roup con|
|00003b30| 73 69 73 74 65 64 20 6f | 66 0d 0a 36 36 20 70 61 |sisted o|f..66 pa|
|00003b40| 74 69 65 6e 74 73 20 77 | 69 74 68 20 6e 6f 20 73 |tients w|ith no s|
|00003b50| 65 72 69 6f 75 73 20 73 | 65 71 75 65 6c 61 65 20 |erious s|equelae |
|00003b60| 61 6e 64 20 74 68 65 20 | 22 62 61 64 20 6f 75 74 |and the |"bad out|
|00003b70| 63 6f 6d 65 22 20 67 72 | 6f 75 70 20 69 6e 63 6c |come" gr|oup incl|
|00003b80| 75 64 65 64 20 31 32 0d | 0a 70 61 74 69 65 6e 74 |uded 12.|.patient|
|00003b90| 73 20 77 68 6f 20 65 69 | 74 68 65 72 20 64 69 65 |s who ei|ther die|
|00003ba0| 64 2c 20 68 61 64 20 43 | 52 46 2c 20 6f 72 20 6e |d, had C|RF, or n|
|00003bb0| 65 75 72 6f 6c 6f 67 69 | 63 20 73 65 71 75 65 6c |eurologi|c sequel|
|00003bc0| 61 65 20 6f 6e 20 64 69 | 73 63 68 61 72 67 65 2e |ae on di|scharge.|
|00003bd0| 0d 0a 20 20 20 20 20 4e | 65 75 72 6f 6c 6f 67 69 |.. N|eurologi|
|00003be0| 63 20 73 69 67 6e 73 2c | 20 6c 6f 6e 67 65 72 20 |c signs,| longer |
|00003bf0| 64 75 72 61 74 69 6f 6e | 20 6f 66 20 64 69 61 6c |duration| of dial|
|00003c00| 79 73 69 73 2c 20 61 6e | 64 20 69 6e 74 75 62 61 |ysis, an|d intuba|
|00003c10| 74 69 6f 6e 20 77 65 72 | 65 20 6d 6f 72 65 0d 0a |tion wer|e more..|
|00003c20| 63 6f 6d 6d 6f 6e 20 69 | 6e 20 74 68 65 20 62 61 |common i|n the ba|
|00003c30| 64 20 6f 75 74 63 6f 6d | 65 20 67 72 6f 75 70 2c |d outcom|e group,|
|00003c40| 20 61 73 20 77 65 72 65 | 20 68 69 67 68 65 72 20 | as were| higher |
|00003c50| 6c 65 76 65 6c 73 20 6f | 66 20 70 6f 74 61 73 73 |levels o|f potass|
|00003c60| 69 75 6d 2c 20 67 6c 75 | 63 6f 73 65 2c 0d 0a 61 |ium, glu|cose,..a|
|00003c70| 6e 64 20 63 72 65 61 74 | 69 6e 69 6e 65 20 61 6e |nd creat|inine an|
|00003c80| 64 20 6c 6f 77 65 72 20 | 6d 65 61 6e 20 73 65 72 |d lower |mean ser|
|00003c90| 75 6d 20 6c 65 76 65 6c | 73 20 6f 66 20 63 61 6c |um level|s of cal|
|00003ca0| 63 69 75 6d 20 28 74 61 | 62 6c 65 29 2e 20 20 54 |cium (ta|ble). T|
|00003cb0| 68 65 20 6d 65 61 6e 20 | 64 61 69 6c 79 0d 0a 75 |he mean |daily..u|
|00003cc0| 72 69 6e 65 20 6f 75 74 | 70 75 74 20 69 6e 20 74 |rine out|put in t|
|00003cd0| 68 65 20 62 61 64 20 6f | 75 74 63 6f 6d 65 20 67 |he bad o|utcome g|
|00003ce0| 72 6f 75 70 20 77 61 73 | 20 37 32 25 20 74 68 61 |roup was| 72% tha|
|00003cf0| 74 20 6f 66 20 74 68 65 | 20 67 6f 6f 64 20 6f 75 |t of the| good ou|
|00003d00| 74 63 6f 6d 65 20 67 72 | 6f 75 70 2e 0d 0a 42 69 |tcome gr|oup...Bi|
|00003d10| 76 61 72 69 61 74 65 20 | 61 6e 61 6c 79 73 69 73 |variate |analysis|
|00003d20| 20 73 68 6f 77 65 64 20 | 74 68 61 74 20 73 65 72 | showed |that ser|
|00003d30| 75 6d 20 70 6f 74 61 73 | 73 69 75 6d 20 61 6e 64 |um potas|sium and|
|00003d40| 20 63 61 6c 63 69 75 6d | 20 6c 65 76 65 6c 73 20 | calcium| levels |
|00003d50| 61 6e 64 20 74 68 65 0d | 0a 68 6f 75 72 6c 79 20 |and the.|.hourly |
|00003d60| 75 72 69 6e 65 20 6f 75 | 74 70 75 74 20 69 6e 20 |urine ou|tput in |
|00003d70| 74 68 65 20 66 69 72 73 | 74 20 34 38 20 68 6f 75 |the firs|t 48 hou|
|00003d80| 72 73 20 62 65 73 74 20 | 64 69 73 63 72 69 6d 69 |rs best |discrimi|
|00003d90| 6e 61 74 65 64 20 62 65 | 74 77 65 65 6e 20 67 72 |nated be|tween gr|
|00003da0| 6f 75 70 73 2e 0d 0a 41 | 73 20 61 20 70 72 6f 67 |oups...A|s a prog|
|00003db0| 6e 6f 73 74 69 63 20 67 | 75 69 64 65 2c 20 61 20 |nostic g|uide, a |
|00003dc0| 73 65 72 75 6d 20 63 61 | 6c 63 69 75 6d 20 6c 65 |serum ca|lcium le|
|00003dd0| 76 65 6c 20 6f 66 20 32 | 20 6d 6d 6f 6c 2f 4c 20 |vel of 2| mmol/L |
|00003de0| 6f 72 20 6c 65 73 73 20 | 61 6e 64 20 75 72 69 6e |or less |and urin|
|00003df0| 65 0d 0a 6f 75 74 70 75 | 74 20 6f 66 20 6c 65 73 |e..outpu|t of les|
|00003e00| 73 20 74 68 61 6e 20 30 | 2e 34 20 6d 6c 2f 6b 67 |s than 0|.4 ml/kg|
|00003e10| 2f 68 6f 75 72 20 69 6e | 20 61 20 32 34 2d 68 6f |/hour in| a 24-ho|
|00003e20| 75 72 20 70 65 72 69 6f | 64 20 70 72 65 64 69 63 |ur perio|d predic|
|00003e30| 74 65 64 20 61 20 62 61 | 64 20 6f 75 74 63 6f 6d |ted a ba|d outcom|
|00003e40| 65 0d 0a 77 69 74 68 20 | 37 35 25 20 73 65 6e 73 |e..with |75% sens|
|00003e50| 69 74 69 76 69 74 79 20 | 61 6e 64 20 39 38 25 20 |itivity |and 98% |
|00003e60| 73 70 65 63 69 66 69 63 | 69 74 79 2c 20 61 6e 64 |specific|ity, and|
|00003e70| 20 68 61 64 20 61 20 70 | 6f 73 69 74 69 76 65 20 | had a p|ositive |
|00003e80| 70 72 65 64 69 63 74 69 | 76 65 20 76 61 6c 75 65 |predicti|ve value|
|00003e90| 0d 0a 6f 66 20 39 30 25 | 2e 0d 0a 20 20 20 20 20 |..of 90%|... |
|00003ea0| 41 20 73 69 6d 70 6c 65 | 20 63 72 69 74 65 72 69 |A simple| criteri|
|00003eb0| 6f 6e 20 62 61 73 65 64 | 20 6f 6e 20 73 65 72 75 |on based| on seru|
|00003ec0| 6d 20 63 61 6c 63 69 75 | 6d 20 6c 65 76 65 6c 73 |m calciu|m levels|
|00003ed0| 20 61 6e 64 20 75 72 69 | 6e 65 20 6f 75 74 70 75 | and uri|ne outpu|
|00003ee0| 74 20 77 69 74 68 69 6e | 0d 0a 74 68 65 20 66 69 |t within|..the fi|
|00003ef0| 72 73 74 20 34 38 20 68 | 6f 75 72 73 20 6f 66 20 |rst 48 h|ours of |
|00003f00| 61 64 6d 69 73 73 69 6f | 6e 20 63 61 6e 20 62 65 |admissio|n can be|
|00003f10| 20 75 73 65 64 20 74 6f | 20 70 72 65 64 69 63 74 | used to| predict|
|00003f20| 20 74 68 65 20 6f 75 74 | 63 6f 6d 65 20 6f 66 0d | the out|come of.|
|00003f30| 0a 68 6f 73 70 69 74 61 | 6c 69 7a 61 74 69 6f 6e |.hospita|lization|
|00003f40| 20 69 6e 20 70 61 74 69 | 65 6e 74 73 20 77 69 74 | in pati|ents wit|
|00003f50| 68 20 48 55 53 2e 20 20 | 46 75 72 74 68 65 72 20 |h HUS. |Further |
|00003f60| 73 74 75 64 69 65 73 20 | 69 6e 20 6f 74 68 65 72 |studies |in other|
|00003f70| 20 70 6f 70 75 6c 61 74 | 69 6f 6e 0d 0a 67 72 6f | populat|ion..gro|
|00003f80| 75 70 73 20 61 72 65 20 | 77 61 72 72 61 6e 74 65 |ups are |warrante|
|00003f90| 64 20 74 6f 20 61 73 73 | 65 73 73 20 69 74 73 20 |d to ass|ess its |
|00003fa0| 72 65 6c 69 61 62 69 6c | 69 74 79 20 61 73 20 61 |reliabil|ity as a|
|00003fb0| 20 70 72 6f 67 6e 6f 73 | 74 69 63 20 67 75 69 64 | prognos|tic guid|
|00003fc0| 65 2e 0d 0a 0d 0a 50 6f | 73 74 2d 43 6f 6d 6d 65 |e.....Po|st-Comme|
|00003fd0| 6e 74 0d 0a 0d 0a 20 20 | 20 20 20 49 20 74 6f 6f |nt.... | I too|
|00003fe0| 6b 20 74 68 65 20 70 75 | 6c 73 65 20 6f 66 20 74 |k the pu|lse of t|
|00003ff0| 68 65 20 61 62 6f 76 65 | 20 61 72 74 69 63 6c 65 |he above| article|
|00004000| 20 61 6e 64 20 66 6f 75 | 6e 64 20 74 68 65 20 72 | and fou|nd the r|
|00004010| 65 70 6f 72 74 20 64 65 | 61 64 2e 20 20 49 74 20 |eport de|ad. It |
|00004020| 69 73 0d 0a 69 6e 63 6c | 75 64 65 64 20 69 6e 20 |is..incl|uded in |
|00004030| 74 68 65 20 59 45 41 52 | 20 42 4f 4f 4b 20 73 69 |the YEAR| BOOK si|
|00004040| 6d 70 6c 79 20 61 73 20 | 61 20 74 6f 75 63 68 73 |mply as |a touchs|
|00004050| 74 6f 6e 65 20 66 6f 72 | 20 61 6e 20 75 70 64 61 |tone for| an upda|
|00004060| 74 65 20 6f 6e 20 48 55 | 53 2e 20 20 53 75 72 65 |te on HU|S. Sure|
|00004070| 2c 0d 0a 69 74 20 69 73 | 20 75 73 65 66 75 6c 20 |,..it is| useful |
|00004080| 74 6f 20 74 72 79 20 74 | 6f 20 65 73 74 61 62 6c |to try t|o establ|
|00004090| 69 73 68 20 70 72 6f 67 | 6e 6f 73 74 69 63 20 63 |ish prog|nostic c|
|000040a0| 68 61 72 61 63 74 65 72 | 69 73 74 69 63 73 20 65 |haracter|istics e|
|000040b0| 61 72 6c 79 20 69 6e 20 | 74 68 65 0d 0a 63 6f 75 |arly in |the..cou|
|000040c0| 72 73 65 20 6f 66 20 61 | 20 64 69 73 65 61 73 65 |rse of a| disease|
|000040d0| 2c 20 62 75 74 2c 20 71 | 75 69 74 65 20 66 72 61 |, but, q|uite fra|
|000040e0| 6e 6b 6c 79 2c 20 77 69 | 74 68 20 61 20 64 69 73 |nkly, wi|th a dis|
|000040f0| 6f 72 64 65 72 20 66 6f | 72 20 77 68 69 63 68 20 |order fo|r which |
|00004100| 74 68 65 20 62 65 73 74 | 0d 0a 6d 61 6e 61 67 65 |the best|..manage|
|00004110| 6d 65 6e 74 20 68 61 73 | 20 79 65 74 20 74 6f 20 |ment has| yet to |
|00004120| 68 61 76 65 20 62 65 65 | 6e 20 64 65 63 69 64 65 |have bee|n decide|
|00004130| 64 20 75 70 6f 6e 2c 20 | 77 68 61 74 20 64 6f 65 |d upon, |what doe|
|00004140| 73 20 69 74 20 6d 65 61 | 6e 20 77 68 65 6e 20 79 |s it mea|n when y|
|00004150| 6f 75 20 63 61 6e 0d 0a | 70 72 65 64 69 63 74 20 |ou can..|predict |
|00004160| 6f 75 74 63 6f 6d 65 20 | 77 69 74 68 20 37 35 25 |outcome |with 75%|
|00004170| 20 73 65 6e 73 69 74 69 | 76 69 74 79 2c 20 39 38 | sensiti|vity, 98|
|00004180| 25 20 73 70 65 63 69 66 | 69 63 69 74 79 2c 20 61 |% specif|icity, a|
|00004190| 6e 64 20 61 20 70 6f 73 | 69 74 69 76 65 0d 0a 70 |nd a pos|itive..p|
|000041a0| 72 65 64 69 63 74 69 76 | 65 20 76 61 6c 75 65 20 |redictiv|e value |
|000041b0| 6f 66 20 39 30 25 3f 20 | 20 59 6f 75 20 63 65 72 |of 90%? | You cer|
|000041c0| 74 61 69 6e 6c 79 20 61 | 72 65 20 6e 6f 74 20 67 |tainly a|re not g|
|000041d0| 6f 69 6e 67 20 74 6f 20 | 63 68 61 6e 67 65 20 79 |oing to |change y|
|000041e0| 6f 75 72 0d 0a 6d 61 6e | 61 67 65 6d 65 6e 74 20 |our..man|agement |
|000041f0| 77 68 65 6e 20 79 6f 75 | 20 6b 6e 6f 77 20 6f 66 |when you| know of|
|00004200| 20 6e 6f 20 62 65 74 74 | 65 72 20 6d 61 6e 61 67 | no bett|er manag|
|00004210| 65 6d 65 6e 74 20 6c 75 | 72 6b 69 6e 67 20 73 6f |ement lu|rking so|
|00004220| 6d 65 77 68 65 72 65 20 | 69 6e 0d 0a 73 6f 6d 65 |mewhere |in..some|
|00004230| 62 6f 64 79 27 73 20 63 | 6c 6f 73 65 74 20 66 6f |body's c|loset fo|
|00004240| 72 20 61 20 70 61 74 69 | 65 6e 74 20 77 68 6f 20 |r a pati|ent who |
|00004250| 69 73 20 6e 6f 74 20 6c | 69 6b 65 6c 79 20 74 6f |is not l|ikely to|
|00004260| 20 64 6f 20 77 65 6c 6c | 2e 0d 0a 20 20 20 20 20 | do well|... |
|00004270| 4e 6f 20 61 73 70 65 63 | 74 20 6f 66 20 48 55 53 |No aspec|t of HUS|
|00004280| 20 69 73 20 6d 6f 72 65 | 20 63 6f 6e 74 72 6f 76 | is more| controv|
|00004290| 65 72 73 69 61 6c 20 74 | 68 61 6e 20 69 74 73 20 |ersial t|han its |
|000042a0| 74 72 65 61 74 6d 65 6e | 74 2e 20 20 49 6e 20 61 |treatmen|t. In a|
|000042b0| 64 64 69 74 69 6f 6e 0d | 0a 74 6f 20 67 65 6e 65 |ddition.|.to gene|
|000042c0| 72 61 6c 20 73 75 70 70 | 6f 72 74 69 76 65 20 6d |ral supp|ortive m|
|000042d0| 65 61 73 75 72 65 73 20 | 73 75 63 68 20 61 73 20 |easures |such as |
|000042e0| 62 6c 6f 6f 64 20 74 72 | 61 6e 73 66 75 73 69 6f |blood tr|ansfusio|
|000042f0| 6e 73 2c 20 61 6e 74 69 | 68 79 70 65 72 74 65 6e |ns, anti|hyperten|
|00004300| 73 69 76 65 0d 0a 64 72 | 75 67 73 2c 20 61 6e 64 |sive..dr|ugs, and|
|00004310| 20 64 69 61 6c 79 73 69 | 73 2c 20 73 65 76 65 72 | dialysi|s, sever|
|00004320| 61 6c 20 73 70 65 63 69 | 66 69 63 20 74 79 70 65 |al speci|fic type|
|00004330| 73 20 6f 66 20 74 68 65 | 72 61 70 79 20 68 61 76 |s of the|rapy hav|
|00004340| 65 20 62 65 65 6e 20 61 | 64 76 6f 63 61 74 65 64 |e been a|dvocated|
|00004350| 3a 0d 0a 70 68 61 72 6d | 61 63 6f 6c 6f 67 69 63 |:..pharm|acologic|
|00004360| 20 61 67 65 6e 74 73 20 | 73 75 63 68 20 61 73 20 | agents |such as |
|00004370| 61 6e 74 69 63 6f 61 67 | 75 6c 61 6e 74 73 2c 20 |anticoag|ulants, |
|00004380| 61 6e 74 69 70 6c 61 74 | 65 6c 65 74 20 61 6e 64 |antiplat|elet and|
|00004390| 20 74 68 72 6f 6d 62 6f | 6c 79 74 69 63 0d 0a 61 | thrombo|lytic..a|
|000043a0| 67 65 6e 74 73 2c 20 70 | 72 6f 73 74 61 63 79 63 |gents, p|rostacyc|
|000043b0| 6c 69 6e 2c 20 61 6e 64 | 20 76 69 74 61 6d 69 6e |lin, and| vitamin|
|000043c0| 20 45 2c 20 61 73 20 77 | 65 6c 6c 20 61 73 20 65 | E, as w|ell as e|
|000043d0| 78 63 68 61 6e 67 65 20 | 74 72 61 6e 73 66 75 73 |xchange |transfus|
|000043e0| 69 6f 6e 2c 20 66 72 65 | 73 68 0d 0a 66 72 6f 7a |ion, fre|sh..froz|
|000043f0| 65 6e 20 70 6c 61 73 6d | 61 2c 20 61 6e 64 20 70 |en plasm|a, and p|
|00004400| 6c 61 73 6d 61 70 68 65 | 72 65 73 69 73 2e 20 20 |lasmaphe|resis. |
|00004410| 41 20 63 65 6e 74 65 72 | 20 69 6e 20 42 65 6c 67 |A center| in Belg|
|00004420| 69 75 6d 20 65 76 65 6e | 20 77 65 6e 74 20 74 6f |ium even| went to|
|00004430| 20 74 68 65 0d 0a 65 78 | 74 72 65 6d 65 73 20 6f | the..ex|tremes o|
|00004440| 66 20 64 6f 69 6e 67 20 | 61 20 31 30 2d 79 65 61 |f doing |a 10-yea|
|00004450| 72 20 65 66 66 69 63 61 | 63 79 20 73 74 75 64 79 |r effica|cy study|
|00004460| 20 6f 66 20 68 65 70 61 | 72 69 6e 20 70 6c 75 73 | of hepa|rin plus|
|00004470| 20 64 69 70 79 72 69 64 | 61 6d 6f 6c 65 20 28 61 | dipyrid|amole (a|
|00004480| 6e 0d 0a 61 6e 74 69 70 | 6c 61 74 65 6c 65 74 20 |n..antip|latelet |
|00004490| 64 72 75 67 29 20 69 6e | 20 74 68 69 73 20 73 79 |drug) in| this sy|
|000044a0| 6e 64 72 6f 6d 65 2e 20 | 20 54 68 65 79 20 66 6f |ndrome. | They fo|
|000044b0| 75 6e 64 20 6e 6f 20 65 | 66 66 65 63 74 20 28 31 |und no e|ffect (1|
|000044c0| 29 2c 20 62 75 74 20 61 | 74 20 6c 65 61 73 74 0d |), but a|t least.|
|000044d0| 0a 74 68 65 79 20 61 72 | 65 20 74 6f 20 62 65 20 |.they ar|e to be |
|000044e0| 63 72 65 64 69 74 65 64 | 20 66 6f 72 20 74 68 65 |credited| for the|
|000044f0| 69 72 20 70 65 72 73 69 | 73 74 65 6e 63 65 2e 0d |ir persi|stence..|
|00004500| 0a 20 20 20 20 20 49 74 | 27 73 20 6e 6f 74 20 68 |. It|'s not h|
|00004510| 61 72 64 20 74 6f 20 75 | 6e 64 65 72 73 74 61 6e |ard to u|nderstan|
|00004520| 64 20 77 68 79 20 6e 6f | 20 6f 6e 65 20 74 68 65 |d why no| one the|
|00004530| 72 61 70 79 20 68 61 73 | 20 63 6c 65 61 72 6c 79 |rapy has| clearly|
|00004540| 20 65 6d 65 72 67 65 64 | 20 61 73 20 74 68 65 0d | emerged| as the.|
|00004550| 0a 64 65 66 69 6e 69 74 | 69 76 65 20 74 68 65 72 |.definit|ive ther|
|00004560| 61 70 79 20 66 6f 72 20 | 48 55 53 2e 20 20 57 68 |apy for |HUS. Wh|
|00004570| 61 74 20 77 65 20 63 61 | 6c 6c 20 63 6c 61 73 73 |at we ca|ll class|
|00004580| 69 63 20 63 68 69 6c 64 | 68 6f 6f 64 20 48 55 53 |ic child|hood HUS|
|00004590| 20 6d 61 79 20 69 6e 20 | 66 61 63 74 0d 0a 72 65 | may in |fact..re|
|000045a0| 70 72 65 73 65 6e 74 20 | 61 20 6e 75 6d 62 65 72 |present |a number|
|000045b0| 20 6f 66 20 73 75 62 73 | 65 74 73 20 6f 66 20 76 | of subs|ets of v|
|000045c0| 61 72 79 69 6e 67 20 64 | 69 73 6f 72 64 65 72 73 |arying d|isorders|
|000045d0| 2e 20 20 49 6e 20 63 6c | 61 73 73 69 63 20 48 55 |. In cl|assic HU|
|000045e0| 53 20 74 68 65 72 65 20 | 69 73 0d 0a 69 6e 63 72 |S there |is..incr|
|000045f0| 65 61 73 69 6e 67 20 65 | 76 69 64 65 6e 63 65 20 |easing e|vidence |
|00004600| 74 68 61 74 20 74 68 65 | 20 64 69 73 6f 72 64 65 |that the| disorde|
|00004610| 72 20 69 73 20 69 6e 69 | 74 69 61 74 65 64 20 62 |r is ini|tiated b|
|00004620| 79 20 62 61 63 74 65 72 | 69 61 6c 20 74 6f 78 69 |y bacter|ial toxi|
|00004630| 6e 73 2e 20 20 49 6e 0d | 0a 73 6f 6d 65 20 72 65 |ns. In.|.some re|
|00004640| 67 69 6f 6e 73 20 6f 66 | 20 4e 6f 72 74 68 20 41 |gions of| North A|
|00004650| 6d 65 72 69 63 61 2c 20 | 66 6f 72 20 65 78 61 6d |merica, |for exam|
|00004660| 70 6c 65 2c 20 6d 61 6e | 79 20 63 61 73 65 73 20 |ple, man|y cases |
|00004670| 61 72 65 20 70 72 65 63 | 65 64 65 64 20 62 79 20 |are prec|eded by |
|00004680| 65 6e 74 65 72 69 63 0d | 0a 69 6e 66 65 63 74 69 |enteric.|.infecti|
|00004690| 6f 6e 73 20 63 61 75 73 | 65 64 20 62 79 20 76 69 |ons caus|ed by vi|
|000046a0| 72 6f 74 6f 78 69 6e 2d | 70 72 6f 64 75 63 69 6e |rotoxin-|producin|
|000046b0| 67 20 45 73 63 68 65 72 | 69 63 68 69 61 20 63 6f |g Escher|ichia co|
|000046c0| 6c 69 2e 20 20 54 68 65 | 72 65 20 69 73 0d 0a 73 |li. The|re is..s|
|000046d0| 70 65 63 75 6c 61 74 69 | 6f 6e 20 74 68 61 74 20 |peculati|on that |
|000046e0| 76 69 72 6f 74 6f 78 69 | 6e 20 67 61 69 6e 73 20 |virotoxi|n gains |
|000046f0| 61 63 63 65 73 73 20 74 | 6f 20 74 68 65 20 63 69 |access t|o the ci|
|00004700| 72 63 75 6c 61 74 69 6f | 6e 20 61 6e 64 20 64 69 |rculatio|n and di|
|00004710| 73 74 75 72 62 73 20 74 | 68 65 0d 0a 72 65 6e 61 |sturbs t|he..rena|
|00004720| 6c 20 76 61 73 63 75 6c | 61 72 20 65 6e 64 6f 74 |l vascul|ar endot|
|00004730| 68 65 6c 69 75 6d 2e 20 | 20 49 6e 20 6f 74 68 65 |helium. | In othe|
|00004740| 72 20 70 61 72 74 73 20 | 6f 66 20 74 68 65 20 77 |r parts |of the w|
|00004750| 6f 72 6c 64 2c 20 69 6e | 66 65 63 74 69 6f 6e 73 |orld, in|fections|
|00004760| 20 77 69 74 68 0d 0a 53 | 68 69 67 65 6c 6c 61 20 | with..S|higella |
|00004770| 6f 72 20 53 61 6c 6d 6f | 6e 65 6c 6c 61 20 70 72 |or Salmo|nella pr|
|00004780| 6f 64 75 63 65 20 61 20 | 73 69 6d 69 6c 61 72 20 |oduce a |similar |
|00004790| 74 6f 78 69 6e 2e 20 20 | 54 6f 20 6c 65 61 72 6e |toxin. |To learn|
|000047a0| 20 6d 6f 72 65 20 61 62 | 6f 75 74 20 74 68 65 0d | more ab|out the.|
|000047b0| 0a 6d 61 6e 61 67 65 6d | 65 6e 74 20 6f 66 20 74 |.managem|ent of t|
|000047c0| 68 69 73 20 64 69 73 6f | 72 64 65 72 2c 20 72 65 |his diso|rder, re|
|000047d0| 61 64 20 74 68 65 20 73 | 75 70 65 72 62 20 63 6f |ad the s|uperb co|
|000047e0| 6d 6d 65 6e 74 61 72 79 | 20 6f 66 20 53 69 65 67 |mmentary| of Sieg|
|000047f0| 6c 65 72 20 28 32 29 2e | 20 20 59 6f 75 0d 0a 77 |ler (2).| You..w|
|00004800| 69 6c 6c 20 66 69 6e 64 | 20 61 20 62 61 6c 61 6e |ill find| a balan|
|00004810| 63 65 64 20 64 69 73 63 | 75 73 73 69 6f 6e 20 6f |ced disc|ussion o|
|00004820| 66 20 61 6c 6c 20 74 68 | 65 20 74 68 65 72 61 70 |f all th|e therap|
|00004830| 69 65 73 20 74 68 61 74 | 20 68 61 76 65 20 62 65 |ies that| have be|
|00004840| 65 6e 20 74 72 69 65 64 | 2c 0d 0a 69 6e 63 6c 75 |en tried|,..inclu|
|00004850| 64 69 6e 67 20 74 68 6f | 73 65 20 6d 65 6e 74 69 |ding tho|se menti|
|00004860| 6f 6e 65 64 20 61 62 6f | 76 65 20 70 6c 75 73 20 |oned abo|ve plus |
|00004870| 66 69 62 72 69 6e 6f 6c | 79 74 69 63 20 61 67 65 |fibrinol|ytic age|
|00004880| 6e 74 73 2c 20 69 6e 74 | 72 61 76 65 6e 6f 75 73 |nts, int|ravenous|
|00004890| 20 49 67 47 0d 0a 69 6e | 66 75 73 69 6f 6e 73 2c | IgG..in|fusions,|
|000048a0| 20 61 6e 64 20 6f 74 68 | 65 72 20 61 73 73 6f 72 | and oth|er assor|
|000048b0| 74 65 64 20 65 6e 74 69 | 74 69 65 73 2e 20 20 53 |ted enti|ties. S|
|000048c0| 69 65 67 6c 65 72 20 63 | 6f 6e 63 6c 75 64 65 73 |iegler c|oncludes|
|000048d0| 20 68 69 73 20 63 6f 6d | 6d 65 6e 74 61 72 79 20 | his com|mentary |
|000048e0| 6f 6e 0d 0a 74 68 69 73 | 20 74 6f 70 69 63 20 62 |on..this| topic b|
|000048f0| 79 20 73 74 61 74 69 6e | 67 20 74 68 61 74 20 63 |y statin|g that c|
|00004900| 61 72 65 66 75 6c 20 61 | 74 74 65 6e 74 69 6f 6e |areful a|ttention|
|00004910| 20 74 6f 20 74 68 65 20 | 6d 61 6e 61 67 65 6d 65 | to the |manageme|
|00004920| 6e 74 20 6f 66 20 74 68 | 65 20 72 65 6e 61 6c 0d |nt of th|e renal.|
|00004930| 0a 66 61 69 6c 75 72 65 | 20 77 69 74 68 20 61 6c |.failure| with al|
|00004940| 6c 20 6f 66 20 69 74 73 | 20 73 69 67 6e 73 20 61 |l of its| signs a|
|00004950| 6e 64 20 73 79 6d 70 74 | 6f 6d 73 20 69 73 20 73 |nd sympt|oms is s|
|00004960| 74 69 6c 6c 20 74 68 65 | 20 6d 61 69 6e 73 74 61 |till the| mainsta|
|00004970| 79 20 6f 66 20 74 72 65 | 61 74 6d 65 6e 74 2c 0d |y of tre|atment,.|
|00004980| 0a 61 6e 64 20 74 68 61 | 74 20 69 6e 76 65 73 74 |.and tha|t invest|
|00004990| 69 67 61 74 69 76 65 20 | 74 68 65 72 61 70 69 65 |igative |therapie|
|000049a0| 73 20 73 68 6f 75 6c 64 | 20 62 65 20 6c 69 6d 69 |s should| be limi|
|000049b0| 74 65 64 20 74 6f 20 6c | 61 72 67 65 72 20 48 55 |ted to l|arger HU|
|000049c0| 53 20 63 65 6e 74 65 72 | 73 20 6f 72 0d 0a 6d 75 |S center|s or..mu|
|000049d0| 6c 74 69 63 65 6e 74 65 | 72 20 63 6f 6c 6c 61 62 |lticente|r collab|
|000049e0| 6f 72 61 74 69 76 65 20 | 74 72 69 61 6c 73 20 69 |orative |trials i|
|000049f0| 66 20 77 65 20 61 72 65 | 20 65 76 65 72 20 67 6f |f we are| ever go|
|00004a00| 69 6e 67 20 74 6f 20 61 | 63 63 75 6d 75 6c 61 74 |ing to a|ccumulat|
|00004a10| 65 0d 0a 73 74 61 74 69 | 73 74 69 63 61 6c 6c 79 |e..stati|stically|
|00004a20| 20 6d 65 61 6e 69 6e 67 | 66 75 6c 20 64 61 74 61 | meaning|ful data|
|00004a30| 2e 20 20 49 20 64 6f 6e | 27 74 20 62 65 6c 69 65 |. I don|'t belie|
|00004a40| 76 65 20 74 68 61 74 20 | 74 68 69 73 20 63 6f 75 |ve that |this cou|
|00004a50| 6c 64 20 68 61 76 65 20 | 62 65 65 6e 20 73 61 69 |ld have |been sai|
|00004a60| 64 0d 0a 61 6e 79 20 62 | 65 74 74 65 72 2e 2d 2d |d..any b|etter.--|
|00004a70| 4a 2e 41 2e 20 53 74 6f | 63 6b 6d 61 6e 2c 20 49 |J.A. Sto|ckman, I|
|00004a80| 49 49 2c 20 4d 2e 44 2e | 0d 0a 24 55 6e 69 71 75 |II, M.D.|..$Uniqu|
|00004a90| 65 5f 49 44 7b 59 52 42 | 31 32 30 30 33 7d 0d 0a |e_ID{YRB|12003}..|
|00004aa0| 24 54 69 74 6c 65 7b 53 | 69 6d 70 6c 69 66 69 65 |$Title{S|implifie|
|00004ab0| 64 20 51 75 61 6e 74 69 | 66 69 63 61 74 69 6f 6e |d Quanti|fication|
|00004ac0| 20 6f 66 20 55 72 69 6e | 61 72 79 20 50 72 6f 74 | of Urin|ary Prot|
|00004ad0| 65 69 6e 20 45 78 63 72 | 65 74 69 6f 6e 20 69 6e |ein Excr|etion in|
|00004ae0| 20 43 68 69 6c 64 72 65 | 6e 7d 0d 0a 24 41 75 74 | Childre|n}..$Aut|
|00004af0| 68 6f 72 7b 45 6c 69 73 | 65 73 2c 20 4a 53 0d 0a |hor{Elis|es, JS..|
|00004b00| 47 72 69 66 66 69 74 68 | 73 2c 20 50 44 0d 0a 48 |Griffith|s, PD..H|
|00004b10| 6f 63 6b 69 6e 67 2c 20 | 4d 44 0d 0a 54 61 79 6c |ocking, |MD..Tayl|
|00004b20| 6f 72 2c 20 43 4d 0d 0a | 57 68 69 74 65 2c 20 52 |or, CM..|White, R|
|00004b30| 48 52 7d 0d 0a 24 41 66 | 66 69 6c 69 61 74 69 6f |HR}..$Af|filiatio|
|00004b40| 6e 7b 54 68 65 20 43 68 | 69 6c 64 72 65 6e 27 73 |n{The Ch|ildren's|
|00004b50| 20 48 6f 73 70 2c 20 42 | 69 72 6d 69 6e 67 68 61 | Hosp, B|irmingha|
|00004b60| 6d 2c 20 45 6e 67 6c 61 | 6e 64 7d 0d 0a 24 4a 6f |m, Engla|nd}..$Jo|
|00004b70| 75 72 6e 61 6c 7b 43 6c | 69 6e 20 4e 65 70 68 72 |urnal{Cl|in Nephr|
|00004b80| 6f 6c 7d 0d 0a 24 56 6f | 6c 75 6d 65 7b 33 30 7d |ol}..$Vo|lume{30}|
|00004b90| 0d 0a 24 50 61 67 65 7b | 32 32 35 2d 32 32 39 7d |..$Page{|225-229}|
|00004ba0| 0d 0a 24 44 61 74 65 7b | 4f 63 74 6f 62 65 72 20 |..$Date{|October |
|00004bb0| 31 39 38 38 7d 0d 0a 24 | 59 42 56 6f 6c 7b 31 39 |1988}..$|YBVol{19|
|00004bc0| 39 30 20 59 65 61 72 20 | 42 6f 6f 6b 20 6f 66 20 |90 Year |Book of |
|00004bd0| 50 65 64 69 61 74 72 69 | 63 73 7d 0d 0a 24 43 68 |Pediatri|cs}..$Ch|
|00004be0| 61 70 74 65 72 7b 31 30 | 2d 31 33 7d 0d 0a 24 59 |apter{10|-13}..$Y|
|00004bf0| 42 45 64 69 74 6f 72 7b | 44 72 73 2e 20 4f 73 6b |BEditor{|Drs. Osk|
|00004c00| 69 20 61 6e 64 20 53 74 | 6f 63 6b 6d 61 6e 7d 0d |i and St|ockman}.|
|00004c10| 0a 24 54 61 62 6c 65 7b | 54 61 62 6c 65 20 4f 6d |.$Table{|Table Om|
|00004c20| 69 74 74 65 64 7d 0d 0a | 24 43 6f 6d 6d 65 6e 74 |itted}..|$Comment|
|00004c30| 61 74 6f 72 7b 43 2e 42 | 2e 20 4c 61 6e 67 6d 61 |ator{C.B|. Langma|
|00004c40| 6e 2c 20 4d 2e 44 2e 7d | 0d 0a 0d 0a 43 6f 70 79 |n, M.D.}|....Copy|
|00004c50| 72 69 67 68 74 20 28 63 | 29 20 59 65 61 72 62 6f |right (c|) Yearbo|
|00004c60| 6f 6b 20 4d 65 64 69 63 | 61 6c 20 50 75 62 6c 69 |ok Medic|al Publi|
|00004c70| 73 68 65 72 73 2c 20 49 | 6e 63 2e 2c 20 31 39 39 |shers, I|nc., 199|
|00004c80| 30 0d 0a 0d 0a 31 39 39 | 30 20 59 65 61 72 20 42 |0....199|0 Year B|
|00004c90| 6f 6f 6b 20 6f 66 20 50 | 65 64 69 61 74 72 69 63 |ook of P|ediatric|
|00004ca0| 73 20 28 52 29 0d 0a 0d | 0a 43 68 61 70 74 65 72 |s (R)...|.Chapter|
|00004cb0| 20 31 30 2d 2d 54 68 65 | 20 47 65 6e 69 74 6f 75 | 10--The| Genitou|
|00004cc0| 72 69 6e 61 72 79 20 54 | 72 61 63 74 0d 0a 0d 0a |rinary T|ract....|
|00004cd0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004ce0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004cf0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004d00| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004d10| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 0d 0a |--------|------..|
|00004d20| 53 69 6d 70 6c 69 66 69 | 65 64 20 51 75 61 6e 74 |Simplifi|ed Quant|
|00004d30| 69 66 69 63 61 74 69 6f | 6e 20 6f 66 20 55 72 69 |ificatio|n of Uri|
|00004d40| 6e 61 72 79 20 50 72 6f | 74 65 69 6e 20 45 78 63 |nary Pro|tein Exc|
|00004d50| 72 65 74 69 6f 6e 20 69 | 6e 20 43 68 69 6c 64 72 |retion i|n Childr|
|00004d60| 65 6e 0d 0a 0d 0a 45 6c | 69 73 65 73 20 4a 53 2c |en....El|ises JS,|
|00004d70| 20 47 72 69 66 66 69 74 | 68 73 20 50 44 2c 20 48 | Griffit|hs PD, H|
|00004d80| 6f 63 6b 69 6e 67 20 4d | 44 2c 20 54 61 79 6c 6f |ocking M|D, Taylo|
|00004d90| 72 20 43 4d 2c 20 57 68 | 69 74 65 20 52 48 52 0d |r CM, Wh|ite RHR.|
|00004da0| 0a 28 54 68 65 20 43 68 | 69 6c 64 72 65 6e 27 73 |.(The Ch|ildren's|
|00004db0| 20 48 6f 73 70 2c 20 42 | 69 72 6d 69 6e 67 68 61 | Hosp, B|irmingha|
|00004dc0| 6d 2c 20 45 6e 67 6c 61 | 6e 64 29 0d 0a 43 6c 69 |m, Engla|nd)..Cli|
|00004dd0| 6e 20 4e 65 70 68 72 6f | 6c 20 33 30 3a 32 32 35 |n Nephro|l 30:225|
|00004de0| 2d 32 32 39 2c 20 4f 63 | 74 6f 62 65 72 20 31 39 |-229, Oc|tober 19|
|00004df0| 38 38 0d 0a 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |88..----|--------|
|00004e00| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004e10| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004e20| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004e30| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004e40| 2d 2d 0d 0a 0d 0a 41 62 | 73 74 72 61 63 74 0d 0a |--....Ab|stract..|
|00004e50| 0d 0a 20 20 20 20 20 43 | 68 61 6e 67 65 73 20 69 |.. C|hanges i|
|00004e60| 6e 20 75 72 69 6e 61 72 | 79 20 70 72 6f 74 65 69 |n urinar|y protei|
|00004e70| 6e 20 65 78 63 72 65 74 | 69 6f 6e 20 70 72 6f 76 |n excret|ion prov|
|00004e80| 69 64 65 20 69 6d 70 6f | 72 74 61 6e 74 20 69 6e |ide impo|rtant in|
|00004e90| 66 6f 72 6d 61 74 69 6f | 6e 20 61 62 6f 75 74 0d |formatio|n about.|
|00004ea0| 0a 74 68 65 20 63 6c 69 | 6e 69 63 61 6c 20 63 6f |.the cli|nical co|
|00004eb0| 75 72 73 65 20 61 6e 64 | 20 70 72 6f 67 6e 6f 73 |urse and| prognos|
|00004ec0| 69 73 20 69 6e 20 70 61 | 74 69 65 6e 74 73 20 77 |is in pa|tients w|
|00004ed0| 69 74 68 20 72 65 6e 61 | 6c 20 64 69 73 65 61 73 |ith rena|l diseas|
|00004ee0| 65 2e 0d 0a 51 75 61 6e | 74 69 66 69 63 61 74 69 |e...Quan|tificati|
|00004ef0| 6f 6e 20 6f 66 20 70 72 | 6f 74 65 69 6e 75 72 69 |on of pr|oteinuri|
|00004f00| 61 20 68 61 73 20 74 72 | 61 64 69 74 69 6f 6e 61 |a has tr|aditiona|
|00004f10| 6c 6c 79 20 72 65 71 75 | 69 72 65 64 20 74 69 6d |lly requ|ired tim|
|00004f20| 65 64 20 75 72 69 6e 65 | 0d 0a 63 6f 6c 6c 65 63 |ed urine|..collec|
|00004f30| 74 69 6f 6e 2e 20 20 42 | 65 63 61 75 73 65 20 6d |tion. B|ecause m|
|00004f40| 69 6c 64 20 6f 72 74 68 | 6f 73 74 61 74 69 63 20 |ild orth|ostatic |
|00004f50| 6f 72 20 61 63 74 69 76 | 69 74 79 2d 72 65 6c 61 |or activ|ity-rela|
|00004f60| 74 65 64 20 70 72 6f 74 | 65 69 6e 75 72 69 61 20 |ted prot|einuria |
|00004f70| 69 73 0d 0a 63 6f 6d 6d | 6f 6e 2c 20 69 74 20 68 |is..comm|on, it h|
|00004f80| 61 73 20 62 65 63 6f 6d | 65 20 73 74 61 6e 64 61 |as becom|e standa|
|00004f90| 72 64 20 70 72 61 63 74 | 69 63 65 20 74 6f 20 71 |rd pract|ice to q|
|00004fa0| 75 61 6e 74 69 66 79 20 | 75 72 69 6e 61 72 79 20 |uantify |urinary |
|00004fb0| 70 72 6f 74 65 69 6e 20 | 65 78 63 72 65 74 69 6f |protein |excretio|
|00004fc0| 6e 0d 0a 75 6e 64 65 72 | 20 62 61 73 61 6c 20 63 |n..under| basal c|
|00004fd0| 6f 6e 64 69 74 69 6f 6e | 73 2c 20 75 73 69 6e 67 |ondition|s, using|
|00004fe0| 20 74 69 6d 65 64 20 6f | 76 65 72 6e 69 67 68 74 | timed o|vernight|
|00004ff0| 20 75 72 69 6e 65 20 73 | 61 6d 70 6c 65 20 63 6f | urine s|ample co|
|00005000| 6c 6c 65 63 74 69 6f 6e | 2e 20 20 54 68 69 73 0d |llection|. This.|
|00005010| 0a 6d 65 74 68 6f 64 20 | 70 72 65 73 65 6e 74 73 |.method |presents|
|00005020| 20 6c 6f 67 69 73 74 69 | 63 61 6c 20 70 72 6f 62 | logisti|cal prob|
|00005030| 6c 65 6d 73 2c 20 70 61 | 72 74 69 63 75 6c 61 72 |lems, pa|rticular|
|00005040| 6c 79 20 69 6e 20 79 6f | 75 6e 67 20 63 68 69 6c |ly in yo|ung chil|
|00005050| 64 72 65 6e 20 77 68 6f | 20 61 72 65 0d 0a 6e 6f |dren who| are..no|
|00005060| 74 20 79 65 74 20 63 6f | 6e 74 69 6e 65 6e 74 20 |t yet co|ntinent |
|00005070| 61 74 20 6e 69 67 68 74 | 2e 20 20 50 72 65 76 69 |at night|. Previ|
|00005080| 6f 75 73 20 73 74 75 64 | 69 65 73 20 68 61 76 65 |ous stud|ies have|
|00005090| 20 73 68 6f 77 6e 20 74 | 68 61 74 20 69 6e 20 61 | shown t|hat in a|
|000050a0| 64 75 6c 74 73 2c 20 74 | 68 65 0d 0a 72 61 6e 64 |dults, t|he..rand|
|000050b0| 6f 6d 20 75 72 69 6e 65 | 20 70 72 6f 74 65 69 6e |om urine| protein|
|000050c0| 2f 63 72 65 61 74 69 6e | 69 6e 65 20 72 61 74 69 |/creatin|ine rati|
|000050d0| 6f 20 28 55 70 2f 55 63 | 29 20 63 6f 72 72 65 6c |o (Up/Uc|) correl|
|000050e0| 61 74 65 73 20 77 65 6c | 6c 20 77 69 74 68 20 74 |ates wel|l with t|
|000050f0| 69 6d 65 64 0d 0a 70 72 | 6f 74 65 69 6e 20 65 78 |imed..pr|otein ex|
|00005100| 63 72 65 74 69 6f 6e 20 | 6d 65 61 73 75 72 65 6d |cretion |measurem|
|00005110| 65 6e 74 73 2e 20 20 54 | 68 65 20 72 65 6c 61 74 |ents. T|he relat|
|00005120| 69 6f 6e 73 68 69 70 20 | 62 65 74 77 65 65 6e 20 |ionship |between |
|00005130| 74 68 65 20 55 70 2f 55 | 63 20 61 6e 64 20 74 69 |the Up/U|c and ti|
|00005140| 6d 65 0d 0a 6f 76 65 72 | 6e 69 67 68 74 20 75 72 |me..over|night ur|
|00005150| 69 6e 65 20 70 72 6f 74 | 65 69 6e 20 65 78 63 72 |ine prot|ein excr|
|00005160| 65 74 69 6f 6e 20 77 61 | 73 20 61 73 73 65 73 73 |etion wa|s assess|
|00005170| 65 64 2e 0d 0a 20 20 20 | 20 20 54 68 65 20 73 74 |ed... | The st|
|00005180| 75 64 79 20 6d 61 74 65 | 72 69 61 6c 20 63 6f 6e |udy mate|rial con|
|00005190| 73 69 73 74 65 64 20 6f | 66 20 37 31 20 65 61 72 |sisted o|f 71 ear|
|000051a0| 6c 79 20 6d 6f 72 6e 69 | 6e 67 20 75 72 69 6e 65 |ly morni|ng urine|
|000051b0| 20 73 61 6d 70 6c 65 73 | 20 63 6f 6c 6c 65 63 74 | samples| collect|
|000051c0| 65 64 0d 0a 66 72 6f 6d | 20 36 36 20 70 61 74 69 |ed..from| 66 pati|
|000051d0| 65 6e 74 73 20 61 67 65 | 64 20 33 2d 32 33 20 79 |ents age|d 3-23 y|
|000051e0| 65 61 72 73 2c 20 6f 66 | 20 77 68 6f 6d 20 33 38 |ears, of| whom 38|
|000051f0| 20 68 61 64 20 61 63 74 | 69 76 65 20 70 72 69 6d | had act|ive prim|
|00005200| 61 72 79 20 67 6c 6f 6d | 65 72 75 6c 61 72 0d 0a |ary glom|erular..|
|00005210| 64 69 73 65 61 73 65 20 | 61 6e 64 20 32 38 20 68 |disease |and 28 h|
|00005220| 61 64 20 65 69 74 68 65 | 72 20 63 68 72 6f 6e 69 |ad eithe|r chroni|
|00005230| 63 20 72 65 6e 61 6c 20 | 66 61 69 6c 75 72 65 20 |c renal |failure |
|00005240| 6f 72 20 6f 74 68 65 72 | 20 6b 69 64 6e 65 79 20 |or other| kidney |
|00005250| 64 69 73 6f 72 64 65 72 | 73 2e 20 20 49 6e 0d 0a |disorder|s. In..|
|00005260| 61 64 64 69 74 69 6f 6e | 2c 20 33 37 37 20 63 6f |addition|, 377 co|
|00005270| 6e 74 72 6f 6c 20 65 61 | 72 6c 79 20 6d 6f 72 6e |ntrol ea|rly morn|
|00005280| 69 6e 67 20 75 72 69 6e | 65 20 73 61 6d 70 6c 65 |ing urin|e sample|
|00005290| 73 20 77 65 72 65 20 63 | 6f 6c 6c 65 63 74 65 64 |s were c|ollected|
|000052a0| 20 66 72 6f 6d 20 68 65 | 61 6c 74 68 79 0d 0a 63 | from he|althy..c|
|000052b0| 68 69 6c 64 72 65 6e 20 | 61 6e 64 20 61 64 6f 6c |hildren |and adol|
|000052c0| 65 73 63 65 6e 74 73 20 | 61 67 65 64 20 33 2d 31 |escents |aged 3-1|
|000052d0| 39 20 79 65 61 72 73 2e | 20 20 53 69 78 74 65 65 |9 years.| Sixtee|
|000052e0| 6e 20 6f 74 68 65 72 20 | 68 65 61 6c 74 68 79 20 |n other |healthy |
|000052f0| 63 68 69 6c 64 72 65 6e | 20 61 67 65 64 0d 0a 33 |children| aged..3|
|00005300| 2d 31 39 20 79 65 61 72 | 73 20 70 72 6f 76 69 64 |-19 year|s provid|
|00005310| 65 64 20 65 61 72 6c 79 | 20 6d 6f 72 6e 69 6e 67 |ed early| morning|
|00005320| 20 75 72 69 6e 65 20 73 | 61 6d 70 6c 65 73 20 6f | urine s|amples o|
|00005330| 76 65 72 20 34 2d 37 20 | 63 6f 6e 73 65 63 75 74 |ver 4-7 |consecut|
|00005340| 69 76 65 20 64 61 79 73 | 2e 0d 0a 20 20 20 20 20 |ive days|... |
|00005350| 54 68 65 20 55 70 2f 55 | 63 20 6d 65 61 73 75 72 |The Up/U|c measur|
|00005360| 65 64 20 69 6e 20 37 31 | 20 65 61 72 6c 79 20 6d |ed in 71| early m|
|00005370| 6f 72 6e 69 6e 67 20 75 | 72 69 6e 65 20 73 61 6d |orning u|rine sam|
|00005380| 70 6c 65 73 20 63 6f 6c | 6c 65 63 74 65 64 20 66 |ples col|lected f|
|00005390| 72 6f 6d 0d 0a 70 61 74 | 69 65 6e 74 73 20 77 69 |rom..pat|ients wi|
|000053a0| 74 68 20 72 65 6e 61 6c | 20 64 69 73 6f 72 64 65 |th renal| disorde|
|000053b0| 72 73 20 63 6f 72 72 65 | 6c 61 74 65 64 20 63 6c |rs corre|lated cl|
|000053c0| 6f 73 65 6c 79 20 77 69 | 74 68 20 74 69 6d 65 64 |osely wi|th timed|
|000053d0| 20 6f 76 65 72 6e 69 67 | 68 74 20 75 72 69 6e 65 | overnig|ht urine|
|000053e0| 0d 0a 70 72 6f 74 65 69 | 6e 20 65 78 63 72 65 74 |..protei|n excret|
|000053f0| 69 6f 6e 20 72 61 74 65 | 73 2e 20 20 54 68 65 20 |ion rate|s. The |
|00005400| 72 65 6c 61 74 69 6f 6e | 73 68 69 70 20 77 61 73 |relation|ship was|
|00005410| 20 6c 69 6e 65 61 72 20 | 74 68 72 6f 75 67 68 6f | linear |througho|
|00005420| 75 74 20 74 68 65 20 65 | 6e 74 69 72 65 0d 0a 72 |ut the e|ntire..r|
|00005430| 61 6e 67 65 20 6f 66 20 | 6e 6f 72 6d 61 6c 20 61 |ange of |normal a|
|00005440| 6e 64 20 61 62 6e 6f 72 | 6d 61 6c 20 70 72 6f 74 |nd abnor|mal prot|
|00005450| 65 69 6e 20 65 78 63 72 | 65 74 69 6f 6e 20 28 46 |ein excr|etion (F|
|00005460| 69 67 20 31 30 2d 32 29 | 2e 20 20 54 68 65 20 75 |ig 10-2)|. The u|
|00005470| 70 70 65 72 20 6c 69 6d | 69 74 20 6f 66 0d 0a 6e |pper lim|it of..n|
|00005480| 6f 72 6d 61 6c 20 66 6f | 72 20 74 68 65 20 55 70 |ormal fo|r the Up|
|00005490| 2f 55 63 20 6d 65 61 73 | 75 72 65 64 20 69 6e 20 |/Uc meas|ured in |
|000054a0| 74 68 65 20 33 37 37 20 | 6e 6f 72 6d 61 6c 20 63 |the 377 |normal c|
|000054b0| 6f 6e 74 72 6f 6c 20 65 | 61 72 6c 79 20 6d 6f 72 |ontrol e|arly mor|
|000054c0| 6e 69 6e 67 20 75 72 69 | 6e 65 0d 0a 73 61 6d 70 |ning uri|ne..samp|
|000054d0| 6c 65 73 20 77 61 73 20 | 32 30 20 6d 67 2f 6d 6d |les was |20 mg/mm|
|000054e0| 6f 6c 2e 20 20 49 6e 20 | 6e 6f 72 6d 61 6c 20 61 |ol. In |normal a|
|000054f0| 64 75 6c 74 73 20 74 68 | 65 20 75 70 70 65 72 20 |dults th|e upper |
|00005500| 6c 69 6d 69 74 20 6f 66 | 20 6e 6f 72 6d 61 6c 20 |limit of| normal |
|00005510| 66 6f 72 20 74 68 65 0d | 0a 72 61 6e 64 6f 6d 20 |for the.|.random |
|00005520| 75 72 69 6e 65 20 55 70 | 2f 55 63 20 72 61 6e 67 |urine Up|/Uc rang|
|00005530| 65 73 20 62 65 74 77 65 | 65 6e 20 31 32 2e 35 20 |es betwe|en 12.5 |
|00005540| 61 6e 64 20 32 32 2e 36 | 20 6d 67 2f 6d 6d 6f 6c |and 22.6| mg/mmol|
|00005550| 2e 0d 0a 20 20 20 20 20 | 43 6f 6d 70 61 72 65 64 |... |Compared|
|00005560| 20 77 69 74 68 20 74 69 | 6d 65 64 20 6f 76 65 72 | with ti|med over|
|00005570| 6e 69 67 68 74 20 75 72 | 69 6e 65 20 73 61 6d 70 |night ur|ine samp|
|00005580| 6c 65 20 63 6f 6c 6c 65 | 63 74 69 6f 6e 2c 20 64 |le colle|ction, d|
|00005590| 65 74 65 72 6d 69 6e 61 | 74 69 6f 6e 20 6f 66 0d |etermina|tion of.|
|000055a0| 0a 74 68 65 20 55 70 2f | 55 63 20 69 6e 20 72 61 |.the Up/|Uc in ra|
|000055b0| 6e 64 6f 6d 20 75 72 69 | 6e 65 20 73 61 6d 70 6c |ndom uri|ne sampl|
|000055c0| 65 73 20 69 73 20 6d 75 | 63 68 20 73 69 6d 70 6c |es is mu|ch simpl|
|000055d0| 65 72 2c 20 70 61 72 74 | 69 63 75 6c 61 72 6c 79 |er, part|icularly|
|000055e0| 20 69 6e 20 63 68 69 6c | 64 72 65 6e 2c 0d 0a 61 | in chil|dren,..a|
|000055f0| 6e 64 20 69 73 20 65 71 | 75 61 6c 6c 79 20 72 65 |nd is eq|ually re|
|00005600| 6c 69 61 62 6c 65 2e 20 | 20 54 68 65 72 65 66 6f |liable. | Therefo|
|00005610| 72 65 2c 20 64 65 74 65 | 72 6d 69 6e 61 74 69 6f |re, dete|rminatio|
|00005620| 6e 20 6f 66 20 74 68 65 | 20 55 70 2f 55 63 20 69 |n of the| Up/Uc i|
|00005630| 73 20 74 68 65 20 6d 65 | 74 68 6f 64 0d 0a 6f 66 |s the me|thod..of|
|00005640| 20 63 68 6f 69 63 65 20 | 66 6f 72 20 71 75 61 6e | choice |for quan|
|00005650| 74 69 66 69 63 61 74 69 | 6f 6e 20 6f 66 20 70 72 |tificati|on of pr|
|00005660| 6f 74 65 69 6e 75 72 69 | 61 20 69 6e 20 70 61 74 |oteinuri|a in pat|
|00005670| 69 65 6e 74 73 20 77 69 | 74 68 20 72 65 6e 61 6c |ients wi|th renal|
|00005680| 20 64 69 73 65 61 73 65 | 2e 0d 0a 0d 0a 50 6f 73 | disease|.....Pos|
|00005690| 74 2d 43 6f 6d 6d 65 6e | 74 0d 0a 0d 0a 20 20 20 |t-Commen|t.... |
|000056a0| 20 20 43 72 61 69 67 20 | 42 2e 20 4c 61 6e 67 6d | Craig |B. Langm|
|000056b0| 61 6e 2c 20 4d 2e 44 2e | 2c 20 41 73 73 6f 63 69 |an, M.D.|, Associ|
|000056c0| 61 74 65 20 50 72 6f 66 | 65 73 73 6f 72 20 6f 66 |ate Prof|essor of|
|000056d0| 20 50 65 64 69 61 74 72 | 69 63 73 2c 20 4e 6f 72 | Pediatr|ics, Nor|
|000056e0| 74 68 77 65 73 74 65 72 | 6e 0d 0a 55 6e 69 76 65 |thwester|n..Unive|
|000056f0| 72 73 69 74 79 20 4d 65 | 64 69 63 61 6c 20 53 63 |rsity Me|dical Sc|
|00005700| 68 6f 6f 6c 2c 20 61 6e | 64 20 44 69 72 65 63 74 |hool, an|d Direct|
|00005710| 6f 72 2c 20 53 65 63 74 | 69 6f 6e 20 6f 66 20 52 |or, Sect|ion of R|
|00005720| 65 73 65 61 72 63 68 20 | 4e 65 70 68 72 6f 6c 6f |esearch |Nephrolo|
|00005730| 67 79 2c 0d 0a 44 69 76 | 69 73 69 6f 6e 20 6f 66 |gy,..Div|ision of|
|00005740| 20 4e 65 70 68 72 6f 6c | 6f 67 79 2c 20 43 68 69 | Nephrol|ogy, Chi|
|00005750| 6c 64 72 65 6e 27 73 20 | 4d 65 6d 6f 72 69 61 6c |ldren's |Memorial|
|00005760| 20 48 6f 73 70 69 74 61 | 6c 2c 20 63 6f 6d 6d 65 | Hospita|l, comme|
|00005770| 6e 74 73 3a 0d 0a 0d 0a | 50 6f 73 74 2d 43 6f 6d |nts:....|Post-Com|
|00005780| 6d 65 6e 74 0d 0a 0d 0a | 20 20 20 20 20 41 20 63 |ment....| A c|
|00005790| 61 72 65 66 75 6c 20 75 | 72 69 6e 61 6c 79 73 69 |areful u|rinalysi|
|000057a0| 73 20 70 65 72 66 6f 72 | 6d 65 64 20 66 6f 72 20 |s perfor|med for |
|000057b0| 69 6e 76 65 73 74 69 67 | 61 74 69 6f 6e 20 6f 66 |investig|ation of|
|000057c0| 20 73 75 73 70 65 63 74 | 65 64 20 6b 69 64 6e 65 | suspect|ed kidne|
|000057d0| 79 0d 0a 64 69 73 65 61 | 73 65 20 70 72 6f 76 69 |y..disea|se provi|
|000057e0| 64 65 73 20 61 6e 20 69 | 6e 76 61 6c 75 61 62 6c |des an i|nvaluabl|
|000057f0| 65 20 77 69 6e 64 6f 77 | 20 69 6e 74 6f 20 74 68 |e window| into th|
|00005800| 65 20 6e 61 74 75 72 65 | 20 6f 66 20 74 68 65 20 |e nature| of the |
|00005810| 70 61 74 68 6f 6c 6f 67 | 69 63 0d 0a 70 72 6f 63 |patholog|ic..proc|
|00005820| 65 73 73 65 73 20 74 68 | 61 74 20 70 72 6f 64 75 |esses th|at produ|
|00005830| 63 65 20 72 65 6e 61 6c | 20 64 79 73 66 75 6e 63 |ce renal| dysfunc|
|00005840| 74 69 6f 6e 2e 20 20 57 | 68 65 6e 20 75 73 65 64 |tion. W|hen used|
|00005850| 20 61 73 20 61 20 73 63 | 72 65 65 6e 69 6e 67 20 | as a sc|reening |
|00005860| 74 6f 6f 6c 2c 0d 0a 74 | 68 65 72 65 20 61 72 65 |tool,..t|here are|
|00005870| 20 61 62 75 6e 64 61 6e | 74 20 64 61 74 61 20 74 | abundan|t data t|
|00005880| 6f 20 73 75 67 67 65 73 | 74 20 61 20 6c 69 6d 69 |o sugges|t a limi|
|00005890| 74 65 64 20 75 73 65 66 | 75 6c 6e 65 73 73 20 66 |ted usef|ulness f|
|000058a0| 6f 72 20 74 68 65 20 22 | 65 61 72 6c 79 22 0d 0a |or the "|early"..|
|000058b0| 64 65 74 65 63 74 69 6f | 6e 20 6f 66 20 72 65 6e |detectio|n of ren|
|000058c0| 61 6c 20 70 61 74 68 6f | 6c 6f 67 79 2e 20 20 54 |al patho|logy. T|
|000058d0| 68 65 20 63 6f 6e 76 65 | 6e 74 69 6f 6e 61 6c 20 |he conve|ntional |
|000058e0| 75 72 69 6e 61 6c 79 73 | 69 73 20 69 73 20 70 65 |urinalys|is is pe|
|000058f0| 72 66 6f 72 6d 65 64 20 | 66 69 72 73 74 0d 0a 62 |rformed |first..b|
|00005900| 79 20 69 6e 73 70 65 63 | 74 69 6f 6e 20 6f 66 20 |y inspec|tion of |
|00005910| 61 20 6d 75 6c 74 69 2d | 74 65 73 74 20 64 69 70 |a multi-|test dip|
|00005920| 73 74 69 63 6b 2c 20 77 | 68 69 63 68 20 79 69 65 |stick, w|hich yie|
|00005930| 6c 64 73 20 69 6e 66 6f | 72 6d 61 74 69 6f 6e 20 |lds info|rmation |
|00005940| 61 62 6f 75 74 20 74 68 | 65 0d 0a 63 6f 6e 63 65 |about th|e..conce|
|00005950| 6e 74 72 61 74 69 6e 67 | 20 61 62 69 6c 69 74 79 |ntrating| ability|
|00005960| 2c 20 61 63 69 64 69 66 | 69 63 61 74 69 6f 6e 20 |, acidif|ication |
|00005970| 63 61 70 61 63 69 74 79 | 2c 20 61 6e 64 20 74 68 |capacity|, and th|
|00005980| 65 20 65 78 63 72 65 74 | 69 6f 6e 20 6f 66 20 72 |e excret|ion of r|
|00005990| 65 64 20 62 6c 6f 6f 64 | 0d 0a 63 65 6c 6c 73 20 |ed blood|..cells |
|000059a0| 61 6e 64 20 70 72 6f 74 | 65 69 6e 20 28 6c 61 72 |and prot|ein (lar|
|000059b0| 67 65 6c 79 20 61 6c 62 | 75 6d 69 6e 29 2c 20 61 |gely alb|umin), a|
|000059c0| 6e 64 20 74 68 65 6e 20 | 62 79 20 6d 69 63 72 6f |nd then |by micro|
|000059d0| 73 63 6f 70 69 63 20 69 | 6e 73 70 65 63 74 69 6f |scopic i|nspectio|
|000059e0| 6e 20 6f 66 20 74 68 65 | 0d 0a 75 72 69 6e 61 72 |n of the|..urinar|
|000059f0| 79 20 73 65 64 69 6d 65 | 6e 74 20 74 6f 20 64 65 |y sedime|nt to de|
|00005a00| 74 65 72 6d 69 6e 65 20 | 61 62 6e 6f 72 6d 61 6c |termine |abnormal|
|00005a10| 20 63 65 6c 6c 75 6c 61 | 72 20 65 6c 65 6d 65 6e | cellula|r elemen|
|00005a20| 74 73 20 61 6e 64 20 63 | 61 73 74 73 2e 20 20 54 |ts and c|asts. T|
|00005a30| 68 65 0d 0a 64 69 70 73 | 74 69 63 6b 20 69 6e 66 |he..dips|tick inf|
|00005a40| 6f 72 6d 61 74 69 6f 6e | 20 69 73 20 71 75 61 6c |ormation| is qual|
|00005a50| 69 74 61 74 69 76 65 2c | 20 61 6e 64 20 74 68 65 |itative,| and the|
|00005a60| 72 65 20 61 72 65 20 6d | 61 6e 79 20 72 65 61 73 |re are m|any reas|
|00005a70| 6f 6e 73 20 66 6f 72 20 | 66 61 6c 73 65 0d 0a 70 |ons for |false..p|
|00005a80| 6f 73 69 74 69 76 65 73 | 20 61 6e 64 20 6e 65 67 |ositives| and neg|
|00005a90| 61 74 69 76 65 73 20 74 | 68 61 74 20 63 6f 75 6c |atives t|hat coul|
|00005aa0| 64 20 6c 65 61 64 20 74 | 68 65 20 63 6c 69 6e 69 |d lead t|he clini|
|00005ab0| 63 69 61 6e 20 61 73 74 | 72 61 79 20 28 31 29 2e |cian ast|ray (1).|
|00005ac0| 20 20 48 6f 77 65 76 65 | 72 2c 20 61 6e 0d 0a 65 | Howeve|r, an..e|
|00005ad0| 61 72 6c 79 20 73 69 67 | 6e 20 6f 66 20 72 65 6e |arly sig|n of ren|
|00005ae0| 61 6c 20 64 79 73 66 75 | 6e 63 74 69 6f 6e 20 69 |al dysfu|nction i|
|00005af0| 73 20 74 68 65 20 65 78 | 63 72 65 74 69 6f 6e 20 |s the ex|cretion |
|00005b00| 6f 66 20 67 72 65 61 74 | 65 72 20 74 68 61 6e 20 |of great|er than |
|00005b10| 6e 6f 72 6d 61 6c 0d 0a | 61 6d 6f 75 6e 74 73 20 |normal..|amounts |
|00005b20| 6f 66 20 70 72 6f 74 65 | 69 6e 20 69 6e 74 6f 20 |of prote|in into |
|00005b30| 74 68 65 20 75 72 69 6e | 65 2e 20 20 54 68 6f 73 |the urin|e. Thos|
|00005b40| 65 20 70 72 6f 74 65 69 | 6e 73 20 61 72 72 69 76 |e protei|ns arriv|
|00005b50| 65 20 69 6e 20 74 68 65 | 20 75 72 69 6e 65 20 62 |e in the| urine b|
|00005b60| 6f 74 68 20 61 73 0d 0a | 74 68 65 20 72 65 73 75 |oth as..|the resu|
|00005b70| 6c 74 20 6f 66 20 6c 65 | 61 6b 61 67 65 20 61 63 |lt of le|akage ac|
|00005b80| 72 6f 73 73 20 61 20 64 | 69 73 65 61 73 65 64 20 |ross a d|iseased |
|00005b90| 67 6c 6f 6d 65 72 75 6c | 75 73 20 61 6e 64 20 66 |glomerul|us and f|
|00005ba0| 72 6f 6d 20 65 6e 68 61 | 6e 63 65 64 20 74 75 62 |rom enha|nced tub|
|00005bb0| 75 6c 61 72 0d 0a 73 65 | 63 72 65 74 69 6f 6e 20 |ular..se|cretion |
|00005bc0| 77 68 65 6e 20 74 68 65 | 72 65 20 69 73 20 69 6e |when the|re is in|
|00005bd0| 74 65 72 73 74 69 74 69 | 61 6c 20 64 61 6d 61 67 |terstiti|al damag|
|00005be0| 65 2e 0d 0a 20 20 20 20 | 20 43 6c 69 6e 69 63 61 |e... | Clinica|
|00005bf0| 6c 20 6e 65 70 68 72 6f | 6c 6f 67 79 20 73 79 6e |l nephro|logy syn|
|00005c00| 64 72 6f 6d 65 73 20 6d | 61 79 20 62 65 20 67 72 |dromes m|ay be gr|
|00005c10| 6f 75 70 65 64 20 69 6e | 74 6f 20 64 69 73 65 61 |ouped in|to disea|
|00005c20| 73 65 73 20 77 69 74 68 | 20 76 65 72 79 0d 0a 6c |ses with| very..l|
|00005c30| 61 72 67 65 20 61 6d 6f | 75 6e 74 73 20 6f 66 20 |arge amo|unts of |
|00005c40| 75 72 69 6e 61 72 79 20 | 70 72 6f 74 65 69 6e 20 |urinary |protein |
|00005c50| 65 78 63 72 65 74 69 6f | 6e 20 28 6e 65 70 68 72 |excretio|n (nephr|
|00005c60| 6f 74 69 63 20 70 72 6f | 74 65 69 6e 75 72 69 61 |otic pro|teinuria|
|00005c70| 2c 20 6f 66 74 65 6e 0d | 0a 61 63 63 6f 6d 70 61 |, often.|.accompa|
|00005c80| 6e 69 65 64 20 62 79 20 | 72 65 64 75 63 74 69 6f |nied by |reductio|
|00005c90| 6e 20 69 6e 20 62 6c 6f | 6f 64 20 61 6c 62 75 6d |n in blo|od album|
|00005ca0| 69 6e 20 63 6f 6e 63 65 | 6e 74 72 61 74 69 6f 6e |in conce|ntration|
|00005cb0| 20 61 6e 64 20 63 6c 69 | 6e 69 63 61 6c 20 65 64 | and cli|nical ed|
|00005cc0| 65 6d 61 29 2c 0d 0a 6d | 6f 64 65 73 74 20 70 72 |ema),..m|odest pr|
|00005cd0| 6f 74 65 69 6e 75 72 69 | 61 20 28 6d 6f 73 74 20 |oteinuri|a (most |
|00005ce0| 72 65 6e 61 6c 20 64 69 | 73 65 61 73 65 73 29 2c |renal di|seases),|
|00005cf0| 20 6f 72 20 76 65 72 79 | 20 73 6d 61 6c 6c 2c 20 | or very| small, |
|00005d00| 62 75 74 20 64 65 74 65 | 63 74 61 62 6c 65 2c 0d |but dete|ctable,.|
|00005d10| 0a 61 6d 6f 75 6e 74 73 | 20 6f 66 20 70 72 6f 74 |.amounts| of prot|
|00005d20| 65 69 6e 75 72 69 61 20 | 28 72 65 70 72 65 73 65 |einuria |(represe|
|00005d30| 6e 74 69 6e 67 20 65 61 | 72 6c 79 20 6d 61 6e 69 |nting ea|rly mani|
|00005d40| 66 65 73 74 61 74 69 6f | 6e 73 20 6f 66 20 72 65 |festatio|ns of re|
|00005d50| 6e 61 6c 20 70 61 74 68 | 6f 6c 6f 67 79 0d 0a 6f |nal path|ology..o|
|00005d60| 72 20 61 73 20 61 20 76 | 61 72 69 61 6e 74 20 6f |r as a v|ariant o|
|00005d70| 66 20 6e 6f 72 6d 61 6c | 29 2e 20 20 49 74 20 69 |f normal|). It i|
|00005d80| 73 20 74 68 65 20 6c 61 | 74 74 65 72 20 63 61 74 |s the la|tter cat|
|00005d90| 65 67 6f 72 79 20 6f 66 | 20 70 72 6f 74 65 69 6e |egory of| protein|
|00005da0| 20 65 78 63 72 65 74 69 | 6f 6e 0d 0a 74 68 61 74 | excreti|on..that|
|00005db0| 20 70 72 6f 76 65 73 20 | 74 72 6f 75 62 6c 65 73 | proves |troubles|
|00005dc0| 6f 6d 65 20 74 6f 20 74 | 68 65 20 63 6c 69 6e 69 |ome to t|he clini|
|00005dd0| 63 69 61 6e 2e 20 20 56 | 61 72 69 61 6e 74 73 20 |cian. V|ariants |
|00005de0| 6f 66 20 6e 6f 72 6d 61 | 6c 20 70 72 6f 74 65 69 |of norma|l protei|
|00005df0| 6e 0d 0a 65 78 63 72 65 | 74 69 6f 6e 20 6d 61 79 |n..excre|tion may|
|00005e00| 20 72 65 73 75 6c 74 20 | 66 72 6f 6d 20 22 6f 72 | result |from "or|
|00005e10| 74 68 6f 73 74 61 74 69 | 63 22 20 70 72 6f 74 65 |thostati|c" prote|
|00005e20| 69 6e 75 72 69 61 2c 20 | 61 20 62 65 6e 69 67 6e |inuria, |a benign|
|00005e30| 20 63 6f 6e 64 69 74 69 | 6f 6e 20 28 32 29 2c 0d | conditi|on (2),.|
|00005e40| 0a 61 6e 64 20 66 72 6f | 6d 20 22 65 78 65 72 63 |.and fro|m "exerc|
|00005e50| 69 73 65 2d 69 6e 64 75 | 63 65 64 22 20 70 72 6f |ise-indu|ced" pro|
|00005e60| 74 65 69 6e 75 72 69 61 | 2c 20 77 68 69 63 68 20 |teinuria|, which |
|00005e70| 69 73 20 70 72 6f 62 61 | 62 6c 79 20 61 20 62 65 |is proba|bly a be|
|00005e80| 6e 69 67 6e 20 63 6f 6e | 64 69 74 69 6f 6e 2c 0d |nign con|dition,.|
|00005e90| 0a 63 6f 6d 6d 6f 6e 20 | 69 6e 20 61 64 6f 6c 65 |.common |in adole|
|00005ea0| 73 63 65 6e 74 73 2e 20 | 20 55 6e 74 69 6c 20 74 |scents. | Until t|
|00005eb0| 68 65 20 6c 61 73 74 20 | 73 65 76 65 72 61 6c 20 |he last |several |
|00005ec0| 79 65 61 72 73 2c 20 63 | 6c 61 73 73 69 63 20 74 |years, c|lassic t|
|00005ed0| 65 61 63 68 69 6e 67 20 | 74 6f 0d 0a 64 69 66 66 |eaching |to..diff|
|00005ee0| 65 72 65 6e 74 69 61 74 | 65 20 6d 69 6c 64 20 70 |erentiat|e mild p|
|00005ef0| 72 6f 74 65 69 6e 75 72 | 69 61 20 6f 66 20 62 65 |roteinur|ia of be|
|00005f00| 6e 69 67 6e 20 6e 61 74 | 75 72 65 20 66 72 6f 6d |nign nat|ure from|
|00005f10| 20 6d 6f 72 65 20 73 65 | 72 69 6f 75 73 20 70 61 | more se|rious pa|
|00005f20| 74 68 6f 6c 6f 67 79 0d | 0a 68 61 73 20 69 6e 76 |thology.|.has inv|
|00005f30| 6f 6c 76 65 64 20 6c 61 | 62 6f 72 69 6f 75 73 20 |olved la|borious |
|00005f40| 28 74 6f 20 74 68 65 20 | 70 61 74 69 65 6e 74 20 |(to the |patient |
|00005f50| 61 6e 64 20 66 61 6d 69 | 6c 79 29 20 63 6f 6c 6c |and fami|ly) coll|
|00005f60| 65 63 74 69 6f 6e 20 6f | 66 20 74 69 6d 65 64 2c |ection o|f timed,|
|00005f70| 20 31 32 2d 0d 0a 61 6e | 64 20 32 34 2d 68 6f 75 | 12-..an|d 24-hou|
|00005f80| 72 20 75 72 69 6e 65 20 | 73 70 65 63 69 6d 65 6e |r urine |specimen|
|00005f90| 73 2e 20 20 4d 6f 72 65 | 20 72 65 63 65 6e 74 6c |s. More| recentl|
|00005fa0| 79 2c 20 73 65 76 65 72 | 61 6c 20 67 72 6f 75 70 |y, sever|al group|
|00005fb0| 73 20 6f 66 20 69 6e 76 | 65 73 74 69 67 61 74 6f |s of inv|estigato|
|00005fc0| 72 73 0d 0a 68 61 76 65 | 20 64 65 6d 6f 6e 73 74 |rs..have| demonst|
|00005fd0| 72 61 74 65 64 20 74 68 | 65 20 61 62 69 6c 69 74 |rated th|e abilit|
|00005fe0| 79 20 74 6f 20 72 65 6c | 61 74 65 20 72 61 6e 64 |y to rel|ate rand|
|00005ff0| 6f 6d 6c 79 20 6f 62 74 | 61 69 6e 65 64 20 75 72 |omly obt|ained ur|
|00006000| 69 6e 65 20 70 72 6f 74 | 65 69 6e 20 74 6f 0d 0a |ine prot|ein to..|
|00006010| 63 72 65 61 74 69 6e 69 | 6e 65 20 72 61 74 69 6f |creatini|ne ratio|
|00006020| 73 20 74 6f 20 6d 6f 72 | 65 20 64 65 66 69 6e 65 |s to mor|e define|
|00006030| 64 20 6d 65 61 73 75 72 | 65 6d 65 6e 74 73 20 6f |d measur|ements o|
|00006040| 66 20 75 72 69 6e 65 20 | 70 72 6f 74 65 69 6e 20 |f urine |protein |
|00006050| 65 78 63 72 65 74 69 6f | 6e 2e 0d 0a 54 68 69 73 |excretio|n...This|
|00006060| 20 68 61 73 20 67 72 65 | 61 74 6c 79 20 73 69 6d | has gre|atly sim|
|00006070| 70 6c 69 66 69 65 64 20 | 74 68 65 20 63 6c 69 6e |plified |the clin|
|00006080| 69 63 61 6c 20 63 61 72 | 65 20 6f 66 20 70 61 74 |ical car|e of pat|
|00006090| 69 65 6e 74 73 20 77 69 | 74 68 20 72 65 6e 61 6c |ients wi|th renal|
|000060a0| 20 64 69 73 65 61 73 65 | 73 2e 0d 0a 20 20 20 20 | disease|s... |
|000060b0| 20 49 6e 20 74 68 69 73 | 20 72 65 70 6f 72 74 2c | In this| report,|
|000060c0| 20 64 61 74 61 20 66 72 | 6f 6d 20 57 68 69 74 65 | data fr|om White|
|000060d0| 27 73 20 70 72 6f 67 72 | 61 6d 20 68 61 73 20 65 |'s progr|am has e|
|000060e0| 78 74 65 6e 64 65 64 20 | 74 68 65 20 6e 6f 72 6d |xtended |the norm|
|000060f0| 61 74 69 76 65 20 64 61 | 74 61 0d 0a 66 6f 72 20 |ative da|ta..for |
|00006100| 74 68 65 20 75 72 69 6e | 65 20 70 72 6f 74 65 69 |the urin|e protei|
|00006110| 6e 20 74 6f 20 63 72 65 | 61 74 69 6e 69 6e 65 20 |n to cre|atinine |
|00006120| 72 61 74 69 6f 20 69 6e | 20 63 68 69 6c 64 72 65 |ratio in| childre|
|00006130| 6e 20 62 79 20 6d 65 61 | 73 75 72 65 6d 65 6e 74 |n by mea|surement|
|00006140| 20 6f 66 20 74 68 65 0d | 0a 72 61 74 69 6f 20 69 | of the.|.ratio i|
|00006150| 6e 20 61 6e 20 65 61 72 | 6c 79 20 6d 6f 72 6e 69 |n an ear|ly morni|
|00006160| 6e 67 20 75 72 69 6e 65 | 20 73 70 65 63 69 6d 65 |ng urine| specime|
|00006170| 6e 20 61 6e 64 20 74 68 | 65 6e 20 62 79 20 73 68 |n and th|en by sh|
|00006180| 6f 77 69 6e 67 20 74 68 | 65 20 73 74 72 69 6b 69 |owing th|e striki|
|00006190| 6e 67 0d 0a 63 6f 72 72 | 65 6c 61 74 69 6f 6e 20 |ng..corr|elation |
|000061a0| 77 69 74 68 20 61 20 74 | 69 6d 65 64 2c 20 6f 76 |with a t|imed, ov|
|000061b0| 65 72 6e 69 67 68 74 20 | 75 72 69 6e 65 20 63 6f |ernight |urine co|
|000061c0| 6c 6c 65 63 74 69 6f 6e | 2e 20 20 54 68 75 73 20 |llection|. Thus |
|000061d0| 74 68 65 20 6e 6f 72 6d | 61 74 69 76 65 0d 0a 72 |the norm|ative..r|
|000061e0| 61 6e 67 65 20 68 61 73 | 20 62 65 65 6e 20 65 73 |ange has| been es|
|000061f0| 74 61 62 6c 69 73 68 65 | 64 20 74 6f 20 62 65 20 |tablishe|d to be |
|00006200| 62 65 74 77 65 65 6e 20 | 30 20 61 6e 64 20 32 30 |between |0 and 20|
|00006210| 20 6d 67 20 6f 66 20 70 | 72 6f 74 65 69 6e 20 70 | mg of p|rotein p|
|00006220| 65 72 20 6d 6d 6f 6c 20 | 6f 66 0d 0a 63 72 65 61 |er mmol |of..crea|
|00006230| 74 69 6e 69 6e 65 2c 20 | 6f 72 20 61 20 72 61 74 |tinine, |or a rat|
|00006240| 69 6f 20 6f 66 20 30 2d | 30 2e 31 38 20 28 6d 67 |io of 0-|0.18 (mg|
|00006250| 2f 6d 67 29 2e 20 20 48 | 6f 77 65 76 65 72 2c 20 |/mg). H|owever, |
|00006260| 50 72 6f 66 65 73 73 6f | 72 20 57 68 69 74 65 27 |Professo|r White'|
|00006270| 73 20 73 74 75 64 79 0d | 0a 64 69 64 20 6e 6f 74 |s study.|.did not|
|00006280| 20 64 65 74 65 72 6d 69 | 6e 65 20 74 68 65 20 72 | determi|ne the r|
|00006290| 61 74 69 6f 20 69 6e 20 | 63 68 69 6c 64 72 65 6e |atio in |children|
|000062a0| 20 77 69 74 68 20 64 6f | 63 75 6d 65 6e 74 65 64 | with do|cumented|
|000062b0| 20 6f 72 74 68 6f 73 74 | 61 74 69 63 0d 0a 70 72 | orthost|atic..pr|
|000062c0| 6f 74 65 69 6e 75 72 69 | 61 2e 20 20 41 6e 65 63 |oteinuri|a. Anec|
|000062d0| 64 6f 74 61 6c 20 65 76 | 69 64 65 6e 63 65 20 66 |dotal ev|idence f|
|000062e0| 72 6f 6d 20 6f 75 72 20 | 6f 77 6e 20 70 72 61 63 |rom our |own prac|
|000062f0| 74 69 63 65 20 73 75 67 | 67 65 73 74 73 20 74 68 |tice sug|gests th|
|00006300| 61 74 20 74 68 6f 73 65 | 0d 0a 63 68 69 6c 64 72 |at those|..childr|
|00006310| 65 6e 20 77 69 74 68 20 | 6f 72 74 68 6f 73 74 61 |en with |orthosta|
|00006320| 74 69 63 20 70 72 6f 74 | 65 69 6e 75 72 69 61 20 |tic prot|einuria |
|00006330| 68 61 76 65 20 61 20 6e | 6f 72 6d 61 6c 20 65 61 |have a n|ormal ea|
|00006340| 72 6c 79 20 6d 6f 72 6e | 69 6e 67 20 72 61 74 69 |rly morn|ing rati|
|00006350| 6f 3b 20 74 68 65 0d 0a | 72 61 74 69 6f 20 6f 62 |o; the..|ratio ob|
|00006360| 74 61 69 6e 65 64 20 61 | 74 20 6f 74 68 65 72 20 |tained a|t other |
|00006370| 74 69 6d 65 73 20 69 6e | 20 74 68 65 20 64 61 79 |times in| the day|
|00006380| 20 69 73 20 6f 6e 6c 79 | 20 6d 69 6c 64 6c 79 20 | is only| mildly |
|00006390| 61 62 6e 6f 72 6d 61 6c | 20 28 3c 3d 20 38 30 20 |abnormal| (<= 80 |
|000063a0| 6d 67 2f 0d 0a 6d 6d 6f | 6c 2c 20 6f 72 20 3c 3d |mg/..mmo|l, or <=|
|000063b0| 20 30 2e 37 32 20 6d 67 | 2f 6d 67 29 2e 20 20 54 | 0.72 mg|/mg). T|
|000063c0| 68 65 72 65 20 61 72 65 | 20 6e 6f 74 20 65 6e 6f |here are| not eno|
|000063d0| 75 67 68 20 64 61 74 61 | 20 79 65 74 20 61 63 63 |ugh data| yet acc|
|000063e0| 75 6d 75 6c 61 74 65 64 | 20 74 6f 0d 0a 64 65 74 |umulated| to..det|
|000063f0| 65 72 6d 69 6e 65 20 74 | 68 65 20 69 6d 70 6f 72 |ermine t|he impor|
+--------+-------------------------+-------------------------+--------+--------+
Only 25.0 KB of data is shown above.