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Unknown  |  1992-06-06  |  334.2 KB

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ConfidenceProgramDetectionMatch TypeSupport
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
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100% file data default



hex view
+--------+-------------------------+-------------------------+--------+--------+
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|00000650| 6f 75 72 6e 61 6c 7b 42 | 72 20 4a 20 44 65 72 6d |ournal{B|r J Derm|
|00000660| 61 74 6f 6c 7d 0d 0a 24 | 56 6f 6c 75 6d 65 7b 31 |atol}..$|Volume{1|
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|00000680| 7d 0d 0a 24 44 61 74 65 | 7b 4a 61 6e 75 61 72 79 |}..$Date|{January|
|00000690| 20 31 39 38 38 7d 0d 0a | 24 59 42 56 6f 6c 7b 31 | 1988}..|$YBVol{1|
|000006a0| 39 38 39 20 59 65 61 72 | 20 42 6f 6f 6b 20 6f 66 |989 Year| Book of|
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|000006c0| 43 68 61 70 74 65 72 7b | 31 35 2d 31 30 7d 0d 0a |Chapter{|15-10}..|
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|00000730| 20 59 65 61 72 20 42 6f | 6f 6b 20 4d 65 64 69 63 | Year Bo|ok Medic|
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|00000760| 39 20 59 65 61 72 20 42 | 6f 6f 6b 20 6f 66 20 44 |9 Year B|ook of D|
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|00001160| 2d 31 30 34 37 7d 0d 0a | 24 44 61 74 65 7b 4d 61 |-1047}..|$Date{Ma|
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|000011e0| 0d 0a 24 43 6f 6d 6d 65 | 6e 74 61 74 6f 72 7b 52 |..$Comme|ntator{R|
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|00001220| 63 61 6c 20 50 75 62 6c | 69 73 68 65 72 73 2c 20 |cal Publ|ishers, |
|00001230| 49 6e 63 2e 2c 20 31 39 | 38 39 0d 0a 0d 0a 31 39 |Inc., 19|89....19|
|00001240| 38 39 20 59 65 61 72 20 | 42 6f 6f 6b 20 6f 66 20 |89 Year |Book of |
|00001250| 44 65 72 6d 61 74 6f 6c | 6f 67 79 20 28 52 29 0d |Dermatol|ogy (R).|
|00001260| 0a 0d 0a 43 68 61 70 74 | 65 72 20 31 35 2d 2d 50 |...Chapt|er 15--P|
|00001270| 68 6f 74 6f 62 69 6f 6c | 6f 67 79 20 61 6e 64 20 |hotobiol|ogy and |
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|000012c0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|000012d0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
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|000012f0| 74 69 6e 67 3a 20 20 41 | 20 53 69 78 2d 59 65 61 |ting: A| Six-Yea|
|00001300| 72 20 45 78 70 65 72 69 | 65 6e 63 65 0d 0a 0d 0a |r Experi|ence....|
|00001310| 4d 65 6e 7a 20 4a 3b 20 | 4d 75 6c 6c 65 72 20 53 |Menz J; |Muller S|
|00001320| 41 3b 20 43 6f 6e 6e 6f | 6c 6c 79 20 53 4d 0d 0a |A; Conno|lly SM..|
|00001330| 28 4d 61 79 6f 20 43 6c | 69 6e 69 63 73 2c 20 4d |(Mayo Cl|inics, M|
|00001340| 61 79 6f 20 46 6f 75 6e | 64 2c 20 52 6f 63 68 65 |ayo Foun|d, Roche|
|00001350| 73 74 65 72 2c 20 4d 69 | 6e 6e 29 0d 0a 4a 20 41 |ster, Mi|nn)..J A|
|00001360| 6d 20 41 63 61 64 20 44 | 65 72 6d 61 74 6f 6c 20 |m Acad D|ermatol |
|00001370| 31 38 3a 31 30 34 34 2d | 31 30 34 37 2c 20 4d 61 |18:1044-|1047, Ma|
|00001380| 79 20 31 39 38 38 0d 0a | 2d 2d 2d 2d 2d 2d 2d 2d |y 1988..|--------|
|00001390| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|000013a0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|000013b0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|000013c0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|000013d0| 2d 2d 2d 2d 2d 2d 2d 2d | 0d 0a 0d 0a 41 62 73 74 |--------|....Abst|
|000013e0| 72 61 63 74 0d 0a 0d 0a | 20 20 20 20 20 54 68 65 |ract....| The|
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|00001e60| 0a 48 79 64 72 6f 61 20 | 76 61 63 63 69 6e 69 66 |.Hydroa |vaccinif|
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|00001ff0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00002000| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00002010| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00002020| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00002030| 2d 2d 2d 2d 2d 2d 2d 0d | 0a 48 79 64 72 6f 61 20 |-------.|.Hydroa |
|00002040| 56 61 63 63 69 6e 69 66 | 6f 72 6d 65 3a 20 20 41 |Vaccinif|orme: A|
|00002050| 20 52 65 76 69 65 77 20 | 6f 66 20 54 65 6e 20 43 | Review |of Ten C|
|00002060| 61 73 65 73 0d 0a 0d 0a | 53 6f 6e 6e 65 78 20 54 |ases....|Sonnex T|
|00002070| 53 3b 20 48 61 77 6b 20 | 4a 4c 4d 0d 0a 28 55 6e |S; Hawk |JLM..(Un|
|00002080| 69 74 65 64 20 4d 65 64 | 20 61 6e 64 20 44 65 6e |ited Med| and Den|
|00002090| 74 61 6c 20 53 63 68 6f | 6f 6c 73 20 6f 66 20 47 |tal Scho|ols of G|
|000020a0| 75 79 27 73 20 61 6e 64 | 20 53 74 20 54 68 6f 6d |uy's and| St Thom|
|000020b0| 61 73 27 73 20 48 6f 73 | 70 73 2c 20 4c 6f 6e 64 |as's Hos|ps, Lond|
|000020c0| 6f 6e 29 0d 0a 42 72 20 | 4a 20 44 65 72 6d 61 74 |on)..Br |J Dermat|
|000020d0| 6f 6c 20 31 31 38 3a 31 | 30 31 2d 31 30 38 2c 20 |ol 118:1|01-108, |
|000020e0| 4a 61 6e 75 61 72 79 20 | 31 39 38 38 0d 0a 2d 2d |January |1988..--|
|000020f0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00002100| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00002110| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00002120| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00002130| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 0d 0a |--------|------..|
|00002140| 0d 0a 41 62 73 74 72 61 | 63 74 0d 0a 0d 0a 20 20 |..Abstra|ct.... |
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|00003650| 20 61 63 74 69 6e 69 63 | 20 70 72 75 72 69 67 6f | actinic| prurigo|
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|00003670| 64 69 61 6e 73 20 73 68 | 6f 75 6c 64 20 70 65 72 |dians sh|ould per|
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|00003690| 65 64 20 73 65 70 61 72 | 61 74 65 6c 79 2c 20 62 |ed separ|ately, b|
|000036a0| 61 73 65 64 20 75 70 6f | 6e 20 74 68 65 20 63 6f |ased upo|n the co|
|000036b0| 6e 73 74 61 6e 74 0d 0a | 70 72 65 73 65 6e 63 65 |nstant..|presence|
|000036c0| 20 6f 66 20 74 68 65 20 | 6c 69 70 20 6c 65 73 69 | of the |lip lesi|
|000036d0| 6f 6e 73 20 69 6e 20 61 | 63 74 69 6e 69 63 20 70 |ons in a|ctinic p|
|000036e0| 72 75 72 69 67 6f 20 62 | 75 74 20 6e 6f 74 20 69 |rurigo b|ut not i|
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|00003710| 61 6e 64 20 75 70 6f 6e | 20 74 68 65 20 61 62 73 |and upon| the abs|
|00003720| 65 6e 63 65 20 6f 66 20 | 6c 65 73 69 6f 6e 73 20 |ence of |lesions |
|00003730| 6f 6e 20 63 6f 76 65 72 | 65 64 20 61 72 65 61 73 |on cover|ed areas|
|00003740| 20 6f 66 20 74 68 65 20 | 62 6f 64 79 20 69 6e 20 | of the |body in |
|00003750| 61 63 74 69 6e 69 63 0d | 0a 70 72 75 72 69 67 6f |actinic.|.prurigo|
|00003760| 2e 20 20 54 68 69 73 20 | 64 69 73 74 69 6e 63 74 |. This |distinct|
|00003770| 69 6f 6e 20 73 65 65 6d | 73 20 72 65 61 73 6f 6e |ion seem|s reason|
|00003780| 61 62 6c 65 20 75 6e 74 | 69 6c 20 6d 6f 72 65 20 |able unt|il more |
|00003790| 69 73 20 6b 6e 6f 77 6e | 20 61 62 6f 75 74 20 65 |is known| about e|
|000037a0| 61 63 68 0d 0a 63 6f 6e | 64 69 74 69 6f 6e 2e 20 |ach..con|dition. |
|000037b0| 20 54 68 65 20 68 69 73 | 74 6f 6c 6f 67 69 63 20 | The his|tologic |
|000037c0| 61 70 70 65 61 72 61 6e | 63 65 73 20 69 6e 20 74 |appearan|ces in t|
|000037d0| 68 65 20 6c 69 70 20 6c | 65 73 69 6f 6e 73 20 6f |he lip l|esions o|
|000037e0| 66 20 61 63 74 69 6e 69 | 63 20 70 72 75 72 69 67 |f actini|c prurig|
|000037f0| 6f 0d 0a 77 65 72 65 20 | 64 69 73 74 69 6e 63 74 |o..were |distinct|
|00003800| 69 76 65 2c 20 77 69 74 | 68 20 6c 61 72 67 65 20 |ive, wit|h large |
|00003810| 6c 79 6d 70 68 6f 69 64 | 20 66 6f 6c 6c 69 63 6c |lymphoid| follicl|
|00003820| 65 73 2e 2d 2d 52 2e 57 | 2e 20 47 61 6e 67 65 2c |es.--R.W|. Gange,|
|00003830| 20 4d 2e 44 2e 0d 0a 24 | 55 6e 69 71 75 65 5f 49 | M.D...$|Unique_I|
|00003840| 44 7b 59 52 42 30 35 30 | 30 34 7d 0d 0a 24 54 69 |D{YRB050|04}..$Ti|
|00003850| 74 6c 65 7b 44 72 75 67 | 2d 49 6e 64 75 63 65 64 |tle{Drug|-Induced|
|00003860| 20 50 68 6f 74 6f 2d 6f | 6e 79 63 68 6f 6c 79 73 | Photo-o|nycholys|
|00003870| 69 73 3a 20 20 54 68 72 | 65 65 20 53 75 62 74 79 |is: Thr|ee Subty|
|00003880| 70 65 73 20 49 64 65 6e | 74 69 66 69 65 64 20 69 |pes Iden|tified i|
|00003890| 6e 20 61 20 53 74 75 64 | 79 20 6f 66 20 31 35 20 |n a Stud|y of 15 |
|000038a0| 43 61 73 65 73 7d 0d 0a | 24 41 75 74 68 6f 72 7b |Cases}..|$Author{|
|000038b0| 0d 0a 42 61 72 61 6e 2c | 20 52 0d 0a 4a 75 68 6c |..Baran,| R..Juhl|
|000038c0| 69 6e 2c 20 4c 7d 0d 0a | 24 41 66 66 69 6c 69 61 |in, L}..|$Affilia|
|000038d0| 74 69 6f 6e 7b 43 61 6e | 6e 65 73 20 47 65 6e 20 |tion{Can|nes Gen |
|000038e0| 48 6f 73 70 2c 20 43 61 | 6e 6e 65 73 2c 20 46 72 |Hosp, Ca|nnes, Fr|
|000038f0| 61 6e 63 65 3b 20 55 6e | 69 76 20 48 6f 73 70 2c |ance; Un|iv Hosp,|
|00003900| 20 55 70 70 73 61 6c 61 | 2c 20 53 77 65 64 65 6e | Uppsala|, Sweden|
|00003910| 7d 0d 0a 24 53 75 62 6a | 65 63 74 7b 50 68 6f 74 |}..$Subj|ect{Phot|
|00003920| 6f 2d 6f 6e 79 63 68 6f | 6c 79 73 69 73 20 64 72 |o-onycho|lysis dr|
|00003930| 75 67 2d 69 6e 64 75 63 | 65 64 3b 0d 0a 4f 6e 79 |ug-induc|ed;..Ony|
|00003940| 63 68 6f 6c 79 73 69 73 | 20 70 68 6f 74 6f 2d 6f |cholysis| photo-o|
|00003950| 6e 79 63 68 6f 6c 79 73 | 69 73 2c 20 64 72 75 67 |nycholys|is, drug|
|00003960| 2d 69 6e 64 75 63 65 64 | 3b 0d 0a 44 72 75 67 28 |-induced|;..Drug(|
|00003970| 73 29 20 70 68 6f 74 6f | 2d 6f 6e 79 63 68 6f 6c |s) photo|-onychol|
|00003980| 79 73 69 73 20 64 75 65 | 20 74 6f 7d 0d 0a 24 4a |ysis due| to}..$J|
|00003990| 6f 75 72 6e 61 6c 7b 4a | 20 41 6d 20 41 63 61 64 |ournal{J| Am Acad|
|000039a0| 20 44 65 72 6d 61 74 6f | 6c 7d 0d 0a 24 56 6f 6c | Dermato|l}..$Vol|
|000039b0| 75 6d 65 7b 31 37 7d 0d | 0a 24 50 61 67 65 7b 31 |ume{17}.|.$Page{1|
|000039c0| 30 31 32 2d 31 30 31 36 | 7d 0d 0a 24 44 61 74 65 |012-1016|}..$Date|
|000039d0| 7b 44 65 63 65 6d 62 65 | 72 20 31 39 38 37 7d 0d |{Decembe|r 1987}.|
|000039e0| 0a 24 59 42 56 6f 6c 7b | 31 39 38 39 20 59 65 61 |.$YBVol{|1989 Yea|
|000039f0| 72 20 42 6f 6f 6b 20 6f | 66 20 44 65 72 6d 61 74 |r Book o|f Dermat|
|00003a00| 6f 6c 6f 67 79 7d 0d 0a | 24 43 68 61 70 74 65 72 |ology}..|$Chapter|
|00003a10| 7b 31 35 2d 31 34 7d 0d | 0a 24 59 42 45 64 69 74 |{15-14}.|.$YBEdit|
|00003a20| 6f 72 7b 44 72 73 2e 20 | 53 6f 62 65 72 20 61 6e |or{Drs. |Sober an|
|00003a30| 64 20 46 69 74 7a 70 61 | 74 72 69 63 6b 7d 0d 0a |d Fitzpa|trick}..|
|00003a40| 24 54 61 62 6c 65 7b 54 | 61 62 6c 65 20 4f 6d 69 |$Table{T|able Omi|
|00003a50| 74 74 65 64 7d 0d 0a 24 | 43 6f 6d 6d 65 6e 74 61 |tted}..$|Commenta|
|00003a60| 74 6f 72 7b 52 2e 57 2e | 20 47 61 6e 67 65 2c 20 |tor{R.W.| Gange, |
|00003a70| 4d 2e 44 2e 7d 0d 0a 0d | 0a 43 6f 70 79 72 69 67 |M.D.}...|.Copyrig|
|00003a80| 68 74 20 28 63 29 20 59 | 65 61 72 20 42 6f 6f 6b |ht (c) Y|ear Book|
|00003a90| 20 4d 65 64 69 63 61 6c | 20 50 75 62 6c 69 73 68 | Medical| Publish|
|00003aa0| 65 72 73 2c 20 49 6e 63 | 2e 2c 20 31 39 38 39 0d |ers, Inc|., 1989.|
|00003ab0| 0a 0d 0a 31 39 38 39 20 | 59 65 61 72 20 42 6f 6f |...1989 |Year Boo|
|00003ac0| 6b 20 6f 66 20 44 65 72 | 6d 61 74 6f 6c 6f 67 79 |k of Der|matology|
|00003ad0| 20 28 52 29 0d 0a 0d 0a | 43 68 61 70 74 65 72 20 | (R)....|Chapter |
|00003ae0| 31 35 2d 2d 50 68 6f 74 | 6f 62 69 6f 6c 6f 67 79 |15--Phot|obiology|
|00003af0| 20 61 6e 64 20 50 68 6f | 74 6f 74 68 65 72 61 70 | and Pho|totherap|
|00003b00| 79 0d 0a 0d 0a 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |y....---|--------|
|00003b10| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003b20| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003b30| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003b40| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003b50| 2d 2d 2d 2d 2d 0d 0a 44 | 72 75 67 2d 49 6e 64 75 |-----..D|rug-Indu|
|00003b60| 63 65 64 20 50 68 6f 74 | 6f 2d 6f 6e 79 63 68 6f |ced Phot|o-onycho|
|00003b70| 6c 79 73 69 73 3a 20 20 | 54 68 72 65 65 20 53 75 |lysis: |Three Su|
|00003b80| 62 74 79 70 65 73 20 49 | 64 65 6e 74 69 66 69 65 |btypes I|dentifie|
|00003b90| 64 20 69 6e 20 61 20 53 | 74 75 64 79 20 6f 66 20 |d in a S|tudy of |
|00003ba0| 31 35 0d 0a 43 61 73 65 | 73 0d 0a 0d 0a 42 61 72 |15..Case|s....Bar|
|00003bb0| 61 6e 20 52 3b 20 4a 75 | 68 6c 69 6e 20 4c 0d 0a |an R; Ju|hlin L..|
|00003bc0| 28 43 61 6e 6e 65 73 20 | 47 65 6e 20 48 6f 73 70 |(Cannes |Gen Hosp|
|00003bd0| 2c 20 43 61 6e 6e 65 73 | 2c 20 46 72 61 6e 63 65 |, Cannes|, France|
|00003be0| 3b 20 55 6e 69 76 20 48 | 6f 73 70 2c 20 55 70 70 |; Univ H|osp, Upp|
|00003bf0| 73 61 6c 61 2c 20 53 77 | 65 64 65 6e 29 0d 0a 4a |sala, Sw|eden)..J|
|00003c00| 20 41 6d 20 41 63 61 64 | 20 44 65 72 6d 61 74 6f | Am Acad| Dermato|
|00003c10| 6c 20 31 37 3a 31 30 31 | 32 2d 31 30 31 36 2c 20 |l 17:101|2-1016, |
|00003c20| 44 65 63 65 6d 62 65 72 | 20 31 39 38 37 0d 0a 2d |December| 1987..-|
|00003c30| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003c40| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003c50| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003c60| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00003c70| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 0d |--------|-------.|
|00003c80| 0a 0d 0a 41 62 73 74 72 | 61 63 74 0d 0a 0d 0a 20 |...Abstr|act.... |
|00003c90| 20 20 20 20 50 68 6f 74 | 6f 2d 6f 6e 79 63 68 6f | Phot|o-onycho|
|00003ca0| 6c 79 73 69 73 20 69 73 | 20 74 68 6f 75 67 68 74 |lysis is| thought|
|00003cb0| 20 74 6f 20 62 65 20 61 | 73 73 6f 63 69 61 74 65 | to be a|ssociate|
|00003cc0| 64 20 77 69 74 68 20 63 | 75 74 61 6e 65 6f 75 73 |d with c|utaneous|
|00003cd0| 0d 0a 70 68 6f 74 6f 73 | 65 6e 73 69 74 69 7a 61 |..photos|ensitiza|
|00003ce0| 74 69 6f 6e 20 69 6e 64 | 75 63 65 64 20 62 79 20 |tion ind|uced by |
|00003cf0| 74 65 74 72 61 63 79 63 | 6c 69 6e 65 73 2c 20 70 |tetracyc|lines, p|
|00003d00| 73 6f 72 61 6c 65 6e 73 | 2c 20 6f 72 20 66 6c 75 |soralens|, or flu|
|00003d10| 6f 72 6f 71 75 69 6e 6f | 6c 6f 6e 65 73 2e 0d 0a |oroquino|lones...|
|00003d20| 20 20 20 20 20 54 68 65 | 20 31 35 20 70 61 74 69 | The| 15 pati|
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|00003d40| 64 79 20 68 61 64 20 62 | 65 65 6e 20 74 72 65 61 |dy had b|een trea|
|00003d50| 74 65 64 20 77 69 74 68 | 20 64 6f 78 79 63 79 63 |ted with| doxycyc|
|00003d60| 6c 69 6e 65 2c 0d 0a 74 | 65 74 72 61 63 79 63 6c |line,..t|etracycl|
|00003d70| 69 6e 65 2c 20 74 72 69 | 6f 78 73 61 6c 65 6e 2c |ine, tri|oxsalen,|
|00003d80| 20 70 65 66 6c 6f 78 61 | 63 69 6e 2c 20 6f 72 20 | pefloxa|cin, or |
|00003d90| 6f 66 6c 6f 78 61 63 69 | 6e 20 62 65 66 6f 72 65 |ofloxaci|n before|
|00003da0| 20 70 68 6f 74 6f 2d 6f | 6e 79 63 68 6f 6c 79 73 | photo-o|nycholys|
|00003db0| 69 73 0d 0a 64 65 76 65 | 6c 6f 70 65 64 2e 20 20 |is..deve|loped. |
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|00003de0| 73 65 6e 74 61 74 69 6f | 6e 2c 20 33 20 64 69 73 |sentatio|n, 3 dis|
|00003df0| 74 69 6e 63 74 20 73 75 | 62 74 79 70 65 73 20 6f |tinct su|btypes o|
|00003e00| 66 0d 0a 6f 6e 79 63 68 | 6f 6c 79 73 69 73 20 63 |f..onych|olysis c|
|00003e10| 6f 75 6c 64 20 62 65 20 | 64 69 73 74 69 6e 67 75 |ould be |distingu|
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|00003e30| 31 20 6f 6e 79 63 68 6f | 6c 79 73 69 73 2c 20 74 |1 onycho|lysis, t|
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|00003ed0| 74 65 64 20 70 72 6f 78 | 69 6d 61 6c 20 62 6f 72 |ted prox|imal bor|
|00003ee0| 64 65 72 20 28 46 69 67 | 0d 0a 31 35 2d 33 29 2e |der (Fig|..15-3).|
|00003ef0| 20 20 49 6e 20 74 79 70 | 65 20 32 20 6f 6e 79 63 | In typ|e 2 onyc|
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|00004060| 2d 31 30 20 64 61 79 73 | 2e 0d 0a 20 20 20 20 20 |-10 days|... |
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|00004350| 0d 0a 42 65 63 61 75 73 | 65 20 74 68 65 20 70 61 |..Becaus|e the pa|
|00004360| 74 68 6f 6c 6f 67 79 20 | 69 73 20 6f 63 63 75 72 |thology |is occur|
|00004370| 72 69 6e 67 20 61 74 20 | 74 68 65 20 73 75 72 66 |ring at |the surf|
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|00004450| 73 75 72 66 61 63 65 2e | 20 20 54 68 65 20 74 72 |surface.| The tr|
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|00004470| 72 65 6d 65 6e 74 73 20 | 6f 66 20 74 68 65 20 6e |rements |of the n|
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|00004490| 69 6e 74 65 72 65 73 74 | 69 6e 67 20 61 6e 64 20 |interest|ing and |
|000044a0| 70 72 6f 62 61 62 6c 79 | 20 75 6e 64 65 72 65 73 |probably| underes|
|000044b0| 74 69 6d 61 74 65 20 74 | 68 65 20 74 6f 74 61 6c |timate t|he total|
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|000044d0| 69 73 73 69 6f 6e 2c 20 | 62 65 63 61 75 73 65 20 |ission, |because |
|000044e0| 74 68 65 20 74 68 65 72 | 6d 6f 63 6f 75 70 6c 65 |the ther|mocouple|
|000044f0| 20 65 6c 65 6d 65 6e 74 | 20 77 6f 75 6c 64 20 70 | element| would p|
|00004500| 72 6f 62 61 62 6c 79 20 | 6e 6f 74 20 68 61 76 65 |robably |not have|
|00004510| 20 64 65 74 65 63 74 65 | 64 0d 0a 73 6f 6d 65 20 | detecte|d..some |
|00004520| 6f 66 20 74 68 65 20 6d | 6f 72 65 20 6c 61 74 65 |of the m|ore late|
|00004530| 72 61 6c 6c 79 20 74 72 | 61 6e 73 6d 69 74 74 65 |rally tr|ansmitte|
|00004540| 64 20 73 63 61 74 74 65 | 72 65 64 20 72 61 64 69 |d scatte|red radi|
|00004550| 61 74 69 6f 6e 2e 2d 2d | 52 2e 57 2e 20 47 61 6e |ation.--|R.W. Gan|
|00004560| 67 65 2c 20 4d 2e 44 2e | 0d 0a 24 55 6e 69 71 75 |ge, M.D.|..$Uniqu|
|00004570| 65 5f 49 44 7b 59 52 42 | 30 35 30 30 35 7d 0d 0a |e_ID{YRB|05005}..|
|00004580| 24 54 69 74 6c 65 7b 48 | 65 74 65 72 6f 67 65 6e |$Title{H|eterogen|
|00004590| 65 69 74 79 20 6f 66 20 | 46 61 6d 69 6c 69 61 6c |eity of |Familial|
|000045a0| 20 50 6f 72 70 68 79 72 | 69 61 20 43 75 74 61 6e | Porphyr|ia Cutan|
|000045b0| 65 61 20 54 61 72 64 61 | 7d 0d 0a 24 41 75 74 68 |ea Tarda|}..$Auth|
|000045c0| 6f 72 7b 0d 0a 52 6f 62 | 65 72 74 73 2c 20 41 47 |or{..Rob|erts, AG|
|000045d0| 0d 0a 45 6c 64 65 72 2c | 20 47 48 0d 0a 4e 65 77 |..Elder,| GH..New|
|000045e0| 63 6f 6d 62 65 2c 20 52 | 47 0d 0a 45 6e 72 69 71 |combe, R|G..Enriq|
|000045f0| 75 65 7a 20 64 65 20 53 | 61 6c 61 6d 61 6e 63 61 |uez de S|alamanca|
|00004600| 2c 20 52 0d 0a 4d 75 6e | 6f 7a 2c 20 4a 4a 7d 0d |, R..Mun|oz, JJ}.|
|00004610| 0a 24 41 66 66 69 6c 69 | 61 74 69 6f 6e 7b 55 6e |.$Affili|ation{Un|
|00004620| 69 76 20 6f 66 20 57 61 | 6c 65 73 2c 20 43 61 72 |iv of Wa|les, Car|
|00004630| 64 69 66 66 3b 20 48 6f | 73 70 20 43 6c 69 6e 69 |diff; Ho|sp Clini|
|00004640| 63 6f 20 64 65 20 53 61 | 6e 20 43 61 72 6c 6f 73 |co de Sa|n Carlos|
|00004650| 2c 20 4d 61 64 72 69 64 | 7d 0d 0a 24 53 75 62 6a |, Madrid|}..$Subj|
|00004660| 65 63 74 7b 50 6f 72 70 | 68 79 72 69 61 20 63 75 |ect{Porp|hyria cu|
|00004670| 74 61 6e 65 61 20 74 61 | 72 64 61 20 66 61 6d 69 |tanea ta|rda fami|
|00004680| 6c 69 61 6c 2c 20 68 65 | 74 65 72 6f 67 65 6e 65 |lial, he|terogene|
|00004690| 69 74 79 20 6f 66 7d 0d | 0a 24 4a 6f 75 72 6e 61 |ity of}.|.$Journa|
|000046a0| 6c 7b 4a 20 4d 65 64 20 | 47 65 6e 65 74 7d 0d 0a |l{J Med |Genet}..|
|000046b0| 24 56 6f 6c 75 6d 65 7b | 32 35 7d 0d 0a 24 50 61 |$Volume{|25}..$Pa|
|000046c0| 67 65 7b 36 36 39 2d 36 | 37 36 7d 0d 0a 24 44 61 |ge{669-6|76}..$Da|
|000046d0| 74 65 7b 4f 63 74 6f 62 | 65 72 20 31 39 38 38 7d |te{Octob|er 1988}|
|000046e0| 0d 0a 24 59 42 56 6f 6c | 7b 31 39 38 39 20 59 65 |..$YBVol|{1989 Ye|
|000046f0| 61 72 20 42 6f 6f 6b 20 | 6f 66 20 44 65 72 6d 61 |ar Book |of Derma|
|00004700| 74 6f 6c 6f 67 79 7d 0d | 0a 24 43 68 61 70 74 65 |tology}.|.$Chapte|
|00004710| 72 7b 31 35 2d 31 35 7d | 0d 0a 24 59 42 45 64 69 |r{15-15}|..$YBEdi|
|00004720| 74 6f 72 7b 44 72 73 2e | 20 53 6f 62 65 72 20 61 |tor{Drs.| Sober a|
|00004730| 6e 64 20 46 69 74 7a 70 | 61 74 72 69 63 6b 7d 0d |nd Fitzp|atrick}.|
|00004740| 0a 24 54 61 62 6c 65 7b | 7d 0d 0a 24 43 6f 6d 6d |.$Table{|}..$Comm|
|00004750| 65 6e 74 61 74 6f 72 7b | 54 2e 42 2e 20 46 69 74 |entator{|T.B. Fit|
|00004760| 7a 70 61 74 72 69 63 6b | 2c 20 4d 2e 44 2e 2c 20 |zpatrick|, M.D., |
|00004770| 50 68 2e 44 2e 7d 0d 0a | 0d 0a 43 6f 70 79 72 69 |Ph.D.}..|..Copyri|
|00004780| 67 68 74 20 28 63 29 20 | 59 65 61 72 20 42 6f 6f |ght (c) |Year Boo|
|00004790| 6b 20 4d 65 64 69 63 61 | 6c 20 50 75 62 6c 69 73 |k Medica|l Publis|
|000047a0| 68 65 72 73 2c 20 49 6e | 63 2e 2c 20 31 39 38 39 |hers, In|c., 1989|
|000047b0| 0d 0a 0d 0a 31 39 38 39 | 20 59 65 61 72 20 42 6f |....1989| Year Bo|
|000047c0| 6f 6b 20 6f 66 20 44 65 | 72 6d 61 74 6f 6c 6f 67 |ok of De|rmatolog|
|000047d0| 79 20 28 52 29 0d 0a 0d | 0a 43 68 61 70 74 65 72 |y (R)...|.Chapter|
|000047e0| 20 31 35 2d 2d 50 68 6f | 74 6f 62 69 6f 6c 6f 67 | 15--Pho|tobiolog|
|000047f0| 79 20 61 6e 64 20 50 68 | 6f 74 6f 74 68 65 72 61 |y and Ph|otothera|
|00004800| 70 79 0d 0a 0d 0a 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |py....--|--------|
|00004810| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004820| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004830| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004840| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004850| 2d 2d 2d 2d 2d 2d 0d 0a | 48 65 74 65 72 6f 67 65 |------..|Heteroge|
|00004860| 6e 65 69 74 79 20 6f 66 | 20 46 61 6d 69 6c 69 61 |neity of| Familia|
|00004870| 6c 20 50 6f 72 70 68 79 | 72 69 61 20 43 75 74 61 |l Porphy|ria Cuta|
|00004880| 6e 65 61 20 54 61 72 64 | 61 0d 0a 0d 0a 52 6f 62 |nea Tard|a....Rob|
|00004890| 65 72 74 73 20 41 47 3b | 20 45 6c 64 65 72 20 47 |erts AG;| Elder G|
|000048a0| 48 3b 20 4e 65 77 63 6f | 6d 62 65 20 52 47 3b 20 |H; Newco|mbe RG; |
|000048b0| 45 6e 72 69 71 75 65 7a | 20 64 65 20 53 61 6c 61 |Enriquez| de Sala|
|000048c0| 6d 61 6e 63 61 20 52 3b | 20 4d 75 6e 6f 7a 20 4a |manca R;| Munoz J|
|000048d0| 4a 0d 0a 28 55 6e 69 76 | 20 6f 66 20 57 61 6c 65 |J..(Univ| of Wale|
|000048e0| 73 2c 20 43 61 72 64 69 | 66 66 3b 20 48 6f 73 70 |s, Cardi|ff; Hosp|
|000048f0| 20 43 6c 69 6e 69 63 6f | 20 64 65 20 53 61 6e 20 | Clinico| de San |
|00004900| 43 61 72 6c 6f 73 2c 20 | 4d 61 64 72 69 64 29 0d |Carlos, |Madrid).|
|00004910| 0a 4a 20 4d 65 64 20 47 | 65 6e 65 74 20 32 35 3a |.J Med G|enet 25:|
|00004920| 36 36 39 2d 36 37 36 2c | 20 4f 63 74 6f 62 65 72 |669-676,| October|
|00004930| 20 31 39 38 38 0d 0a 2d | 2d 2d 2d 2d 2d 2d 2d 2d | 1988..-|--------|
|00004940| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004950| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004960| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004970| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00004980| 2d 2d 2d 2d 2d 2d 2d 0d | 0a 0d 0a 41 62 73 74 72 |-------.|...Abstr|
|00004990| 61 63 74 0d 0a 0d 0a 20 | 20 20 20 20 50 6f 72 70 |act.... | Porp|
|000049a0| 68 79 72 69 61 20 63 75 | 74 61 6e 65 61 20 74 61 |hyria cu|tanea ta|
|000049b0| 72 64 61 20 28 50 43 54 | 29 20 63 6f 6d 62 69 6e |rda (PCT|) combin|
|000049c0| 65 73 20 74 68 65 20 74 | 79 70 69 63 61 6c 20 73 |es the t|ypical s|
|000049d0| 6b 69 6e 20 6c 65 73 69 | 6f 6e 73 20 6f 66 20 74 |kin lesi|ons of t|
|000049e0| 68 65 0d 0a 68 65 70 61 | 74 69 63 20 70 6f 72 70 |he..hepa|tic porp|
|000049f0| 68 79 72 69 61 73 20 77 | 69 74 68 20 64 65 63 72 |hyrias w|ith decr|
|00004a00| 65 61 73 65 64 20 68 65 | 70 61 74 69 63 20 61 63 |eased he|patic ac|
|00004a10| 74 69 76 69 74 79 20 6f | 66 20 75 72 6f 70 6f 72 |tivity o|f uropor|
|00004a20| 70 68 79 72 69 6e 6f 67 | 65 6e 0d 0a 64 65 63 61 |phyrinog|en..deca|
|00004a30| 72 62 6f 78 79 6c 61 73 | 65 2e 20 20 50 72 6f 62 |rboxylas|e. Prob|
|00004a40| 61 62 6c 79 20 6e 6f 20 | 6d 6f 72 65 20 74 68 61 |ably no |more tha|
|00004a50| 6e 20 6f 6e 65 20 66 6f | 75 72 74 68 20 6f 66 20 |n one fo|urth of |
|00004a60| 74 68 65 20 63 61 73 65 | 73 20 61 72 65 20 74 79 |the case|s are ty|
|00004a70| 70 65 20 49 49 20 6f 72 | 0d 0a 66 61 6d 69 6c 69 |pe II or|..famili|
|00004a80| 61 6c 20 50 43 54 2e 20 | 20 49 6e 20 74 79 70 65 |al PCT. | In type|
|00004a90| 20 49 2c 20 6f 72 20 73 | 70 6f 72 61 64 69 63 20 | I, or s|poradic |
|00004aa0| 63 61 73 65 73 2c 20 74 | 68 65 20 63 6f 6e 63 65 |cases, t|he conce|
|00004ab0| 6e 74 72 61 74 69 6f 6e | 20 6f 66 20 72 65 64 20 |ntration| of red |
|00004ac0| 62 6c 6f 6f 64 20 63 65 | 6c 6c 0d 0a 75 72 6f 70 |blood ce|ll..urop|
|00004ad0| 6f 72 70 68 79 72 69 6e | 6f 67 65 6e 20 64 65 63 |orphyrin|ogen dec|
|00004ae0| 61 72 62 6f 78 79 6c 61 | 73 65 20 69 73 20 6e 6f |arboxyla|se is no|
|00004af0| 72 6d 61 6c 2e 20 20 41 | 20 63 6f 6d 70 61 72 69 |rmal. A| compari|
|00004b00| 73 6f 6e 20 6f 66 20 34 | 20 53 70 61 6e 69 73 68 |son of 4| Spanish|
|00004b10| 20 66 61 6d 69 6c 69 65 | 73 0d 0a 77 69 74 68 20 | familie|s..with |
|00004b20| 36 20 74 79 70 65 20 49 | 49 20 50 43 54 20 66 61 |6 type I|I PCT fa|
|00004b30| 6d 69 6c 69 65 73 20 69 | 6e 64 69 63 61 74 65 64 |milies i|ndicated|
|00004b40| 20 74 68 65 20 65 78 69 | 73 74 65 6e 63 65 20 6f | the exi|stence o|
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|00004b70| 2c 20 69 6e 20 77 68 69 | 63 68 20 69 6e 64 69 76 |, in whi|ch indiv|
|00004b80| 69 64 75 61 6c 20 70 61 | 74 69 65 6e 74 73 2c 20 |idual pa|tients, |
|00004b90| 61 70 61 72 74 20 66 72 | 6f 6d 20 74 68 65 69 72 |apart fr|om their|
|00004ba0| 20 66 61 6d 69 6c 79 20 | 68 69 73 74 6f 72 79 2c | family |history,|
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|00004bc0| 67 75 69 73 68 61 62 6c | 65 20 66 72 6f 6d 20 74 |guishabl|e from t|
|00004bd0| 68 6f 73 65 20 77 69 74 | 68 20 74 68 65 20 74 79 |hose wit|h the ty|
|00004be0| 70 65 20 49 20 66 6f 72 | 6d 2e 0d 0a 20 20 20 20 |pe I for|m... |
|00004bf0| 20 49 6e 20 31 37 20 70 | 61 74 69 65 6e 74 73 20 | In 17 p|atients |
|00004c00| 77 69 74 68 20 50 43 54 | 20 66 72 6f 6d 20 31 30 |with PCT| from 10|
|00004c10| 20 66 61 6d 69 6c 69 65 | 73 20 74 68 65 20 63 6f | familie|s the co|
|00004c20| 6e 63 65 6e 74 72 61 74 | 69 6f 6e 20 6f 66 0d 0a |ncentrat|ion of..|
|00004c30| 69 6d 6d 75 6e 6f 72 65 | 61 63 74 69 76 65 20 75 |immunore|active u|
|00004c40| 72 6f 70 6f 72 70 68 79 | 72 69 6e 6f 67 65 6e 20 |roporphy|rinogen |
|00004c50| 64 65 63 61 72 62 6f 78 | 79 6c 61 73 65 20 77 61 |decarbox|ylase wa|
|00004c60| 73 20 6d 65 61 73 75 72 | 65 64 20 69 6e 20 72 65 |s measur|ed in re|
|00004c70| 64 20 62 6c 6f 6f 64 20 | 63 65 6c 6c 73 2e 0d 0a |d blood |cells...|
|00004c80| 53 65 76 65 6e 74 79 2d | 66 6f 75 72 20 72 65 6c |Seventy-|four rel|
|00004c90| 61 74 69 76 65 73 20 61 | 6e 64 20 34 37 20 63 6f |atives a|nd 47 co|
|00004ca0| 6e 74 72 6f 6c 73 20 61 | 6c 73 6f 20 77 65 72 65 |ntrols a|lso were|
|00004cb0| 20 73 74 75 64 69 65 64 | 2e 20 20 49 6e 20 36 20 | studied|. In 6 |
|00004cc0| 66 61 6d 69 6c 69 65 73 | 0d 0a 64 65 63 72 65 61 |families|..decrea|
|00004cd0| 73 65 64 20 65 6e 7a 79 | 6d 65 20 61 63 74 69 76 |sed enzy|me activ|
|00004ce0| 69 74 79 20 73 65 67 72 | 65 67 61 74 65 64 20 61 |ity segr|egated a|
|00004cf0| 73 20 61 6e 20 61 75 74 | 6f 73 6f 6d 61 6c 20 64 |s an aut|osomal d|
|00004d00| 6f 6d 69 6e 61 6e 74 20 | 74 72 61 69 74 2e 20 20 |ominant |trait. |
|00004d10| 49 6e 20 74 68 65 0d 0a | 6f 74 68 65 72 20 34 20 |In the..|other 4 |
|00004d20| 66 61 6d 69 6c 69 65 73 | 20 61 74 20 6c 65 61 73 |families| at leas|
|00004d30| 74 20 32 20 73 75 62 6a | 65 63 74 73 20 68 61 64 |t 2 subj|ects had|
|00004d40| 20 6f 76 65 72 74 20 50 | 43 54 2e 20 20 41 6c 6c | overt P|CT. All|
|00004d50| 20 6d 65 6d 62 65 72 73 | 20 6f 66 20 74 68 65 73 | members| of thes|
|00004d60| 65 0d 0a 66 61 6d 69 6c | 69 65 73 2c 20 69 6e 63 |e..famil|ies, inc|
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|00004d80| 68 20 6f 76 65 72 74 20 | 50 43 54 20 68 61 64 20 |h overt |PCT had |
|00004d90| 6e 6f 72 6d 61 6c 20 63 | 6f 6e 63 65 6e 74 72 61 |normal c|oncentra|
|00004da0| 74 69 6f 6e 73 20 6f 66 | 20 72 65 64 20 62 6c 6f |tions of| red blo|
|00004db0| 6f 64 0d 0a 63 65 6c 6c | 20 75 72 6f 70 6f 72 70 |od..cell| uroporp|
|00004dc0| 68 79 72 69 6e 6f 67 65 | 6e 20 64 65 63 61 72 62 |hyrinoge|n decarb|
|00004dd0| 6f 78 79 6c 61 73 65 2e | 20 20 54 68 65 20 70 61 |oxylase.| The pa|
|00004de0| 74 69 65 6e 74 73 20 77 | 65 72 65 20 63 6c 69 6e |tients w|ere clin|
|00004df0| 69 63 61 6c 6c 79 20 61 | 6e 64 0d 0a 62 69 6f 63 |ically a|nd..bioc|
|00004e00| 68 65 6d 69 63 61 6c 6c | 79 20 73 69 6d 69 6c 61 |hemicall|y simila|
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|00004e20| 20 77 69 74 68 20 73 70 | 6f 72 61 64 69 63 20 50 | with sp|oradic P|
|00004e30| 43 54 2e 0d 0a 20 20 20 | 20 20 50 61 74 69 65 6e |CT... | Patien|
|00004e40| 74 73 20 77 69 74 68 20 | 50 43 54 20 77 68 6f 20 |ts with |PCT who |
|00004e50| 68 61 76 65 20 61 66 66 | 65 63 74 65 64 20 72 65 |have aff|ected re|
|00004e60| 6c 61 74 69 76 65 73 20 | 64 6f 20 6e 6f 74 20 61 |latives |do not a|
|00004e70| 6c 77 61 79 73 20 68 61 | 76 65 20 61 6e 0d 0a 61 |lways ha|ve an..a|
|00004e80| 75 74 6f 73 6f 6d 61 6c | 20 64 6f 6d 69 6e 61 6e |utosomal| dominan|
|00004e90| 74 6c 79 20 69 6e 68 65 | 72 69 74 65 64 20 64 65 |tly inhe|rited de|
|00004ea0| 66 69 63 69 65 6e 63 79 | 20 6f 66 20 72 65 64 20 |ficiency| of red |
|00004eb0| 62 6c 6f 6f 64 20 63 65 | 6c 6c 20 75 72 6f 70 6f |blood ce|ll uropo|
|00004ec0| 72 70 68 79 72 69 6e 6f | 67 65 6e 0d 0a 64 65 63 |rphyrino|gen..dec|
|00004ed0| 61 72 62 6f 78 79 6c 61 | 73 65 2e 20 20 54 68 65 |arboxyla|se. The|
|00004ee0| 20 70 72 65 73 65 6e 74 | 20 70 61 74 69 65 6e 74 | present| patient|
|00004ef0| 73 20 68 61 64 20 6e 6f | 20 65 76 69 64 65 6e 63 |s had no| evidenc|
|00004f00| 65 20 6f 66 20 65 78 70 | 6f 73 75 72 65 20 74 6f |e of exp|osure to|
|00004f10| 20 74 6f 78 69 63 0d 0a | 63 68 65 6d 69 63 61 6c | toxic..|chemical|
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|00004f30| 61 6d 69 6c 69 61 6c 20 | 50 43 54 20 69 73 20 68 |amilial |PCT is h|
|00004f40| 65 74 65 72 6f 67 65 6e | 65 6f 75 73 2e 0d 0a 0d |eterogen|eous....|
|00004f50| 0a 50 6f 73 74 2d 43 6f | 6d 6d 65 6e 74 0d 0a 0d |.Post-Co|mment...|
|00004f60| 0a 20 20 20 20 20 54 68 | 65 20 63 6c 69 6e 69 63 |. Th|e clinic|
|00004f70| 61 6c 20 61 6e 64 20 62 | 69 6f 63 68 65 6d 69 63 |al and b|iochemic|
|00004f80| 61 6c 20 61 62 6e 6f 72 | 6d 61 6c 69 74 69 65 73 |al abnor|malities|
|00004f90| 20 6f 66 20 70 6f 72 70 | 68 79 72 69 61 20 63 75 | of porp|hyria cu|
|00004fa0| 74 61 6e 65 61 20 74 61 | 72 64 61 20 28 50 43 54 |tanea ta|rda (PCT|
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|00004ff0| 63 61 72 62 6f 78 79 6c | 61 73 65 20 28 55 52 4f |carboxyl|ase (URO|
|00005000| 44 29 0d 0a 69 6e 20 74 | 68 65 20 68 65 70 61 74 |D)..in t|he hepat|
|00005010| 6f 63 79 74 65 73 2e 20 | 20 54 77 6f 20 6b 6e 6f |ocytes. | Two kno|
|00005020| 77 6e 20 65 74 69 6f 6c | 6f 67 69 65 73 20 6f 66 |wn etiol|ogies of|
|00005030| 20 74 68 69 73 20 65 6e | 7a 79 6d 65 20 64 65 66 | this en|zyme def|
|00005040| 65 63 74 20 61 72 65 20 | 28 31 29 0d 0a 70 6f 69 |ect are |(1)..poi|
|00005050| 73 6f 6e 69 6e 67 20 77 | 69 74 68 20 70 6f 6c 79 |soning w|ith poly|
|00005060| 68 61 6c 6f 67 65 6e 61 | 74 65 64 20 61 72 6f 6d |halogena|ted arom|
|00005070| 61 74 69 63 20 68 79 64 | 72 6f 63 61 72 62 6f 6e |atic hyd|rocarbon|
|00005080| 73 20 73 75 63 68 20 61 | 73 20 68 65 78 61 63 68 |s such a|s hexach|
|00005090| 6c 6f 72 6f 62 65 6e 7a | 65 6e 65 0d 0a 28 61 20 |lorobenz|ene..(a |
|000050a0| 72 61 72 65 20 63 61 75 | 73 65 29 20 61 6e 64 20 |rare cau|se) and |
|000050b0| 28 32 29 20 61 75 74 6f | 73 6f 6d 61 6c 20 64 6f |(2) auto|somal do|
|000050c0| 6d 69 6e 61 6e 74 20 69 | 6e 68 65 72 69 74 61 6e |minant i|nheritan|
|000050d0| 63 65 20 6f 66 20 55 52 | 4f 44 20 64 65 66 69 63 |ce of UR|OD defic|
|000050e0| 69 65 6e 63 79 20 62 65 | 69 6e 67 0d 0a 64 65 63 |iency be|ing..dec|
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|00005170| 2e 20 20 54 68 65 72 65 | 66 6f 72 65 20 74 68 65 |. There|fore the|
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|00005190| 65 66 69 63 69 65 6e 63 | 79 20 6f 66 20 55 52 4f |eficienc|y of URO|
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|000051e0| 70 6f 72 61 64 69 63 29 | 20 50 43 54 20 74 68 6f |poradic)| PCT tho|
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|00005200| 20 6e 6f 72 6d 61 6c 20 | 61 6d 6f 75 6e 74 73 20 | normal |amounts |
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|00005230| 20 61 6e 64 20 6e 6f 20 | 68 69 73 74 6f 72 79 20 | and no |history |
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|00005250| 6f 72 70 68 79 72 69 6e | 6f 67 65 6e 69 63 20 63 |orphyrin|ogenic c|
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|00005270| 0d 0a 68 65 78 61 63 68 | 6c 6f 72 6f 62 65 6e 7a |..hexach|lorobenz|
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|00005290| 63 61 72 65 66 75 6c 20 | 61 6e 64 20 63 6f 6d 70 |careful |and comp|
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|000052d0| 77 65 72 65 20 32 0d 0a | 67 72 6f 75 70 73 3a 20 |were 2..|groups: |
|000052e0| 36 20 66 61 6d 69 6c 69 | 65 73 20 69 6e 20 77 68 |6 famili|es in wh|
|000052f0| 69 63 68 20 61 6c 6c 20 | 68 61 64 20 64 65 63 72 |ich all |had decr|
|00005300| 65 61 73 65 64 20 65 72 | 79 74 68 72 6f 63 79 74 |eased er|ythrocyt|
|00005310| 65 20 55 52 4f 44 20 28 | 74 68 65 73 65 20 61 72 |e UROD (|these ar|
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|00005340| 74 68 65 72 20 67 72 6f | 75 70 20 6f 66 20 34 20 |ther gro|up of 4 |
|00005350| 66 61 6d 69 6c 69 65 73 | 20 69 6e 20 77 68 69 63 |families| in whic|
|00005360| 68 20 32 20 68 61 64 20 | 6f 76 65 72 74 20 50 43 |h 2 had |overt PC|
|00005370| 54 20 62 75 74 0d 0a 61 | 6c 6c 20 68 61 64 20 6e |T but..a|ll had n|
|00005380| 6f 72 6d 61 6c 20 61 6d | 6f 75 6e 74 73 20 6f 66 |ormal am|ounts of|
|00005390| 20 55 52 4f 44 20 69 6e | 20 65 72 79 74 68 72 6f | UROD in| erythro|
|000053a0| 63 79 74 65 73 2c 20 66 | 69 62 72 6f 62 6c 61 73 |cytes, f|ibroblas|
|000053b0| 74 73 2c 20 61 6e 64 20 | 6c 69 76 65 72 2e 20 20 |ts, and |liver. |
|000053c0| 54 68 65 72 65 0d 0a 69 | 73 2c 20 74 68 65 72 65 |There..i|s, there|
|000053d0| 66 6f 72 65 2c 20 6e 6f | 77 20 61 20 74 79 70 65 |fore, no|w a type|
|000053e0| 20 49 20 6f 72 20 73 70 | 6f 72 61 64 69 63 20 50 | I or sp|oradic P|
|000053f0| 43 54 20 77 68 69 63 68 | 2c 20 69 6e 20 66 61 63 |CT which|, in fac|
|00005400| 74 2c 20 6f 63 63 75 72 | 73 20 69 6e 20 66 61 6d |t, occur|s in fam|
|00005410| 69 6c 69 65 73 0d 0a 61 | 6e 64 20 69 6e 20 77 68 |ilies..a|nd in wh|
|00005420| 69 63 68 20 74 68 65 72 | 65 20 69 73 20 6e 6f 74 |ich ther|e is not|
|00005430| 20 61 20 64 65 6d 6f 6e | 73 74 72 61 62 6c 65 20 | a demon|strable |
|00005440| 64 65 66 69 63 69 65 6e | 63 79 20 6f 66 20 55 52 |deficien|cy of UR|
|00005450| 4f 44 2e 20 20 49 6e 20 | 74 68 69 73 20 67 72 6f |OD. In |this gro|
|00005460| 75 70 20 6f 66 0d 0a 74 | 79 70 65 20 49 20 28 73 |up of..t|ype I (s|
|00005470| 70 6f 72 61 64 69 63 29 | 20 50 43 54 2c 20 62 75 |poradic)| PCT, bu|
|00005480| 74 20 6f 63 63 75 72 72 | 69 6e 67 20 69 6e 20 66 |t occurr|ing in f|
|00005490| 61 6d 69 6c 69 65 73 2c | 20 74 68 65 20 64 69 73 |amilies,| the dis|
|000054a0| 6f 72 64 65 72 20 69 73 | 20 70 6f 73 73 69 62 6c |order is| possibl|
|000054b0| 79 0d 0a 69 6e 68 65 72 | 69 74 65 64 20 61 73 20 |y..inher|ited as |
|000054c0| 61 6e 20 61 75 74 6f 73 | 6f 6d 61 6c 20 64 6f 6d |an autos|omal dom|
|000054d0| 69 6e 61 6e 74 20 74 72 | 61 69 74 20 77 69 74 68 |inant tr|ait with|
|000054e0| 20 69 6e 63 6f 6d 70 6c | 65 74 65 20 70 65 6e 65 | incompl|ete pene|
|000054f0| 74 72 61 6e 63 65 20 6f | 72 20 61 73 20 61 6e 0d |trance o|r as an.|
|00005500| 0a 61 75 74 6f 73 6f 6d | 61 6c 20 72 65 63 65 73 |.autosom|al reces|
|00005510| 73 69 76 65 20 74 72 61 | 69 74 2e 20 20 46 6f 72 |sive tra|it. For|
|00005520| 20 61 20 72 65 63 65 73 | 73 69 76 65 20 69 6e 68 | a reces|sive inh|
|00005530| 65 72 69 74 61 6e 63 65 | 20 69 74 20 77 6f 75 6c |eritance| it woul|
|00005540| 64 20 62 65 20 6e 65 63 | 65 73 73 61 72 79 20 74 |d be nec|essary t|
|00005550| 6f 0d 0a 70 6f 73 74 75 | 6c 61 74 65 20 74 68 61 |o..postu|late tha|
|00005560| 74 20 32 20 6f 66 20 74 | 68 65 20 66 61 6d 69 6c |t 2 of t|he famil|
|00005570| 69 65 73 20 72 65 73 75 | 6c 74 20 66 72 6f 6d 20 |ies resu|lt from |
|00005580| 68 6f 6d 6f 7a 79 67 6f | 74 65 2d 68 65 74 65 72 |homozygo|te-heter|
|00005590| 6f 7a 79 67 6f 74 65 20 | 6d 61 72 72 69 61 67 65 |ozygote |marriage|
|000055a0| 73 2c 0d 0a 77 68 69 63 | 68 2c 22 69 6e 20 74 68 |s,..whic|h,"in th|
|000055b0| 65 20 61 62 73 65 6e 63 | 65 20 6f 66 20 65 76 69 |e absenc|e of evi|
|000055c0| 64 65 6e 63 65 20 6f 66 | 20 63 6f 6e 73 61 6e 67 |dence of| consang|
|000055d0| 75 69 6e 69 74 79 2c 20 | 77 6f 75 6c 64 20 73 75 |uinity, |would su|
|000055e0| 67 67 65 73 74 20 74 68 | 61 74 20 74 68 65 20 67 |ggest th|at the g|
|000055f0| 65 6e 65 0d 0a 69 73 20 | 6e 6f 74 20 75 6e 63 6f |ene..is |not unco|
|00005600| 6d 6d 6f 6e 20 69 6e 20 | 74 68 65 20 70 6f 70 75 |mmon in |the popu|
|00005610| 6c 61 74 69 6f 6e 2e 22 | 2d 2d 54 2e 42 2e 20 46 |lation."|--T.B. F|
|00005620| 69 74 7a 70 61 74 72 69 | 63 6b 2c 20 4d 2e 44 2e |itzpatri|ck, M.D.|
|00005630| 2c 20 50 68 2e 44 2e 0d | 0a 24 55 6e 69 71 75 65 |, Ph.D..|.$Unique|
|00005640| 5f 49 44 7b 59 52 42 30 | 35 30 30 36 7d 0d 0a 24 |_ID{YRB0|5006}..$|
|00005650| 54 69 74 6c 65 7b 4c 6f | 6e 67 2d 54 65 72 6d 20 |Title{Lo|ng-Term |
|00005660| 45 66 66 65 63 74 20 6f | 66 20 48 69 67 68 2d 44 |Effect o|f High-D|
|00005670| 6f 73 65 2c 20 53 68 6f | 72 74 2d 43 6f 75 72 73 |ose, Sho|rt-Cours|
|00005680| 65 20 43 68 6c 6f 72 6f | 71 75 69 6e 65 20 54 68 |e Chloro|quine Th|
|00005690| 65 72 61 70 79 20 6f 6e | 20 50 6f 72 70 68 79 72 |erapy on| Porphyr|
|000056a0| 69 61 20 43 75 74 61 6e | 65 61 20 54 61 72 64 61 |ia Cutan|ea Tarda|
|000056b0| 7d 0d 0a 24 41 75 74 68 | 6f 72 7b 0d 0a 54 73 65 |}..$Auth|or{..Tse|
|000056c0| 67 61 2c 20 45 7d 0d 0a | 24 41 66 66 69 6c 69 61 |ga, E}..|$Affilia|
|000056d0| 74 69 6f 6e 7b 41 64 64 | 69 73 20 41 62 61 62 61 |tion{Add|is Ababa|
|000056e0| 20 55 6e 69 76 2c 20 41 | 64 64 69 73 20 41 62 61 | Univ, A|ddis Aba|
|000056f0| 62 61 2c 20 45 74 68 69 | 6f 70 69 61 7d 0d 0a 24 |ba, Ethi|opia}..$|
|00005700| 53 75 62 6a 65 63 74 7b | 50 6f 72 70 68 79 72 69 |Subject{|Porphyri|
|00005710| 61 20 63 75 74 61 6e 65 | 61 20 74 61 72 64 61 20 |a cutane|a tarda |
|00005720| 63 68 6c 6f 72 6f 71 75 | 69 6e 65 20 69 6e 3b 0d |chloroqu|ine in;.|
|00005730| 0a 43 68 6c 6f 72 6f 71 | 75 69 6e 65 20 69 6e 20 |.Chloroq|uine in |
|00005740| 70 6f 72 70 68 79 72 69 | 61 20 63 75 74 61 6e 65 |porphyri|a cutane|
|00005750| 61 20 74 61 72 64 61 7d | 0d 0a 24 4a 6f 75 72 6e |a tarda}|..$Journ|
|00005760| 61 6c 7b 51 20 4a 20 4d | 65 64 7d 0d 0a 24 56 6f |al{Q J M|ed}..$Vo|
|00005770| 6c 75 6d 65 7b 36 35 7d | 0d 0a 24 50 61 67 65 7b |lume{65}|..$Page{|
|00005780| 39 35 33 2d 39 35 37 7d | 0d 0a 24 44 61 74 65 7b |953-957}|..$Date{|
|00005790| 4e 6f 76 65 6d 62 65 72 | 20 31 39 38 37 7d 0d 0a |November| 1987}..|
|000057a0| 24 59 42 56 6f 6c 7b 31 | 39 38 39 20 59 65 61 72 |$YBVol{1|989 Year|
|000057b0| 20 42 6f 6f 6b 20 6f 66 | 20 44 65 72 6d 61 74 6f | Book of| Dermato|
|000057c0| 6c 6f 67 79 7d 0d 0a 24 | 43 68 61 70 74 65 72 7b |logy}..$|Chapter{|
|000057d0| 31 35 2d 31 36 7d 0d 0a | 24 59 42 45 64 69 74 6f |15-16}..|$YBEdito|
|000057e0| 72 7b 44 72 73 2e 20 53 | 6f 62 65 72 20 61 6e 64 |r{Drs. S|ober and|
|000057f0| 20 46 69 74 7a 70 61 74 | 72 69 63 6b 7d 0d 0a 24 | Fitzpat|rick}..$|
|00005800| 54 61 62 6c 65 7b 54 61 | 62 6c 65 20 4f 6d 69 74 |Table{Ta|ble Omit|
|00005810| 74 65 64 7d 0d 0a 24 43 | 6f 6d 6d 65 6e 74 61 74 |ted}..$C|ommentat|
|00005820| 6f 72 7b 54 2e 42 2e 20 | 46 69 74 7a 70 61 74 72 |or{T.B. |Fitzpatr|
|00005830| 69 63 6b 2c 20 4d 2e 44 | 2e 2c 20 50 68 2e 44 2e |ick, M.D|., Ph.D.|
|00005840| 7d 0d 0a 0d 0a 43 6f 70 | 79 72 69 67 68 74 20 28 |}....Cop|yright (|
|00005850| 63 29 20 59 65 61 72 20 | 42 6f 6f 6b 20 4d 65 64 |c) Year |Book Med|
|00005860| 69 63 61 6c 20 50 75 62 | 6c 69 73 68 65 72 73 2c |ical Pub|lishers,|
|00005870| 20 49 6e 63 2e 2c 20 31 | 39 38 39 0d 0a 0d 0a 31 | Inc., 1|989....1|
|00005880| 39 38 39 20 59 65 61 72 | 20 42 6f 6f 6b 20 6f 66 |989 Year| Book of|
|00005890| 20 44 65 72 6d 61 74 6f | 6c 6f 67 79 20 28 52 29 | Dermato|logy (R)|
|000058a0| 0d 0a 0d 0a 43 68 61 70 | 74 65 72 20 31 35 2d 2d |....Chap|ter 15--|
|000058b0| 50 68 6f 74 6f 62 69 6f | 6c 6f 67 79 20 61 6e 64 |Photobio|logy and|
|000058c0| 20 50 68 6f 74 6f 74 68 | 65 72 61 70 79 0d 0a 0d | Phototh|erapy...|
|000058d0| 0a 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |.-------|--------|
|000058e0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|000058f0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00005900| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00005910| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00005920| 2d 0d 0a 4c 6f 6e 67 2d | 54 65 72 6d 20 45 66 66 |-..Long-|Term Eff|
|00005930| 65 63 74 20 6f 66 20 48 | 69 67 68 2d 44 6f 73 65 |ect of H|igh-Dose|
|00005940| 2c 20 53 68 6f 72 74 2d | 43 6f 75 72 73 65 20 43 |, Short-|Course C|
|00005950| 68 6c 6f 72 6f 71 75 69 | 6e 65 20 54 68 65 72 61 |hloroqui|ne Thera|
|00005960| 70 79 20 6f 6e 20 50 6f | 72 70 68 79 72 69 61 0d |py on Po|rphyria.|
|00005970| 0a 43 75 74 61 6e 65 61 | 20 54 61 72 64 61 0d 0a |.Cutanea| Tarda..|
|00005980| 0d 0a 54 73 65 67 61 20 | 45 0d 0a 28 41 64 64 69 |..Tsega |E..(Addi|
|00005990| 73 20 41 62 61 62 61 20 | 55 6e 69 76 2c 20 41 64 |s Ababa |Univ, Ad|
|000059a0| 64 69 73 20 41 62 61 62 | 61 2c 20 45 74 68 69 6f |dis Abab|a, Ethio|
|000059b0| 70 69 61 29 0d 0a 51 20 | 4a 20 4d 65 64 20 36 35 |pia)..Q |J Med 65|
|000059c0| 3a 39 35 33 2d 39 35 37 | 2c 20 4e 6f 76 65 6d 62 |:953-957|, Novemb|
|000059d0| 65 72 20 31 39 38 37 0d | 0a 2d 2d 2d 2d 2d 2d 2d |er 1987.|.-------|
|000059e0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|000059f0| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00005a00| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00005a10| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 2d 2d 2d 2d 2d 2d 2d |--------|--------|
|00005a20| 2d 2d 2d 2d 2d 2d 2d 2d | 2d 0d 0a 0d 0a 41 62 73 |--------|-....Abs|
|00005a30| 74 72 61 63 74 0d 0a 0d | 0a 20 20 20 20 20 50 6f |tract...|. Po|
|00005a40| 72 70 68 79 72 69 61 20 | 63 75 74 61 6e 65 61 20 |rphyria |cutanea |
|00005a50| 74 61 72 64 61 2c 20 77 | 68 69 63 68 20 69 73 20 |tarda, w|hich is |
|00005a60| 72 65 6c 61 74 65 64 20 | 74 6f 20 61 6c 63 6f 68 |related |to alcoh|
|00005a70| 6f 6c 20 61 62 75 73 65 | 2c 20 69 73 20 63 6f 6d |ol abuse|, is com|
|00005a80| 6d 6f 6e 20 69 6e 0d 0a | 45 74 68 69 6f 70 69 61 |mon in..|Ethiopia|
|00005a90| 2e 20 20 49 6e 20 61 64 | 64 69 74 69 6f 6e 20 74 |. In ad|dition t|
|00005aa0| 6f 20 74 68 65 20 75 73 | 75 61 6c 20 63 75 74 61 |o the us|ual cuta|
|00005ab0| 6e 65 6f 75 73 20 6d 61 | 6e 69 66 65 73 74 61 74 |neous ma|nifestat|
|00005ac0| 69 6f 6e 73 2c 20 74 68 | 65 20 6c 69 76 65 72 20 |ions, th|e liver |
|00005ad0| 69 73 0d 0a 61 6c 6d 6f | 73 74 20 61 6c 77 61 79 |is..almo|st alway|
|00005ae0| 73 20 61 66 66 65 63 74 | 65 64 2c 20 77 69 74 68 |s affect|ed, with|
|00005af0| 20 76 61 72 69 61 62 6c | 65 20 69 6e 66 6c 61 6d | variabl|e inflam|
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|00005b10| 6f 73 69 73 2e 20 20 53 | 65 72 75 6d 0d 0a 74 72 |osis. S|erum..tr|
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|00005b30| 72 69 6e 61 72 79 20 70 | 6f 72 70 68 79 72 69 6e |rinary p|orphyrin|
|00005b40| 20 6c 65 76 65 6c 73 20 | 61 72 65 20 73 69 67 6e | levels |are sign|
|00005b50| 69 66 69 63 61 6e 74 6c | 79 20 72 61 69 73 65 64 |ificantl|y raised|
|00005b60| 20 69 6e 20 6d 6f 73 74 | 0d 0a 70 61 74 69 65 6e | in most|..patien|
|00005b70| 74 73 20 61 6e 64 20 61 | 72 65 20 67 6f 6f 64 20 |ts and a|re good |
|00005b80| 69 6e 64 69 63 61 74 6f | 72 73 20 6f 66 20 74 68 |indicato|rs of th|
|00005b90| 65 20 68 65 70 61 74 69 | 63 20 61 6e 64 20 63 75 |e hepati|c and cu|
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|00005bc0| 2e 20 20 54 68 65 73 65 | 20 69 6e 64 69 63 61 74 |. These| indicat|
|00005bd0| 6f 72 73 20 72 65 76 65 | 72 74 20 72 61 70 69 64 |ors reve|rt rapid|
|00005be0| 6c 79 20 74 6f 20 6e 6f | 72 6d 61 6c 20 61 66 74 |ly to no|rmal aft|
|00005bf0| 65 72 20 63 68 6c 6f 72 | 6f 71 75 69 6e 65 20 74 |er chlor|oquine t|
|00005c00| 68 65 72 61 70 79 2e 0d | 0a 48 6f 77 65 76 65 72 |herapy..|.However|
|00005c10| 2c 20 74 68 65 72 65 20 | 61 72 65 20 6e 6f 20 64 |, there |are no d|
|00005c20| 61 74 61 20 6f 6e 20 74 | 68 65 20 6c 6f 6e 67 2d |ata on t|he long-|
|00005c30| 74 65 72 6d 20 65 66 66 | 65 63 74 20 6f 66 20 63 |term eff|ect of c|
|00005c40| 68 6c 6f 72 6f 71 75 69 | 6e 65 20 6f 6e 20 74 68 |hloroqui|ne on th|
|00005c50| 65 0d 0a 63 75 74 61 6e | 65 6f 75 73 20 61 6e 64 |e..cutan|eous and|
|00005c60| 20 68 65 70 61 74 69 63 | 20 6d 61 6e 69 66 65 73 | hepatic| manifes|
|00005c70| 74 61 74 69 6f 6e 73 20 | 6f 66 20 70 6f 72 70 68 |tations |of porph|
|00005c80| 79 72 69 61 20 63 75 74 | 61 6e 65 61 20 74 61 72 |yria cut|anea tar|
|00005c90| 64 61 2e 20 20 41 20 73 | 74 75 64 79 20 77 61 73 |da. A s|tudy was|
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|00005cb0| 69 67 61 74 65 20 74 68 | 69 73 2e 0d 0a 20 20 20 |igate th|is... |
|00005cc0| 20 20 46 6f 72 74 79 2d | 73 69 78 20 70 61 74 69 | Forty-|six pati|
|00005cd0| 65 6e 74 73 20 77 65 72 | 65 20 6f 62 73 65 72 76 |ents wer|e observ|
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|00005cf0| 20 33 32 20 6d 6f 6e 74 | 68 73 20 61 66 74 65 72 | 32 mont|hs after|
|00005d00| 20 74 72 65 61 74 6d 65 | 6e 74 0d 0a 77 69 74 68 | treatme|nt..with|
|00005d10| 20 68 69 67 68 2d 64 6f | 73 65 2c 20 73 68 6f 72 | high-do|se, shor|
|00005d20| 74 2d 63 6f 75 72 73 65 | 20 63 68 6c 6f 72 6f 71 |t-course| chloroq|
|00005d30| 75 69 6e 65 20 70 68 6f | 73 70 68 61 74 65 20 66 |uine pho|sphate f|
|00005d40| 6f 72 20 70 6f 72 70 68 | 79 72 69 61 20 63 75 74 |or porph|yria cut|
|00005d50| 61 6e 65 61 20 74 61 72 | 64 61 2e 0d 0a 45 61 72 |anea tar|da...Ear|
|00005d60| 6c 79 20 69 6d 70 72 6f | 76 65 6d 65 6e 74 20 77 |ly impro|vement w|
|00005d70| 61 73 20 6e 6f 74 65 64 | 20 69 6e 20 74 68 65 20 |as noted| in the |
|00005d80| 63 75 74 61 6e 65 6f 75 | 73 20 6d 61 6e 69 66 65 |cutaneou|s manife|
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|00005db0| 74 73 20 77 69 74 68 20 | 68 79 70 65 72 70 69 67 |ts with |hyperpig|
|00005dc0| 6d 65 6e 74 61 74 69 6f | 6e 20 61 6e 64 20 68 79 |mentatio|n and hy|
|00005dd0| 70 65 72 74 72 69 63 68 | 6f 73 69 73 2c 20 77 68 |pertrich|osis, wh|
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|00005df0| 72 20 33 2d 36 20 6d 6f | 6e 74 68 73 2e 0d 0a 4d |r 3-6 mo|nths...M|
|00005e00| 65 61 6e 20 73 65 72 75 | 6d 20 74 72 61 6e 73 61 |ean seru|m transa|
|00005e10| 6d 69 6e 61 73 65 20 61 | 6e 64 20 6d 65 61 6e 20 |minase a|nd mean |
|00005e20| 75 72 69 6e 61 72 79 20 | 70 6f 72 70 68 79 72 69 |urinary |porphyri|
|00005e30| 6e 20 6c 65 76 65 6c 73 | 20 72 65 6d 61 69 6e 65 |n levels| remaine|
|00005e40| 64 20 62 65 6c 6f 77 0d | 0a 70 72 65 74 72 65 61 |d below.|.pretrea|
|00005e50| 74 6d 65 6e 74 20 6c 65 | 76 65 6c 73 2e 20 20 4e |tment le|vels. N|
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