Literature DB >> 1517489

Photosensitivity, abnormal porphyrin profile, and sideroblastic anemia.

H W Lim1, D Cooper, S Sassa, H Dosik, M R Buchness, N A Soter.   

Abstract

Cutaneous photosensitivity in a 43-year-old man with idiopathic sideroblastic anemia associated with an abnormal porphyrin profile is reported. This condition was associated with elevated free erythrocyte porphyrin, plasma protoporphyrin, urine porphyrins (predominantly coproporphyrin), stool porphyrins (predominantly protoporphyrin), decreased ferrochelatase activity, and deletion of portions of the long arms of chromosomes 18 and 20. Five other patients with sideroblastic anemia and abnormal porphyrin profiles have been described; all but one of these patients had photosensitivity. The porphyrin profile of this patient is similar to that of three other previously described patients.

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Year:  1992        PMID: 1517489     DOI: 10.1016/0190-9622(92)70182-f

Source DB:  PubMed          Journal:  J Am Acad Dermatol        ISSN: 0190-9622            Impact factor:   11.527


  5 in total

1.  Production and characterization of erythropoietic protoporphyric heterodimeric ferrochelatases.

Authors:  Wided Najahi-Missaoui; Harry A Dailey
Journal:  Blood       Date:  2005-04-14       Impact factor: 22.113

Review 2.  Porphyrias in Japan: compilation of all cases reported through 2002.

Authors:  Masao Kondo; Yuzo Yano; Masuo Shirataka; Gumpei Urata; Shigeru Sassa
Journal:  Int J Hematol       Date:  2004-06       Impact factor: 2.490

Review 3.  Recent advances on porphyria genetics: Inheritance, penetrance & molecular heterogeneity, including new modifying/causative genes.

Authors:  Makiko Yasuda; Brenden Chen; Robert J Desnick
Journal:  Mol Genet Metab       Date:  2018-11-30       Impact factor: 4.797

4.  Photosensitivity and acute liver insufficiency in late-onset erythropoietic protoporphyria with a chromosome 18q abnormality.

Authors:  Yuka Oshikawa; Satoshi Fukushima; Taiga Miyake; Takeshi Kawaguchi; Kenta Motomura; Yasuhiro Nakashima; Kenichi Nakamura; Masatoshi Jinnin; Hironobu Ihn
Journal:  Case Rep Dermatol       Date:  2012-07-10

5.  From the dermatologikum hamburg: quiz.

Authors:  Wendemagegn E Yeshanehe; Rajalakshmi Tirumalae; Almut Böer-Auer
Journal:  Dermatol Pract Concept       Date:  2012-10-31
  5 in total

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