Literature DB >> 7629774

Adult-onset congenital erythropoietic porphyria (Günther's disease) presenting with thrombocytopenia.

A Murphy1, G Gibson, G H Elder, B A Otridge, G M Murphy.   

Abstract

Cutaneous signs of Günther's disease (congenital erythropoietic porphyria) developing 5 years after the onset of symptomatic thrombocytopenia are described in a 65-year-old man. Persistent thrombocytopenia unresponsive to corticosteroids and immunoglobulin necessitated a splenectomy.

Entities:  

Mesh：

Year:  1995        PMID： 7629774      PMCID： PMC1295249     

Source DB:  PubMed          Journal:  J R Soc Med        ISSN： 0141-0768            Impact factor:   5.344


  6 in total

1.  Erythropoietic uroporphyria of Gunther first presenting at 58 years with positive family studies.

Authors:  R W Pain; F W Welch; A J Woodroffe; D A Handley; W H Lockwood
Journal:  Br Med J       Date:  1975-09-13

2.  Hematin therapy in late onset congenital erythropoietic porphyria.

Authors:  J M Rank; J G Straka; M K Weimer; I Bossenmaier; B L Taddeini; J R Bloomer
Journal:  Br J Haematol       Date:  1990-08       Impact factor: 6.998

3.  Late onset erythropoietic uroporphyria.

Authors:  A MacDonald; D Nicholson; R Williams
Journal:  Br J Dermatol       Date:  1976-07       Impact factor: 9.302

4.  Congenital erythropoietic porphyria. I. Case report, special studies and therapy.

Authors:  R G Haining; M L Cowger; D B Shurtleff; R F Labbe
Journal:  Am J Med       Date:  1968-10       Impact factor: 4.965

5.  The anaemia of erythropoietic prophyria with the description of the disease in an elderly patient.

Authors:  S Kramer; E Viljoen; A M Meyer; J Metz
Journal:  Br J Haematol       Date:  1965-11       Impact factor: 6.998

Review 6.  Late onset erythropoietic porphyria.

Authors:  Y Horiguchi; T Horio; M Yamamoto; T Tanaka; Y Seki; S Imamura
Journal:  Br J Dermatol       Date:  1989-08       Impact factor: 9.302
  6 in total
  3 in total

Review 1.  Porphyria Diagnostics-Part 1: A Brief Overview of the Porphyrias.

Authors:  Vaithamanithi-Mudumbai Sadagopa Ramanujam; Karl Elmo Anderson
Journal:  Curr Protoc Hum Genet       Date:  2015-07-01

Review 2.  Congenital erythropoietic porphyria: Recent advances.

Authors:  Angelika L Erwin; Robert J Desnick
Journal:  Mol Genet Metab       Date:  2018-12-27       Impact factor: 4.797

Review 3.  Dental-craniofacial manifestation and treatment of rare diseases.

Authors:  En Luo; Hanghang Liu; Qiucheng Zhao; Bing Shi; Qianming Chen
Journal:  Int J Oral Sci       Date:  2019-02-20       Impact factor: 6.344
  3 in total

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