Literature DB >> 35991856

Porphyria cutanea tarda associated with elevated serum ferritin, iron overload, and a bone morphogenetic protein 6 genetic variant.

Paul C Adams1, Carolyn Horgan-Bell2, Scott Walsh3, Bekim Sadikovic4.   

Abstract

A man aged 51 years was referred to dermatology for hand dermatitis. The dorsal hands and fingers had superficial erosions with pale pink shallow scars and milia suggestive of porphyria cutanea tarda (PCT). Urine and fecal studies were typical of PCT. The patient had daily alcohol use and was found to have elevated serum ferritin, aspartate aminotransferase, and alanine transaminase. Genetic testing for common hemochromatosis genetic variants (HFE C282Y and H63D) was normal. He underwent next-generation sequencing analysis using the 16-gene hyperferritinemia gene panel for genes known to be associated with hereditary hyperferritinemia, iron overload, or both and was discovered to have a genetic variant in bone morphogenetic 6 (BMP6, c.287T> C, p.Leu96Pro). The skin lesions improved with phlebotomy therapy.
Copyright © 2020 Canadian Association for the Study of the Liver.

Entities:  

Keywords:  BMP6; Leu96Pro Author Affiliation ; PCT; iron overload; porphyria cutanea tarda

Year:  2020        PMID: 35991856      PMCID: PMC9202784          DOI: 10.3138/canlivj-2019-0018

Source DB:  PubMed          Journal:  Can Liver J        ISSN: 2561-4444


  7 in total

Review 1.  Haemochromatosis.

Authors:  Pierre Brissot; Antonello Pietrangelo; Paul C Adams; Barbara de Graaff; Christine E McLaren; Olivier Loréal
Journal:  Nat Rev Dis Primers       Date:  2018-04-05       Impact factor: 52.329

2.  The p.Leu96Pro Missense Mutation in the BMP6 Gene Is Repeatedly Associated With Hyperferritinemia in Patients of French Origin.

Authors:  Gérald Le Gac; Isabelle Gourlaouen; Chandran Ka; Claude Férec
Journal:  Gastroenterology       Date:  2016-08-30       Impact factor: 22.682

3.  Identification of new BMP6 pro-peptide mutations in patients with iron overload.

Authors:  Chiara Piubelli; Annalisa Castagna; Giacomo Marchi; Monica Rizzi; Fabiana Busti; Sadaf Badar; Monia Marchetti; Marco De Gobbi; Antonella Roetto; Luciano Xumerle; Eda Suku; Alejandro Giorgetti; Massimo Delledonne; Oliviero Olivieri; Domenico Girelli
Journal:  Am J Hematol       Date:  2017-04-29       Impact factor: 10.047

4.  Bone morphogenetic protein signaling is impaired in an HFE knockout mouse model of hemochromatosis.

Authors:  Elena Corradini; Cinzia Garuti; Giuliana Montosi; Paolo Ventura; Billy Andriopoulos; Herbert Y Lin; Antonello Pietrangelo; Jodie L Babitt
Journal:  Gastroenterology       Date:  2009-07-07       Impact factor: 22.682

5.  Clinical evaluation of a hemochromatosis next-generation sequencing gene panel.

Authors:  Matthew B Lanktree; Bekim Sadikovic; John S Waye; Alexander Levstik; Bruce B Lanktree; Jovana Yudin; Mark A Crowther; Guillaume Pare; Paul C Adams
Journal:  Eur J Haematol       Date:  2016-11-11       Impact factor: 2.997

6.  Heterozygous Mutations in BMP6 Pro-peptide Lead to Inappropriate Hepcidin Synthesis and Moderate Iron Overload in Humans.

Authors:  Raed Daher; Caroline Kannengiesser; Dounia Houamel; Thibaud Lefebvre; Edouard Bardou-Jacquet; Nicolas Ducrot; Caroline de Kerguenec; Anne-Marie Jouanolle; Anne-Marie Robreau; Claire Oudin; Gerald Le Gac; Boualem Moulouel; Veronique Loustaud-Ratti; Pierre Bedossa; Dominique Valla; Laurent Gouya; Carole Beaumont; Pierre Brissot; Hervé Puy; Zoubida Karim; Dimitri Tchernitchko
Journal:  Gastroenterology       Date:  2015-11-12       Impact factor: 22.682

7.  Evaluation of a bone morphogenetic protein 6 variant as a cause of iron loading.

Authors:  Cameron J McDonald; Gautam Rishi; Eriza S Secondes; Lesa Ostini; Daniel F Wallace; Darrell H G Crawford; Hanlon Sia; Paul Clark; V Nathan Subramaniam
Journal:  Hum Genomics       Date:  2018-04-25       Impact factor: 4.639

  7 in total

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