نتایج جستجو برای: dystrophic epidermolysis bullosa

تعداد نتایج: 6516  

Journal: :The Journal of investigative dermatology 2008
Tracy Wong Luke Gammon Lu Liu Jemima E Mellerio Patricia J C Dopping-Hepenstal John Pacy George Elia Rosemary Jeffery Irene M Leigh Harshad Navsaria John A McGrath

Recessive dystrophic epidermolysis bullosa (RDEB) is a severe inherited skin-blistering disorder caused by mutations in the COL7A1 gene that lead to reduced type-VII collagen and defective anchoring fibrils at the dermal-epidermal junction (DEJ). Presently there are no effective treatments for this disorder. Recent mouse studies have shown that intradermal injections of normal human fibroblasts...

Journal: :Actas dermo-sifiliograficas 2017
A Batalla A Vicente J Bartrons F Prada C Fortuny M A González-Enseñat

INTRODUCTION AND OBJECTIVE In recent decades, an association has been reported between epidermolysis bullosa (EB) and dilated cardiomyopathy (DC). DC is typically in an advanced phase when detected, leading to a poorer prognosis. Our objective was to determine the prevalence of DC in patients with EB seen in Hospital San Joan de Déu in Barcelona, Spain, between May 1986 and April 2015. METHOD...

2013
Klaas Heeres Hendri H. Pas Katsushi Owaribe Ana M. Martinez de Velasco

Introduction Generalized atrophic benign epidermolysis bullosa (GABEB) is a form of nonlethal junctional epidermolysis bullosa characterized by universal alopecia and atrophy of the skin. We report a deficiency of the 180-kD bullous pemphigoid antigen in three patients with GABEB from unrelated families. We screened specimens of clinically normal skin from nine junctional epidermolysis bullosa ...

Journal: :The Journal of investigative dermatology 2001
P B Cserhalmi-Friedman M C Garzon E Guzman A Martinez-Mir W K Chung K Anyane-Yeboa A M Christiano

Peter B Cserhalmi-Friedman, Maria C Garzon, Edwin Guzman, Amalia Martinez-Mir, Wendy K Chung, Kwame Anyane-Yeboa and Angela M Christiano Department of Dermatology, New York, New York, U.S.A. Department of Genetics and Development, New York, New York, U.S.A. Department of Pediatrics, Division of Clinical Genetics, College of Physicians and Surgeons, Columbia University, New York, New York, U.S.A...

Journal: :Pediatric dentistry 2000
A Kostara G J Roberts M Gelbier

PURPOSE This study was performed to compare Dental Age (DA) of children with Dystrophic Epidermolysis Bullosa recessiva (DEBr) with the DA of healthy children. METHODS Orthopantomographs (OPG's) of children with DEBr were compared with those of healthy children. Dental maturity was estimated using Dermirjian's method. A total of 48 pairs of OPG's were compared. RESULTS There was a considera...

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