Results 21 to 30 of about 9,316 (182)
Surgical management of hand deformities in hereditary dystrophic epidermolysis bullosa [PDF]
Vojnosanitetski Pregled, 2003 In the period 1996-2001 in the Clinic for Plastic Surgery and Burns of the Military Medical Academy, 18 patients. 12 male and 6 female, with hereditary dystrophic epidermolysis bullosa (HDEB) and hand deformities were surgically treated, to achieve the ...
Panajotović Ljubomir +2 more
doaj +1 more source
Scanning electron microscopy of a blister roof in dystrophic epidermolysis bullosa [PDF]
Anais Brasileiros de Dermatologia, 2013 In dystrophic epidermolysis bullosa the genetic defect of anchoring fibrils leads to cleavage beneath the basement membrane, with its consequent loss.
Hiram Larangeira de Almeida Jr. +4 more
doaj +1 more source
Genética Molecular das Epidermólises Bolhosas Molecular Genetics of Epidermolysis Bullosa
Anais Brasileiros de Dermatologia, 2002 O estudo das alterações moleculares das epidermólises bolhosas tem contribuído para que se compreenda melhor essas enfermidades. Na epidermólise bolhosa simples a maioria dos casos está associada com alteração nas citoqueratinas basais 5 (gen KRT5) e 14 (
Hiram Larangeira de Almeida Jr
doaj +1 more source
A REPORT OF TWO CASES OF SQUAMOUS CELL CARCINOMA OF THE HAND IN PATIENTS WITH EPIDERMOLYSIS BULLOSA DYSTROPHICA [PDF]
Basrah Journal of Surgery, 2016 This is a report of two patients (brothers) with unusual generalized skin disorder, a recessive dystrophic epidermolysis bullosa (RDEB) that appeared at childhood, who developed a progressive squamous cell carcinoma (SCC) in their right hands. Later both
Avadis A Muradian
doaj +1 more source
Special Care in Dentistry, Volume 40, Issue S1, Page 3-81, November 2020., 2020 ABSTRACT Background Inherited epidermolysis bullosa (EB) is a genetic disorder characterized by skin fragility and unique oral features. Aims To provide (a) a complete review of the oral manifestations in those living with each type of inherited EB, (b) the current best practices for managing oral health care of people living with EB, (c) the current ...
Susanne Krämer +21 more
wiley +1 more source
Esophageal involvement in epidermolysis bullosa dystrophica: Clinical and roentgenographic manifestations [PDF]
, 1983 Epidermolysis bullosa is a rare hereditary mechanobullous skin disorder. Four patients with esophageal involvement are reported. These cases and review of reported cases of epidermolysis bullosa dystrophica-recessive (EBD-R) showed distinct clinical and ...
Agha, Farooq P. +2 more
core +1 more source
Genetic Disorders of the Extracellular Matrix
The Anatomical Record, Volume 303, Issue 6, Page 1527-1542, June 2020., 2020 ABSTRACT Mutations in the genes for extracellular matrix (ECM) components cause a wide range of genetic connective tissues disorders throughout the body. The elucidation of mutations and their correlation with pathology has been instrumental in understanding the roles of many ECM components. The pathological consequences of ECM protein mutations depend
Shireen R. Lamandé, John F. Bateman
wiley +1 more source
ATP-Induced Cell Contraction with Epidermolysis Bullosa Dystrophica Recessive and Normal Dermal Fibroblasts [PDF]
, 1986 Human dermal fibroblasts cultured on glass coverslips and permeabilized by glycerol can be induced to undergo cell shrinkage by the addition of ATP in buffer containing calcium and magnesium. They reduce in size by 72% in 10min.
Rajaratnam, Joseph +2 more
core +1 more source
Translating the combination of gene therapy and tissue engineering for treating recessive dystrophic epidermolysis bullosa [PDF]
European Cells & Materials, 2018 The combination of gene therapy and tissue engineering is one of the most promising strategies for the treatment of recessive dystrophic epidermolysis bullosa (RDEB).
A Dakiw Piaceski +8 more
doaj +1 more source
Case Reports in Pediatrics, Volume 2020, Issue 1, 2020., 2020 We report two Hong Kong children with severe generalized epidermolysis bullosa simplex (EBS), the most severe form of EBS, without a family history of EBS. EBS is a rare genodermatosis usually inherited in an autosomal dominant fashion although rare autosomal recessive cases have been reported.
Shuk Ching Chong +7 more
wiley +1 more source