Urinary acid glycosaminoglycan in epidermolysis bullosa dystrophica (recessive type).
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چکیده
منابع مشابه
Anesthetic implications in epidermolysis bullosa dystrophica.
Epidermolysis bullosa is a genetic mechanobullous disease of the stratified squamous keratinizing epithelium that affects the skin and mucous membranes. Its primary feature is the formation of blisters after minor shearing trauma to the skin or mucous membranes that can result in debilitating, even life-threatening scarring. The disease presents special problems for the anesthesia provider beca...
متن کاملClinical Management for Epidermolysis Bullosa Dystrophica
Epidermolysis bullosa (EB) consists of a group of genetic hereditary disorders in which patients frequently present fragile skin and mucosa that form blisters following minor trauma. More than 20 subtypes of EB have been recognized in the literature. Specific genetic mutations are well characterized for most the different EB subtypes and variants. The most common oral manifestations of EB are p...
متن کاملAnaesthesia for correction of oesophageal stricture in a patient with recessive epidermolysis bullosa dystrophica: case report.
Epidermolysis bullosa dystrophica is a rare hereditary disorder which presents significant anaesthetic problems. These include malnutrition and anaemia; electrolyte imbalance in severe cases; renal failure and amyloidosis in progressive disease; association with porphyria; a history of steroid therapy. Technical problems associated with the necessity to avoid trauma to the skin and mucous membr...
متن کاملAcquired double-barrel oesophagus in epidermolysis bullosa dystrophica.
An unusual case of epidermolysis bullosa dystrophica with extensive stenosis, high perforation, and dissection of the oesophagus forming a "double-barrel" structure is described. Gastric epithelium found in the upper oesophagus is thought to be of metaplastic origin and caused by repeated minor trauma with repair.
متن کاملDeath from colonic disease in epidermolysis bullosa dystrophica
BACKGROUND Squamous cell carcinomas and renal failure were reported the causes of death in patients with recessive dystrophic epidermolysis bullosa (RDEB). Death from colonic disease in epidermolysis bullosa (EB) is never reported. CASE PRESENTATION We demonstrate a male patient with RDEB. He suffered megacolon due to fecal impaction and died from sigmoid colon perforation with peritonitis at...
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ژورنال
عنوان ژورنال: The Tohoku Journal of Experimental Medicine
سال: 1978
ISSN: 0040-8727,1349-3329
DOI: 10.1620/tjem.124.91