Epidermolysis Bullosa - An anaesthetic challenge

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Anaesthetic management of an infant with epidermolysis bullosa undergoing inguinal hernia repair.

Epidermolysis bullosa is a group of inherited disorders characterized by blistering of the skin as a result of minor trauma. We managed an infant with epidermolysis bullosa undergoing inguinal hernia repair. Anaesthesia was induced with oxygen/nitrous oxide mixture and sevoflurane. Oral tracheal intubation was done with a lubricated laryngoscope blade with KY jelly using atracurium 0.5 mg/kg an...

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Epidemiology of epidermolysis bullosa in the antipodes: the Australasian Epidermolysis Bullosa Registry with a focus on Herlitz junctional epidermolysis bullosa.

OBJECTIVE To present epidemiologic and clinical data from the Australasian Epidermolysis Bullosa (EB) Registry, the first orphan disease registry in Australia. DESIGN Observational study (cross-sectional and longitudinal). SETTING Australian private dermatology practice, inpatient ward, and outpatient clinic. PATIENTS Systematic case finding of patients with EB simplex, junctional EB (JEB...

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Epidermolysis bullosa acquisita.

Autoimmunity to type-WI collagen is characterized by autoantibodies predominantly of lgG class to the non-collagenous domain of type-WI collagen present in the anchoring fibrils which bind basement membrane lamina densa to the anchoring plaques in the dermis. This results in a sublami.na densa split with a blister formation. Type WI collagen autoim.munity is heterogenous in its clinical spectru...

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Junctional epidermolysis bullosa.

Epidermolysis bullosa (EB) encompasses a heterogeneous group of genodermatoses, characterized by fragility and blistering of the skin, often associated with extracutaneous manifestations. The level of vesiculation within the skin defines 3 major subtypes of EB: EB simplex, junctional EB, and dystrophic EB. We present the case of a male neonate of 36 weeks of gestation, who was born with a few b...

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Inherited epidermolysis bullosa

Inherited epidermolysis bullosa (EB) encompasses a number of disorders characterized by recurrent blister formation as the result of structural fragility within the skin and selected other tissues. All types and subtypes of EB are rare; the overall incidence and prevalence of the disease within the United States is approximately 19 per one million live births and 8 per one million population, r...

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ژورنال

عنوان ژورنال: Sri Lankan Journal of Anaesthesiology

سال: 2019

ISSN: 2279-1965,1391-8834

DOI: 10.4038/slja.v27i2.8405