Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases

Autor:	Christine Bodemer, Cristina Has, Jemima E. Mellerio, N. Bellon, Céline Greco, Tamara Caldaro, Maya El Hachem, Hana Buckova, Rudolf Autrata, Giovanna Zambruno, Carmen Salavastru
Jazyk:	angličtina
Rok vydání:	2020
Předmět:	Adult medicine.medical_specialty Consensus Urinary retention Pain lcsh:Medicine Context (language use) Review Sepsis 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine Rare Diseases Blister 030225 pediatrics medicine Humans Pharmacology (medical) Epidermolysis bullosa Intensive care medicine Child Genetics (clinical) Esophageal Obstruction Skin Mucous Membrane Esophageal obstruction Emergency management business.industry lcsh:R General Medicine Airway obstruction medicine.disease Corneal erosion Esophageal blisters ERN-skin medicine.symptom business
Zdroj:	Orphanet Journal of Rare Diseases, Vol 15, Iss 1, Pp 1-10 (2020) Orphanet Journal of Rare Diseases
ISSN:	1750-1172
DOI:	10.1186/s13023-020-01403-x
Popis:	Epidermolysis bullosa (EB) comprises a group of genetic disorders with the hallmark of fragility of the skin and mucosal surfaces. The severity of different types of EB varies markedly as does the occurrence of extra-cutaneous involvement and complications. A number of emergency situations may occur in the context of EB including obstruction to oral intake from oral or esophageal blisters or scarring, acute airway obstruction, acute urinary retention, sepsis and corneal erosions. Whilst general management principles apply in each of these settings, specific considerations are essential in managing EB to avoid undue trauma or damage to delicate tissues. These recommendations have been developed from a literature review and consensus from experts of the European Network for Rare Skin Disorders (ERN-Skin) to aid decision-making and optimize clinical care by non-EB expert health professionals encountering emergency situations in babies, children and adults with EB.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e7ec45d70021db213dae82aa2f26f879 http://link.springer.com/article/10.1186/s13023-020-01403-x Zobrazit plný text záznamu Plný text ve formátu PDF Plný text ve formátu HTML
Nepřihlášeným uživatelům se plný text nezobrazuje	K zobrazení výsledku je třeba se přihlásit.