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

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F65269705%3A_____%2F20%3A00072816" target="_blank" >RIV/65269705:_____/20:00072816 - isvavai.cz</a>

Výsledek na webu

<a href="https://ojrd.biomedcentral.com/track/pdf/10.1186/s13023-020-01403-x" target="_blank" >https://ojrd.biomedcentral.com/track/pdf/10.1186/s13023-020-01403-x</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1186/s13023-020-01403-x" target="_blank" >10.1186/s13023-020-01403-x</a>

Jazyk výsledku

angličtina

Název v původním jazyce

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

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

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

Popis výsledku anglicky

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.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30100 - Basic medicine

Projekt

—

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2020

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

Orphanet Journal of Rare Diseases

ISSN

1750-1172

e-ISSN

—

Svazek periodika

15

Číslo periodika v rámci svazku

1

Stát vydavatele periodika

GB - Spojené království Velké Británie a Severního Irska

Počet stran výsledku

10

Strana od-do

142

Kód UT WoS článku

000540450100001

EID výsledku v databázi Scopus

2-s2.0-85086354153