Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases
The result's identifiers
Result code in 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>
Result on the web
<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>
Alternative languages
Result language
angličtina
Original language name
Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases
Original language description
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.
Czech name
—
Czech description
—
Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
—
OECD FORD branch
30100 - Basic medicine
Result continuities
Project
—
Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2020
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Orphanet Journal of Rare Diseases
ISSN
1750-1172
e-ISSN
—
Volume of the periodical
15
Issue of the periodical within the volume
1
Country of publishing house
GB - UNITED KINGDOM
Number of pages
10
Pages from-to
142
UT code for WoS article
000540450100001
EID of the result in the Scopus database
2-s2.0-85086354153