Fatal Primary Capillary Leak Syndrome in a Late Preterm Newborn

Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064190%3A_____%2F16%3AN0000002" target="_blank" >RIV/00064190:_____/16:N0000002 - isvavai.cz</a>
Alternative codes found
RIV/00216208:11110/16:10328063
Result on the web
<a href="http://dx.doi.org/10.1007/s12098-016-2134-y" target="_blank" >http://dx.doi.org/10.1007/s12098-016-2134-y</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1007/s12098-016-2134-y" target="_blank" >10.1007/s12098-016-2134-y</a>

Result language
angličtina
Original language name
Fatal Primary Capillary Leak Syndrome in a Late Preterm Newborn
Original language description
Primary capillary leak syndrome is a rare disease of unknown etiology, characterized by episodes of vascular collapse and plasma extravasation, which may lead to multiple organ failure. Primary capillary leak is extremely rare in children. The authors report a case of a late preterm newborn with fatal capillary leak syndrome of unknown etiology, manifesting as hypotension unresponsive to treatment, extravasation leading to generalised edema, disseminated intravascular coagulation and finally, multiple organ dysfunction syndrome. Aggressive volumotherapy and a combination of inotropes and high doses of terlipressin did not influence systemic vascular collapse and plasma extravasation. The newborn developed multiple organ failure and died on day 27 of life. Investigations performed failed to reveal any specific cause of capillary leak. This is the first report of a fatal primary capillary leak syndrome in a newborn.
Czech name
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Czech description
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Type
J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)
CEP classification
FG - Paediatrics
OECD FORD branch
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Publication year
2016
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

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