title: Fatal Primary Capillary Leak Syndrome in a Late Preterm Newborn.
abstract: 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.
authors.affiliation: Department of Neonatology, Thomayer Hospital, Videnska 800, 14059, Prague, Praha 4, Czech Republic.
authors.forename: Katarina
authors.initials: K
authors.lastname: Kulihova
authors.affiliation: Department of Neonatology, Thomayer Hospital, Videnska 800, 14059, Prague, Praha 4, Czech Republic.
authors.forename: Martina
authors.initials: M
authors.lastname: Prochazkova
authors.affiliation: Department of Neonatology, Thomayer Hospital, Videnska 800, 14059, Prague, Praha 4, Czech Republic.
authors.forename: Jana
authors.initials: J
authors.lastname: Semberova
authors.affiliation: Department of Neonatology, Thomayer Hospital, Videnska 800, 14059, Prague, Praha 4, Czech Republic. janjanota@yahoo.com.|Department of Pathophysiology, 1st Medical Faculty, Charles University in Prague, U Nemocnice 5, 12853, Prague, Praha 2, Czech Republic. janjanota@yahoo.com.
authors.forename: Jan
authors.initials: J
authors.lastname: Janota
mesh_terms: D019559:Capillary Leak Syndrome; D004487:Edema; D006801:Humans; D007022:Hypotension; D007231:Infant, Newborn; D009102:Multiple Organ Failure; D012769:Shock
keywords: Capillary leak syndrome; Disseminated intravascular coagulation; Late preterm newborn