Perinatal asphyxia may present with features of neonatal atypical hemolytic uremic syndrome.
Hemolytic uremic syndrome (HUS) is the consequence of platelet consumption at sites of endothelial injury. Perinatal asphyxia (PA) may cause renal failure after birth and can be associated with disseminated intravascular coagulopathy (DIC) with platelet consumption. No biological investigation permits us to distinguish clearly between neonatal HUS and DIC. We report on three neonates with renal failure due to different degrees of PA. They presented biological features compatible with HUS, such as fragmentocytes ( approximately 2%), thrombopenia (<50,000/mm(3)), and anemia (<8 g/dl). One patient required peritoneal dialysis. Haptoglobin was undetectable for all three patients. Factor H and factor I, as well as components of the complement system (C3 and C4) and ADAMTS13 activity, were decreased. Two patients received daily fresh frozen plasma infusions over the first 4 weeks. Renal function improved in two patients; one patient had chronic renal failure. No neurological sequelae were noted. All blood parameters suggestive of thrombotic microangiopathy (TMA) were normal on days 12, 30, and 60. We hypothesize that endothelial cell damage concomitant with PA may lead to a vicious circle that results in consumption of platelets and plasma factors involved in hemostasis and/or fibrinolysis. In conclusion, PA, DIC and HUS are difficult to distinguish, and endothelial cell damage may be their common pathophysiological pathway.
Biran
Valérie
V
Department of Neonatology, Hôpital Trousseau, AP-HP, University of Paris VI, Paris, France.
Fau
Sébastien
S
Jamal
Taleb
T
Veinberg
Frédérique
F
Renolleau
Sylvain
S
Gold
Francis
F
Bensman
Albert
A
Ulinski
Tim
T
D001238:Asphyxia Neonatorum / Q000150:complications / Q000473:pathology* / Q000628:therapy; D005865:Gestational Age; D006463:Hemolytic-Uremic Syndrome / Q000150:complications / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010530:Peritoneal Dialysis; D010951:Plasma Exchange; D051437:Renal Insufficiency / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D016896:Treatment Outcome