title: Neonatal asphyxia and renal failure as the presentation of non-inherited protein C deficiency.
abstract: Inherited or acquired protein C (PC) deficiency leads to thromboembolic events. Plasma PC activity in infancy is physiologically lower than in adults. We describe a case of neonatal asphyxia and acute renal failure associated with isolated PC deficiency. A full-term male infant was born to a healthy mother by caesarean section because of fetal distress. The small-for-gestational age infant showed 2 and 7 of Apgar scores at 1 and 5 minutes, respectively. Hypercoagulability required repeated infusions of fresh frozen plasma. Coagulation study revealed PC activity, 6%, protein S activity, 61%, and high D-dimer levels, along with normal factor VII activity and absent vitamin K deficiency. Anticoagulant and activated PC therapy improved coagulopathy and nephropathy. Imaging analyses indicated no visceral infarctions. Renal function and PC activity have been slowly normalized until 6 months of age. He had no PROC mutation or PC-deficient parents. Selective PC deficiency may occur as an acquired cause of hypercoagulable crisis in the stressed newborn.
authors: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors: Y
authors: Y
authors: Matsunaga
authors: S
authors: S
authors: Ohga
authors: T
authors: T
authors: Kinjo
authors: M
authors: M
authors: Ochiai
authors: N
authors: N
authors: Ito
authors: T
authors: T
authors: Doi
authors: D
authors: D
authors: Kang
authors: T
authors: T
authors: Hara
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology* / Q000628:therapy; D001034:Apgar Score; D001238:Asphyxia Neonatorum / Q000209:etiology*; D004211:Disseminated Intravascular Coagulation / Q000150:complications / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn; D007236:Infant, Small for Gestational Age; D008297:Male; D020151:Protein C Deficiency / Q000150:complications* / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy