Treatment of Congenital Afibrinogenemia in a Neonate With Critical Pulmonary Stenosis

J Pediatr Pharmacol Ther. 2023;28(3):268-271. doi: 10.5863/1551-6776-28.3.268. Epub 2023 Jun 2.

Abstract

Fibrinogen deficiencies in neonates can lead to bleeding complications. In this report, we describe a case of congenital afibrinogenemia in a newborn with critical pulmonary stenosis who presented with bilateral cephalohematomas after an uncomplicated delivery. The initial use of cryoprecipitate was followed by administration of fibrinogen concentrate. We estimated a half-life of 24 to 48 hours with the concentrate product. This patient received fibrinogen replacement and had a subsequent successful cardiac repair. The drug's shorter half-life in this neonate contrasts with prior reports of longer half-life in older patients and is important to note in treating future neonatal patients with this diagnosis.

Keywords: afibrinogenemia; fibrinogens, abnormal; infant, newborn, diseases; pharmacokinetics; pulmonary valve stenosis.

Grants and funding

Dr Balasa has served on Advisory Boards and received speaker honoraria from CSL, SanofiGenzyme, and Takeda.