• Leslie J Raffini, Alison E Niebanck, Joanne Hrusovsky, Amanda Stevens, Anne Blackwood-Chirchir, Kwaku Ohene-Frempong, and Janet L Kwiatkowski.
• Division of Hematology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4399, and Duke University School of Medicine, Durham, NC, USA. raffini@email.chop.edu
• Pediatr Blood Cancer. 2006 Oct 15; 47 (5): 589-93.

BackgroundPatients with sickle cell disease (SCD) have high rates of perioperative complications, including bleeding 1,2.ProceduresWe conducted a retrospective review of pre-operative coagulation studies in pediatric patients with SCD followed by a prospective study of 100 well children with SCD to determine the prevalence of abnormal coagulation screening tests, and to evaluate potential etiologies.ResultsIn the retrospective study, 32/84 (38.1%) had a prolonged prothrombin time (PT), compared to 8/100 in the prospective study. Prolongations of the activated partial thromboplastin time (aPTT) were less common. Children in the prospective study with prolonged PTs had significantly lower levels of Factor V and VII compared to those with normal PTs. Factor VII levels were <50% in 4/8 with long PTs, compared to 3/92 with normal PTs, P=0.001. Though retrospectively, several patients had normalization of their PT with vitamin K, there was no laboratory evidence of vitamin K deficiency in the prospective study. In the retrospective analysis, six of seven children who had pre-operative coagulation studies and significant intraoperative blood loss had prolonged PTs (P=0.04).ConclusionsChildren with SCD admitted for surgical procedures were more likely to have prolonged PTs than those tested at a well visit. There was intra-patient variability in coagulation studies that may be related to clinical status, hepatocellular dysfunction, and/or increased clotting factor consumption. Future well-designed prospective studies to determine whether abnormal coagulation studies are associated with an increased risk of perioperative bleeding in children with SCD are necessary.Copyright (c) 2005 Wiley-Liss, Inc.

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