Your browser doesn't support javascript.
loading
Factor XIII deficiency in children-clinical presentation and outcome
JCPSP-Journal of the College of Physicians and Surgeons Pakistan. 2008; 18 (9): 565-568
Dans Anglais | IMEMR | ID: emr-102966
ABSTRACT
To determine the demographic features and clinical outcome of children with Factor XIII deficiency. Observational case series. The Aga Khan University Hospital, Karachi, from January 1996 to December 2006. Records of all hospitalized pediatric patients with discharge diagnosis of FXIII D, on the basis of factor XIII assay 5 mol/L urea test were retrospectively reviewed and abstracted on a pre-specified proforma. Demographic features, coagulation profile, family history and outcomes were noted. A total of 10 charts were reviewed. There were 5 boys and 5 girls. Almost all the children [9/10] were less than 5 years of age, out of whom 5 [50%] were infants, and 3 were neonates. Bruises and prolonged bleeding after trauma was the major presenting complaints in 80%, followed by prolonged bleeding from the umbilical stump in 2 patients. Nine patients had past history of prolonged umbilical bleeding. Two patients had history of FXIII D in siblings, while 2 had history of prolonged bleeding in other family members [cause unknown]. Consanguinity was present in 80% of the families. Initial coagulation screen were normal in all patients. Two patients had intracranial hemorrhage, proved on neuro-imaging, were managed with plasma infusions and required craniotomy. The rest were managed conservatively with plasma transfusions. All were discharged alive in good clinical condition. Almost all were followed regularly in clinic with monthly cryoprecipitate transfusions. Although factor XIII deficiency is a rare genetic disorder in children with history of brui sing, prolonged umbilical bleeding, family history of bleeding and consanguinity with normal initial coagulation screen [PT, APTT and platelets], FXIII D should be ruled out
Sujets)
Recherche sur Google
Indice: Méditerranée orientale Sujet Principal: Temps partiel de thromboplastine / Temps de prothrombine / Enfant / Études rétrospectives / Facteurs de risque / Résultat thérapeutique / Consanguinité / Contusions / Hémorragies intracrâniennes / Déficit en facteur XIII Limites du sujet: Femelle / Humains / Mâle langue: Anglais Texte intégral: J. Coll. Physicians Surg. Pak. Année: 2008

Documents relatifs à ce sujet

MEDLINE

...
LILACS

LIS

Recherche sur Google
Indice: Méditerranée orientale Sujet Principal: Temps partiel de thromboplastine / Temps de prothrombine / Enfant / Études rétrospectives / Facteurs de risque / Résultat thérapeutique / Consanguinité / Contusions / Hémorragies intracrâniennes / Déficit en facteur XIII Limites du sujet: Femelle / Humains / Mâle langue: Anglais Texte intégral: J. Coll. Physicians Surg. Pak. Année: 2008