THE FACTOR VII DEFICIENCY IN MOROCCAN CHILDREN

Authors

  • Mohamed Khorassani Professor of pediatrics, Pediatric Hematology and Oncology Center, Children’s Hospital of Rabat, Morocco
  • Maria Elkababri Professor of pediatrics, Pediatric Hematology and Oncology Center, Children’s Hospital of Rabat, Morocco
  • Amina Kili Professor of pediatrics, Pediatric Hematology and Oncology Center, Children’s Hospital of Rabat, Morocco
  • Laila Hessissen Professor of pediatrics, Pediatric Hematology and Oncology Center, Children’s Hospital of Rabat, Morocco
  • Mohamed Khattab Professor of pediatrics, Pediatric Hematology and Oncology Center, Children’s Hospital of Rabat, Morocco

DOI:

https://doi.org/10.53555/eijmhs.v2i1.108

Keywords:

factor VII, deficiency, children, bleeding disorders

Abstract

The Factor VII deficiency is a rare causes of bleeding disorders in children. 12 cases were diagnosed and treated in hematology and oncology service between 2003 and 2016. The clinical manifestations are extremely variable. The severity of bleeding is variable and does not seem related to the importance of the deficit; a deep deficit may remain asymptomatic or result in more or less severe hemorrhagic syndrom. 33% of cases were asymptomatic, 66% of cases had a hemorrhagic syndrom:  its Mucocutaneous bleeding (33%), gastrointestinal bleeding (17%), Urinary bleeding (hematuria) (8%), Articular bleeding (haemarthrosis ankle) (8%). Our patients have low PT ranging from 12% to 55%. Family investigation is required, including the dosage of Factor VII in parents and siblings. The  Factor VII  deficiencyis suspected ahead of the combination of an elongated prothrombin time and  a  normal  PTT,  it  is confirmed  by  the  dosage  of  factor  VII  activity,  by  chronometric  method.Replacement  therapyof  this  deficit  is  not  systematic, it  is  indicated  in  the  case  of  an  acute hemorrhagic stroke, or prophylactically prior to surgery, it is based on the recombinant Factor.

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Published

2016-03-27

Issue

Vol. 2 No. 1 (2016): EPH - International Journal of Medical and Health Science (ISSN: 2456 - 6063)

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