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Hereditary factor XIII deficiency

A P Patel1, M Chandy, P Raghupathy

  • 1Department of Clinical Pathology, Christian Medical College and Hospital, Vellore.

Indian Journal of Pediatrics
|May 1, 1993
PubMed
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Hereditary Factor XIII (FX III) deficiency, characterized by bleeding issues, was diagnosed in twelve cases. Despite symptoms like umbilical cord bleeding, diagnosis was often delayed, necessitating transfusions for most patients.

Area of Science:

  • Hematology
  • Genetics
  • Pediatrics

Background:

  • Hereditary Factor XIII (FX III) deficiency is a rare bleeding disorder.
  • Delayed diagnosis can lead to severe bleeding complications.

Purpose of the Study:

  • To present twelve cases of hereditary FX III deficiency diagnosed over five years.
  • To highlight diagnostic challenges and clinical features.
  • To discuss the utility of the Urea solubility test and prophylactic transfusion.

Main Methods:

  • Retrospective review of twelve diagnosed cases of hereditary FX III deficiency.
  • Analysis of clinical features, family history, and diagnostic methods.
  • Evaluation of treatment outcomes, including transfusions and prophylactic measures.

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Main Results:

  • Twelve cases diagnosed between 1986-1990.
  • All patients experienced umbilical cord bleeding and frequent bleeding episodes.
  • Diagnosis was significantly delayed in most cases.
  • Ten patients had consanguineous parents.
  • Most patients required transfusions for bleeding episodes.
  • The Urea solubility test was found to be easy to perform but presented interpretation challenges.

Conclusions:

  • Hereditary FX III deficiency diagnosis is often delayed despite characteristic bleeding symptoms.
  • Consanguinity is a significant factor in hereditary FX III deficiency.
  • The Urea solubility test is a valuable diagnostic tool, but careful interpretation is crucial.
  • Prophylactic transfusion may play a role in managing patients with FX III deficiency.