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Acute massive haemolysis in children with glucose-6-phosphate dehydrogenase deficiency

H K Y Lau1, C H Li, A C W Lee

  • 1Department of Paediatrics and Adolescent Medicine, Tuen Mun Hospital, Tuen Mun, Hong Kong.

Hong Kong Medical Journal = Xianggang Yi Xue Za Zhi
|April 11, 2006
PubMed

Insights

Acute massive hemolysis in children with glucose-6-phosphate dehydrogenase deficiency can be life-threatening. Prompt recognition and management are crucial for recovery, with triggers including fava beans and infections.

Area of Science:

  • Pediatrics
  • Hematology
  • Genetics

Background:

  • Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited disorder.
  • It can lead to hemolytic anemia, particularly when exposed to certain triggers.
  • Acute massive hemolysis is a severe but uncommon manifestation in children.

Purpose of the Study:

  • To describe episodes of acute massive hemolysis in boys with G6PD deficiency.
  • To identify potential triggers for these hemolytic episodes.
  • To highlight the clinical course and management of affected children.

Main Methods:

  • Retrospective case series analysis.
  • Review of medical records for six boys over a 12-year period.
  • Documentation of clinical presentation, laboratory values, and outcomes.

Main Results:

  • Seven episodes of acute massive hemolysis, anemia, and hemoglobinuria observed.
  • Mean age of presentation was 5.5 years.
  • Triggers included fava beans, mothballs, herbal medicine, injections, and URTI.
  • Two patients had transient renal impairment; one required exchange transfusion.
  • All patients recovered after intensive care and transfusions.

Conclusions:

  • Acute massive hemolysis is a serious, potentially fatal complication in G6PD-deficient children.
  • Early diagnosis and intervention are vital for favorable outcomes.
  • Enhanced patient education and public health initiatives are recommended.

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