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Acute intravascular haemolysis in glucose-6-phosphate dehydrogenase deficiency
S Sarkar1, D Prakash, R K Marwaha
1Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Insights
Glucose-6-phosphate dehydrogenase (G6PD) deficiency in children can cause acute intravascular hemolysis. Common triggers include infections and certain medications, with potential for kidney complications.
Area of Science:
- Pediatrics
- Hematology
- Nephrology
Background:
- Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited disorder.
- Acute intravascular hemolysis is a serious complication in children with G6PD deficiency.
Purpose of the Study:
- To investigate the causes, clinical presentation, and outcomes of acute intravascular hemolysis in children with G6PD deficiency.
- To identify risk factors and complications associated with this condition.
Main Methods:
- Retrospective evaluation of 35 boys diagnosed with G6PD deficiency and acute intravascular hemolysis.
- Analysis of clinical data including symptoms, laboratory findings, predisposing factors, and treatment outcomes.
Main Results:
- Drug intake (24 children) and infections like hepatitis (7), malaria (4), and bacterial sepsis (3) were primary triggers.
- Pallor and cola-colored urine were universal symptoms.
- Renal impairment, including azotaemia and acute renal failure, occurred in a significant proportion of patients, particularly those with malaria or oliguria.
- Most children (32/35) recovered with supportive care, including blood transfusions and management of underlying conditions.
Conclusions:
- G6PD deficiency hemolysis in children is often triggered by infections or drugs.
- Prompt recognition and management of hemolysis and its complications, such as renal failure, are crucial for favorable outcomes.
- Supportive care and treatment of precipitating factors are key to recovery.
Abstract:
Thirty-five children with G6PD deficiency, who presented with acute intravascular haemolysis, were evaluated to define its aetiology, clinical features and ultimate outcome. All were boys with ages ranging from 6 months to 12 years. Pallor of abrupt onset and passage of cola-coloured urine were universal presenting symptoms. Incriminating factors responsible for haemolysis include hepatitis (7), malaria (4), bacterial sepsis (3) and drug intake (24), with more than one predisposing condition existing in some children. Marked elevations in serum bilirubin, coinciding with intravascular haemolysis, was a feature in all the seven children with hepatitis. Azotaemia was noted in 20 patients, of whom 14 did not have oliguria. All four children with malaria presented with protracted renal failure. Therapy focused on maintaining a high urine output in those without oliguria. A total of 15 peritoneal dialyses and five haemodialyses were required in six patients with acute renal failure, all of whom were oliguric. Supportive therapy consisted of blood transfusions and treatment of the predisposing diseases. Thirty-two children recovered completely while three died, the cause of death being severe anaemia and congestive cardiac failure, malaria with oliguric renal failure and hepatic encephalopathy, respectively.