Vol. 4, Issue 8, Part D (2018)

Abstract
Introduction: G6pd deficiency is a X linked chromosomal disorder affecting males and females are carriers. It presents with haemolytic anaemia and intravascular hemolysis. The enzyme deficiency causes decreased production of NADPH in the erythrocytes which keeps glutathione in reduced form preventing oxidative stress in cells. Secondary meth-haemoglobinaemia occurs in susceptible individuals, due to decreased active form of haemoglobin available for caring oxygen to cells. A 2 years old male child presented in the hospital emergency with complaints of cough, cold and fever for 3days and increased work of breathing with scanty brown coloured urine since 24hours. On examination there was severe pallor, mild perioral cyanosis and icterus. He had respiratory distress with bilateral crepitation, on auscultation the heart sounds are normal with S3 gallop rhythm. Child was managed in PICU by the standard protocols. Investigations revealed g6pd deficiency, meth-haemoglobinaemia, slight increase in bilirubin levels, normal complement levels. Uri analysis had protein 2+, blood 3+, raised urine: creatinine ratio and on microscopy 10-12RBCS. The child was managed conservatively and gradually improved with high concentration oxygen therapy and blood transfusion.