Glucose 6- Phosphate dehydrogenase deficiency associated with neonatal jaundice

Glucose-6 Phosphate Dehydrogenase [G-6PD] deficiency is the commonest enzymopathy in human beings. It is transmitted as X-linked recessive disorder. Acute hemolytic crisis is the most common presentation of G-6PD deficiency, but in neonatal period it usually presents as jaundice. To find out the proportion of G-6PD deficiency cases in patients with neonatal pathological hyperbilirubinemia and study the clinical course of disease. The study was conducted at the neonatal unit of The Children's Hospital and Institute of Child Health, Lahore from January 2000 - April 2001. One hundred jaundiced neonates with unconjugated hyperbilirubinemia in pathologic range [peak serum bilirubin more than 12 mg/dl in full term and more than 15 mg/dl in preterm neonates] were included. Screening for G-6PD deficiency was done by dye decolorization test, which is semi quantitative, visual colorimetric assay. Out of 100 study cases, 62% were male and 38% were female. 10% of the cases were found to be G-6PD deficient; all were male. One case of G-6PD deficiency developed jaundice during first 24 hrs of life, 8 cases between 1 -7 days and one case after 7 days of life. Peak serum bilirubin levels in neonates with G-6PD deficiency were < 20 mg/dl in 2 cases, 20-30 mg/dl in 6 cases and >30 mg/dl in 2 cases. Evidence of hemolysis [reticulocyte count >5% and Hb% <12.5 gm%] was present in two neonates. In the G-6PD deficiency group, 40% of the cases underwent exchange transfusion compared to 26.6% of cases in the G-6PD normal group. One neonate with G-6PD deficiency had kernicterus at admission. Two neonates with G-6PD deficiency died, due to culture proven sepsis. G-6PD assay should be included in all jaundiced neonates with unexplained neonatal unconjugated pathological hyperbilirubinemia. G-6PD deficiency associated neonatal jaundice is not only hemolytic in origin, but is also related to the impairment of hepatic bilirubin conjugation and excretion

Remission and survival rates of acute myeloid leukemia patients in Peshawar

Thirty four patients were diagnosed as having acute myeloid leukemia [AML] between July 1994 and June 1996 at Lady Reading Hospital, Peshawar. Aim of the study was to determine the remission rate, leukemia-free survival and overall survival of patients suffering from AML in the center. 20 [58%] patients were male while 14 [42%] patients were female. Median age was 30 years [range 5-59 years]. Twenty one [62%] of our patients were between the age of 17 and 36 years, thus more than half of our patients belonged to the younger age group. M1 and M2 sub-types were the most commonly encountered sub-types of AML in our patients. Minimum follow-up was for 12 months. All 34 evaluable patients received Cytarabine 100mg/m2 for 7 days and Daunorubicin 45 mg/m2 for 3 days [standard 7+3 regime]. Patients achieving a complete remission received four cycles of monthly Cytarabine 100mg/m2 for 5 days and Daunorubicin 45mg/m2 for 2 days [5+2 regime] as maintenance chemotherapy. Daunorubicin was replaced with 6-Thioguanine after 4 months of maintenance therapy. An overall response rate of 62% was achieved [complete remission in 50% patients and partial response in 12% patients]. Leukemia-free survival at 6 months and twelve months was 50% and 30% respectively. Our treatment strategies, results and difficulties involved in the treatment of acute myeloid leukemia patients in developing countries are discussed

Hodgkin's disease presenting as nephrotic syndrome: a case report

Nephrotic syndrome is a rare presentation of Hodgkin's disease. Majority of these cases have minimal change disease and some cases present as membranous glomerulopathy. This report presents the case of a child with nephrotic syndrome associated with Hodgkin's disease