Glucose-6-phosphate dehydrogenase deficiency as a cause of neonatal jaundice in Zagazig university hospital

Jaundice is a common problem affecting 50% to 70% of term infants and more than 80% of preterms, its mechanism is multifactorial; increased production, impaired conjugation and impaired excretion of bilirubin. Glucose-6-phosphate dehydrogenase [G-6-PD] deficiency the most common red cell enzyme abnormality associated with hemolysis. It is also known to be associated with neonatal jaundice, kernicterus, and even death. Identify neonates suffering pathological unconjugated hyperbilirubinemia particularly due to G-6-PD deficiency in Zagazig University Hospital. Two hundred clinically jaundiced neonates were enrolled in this study. Their mean gestational age was 36.65 +/- 1.76 weeks, mean birth weight was 2.9 +/- 0.49 kg, mean age at time of admission was 6.5 +/- 3.72 days, 121 were males and 79 were females [representing 60.5% and 39.5% respectively]. In addition 60 age and sex matched healthy neonates served as a control group. AII neonates were subjected to history taking, clinical examination, and measurement of total and direct serum bilirubin. Upon plotting total serum bilirubin [TSB] on bilirubin nomogram 123 neonates were identified as having pathological hyperbilirubinemia and the remaining 77 neonates were excluded from the study in addition, 22 neonates were identified as having direct [conjugated] hyperbilirubinemia and were also excluded from the study. The remaining 101 [50.5%] neonates [61 males and 40 females] were diagnosed as having pathological unconjugated hyperbilirubinemia and were subjected to laboratory investigations in the form of; CBC, peripheral blood smear, reticulocytic count, direct Coombs' test, ABO and Rh blood grouping for mothers and neonates, liver function test, urine analysis, sepsis screen [C-reactive protein, total and differential leucocytic count and band cell count], serum TSH and T4, and G-6-PD enzyme assay. Results: Out of 101 neonates wfth pathological unconjugated hyperbilirubinemia G-6-PD deficiency was detected in 13 [12.9%] neonates, 10 of them were males and 3 were females [representing 76.9% and 23.1% respectively]. G-6-PD deficiency is an important cause of neonatal jaundice in both males and females

P-selectin in preterm infants suffering necrotizing enterocolitis

Platelet selectin [P-selectin], an adhesion molecule expressed by activated endothelial cells, mediates the early phases of leukocyte adherence to the endothelium. Expression of P-selectin has been shown to be crucial to neutrophil recruitment in many human inflammatory processes as well as in animal models of intestinal ischemia-reperfusion, intestinal transplantation, and sepsis, but its role in NEC is unknown. To study P-selectin, a possible cause of NEC, in the blood of preterm infants. Twenty-four consecutive preterms, clinically suspected or proven to have NEC, were enrolled in this pilot study. Their weight ranged from 1 to 2.3 Kg [mean +/- SD: 1.7 +/- 0.5 Kg], age ranged from 2 to 21 days [mean +/- SD: 12 +/- 3.5 days] and their gestational age [GA] ranged from 29 to 33 weeks [mean +/- SD: 31 +/- 3 weeks]. In addition, 12 age- and weight-matched apparently healthy preterm infants served as a control group. Written consents were obtained from the parents of infants included in the study. All neonates were subjected to perinatal history, clinical examination, routine investigations [CBC, plain X-ray and abdominal ultrasonography [US], arterial blood gases and serum bicarbonate, serum sodium, CRP and blood culture], and measurement of blood P-selectin by direct immunofluorescent staining. Infants with NEC clinically presented with significant PROM, gastric residual, abdominal distensions, hypoperfusion, hematochezia and evidence of NEC in abdominal X-ray and/or US, compared to control infants. Significant abnormal laboratory investigations in NEC cases included high CRP, hyponatremia, bandemia, thrombocytopenia, metabolic acidosis, and blood culture-proven neonatal sepsis. Abnormal blood P-selectin [>20 units] was detected in 21 [87.5%] infants with NEC, with a mean level of 51 +/- 12.4 units that was significantly higher than that of control infants, P<0.001. A strong significant negative correlation was observed between blood P-selectin and each of GA, body weight, platelet count, arterial blood pH and bicarbonate, while it was a significant positive correlation with each of CRP and band cell count. P-selectin may have a role in the pathogenesis of NEC in preterm infants and may be used as a diagnostic tool