Treatment of acute infectious purpura fulminans with activated protein C.

Infectious purpura fulminans is associated with high mortality and morbidity despite standard antimicrobial therapy. We report satisfactory clinical outcome in two children with sepsis associated purpura fulminans who were treated with activated protein C (APC). There is need for proper evaluation of the efficacy of this extremely expensive therapeutic modality by randomized controlled trials before it is made standard of care in childhood infectious purpura fulminans.

Anemia in the newborn.

In neonatal period anemia is a complex problem owing to the unique blood picture. The erythrocytic system undergoes serial adaptation to meet progressively changing demands of oxygen in the embryo, the fetus and neonate. This leads to rapid change in normal hematological change in post-birth period. Definition of anemia is difficult because as described earlier, several important factors influence normal blood in the newborn infants. The etiology of neonatal anemia can be classified into i) hemorrhage (ii) hemolysis (iii) failure of red cell production. Severe fetal hemorrhage may accompany various placental anomalies like placenta praevia, abruptio placenta and accidental incision of placenta during the caesarian section. It is reported that 10% of all infants born following placenta praevia and 4% of infants born following abruptio placenta present with severe anemia. The passage of fetal erythrocytes in maternal circulation occurs commonly during pregnancy. In 50% of pregnancies some fetal cells are passed in maternal circulation sometimes during gestation or during birth process. Treatment of a neonate with anemia due to blood depends on the degree of hypovolemia or anemia and whether the blood loss has been acute or chronic. Newborn with pale skin should be differentiated from an asphyxiated baby.

Diamond-Blackfan anemia: report of 6 cases.

Diamond-Blackfan anemia is a rare congenital hypoplastic anemia. We report 6 children diagnosed as Diamond-Blackfan anemia at our clinic. All had severe pallor at presentation, with mild hepatomegaly and just palpable spleen in one child. Thumb anomaly was present in one of them. All of them had macrocytic or normocytic anemia with reticulocytopenia, and bone marrow examination revealed marked erythroid hypoplasia. All of them were treated with oral steroids with a good response.

Age related seroprevalence of antibodies to varicella in India.

OBJECTIVE: To determine the age related prevalence of Varicella Zoster Virus (VZV) antibodies in India. SETTING: This was a cross sectional multicentric study performed in 4 major cities of India: Calcutta (outpatients), Mumbai (outpatients), Lucknow (walk-in patients to a diagnostic laboratory, orphanage and factory workers) and Bangalore (outpatients and walk-in patients to a diagnostic laboratory). METHODS: A total of 1609 volunteers from birth to 40 years of age were included into the study. IgG antibodies against VZV were determined using commercial kits (ELISA-Enzygnost). RESULTS: Overall seroprevalence of anti VZV antibodies was 68. 22percnt. The age related seroprevalence rate of anti VZV antibodies was 29percnt in the age group of 1-5 years, 51.1percnt in 5-10 years, 71.7percnt in 11-15 years, 79.8percnt in 16-20 years, 88.1percnt in 21-30 years and 91.1percnt in 31-40 years. CONCLUSION: A significant proportion of adolescents and adults are susceptible to varicella in India, as in other tropical countries

Anemia in newborn.

Various blood indices vary in a newborn as compared to older child or adult. It depends on the gestational age, day of life, maternal factors, mode of delivery and site of blood collection. Hemoglobin, HCT & MCV tend to be higher in newborns. They further increase in first 2 days of life. Reticulocytosis and presence of nucleated red cells are normally seen in first week of life. Neonatal anemia is a common problem in NICU. It is usually caused by either hemorrhage or hemolysis and rarely due to decreased production. Hemorrhage can be ante or intra or post natal and it could be external or internal. It could be acute or chronic. Management of acute severe hemorrhage includes packed cell transfusion. Hemolysis is usually due to isoimmune hemolysis, G6PD deficiency or rarely due to the hemoglobinopathy like alpha-thalassemia or due to spherocytosis. Usually patients will have indirect hyperbilirubinemia which needs phototherapy or exchange transfusion. Rarely congenital pure red cell aplasia can present at birth with physical anomalies and anemia. Treatment of neonatal anemia depends on the arteriology.

Immunogenicity study of Haemophilus influenzae type B conjugate vaccine in Indian infants.

OBJECTIVE: To assess the immunogenicity in Indian infants to Haemophilus influenzae b oligosaccharide conjugate vaccine (HbOC). DESIGN: Prospective multicenter study. SETTING: Pediatric Out Patient Department of general hospitals in Pune and Mumbai. SUBJECTS: 124 full term healthy infants brought for routine DPT/OPV immunization. METHODS: Infants were administered 3 doses of 0.5 ml of HbOC, on the same day as their DPT/OPV immunization, injected intramuscularly on the limb opposite to that where DPT vaccine was administered. Data on local reactions and general symptoms was collected for three days after every dose. The children had their blood collected for assay of anti PRP (polyribosil ribitol phosphate) antibody titers, along with the first injection and one month after the third injection. One hundred and three infants completed the study protocol with two blood collections. RESULTS: The initial geometric mean titers (GMT) of 0.124 mcg/ml rose by 37 times to 4.552 mcg/ml. Ninety eight children (95.1%) had a final titer of > or = 0.15 mcg/ml, the minimum level associated with protection, and 77 children (74.8%) had a final level of > or = 1.0 mcg/ml, a level associated with long term protection. CONCLUSION: HbOC is immunogenic in Indian infants when used as per the locally recommended DPT/OPV immunization schedule.

'NESTROFT'--an effective screening test for beta thalassemia trait.

OBJECTIVE: To evaluate the efficacy of NESTROFT (Naked Eye Single Tube Red Cell Osmotic Fragility Test) as a screening tool for detection of beta thalassemia trait. DESIGN: Prospective study. SETTING: Field camps in various parts of Gujarat and Maharashtra States. METHODS: A total of 2525 subjects were screened. NESTROFT, complete hemogram including red cell indices and calculation of Mentzer's Fraction (MF) and discriminant functions (DF1-4) were done in all subjects. HbA2 was performed in 830 initial subjects to compute sensitivity, specificity and predictive values for various parameters. RESULTS: NESTROFT (sensitivity 94.4%), as a single screening parameter was superior to any of the other evaluated parameters individually, besides being cost effective. Mean corpuscular volume (MCV) < 80 fl followed NESTROFT closely (sensitivity 93.7; p > 0.05). MCV < 75 fl had a significantly (p < 0.001) lower sensitivity (87.3%) in comparison to both of these parameters. In contrast, MF, DF1, DF2, DF3 and DF4 did not meet the requirements of a good screening test with sensitivity values of 66.2%, 54.9%, 47.2%, 64.1% and 55.6%, respectively. NESTROFT in combination with MCV < 80 fl proved 100% sensitive. However, the combination was not cost effective. CONCLUSION: NESTROFT is a sensitive, cost effective, rapid and reliable screening test for detection of beta thalassemia trait in a population.

Evaluation of naked eye single tube red cell osmotic fragility test in detecting beta-thalassemia trait.

The Naked Eye Single Tube Red Cell Osmotic Fragility Test (NESTROFT) was applied to 4 groups of subjects: (i) Normal; (ii) Proven beta-thalassemia trait carriers; (iii) Iron deficiency anemia; and (iv) other hemoglobinopathies, to evaluate its effectiveness as a screening test for beta-thalassemia minor. The test was successful in detecting 105/110 subjects with beta-thalassemia trait. The sensitivity of the test was 95.5% and specificity was 87%. The predictive value of the positive test was 70.5% and that of the negative test was 98.3%. NESTROFT was also positive in 9/17 subjects with HbS trait, in 3/3 subjects with HbD trait and in 1/1 subjects with HbE trait. The test proved to be simple, cheap, easy to perform and adaptable for field surveys, coming close to an ideal screening test for beta-thalassemia minor.