An alarming rise of dengue has been seen in Mumbai during the post-monsoon season. We undertook this prospective study in the pediatric wards and pediatric intensive care unit of B. J. Wadia Hospital for Children between 27 August 2003 and 10 October 2003 to determine the clinical features, laboratory abnormalities, and outcome of children affected with dengue and to determine the predictive markers for dengue shock syndrome. Fifty-one suspected dengue cases were tested for positivity of dengue by determination of dengue IgM antibodies by ELISA test. These positive cases were analysed for common clinical features, laboratory derangements, and outcome. Patients were subdivided into three subgroups: dengue fever (DF), dengue hemorrhagic fever (DHF), and dengue shock syndrome (DSS) as per WHO classification. Predictive markers for DSS were also determined. Thirty-nine patients had a positive dengue IgM titre, 20 patients had DHF, 18 patients had DSS, and one patient had DF The mean age of presentation was 4.9 years. Fever, hepatomegaly, vomiting, bleeding tendencies, erythematous rash, thrombocytopenia, elevated liver enzymes, and deranged PT and PTT were the predominant clinical and laboratory features. Predictive markers for DSS were younger age at onset, altered sensorium, paralytic ileus, and significantly deranged PT. Patients with DSS also had a longer recovery period and required more supportive management in the form of component therapy and ionotropic support. All three patients who died belonged to the DSS subgroup with case fatality rate for DSS being 16.6 per cent. None of the patients in the DHF or DF subgroup died. Endemicity of dengue fever is on the rise in Mumbai with increased incidence among children. Appropriate investigations, strict monitoring and prompt supportive management can reduce mortality in dengue. Predictive markers of DSS can reduce the mortality if promptly treated. Also prevention of transmission by mosquito control and maintaining water sanitation is required to effectively control this epidemic.

The aim of this prospective study was to determine the adverse effects of antiretroviral therapy in HIV-1 infected children and factors associated with adverse effects. The study was performed in a pediatric and perinatal HIV clinic in a tertiary general hospital. Forty-three HIV positive children from the age group of 5 months to 14 years were started on antiretroviral therapy ART. Thirteen patients (30%) had adverse effects related to the ART. Seven patients (16%) had hepatotoxicity, 5 patients (12%) had raised serum amylase without symptomatic pancreatitis, 5 patients (12%) had zidovudine AZT induced anemia, 4 patients (9%) had Nevirapine NVP induced rash, 1 patient (2%) had Didanosine ddI induced pain in abdomen, 1 patient (2%) had Stavudine d4T induced angioedema, and 1 patient (2%) had hepatic steatosis. Five patients (71%) with hepatotoxicity responded to dose adjustment of ART whereas in 2 patients (29%), the elevated liver enzymes resolved on its own. Two patients (40%) with AZT induced anemia required omission of AZT and remaining 3 patients (60%) responded to dosage adjustment. ddI induced abdominal pain, d4T induced angioedema and hepatic steatosis resolved on omitting the respective antiretroviral drug. NVP induced rash and raised serum amylase subsided without any intervention. Hepatotoxicity was seen at higher viral load (Mean = 118608 copies/ml) whereas elevated serum amylase was seen at lower viral load (mean = 37631 copies/ml), which was statistically significant (p < 0.0001). NVP induced rash was seen in early weeks of therapy, serum amylase abnormalities were seen at a mean interval of 0.9 years after starting therapy, hepatotoxicity was seen at a mean interval of 1.7 years and AZT induced anemia was seen at a mean interval of 2.0 years after starting therapy. Adverse effects with antiretroviral drugs in HIV-infected children are quite common. Hepatotoxicity is the commonest adverse effect noted followed by elevated serum amylase and zidovudine induced anemia. Hepatotoxicity is seen at higher viral load as compared to other adverse effects. Most of the adverse effects are reversible on dosage modification or omitting the offending drug.

i.m. midazolam is an effective agent for controlling acute convulsions in children especially in children with febrile convulsions. It has relatively no side effects as compared to Intravenous diazepam and can be used as a first line agent for treatment of acute convulsions in patients with difficult intravenous access.

We report a male neonate who had liver abscess that resolved with intravenous antibiotics and surgical drainage. However, the child developed complete thrombosis of portal vein with cavernous formation within 16 days of therapy and portal hypertension subsequently. The child is now 2 1/2 years and has extra hepatic portal hypertension but is otherwise asymptomatic.

Nevirapine blood concentrations are affected by many factors, most notably age ≤ 3 years; a combination of young age, stunting and CYP2B6 GG or GT genotype could potentially result in sub-therapeutic nevirapine concentrations. Dosing recommendations for children should be reviewed in the light of these findings.

Chronic granulomatous disease (CGD) is a group of inherited disorder of phagocytes, resulting from mutations in the components of the NADPH oxidase complex. Reduced or absent oxygen radical synthesis seen in these patients leads to impaired killing of intracellular bacteria and fungi. CGD clinically presents with recurrent and life-threatening infections as well as granulomatous inflammatory responses. p47 encoded by the NCF1 gene is the most common autosomal recessive form of CGD which is often clinically milder. Here, we are presenting the data on clinical and immunological findings in 21 Indian patients with Del GT mutation in the NCF1 gene. Diagnosis of these patients was based on detailed clinical evaluation, measurement of respiratory burst activity by nitro blue tetrazolium and dihydrorhodamine-1,2,3 assay, expression of p47 by flow cytometry, and molecular confirmation by GeneScan method. Seventeen male and four female patients with median age of onset of 1 year ranging from 1.5 months to 6 years were included in the study. Sixty-two percent (13 out of 21) of patients belonged to a consanguineous marriage with only one family having a history of a previous sibling death. Significant variability in clinical presentation was observed in spite of identical genetic defect ranging from asymptomatic to very severe presentation leading to early death or requiring transplantation. However, none of these patients showed difference in immunological parameters to account for this variability. Thus, this study highlights the phenotypic heterogeneity seen in these patients with Del GT mutation in the NCF1 gene and its implication in management of these patients.

CMV is present in the liver tissues of more than half the patients with NC. Serology or blood CMV PCR is apparently not an accurate marker of CMV in the liver tissue. Also, CMV infection in children seems to be associated equally with BA or non-BA neonatal hepatitis.

Acute hepatitis and hepatic encephalopathy are rare manifestations of dengue haemorrhagic fever (DHF). We report 4 children aged 8 months to 3 y who presented with severe hepatic dysfunction. Three male infants had in addition hepatic encephalopathy and 2 of them succumbed to their disease suggesting that hepatitis with encephalopathy has a very high mortality.