Background of the study: Preterm infants are managed with antibiotics for sepsis, including suspected or probable sepsis. This leads to a delayed and abnormal colonization of the gut with potentially pathogenic organisms and a microbiome, which lacks biodiversity and increases the risk for late-onset sepsis (LOS). Probiotics have been proven to reduce the risk for necrotizing enterocolitis, but evidence for prevention of LOS is inconclusive. Probiotic effect depends also on the strain used, dose and indication for use. This study evaluated Bacillus clausii probiotic administered prophylactically to preterm neonates for prevention of LOS. Objectives: To study B.clausii given prophylactically to preterm neonates for prevention of LOS. Design: Double-blinded, placebo-controlled, randomized trial. Settings: Tertiary care neonatal unit in India. Participants: Consecutive preterm neonates <34 weeks, admitted from 1 March 2012 to 28 February 2014 were stratified as extreme preterm and very preterm. Intervention: Randomized to receive either probiotic or placebo for 6 weeks, discharge from hospital, death or occurrence of sepsis, whichever was earlier. Primary outcome: Incidence of definite and probable LOS in probiotic group compared with placebo. Results: Of 326 eligible preterm infants, 244 were enrolled and 82 were excluded. Of these, 120 were stratified as extreme preterm and randomized to receive placebo (n = 59) and probiotic (n = 61). Of 124 babies stratified as very preterm, an equal number was randomized to receive placebo (n = 62) and probiotic (n = 62). There was no significant difference in the incidence of LOS between the two arms in the extreme preterm group [29% vs. 23%; relative risk (RR) 1.27; 95% confidence interval (CI) 0.88–1.66; p = 0.36] and the very preterm group (13% vs. 10%; RR 1.33; 95% CI 0.96–1.70; p = 0.32). Full feeds were achieved significantly faster in the probiotic group in both the extreme preterm (RR 0.82; 95% CI 0.74–0.88) and the very preterm (RR 0.67; 95% 0.32–0.77). Conclusions: Prophylactic administration of B.clausii to preterm neonates did not result in a significant difference in the incidence of LOS as compared with placebo.

Enteral feeding in preterm neonates with intrauterine growth restriction (IUGR) and absent or reversed end diastolic flow (AREDF) on umbilical artery (UA) Doppler is delayed owing to an increased risk of necrotizing enterocolitis (NEC). Delaying enteral feeding with longer duration of parenteral nutrition (PN) carries an increased risk of sepsis. To study early versus late feeding in preterm IUGR neonates for time required to attain sufficient feed volume to discontinue PN and increased risk of NEC or feed intolerance (FI). Open-label randomized controlled trial. Tertiary care neonatal unit and fetal-maternal medicine unit in India. Preterm intrauterine growth restricted neonates’ ≤32 weeks with AREDF on UA Doppler enrolled from 1 January 2014 to 31 July 2015. Randomized to receive early or late feeding using mothers own or donor breast milk as per a feed initiation and advancement protocol. Time in days required to attain sufficient feed volume allowing discontinuation of PN and incidence of NEC in neonates fed early versus late. There were 77 eligible neonates. Sixty-two neonates were included and stratified as extreme preterm (27–29 weeks) (n = 20) and very preterm (30–32 weeks) (n = 42). Ten extreme preterm and 21 very preterm neonates were randomized to each early feeding and late feeding arm. There was a significantly faster attainment of sufficient feeds in the early feeding arm of both the stratified groups [extreme preterm: median 14 days (Interquartile range IQR: 12–15) compared with 18 days (IQR: 18–20), hazard ratio (HR): 1.59, 95% CI: 0.626–4.078; very preterm: 12 days (IQR: 10–14) as compared with 16 days (IQR 15–17), HR: 1.89, 95% CI: 1.011–3.555]. There was no difference in the incidence of NEC, FI and combined outcome of NEC and FI. Early feeding in preterm IUGR neonates with AREDF on antenatal UA Doppler allowed earlier discontinuation of PN, allowing birth weight to be regained earlier and did not increase the incidence of NEC and FI.

Background: Dengue is a major health issue with seasonal rise in dengue fever cases imposing an additional burden on hospitals, necessitating bolstering of services in the emergency department, laboratory with creation of additional dengue fever wards.Objectives: To study the clinical and hematological profile of dengue fever cases presenting to a hospital.Methods: Patients with fever and other signs of dengue with either positive NS1 antigen test or IgM or IgG antibody were included. Age, gender, clinical presentation, platelet count and hematocrit were noted and patients classified as dengue fever (DF), dengue hemorrhagic fever (DHF) or dengue shock syndrome (DSS). Duration of hospitalization, bleeding manifestations, requirement for platelet component support and mortality were recorded.Results: There were 443 adults and 57 children between 6 months to 77 year age. NS1 was positive in 115 patients (23%). Fever (99.8%) and severe bodyache (97.4%) were the commonest presentation. DF was seen in 484 (96.8 %), DHF in 10 (2%) and DSS in 6 cases (1.2%). OPD treatment was needed in 412 (82%) and hospitalization in 88 (18%). Intravenous fluid resuscitation was needed in 16 (3.2%) patients. Thrombocytopenia was seen in 335 (67%) patients at presentation. Platelet transfusion was needed in 46 (9.2%). PRC transfusion was given in 3 patients with DF and 10 of DHF. Death occurred in 03 DSS and 2 DHF patients. Conclusions: Majority of DF cases can be managed on OPD basis. DHF and DSS carry high mortality. Hospitals can analyze annual data for resource allocation for capacity expansion.

Background of the study: Neonates at risk for early-onset sepsis are started on antibiotics empirically. Antibiotic resistance to conventionally used antibiotics is increasingly being reported. Antenatal maternal antibiotic exposure in this setting contributes to low yield on blood culture drawn at birth, limiting the planning of antibiotics based on culture reports. A head-to-head comparison for selecting the appropriate antibiotic is one strategy. Objectives: To compare monotherapy with amikacin against piperacillin-tazobactum as an empirical therapy in neonates at risk for early-onset sepsis. Design: Randomized open-label controlled trial with stratification and block randomization. Settings: Tertiary care neonatal unit in India Participants: All consecutive inborn neonates delivered between 01 May 2009 and 30 April 2011 who were ≥28 week gestation and/or ≥1000 g birth weight with risk factors for early-onset sepsis. Intervention: Randomized to receive either amikacin or piperacillin-tazobactum, after stratifying as asymptomatic or symptomatic within 1 h of birth. Primary outcome: Incidence of treatment failure to the allocated antibiotic defined as blood culture isolate reported resistant to the allocated antibiotic or progression of the illness, necessitating a change of antibiotic. Results: Of 204 eligible cases, 187 were enrolled. Seventeen babies were excluded. A total of 128 neonates were stratified as asymptomatic and 59 as symptomatic. In all, 64 of the asymptomatic cases received amikacin and 64 received piperacillin-tazobactum, while 29 symptomatic babies received amikacin and 30 received piperacillin-tazobactum. Five babies had blood culture–positive sepsis, and 28 babies had strong suspicion of sepsis. There was no difference in the treatment failure in the amikacin group (3 of 93; 3.2%) compared with piperacillin-tazobactum group (2 of 94; 2.1%) (p > 0.01) and no difference in the incidence of second infection, fungal sepsis and all-cause mortality at day 7 and 28 between the two study groups (p > 0.01). Conclusions: Monotherapy with amikacin as an empirical antibiotic did not result in a higher incidence of treatment failure in neonates at risk for early-onset sepsis as compared with piperacillin-tazobactum. Both antibiotics were effective in management of babies with early-onset sepsis.

4H syndrome is a congenital hypomyelinating leukodystrophy characterized by hypodontia, hypomyelination and hypogonadotropic hypogonadism belonging to the Pol III-related leukodystrophies which arise due to mutations in the POLR3A or POLR3B gene. The clinical presentation is of neurodevelopmental delay or regression with ataxia, dystonia, nystagmus, delayed deciduous dentition and abnormal order of eruption of teeth. MRI brain shows a characteristic hypomyelination pattern. Several mutations have been described in the implicated genes but there are no reports on mutations seen in patients from India. We report a 1½ year old girl, only child of a non-consanguinous couple who presented with delayed developmental milestones and delayed dentition. On physical examination she had downward slanting palpebral fissures, low set ears, smooth philtrum, hypodontia, prominent body hair and clitoromegaly. There was prominent horizontal nystagmus, hypertonia of both upper and lower limbs, exaggerated deep tendon jerks and flexor planter response. She had not attained complete head control and required support to sit. She showed absent waves on brainstem evoked response audiometry and her fundus examination showed bilateral optic atrophy with prolongation of P100 latencies on visual evoked potentials. MRI Brain showed hyperintensity of entire white matter with involvement of the internal and external capsule, frontal deep white matter and corpus callosum. Her karyotype was 46 XX and her endocrinal profile was unremarkable. Clinical exome sequencing identified an unreported mutation in the POLR3A gene. The same mutation was identified by Sanger sequencing in heterozygous state in both parents. The child is being managed with physiotherapy and developmental therapy. She has been provided with hearing aids and started on speech therapy. Parents were provided anticipatory guidance and genetic counselling about autosomal recessive nature of inheritance, risk of recurrence and need for follow-up. 4H syndrome is a rare congenital hypomyelinating leukodystrophy inherited as an autosomal recessive disorder due to mutations in the POLR3A and POLR3B gene. Delay or regression of milestones, abnormalities in dentition and endocrinal perturbations are its hallmark. A novel mutation in the POLR3A gene resulting in amino acid substitution of arginine for glutamine at codon 808 (p.R808Q) was detected in exon 18 in our case.