We report the outcome of VLBWI born in a developing country with high resources. The rates of CLD, IVH and ROP were < or =25th percentile of the VON and mortality, NEC and PVL were in the 50th percentile.

Background: Therapeutic hypothermia provides up to 30% neuroprotection in moderate to severe hypoxic ischemic encephalopathy (HIE). Additional neuroprotection may be achieved by using concomitant pharmacologic neuroprotective agents. Aim: The aim was to evaluate the safety of concomitant neuroprotective therapy of therapeutic hypothermia and magnesium sulfate (MgSO 4) in the management of moderate and severe HIE in term and near-term infants. Study Design: Multicenter double-blind randomized controlled trial. Methodology: Term and near-term newborn infants (≥35 weeks) with a clinical diagnosis of moderate or severe HIE were randomized to either Arm A (therapeutic hypothermia plus MgSO 4) or Arm B (therapeutic hypothermia plus placebo) using a net-based randomization system. Both groups received, within 6 h of birth, standard hypothermia therapy (72 h of cooling to 33.5°C followed by slow rewarming over a period of 8 h) plus either MgSO 4 (250 mg/kg/dose ×3 doses) or placebo (normal saline). The groups were compared for short-term predischarge adverse outcomes. Results: A total of 60 patients were randomized (29 in Arm A and 31 in Arm B). Both groups had similar baseline characteristics (P > 0.05) including severity of HIE. There were no differences in the short-term adverse outcomes (death, seizures, thrombocytopenia, coagulopathy, renal failure, elevated liver function test's, hypotension, intracranial hemorrhage, necrotizing enterocolitis, pulmonary hemorrhage, persistent pulmonary hypertension, and pulmonary air leak syndromes) between the two groups (P > 0.05). Conclusions: The combined use of therapeutic hypothermia and MgSO 4 appears to be safe particularly with respect to maintaining normal blood pressure and coagulopathy. Long-term survival and neurodevelopmental outcomes remain to be evaluated.

BackgroundEllis-van Creveld (EvC) syndrome is an autosomal recessive chondrodysplastic condition with clinical manifestations that include short-limbs and ribs, postaxial polydactyly and dysplastic nails and teeth. In about two thirds of patients, mutations in either EVC or EVC2 genes have been found to be the underlying cause.MethodsIn this paper, we describe the molecular (DNA sequencing) and clinical analysis of six children diagnosed with EvC from four different families from the United Arab Emirates (UAE).ResultsAll the children had the common clinical and radiological features of this syndrome. However, DNA sequence analysis of the genes shown to be involved (EVC and EVC2) revealed a novel splice site mutation (c.2047-1G>T) in intron 13 of EVC2 gene in one family. In addition, we confirm previous mutational analyses that showed a truncating mutation in exon 13 of EVC gene (c.1813C>T; p.Q605X) in the second family and a single nucleotide deletion (c.981delG; p.K327fs) in exon 8 of EVC2 gene in the third family. No mutations in the exons, splice sites or the promoter regions of either gene have been found in the index case of the fourth family who exhibited "EvC-like" features.ConclusionsGiven the small population size of UAE, our data illustrates further the molecular heterogeneity observed in EvC patients and excludes the possibility of a common founder effect for this condition in the UAE reflecting the current ethnic diversity of the country.

Palivizumab may be an effective intervention during nosocomial outbreaks of RSV in a subgroup of extremely low-birth-weight male infants with hemodynamically significant congenital heart disease.

Implementing a structured comprehensive multidisciplinary program to manage GDM women has a positive impact on improving the care and outcomes of neonates.