Studies are supporting neuroprotective benefi t of therapeutic hypothermia in term newborns with hypoxic-ischemic encephalopathy.We assessed survival and neurodevelopmental outcome of neonates subjected to the procedure and factors that may haveinfl uenced it. Newborns with gestational age of more than 36 weeks and less than 6 hours of age with moderate to severe asphyxialencephalopathy underwent cooling protocol at a temperature of 33.5 °C for 72 hours and rewarming period of 6 hours. The outcomemeasures assessed were death and neurodevelopmental characteristics. Twenty-fi ve children were assessed during the period fromOctober 2010 to October 2013. Median gestational age was 40 weeks, birth weight 3470 g, Apgar score 2/4 and pH on admission tothe hospital 7.02. Four (16%) children died and two were lost for follow up. At the age of fi nal assessment, developmental categoriesof communication were normal in 68.4%, problem solving in 73.7%, personal-social in 68.4%, gross motor in 57.9%, and fi ne motorin 36.8% but with a high need of retesting in this area. Seven of 19 patients (36.8%) had completely normal results for all fi ve categories,while three (15.8%) had abnormal results for all categories. None of the 18 parameters that were correlated with neurodevelopmentaloutcome showed statistical signifi cance. Amplitude integrated electroencephalography was done in ten patients and themost prominent fi nding was discontinuous activity in eight patients. In conclusion, a relatively small number of patients and limitationsof this study design precluded any far-reaching conclusions, but we think that this method can provide better survival and lessneurologic sequels in hypoxic-ischemic encephalopathy patients.
Wolf-Hirschhorn Syndrome: Report of two Cases in Bosnia and Herzegovina Wolf-Hirschhorn syndrome (WHS) is a rare developmental disorder caused by a partial deletion of the short arm of chromosome 4 (4p-). The main phenotypic characteristics of WHS are: intrauterine growth retardation, mental retardation, typical facial dysmorphism, microcephaly and midline fusion defects (cleft lip or palate, cardiac septal defects). Other abnormalities, such as agenesis of the corpus callosum, dysplastic kidneys, iris coloboma and skeletal abnormalities have occasionally been described. We describe two female newborn babies with a 4p deletion, who have a majority of the main phenotypic features of WHS. Prenatal diagnosis of the syndrome is very important, because dysmorphologic features are associated with profound mental retardation. Postnatal recognition of the syndrome requires genetic counseling of the parents and supportive multidisciplinary treatment.
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