Indian journal of pediatrics
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Case Reports
Intermittent hyperammonemic encephalopathy in a child with ornithine transcarbamylase deficiency.
Ornithine transcarbamylase (OTC) deficiency is an X-linked disorder and the most common inherited cause of hyperammonemia. Clinical manifestations are more severe in hemizygous males who often present in neonatal period. ⋯ Fluctuating concentration of ammonia, glutamine and other excitotoxic amino acids result in a chronic or episodically recurring encephalopathy. The authors report a heterozygous female with OTC deficiency who presented with recurrent encephalopathy.
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To study the profile and outcome of children admitted to a tertiary level pediatric intensive care unit (PICU) in India. ⋯ Our data appears to be similar with regards to PRISMIII scores and adjusted mortality, length of the PICU stay, and duration of ventilation, to previously published western data. Multiorgan failure remains a major cause of death. As expected, Dengue and malaria were common. Incidence of nosocomial infections was somewhat high. Interestingly, more boys got admitted to the PICU as compared to girls. Clearly more studies are required to assess the overall outcomes of critically ill children in India.