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Transient hyperammonemia of the newborn
Transient hyperammonemia of the newborn (THAN) is an idiopathic disorder occasionally present in preterm newborns but not always symptomatic.〔 Continuous dialysis or hemofiltration have proven to be the most effective treatment.〔 Nutritional support and sodium benzoate have also been used to treat THAN.
==Signs and symptoms==
Hyperammonemia occurs when the body produces excess ammonia. This ammonia primarily exists as ammonium ion that has a concentration less than or around 35 µmol/L in normal referenced serum levels. Excess ammonia is processed in the liver through the urea cycle to produce urea.〔 Excess ammonia can be produced by bacterial hydrolysis of intestinal compounds, purine nucleotide cycles, the transamination of amino acid in voluntary muscles, and other metabolic events of filtration organs(kidneys and liver).〔 In THAN, symptoms of hyperammonemia are observed within 24 hours of birth, and the causation of hyperammonemia must exclude urea cycle disorders. If the amount of ammonia entering the brain is increased, neurological disorders such as urea cycle enzyme deficiencies, Reye syndrome, seizures, and encephalopathies may occur.〔 The most common indicator of THAN is respiratory distress syndrome. In newborns with THAN, the primary cause is thought to be genetic, but it has not been narrowed down to one gene or locus so the exact cause remains unknown. Observable CNS depression, comatose, metabolic acidosis, feeding difficulties, cyanosis, abnormal EEG, increased intraventricular hemorrhage, hypotonia, and irratibility are common symptoms of THAN. Individuals that develop hyperammonemia after birth are more likely to have hyperammonemia as a result in urea cycle enzyme deficiency (UCED).〔
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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