Idiopathic hyperammonemia is characterized by increased serum ammonia levels (>200umol/L) and is sometimes associated with normal to slightly elevated liver function tests [1]. These patients often present with encephalopathy, cerebral edema, seizures, and coma. Idiopathic hyperammonemia has been reported after high dose chemotherapy, and organ transplants including Orthotopic lung transplant [...

Background Literature suggests that proinflammatory mechanisms are implicated in the pathophysiology of hepatic encephalopathy. This is mainly caused by high circulating levels of ammonia (hyperammonemia-HA), due to liver failure [1,2]. In addition, NMDA receptors are excessively activated during acute hyperammonemia and thus significally contribute to the brain damage [3]. In fact, blockage of...

Hyperammonemia can be secondary to hepatic or non hepatic and idiopathic causes. Among non hepatic or non cirrhotic causes of hyperammonemia, excess of ammonia production or decrease in ammonia excretion predominates to produce the clinical syndrome. Cirrhosis is the commonest cause of hyperammonemia in adults. In children, disorders of urea cycle need to be considered as a suspecting but rare ...

Ornithine transcarbamylase deficiency (OTCD) is an X-linked disorder, with an estimated prevalence of 1 per 80000 live births. Female patients with OTCD develop metabolic crises that are easily provoked by non-predictable common disorders, such as genetic (private mutations and lyonization) and external factors; however, the outcomes of these conditions may differ. We resuscitated a female pati...

Hyperammonemia is considered to be central to the pathophysiology of hepatic encephalopathy in patients exhibiting hepatic failure (HF). It has previously been determined that hyperammonemia is a serious metabolic disorder commonly observed in patients with HF. However, it is unclear whether hyperammonemia has a direct adverse effect on hepatic cells or serves as a cause and effect of HF. The p...

how to cite this article: khalessi n, khosravi n, mirjafari m, afsharkhas l. plasma ammonia levels in newborns with asphyxia. iran j child neurol. winter 2016; 10(1):42-46. abstract objective perinatal asphyxia may result in hypoxic damage in various body organs, especially in the central nervous system. it could induce cascade of biochemical events leading to the cell death and metabolic chang...

Hepatic encephalopathy in patients with liver disease is associated with poor prognosis. This could be due to the induction by the transient episode of hepatic encephalopathy of long-lasting alterations making patients more susceptible. We show that a single transient episode of hyperammonemia induces long-lasting alterations in signal transduction. The content of the regulatory subunit of the ...

Hyperammonemia syndrome is one of the causes of metabolic encephalopathy that is rarely observed after high dose chemotherapy for the treatment of hematologic malignancies. Two cases, who developed neurologic symptoms and coma due to hyperammonemia subsequent to chemotherapy for Burkitt’s lymphoma and acute myeloid leukemia, respectively are being presented in this report. In case respiratory a...

CONTEXT Idiopathic hyperammonemia is characterized by elevated serum ammonia associated with neurological deterioration of no other obvious etiology associated with relatively normal liver function tests and normal amino-acid levels. CASE REPORT We report a case of a 32-year-old woman who presented with acute mental status changes with a pelvic abscess approximately a year following her total...

Hyperammonemia is commonly encountered in active liver disease. Evaluation of patients having hyperammonemia with normal liver function is difficult. We present a case referred to us as undiagnosed hyperammonemic coma with normal liver function, who was subsequently diagnosed to have Hashimoto's encephalopathy. In patients with hyperammonemia without hepatic dysfunction, one must search for the...