Hyperammonaemia in children can lead to grave consequences in the form of cerebral
oedema, severe neurological impairment and even death. In infants and children, common …

Hyperammonemia occurs frequently in the critically ill but is largely confined to patients with
hepatic dysfunction or failure. Non-hepatic hyperammonemia (NHHA) is far less common …

This study develops a novel fluorometric method for the sensitive and selective
determination of urea, based on unique system comprising nitrogen doped red-emissive …

Citrin deficiency (CD) is a complex metabolic condition due to defects in SLC25A13
encoding citrin, an aspartate/glutamate carrier located in the mitochondrial inner membrane …

The pathway of ammonia disposal in the mammalian organism has been described in 1932
as a metabolic cycle present in the liver in different compartments. In 1958, the first human …

Hepatic encephalopathy (HE) is a severe metabolic syndrome linked with acute/chronic
hepatic disorders. HE is also a pernicious neuropsychiatric complication associated with …

Neonatal onset hyperammonemia in patients with urea cycle disorders (UCDs) is still
associated with high morbidity and mortality. Current protocols consistently recommend …

Aim Clinical practice guidelines (CPGs) have progressively become a popular tool for
making optimal clinical decisions. The literature shows that the poor quality of CPGs can …

Background Continuous kidney replacement therapies (CKRT) have been reported to be an
effective approach to removing toxic metabolites in inborn errors of metabolism (IEM). The …

Effective point-of-care (POC) methods for detecting metabolic disorders, such as
hyperammonemia, are crucial. Urinary ammonia is an underexplored but physiologically …