[Plasma ammonia concentrations in mucoviscidosis]. / Plasma-Ammoniakkonzentration bei Mukoviszidose.

In 16 children and adolescents suffering from mucoviscidosis the ammonia concentration of the plasma determined by a modified ion exchange method was compared with the clinical degree of severity. The ammonia values of the plasma were compared with several parameters, such as Shwachman stage, the presence of cardio-respiratory insufficiency, blood gas values and liver function tests. In 5 children with cystic fibrosis and cardio-respiratory insufficiency high plasma ammonia values were found. In older children with the disease lasting for a longer time with medium Shwachman stage (stage III) but without cardiorespiratory insufficiency the ammonia values were also relatively high. Cardiorespiratory insufficiency and disturbance of the liver function may play a role in the development of hyperammonemia. A distinct hyperammonemia refers to a bad prognosis in children with cystic fibrosis.