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[Plasma ammonia concentrations in mucoviscidosis]
Zeitschrift Fur Die Gesamte Innere Medizin Und Ihre Grenzgebiete
|August 1, 1983
Summary
High plasma ammonia levels in children with cystic fibrosis indicate a poor prognosis, especially with cardiorespiratory insufficiency or liver dysfunction.
Area of Science:
- Biochemistry
- Pediatrics
- Pulmonology
Context:
- Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs and digestive system.
- Hyperammonemia, elevated ammonia levels in the blood, is a potential complication in various chronic diseases.
- Assessing ammonia levels in pediatric CF patients can offer insights into disease severity and progression.
Purpose:
- To investigate the correlation between plasma ammonia concentration and the clinical severity of cystic fibrosis in pediatric patients.
- To compare ammonia levels with established clinical parameters like Shwachman stage, cardiorespiratory status, blood gas values, and liver function tests.
Summary:
- This study analyzed plasma ammonia levels in 16 children and adolescents with cystic fibrosis using a modified ion exchange method.
- Elevated ammonia levels were observed in patients with cardiorespiratory insufficiency and in older patients with longer disease duration and moderate Shwachman stage (III).
- Cardiorespiratory insufficiency and impaired liver function appear to contribute to hyperammonemia in cystic fibrosis.
Impact:
- Hyperammonemia serves as a significant indicator of a poor prognosis in pediatric patients with cystic fibrosis.
- These findings highlight the potential of monitoring plasma ammonia as a biomarker for disease severity and outcome prediction in CF.
- Understanding the link between hyperammonemia and CF complications can inform clinical management and therapeutic strategies.