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[Galactose loading test in infants and small children suffering in recurrent bronchitis and other chronic illness
Insights
Minor galactose metabolism deviations cause early-life symptoms in children. A lactose-free diet improved most patients with chronic diseases, suggesting altered galactose pathways.
Area of Science:
- Biochemistry
- Pediatrics
- Metabolic Disorders
Context:
- Investigated children with chronic conditions like recurrent bronchitis, vomiting, diarrhea, milk intolerance, and developmental delays.
- Assessed galactose metabolism through loading tests in 92 children, identifying pathological levels in 32 cases.
- Observed family history of stillbirth, cataracts, hyperbilirubinemia, and convulsions in 10 patients, suggesting potential genetic links.
Purpose:
- To evaluate galactose metabolism in children with chronic diseases.
- To identify potential alternative metabolic pathways for galactose when galactose-1-phosphat-uridyl-transferase levels are normal.
- To assess the efficacy of a lactose-free diet in managing symptoms related to galactose metabolism deviations.
Summary:
- Galactose loading tests revealed pathological, pseudo-diabetic levels in 32 of 92 children with chronic illnesses.
- Galactose-1-phosphat-uridyl-transferase levels were decreased in only 4 of 17 patients, indicating other metabolic pathways may be involved.
- A lactose-free diet led to complete symptom remission in 29 patients, with one infant death and two remaining mentally retarded.
Impact:
- Highlights that minor galactose metabolism deviations are more common causes of early-life clinical symptoms than previously thought.
- Demonstrates the therapeutic potential of dietary interventions (lactose restriction) for specific pediatric metabolic disorders.
- Suggests the need for broader screening of galactose metabolism in infants and children presenting with unexplained chronic symptoms.
Abstract:
The authors performed galactose loading tests in children suffering from chronic diseases: recurrent bronchitis vomiting, diarrhoea, milk-intolerance, somatic and mental retardation, cramps. In 32 of the 92 examined cases galactose levels rose until pathological, pseudo- diabetic levels. Stillbirth, cataract, hyperbilirubinaemia, convulsions occurred among family members of 10 patients. Galactose-1-phosphat-uridyl-transferase levels were decreased only in 4 of the 17 patients examined. In the other cases some different pathway of galactose metabolism is suspected. Complete remission of symptoms was achieved with diet devoid of milk sugar (lactose) in 29 patients: one infant died and two others remained mentally retarded. According to the examinations presented minor deviations of galactose metabolism cause clinical symptoms more frequently in early life as it was supposed until now.