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Congenital selective malabsorption of glucose and galactose
Journal of Pediatric Gastroenterology and Nutrition
|December 1, 1985
Summary
Congenital selective glucose and galactose malabsorption (GGM) causes severe diarrhea in infants. Dietary restriction of these sugars is essential, as transport defects are permanent but may improve with age.
Area of Science:
- Pediatric Gastroenterology
- Human Physiology
- Molecular Biology
Background:
- Congenital selective glucose and galactose malabsorption (GGM) is a rare inherited disorder.
- It presents in neonates with intractable, acidic diarrhea triggered by glucose and galactose intake.
- Symptoms resolve with dietary elimination of these specific sugars.
Purpose of the Study:
- To review case histories and in vivo/in vitro test results of eight children with GGM.
- To elucidate the underlying mechanisms of defective sugar transport in GGM.
- To investigate the long-term clinical course and potential for age-related improvement.
Main Methods:
- Longitudinal follow-up of eight pediatric patients diagnosed with GGM since 1971.
- In vivo clinical observations including dietary response and symptom monitoring.
- In vitro intestinal tissue analysis to assess glucose and galactose transport kinetics.
Main Results:
- GGM patients exhibited significantly reduced intracellular glucose concentrations in jejunal tissue.
- Defective sodium-glucose cotransport at the brush border membrane was identified as the primary defect.
- A small residual active transport system may be present, particularly at low glucose concentrations.
Conclusions:
- GGM is characterized by permanent defects in jejunal glucose and galactose absorption.
- Dietary management is critical for symptom control in affected infants.
- Further research is needed on the role of residual transport systems and genetic factors.