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Published on: November 27, 2016
Five Arab children with glucose-galactose malabsorption
Asaad Assiri1, Anjum Saeed, Abdulrehman Alnimri
1Department of Pediatrics, Faculty of Medicine, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia. prof-asaad@hotmail.com
Insights
Glucose-galactose malabsorption (GGM) is a rare condition causing chronic diarrhea in infants. Early diagnosis and fructose-based formulas are crucial for managing this glucose malabsorption disorder.
Area of Science:
- Pediatrics
- Gastroenterology
- Human Genetics
Background:
- Glucose-galactose malabsorption (GGM) is an inherited disorder affecting nutrient absorption.
- It presents in infancy with severe gastrointestinal symptoms.
Observation:
- Five infants with GGM from diverse origins were studied.
- All infants exhibited chronic diarrhea, failure to thrive, and reducing substances in stools.
- Sugar chromatography confirmed glucose and galactose malabsorption, with normal small bowel biopsies.
Findings:
- Complications included dehydration, hypernatremia, gangrene requiring amputation, and nephrolithiasis.
- All infants showed significant improvement with fructose-based formulas.
- This highlights the critical role of dietary management.
Implications:
- GGM should be considered in the differential diagnosis of infant chronic diarrhea.
- Prompt diagnosis and appropriate management with fructose-based diets can prevent severe complications.
- Genetic counseling may be beneficial for affected families.
Abstract:
Five children with glucose-galactose malabsorption (GGM) are presented. Two infants from Saudi Arabia were first-degree relatives, the third infant was unrelated and the other two were of Yemeni and Syrian origin, respectively. All the infants had chronic diarrhoea and four had failed to thrive since early infancy. All had stools positive for reducing substances, and sugar chromatography showed glucose and galactose malabsorption. Small bowel biopsies were normal in all. One infant developed gangrene of both legs as a complication of hypernatraemia and dehydration, necessitating bilateral amputation. Two infants had nephrolithiasis. All the infants responded well to fructose-based formulae. GGM should be considered in the differential diagnosis of chronic diarrhoea in infants breastfed or artificially fed from early life.
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