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Treatment of an infant with congenital sodium diarrhea by oral rehydration
T Kidowaki1, H Funaki, R Mizuta
1Pediatric Department, Ohtsu Municipal Hospital, Japan.
Insights
Congenital sodium diarrhea (CNaD) is a rare condition causing watery stools and metabolic issues. Early electrolyte replacement and careful medication management are key for infant health.
Area of Science:
- Pediatric Gastroenterology
- Neonatology
- Genetics
Background:
- Congenital sodium diarrhea (CNaD) is a rare inherited disorder.
- Characterized by severe watery diarrhea and electrolyte imbalances from birth.
Observation:
- A male infant presented with abdominal distension, watery stools (high sodium), and metabolic acidosis within the first week of life.
- Hyponatremia, low urine sodium, elevated serum aldosterone, and high renin activity were noted.
- Other causes of secretory diarrhea were excluded.
Findings:
- Initial treatment with oral water and electrolyte supplements corrected hyponatremia and metabolic acidosis, leading to weight gain.
- Administration of loperamide hydrochloride, due to high serum prostaglandin, improved diarrhea but led to acholic stools.
- Discontinuation of loperamide resulted in a return of watery stools, indicating persistent CNaD.
Implications:
- This case highlights the diagnostic challenges and treatment strategies for congenital sodium diarrhea.
- Early and appropriate fluid and electrolyte management is crucial for infant survival and development.
- Further research into prostaglandin's role and targeted therapies for CNaD is warranted.
Abstract:
We have been following a male case of congenital sodium diarrhea (CNaD), who had a distended abdomen, passed watery stools with high sodium concentration, and showed metabolic acidosis in the first week of life. He also showed hyponatremia, low urine sodium, high serum aldosterone and high renin activity. Other possible causes of secretory diarrhea were ruled out. The initial effective treatment was oral supplements of water and electrolytes lost in the fecal fluid: with this he gained weight, and hyponatremia and metabolic acidosis were corrected. Loperamide hydrochloride was administered to increase intestinal absorption of sodium, as the serum prostaglandin was high. Watery diarrhea then improved, but as he passed acholic stools, we stopped and postponed the treatment for a while; the stools then became watery again. Although diarrhea has persisted, he has not shown any abnormalities in growth or psychomotor development for the first two years of life.