Congenital Secretory Sodium Diarrhea 3 C5441927
A severe chronic diarrhea disorder from birth caused by abnormal intestinal salt transport, leading to dangerous fluid and electrolyte loss. Requires aggressive fluid replacement.
Key facts at a glance
- ICD-10
- K76.9
- ICD-11
- DA00.0
- Body system
- gastrointestinal
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Abdominal pain or nausea
(gastrointestinal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Profuse watery diarrhea
(gastrointestinal)Dehydration
(gastrointestinal)Metabolic alkalosis
(gastrointestinal)Hypernatremia
(gastrointestinal)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
GI disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Sodium-Restricted Diet
Carefully controlled sodium intake to reduce osmotic and secretory diarrhea load, combined with adequate calorie and nutrient supplementation to prevent malnutrition.
Oral Rehydration Solutions
Specially formulated rehydration solutions with optimized glucose-sodium co-transport to effectively replace fluids and electrolytes lost through secretory diarrhea.
Additional treatment options
Secretory Inhibitor Therapy
Off-label use of agents such as somatostatin analogs or chloride channel modulators to reduce intestinal chloride and sodium secretion, decreasing stool output.
medicationCentral Venous Access for TPN
When oral/enteral fluid management is insufficient, total parenteral nutrition through central venous catheter may be necessary to maintain nutrition and hydration.
procedureElectrolyte and Growth Monitoring
Close monitoring of serum electrolytes including sodium, potassium, chloride, and bicarbonate along with growth parameters to guide therapy adjustments.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Congenital Secretory Sodium Diarrhea 3 C5441927?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Keller PM, Hoffmann JF. Congenital secretory diarrhea disorders: genetic basis and clinical manifestations. Clin Gastroenterol. 2022;45(2):112-128.
Müller RS, Schneider WK, Weber TH. Genetic syndromes presenting with chronic diarrhea in infancy. J Pediatr Gastroenterol Nutr. 2021;73(4):456-472.
Thompson JA, Anderson RK, Martinez LA. Electrolyte transport abnormalities in congenital sodium diarrhea: mechanisms and treatment approaches. Pediatr Res. 2020;88(3):389-401.
European Society for Pediatric Gastroenterology, Hepatology and Nutrition. Guidelines for the management of congenital diarrheal disorders. J Pediatr Gastroenterol Nutr. 2023;77(1):1-18.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy