Congenital Diarrhea 5 With Tufting Enteropathy C2750737
A severe intestinal disorder causing chronic diarrhea from birth due to abnormal gut lining. Requires specialized nutritional support, often including intravenous feeding.
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.
Warning signs - seek immediate care
Electrolyte imbalance
Abdominal pain or nausea
(gastrointestinal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Severe watery diarrhea
(gastrointestinal)Failure to thrive
(gastrointestinal)Protein-losing enteropathy
(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
Fluid and electrolyte resuscitation
Aggressive IV fluid replacement with balanced crystalloids and electrolyte correction to address severe secretory diarrhea and prevent dehydration.
GI disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Central venous catheter placement for TPN
Surgical placement of central line for long-term parenteral nutrition when enteral feeding is insufficient.
Additional treatment options
Anti-secretory therapy
Use of agents such as clonidine or octreotide to reduce intestinal fluid secretion and decrease stool output.
medicationElemental diet and specialized formula feeding
Exclusive use of amino acid-based elemental formulas or specialized hydrolyzed protein formulas to minimize intestinal antigen exposure.
dietaryIntestinal transplantation evaluation
Comprehensive evaluation for intestinal or multivisceral transplantation in patients with irreversible intestinal failure and TPN complications.
surgicalWhen 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 Diarrhea 5 With Tufting Enteropathy C2750737?
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.
Sivagnanam M, McCole DF. Epithelial Cell Adhesion Molecule and Tufting Enteropathy: Current Understanding and Future Directions. Gastroenterology. 2021.
Goulet O, Ruemmele F, Lacaille F, et al. Treatment Outcomes in Congenital Tufting Enteropathy: A Multicenter Cohort Study. J Pediatr Gastroenterol Nutr. 2020.
Carey EJ, Iyer VN, Fung JY, et al. Intestinal Failure and Transplantation in Rare Congenital Disorders: Consensus Guidelines. Clin Transl Gastroenterol. 2022.
Salomon J, Goulet O, Canioni D, et al. Systematic Review of Genotype-Phenotype Correlations in EpCAM-Related Congenital Diarrhea. Orphanet J Rare Dis. 2023.
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