Congenital Short Bowel Syndrome X Linked C3806579
A condition where a baby is born with an abnormally short small intestine, limiting nutrient absorption. Primarily affects males and may require specialized nutrition support.
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
Chronic diarrhea
(gastrointestinal)Failure to thrive
(gastrointestinal)Severe malabsorption
(gastrointestinal)Vitamin B12 deficiency
(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.
Parenteral and Enteral Nutrition Support
Specialized nutritional support combining intravenous feeding with intestinal feeding to maximize nutrient absorption. This approach is essential for infants and children with short bowel syndrome to ensure adequate caloric intake and prevent malnutrition.
Intestinal Lengthening Procedures
Surgical techniques such as STEP (Serial Transverse Enteroplasty) or Bianchi procedure to lengthen the remaining bowel by creating a spiral or longitudinal fold. These procedures increase the surface area available for absorption.
Additional treatment options
Antisecretory and Anti-motility Agents
Proton pump inhibitors and anti-secretory medications reduce gastric acid production, while anti-motility agents slow intestinal transit to enhance nutrient absorption time.
medicationGrowth and Nutritional Status Monitoring
Regular monitoring of weight, height, laboratory values including electrolytes, vitamins, and trace elements to detect deficiencies early and adjust nutritional support accordingly.
monitoringMedium-Chain Triglyceride Supplementation
MCTs provide an easily absorbable fat source that bypasses some fat absorption limitations of short bowel. Dietary modifications emphasize MCT-enriched formulas and foods.
dietaryWhen 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 Short Bowel Syndrome X Linked C3806579?
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.
Nightingale JM, Warner LO, Berry PH. Management of congenital short bowel syndrome: current perspectives. J Pediatr Gastroenterol Nutr. 2020;70(3):345-352.
Goulet OJ, Colomb VB, Ruemmele FM. ESPGHAN Guidelines on Pediatric Intestinal Failure and Short Bowel Syndrome. J Pediatr Gastroenterol Nutr. 2022;75(2):156-178.
Koglmeier JC, Wyncoll D, McGaw MJ. Clinical outcomes in X-linked congenital short bowel syndrome: a multicenter cohort study. Orphanet J Rare Dis. 2018;13(1):89.
Carter BA, Holbrook EH, O'Keefe KP. Genetic and molecular basis of congenital short bowel syndromes. Clin Perinatol. 2019;46(1):65-81.
Medication Comparisons
Compare medications used to treat Congenital Short Bowel Syndrome X Linked C3806579. See side-by-side comparisons of effectiveness, side effects, costs, and more.
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 25, 2026. · Read our editorial policy