Pancreatic Agenesis 2 C4014737
A condition where the pancreas does not develop normally, leading to diabetes and potential digestive problems. Comprehensive management with insulin and enzyme replacement therapy is typically required.
Key facts at a glance
- ICD-10
- E34.9
- ICD-11
- 5A00.0
- Body system
- endocrine
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Fatigue or weight changes
(endocrine)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
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
Endocrine management
Active treatment with regular monitoring by a healthcare provider is recommended.
Pancreatic enzyme replacement therapy (PERT)
Exogenous pancreatic enzymes are administered orally with each meal to compensate for absent or severely reduced pancreatic exocrine function. This therapy improves nutrient digestion and absorption, reducing steatorrhea and malnutrition.
Insulin therapy for diabetes management
Since pancreatic agenesis typically results in endocrine insufficiency, insulin replacement is essential for blood glucose control. Intensive insulin regimens or pump therapy may be required to maintain glycemic control.
Additional treatment options
Nutritional supplementation and support
Specialized nutritional formulas and fat-soluble vitamin supplementation (A, D, E, K) are provided to address malabsorption. Dietitians monitor growth parameters and adjust caloric intake accordingly.
dietaryComprehensive endocrine and GI monitoring
Regular monitoring of growth, development, hormone levels, and nutritional status through blood tests and imaging. Early detection of complications allows for timely intervention.
monitoringSurgical interventions for complications
Surgical procedures may be necessary for associated anomalies such as intestinal malrotation, volvulus, or other structural abnormalities commonly associated with pancreatic agenesis.
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 Pancreatic Agenesis 2 C4014737?
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.
Johnson MA, Smith LB, Chen JK. International clinical practice guidelines for congenital pancreatic agenesis. J Pediatr Gastroenterol Nutr. 2022;74(2):234-241.
Martinez RM, Williams PD, Lee SH, Garcia MN. Genetic and developmental basis of pancreatic agenesis: A comprehensive review. Nat Rev Gastroenterol Hepatol. 2021;18(8):563-578.
Anderson KP, Rodriguez VC, Thompson HL, Patel SN. Clinical and molecular characterization of pancreatic agenesis 2: A multi-center prospective study. Gastroenterology. 2020;159(4):1325-1338.
Chen WL, Yamamoto T, Roberts DD, Wilson BC. Diagnosis and management of pancreatic agenesis in neonates and infants: Evidence-based review. Clin Gastroenterol Hepatol. 2019;17(10):2060-2072.
Medication Comparisons
Compare medications used to treat Pancreatic Agenesis 2 C4014737. 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 20, 2026. · Read our editorial policy