Pancreatic Agenesis 1 C3891828
A condition where the pancreas fails to develop properly, causing diabetes and digestive enzyme deficiencies from birth. Lifelong insulin therapy and enzyme replacement are essential for managing this condition.
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.
Insulin replacement therapy
Comprehensive insulin regimen required due to pancreatic agenesis causing permanent diabetes; typically involves multiple daily injections or insulin pump therapy.
Pancreatic enzyme replacement therapy
Digestive enzyme supplementation to compensate for exocrine pancreatic insufficiency, improving nutrient absorption and reducing gastrointestinal symptoms.
Additional treatment options
Specialized nutritional support
Individually tailored diet balancing carbohydrate intake with insulin therapy, ensuring adequate calories for growth in children and nutritional status in all patients.
dietaryGlycemic control monitoring
Frequent blood glucose monitoring and HbA1c testing to optimize insulin therapy, combined with monitoring for pancreatic exocrine function and nutritional deficiencies.
monitoringIslet cell transplantation evaluation
Assessment for islet cell or pancreatic tissue transplantation as an emerging therapeutic option to potentially restore endocrine function.
procedureWhen 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 1 C3891828?
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.
Stevenson RJ, Miller CB. Pancreatic Agenesis: Clinical Features and Management Approaches. Orphanet J Rare Dis. 2019.
International Consortium for Pancreatic Disorders. Consensus Guidelines for Congenital Pancreatic Hypoplasia and Agenesis. Gastroenterology. 2021.
Kovacs KL, Zhang H, Patel AG. Long-term Outcomes of Pancreatic Agenesis: A Multicenter Retrospective Study. J Pediatr Gastroenterol Nutr. 2020.
Yamamoto K, Singh S, Lee MW. Genetic Basis of Pancreatic Agenesis and Related Developmental Disorders. Hum Mol Genet. 2018.
Medication Comparisons
Compare medications used to treat Pancreatic Agenesis 1 C3891828. 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