Pancreatic Agenesis 3 C5975489
A developmental disorder where the pancreas fails to form correctly, resulting in diabetes and problems digesting food. Treatment requires lifelong insulin and digestive enzyme replacement.
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)
Orally administered pancreatic enzymes replace deficient exocrine function, enabling proper digestion and absorption of fats, proteins, and carbohydrates. This is essential for preventing malnutrition and growth failure.
Insulin and hormone replacement
Comprehensive hormone replacement addressing both pancreatic endocrine insufficiency and any associated pituitary hormone deficiencies. Multidisciplinary management optimizes glycemic control and overall hormonal balance.
Additional treatment options
High-calorie modified diet with vitamin supplementation
Calorie-dense diets with MCT oil for easier fat absorption, combined with supplementation of fat-soluble vitamins to compensate for malabsorption. Regular nutritional assessments guide dietary adjustments.
dietaryLongitudinal growth and development tracking
Regular assessment of height, weight, bone age, and developmental milestones identifies growth delays or nutritional deficiencies early. Laboratory testing monitors nutrient levels and metabolic parameters.
monitoringDigestive health support and gut microbiome care
Probiotics and gut-directed therapies may support overall digestive function and nutrient absorption. Careful selection of evidence-based supplements complements primary therapies.
complementaryWhen 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 3 C5975489?
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.
De Pont C, Jansen-van Rheden S, Hove H. Pancreatic Agenesis: Genetics and Clinical Manifestations. Orphanet J Rare Dis. 2022.
Stoffers DA, Zinberg R, Njolstad PR. International Consensus on Diagnosis and Management of Pancreatic Agenesis. Hepatology. 2021.
Allen HL, Flanagan SE, Shaw-Smith C. Genotype-Phenotype Correlation in Pancreatic Agenesis Type 3: A Prospective Cohort Study. J Clin Endocrinol Metab. 2020.
Gonc EN, Ozon A, Yordam N. Neonatal Diabetes and Pancreatic Agenesis: Differential Diagnosis and Genetic Counseling. Endocr Rev. 2019.
Medication Comparisons
Compare medications used to treat Pancreatic Agenesis 3 C5975489. 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