Diazoxide Resistant Focal Hyperinsulinism Due To Kir6 2 Deficiency C5191060
A severe genetic condition causing dangerously low blood sugar due to excess insulin production. Often requires surgical treatment when medications like diazoxide are ineffective.
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.
Focal lesion resection
Surgical removal of the focal hyperinsulinemic lesion identified by 18F-DOPA PET imaging offers the possibility of cure. Careful intraoperative monitoring ensures complete excision while preserving normal pancreatic tissue.
Continuous glucose monitoring
Real-time glucose monitoring devices track glucose trends and detect asymptomatic hypoglycemia. Data guides treatment adjustments and reduces hypoglycemic events between meals.
Additional treatment options
Somatostatin analog therapy
Octreotide or pasireotide suppress insulin secretion through somatostatin receptor activation. Subcutaneous or continuous infusion provides alternative glycemic control when surgical cure is not achievable.
medicationFrequent feeding regimen
Scheduled small frequent feedings with complex carbohydrates maintain stable glucose levels. Enteral feeds via nasogastric or gastrostomy tube may be necessary for reliable overnight glucose delivery.
dietaryPancreatectomy
Partial or near-total pancreatectomy reduces insulin-secreting tissue mass when focal surgery is not feasible or has failed. Reserved for severe cases given significant diabetes risk.
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 Diazoxide Resistant Focal Hyperinsulinism Due To Kir6 2 Deficiency C5191060?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Diazoxide Resistant Focal Hyperinsulinism Due To Kir6 2 Deficiency C5191060. See side-by-side comparisons of effectiveness, side effects, costs, and more.
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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