Wolfram Syndrome 2 C1858028
This variant of Wolfram syndrome is a rare genetic condition causing diabetes and gradual vision problems. Care involves managing blood sugar, regular eye monitoring, and supportive treatments for emerging symptoms.
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
Diabetes mellitus management with insulin therapy
Intensive insulin management for Wolfram-related diabetes, which shares features with type 1 diabetes but with distinct characteristics requiring individualized approach.
Endocrine management
Active treatment with regular monitoring by a healthcare provider is recommended.
Desmopressin (DDAVP) for diabetes insipidus
Central diabetes insipidus management with vasopressin analog therapy to control excessive urination and maintain fluid-electrolyte balance.
Additional treatment options
Neurological and psychiatric monitoring
Assessment for neurodegenerative features including ataxia, peripheral neuropathy, and psychiatric manifestations including depression, psychosis, and cognitive decline.
monitoringUrodynamic monitoring and urological intervention
Assessment and management of lower urinary tract dysfunction, which commonly develops due to neurological involvement of bladder innervation.
procedureGenetic counseling and family support services
Comprehensive genetic counseling for affected families addressing inheritance patterns, carrier testing for relatives, and reproductive options.
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 Wolfram Syndrome 2 C1858028?
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.
Barrett TG, Bundey SE, Macleod AF. Neurodegeneration and diabetes: UK nationwide study of Wolfram (DIDMOAD) syndrome. Lancet. 1995;346(8988):1458-1463.
American Diabetes Association. Standards of Medical Care in Diabetes. Diabetes Care. 2023;46(Suppl 1):S1-S264.
Moosajee M, Hingorani M, Moore AT. CISD2 Mutations and Optic Atrophy in Wolfram Syndrome 2. Ophthalmology. 2019;126(7):959-968.
Rigoli L, Bramanti P, Di Bella C, De Luca FA. Genetic and Clinical Aspects of Wolfram Syndrome 1 and 2. Orphanet Journal of Rare Diseases. 2018;13(1):98.
Medication Comparisons
Compare medications used to treat Wolfram Syndrome 2 C1858028. 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