Diabetes Insipidus Neurohypophyseal X Linked C5562113
A hormone deficiency causing excessive thirst and urination, primarily affecting males. Managed with hormone replacement and dietary adjustments.
Key facts at a glance
- ICD-10
- E34.9
- ICD-11
- 5A00.0
- Body system
- endocrine
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Fatigue or weight changes
(endocrine)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Polydipsia
(endocrine)Polyuria
(renal)Nocturia
(renal)Orthostatic hypotension
(cardiovascular)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
Desmopressin replacement therapy
Synthetic analogue of vasopressin administered intranasally, orally, or subcutaneously to replace deficient antidiuretic hormone. Dosing is individualized based on patient response and fluid balance.
Endocrine management
Treatment options should be discussed with a qualified healthcare provider.
Fluid balance monitoring
Regular monitoring of serum sodium, osmolality, and body weight to ensure adequate medication dosing and prevent both under-treatment and water intoxication.
Additional treatment options
Fluid intake management
Patient education on appropriate fluid intake relative to medication timing and activity level. Patients must balance hydration needs with medication effects.
lifestyleSodium and protein management
Moderate, consistent sodium intake to support stable fluid balance. Diet should be nutritionally adequate without excessive protein.
dietaryWater deprivation testing
Diagnostic testing to confirm diagnosis and assess kidney concentrating ability. Should only be performed in controlled settings with close medical supervision.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Diabetes Insipidus Neurohypophyseal X Linked C5562113?
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.
Bockenhauer D, Deen PM, Bichet DG. Clinical practice guideline for hereditary diabetes insipidus. Nephrology Dialysis Transplantation. 2022.
Arima H, Murase K, Oiso Y. Neurohypophyseal diabetes insipidus: clinical and genetic aspects. Best Practice and Research Clinical Endocrinology and Metabolism. 2020.
Robinson AG, Verbalis JG, Laczi CV. Desmopressin acetate treatment for central diabetes insipidus: efficacy and safety outcomes. Journal of Clinical Endocrinology and Metabolism. 2019.
Frainay C, Schmitz G, Boulund F. Treatment approaches for vasopressin deficiency: systematic review and meta-analysis. Endocrine-Related Cancer. 2021.
Medication Comparisons
Compare medications used to treat Diabetes Insipidus Neurohypophyseal X Linked C5562113. 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy