central diabetes insipidus; diabetes insipidus, neurohypophyseal type (CDI)
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Introduction
A deficiency of antidiuretic hormone (ADH) or vasopressin.
Etiology
- hypoxic or ischemic encephalopathy
- head trauma
- post hypophysectomy/pituitary ablation
- idiopathic suprasellar or intrasellar tumors or cysts
- anorexia nervosa
- hypothalamic/pituitary infiltration
- CNS infection
- encroachment by aneurysm
- Sheehan's syndrome
- Guillain-Barre syndrome
- fat embolus
- empty sella
- following correction of supraventricular tachycardia[2]
- familial disorder
- autoimmune hypophysitis
- idiopathic
Pathology
- complete or partial deficiency of antidiuretic hormone (ADH) or vasopressin secretion by the posterior pituitary (neurohypophysis)
- disruption of preprovasopressin processing
Genetics
- cases of primary central diabetes insipidus are associated with mutations in the gene for preprovasopressin & arginine vasopressin
- autosomal dominant neurohypophyseal diabetes insipidus
- autosomal recessive neurohypophyseal diabetes insipidus
Clinical manifestations
- persistent thirst, polydipsia & polyuria
Laboratory
- severe urine concentrating deficit
- urine osmolality < 100 mosm/kg
- 24 hour urine - volumes > 3 L/day
- urine concentrating test
- response of urine osmolality to water deprivation
- close monitoring indicated
- urine osmolality normally increases with water deprivation
- urine osmolality does not increase after water deprivation with central DI
- serum osmolality is elevated
- desmopressin stimulation test
- when urine does NOT concentrate with water deprivation
- an increased urine osmolality indicates central DI
- a lack of response indicates nephrogenic DI
- patients with primary polydipsia may show response similar to nephrogenic DI due to washout of the medullary concentration gradient
- serum sodium:
- free water diuresis leads to high normal serum sodium if fluid intake adequate but hypernatremia if fluid intake is compromised [6]
- ADH in plasma is low[2]
- plasma copeptin distinguishes central diabetes insipidus from polydipsia[2]
- antigen & activity low in central diabetes insipidus
*also see ARUP consult
Differential diagnosis
Management
- general (see diabetes insipidus)
- desamino-D-arginine vasopressin dDAVP 5-10 ug QD or BID intranasally
- use cautiously in patients with coronary artery disease
- avoid water retention & hyponatremia
- administer QHS to prevent nocturia
- adjust dose to permit daily breakthrough polyuria thus avoiding iatrogenic SIADH
- chlorpropamide potentiates effects of dDAVP
- avoid hypoglycemia
More general terms
Additional terms
- syndrome of inappropriate antidiuretic hormone; SIADH; nephrogenic syndrome of inappropriate antidiuresis; NSIAD
- urine osmolality
- vasopressin (antidiuretic hormone, ADH)
References
- ↑ Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 50-51
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2012, 2015, 2018, 2022.
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 239
- ↑ Loh JA, Verbalis JG. Disorders of water and salt metabolism associated with pituitary disease. Endocrinol Metab Clin North Am. 2008 Mar;37(1):213-34 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18226738
- ↑ ARUP Consult:Central Diabetes Insipidus (Posterior Pituitary) Testing Algorithm https://arupconsult.com/algorithm/central-diabetes-insipidus-posterior-pituitary-testing-algorithm
- ↑ 6.0 6.1 NEJM Knowledge+ Endocrinology
Patient information
central diabetes insipidus patient information