GB90.4A

ICD-11 MMS 2026-01

Nephrogenic diabetes insipidus

Description

Nephrogenic diabetes insipidus is a condition in which the kidney tubules respond poorly to pituitary secreted anti-diuretic hormone, resulting in a failure to concentrate the urine, and water loss. Polyuria with dilute urine and polydypsia (excessive thirst) are present. It can be congenital or acquired with many causes. The congenital forms may be attributed to vasopressin receptor or aquaporin-2 defects. They are characterised by polyuria with polydipsia, recurrent bouts of fever, constipation, and acute hypernatraemic dehydration after birth that may cause neurological sequelae.

Index terms

  • Nephrogenic diabetes insipidus
  • ADH - [antidiuretic-hormone] resistant diabetes insipidus
  • NDI - [nephrogenic diabetes insipidus]
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  • adiuretin-resistant diabetes insipidus
  • antidiuretic-hormone-resistant diabetes insipidus
  • congenital nephrogenic diabetes insipidus
  • diabetes tenuifluus
  • familial nephrogenic diabetes
  • familial nephrogenic diabetes insipidus
  • hereditary nephrogenic diabetes insipidus
  • primary nephrogenic diabetes insipidus
  • renal diabetes insipidus
  • Nephrogenic diabetes insipidus - intracranial calcification
  • Nephrogenic Diabetes Insipidus associated with familial or genetic disorders elsewhere classified
  • AQP2 mutation
  • X-linked AVPR2 mutation
  • Acquired nephrogenic diabetes insipidus due to causes classified elsewhere
  • Vasopressin-resistant hyposthenuria
  • vasopressin-resistant diabetes insipidus
  • Vasopressin-unresponsiveness hyposthenuria

Excludes

  • 5A61.5 – Central diabetes insipidus

Exclusions from above levels

  • http://id.who.int/icd/entity/1746821938 – Urolithiasis
  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
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  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • GB90.40 – Hypotonia-cystinuria type 1
  • GB90.41 – Pseudohypoaldosteronism type 1
  • GB90.42 – Fanconi syndrome
  • GB90.43 – Bartter syndrome
  • GB90.44 – Renal tubular acidosis
  • GB90.45 – Renal glycosuria
  • GB90.46 – Tubular disorders of sodium or potassium transport
  • GB90.47 – Aminoaciduria
  • GB90.48 – Disorders of calcium or phosphate excretion
  • GB90.49 – Renal hypocalciuria
  • GB90.4Y – Other specified renal tubular function disorders
  • GB90.4Z – Renal tubular function disorders, unspecified

Foundation entity

Nephrogenic diabetes insipidus – http://id.who.int/icd/entity/1417669099

Part of: GB90.4: Renal tubular function disorders › GB90: Certain specified disorders of kidney or ureter › 16: Diseases of the genitourinary system