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Nephrogenic diabetes insipidus

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Escalate

Hypernatraemia with confusion, seizure, coma, marked volume depletion or inability to access water is a medical emergency. Restore circulation first if shocked, then replace free water through a monitored plan that accounts for chronicity and ongoing urine loss; overly rapid sodium correction can cause neurological harm. Suspected lithium toxicity, severe hypokalaemia or hypercalcaemia, and an infant with fever, weight loss or dehydration also require urgent specialist care.

Synopsis

Distinguish renal vasopressin resistance from other polyuria, protect against hypernatraemic dehydration, and manage acquired or inherited disease with specialist safeguards.

  • Nephrogenic diabetes insipidus is renal resistance to arginine vasopressin, now also termed AVP resistance, causing hypotonic polyuria despite an intact thirst and hormone response.
  • Confirm true polyuria by measured volume rather than urinary frequency; in adults a 24-hour output above about 3 litres is a useful threshold but size and intake matter.
  • Exclude osmotic diuresis from glucose, urea, mannitol or diuretics and primary polydipsia before undertaking specialised water-balance testing.

Key red flags

Hypernatraemic dehydration

Dry mucosae, hypotension, tachycardia, lethargy, irritability, confusion or seizure after reduced water access signals dangerous uncompensated loss.

Investigation priorities

01
Measured 24-hour urineFirst step

Confirm polyuria and quantify ongoing water loss.

Management branches

VerifyProve hypotonic polyuria

A patient reports frequent urination and excessive thirst.

  1. Distinguish urinary frequency from high volume using a bladder history, fluid chart or complete 24-hour urine measurement.
  2. Pair urine osmolality with plasma sodium or osmolality and screen glucose, calcium, potassium, renal function and diuretic or SGLT2 use.

Key medicines

Thiazide-type diureticSelect a locally available agent and low starting dose only under renal or endocrine advice, then titrate against measured urine volume, blood pressure and electrolytes.
AmilorideUse specialist-selected dosing from the current formulary, particularly in lithium-associated disease, with early and repeated potassium, creatinine and lithium checks.
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Sources and review status6 sources · checked 27 Aug 2026 · clinical review pending
Sources

Sources and review status

National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom