What Is Hereditary Nephrogenic Diabetes Insipidus?

Lithium is the most common cause of acquired nephrogenic diabetes insipidus. It’s a medication often used to treat bipolar disorder. Long-term lithium use can damage the cells of the kidneys so they no longer respond to AVP.

What causes nephrogenic diabetes insipidus?

Nephrogenic diabetes insipidus occurs when there’s a defect in the structures in your kidneys that makes your kidneys unable to properly respond to ADH . The defect may be due to an inherited (genetic) disorder or a chronic kidney disorder.

What type of disorder is nephrogenic diabetes insipidus?

Nephrogenic diabetes insipidus (NDI) is a rare kidney disorder that may be inherited or acquired. NDI is not related to the more common diabetes mellitus (sugar diabetes), in which the body does not produce or properly use insulin.

How do you differentiate neurogenic and nephrogenic diabetes insipidus?

Generally, an increase in urine osmolality of greater than 50% reliably indicates central diabetes insipidus, while an increase of less than 10% indicates nephrogenic diabetes insipidus; responses between 10% and 50% are indeterminate.

What is the difference between central and nephrogenic diabetes insipidus?

Central DI (CDI) is due to a lack of the hormone vasopressin (antidiuretic hormone). This can be due to injury to the hypothalamus or pituitary gland or genetics. Nephrogenic DI (NDI) occurs when the kidneys do not respond properly to vasopressin.

What happens in nephrogenic diabetes insipidus?

In nephrogenic diabetes insipidus, the kidneys produce a large volume of dilute urine because the kidney tubules fail to respond to vasopressin (antidiuretic hormone) and are unable to reabsorb filtered water back into the body.

How is nephrogenic diabetes insipidus detected?

Diagnosis of nephrogenic diabetes insipidus is based on tests of blood and urine. Drinking increased amounts of water helps prevent dehydration. To treat nephrogenic diabetes insipidus, people restrict salt in their diet and sometimes take drugs to reduce the amount of urine excreted.

Is nephrogenic diabetes insipidus reversible?

Disordered water channel expression and distribution in acquired nephrogenic diabetes insipidus. Many of the implicated causes are reversible and are caused by isolated effects on the cortical collecting duct without broad damage to the medullary countercurrent system.

What is ICD 10 code for nephrogenic diabetes insipidus?

Nephrogenic diabetes insipidus

N25. 1 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

How do thiazide diuretics treat nephrogenic diabetes insipidus?

Thiazides have been used in patients with nephrogenic diabetes insipidus (NDI) to decrease urine volume, but the mechanism by which it produces the paradoxic antidiuretic effect remains unclear.

How does hypercalcemia cause nephrogenic diabetes insipidus?

Chronic hypercalcemia can cause nephrogenic DI through multiple mechanisms, including acute interference with vasopressin-stimulated water flow, chronic downregulation of aquaporin-2 channel expression in the cortical collecting duct,21 and direct or indirect (e.g. via prostaglandins) inhibition of sodium chloride …

Is Alport syndrome dominant or recessive?

Alport syndrome has autosomal dominant inheritance in about 5 percent of cases. People with this form of Alport syndrome have one mutation in either the COL4A3 or COL4A4 gene in each cell.

What does nephrogenic mean?

Medical Definition of nephrogenic

1 : originating in the kidney : caused by factors originating in the kidney nephrogenic hypertension. 2 : developing into or producing kidney tissue strands of nephrogenic cells.

What is the management of nephrogenic diabetes insipidus?

Some treatments can reduce the symptoms of nephrogenic diabetes insipidus, at least somewhat: Diet. A low-salt, low-protein diet reduces urine output. Nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen (Motrin), indomethacin (Indocin), and naproxen (Naprosyn) also can reduce urination.

How common is nephrogenic diabetes insipidus?

The prevalence of nephrogenic diabetes insipidus is unknown, although the condition is thought to be rare. The acquired form occurs more frequently than the hereditary form.

Why are his kidneys so active at night?

Look for a specific hormonal problem involving the pituitary. CQ#5: His kidneys are so active at night because‚Ķ He drinks too much because he is unhappy. He doesn’t make enough cortisone, which is a stress response manager.

Which diagnostic finding helps distinguish nephrogenic diabetes insipidus DI from Central Di?

Diagnosis is by water deprivation test showing failure to maximally concentrate urine; vasopressin levels and response to exogenous vasopressin help distinguish central from nephrogenic diabetes insipidus.

What hormone is either not binding with its receptor or not produced at all in patients with diabetes insipidus?

Diabetes insipidus is caused by a lack of antidiuretic hormone (ADH), also called vasopressin, which prevents dehydration, or the kidney’s inability to respond to ADH. ADH enables the kidneys to retain water in the body. The hormone is produced in a region of the brain called the hypothalamus.

How does amiloride treat nephrogenic diabetes insipidus?

Amiloride blocks lithium entry through the sodium channel thereby attenuating the resultant nephrogenic diabetes insipidus.

How is nephrogenic diabetes insipidus Usmle treated?


  1. Management approach. treat underlying cause when appropriate. remove offending agent if possible.
  2. Central DI. first-line. desmopressin (DDAVP) mechanism. ADH analog. other. hydration.
  3. Nephrogenic DI. first-line. hydrochlorothiazide. mechanism. enhances sodium excretion over water excretion. indomethacin. mechanism.

Is diabetes insipidus primary or secondary?

Central diabetes insipidus is caused by reduced or absent levels of vasopressin. The condition can be present from birth, or primary. Secondary central diabetes insipidus is acquired later in life. The cause of primary central diabetes insipidus is often unknown.

What’s the difference between diabetes mellitus and diabetes insipidus?

Diabetes mellitus is more commonly known simply as diabetes. It’s when your pancreas doesn’t produce enough insulin to control the amount of glucose, or sugar, in your blood. Diabetes insipidus is a rare condition that has nothing to do with the pancreas or blood sugar.

What drugs antagonize the effects of ADH on the renal tubules and thus could cause nephrogenic diabetes insipidus?

Antagonizing antidiuretic hormone effect in the kidneys

Demeclocycline is a tetracycline derivative that induces nephrogenic diabetes insipidus by inhibiting cAMP.