Nephrogenic Diabetes Insipidus

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Have you been lately suffering from excessive urination along with severe thirst? You are probably suffering from Nephrogenic Diabetes Insipidus (NDI), a major diabetic disorder that you should not neglect. Get detailed information about this syndrome including its causes, symptoms, diagnosis and treatment.

Nephrogenic Diabetes Insipidus Definition

It is a common kidney disorder characterized by disturbed water-balance in the body. In this type of ailment, a small defect in the tubule of the kidney causes a person to release large amounts of urine. Such small tubes remove water in large quantities which dehydrates the body and causes excessive thirst, especially in hot weather. This disorder is related to a hormone, known as ADH- Antidiuretic Hormone. Though Diabetes Insipidus Nephrogenic is a type of diabetes mellitus, both the disorders have separate reasons for their cause.

Nephrogenic Diabetes Insipidus ICD9 Code

The ICD9 code for this condition is 588.1.

Nephrogenic Diabetes Insipidus Incidence

NDI is an uncommon ailment. Compared to its hereditary form, Acquired Nephrogenic Diabetes Insipidus occurs more frequently among the patients. Approximately it is found to arise in 1 in every 25000 inhabitants of the US.

Nephrogenic Diabetes Insipidus Types

This progressive disorder can be categorized into two major forms:

Picture of Nephrogenic Diabetes Insipidus

Picture – Nephrogenic Diabetes Insipidus

  • Congenital NDI
  • Acquired NDI

Nephrogenic Diabetes Insipidus Causes

NDI occurs when the small tubes of the kidney fail to respond to a chemical compound in the body, known as ADH (Antidiuretic Hormone, also known as Vasopressin). The main function of this hormone is to send special signals to the kidney. In people with NDI, the kidneys discharge large amounts of water into the urine making it more diluted.

The cause for the development of this disorder can depends on the type of the condition that a person suffers from.

Acquired NDI

The main causes for its occurrence are:

  • Hypokalaemia
  • Idiopathic
  • Hypercalcaemia
  • Post-obstructive uropathy
  • Renal tubular acidosis
  • Drugs (such as Foscarnet, Clozapine, Antifungals, Antineoplastic agents, Lithium2, 3 and Antiviral medications)

Congenital NDI

It is triggered by:

  • Irregular NDI, along with intracerebral calcification and mental retardation.
  • X-linked mutation in the V2 ADH-receptor gene.

Nephrogenic Diabetes Insipidus Symptoms

Patients suffering from NDI have an uncontrollable thirst for water, particularly ice water. The urine production in such patients is around 3-15 liters per day.

A few patients try to avoid drinking large amounts of water to evade excessive urination. Such individuals suffer from severe dehydration due to lack of enough fluids in their body. The symptoms that indicate the presence of NDI include:

  • Sunken eyes
  • Dry mucous membranes
  • Sunken fontanelles in newborns
  • Dry skin

There are a number of other symptoms that can occur due to the absence of adequate fluids in the body. These problems, which serve as warning signs for patients and indicate the presence of NDI to physicians, include:

  • Irritability
  • Fatigue
  • Weight Loss
  • Muscle pains
  • Headache
  • Fast heart rate
  • Low body temperature

Nephrogenic Diabetes Insipidus Diagnosis

A proper diagnosis of this syndrome is essential for doctors to determine the intensity of the condition and provide patients with appropriate treatment for it. The necessary diagnostic tests for NDI include:

  • Urine specific gravity
  • Urine 24 hour volume
  • Urine concentration test
  • Serum Sodium

The above-mentioned tests help display a number of symptoms characteristic of the disorder, such as:

  • ADH level
  • High urine flow, irrespective of the amount of fluid consumed.
  • Low urine osmolality
  • High serum osmolality

Nephrogenic Diabetes Insipidus Treatment

The main objective of treating NDI patients is to monitor the level of fluids in their body. This disease can be successfully treated by drinking large volumes of water. The fluid intake should be equal to the total amount of urine produced in the body. Intake of water should be accompanied by a healthy low sodium diet.

In a few cases, this disease may arise due to certain medicines and drugs. Such medications should immediately be stopped after consulting a doctor, to avoid further complications arising out of this disorder.

Medicines, such as Hydrochlorothiazide, may help reduce the occurrence of NDI symptoms and prevent any damage to the urinary tract due to dilatation. This is done by minimizing the production of urine by up to 50% through hydrochlorothiazide drug therapy. Non-steroidal anti-inflammatory drugs (NSAIDs), such as Indomethicin, can also be used to enhance the concentration of urine in patients.

Dehydration, caused by NDI, can be cured by drinking lots of water. During extreme dehydration, intravenous fluids are administered to affected individuals to stabilize their condition.

Nephrogenic Diabetes Insipidus Prognosis

If enough fluids are consumed and the electrolyte balance is maintained, NDI does not have any severe impact on the water balance of the body. Not drinking enough fluid can make a patient suffer from multiple physical complications.

Nephrogenic Diabetes Insipidus Complications

The numerous complications arising in an NDI affected patient, due to improper treatment or absence of cure, include:

  • Dilation of the ureters and bladder
  • Severe dehydration
  • High blood sodium (hypernatremia)
  • Shock

Nephrogenic Diabetes Insipidus Risk Factors

The presence of a number of other factors can increase the risk of having NDI. These include:

Heredity

A genetic defect can make a permanent change in the ability of the kidney to make the urine concentrated.

Being male

The disorder is found to afflict males more than females.

Nephrogenic Diabetes Insipidus Prevention

There is no possible option to prevent the occurrence of Congenital NDI. In a few cases, however, the acquired form of the disorder can be prevented from worsening with medicines under the guidance of a health care provider.

NDI is a rare but a complicated diabetic disorder. Unless managed in time, the condition can destabilize the balance of water in the body and give rise to complications. If you or any of your family members experience excessive thirst or any other symptoms of this disorder, consult a professional medical care provider immediately.

References:

http://en.wikipedia.org/wiki/Nephrogenic_diabetes_insipidus

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001540/

http://www.patient.co.uk/doctor/Nephrogenic-Diabetes-Insipidus.htm

http://ghr.nlm.nih.gov/condition/nephrogenic-diabetes-insipidus

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