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Diabetes insipidus (DI) (Greek διαβαίνειν diabainein - to pass through and Latin insipidus - without taste) is a condition characterized by excretion of large amounts of severely diluted urine, which cannot be reduced when fluid intake is reduced. It denotes inability of the kidney to concentrate urine. DI is caused by a deficiency of antidiuretic hormone (ADH), also known as vasopressin, due to the destruction of the back or "posterior" part of the pituitary gland where vasopressin is normally released from, or by an insensitivity of the kidneys to that hormone. It can also be induced iatrogenically by various drugs.
Signs and symptoms
Excessive urination and extreme thirst (especially for cold water and sometimes ice or ice water) are typical for DI. Symptoms of diabetes insipidus are quite similar to those of untreated diabetes mellitus, with the distinction that the urine is not sweet as it does not contain glucose and there is no hyperglycemia (elevated blood glucose). Blurred vision is a rarity. Signs of dehydration may also appear in some individuals since the body cannot conserve much (if any) of the water it takes in.
The extreme urination continues throughout the day and the night. In children, DI can interfere with appetite, eating, weight gain, and growth as well. They may present with fever, vomiting, or diarrhea. Adults with untreated DI may remain healthy for decades as long as enough water is drunk to offset the urinary losses. However, there is a continuous risk of dehydration.
In order to distinguish DI from other causes of excess urination, blood glucose levels, bicarbonate levels, and calcium levels need to be tested. Measurement of blood electrolytes can reveal a high sodium level (hypernatremia as dehydration develops). Urinalysis demonstrates a dilute urine with a low specific gravity. Urine osmolarity and electrolyte levels are typically low.
A fluid deprivation test helps determine whether DI is caused by:
This test measures changes in body weight, urine output, and urine composition when fluids are withheld and as dehydration occurs. The body's normal response to dehydration is to concentrate urine and conserve water, so urine becomes more concentrated and urination becomes less frequent. Those with DI continue to urinate large amounts of dilute urine in spite of not drinking any fluids. Sometimes measuring blood levels of ADH during this test is also necessary.
To distinguish between the main forms, desmopressin stimulation is also used; desmopressin can be taken by injection, a nasal spray, or a tablet. While taking desmopressin, a patient should drink fluids or water only when thirsty and not at other times, as this can lead to sudden fluid accumulation in central nervous system. If desmopressin reduces urine output and increases osmolarity, the pituitary production of ADH is deficient, and the kidney responds normally. If the DI is due to renal pathology, desmopressin does not change either urine output or osmolarity.
If central DI is suspected, testing of other hormones of the pituitary, as well as magnetic resonance imaging (MRI), is necessary to discover if a disease process (such as a prolactinoma, or histiocytosis, syphilis, tuberculosis or other tumor or granuloma) is affecting pituitary function. Thankfully most people with this form either have experienced past head trauma or simply have stopped ADH production for no apparent reason.
Habit drinking (in its severest form termed psychogenic polydipsia) is the most common imitator of diabetes insipidus at all ages. While many adult cases in the medical literature are associated with mental disorders, most patients with habit polydipsia have no other detectable disease. The distinction is made during the water deprivation test, as some degree of urinary concentration above isosmolar is usually obtained before the patient becomes dehydrated.
Electrolyte and volume homeostasis is a complex mechanism that balances the body's requirements for blood pressure and the main electrolytes sodium and potassium. In general, electrolyte regulation precedes volume regulation. When the volume is severely depleted, however, the body will retain water at the expense of deranging electrolyte levels.
The regulation of urine production occurs in the hypothalamus, which produces antidiuretic hormone (ADH or vasopressin) in the supraoptic and paraventricular nuclei. After synthesis, the hormone is transported in neurosecretory granules down the axon of the hypothalamic neuron to the posterior lobe of the pituitary gland where it is stored for later release. In addition, the hypothalamus regulates the sensation of thirst in the ventromedial nucleus by sensing increases in serum osmolarity and relaying this information to the cortex.
The main effector organ for fluid homeostasis is the kidney. ADH acts by increasing water permeability in the collecting ducts and distal convoluted tubule, specifically it acts on proteins called aquaporins which open to allow water into the collecting duct cells. This increase in permeability allows for reabsorption of water into the bloodstream, thus concentrating the urine.
There are several forms of DI:
Cranial diabetes insipidus
Cranial diabetes insipidus (sometimes central or neurogenic DI) is due to damage to the hypothalamus or pituitary due to a tumor, trauma (such as a fracture to the base of the skull), stroke, neurosurgery or some rather rare causes (which include hemochromatosis, sarcoidosis, histiocytosis, diseases that can form masses in the vicinity like a tuberculoma or syphilis and some genetic disorders). If the hypothalamus is damaged, the feeling of thirst may be completely absent.
Nephrogenic diabetes insipidus is due to the inability of the kidney to respond normally to ADH.
- There are hereditary causes. Inherited forms have been associated with AVPR2 (X-linked) and AQP2 (autosomal).. Approximately 90% are due to mutations of the ADH V2 receptor, and 10% mutations of the aquaporin 2 water channel), but these are rare (incidence is around 4 per million live births). Most are male, because V2 receptor mutations are x-linked recessive defects.
- More common are acquired forms of NDI, which occur as a side-effect to some medications (such as lithium citrate and amphotericin B), as well as in polycystic kidney disease (PKD) and sickle-cell disease, and electrolyte disturbances such as hypokalemia and hypercalcemia. In some cases, no cause is found.
Dipsogenic DI is due to a defect or damage to the thirst mechanism, which is located in the hypothalamus. This defect results in an abnormal increase in thirst and fluid intake that suppresses ADH secretion and increases urine output. Desmopressin is ineffective, and can lead to fluid overload as the thirst remains.
Most cases of gestational DI can be treated with desmopressin. In rare cases, however, an abnormality in the thirst mechanism causes gestational DI, and desmopressin should not be used.
Central DI and gestational DI respond to desmopressin. Carbamazepine, an anti-convulsive medication, has also had some success in this type of DI. Also gestational DI tends to abate on its own 4 to 6 weeks following labour, though some women may develop it again in subsequent pregnancies. In dipsogenic DI, desmopressin is not usually an option.
Desmopressin will be ineffective in nephrogenic DI. Instead, the diuretic hydrochlorothiazide (HCT or HCTZ) or indomethacin can improve nephrogenic diabetes insipidus; HCT is sometimes combined with amiloride to prevent hypokalemia. Again, adequate hydration is important for patients with DI, as they may become dehydrated easily.
- Sweeney AT, Blake MA, Adelman LS, et al (2004). Pituitary apoplexy precipitating diabetes insipidus. Endocr Pract 10 (2): 135–8.
- OMIM 304800
- OMIM 125800
- Christensen S, Kusano E, Yusufi AN, Murayama N, Dousa TP (June 1985). Pathogenesis of nephrogenic diabetes insipidus due to chronic administration of lithium in rats. J. Clin. Invest. 75 (6): 1869–79.
- Marples D, Frøkiaer J, Dørup J, Knepper MA, Nielsen S (April 1996). Hypokalemia-induced downregulation of aquaporin-2 water channel expression in rat kidney medulla and cortex. J. Clin. Invest. 97 (8): 1960–8.
- Carney S, Rayson B, Morgan T (October 1976). A study in vitro of the concentrating defect associated with hypokalaemia and hypercalcaemia. Pflugers Arch. 366 (1): 11–7.
- Perkins RM, Yuan CM, Welch PG (March 2006). Dipsogenic diabetes insipidus: report of a novel treatment strategy and literature review. Clin. Exp. Nephrol. 10 (1): 63–7.
- Kalelioglu I, Kubat Uzum A, Yildirim A, Ozkan T, Gungor F, Has R (2007). Transient gestational diabetes insipidus diagnosed in successive pregnancies: review of pathophysiology, diagnosis, treatment, and management of delivery. Pituitary 10 (1): 87–93.
Endocrine pathology of psychological interest (E00-35)
thyroid Hypothyroidism (Iodine deficiency, Cretinism, Congenital hypothyroidism, Goitre) - Hyperthyroidism (Graves-Basedow disease, Toxic multinodular goitre) - Thyroiditis (De Quervain's thyroiditis, Hashimoto's thyroiditis)
Urinary system - Pathology - Nephrology (N00-N39, 580-599)
|Diseases of the glomerulus||
Glomerulonephritis - Focal segmental glomerulosclerosis - Membranoproliferative glomerulonephritis - Membranous glomerulonephritis - Nephritic syndrome - Post-streptococcal glomerulonephritis - Nephrotic syndrome (Minimal change disease) - IgA nephropathy - Lupus nephritis - Diabetic nephropathy - Rapidly progressive glomerulonephritis
|Tubulointerstitial diseases of the kidney||
Interstitial nephritis - Pyelonephritis - Hydronephrosis - Pyonephrosis - Balkan nephropathy - Reflux nephropathy
Acute renal failure (Acute tubular necrosis) - Chronic renal failure
|Diseases of the renal tubule and
other disorders of kidney and ureter
Renal osteodystrophy - Nephrogenic diabetes insipidus - Renal tubular acidosis - Nephroptosis - Ureterocele
|Other diseases and
disorders of urinary system
|Tumors of the kidney||
Renal cell carcinoma - Wilms' tumor (children)
See also congenital conditions (Q60-Q64, 753)
Sex linkage: X-linked recessive disorders
Chronic granulomatous disease (CYBB) · Wiskott-Aldrich syndrome · X-linked Severe Combined Immunodeficiency · X-linked agammaglobulinemia · Hyper-IgM syndrome type 1 · IPEX
Haemophilia A · Haemophilia B · X-linked sideroblastic anemia · X-linked lymphoproliferative disease
Androgen insensitivity syndrome/Kennedy disease · Diabetes insipidus
amino acid: Ornithine transcarbamylase deficiency · Oculocerebrorenal syndrome
lipid storage disorder: Fabry's disease
mucopolysaccharidosis: Hunter syndrome
purine-pyrimidine metabolism: Lesch-Nyhan syndrome
eye disorders: Color blindness (red and green, but not blue) · Ocular albinism (1) · Norrie disease · Choroideremia
Dyskeratosis congenita · Hypohidrotic ectodermal dysplasia (EDA) · X-linked ichthyosis
Alport syndrome · Dent's disease
|No primary system||
Barth syndrome · McLeod syndrome · Simpson-Golabi-Behmel syndrome
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