Diabetes insipidus (DI) is a condition characterized by excretion of large amounts of severely diluted urine, which cannot be reduced when fluid intake is reduced. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings E00-E35 - Endocrine diseases (E00-E07 Thyroid gland / Thyroid hormone ( Congenital iodine-deficiency syndrome ( N00-N39 - Diseases of the Genitourinary system: urinary system (N00-N08 Glomerular diseases Prefixes. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. The Diseases Database is a free Website that provides information about the relationships between medical conditions Symptoms, and Medications. MedlinePlus, with the MedlinePlus Medical Encyclopedia, is a website network containing Health information from the world's largest medical Library eMedicine is an online clinical medical knowledge base that was founded in 1996 by Scott Plantz and Richard Lavely two medical doctors Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books A disease is an abnormal condition of an organism that impairs bodily functions and can be deadly Urine is a liquid waste product of the body secreted by the Kidneys by a process of filtration from Blood and Excreted through the Urethra. 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. Arginine vasopressin ( AVP) also known as vasopressin, argipressin or antidiuretic hormone ( ADH) is a Hormone found in The pituitary gland, or hypophysis, is an Endocrine gland about the size of a Pea. The kidneys are complicated organs that have numerous biological roles It can also be induced iatrogenically by various drugs. The terms iatrogenesis and iatrogenic artifact refer to adverse effects or complications caused by or resulting from medical treatment or advice

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). Diabetes mellitus (ˌdaɪəˈbiːtiːz or /ˌdaɪəˈbiːtəs/ /məˈlaɪtəs/ or /ˈmɛlətəs/ often referred to simply as diabetes ( Ancient Greek: grc Hyperglycemia, hyperglycaemia, or high blood sugar is a condition in which an excessive amount of Glucose circulates in the Blood plasma Blood sugar, used in a physiological context is a misnomer and misleading 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. Dehydration ( hypohydration) is the removal of Water ( hydro in ancient Greek) from an object

The extreme urination continues throughout the day and the night. In children, DI can interfere with appetite, eating, weight gain, and growth as well. Human development is the process of Growing to maturity In biological terms this entails growth from a one-celled Zygote to an adult Human being They may present with fever, vomiting, or diarrhea. Fever (also known as pyrexia, from the Greek pyretos meaning fire or a febrile response, from the Latin word Febris Vomiting (also called throwing up, emesis) is the forceful expulsion of the contents of one's Stomach through the Mouth and sometimes the In Medicine, diarrhea, also spelled diarrhoea (see spelling differences) is frequent loose or liquid Bowel movements Acute 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. Dehydration ( hypohydration) is the removal of Water ( hydro in ancient Greek) from an object

Diagnosis

In order to distinguish DI from other causes of excess urination, blood glucose levels, bicarbonate levels, and calcium levels need to be tested. Blood sugar, used in a physiological context is a misnomer and misleading In Inorganic chemistry, bicarbonate ( IUPAC -recommended nomenclature hydrogencarbonate) is an intermediate form in the Deprotonation Calcium (ˈkælsiəm is the Chemical element with the symbol Ca and Atomic number 20 Measurement of blood electrolytes can reveal a high sodium level (hypernatremia as dehydration develops). An electrolyte is any substance containing free Ions that behaves as an electrically conductive medium Sodium (ˈsoʊdiəm is an element which has the symbol Na( Latin natrium, from Arabic natrun) atomic number 11 atomic mass 22 Hypernatremia is an Electrolyte disturbance that is defined by an elevated Sodium level in the blood Dehydration ( hypohydration) is the removal of Water ( hydro in ancient Greek) from an object Urinalysis demonstrates a dilute urine with a low specific gravity. A urinalysis (or "UA" is an array of tests performed on Urine and one of the most common methods of Medical Diagnosis. Specific gravity is defined as the ratio of the Density of a given solid or liquid substance to the density of water at a specific temperature and pressure typically Urine osmolality and electrolyte levels are typically low. Osmolarity is a measure of the osmoles of solute per Liter of solution while the osmolality is a measure of the osmoles of Solute per Kilogram

A fluid deprivation test helps determine whether DI is caused by:

1. excessive intake of fluid
2. a defect in ADH production
3. a defect in the kidneys' response to ADH

This test measures changes in body weight, urine output, and urine composition when fluids are withheld and as dehydration occurs. Arginine vasopressin ( AVP) also known as vasopressin, argipressin or antidiuretic hormone ( ADH) is a Hormone found in Arginine vasopressin ( AVP) also known as vasopressin, argipressin or antidiuretic hormone ( ADH) is a Hormone found in 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. Desmopressin (trade names DDAVP, Stimate, Minirin) is a Synthetic replacement for antidiuretic hormone, the Hormone that 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. The pituitary gland, or hypophysis, is an Endocrine gland about the size of a Pea. A prolactinoma is a Benign Tumor ( Adenoma) of the Pituitary gland that produces a Hormone called Prolactin. In Medicine, histiocytosis is an excessive number of Histiocytes that is an excessive number of tissue Macrophages, and is typically used Syphilis is a Sexually transmitted disease caused by the spirochetal Bacterium Treponema pallidum pallidum. Tuberculosis (abbreviated as TB for tubercle bacillus or T u' b' erculosis Bacillus --> is a common See also Cancer A tumor or tumour is the name for a swelling or lesion formed by an abnormal growth of cells (termed neoplastic In Medicine ( Anatomical pathology) a granuloma (classical Latin plural granulomata; modern anglicized plural granulomas, also accepted 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. Psychogenic polydipsia is a special form of Polydipsia, caused by mental disorders 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.

Pathophysiology

Electrolyte and volume homeostasis is a complex mechanism that balances the body's requirements for blood pressure and the main electrolytes sodium and potassium. Homeostasis (from Greek: ὅμος hómos, "equal" and ιστημι istēmi, "to stand" lit Blood pressure is also the title of a short story by Damon Runyan in Guys and Dolls and Other Stories Sodium (ˈsoʊdiəm is an element which has the symbol Na( Latin natrium, from Arabic natrun) atomic number 11 atomic mass 22 Potassium (pəˈtæsiəm is a Chemical element. It has the symbol K (kalium from qalīy Atomic number 19 and Atomic mass 39 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. The hypothalamus links the Nervous system to the Endocrine system via the Pituitary gland (hypophysis Arginine vasopressin ( AVP) also known as vasopressin, argipressin or antidiuretic hormone ( ADH) is a Hormone found in The supraoptic nucleus ( SON) is a nucleus of Magnocellular neurosecretory cells in the Hypothalamus of the mammalian brain The paraventricular nucleus (PVN is an aggregation of neurons in the Hypothalamus which produces many Hormones. 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. The pituitary gland, or hypophysis, is an Endocrine gland about the size of a Pea. 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 ventromedial nucleus (sometimes referred to as the ventromedial hypothalamus) is a nucleus of the Hypothalamus. The cerebral cortex is a structure within the Brain that plays a key role in Memory, Attention, perceptual Awareness, Thought,

The main effector organ for fluid homeostasis is the kidney. In medicine body water is all of the Water content of the Human body. The kidneys are complicated organs that have numerous biological roles 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. The collecting duct system of the Kidney consists of a series of tubules and ducts that connect the Nephrons to the Ureter. Aquaporins are Integral membrane proteins from a larger family of Major intrinsic proteins (MIP that form Pores in the membrane This increase in permeability allows for reabsorption of water into the bloodstream, thus concentrating the urine.

There are several forms of DI:

• Central diabetes insipidus is due to damage to the hypothalamus or pituitary due to a tumor, 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). See also Cancer A tumor or tumour is the name for a swelling or lesion formed by an abnormal growth of cells (termed neoplastic A stroke is the rapidly developing loss of brain functions due to a disturbance in the blood vessels supplying blood to the brain Neurosurgery is the surgical discipline focused on treating those central, Peripheral nervous system and spinal column diseases amenable to surgical Haemochromatosis, also spelled hemochromatosis (see spelling differences) also called siderophilia Sarcoidosis, also called sarcoid (from the Greek sarx, meaning "flesh" or Besnier-Boeck disease, is an immune system disorder characterized In Medicine, histiocytosis is an excessive number of Histiocytes that is an excessive number of tissue Macrophages, and is typically used Tuberculosis (abbreviated as TB for tubercle bacillus or T u' b' erculosis Bacillus --> is a common Syphilis is a Sexually transmitted disease caused by the spirochetal Bacterium Treponema pallidum pallidum. A genetic disorder is a condition caused by abnormalities in Genes or Chromosomes While some diseases such as Cancer, are due to genetic abnormalities acquired 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 (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). Aquaporins are Integral membrane proteins from a larger family of Major intrinsic proteins (MIP that form Pores in the membrane 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 hypokalaemia and hypercalcaemia. Medication, also referred to as medicine, can be loosely defined as any substance intended for use in the diagnosis cure mitigation treatment or prevention of disease Lithium citrate (Li3C6H5O7 is a chemical compound of Lithium and Citrate that is used as a Mood stabilizer Amphotericin B (Fungilin Fungizone Abelcet AmBisome Fungisome Amphocil Amphotec is a Polyene Antifungal drug, often used Intravenously Polycystic kidney disease ( PKD, also known as polycystic kidney syndrome is a progressive ciliopathic, Genetic disorder of the Kidneys Sickle-cell disease or sickle-cell anaemia (or anemia) is a Blood disorder characterized by Red blood cells that assume an abnormal rigid 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.
• Gestational DI only occurs during pregnancy. Pregnancy ( Latin graviditas) is the carrying of one or more offspring known as a Fetus or Embryo, inside the Uterus of a Female While all pregnant women produce vasopressinase in the placenta, which breaks down ADH, this can assume extreme forms in GDI. The placenta is an Ephemeral organ present in placental Vertebrates, such as Eutherial Mammals and Sharks during Gestation 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.

Treatment

Central DI and gestational DI respond to desmopressin. Desmopressin (trade names DDAVP, Stimate, Minirin) is a Synthetic replacement for antidiuretic hormone, the Hormone that 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 NDI; HCT is sometimes combined with amiloride to prevent hypokalemia. A diuretic is any Drug that elevates the rate of urination ( Diuresis) Hydrochlorothiazide, sometimes abbreviated HCT, HCTZ, or HZT is a popular Diuretic drug of the Thiazide class that acts by inhibiting Indometacin ( INN Amiloride is a potassium-sparing diuretic, first approved for use in 1967 (then known as MK 870 used in the management of Hypertension and Congestive Hypokalemia refers to the condition in which the concentration of Potassium in the blood is low Again, adequate hydration is important for patients with DI, as they may become dehydrated easily.

Sources

• The public domain document "Diabetes Insipidus", NIH Publication No. The public domain is a range of abstract materials &ndash commonly referred to as Intellectual property &ndash which are not owned or controlled by anyone "NIH" redirects here For other meanings of NIH see NIH (disambiguation. 01-4620, December 2000.

External links

• The Diabetes Insipidus Foundation, Inc
• Information on Diabetes Insipidus