Nephrogenic Diabetes Insipidus
Nephrogenic diabetes insipidus (NDI) is a kidney disorder characterized by the organ's inability to respond to the antidiuretic hormone (ADH), also called arginine vasopressin (AVP), produced in the hypothalamus, a structure of the brain. NDI involves an abnormality in the kidney tubules which prevents the proper amount of water from being reabsorbed from the kidneys back into the body. Instead, the water is excreted in large amounts as diluted urine. There are two categories of nephrogenic diabetes insipidus: inherited and acquired. Within the inherited group, there are three types of NDI: X-linked, autosomal recessive, and autosomal dominant. Unlike the more common diabetic disorder diabetes mellitus, NDI is not related to insulin production or levels of sugar in the blood and urine. Ninety percent of inherited NDI is X-linked, meaning it is caused by an alteration in a gene carried on the X chromosome . Since women have two X chromosomes and men have only one, an X-linked recessive condition is expected to effect men since they do not have a second X chromosome with a normal copy of the gene to produce the needed substance. Autosomal recessive NDI is rarer and equally affects males and females. Autosomal dominant NDI is the most rare of the three and affects both males and females. Inherited NDI is present from birth and symptoms usually manifest within the first several days of life. If the disorder is not diagnosed and treated early, it will cause the body to lose too much water. This dehydration can lead to brain damage and eventually death. But, with early diagnosis and treatment to avoid severe dehydration, the person can live a normal life span without any mental impairment. Acquired NDI is the most common type of the disease and can be acquired at any age. It Continue reading >>
Diabetes Insipidus: Definition, Causes & Types
Although you have heard of diabetes, chances are you haven't heard of an unrelated disease called diabetes insipidus. This lesson will define this disease as well as list the different types, while trying to ascertain the cause of diabetes insipidus in a three-year old child. Case Study, Definition and Symptoms A three-year-old male won't stop drinking water and juice. He urinates more than he used to, his urine is diluted, and he becomes hysterical when fluids are withheld. What's going on here? There are several possibilities, and doctors would need to run tests to pinpoint the exact cause. However, these symptoms sound like the three-year old could have diabetes insipidus, which is a condition that causes excessive thirst and increased urination. Sometimes, you may hear doctors refer to diabetes insipidus as DI. Urine can be dilute, or water-colored, or concentrated, or really yellow. The urine here slightly concentrated While part of its name is 'diabetes,' it isn't the type of diabetes you're probably thinking about. And while some of the symptoms are similar, its causes are quite different. So, when you hear 'diabetes insipidus,' realize we are not talking about the diabetes that requires sugar-levels and insulin to be monitored. So, why do they have the same name? Well, an ancient Greek doctor used the term 'diabetes' to describe a condition where someone urinated a lot (which also occurs in the type of diabetes you've probably heard of). Diabetes means 'to pass through,' so urine passing through the body seemed to fit. And 'insipidus' means 'no taste' in Latin, which is fitting because there is no extra sugar in the urine when a patient has diabetes insipidus, unlike the more common types of diabetes. Before we go into the specific types of diabetes insipidus, l Continue reading >>
Diabetes Insipidus
On this page: What is diabetes insipidus? Diabetes insipidus is a rare disorder that occurs when a person's kidneys pass an abnormally large volume of urine that is insipid—dilute and odorless. In most people, the kidneys pass about 1 to 2 quarts of urine a day. In people with diabetes insipidus, the kidneys can pass 3 to 20 quarts of urine a day. As a result, a person with diabetes insipidus may feel the need to drink large amounts of liquids. Diabetes insipidus and diabetes mellitus—which includes both type 1 and type 2 diabetes—are unrelated, although both conditions cause frequent urination and constant thirst. Diabetes mellitus causes high blood glucose, or blood sugar, resulting from the body's inability to use blood glucose for energy. People with diabetes insipidus have normal blood glucose levels; however, their kidneys cannot balance fluid in the body. What are the kidneys and what do they do? The kidneys are two bean-shaped organs, each about the size of a fist. They are located just below the rib cage, one on each side of the spine. Every day, the kidneys normally filter about 120 to 150 quarts of blood to produce about 1 to 2 quarts of urine, composed of wastes and extra fluid. The urine flows from the kidneys to the bladder through tubes called ureters. The bladder stores urine. When the bladder empties, urine flows out of the body through a tube called the urethra, located at the bottom of the bladder. How is fluid regulated in the body? A person's body regulates fluid by balancing liquid intake and removing extra fluid. Thirst usually controls a person’s rate of liquid intake, while urination removes most fluid, although people also lose fluid through sweating, breathing, or diarrhea. The hormone vasopressin, also called antidiuretic hormone, con Continue reading >>
Diabetes Insipidus
Overview Diabetes insipidus is a rare condition where you produce a large amount of urine and often feel thirsty. Diabetes insipidus isn't related to diabetes mellitus (usually just known as diabetes), but it does share some of the same signs and symptoms. The two main symptoms of diabetes insipidus are: passing large amounts of urine, even at night (polyuria) In very severe cases of diabetes insipidus, up to 20 litres of urine can be passed in a day. Read more about the symptoms of diabetes insipidus. When to seek medical advice You should always see your GP if you're feeling thirsty all the time. Although it may not be diabetes insipidus, it should be investigated. Also see your GP if you're: passing more urine than normal – most healthy adults pass urine four to seven times in a 24 hour period passing small amounts of urine at frequent intervals – sometimes, this can occur along with the feeling that you need to pass urine immediately Children tend to urinate more frequently because they have smaller bladders. However, seek medical advice if your child urinates more than 10 times a day. Your GP will be able to carry out a number of tests to help determine what's causing the problem. Read more about diagnosing diabetes insipidus. What causes diabetes insipidus? Diabetes insipidus is caused by problems with a hormone called vasopressin (AVP), also called antidiuretic hormone (ADH). AVP plays a key role in regulating the amount of fluid in the body. It's produced by specialist nerve cells in a part of the brain known as the hypothalamus. AVP passes from the hypothalamus to the pituitary gland where it's stored until needed. The pituitary gland releases AVP when the amount of water in the body becomes too low. It helps retain water in the body by reducing the amount Continue reading >>
Diabetes Insipidus
Diabetes insipidus (DI) is a condition characterized by large amounts of dilute urine and increased thirst.[1] The amount of urine produced can be nearly 20 liters per day.[1] Reduction of fluid has little effect on the concentration of the urine.[1] Complications may include dehydration or seizures.[1] There are four types of DI, each with a different set of causes.[1] Central DI (CDI) is due to a lack of the hormone vasopressin (antidiuretic hormone).[1] This can be due to damage to the hypothalamus or pituitary gland or genetics.[1] Nephrogenic diabetes insipidus (NDI) occurs when the kidneys do not respond properly to vasopressin.[1] Dipsogenic DI is due to abnormal thirst mechanisms in the hypothalamus while gestational DI occurs only during pregnancy.[1] Diagnosis is often based on urine tests, blood tests, and the fluid deprivation test.[1] Diabetes mellitus is a separate condition with an unrelated mechanism, though both can result in the production of large amounts of urine.[1] Treatment involves drinking sufficient fluids to prevent dehydration.[1] Other treatments depend on the type.[1] In central and gestational disease treated is with desmopressin.[1] Nephrogenic disease may be treated by addressing the underlying cause or the use of a thiazide, aspirin, or ibuprofen.[1] The number of new cases of diabetes insipidus each year is 3 in 100,000.[4] Central DI usually starts between the ages of 10 and 20 and occurs in males and females equally.[2] Nephrogenic DI can begin at any age.[3] The term "diabetes" is derived from the Greek word meaning siphon.[5] Signs and symptoms[edit] Excessive urination and extreme thirst and increased fluid intake (especially for cold water and sometimes ice or ice water) are typical for DI.[6] The symptoms of excessive urination Continue reading >>
Diabetes Insipidus
Diabetes Insipidus Definition Diabetes insipidus (DI) is a disorder that causes the patient to produce tremendous quantities of urine. The massively increased urine output is usually accompanied by intense thirst. Description The balance of fluid within the body is maintained through a number of mechanisms. One important chemical involved in fluid balance is called antidiuretic hormone (ADH). ADH is produced by the pituitary, a small gland located at the base of the brain. In a healthy person and under normal conditions, ADH is continuously released. ADH influences the amount of fluid that the kidneys reabsorb into the circulatory system and the amount of fluid that the kidneys pass out of the body in the form of urine. Production of ADH is regulated by the osmolality of the circulating blood. Osmolality refers to the concentration of dissolved chemicals (such as sodium, potassium, and chloride; together called solute) circulating in the fluid base of the blood (plasma). When there is very little fluid compared to the concentration of solute, the pituitary will increase ADH production. This tells the kidneys to retain more water and to decrease the amount of urine produced. As fluid is retained, the concentration of solute will normalize. At other times, when the fluid content of the blood is high in comparison to the concentration of solute, ADH production will decrease. The kidneys are then free to pass an increased amount of fluid out of the body in the urine. Again, this will allow the plasma osmolality to return to normal. Diabetes insipidus occurs when either the amount of ADH produced by the pituitary is below normal (central DI), or the kidneys' ability to respond to ADH is defective (nephrogenic DI). In either case, a person with DI will pass extraordinarily la Continue reading >>
Everything You Should Know About Diabetes Insipidus
What is diabetes insipidus? Diabetes insipidus (DI) is a rare condition that occurs when your kidneys are not able to conserve water. DI is not related to diabetes mellitus, which is often referred to simply as diabetes. That means you can have DI without having diabetes. In fact, the condition can occur in anyone. DI results in extreme thirst and frequent urination of dilute and odorless urine. There are several types of DI, and they can often be successfully treated. Keep reading to learn more about this condition. The main symptoms of DI are excessive thirst, which can cause an uncontrollable craving for water, and excessive urine volume. A healthy adult will typically urinate less than 3 quarts of urine a day. People with DI may eliminate up to 16 quarts of urine a day. You may need to get up during the night to urinate frequently, or you may experience bed-wetting. Possible symptoms in young children and infants include: fussiness and irritability unusually wet diapers or bed-wetting, or excessive urine output excessive thirst dehydration high fever dry skin delayed growth Adults can experience some of the above symptoms, plus confusion, dizziness, or sluggishness. DI can also lead to severe dehydration, which can lead to seizures, brain damage, and even death if not treated. You should contact your doctor immediately if you or your child is experiencing these symptoms. To understand diabetes insipidus, it helps to understand how your body normally uses and regulates fluids. Fluids make up as much as 60 percent of your overall body mass. Maintaining the proper amount of fluid in your body is key to your overall health. Consuming water and food throughout the day helps provide fluid to your body. Urinating, breathing, and sweating help to eliminate fluid from your b Continue reading >>
Diabetes Insipidus
Print Overview Diabetes insipidus (die-uh-BEE-teze in-SIP-uh-dus) is an uncommon disorder that causes an imbalance of water in the body. This imbalance leads to intense thirst even after drinking fluids (polydipsia), and excretion of large amounts of urine (polyuria). While the names diabetes insipidus and diabetes mellitus sound similar, they're not related. Diabetes mellitus — which can occur as type 1 or type 2 — is the more common form of diabetes. There's no cure for diabetes insipidus, but treatments are available to relieve your thirst and normalize your urine output. Symptoms The most common signs and symptoms of diabetes insipidus are: Extreme thirst Excretion of an excessive amount of diluted urine Depending on the severity of the condition, urine output can be as much as 16 quarts (about 15 liters) a day if you're drinking a lot of fluids. Normally, a healthy adult will urinate an average of less than 3 quarts (about 3 liters) a day. Other signs may include needing to get up at night to urinate (nocturia) and bed-wetting. Infants and young children who have diabetes insipidus may have the following signs and symptoms: Unexplained fussiness or inconsolable crying Trouble sleeping Fever Vomiting Diarrhea Delayed growth Weight loss When to see a doctor See your doctor immediately if you notice the two most common signs of diabetes insipidus: excessive urination and extreme thirst. Causes Diabetes insipidus occurs when your body can't regulate how it handles fluids. Normally, your kidneys remove excess body fluids from your bloodstream. This fluid waste is temporarily stored in your bladder as urine, before you urinate. When your fluid regulation system is working properly, your kidneys conserve fluid and make less urine when your body water is decreased, suc Continue reading >>
Diabetes Insipidus
During the day, your kidneys filter all your blood many times. Normally, most of the water is reabsorbed, and only a small amount of concentrated urine is excreted. DI occurs when the kidneys cannot concentrate the urine normally, and a large amount of dilute urine is excreted. The amount of water excreted in the urine is controlled by antidiuretic hormone (ADH). ADH is also called vasopressin. ADH is produced in a part of the brain called the hypothalamus. It is then stored and released from the pituitary gland. This is a small gland just below the base of the brain. DI caused by a lack of ADH is called central diabetes insipidus. When DI is caused by a failure of the kidneys to respond to ADH, the condition is called nephrogenic diabetes insipidus. Nephrogenic means related to the kidney. Central DI can be caused by damage to the hypothalamus or pituitary gland as a result of: Head injury Infection Loss of blood supply to the pituitary gland Surgery Nephrogenic DI involves a defect in the kidneys. As a result, the kidneys do not respond to ADH. Like central DI, nephrogenic DI is very rare. Nephrogenic DI may be caused by: Certain drugs, such as lithium Genetic problems Continue reading >>
Diabetes Insipidus
Practice Essentials Diabetes insipidus (DI) is defined as the passage of large volumes (>3 L/24 hr) of dilute urine (< 300 mOsm/kg). It has the following 2 major forms: Two other forms are gestational DI and primary polydipsia (dipsogenic DI); both are caused by deficiencies in AVP, but the deficiencies do not result from a defect in the neurohypophysis or kidneys. Signs and symptoms The predominant manifestations of DI are as follows: The most common form is central DI after trauma or surgery to the region of the pituitary and hypothalamus, which may exhibit 1 of the following 3 patterns: In infants with DI, the most apparent signs may be the following: In children, the following manifestations typically predominate: If the condition that caused DI also damaged the anterior pituitary or hypothalamic centers that produce releasing factors, patients may present with the following: Physical findings vary with the severity and chronicity of DI; they may be entirely normal or may include the following: See Clinical Presentation for more detail. If the clinical presentation suggests DI, laboratory tests must be performed to confirm the diagnosis, as follows: Additional studies that may be indicated include the following: See Workup for more detail. Management Most patients with DI can drink enough fluid to replace their urine losses. When oral intake is inadequate and hypernatremia is present, provide fluid replacement as follows: Give dextrose and water or an intravenous fluid that is hypo-osmolar with respect to the patient’s serum; do not administer sterile water without dextrose IV Administer fluids at a rate no greater than 500-750 mL/hr; aim at reducing serum sodium by approximately 0.5 mmol/L (0.5 mEq/L) every hour Pharmacologic therapeutic options include the follo Continue reading >>
Medical Definition Of Diabetes Insipidus
Diabetes insipidus: Excessive urination and extreme thirst as a result of inadequate output of the pituitary hormone ADH (antidiuretic hormone, also called vasopressin) or the lack of the normal response by the kidney to ADH. There are two types of diabetes insipidus -- central and nephrogenic. Central diabetes insipidus is a lack of ADH production and is due to damage to the pituitary gland or hypothalamus where ADH is produced. Nephrogenic diabetes insipidus is lack of response of the kidney to the fluid-conserving action of ADH. Nephrogenic diabetes insipidus can be due to diseases of the kidney (such as polycystic kidney disease), certain drugs (such as lithium), and can also occur an inherited disorder. In both central and nephrogenic diabetes insipidus, patients excrete extraordinarily large volumes of very dilute urine. They feel thirsty and drink very large amounts of water to compensate for the water they lose in the urine. The main danger with diabetes insipidus comes when fluid intake does not keep pace with urine output, resulting in dehydration and high blood sodium. The treatment of central diabetes insipidus is with vasopressin used as a nasal spray or as tablets. Nephrogenic diabetes insipidus does not respond to vasopressin treatment. In cases of nephrogenic diabetes insipidus caused by a drug (such as lithium), stopping the drug usually leads to recovery. In cases of hereditary nephrogenic diabetes insipidus, treatment is with fluid intake to match urine output and drugs that lower urine output. Effective treatment is important because the dehydration and high blood sodium can cause brain damage and death. Last Editorial Review: 5/13/2016 Continue reading >>
Diabetes Insipidus - Symptoms, Diagnosis And Treatment | Bmj Best Practice
Disorder characterized by polydipsia, polyuria, increased thirst, and formation of hypotonic urine. Two types exist: central DI, due to defective synthesis or release of arginine vasopressin (AVP) from the hypothalamo-pituitary axis; and nephrogenic DI, due to renal insensitivity to AVP. Recognized risk factors for central DI include pituitary surgery, craniopharyngioma, infiltrative pituitary stalk lesions, traumatic brain injury, subarachnoid hemorrhage, congenital hypothalamo-pituitary defects, autoimmune disorders, and Wolfram syndrome. Risk factors for nephrogenic DI include lithium therapy, chronic renal disease, and chronic hypercalcemia or hypokalemia. Genetic mutations are responsible for inherited forms of both types. Both types of DI may be associated with hypernatremia, and this may present as a medical emergency. Treatment goals are correction of any pre-existing water deficits and reduction in ongoing excessive urinary water losses. In central DI, desmopressin is the treatment of choice. Nephrogenic DI is treated with an adequate fluid intake; salt restriction and diuretics may help reduce polyuria. Diabetes insipidus (DI) is a metabolic disorder characterized by defective ability to concentrate urine in the kidneys, resulting in the production of large quantities of dilute urine. It may result from an absolute or relative deficiency of arginine vasopressin (AVP), also known as antidiuretic hormone (ADH), which is produced by the hypothalamus and secreted via the posterior pituitary, or by resistance to its action within the renal collecting ducts. Clinically it manifests as polydipsia, polyuria, and hypotonic urine. [1] Robinson A, Verbalis J. Posterior pituitary. In: Kronenberg H, Melmed S, Polonsky K, et al., eds. Williams Textbook of endocrinology. 11 Continue reading >>
Diabetes Insipidus
A disease characterized by excretion of large amount of severely diluted urine, which cannot be reduced when fluid intake is reduced. It is generally related to a tumor or insult to the pituitary gland, where the hormonal signals the pituitary gives to the kidneys break down, and the kidneys do not conserve water, but express all water as urine. This is a rather rare disease. Continue reading >>
Diabetes Insipidus: Causes, Symptoms And Treatment
Diabetes insipidus is a condition where the body loses too much fluid through urination, causing a significant risk of dangerous dehydration as well as a range of illnesses and conditions. There are two forms of the disease: nephrogenic diabetes insipidus and central diabetes insipidus (also known as neurogenic diabetes insipidus). A number of factors have been linked to the development of diabetes insipidus, which may also occur in pregnancy or with the use of certain medications. Establishing the cause of the problem can help determine the most appropriate treatment to support the regulation of water balance in the body. Diabetes insipidus is a condition that can be managed successfully. Contents of this article: What is diabetes insipidus? An uncommon condition, diabetes insipidus is a disorder affecting the regulation of body fluid levels. Two key symptoms resemble those of the more common forms of diabetes that affect blood sugar levels (diabetes mellitus types 1 and 2).1-5 People with diabetes insipidus produce excessive amounts of urine (polyuria), resulting in frequent urination and, in turn, thirst (polydipsia). However, the underlying cause of these two symptoms is quite different from the causes in types 1 and 2 diabetes. In diabetes mellitus, elevated blood sugar prompts the production of large volumes of urine to help remove the excess sugar from the body. In diabetes insipidus, it is the body's water balance system itself that is not working properly. Here are some key points about diabetes insipidus. More detail and supporting information is in the body of this article. Diabetes insipidus is a condition where the body fails to properly control water balance, resulting in excessive urination. Diabetes insipidus can be caused by low or absent secretion of t Continue reading >>
Diagnosis Of Polyuria And Diabetes Insipidus
DEFINITION Polyuria has generally been defined as a urine output exceeding 3 L/day in adults and 2 L/m2 in children. It must be differentiated from the more common complaints of frequency or nocturia, which are not associated with an increase in the total urine output. The following is an overview of the diagnosis of polyuria and diabetes insipidus (DI). The causes and treatment of polyuria due to central or nephrogenic DI are presented separately. (See "Clinical manifestations and causes of central diabetes insipidus" and "Clinical manifestations and causes of nephrogenic diabetes insipidus" and "Treatment of central diabetes insipidus" and "Treatment of nephrogenic diabetes insipidus".) CAUSES In the absence of a glucose-induced osmotic diuresis in uncontrolled diabetes mellitus, there are three major causes of polyuria in the outpatient setting, each of which is due to a defect in water balance leading to the excretion of large volumes of dilute urine (urine osmolality usually below 250 mosmol/kg): primary polydipsia, which is primarily seen in adults and adolescents; central DI; and nephrogenic DI [1]. Primary polydipsia — Primary polydipsia (sometimes called psychogenic polydipsia) is characterized by a primary increase in water intake. This disorder is most often seen in middle-aged women and in patients with psychiatric illnesses, including those taking a phenothiazine, which can lead to the sensation of a dry mouth. Primary polydipsia can also be induced by hypothalamic lesions that directly affect the thirst center, as may occur with an infiltrative disease such as sarcoidosis [1]. (See "Causes of hyponatremia in adults".) Central DI — Central DI (also called neurohypophyseal or neurogenic DI) is associated with deficient secretion of antidiuretic hormone ( Continue reading >>
