Diabetes insipidus
Introduction
Introduction Diabetes collapse, generally referred to as diabetes insipidus. Diabetes insipidus refers to vasopressin (VP), also known as insufficient secretion of antidiuretic hormone (ADH) (also known as central or pituitary diabetes insipidus), or renal vasopressin deficiency ( Also known as renal diabetes insipidus, a group of syndromes characterized by polyuria, polydipsia, low specific gravity urine and hypotonic urine.
Cause
Cause
1, primary (unexplained or idiopathic diabetes insipidus) diabetes insipidus: about 1/3 to 1/2. Usually in children with onset, very few (<20%) with anterior pituitary dysfunction. This diagnosis can only be determined after careful search for secondary causes that do not exist. When there is anterior pituitary dysfunction or hyperprolactinemia or radiographic examination with evidence of lesions in the sella or on the sella, the cause should be searched as much as possible. The longer the follow-up can not find the primary factor, The diagnosis of primary diabetes insipidus is affirmed. It has been reported that in patients with primary diabetes insipidus, neurons in the supraoptic nucleus and paraventricular nucleus are reduced, and antibodies to the hypothalamic nucleus are present in the circulation.
2, secondary diabetes insipidus: occurs in the hypothalamus or pituitary new organisms or invasive damage, including: chromoblastoma, craniopharyngioma, embryonal tumor, pineal tumor, glioma, meningioma, metastasis Tumor, leukemia, histiocytosis, sarcoma, xanthoma, sarcoidosis, and brain infectious diseases (tuberculosis, syphilis, vascular disease).
3, hereditary diabetes insipidus: hereditary diabetes insipidus is very rare, can be a single genetic defect, but also a part of DIDMOAD syndrome. (Can be expressed as diabetes insipidus, diabetes, optic atrophy, deafness, also known as Wolfram syndrome).
4, physical damage: common in the brain, especially in the pituitary, hypothalamic site surgery, isotope treatment, after severe brain trauma. Diabetes insipidus caused by surgery usually occurs 1 to 6 days after surgery and disappears after a few days. After a period of 1 to 5 days, the symptoms of diabetes insipidus disappeared permanently or relapsed into chronic. Severe brain trauma, often accompanied by skull fractures, can occur with diabetes insipidus, and a small number of patients with anterior pituitary dysfunction. Diabetes-induced diabetes insipidus can recover on its own, sometimes disappearing completely after 6 months.
Examine
an examination
Related inspection
Blood vasopressin test urine routine nephropathy
Symptoms of diabetes insipidus may occur during pregnancy, and symptoms disappear after several days of delivery. Xihan syndrome can show symptoms of diabetes insipidus after treatment with cortisone. AVP-resistant diabetes insipidus may occur during pregnancy, which may be due to increased vasopressin activity in the circulation during pregnancy. This patient's plasma AVP level increased, lack of response to high-dose AVP, but responded to desmopressin treatment, and the symptoms were relieved after delivery.
Pituitary diabetes insipidus can be seen at any age, usually in childhood or early adulthood, males are more common than females, and the ratio of male to female is about 2:1. The general onset date is clear. Most patients have polydipsia, polydipsia, and polyuria. Nocturia is significant, urine volume is relatively fixed, generally 4L / d or more, up to 18L / d, but there are reports of up to 40L / d. The urine weight is less than 1.006, and partial diabetes insipidation can reach 1.010 in severe dehydration. Urine osmotic pressure is mostly <200mOsm/kg?H2O. Thirst is often severe, and the amount of water in the normal center of the thirst is roughly equal to the amount of water. General people with diabetes insipidus like cold drinks. If drinking water is not restricted, it only affects sleep and causes physical weakness. Intelligence, physical development is close to normal. Polydipsia, polyuria can be aggravated during fatigue, infection, menstrual cycle and pregnancy. Hereditary diabetes insipidus starts from a young age, and dehydration fever and hypernatremia can occur due to hypoplasia of the central nervous system. In addition to localized symptoms, tumor and craniocerebral trauma surgery may also cause hypernatremia (, sputum, vomiting, etc.). Once the diabetes insipidus combined with anterior pituitary dysfunction, the symptoms of diabetes insipidus will be reduced, and the symptoms will reappear or worsen after glucocorticoid replacement therapy.
Diagnosis
Differential diagnosis
Diabetes insipidus must be differentiated from other types of polyuria. Some can be identified by medical history (such as the recent use of lithium or mannitol, surgery under methoxyflurane anesthesia or recent kidney transplantation). In other patients, a physical examination or a simple laboratory examination will prompt a diagnosis (such as diabetes, kidney disease, sickle cell anemia, hypercalcemia, hypokalemia, primary aldosteronism).
Congenital renal diabetes insipidus is a rare form of polyuria due to no response to AVP. Women are milder than men, and can concentrate urine when the water is banned, and is effective with a large amount of desmopressin. A family with this disease has an abnormal gene on the short arm of the X-ray chromosome. Most patients have V2 receptor abnormalities, and some patients have defects in the recipient's post. All patients had normal V1 receptor function. When renal diabetes insipidus and central diabetes insipidus cannot be identified by osmotic pressure measurement, the blood or urine AVP concentration associated with plasma osmotic pressure is increased, and the diagnosis of renal diabetes insipidus can be confirmed.
Primary polydipsia or polydipsia may sometimes be difficult to distinguish from diabetes insipidus, or both forms may exist simultaneously. Excessive long-term water intake causes hypotonic polyuria to be confused with diabetes insipidus. Intermittent large amounts of drinking water, even if the ability to dilute urine is normal, can lead to water intoxication and dilute hyponatremia. This phenomenon is rare, but the tendency of these patients to have low sodium increases. These patients often drink more urine and are often unstable, and often do not have nighttime polyuria, which is different from long-term polydipsia in diabetes insipidus. Combined with low plasma osmotic pressure and low osmotic pressure, the diagnosis of primary polydipsia can be clarified. The relationship is normal or often normal. In the water-free test, when the osmolality was stable, the osmolality did not increase or increase after injection of vasopressin. Due to long-term large amount of water intake inhibiting the release of AVP and long-term polyuria leading to loss of renal medullary osmotic pressure gradient, urine osmotic pressure can be lower than normal compared with blood osmotic pressure. Therefore, it is sometimes difficult to identify primary polydipsia and incomplete central diabetes insipidus, and some patients may have both cases.
Symptoms of diabetes insipidus may occur during pregnancy, and symptoms disappear after several days of delivery. Xihan syndrome can show symptoms of diabetes insipidus after treatment with cortisone. AVP-resistant diabetes insipidus may occur during pregnancy, which may be due to increased vasopressin activity in the circulation during pregnancy. This patient's plasma AVP level increased, lack of response to high-dose AVP, but responded to desmopressin treatment, and the symptoms were relieved after delivery.
Pituitary diabetes insipidus can be seen at any age, usually in childhood or early adulthood, males are more common than females, and the ratio of male to female is about 2:1. The general onset date is clear. Most patients have polydipsia, polydipsia, and polyuria. Nocturia is significant, urine volume is relatively fixed, generally 4L / d or more, up to 18L / d, but there are reports of up to 40L / d. The urine weight is less than 1.006, and partial diabetes insipidation can reach 1.010 in severe dehydration. Urine osmotic pressure is mostly <200mOsm/kg?H2O. Thirst is often severe, and the amount of water in the normal center of the thirst is roughly equal to the amount of water. General people with diabetes insipidus like cold drinks. If drinking water is not restricted, it only affects sleep and causes physical weakness. Intelligence, physical development is close to normal. Polydipsia, polyuria can be aggravated during fatigue, infection, menstrual cycle and pregnancy. Hereditary diabetes insipidus starts from a young age, and dehydration fever and hypernatremia can occur due to hypoplasia of the central nervous system. In addition to localized symptoms, tumor and craniocerebral trauma surgery may also cause hypernatremia (, sputum, vomiting, etc.). Once the diabetes insipidus combined with anterior pituitary dysfunction, the symptoms of diabetes insipidus will be reduced, and the symptoms will reappear or worsen after glucocorticoid replacement therapy.
