What Is Central Diabetes Insipidus?

CDI is divided into three types: congenital CDI, acquired CDI, and hereditary CDI.

Xingbing

(Chief physician)

Department of Neurosurgery, Peking Union Medical College Hospital

Diabetes inspidus (DI) is a group of symptoms caused by various reasons affecting the insufficient secretion of AVP (vasopressin, also known as antidiuretic hormone (ADH)) or the deficiency of the kidney's response to vasopressin. It is characterized by polydipsia, polydipsia, thirst, low specific gravity urine and hypotonic urine. Central diabetes insipidus (also known as vasopressin deficiency, hypothalamic diabetes insipidus, Central DI, CDI; Vasopressin deficiency; Hypothalamic DI) is caused by a variety of reasons such as trauma, tumors, surgery, etc. Insufficient posterior pituitary injury causes diabetes insipidus caused by insufficient AVP synthesis, transport, and secretion. The incidence of men and women is similar, and can occur in all ages, with 10 to 20 years of age being the highest incidence.

Western Medicine Name: Central diabetes insipidus
English name: Central DI, CDI

The main symptoms: Pee more and drink more
Multiple groups: 10-20 years old
Contagious: Non-contagious

Central diabetes insipidus disease classification

CDI is divided into three types: congenital CDI, acquired CDI, and hereditary CDI.

Congenital central diabetes insipidus: mainly familial central diabetes insipidus, familial hypopituitarism, and diabetes insipidus caused by congenital cytomegalovirus infection, accounting for 50% to 60% of diabetes insipidus

Acquired central diabetes insipidus: common in: head trauma and pituitary hypothalamus surgery: a common cause of CDI. Transient CDI is most common after pituitary surgery. Damage to the pituitary stem above the median bulge can cause permanent CDI. Tumors: craniopharyngioma, pituitary metastasis cancer, pituitary sarcoma, and lymphoma. Granuloma: sarcoidosis, histiocytosis, sarcoma, xanthomas, etc. Infectious diseases: encephalitis, meningitis, tuberculosis, syphilis, toxoplasmosis, etc. Angioplasty: aneurysm, aortic coronary artery bypass. Inflammatory: Lymphocytic funnel neurohypophysitis, granulomatosis, lupus erythematosus, scleroderma, etc .; chemical poisons; idiopathic; others: autoimmune diseases can also cause CDI, anti-serum exists in serum AVP cell antibodies.

Hereditary central diabetes insipidus: can be X-linked recessive, autosomal dominant, or autosomal recessive

Congenital central diabetes insipidus, idiopathic central diabetes insipidus, and autoimmune central diabetes insipidus are all caused by the pathological changes of the pituitary system, also known as primary central diabetes insipidus; trauma, Diabetes insipidus caused by tumors, surgery, infections, granulomas, and angiopathy is also called secondary central diabetes insipidus. ^[1-2]

Clinical manifestations of central diabetes insipidus

The age of onset of CDI is mostly between 8 and 12 years, and most in adults are between 25 and 40 years.

The clinical manifestations of central diabetes insipidus are twofold: polydipsia, polyuria, and thirst caused by insufficient antidiuretic hormone (AVP); performance related to the etiology, such as headaches caused by mass lesions. Most patients increase the frequency of urination at the beginning and increase their urine output. Children with onset of illness can cause dilation of the bladder, ureter, and renal pelvis due to long-term polyuria, impair renal function, and may be associated with osteoporosis. Children may develop bedwetting. If the patient cannot or cannot drink water, hypovolemic manifestations such as palpitations, palpitation, decreased blood pressure, cold limbs, shock, and prerenal azotemia can be promptly corrected at this time. If the hypovolemia is not corrected in time, headaches, irritability, lookouts, and coma can occur.

Patients with CDI have low anterior pituitary function. The incidence of pituitary hormone deficiency is ranked from high to low as growth hormone deficiency, glucocorticoid deficiency, gonadotropin deficiency, prolactin deficiency, and thyroid hormone deficiency. Patients with bite-onset diabetes insipidus and hypopituitarism are more common.

According to the occurrence and duration of diabetes insipidus, it is divided into temporary, persistent and three-phase diabetes insipidus. Transient diabetes insipidus is caused by injury to the hypothalamus, pituitary stalk, or nerve pituitary due to trauma and surgery. It usually occurs suddenly after surgery or after injury and recovers within a few days. Persistent diabetes insipidus is the result of permanent damage to AVP-producing neurons. It usually occurs within 1 to 3 days. It improves after a few days, but it does not return to normal, and it is often associated with water and electrolyte disorders. Triple-phase diabetes insipidus, including acute, intermediate, and persistent.

According to the disappearance of 24 patients, urine volume can be divided into three types: light, medium and heavy. Mild urine output is 3000 ~ 4000ml; moderate urine output is 4000 ~ 6000ml; severe urine output is above 6000ml. ^[3]

Central diabetes insipidus diagnosis test

Central Diabetes Insipidus Water Test

All drugs that affect urine output were discontinued a few days before the test. Body weight, blood pressure, plasma osmotic pressure, urine specific gravity and urine osmotic pressure were measured before the test was started, and urine volume, urine specific gravity, urine osmotic pressure and electrolytes were measured every 1 to 2 hours thereafter. During the test, drinking water and various beverages were forbidden, and foods with less water content could be eaten normally. If the osmotic pressure difference of the urine sample is less than 30 mmol / L for two consecutive times, the test may be terminated. Normal people show a decrease in urine output within a few hours after water deprivation (often below 0.5ml / min), a significant increase in urine specific gravity (> 1.020), a significant increase in urine osmotic pressure (> 800mmol / L), and no significant increase in plasma osmotic pressure. High (<300mmol / L). In patients with complete central diabetes insipidus, the urine cannot be fully concentrated after the water is absent, and the urine output is not significantly reduced. The urine specific gravity is still less than 1.010, the urine osmolarity is <300mmol / L, the plasma osmotic pressure is> 300mmol / L, and the urine osmotic pressure and plasma osmosis Pressure ratio <1. In some cases of diabetes insipidus, the peak of urine specific gravity does not exceed 1.020, and the peak of osmotic pressure of urine does not exceed 750 mmol / L. ^[4]

-AVP Central Diabetes Insipidus Water-AVP Test

AVP was given after sufficient water abstinence, and the patient's response to AVP was observed. The urine of normal people has been fully concentrated after the water is absent, and the injection of AVP does not make the urine further concentrated, and the urine osmotic pressure and urine specific gravity do not increase further. Patients with complete central diabetes insipidus have significantly reduced urine output after injection of AVP, the urine specific gravity has increased above 1.020, and the urine osmotic pressure has increased by more than 50%; the urine osmotic pressure of partial diabetes insipidus has increased by less than 50%

Hypertonic saline test: After instillation of hypertonic saline in normal people, the plasma AVP level is significantly increased, the reabsorption of free water by the kidney is increased, and the urine volume is reduced by more than 70% compared with that before infusion. At the same time, the urine specific gravity and urine osmotic pressure increase high. Patients with central diabetes insipidus have mildly elevated or non-increased plasma AVP levels, so there is no sudden decrease in urine output, increased urine specific gravity, and increased urine osmolarity; however, after AVP injection, urine volume decreases, urine specific gravity, and urine penetration The pressure rises. Patients with hypertension and heart failure are forbidden to do this test, and normal patients also need to watch closely when doing this test.

Central Diabetes Insipidus Diagnostic Criteria

1. Drink more urine, urine output> 4000ml (or 200ml / hr or 6ml / kg / h), last for more than 24 hours;

2. Urine specific gravity1.005, urine osmotic pressure200mOsm / kg.H2O;

3. Plasma osmotic pressure 300mOsm / kg.H2O;

4. Urinary osmotic pressure / blood osmotic pressure <1;

5. Water ban test: 4-6 hours after water ban, symptoms of dehydration, constant urine output, urine specific gravity not exceeding 1.015, and urine osmotic pressure not exceeding plasma osmotic pressure;

6. Pituitary vasopressin test: rapid increase in urine specific gravity 1.018, urine osmotic pressure> 9%, urine osmotic pressure / plasma osmotic pressure> 1;

7. Blood sodium concentration 150mmol / L;

8. Determination of plasma vasopressin: AVP value is lower than normal (normal person 1 ~ 1.5ng / L)

9.MRI: disappearance of high signal of posterior pituitary;

10. Kidney function is normal.

Central Diabetes Insipidus Imaging

1. Patients with suspected central diabetes insipidus should undergo craniocerebral / saddle region enhanced MRI

2. MRI manifestations of central diabetes insipidus.

Hereditary or idiopathic CDI: Because the posterior lobe of the pituitary gland contains neuroendocrine particles that store hormones, the T1 weighted image of the saddle region magnetic resonance imaging is a bright high signal (provided that the thin layer scan of the saddle region), this signal is It is present in nearly 80% of normal people and is absent in most patients with diabetes insipidus. In patients with familial central diabetes insipidus, a bright signal of the pituitary gland exists in the early stages of the disease, but it usually disappears with the severity of diabetes insipidus. The persistent bright signal of the pituitary gland in some patients with diabetes insipidus may originate from the contained oxytocin. The thickening of the pituitary stalk is usually accompanied by the lack of bright signals from the pituitary gland. At this time, we should look for possible systemic diseases. For diabetes insipidus with only pituitary thickening, MRI should be reviewed every 3 to 6 months to exclude neoplastic lesions such as germ cell tumors. If the pituitary stalk shrinks during follow-up, it may be lymphocytic pituitary.

Tumorous: Can find lesions occupying the saddle area (tumorous lesions such as craniopharyngioma, germ cell tumor, or metastatic carcinoma in the saddle area). MRI showed a thickening of the hypothalamic mass and pituitary stalk. In metastatic pituitary cancer, tumor cells are twice as likely to metastasize to the pituitary gland as the arteries supplying the pituitary gland are more straightforward. Most of the primary tumors in the pituitary area grow relatively slowly, so metastatic carcinoma in the saddle area should be considered for tumors with diabetes insipidity and rapid growth in a short time.

Inflammatory lesions: The pituitary stem is thickened or nodular-like, and some lesions are misdiagnosed as MRI with a pituitary adenoma.

Three- quarters of patients with closed head trauma are due to motorcycle accidents, with young men mostly. MRI / CT can manifest as hypothalamic or neural pituitary hemorrhage, pituitary stem severance, or neural pituitary infarction.

Central diabetes insipidus disease treatment

Treatment and prevention of central diabetes insipidus

Caused by tumor compression, inflammatory invasion, or traumatic brain injury, the primary disease must be managed. In patients with saddle tumors, try not to damage the posterior pituitary lobe, pituitary stalk, and pituitary portal system during surgery. Radiotherapy, surgery and drug treatment can be done for tumors; hormone treatment can be used for inflammatory lesions. ^[5]

Symptomatic treatment of central diabetes insipidus

Hormone replacement therapy

Desmopressin Acetate Tablets: The trade name is Mi Ning, which has been proven in clinical applications for more than 20 years, and has good curative effect and few side effects. It is considered to be the drug of choice for the treatment of CDI. Generally, the appropriate initial dose for adults and children is 0.05 to 0.1 mg three times a day. Use with caution in pregnant women.

Desmopressin acetate injection: trade name Mi lemon. The dosage form is 4 & micro; g / 1ml; adults 1-2 times a day, 1-4 micrograms (0.25-1 ml) each time; children over one year old, 1-2 times a day, 0.1-1 micrograms (0.025-0.25 ml) . It is usually administered intravenously, but can also be administered intramuscularly or subcutaneously if necessary.

Nerve pituitary hormone: Also called posterior pituitary hormone, usually injected 5 ~ 10U subcutaneously for 4 ~ 6 hours. Disable pregnant women.

Water AVP: 5 ~ 10 U subcutaneously or intramuscularly, once every 6 ~ 8 hours.

Oil agent tannic acid AVP: also known as diarrhea, one injection of 0.3ml for 36 to 72 hours; 1ml of injection for 5 to 10 days. The starting dose should be small.

2. Non-AVP oral drugs

2.1 Thiazine diuretics: Hydrochlorothiazide, usually 2 to 3 times a day, 25mg each time.

2.2 Carbamazepine: Usually 2 ~ 3 times a day, 0.1 ~ 0.2g each time. ^[6]