Nephrogenic diabetes insipidus

Introduction Diabetes insipidus refers to the discharge of a large amount of diluted body fluid from the body. This state of excessive water intake and hypotonic polyuria may be caused by the release of antidiuretic hormone (ADH) due to normal physiological stimulation (central or nerve). Sexually, or the kidneys do not respond to diuretic hormones, ie, renal diabetes insipidus. Renal diabetes collapse refers to the latter. That is, in the case where the plasma ADH is normal or increased, the kidney cannot concentrate the urine and continuously discharges the pathological state of the diluted urine. Diabetes insipidus refers to the pathological state in which the kidney can not concentrate urine and continuously discharge a large amount of diluted urine in the case of normal or elevated plasma ADH. It is a rare renal tubular dysfunction disease. This state of excessive water intake and hypotonic polyuria may be due to normal physiological stimulation, which may cause the release of antidiuretic hormone. Nephrogenic diabetes insipidus is a disease of renal tubular hydration dysfunction. , manifested as polyuria, polydipsia, and persistent hypotension. It can be hereditary and secondary hereditary as a hereditary tubular disease. It is also called hereditary or primary anti-pituitary vasospasm. Also known as familial renal diabetes insipidus. Secondary can occur in a variety of chronic kidney diseases (such as obstructive nephropathy, interstitial nephritis, chronic pyelonephritis hypercalcemia, potassium-deficient nephropathy, renal tuberculosis, renal medullary cystic disease, etc.), multiple myeloma kidney starch Acquired drug damage (such as demecycline, methoxyflurane vincristine) and other acquired patients due to the anti-ADH effect of kidney and extra-renal disease and / or destruction of the hyperosmolar state of the renal medullary fluid makes the urine Liquid concentration is affected by certain factors, so it is also called secondary or incomplete anti-ADH diabetes insipidus.

Cause

1. Primary congenital and familial.

2. Secondary

(1) A variety of chronic kidney diseases (polycystic kidney disease, medullary cystic disease, chronic interstitial disease, severe renal failure).

(2) Obstructive uropathy, after the obstruction is relieved.

(3) unilateral renal artery stenosis.

(4) After kidney transplantation.

(5) Acute tubular necrosis.

(6) Low potassium (including primary aldosteronism).

(7) Chronic hypercalcemia (including hyperparathyroidism).

(8) Drugs (lithium, methoxyflurane, dexamethasone, rhizome, amphotericin B, gentamicin, etc.).

(9) Systemic diseases (multiple myeloma, amyloidosis, Sjogren's syndrome, etc.).

an examination

Related inspection

Urine routine water vasopressin test

Typical case

(1) According to clinical manifestations.

(2) Laboratory examination.

(3) Positive family history, generally can be diagnosed.

2. Atypical cases

(1) Young JEan has repeated dehydration, polydipsia, and vomiting.

(2) fever, convulsions and developmental disorders.

(3) Especially in the case of water loss, the urine is still low-tension urine, which has certain value for the diagnosis.

Differential diagnosis

Renal diabetes insipidus needs to be differentiated from pituitary diabetes insipidus with neurological polydipsia and diabetes.

1. Pituitary diabetes insipidus: This disease is caused by the lack of antidiuretic hormone, low blood ADH level and low urine cAMP. After the injection of antidiuretic hormone, the symptoms of polydipsia are obviously improved, urine cAMP is increased, and urine osmotic pressure is increased. Pituitary diabetes insipidus is more common than adolescent disease. Sudden onset of sudden onset of urine and polydipsia may have signs of hypothalamic-neuronal pituitary damage.

2. Psychotic polydipsia, polyuria: neurological polydipsia, polyuria often occur in adult women often have a history of trauma, first polydipsia after polydipsia and polydipsia, and is closely related to mental factors It responds to the vasopressin test, and the rapid osmolality of the hypertonic saline test results in a slight decrease in urine volume, which can be naturally reduced without drinking water at night.

3. Diabetes: There may also be polydipsia, but elevated blood sugar and abnormal glucose tolerance can be differentiated from this disease.

For polydipsia, pituitary diabetes insipidus or renal diabetes insipidus or neuropathic polydipsia can be identified by measuring the urine osmotic pressure after water inhalation or injection of vasopressin. The above test results showed that there was no significant change in urine osmotic pressure after normal blood test, patients with neurological polydipsia, polyuria and renal diabetes insipidus and after vasopressin injection, and urine perfusion of two test results in patients with pituitary diabetes insipidus The pressure has a significant change and the latter is significantly larger than the former. Renal diabetes insipidus and neurogenic polydipsia have significantly different urine osmotic pressure after water insufficiency. The former is lower than the latter.

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