Hydropexic syndrome is a pathological condition characterized by a violation of the hypothalamus and pituitary gland and, as a result, increased vasopressin production. The disease is manifested by the appearance of edema, decreased diuresis, and an increase in body weight. Fluid retention is accompanied by headache, dryness and pallor of the skin. Diagnostics includes physical examination, determination of the density and osmolarity of urine, the level of electrolytes and aldosterone in the blood, a sample with a water load. Treatment involves strict restriction of fluid and salt intake, the appointment of diuretics, potassium and magnesium preparations.
Meaning
Hydropexic syndrome (Parkhon’s syndrome, primary adiuretism) is a rare endocrine disease in which fluid retention occurs in the body due to elevated levels of antidiuretic hormone (ADH). This pathological condition was first described in the middle of the twentieth century by the Romanian scientist Konstantin Parhon, and subsequently it was studied in detail by American endocrinologists Benjamin Schwartz and Frederick Butter. The disease most often affects women aged 30 to 50 years. Primary adiuretism is practically not found among children and adolescents.
Causes
The main cause of the disease is an increased release of vasopressin, which may be due to a number of factors:
- Violation of the hypothalamus function. Meningitis, encephalitis, influenza, subarachnoid hemorrhages, hematomas, injuries involving the hypothalamus in the pathological process can become a trigger mechanism in the development of the disease. Women have known cases of Parkhon syndrome during pregnancy and childbirth, as well as after extirpation of the uterus with appendages.
- Benign and malignant neoplasms. Pancreatic tumor, lymphosarcoma, thymoma, bronchogenic cancer can independently produce vasopressin.
- Endocrine disorders. Decreased thyroid function, insufficiency of adrenal cortex hormones, inhibition of the endocrine function of the adenohypophysis affect the production of vasopressin.
- Taking medications. Systematic use of certain medications (neuroleptics, tranquilizers) it can cause an increase in the level of ADH.
- Mental disorders. Psychosis, emotional trauma, depression, chronic fatigue and constant stress affect the endocrine function of the hypothalamus.
- Other reasons. There are cases of pathology in tuberculosis, sarcoidosis, chronic renal failure.
Pathogenesis
The pathogenesis of the hydropexic syndrome depends on the causes of the disease. The main link in all cases is a violation in the work of the hypothalamus and increased secretion of the hormone vasopressin. Antidiuretic hormone is involved in the regulation of osmotic pressure in the body. Increased production of ADH and an increase in osmotic pressure lead to reabsorption of water by the tubules of the kidneys and its retention in the body. This causes a decrease in diuresis and a decrease in the concentration of sodium ions in the blood plasma. These disorders lead to excessive release of aldosterone and the development of electrolyte disorders. The production of ADH by tumors also leads to an increase in its level in the blood and shifts in the homeostasis system.
Symptoms
The disease is manifested by the development of moderate edema (face, trunk, limbs), an increase in body weight. Water retention causes intoxication, accompanied by dizziness, headache, insomnia, weakness, periodic nausea and vomiting. A decrease in the amount of daily urine (oliguria) is replaced by attacks of polyuria. Loose stools may be observed. In women, menstrual function is disrupted up to amenorrhea. Men have a decrease in libido and the development of impotence. Externally, patients look swollen, the face is puffy, the skin is dry, pale with a bluish tinge.
Complications
An increase in the production of antidiuretic hormone and a decrease in the amount of sodium lead to a decrease in body temperature, slowing down the conduction of the heart, mental disorders (psychoses, confusion, disorientation) and the development of seizures. The progression of the disease and a decrease in sodium below 110-115 mmol / l cause the development of cerebral edema, a violation of cardiac activity, which entails the onset of coma and death. Water retention in the body and the occurrence of edematous syndrome provoke the development of acute heart failure, pulmonary edema. Weight gain due to excessive accumulation of fluid causes joint damage, the development of arthritis, protrusions and hernias of the spine.
Diagnostics
The diagnosis of hydropexic syndrome is carried out on the basis of complaints, anamnesis of life and disease, laboratory analysis data. Patients have a puffy appearance, the skin is pale in color. Patients complain of periodic headaches and a decrease in the amount of urine (less than 500-600 ml / day). The blood test shows a reduced amount of sodium ions, plasma osmolarity is reduced. In the general analysis of urine, hypernatremia, hyperchloremia, increased density and osmolarity are detected.
In some cases, a test with a water load is performed (the patient drinks about 1.5 liters of water in 30 minutes), while there is a delay in urination of more than 4-5 hours. In the McClure-Aldrich test, the subcutaneously injected fluid resolves with a delay. The absence of a decrease in urine density and characteristic urination disorders indicate inadequate production of ADH. Additional studies include tomography of the hypothalamus and pituitary gland, assessment of hormonal status, lung radiography, ECG, abdominal ultrasound and adrenal glands.
Differential diagnosis of Parkhon syndrome is carried out with idiopathic edema, which is characterized by increased thirst, pronounced swelling of tissues and adequate urine density. Hypofunction of the thyroid gland is manifested by an increased content of TSH, a decrease in T4 levels, the development of constipation, drowsiness and apathy. With hyperaldosteronism, blood pressure indicators increase, the content of renin in plasma decreases. To exclude this pathology, tests with hypothyazid and spironolactone are performed.
Treatment
To reduce swelling for a long time or for life, potassium-sparing diuretics are prescribed. Thiazide and loop diuretics are periodically used. In severe cases, ADH antagonists (oxytocin, glucocorticoids) are used. Additionally, metabolic therapy is carried out, potassium and magnesium preparations are used. Seizures are stopped by the introduction of benzodiazepines, sodium chloride, osmotic laxative (sorbitol). With the development of cerebral edema, intravenous infusion of mannitol, a hypertonic solution of sodium chloride is carried out.
All patients are prescribed a salt-free low-calorie diet with a reduced content of carbohydrates and fats. It is recommended to reduce fluid intake to 0.5-1 liters per day, including soups, compotes and tea. Fresh vegetables, unsweetened fruits, low-fat meats are introduced into the diet. Patients with excess weight are shown physical therapy, swimming, aerobics, spa treatment. Good results were noted during X-ray therapy of the pituitary region. In the presence of tumors, etiotropic treatment is carried out (removal of neoplasms, radiation therapy, chemotherapy).
Prognosis and prevention
The prognosis for vital activity and working capacity in hydropexic syndrome depends on the underlying disease and the neglect of the pathological process. With timely diagnosis and adequate therapy, it is possible to reduce the severity of edema, normalize diuresis. Severe forms of the disease, poorly amenable to etiotropic and symptomatic treatment, lead to gradual disability. Prevention of complications includes compliance with the drinking regime, restriction of salt intake, the use of potassium preparations and diuretics, treatment of the underlying pathology.