DIABETES NOT SUGAR (diabetes insipidus; grech to pass diabetes from diabaino through; synonym mocheiznureniye not sugar) — the clinical syndrome which is characterized by a polyuria and a secondary polydipsia.
Distinguish two forms D. of N — central and peripheral. The central form D. of N is caused by absence or decrease in secretion vasopressin (see) that is caused by hereditary or secondary defeat gipotalamo-pituitary system (see). The peripheral form is connected with hereditary or secondary damage of kidneys and nonsensitivity of an epithelium of renal tubules to vasopressin.
D.'s Frequency of N among other diseases of endocrine system makes 0,7 — 0,8%. Incidence on 10 000 population, according to various researchers, fluctuates from 2,5 to 16.
various destructive patol, the processes which are localized in a hypothalamus and a hypophysis can be the Reason of the central form D. of N: primary or metastatic tumors, inflammatory processes and infectious granulomas (e.g., syphiloma), effects of an injury, radiative effects, vascular defeats, xanthomatosis, lymphogranulomatosis, sarcoidosis, myeloid leukosis. Approximately at a half of patients it is not possible to establish a cause of illness, and such form D. of N call idiopathic or genuinical.
Inflammatory processes in diencephalic area (see. Hypothalamic syndromes ), the causing D. and., can develop as a result of acute respiratory diseases, hron, focal infection, and also at tuberculosis, belly, sypny and a typhinia, scarlet fever, whooping cough, malaria, a brucellosis, a puerperal septicaemia, pneumonia.
At children D.'s reason for N, especially at early age, a thicket defects of embryonic development of a hypothalamus or genetic disorders are; hereditary transfer in such cases goes on dominant type.
Nephrogenic vazopressinrezistentny D. of N — the rare inborn hereditary family disease linked to a floor; perhaps, it is connected with damage of X-chromosome.
Pathogeny and pathological anatomy
Development of the central form D. of N is connected with insufficiency of the vasopressin possessing antidiuretic action. Synthesis and (or) secretion of vasopressin by neurons of a supraoptic (nadzritelny) kernel of a hypothalamus with the subsequent insufficient receipt it in a back share of a hypophysis and from there in blood are most often broken. Also isolated disturbance of transport of vasopressin through a gipotalamo-pituitary path and a back share of a hypophysis is possible. Occasionally D. of N develops as a result of lack of reaction to vasopressin of the canalicular device of a kidney. Nephrogenic vazopressinrezistentiy D. of N is characterized by nonsensitivity of kidneys to effect of vasopressin and impermeability of distal nephron for water. Insufficient products cyclic 3'5'AMF in an epithelium of tubules of distal nephron are biochemical noted.
Of N arises at preservation of activity of the front share of a hypophysis providing sufficient products of cortisol which slows down allocation in blood of vasopressin and reduces sensitivity of kidneys to its action. Emergence polyurias (see) at D. by N it is connected with the insufficiency of vasopressin leading to falloff of a reabsorption of water in distal department of tubules of kidneys owing to reduction of permeability of intercellular substance. Polydipsia (see) develops for the second time as a result of removal of large amounts of water and prevents development of dehydration of an organism.
The central form D. of N results from damage of the supraoptic kernels of a hypothalamus cosecreting vasopressin, a supraoptiko-pituitary path, a back share of a hypophysis. In an experiment it is received destruction or removal of any of these departments. Macroscopically the hypothalamus at primary D. of N is not changed, gistol, changes can also not be, but usually at primary D. dystrophies and destructions (fig.) are exposed to N of a cell of supraoptic kernels, their deformation with destruction of basphilic substance (Nissl's substance) before its total disappearance is observed. Kernels of cells are wrinkled or lizirovana, cells do not contain neurosecretion, i.e. connected with carrier protein of antidiuretic hormone; proliferation of astrocytes in a zone of supraoptic kernels is sometimes observed. In paraventrikulyarny kernels of change are expressed more weakly. In a neurohypophysis cysts can be defined, it does not contain neurosecretion; thus it is about absolute insufficiency of antidiuretic hormone. The adenohypophysis is not changed. From other bodies of changes it is not noted.
Of N can be observed at the relative insufficiency of vasopressin connected with increase in an inactivation of hormone in a liver. In these cases in a liver there is a picture hron, hepatitis, the nature to-rogo, according to Richards and Sloper (M. of Richards, J. Sloper, 1969), has autoimmune character.
Secondary nephrogenic D. of N can develop as a result of intersticial nephrite with fibrosis of a medulla of kidneys, a polycystosis of kidneys or an inborn hydronephrosis. Folkoner (D. Falconer, 1964) with soavt, and Nike (D. Naik, 1970) N at 3 genotypes of mice described hereditary nephrogenic D. It is characterized by an oligosindaktiliya, small kidneys, small nephron and accumulation of proteinaceous masses in an epithelium of distal tubules; the juxtaglomerular index at them is changed. Supraoptic kernels are hypertrophied, the hypertrophy of all departments of a hypophysis is noted. The glomerular zone of bark of Adrenal glands is reduced.
At nephrogenic vazopressinrezistentny D. of N macroscopically kidneys are not changed, a renal pelvis is sometimes expanded. Microscopic changes can also not be. Dilatation of collective tubes and a cyst in marrow is in some cases observed; at the same time supraoptic kernels are usually not changed, seldom hypertrophied; products and secretion of vasopressin are not changed and are even raised.
The clinical picture
the Disease can quickly begin, within several days, or gradually. Main symptoms: painful thirst and allocation of large amounts of urine with low specific weight. The amount of daily urine not less than 3 — 4 l, specific weight does not exceed it 1,005 — 1,008 (at Zimnitsky's test); more than at a half of patients the diuresis fluctuates from 4 to 12 l a day, at individuals — to 40 l. Even at sufficient administration of liquid at many patients the xeroderma and the general weakness is noted. At children bed wetting quite often takes place, physical development sometimes is late.
In the presence of a tumor, a xanthomatosis or another patol, process in diencephalic area D. of N develops against the background of the corresponding symptoms. Renal D. of N is shown on 3 — - m of month of life by a plentiful diuresis, tendency to locks, fervescence. At an otnyatiya of the child from a breast the wedge, manifestations amplify. Thirst can not be.
Complications are possible at restriction of reception of liquid that quickly leads to dehydration with a lose of weight, to emergence of the general weakness, a headache, a hyperthermia; long restriction of drink can cause mental disturbances and a collapse.
Diagnostic value has reduction of a polyuria with increase in specific weight of urine higher than 1,010 at administration of drugs of vasopressin. At a renal form D. of N at children administration of vasopressin does not reduce a polyuria. However characteristic symptoms, a sharp aggravation of symptoms of the child at deprivation of its liquid allow to differentiate forms D. of N. Differential diagnosis is carried out with the diseases proceeding with a polyuria. The diabetes mellitus is differentiated on the basis of a hyperglycemia, a glycosuria, the high specific weight of urine (see. diabetes mellitus ); at hron, insufficiency of kidneys in urine find protein and cylinders; hyperparathyreosis (see) and primary aldosteronism (see. Aldosteroma ) differ thanks to characteristic a wedge, a picture (at the first disease there is a hypercalcemia, at the second — a hypopotassemia). Differential diagnosis with a psychogenic polydipsia is difficult, at a cut thirst develops initially, and a polyuria — for the second time; in these cases use test with xerophagia or intravenous administration of 50 ml of 2,5% of solution of sodium chloride within 45 min. In the presence of a psychogenic polyuria during conducting test release of urine decreases, its specific weight (more than 1,020) while at sick D. the N of such phenomena is not observed increases. Considering a possibility of dehydration, these tests are carried out only in the conditions of a hospital. Diagnostic criterion is test with inhalation Adiurecrinum (see); at the same time at patients with the central form D. of N the amount of urine decreases, its specific weight increases to 1,010 — 1,015, and at a renal form reaction to Adiurecrinum is absent.
At distinguished patol, the process which led N to D. is carried out etiol, treatment: at a tumor — its removal or radiation therapy, at a hypothalamic syndrome — the corresponding treatment; recovery after specific therapy at D. is described in N of a syphilitic, tubercular and malarial etiology. Recovery at a hypothalamic syndrome with D. is described the N called by an acute respiratory infection after use of antibiotics and intravenous administration of hexamethylenetetramine: penicillin of 6 000 000 — 10 000 000 PIECES and intravenous administration of 40% of solution of hexamethylenetetramine on 10,0 ml within 6 — 10 days.
At an idiopathic form or lack of effect of antiinflammatory therapy appoint drugs of vasopressin. Adiurecrinum is inhaled in Nov on 0,03 — 0,05 g by 2 — 4 times a day, at inflammatory process in a nasal cavity appoint injections of Pituitrinum under skin or in muscles on 0,5 — 1,0 ml (5 — 10 PIECES) each 8 — 12 hour. Apply also oil suspension vasopressin-tannate on 0,2 — 0,6 ml (1 — 3 PIECE) in muscles once in 1 — 3 day. The effect is reached in 1 — 2 week then the dose is reduced. Saluretics (paradoxical action) can render moderate symptomatic effect; hypothiazid is appointed on 50 — 100 mg a day, at long therapy purpose of salts of potassium is necessary. The mechanism of action of saluretics is not clear. At a part of patients use of Chlorproramidum in a dose 0,125 — 0,5, occasionally 0,75 g a day is effective. Introduction of Chlorproramidum reduces a reabsorption of water in tubules of kidneys. Believe that its action is connected with strengthening of activity of small amounts of vasopressin. At use of Chlorproramidum the hypoglycemia is possible. Purpose of Chlorproramidum at a combination is the most reasonable not sugar and a diabetes mellitus.
To children Adiurecrinum and Pituitrinum dose depending on age. Aged from 3 up to 7 years Adiurecrinum is appointed on 0,01 — 0,02 g, from 7 to 12 years — 0,02 — 0,03 g, 12 years — 0,03 — 0,04 g are more senior (2 — 3 times per day to inhale in Nov). To children up to 3 years appoint the adiurekrinovy ointment containing 100 PIECES in 1 g (on 0,1 — 0,2 g 2 — 3 times a day in a nasal cavity). Pituitrinum for children is used (containing 5 PIECES in 1 ml) aged till 1 year — on 0,1 — 0,15 ml, 2 — 5 laid down — on 0,2 — 0,4 ml, 6 — 12 years — on 0,4 — 0,6 ml (1 — 2 time a day under skin or in muscles). Pathogenetic treatment of a renal form D. of N is not developed; by means of sulfanamide diuretic drugs it is possible to lower a diuresis considerably; for the warning of dehydration to children allow to drink liquids without restriction.
the Forecast depends on the reason which caused D. of N and a basic disease. At D. of N of an infectious etiology recovery is possible. Can lead a successful oncotomy to recovery. Quite often the disease proceeds all life, but at replacement hormonal therapy preservation of working capacity is possible. The forecast for life at a renal form at children is doubtful.
Prevention it is not developed. Patients need to create the guarding mode.
Bibliography: Atabek A. A. Not diabetes mellitus, M., 1951, bibliogr.; Zhukovsky M. A. Children's endocrinology, M., 1971; The Multivolume guide to internal diseases, under the editorship of E. M. Tareeva, t. 7, page 399, L., 1966, bibliogr.; The guide to clinical endocrinology, under the editorship of B. G. Baranov, page 209, L., 19 77; The Guide to endocrinology, under the editorship of B. V. Alyoshin, etc., page 88, M., 1973; Green J. R. and. lake of Hereditary and idiopathic types of diabetes insipidus, Brain, y. 90, p. 707, 1967; Labhart A. Klinik der inneren Sekretion, B. — N. Y., 1971; RichardsM.A.a. SloperJ.G. Diabetes insipidus — the complexity of the syndrome, Acta endocr. (Kbh.), v. 62, p. 627, 1969.
Century of P. Klyachko; H. K. Bogdanovich (stalemate. An.).