MAURIAC SYNDROME (P. Mauriac, the fr. doctor, 1882 — 1963) — the heavy complication of a diabetes mellitus at children, to-rogo is the cornerstone a lack of insulin and nek-ry tropny hormones of a hypophysis, characterized by a growth inhibition and sexual development, increase in a liver. Mauriac in 1930 for the first time described peculiar a wedge, the picture observed at children in several years after emergence of a diabetes mellitus (see. a diabetes mellitus, at children ).
The etiology and a pathogeny
M. of page develops at the wrong treatment of a diabetes mellitus insulin (see) or absence and non-compliance with the mode of clinical nutrition. Insufficient doses of insulin, purpose of a type of the insulin which is not corresponding to features of a glycemic profile matter; administration of simple insulin only in the morning that does not provide compensation of metabolic disturbances in evening and night hours. At a lack of insulin and connected with this disturbance of use by fabrics of glucose products of kontrinsulyarny hormones raise, first of all cortisone (see), glucagon (see), somatotropic hormone (see), catecholamines (see). At the same time processes of a catabolism of proteins, a gluconeogenesis, lipolysis amplify; content in blood of beta lipoproteids and free fat to - t increases. Increase in a liver is connected with adjournment in it of a glycogen and neutral fats.
The clinical picture
Develops obesity (see) with characteristic redistribution of fat (fatty deposits on a trunk at thin extremities), osteoporosis (see), lag in growth, a delay of sexual development. Early symptoms of M. of page are shown in a delay of physical and sexual development, emergence of fatty deposits on a trunk and cheeks, increase in a liver; further manifestations become heavier: borders of a liver can extend up to the level of a navel, a liver at a palpation dense, fatty deposits increase by a trunk; a growth inhibition and sexual development become more expressed, up to development infantility (see).
M.'s Diagnosis of page at a diabetes mellitus against the background of absence or incorrectly carried out insulin therapy, improper feeding and a decompensation of metabolism does not present difficulty. Also correct assessment a lab is of great importance. analyses: at M. villages usually note high figures of a sugar content in blood and urine, increase in content in blood of ketone bodies, cholesterol, decrease in content of albumine, increase ah - and az globulins. M.C. needs to be differentiated with glycogenoses (see), including with a disease to Awnless wheat.
the Main attention should be concentrated on achievement of permanent compensation of a diabetes mellitus. Appoint fiziol, the diet adequate to age and weight of the child, with precisely established maintenance of products, carbohydrate-rich, an exception of easily assimilable carbohydrates and restriction of fats. Insulins therapy) carry out by drugs of the prolonged action and — if necessary — their combination with simple insulin or moderately prolonged its drugs according to feature of a glycemic and glikozurichesky profile in the doses providing compensation of disturbances of metabolism. Appoint vitamins of group B, anabolic steroids (Nerobolum, retabolil), physical therapy (an inductothermy to area of a liver), massage of the lower and upper extremities, remedial gymnastics. After 14 — 15 years at a delay of sexual development it is possible to appoint a chorionic gonadotrophin.
The forecast and Prevention
the Forecast at timely begun treatment can be favorable. The duration of a disease of a diabetes mellitus is more and the more its manifestations are expressed, the worse than M. the page will respond to treatment. In the started cases the forecast adverse.
Prevention consists in timely transition to treatment by insulin with the prolonged action that promotes achievement of the fullest and permanent compensation of diabetes. Due to broad implementation in modern medical practice of the prolonged types of insulin M. of page meets more and more seldom.
Bibliography: Zhukovsky M. A. Children's endocrinology, page 234, M., 1971; Knyazevskaya Ie. G. Sindr Moriaca v of children and its treatment, Pediatrics, No. 3, page 38, 1972; Martynova M. I., Lapchenko L. N. and Luzyanin G. A. Sindr of physical and sexual infantility at severe forms of a diabetes mellitus at children, Vopr. okhr. mat. also it is put., t. 21, No. 8, page 3, 1976; To u p e t z G. W. u. A u e r s w a 1 d W. Beobachtung zum Mauriac-Syndrom, Dtsch. Gesundh. - Wes., Bd 27, S. 535, 1972; M an u r i and with P. Recherches sur ie pouvoir glycolytique du sang in vitro chez les diabetiques et les can-syogey, Page R. Soc. Biol. (Paris), v. 105, p. 28, 1930; Windorf er A. Das Syndrom Mauriac (Diabetes im Kindersalter mit se-kundarer Glykogenose), Ergebn. inn. Med. Kinderheilk., Bd 4, S. 392, 1953.
M. A. Zhukovsky.