Mauriac's syndrome in diabetes mellitus

Mauriac's syndrome is a disease that develops as a complication as a result of improper treatment of diabetes at a young age. For the first time the disease was described in 1930 by a doctor of French origin Pierre Mauriac. He described a peculiar clinical picture in which certain external signs appear in children suffering from diabetes mellitus and having undergone insulin therapy of the wrong dosage. He noticed that all children outwardly have a similarity, which manifests itself in low growth, obesity, lag in sexual development.

Causes of the disease

The main cause of severe complications is the incorrect treatment of diabetes mellitus. With this disease, the pancreas does not work properly, without producing enough insulin. Due to pathology, there is a deficiency of glucose in the cells due to the fact that it is concentrated in large quantities in the blood.

The development of Mauriac syndrome in type 1 diabetes is associated with inadequate therapy. A sick child was prescribed an insufficient dose of insulin for a long time, or low quality and poorly purified preparations were used, which led to a chronic deficiency of insulin.

A prolonged lack of insulin in the body leads to the following processes:

• Metabolic disorders, in particular, a disorder of carbohydrate metabolism.
• Increase in liver size and fatty degeneration due to increased disintegration of glycogen.
• Changes in blood composition - an increase in glucose in the blood, cholesterol, fatty acids.

However, this is not all pathological processes that occur with the body in the syndrome of Moriak, and the following deviations are observed in patients with children:

• Insufficient production of important hormones - cortisol, somatotropin, glucagon and, as a consequence, disruption of growth processes.
• The breakdown of proteins and the abundant release of calcium and phosphorus from the bones, eventually leads to the development of osteoporosis and atrophy of certain muscles.
• Inability to absorb vitamins in the intestine.

The disease most often manifests itself at the age of 15-18 years, but dysfunctional processes begin much earlier. Currently, for the treatment of early diabetes, modern and thoroughly purified medications are used that practically exclude the development of the Moriak syndrome.

Symptomatic of the disease

The syndrome of Moriak in diabetes mellitus has a number of characteristic manifestations:

• The child has a lag in development and inhibition of growth. Obesity and low growth are often observed. In this case, a sick child may lag behind peers in growth by 10-30 centimeters.
• Lag in sexual development (underdeveloped sexual characteristics and lack of menstruation in girls).
• Prolonged pubertal period.
• Obesity, especially in the face and upper half of the trunk with thin extremities. Sick children are very similar to each other, have a "moon-shaped" face, short neck, fat deposits in the area of the hands, shoulders, abdomen. The lower part of the body remains very thin.
• Enlargement of the liver, accompanied by roundabout venous circulation.
• Development of osteoporosis (slowing the development of bone tissue).
• Diseases of the eye, including retinal diseases, and subsequently the development of cataracts.

The most common occurrence of Moriak's syndrome in children, a photo of a sick child clearly shows what external manifestations this disease has.

Diagnosis of the disease

The diagnosis of "Mauriac's syndrome" is made with obvious external signs of the development of the disease, such as: insufficient growth for one's age, the presence of obesity, especially in the face, sexual immaturity, an obvious increase in the liver.

To confirm the diagnosis, a blood test is performed, as a result of which the following manifestations can be distinguished:

• The level of glucose is unstable, manifested by constant jumps, then to a large, then to a smaller side.
• Excessive lipid levels in the blood (hyperlipemia).
• Significant increase in the level of cholesterol in the blood (hypercholesterolemia).

In some cases, the liver is additionally examined, for this purpose a biopsy is performed to identify the fat load

Course of the disease

For a young organism, the disease is extremely difficult. Being a severe form of diabetes, it is difficult to compensate. Often becomes the cause of acidosis and hyperglycaemic coma.

Moriak's syndrome occurs in severe form due to the frequent attachment of various infectious diseases, as well as the complex and prolonged recovery of biochemical processes in the human body.

The syndrome of Moriak and Nobukura: similarities and differences

The syndrome of Moriak and Nobelcur often develops in childhood, both diseases are a serious complication of diabetes, which occurs against the background of improper treatment. Both syndromes have similar manifestations, including a lag in growth and sexual development, a protracted pubertal period, fatty liver dystrophy. At the same time, the main difference of the Nobelcour syndrome is the absence of excess subcutaneous fat. In this treatment of both syndromes is aimed at compensated treatment of diabetes mellitus.

Treatment of the Mauriac Syndrome

Direct treatment of the syndrome as such does not exist. All therapy is aimed at eliminating the root cause of the disease and the complications caused by it. To do this, the patient is prescribed correct insulin therapy in the correct dosage and quality modern drugs.

To prevent lipodystrophy, a number of preventive procedures are carried out, for this purpose massage, physiotherapeutic procedures are prescribed and a scheme for injections with insulin is made. In addition to treatment, a special diet can be prescribed that excludes from the diet all foods containing animal fats. In this diet, the emphasis is on protein and carbohydrate foods.

Therapeutic therapy is also designed to compensate for the complications that develop on the background of the underlying disease, for this the patient is appointed:

• Reception of hepatoprotectors in order to restore liver cells.
• B group vitamins to restore normal metabolism.
• A complex of drugs aimed at normalizing the level of lipids and cholesterol in the blood.
• Steroid drugs to provoke body growth.
• Admission of hormonal drugs to restore important sexual functions.

Prophylaxis and prognosis

To prevent the development of such a dangerous complication of diabetes mellitus, like Moriak's syndrome, it is necessary to conduct correct treatment of the underlying disease and not to allow insulin deficiency.

With the comprehensive treatment of the already developed syndrome with modern drugs, the prognosis is quite favorable, but provided all the recommendations of the doctor are fulfilled. Proper dosage of insulin and restoration of the functionality of all affected organs can restore health to the child, both external and internal.