Mauriac's syndrome is a disease that develops as a complication as a result of improper treatment of diabetes at a young age. The disease was first described in 1930 by French-born physician Pierre Mauriac. He described a peculiar clinical picture in which certain external signs appear in children with diabetes mellitus who have undergone insulin therapy with the wrong dosage. He noticed that all children outwardly have a similarity, which manifests itself in short stature, obesity, lag in sexual development.
Causes of disease development
The main cause of severe complications is the incorrect treatment of diabetes mellitus. In this disease, the pancreas does not work properly, not producing enough insulin. Due to the pathologydeficiency of glucose in cells due to the fact that it is concentrated in large quantities in the blood.
The development of Mauriac's syndrome in type 1 diabetes mellitus is associated with inadequate therapy. A sick child was given an insufficient dose of insulin for a long time, or low-quality and poorly purified preparations were used, which led to chronic insulin deficiency.
Prolonged lack of insulin in the body leads to the following processes:
- Metabolic disorders, in particular, a disorder of carbohydrate metabolism.
- Increase in the size of the liver and its fatty degeneration due to increased breakdown of glycogen.
- Changes in blood composition - an increase in blood glucose, cholesterol, fatty acids.
At the same time, these are far from all the pathological processes that occur with the body in Mauriac's syndrome, and the following deviations are also observed in sick children:
- Insufficient production of important hormones - cortisol, somatotropin, glucagon and, as a result, disruption of growth processes.
- The breakdown of proteins and the abundant removal of calcium and phosphorus from the bones, eventually leading to the development of osteoporosis and atrophy of some muscles.
- Inability to absorb vitamins in the intestines.
The disease most often manifests itself at the age of 15-18, but dysfunctional processes begin much earlier. Currently, modern and carefully purified drugs are used to treat early diabetes.which practically exclude the development of Mauriac's syndrome.
Symptoms of the disease
Mauriac's syndrome in diabetes mellitus has a number of characteristic manifestations:
- The child has a developmental delay and growth inhibition. Obesity and short stature are often observed. At the same time, a sick child may lag behind peers in growth by 10-30 centimeters.
- Sexual retardation (underdeveloped sexual characteristics and lack of menstruation in girls).
- Protracted puberty.
- Obesity, especially in the face and upper torso with thin limbs. Sick children are very similar to each other, they have a "moon-shaped" face, a short neck, fat deposits in the arms, shoulders, and abdomen. At the same time, the lower part of the body remains very thin.
- Liver enlargement with roundabout venous circulation.
- Development of osteoporosis (delayed bone development).
- Eye diseases, including diseases of the retina, and subsequently the development of cataracts.
Moriac's syndrome is most common in children, a photo of a sick child clearly shows what external manifestations this disease has.
Diagnosis of 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, a clear enlargement of the liver.
To confirm the diagnosis, a blood test is performed, inas a result of which the following manifestations can be distinguished:
- Glucose level is unstable, manifested by constant jumps up and down.
- Excessive blood lipids (hyperlipemia).
- Significant increase in blood cholesterol levels (hypercholesterolemia).
In some cases, the liver is additionally studied, for this a biopsy is performed to reveal the fat load
Course of illness
For a young organism, the disease is extremely difficult. Being a severe form of diabetes mellitus, it is difficult to compensate. Often causes acidosis and hyperglycemic coma.
Mauriac's syndrome occurs in severe form due to the frequent addition of various infectious diseases, as well as the complex and lengthy recovery of biochemical processes in the human body.
Mauriac and Nobecourt syndrome: similarities and differences
The syndrome of Mauriac and Nobekur often develops in childhood, both diseases are a severe complication of diabetes mellitus, which occurs against the background of improper treatment. Both syndromes have similar manifestations, they include growth retardation and sexual development, prolonged puberty, fatty degeneration of the liver. At the same time, the main difference between Nobekur's syndrome is the absence of excess subcutaneous fat. In this case, the treatment of both syndromes is aimed at compensated treatment of diabetes mellitus.
Treatment of Mauriac's Syndrome
Direct treatment of thethere is no such thing as a syndrome. All therapy is aimed at eliminating the root cause of the disease and the complications caused by it. For this, the patient is prescribed the correct insulin therapy in the correct dosage and high-quality modern drugs.
To prevent lipodystrophy, a number of preventive procedures are carried out, for this purpose massage, physiotherapy procedures are prescribed and a scheme for insulin injections is drawn up. In addition to treatment, a special diet can be prescribed, excluding from the diet all products containing animal fats. At the same time, the diet focuses on protein and carbohydrate foods.
Therapeutic therapy is also designed to compensate for the complications that have developed against the background of the underlying disease, for this the patient will be prescribed:
- Reception of hepatoprotectors in order to restore liver cells.
- B vitamins to restore normal metabolism.
- A complex of drugs aimed at normalizing the level of lipids and cholesterol in the blood.
- Steroid drugs to induce body growth.
- Taking hormonal drugs to restore important sexual functions.
Prevention and prognosis
To prevent the development of such a dangerous complication of diabetes as Mauriac's syndrome, it is necessary to carry out the correct treatment of the underlying disease and prevent insulin deficiency.
In the complex treatment of an already developed syndrome with modern drugs, the prognosis is quitefavorable, but subject to the implementation of all doctor's recommendations. The correct dosage of insulin and the restoration of the functionality of all affected organs can restore the child to he alth, both external and internal.
