Moriak'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 a doctor of French origin Pierre Moriac. He described a peculiar clinical picture, in which children suffering from diabetes mellitus and undergoing insulin therapy of the wrong dosage exhibit certain external signs. He noted that all children externally have similarities, which are manifested in low growth, obesity, and lag in sexual development.
Causes of the disease
The main cause of serious complications is the incorrect therapy for diabetes. With this disease, the pancreas does not work properly without producing enough insulin. Due to the 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 Moriak's syndrome in type 1 diabetes is associated with inadequate therapy. An ill dose of insulin was given to a sick child for a long time, or poor quality and poorly purified preparations were used, which led to chronic insulin deficiency.
Long-term lack of insulin in the body leads to the following processes:
- Metabolic disorders, in particular, metabolic disorders of the carbohydrate.
- An increase in the size of the liver and its fatty degeneration due to increased breakdown of glycogen.
- Change in blood composition - an increase in blood glucose, cholesterol, fatty acids.
At the
same time, this is far from all the pathological processes that occur with the body with Moriak's syndrome, and the following deviations are also observed in sick children:
- Insufficient production of important hormones - cortisol, growth hormone, glucagon and, as a result, a violation of growth processes.
- The breakdown of proteins and the abundant withdrawal of calcium and phosphorus from bones, ultimately leads 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 years, but dysfunctional processes begin much earlier. Currently, modern and carefully purified preparations are used to treat early diabetes, which virtually exclude the development of Moriak's syndrome.
Symptoms of the disease
Moriak's syndrome in diabetes has a number of characteristic manifestations:
- The child has a developmental delay and growth inhibition. Obesity and short stature 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).
- Protracted puberty.
- Obesity, especially in the face and upper body with thin limbs. Sick children are very similar to each other, have a “moon-shaped" face, a short neck, fat deposits in the arms, shoulders, and abdomen. In this case, the lower body remains very thin.
- Enlarged liver, accompanied by roundabout venous circulation.
- The development of osteoporosis (slowing down the development of bone tissue).
- Eye diseases, including retinal diseases, and subsequently the development of cataracts.
Most often, Moriak syndrome is found in children, a photo of a sick child clearly shows what external manifestations this disease has.
Diagnosis of the disease
The diagnosis of Moriak’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, and a clear enlargement of the liver.
To confirm the diagnosis, a blood test is performed, as a result of which the following manifestations can be distinguished:
- The glucose level is unstable, it manifests itself in constant jumps, either up or down.
- Excessive blood lipids (hyperlipemia).
- A significant increase in blood cholesterol (hypercholesterolemia).
In some cases, the liver is additionally studied, for this a biopsy is performed to reveal the fat load.
Course of the disease
For a young body, the disease is extremely difficult. Being a severe form of diabetes, it is difficult to compensate for. Often it causes acidosis and hyperglycemic coma.
Moriak's syndrome occurs in severe form due to the frequent accession of various infectious diseases, as well as the complex and long-term restoration of biochemical processes in the human body.
Moriak and Nobekur syndrome: similarities and differences
Moriak and Nobekura syndrome often develops in childhood, both diseases are a serious complication of diabetes mellitus, which occurs against the background of improper treatment. Both syndromes have similar manifestations, they include lag in growth and sexual development, prolonged puberty, fatty degeneration of the liver. In this case, the main difference between Nobekur syndrome is the lack of excess subcutaneous fat. With this treatment of both syndromes, it aims to compensate for diabetes.
Treatment for Moriak's Syndrome
Direct treatment of the syndrome itself as such does not exist. All therapy is aimed at eliminating the root cause of the disease and the complications it causes. 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 performed, for this, massage, physiotherapeutic procedures are prescribed and a scheme for insulin injections is drawn up. In addition to treatment, a special diet can be prescribed that excludes all foods containing animal fats from the diet. Moreover, the diet focuses on protein and carbohydrate products.
Therapeutic therapy is also designed to compensate for the complications developed against the background of the underlying disease, for this the patient is prescribed:
- Reception of hepatoprotectors in order to restore liver cells.
- B vitamins to restore normal metabolism.
- A complex of drugs aimed at normalizing blood lipids and cholesterol.
- Steroid drugs to provoke the growth of the body.
- Taking hormonal drugs to restore important sexual functions.
Prevention and prognosis
To prevent the development of such a dangerous complication of diabetes mellitus as Moriak’s syndrome, it is necessary to carry out the correct treatment of the underlying disease and prevent insulin deficiency.
With the complex treatment of the already developed syndrome with modern drugs, the prognosis is quite favorable, but subject to the fulfillment of all the doctor's recommendations. The correct dosage of insulin and restoration of the functionality of all affected organs can restore the child’s health, both external and internal.