What is Moriak syndrome: causes, symptoms, treatment

Secondary diabetic glycogenosis or Moriak syndrome is a complication of insulin-dependent diabetes mellitus in children. How the pathology manifests itself, is diagnosed and treated, you will learn from the article.

Contents:

1. General information.
2. Causes.
3. Pathogenesis.
4. Symptoms.
5. Complications.
6. Diagnosis.
7. Treatment.
8. Prognosis and prevention.

General information

Moriac syndrome is named after the French physician P. Moriac, who first described the symptoms in the middle of the 20th century. The debut of the disease occurs during adolescence and is diagnosed in patients with type 1 diabetes mellitus against the background of delayed puberty. Thanks to the creation of innovative insulin-based medications and methods of their administration, and the education of patients in diabetic schools, Moriak syndrome is diagnosed less and less frequently today. The disease is not gender-specific, endemic, seasonal, or racial.

Causes

Moriak syndrome develops in cases of decompensation of type 1 diabetes mellitus in children.

In this case, the pathology is correlated with improper treatment of diabetes, and the triggers of the disease are:

1. Incorrect insulin dosages.
2. Substandard drugs for the treatment of syndrome or insulin-dependent diabetes.
3. Lack of a responsible attitude towards treatment: infrequent monitoring of blood sugar levels, skipping injections.
4. Lack of skills of administering insulin parenterally, which provokes low efficiency and complications.
5. Ignorance of the equivalent of bread units, non-compliance with the diet.

Pathogenesis

The main trigger of the pathology is a permanent deficiency of insulin, which provokes a disturbance of all metabolic processes, but first of all – of carbohydrate. The blood registers hyperglycemia, but glucose can not penetrate into the cell. To compensate for the lack of sugar, glycogen decomposition increases in cells, the number of intermediate products of lipid metabolism in the blood increases.

Increases the synthesis of cholesterol, lipid fractions, the accumulation of which leads to fatty liver dystrophy with an increase in its size. Chronic insulin deficiency provokes growth of counterinsulin hormones. Adrenal glucocorticosteroids in this case become a trigger of glucogenesis, they synthesize glucose from non-carbohydrate precursors.

As a result of the blockage of the pituitary-adrenal system, growth and puberty of the child are retarded. Cortisol acts catabolic and promotes protein breakdown in tissues. Calcium and phosphorus are washed out of the bones, leading to osteoporosis and muscle atrophy.

Symptoms

Clinically, Moriak syndrome is visualized by a number of signs:

• delayed growth of up to 25 cm compared to normal;
• late bone formation;
• brittle bones;
• cataract;
• retinal abnormalities.

Cushingoid overweight gain is another symptom of the pathology, which is associated with leptin and other adipose tissue hormones, as well as with adrenal gland function. Lipids are localized in the face and neck, the upper third of the torso. At the same time, the arms and legs remain the same. On the child’s body, hair growth intensifies, and hair striae form, and some have folliculitis with itching. Hepatomegaly is typical of Moriak syndrome, in extreme cases, the edge of the organ reaches the navel without impairment of function.

Sexual infantilism with underdeveloped or absent secondary sexual characteristics is formed under the influence of sex hormones. Puberty is delayed, as is menstruation in girls.

Complications

Moriak syndrome itself is a complication that provokes obesity, hypercholesterolemia, heart and vascular pathology, and osteoporosis. In late diagnosis of the disease or ineffective therapy, fatty infiltration and liver fibrosis are formed, metabolism with accumulation of decay products leads to acidosis, ketoacidosis coma up to fatal outcome.

Diagnosis

It is not difficult to make a correct diagnosis of the syndrome based on the history and specific clinic: short stature, obesity, hirsutism, underdevelopment of sexual characteristics, hepatomegaly on palpation.

To confirm the diagnosis, one should:

1. Blood biochemical testing: sugar spikes throughout the day, hyperlipid and hypercholesterolemia.
2. Densitometry – X-ray diagnosis of osteoporosis: minimum bone density and risk of fractures with little traumatic stress.
3. Ultrasound examination of the liver to determine its size, signs of lipid hepatosis, and fibroelastometry to assess the severity of fibrosis.
4. Determination of bone age using hand and wrist X-rays with visualization of ossification nuclei. This is a reliable study to know a person’s bio-age. In Moriak syndrome, it is less than calendar age.
5. Hepatobiopsy – performed when indicated for invasive determination of glycogen and fat burden, differentiation with Hirke’s disease.

Treatment

The goal of therapy is to compensate for the underlying pathology that caused the syndrome. For this purpose, optimal doses of insulin are prescribed, first of all. Basal bolus correction with imitation of natural secretion of the hormone by the pancreas is used. Long-acting insulin replaces its basal secretion, and a short-acting one replaces its food component: it is administered after a meal.

Prolonged preparations guarantee a stable insulin concentration and prevent sudden daily fluctuations in glucose, which minimizes the risk of polyneuropathies and heart and vascular disease. The dosage of short and ultra-short insulin is calculated on the basis of the number of bread units eaten.

Preparations of high purity and quality are used. Also used:

1. Additional correction with medications, which is carried out with hypolipidemic drugs that normalize the level of cholesterol and other blood fats, hepatoprotectors that restore liver cells, B vitamins that improve trophism, steroids that accelerate growth and puberty (they are used in children over 14-15 years).
2. Physiotherapy is represented by massage, inductothermy to restore lipid metabolism and blood flow rate;
   diabetic school is the training of parents and children in self-monitoring and diet therapy.

Prognosis and prevention

With timely and high-quality therapy of the pathology, the prognosis is favorable. Compensation of diabetes occurs when blood glucose is normal without sharp fluctuations, which avoids complications. Prevention is reduced to the monitoring of blood glucose concentrations and adequate therapy of diabetes in children.