Limfatiko-gipoplastichesky diathesis – anomaly of the constitution which is shown by limfoproliferativny processes and a hypoplasia of endocrine and cardiovascular systems. The main symptoms – a limfatizm and violation of immunity, violation of water-salt exchange, cardiovascular insufficiency. It is diagnosed on the basis of the anamnesis, including family, and on clinical signs. Is confirmed biochemical and radiological (the roentgenogram of a thymus). Treatment is complex, it is directed to maintenance of immunity and ensuring normal physical and psychological development of the child.
Limfatiko-gipoplastichesky diathesis, in fact, is not a separate nosological form, and represents a combination of various pathologies, each of which can be expressed to a greater or lesser extent. The term "diathesis" means hereditary predisposition, and it reflects at the same time versatility of clinical manifestations and a lack of data on the nature of this state. The concept "limfatiko-hypoplastic diathesis" is entered into pediatrics in 1889-1890 by A. Paltauf and T. Esherikh. This anomaly of the constitution usually demonstrates clinically at children of 2-7 years, boys and girls equally often suffer. High relevance of limfatiko-hypoplastic diathesis is caused it not until the end of the studied nature and heavy complications in the form of a timomegaliya and sharp nadpochechnikovy insufficiency which can lead to sudden children's death.
Reasons of limfatiko-hypoplastic diathesis
The etiology remains until the end of not studied now. Influence of pre-natal factors is proved: infections of mother during pregnancy, toxicoses and gestoza of pregnant women, etc. The patrimonial trauma of newborns as at the same time the hypothalamic area of a brain which is responsible for pathogenesis of limfatiko-hypoplastic diathesis is often damaged is of great importance. After the child's birth anomaly of the constitution can be provoked by the wrong diet, in particular – artificial feeding there is a lot of protein. A part is played by frequent SARS and other diseases in the first year of life. There is a hereditary predisposition to limfatiko-hypoplastic diathesis. Quite often in a family there are already ill children or cases on one of parental lines.
The pathogenesis of limfatiko-hypoplastic diathesis consists of several components. First of all, the central hormonal regulation in a hypothalamus is broken that is shown by an imbalance of hormones of adrenal glands with a lack of glucocorticoids and surplus of mineralkortikoid. Same is the reason of insufficient T-cellular immunity. At the same time limfatiko-hypoplastic diathesis is followed by an underdevelopment of cardiovascular and endocrine system that, eventually, forms a diverse clinical picture.
Symptoms of limfatiko-hypoplastic diathesis
Limfatiko-gipoplastichesky diathesis arises since the birth, but the main simptomokompleks develops more often by 2-7 years. In the first year of life big weight at the birth can attract attention and a considerable increase in weight every month, existence of several stigmata of a dizembriogenez, decrease in turgor of fabrics and a muscular tone. Approximately by 2-3 years the child usually treats group of often ill children. Can seem that the kid lags behind in development, but actually he just sluggish and sluggish. At planned survey the increased lymph nodes often are found, at the same time their increase is not connected with inflammatory process. Such state is called a limfatizm.
As a rule, the child has adenoides that can become the reason of the complicated nasal breath. At first it does not exert impact on a condition of the patient, but can lead to lag in development because of a chronic hypoxia of a brain later. The noisy breath (stridor) connected with narrowing of a gleam of airways because of the increased timus meets. Because of surplus of mineralkortikoid water-salt exchange is broken that leads to a water delay in an organism. The child at the same time looks pastose, in total with a wide thorax, a horizontal arrangement of edges, a short neck, a trunk and long legs it forms characteristic appearance.
The timomegaliya in combination with dysfunction of adrenal glands can become the reason of sudden death of the child at limfatiko-hypoplastic diathesis though it infrequently meets at this disease. Partly the mechanical sdavleniye of a trachea, partly – the acute shortage of glucocorticoids leading to delay of a warm rhythm and critical falling of arterial pressure becomes the reason of a lethal outcome. Here it should be noted that anomalies of development of heart and vessels in the form of a prolapse of the mitralny valve, additional chords, etc. often occur at children with limfatiko-plastic diathesis, is more rare – congenital heart diseases, but, anyway, cardiovascular insufficiency always takes place.
Diagnosis of limfatiko-hypoplastic diathesis
This anomaly of the constitution comes to light clinically. The pediatrician can suspect limfatiko-hypoplastic diathesis at characteristic appearance of the child. The limfatizm, several stigmata of a dizembriogenez (horizontal edges, a prolapse of the mitralny valve, etc.) and the accompanying timomegaliya which is found perkutorno and is confirmed radiological are defined. As a rule, the child has adenoides, vegetovascular manifestations in the form of an arterialnoya of hypotonia, tranzitorny violations of a warm rhythm, perspiration, causeless subfebrilny temperature, etc.
The large role in diagnosis of limfatiko-hypoplastic diathesis is played by anamnestichesky data. It and the burdened heredity (diabetes, obesity, bronchial asthma, allergic diseases), and infections of mother during pregnancy, and the complicated childbirth, and violations of a diet in the first years of life. In the general blood test of the child with limfatiko-hypoplastic diathesis the increased maintenance of lymphocytes against the background of deficiency of neutrophils is found. Biochemical blood test reveals a dislipidemiya: a hypercholesterolemia, the increased content of phospholipids. Also the lowered glucose level in blood takes place. In an immunogramma – decrease in quantity of lymphocytes and immunoglobulins that confirms insufficiency of cellular immunity.
Treatment of limfatiko-hypoplastic diathesis
Complex therapy is carried out. Dispensary observation at the pediatrician is obligatory. Courses apply adaptogens, in more hard cases – immunostimulators and immunoglobulins. For increase in immunity sanatorium treatment is appointed, the hardening is shown. The large role in therapy of limfatiko-hypoplastic diathesis is played by a diet with the increased content of cellulose. The standard daily rate of protein, water and salt is regulated. Visit of preschool institutions for decrease in risk of infection and the best adaptation of the child is often contraindicated. At considerable growth of adenoides their surgical removal is recommended. Congenital heart diseases and a timomegaliya can also become the indication to expeditious treatment.
Forecast and prevention of limfatiko-hypoplastic diathesis
The forecast strongly varies and depends on expressiveness of a limfatizm, existence of complications from cardiovascular system, a thymus, etc. Prevention of limfatiko-hypoplastic diathesis is possible only during pregnancy of mother, especially for women from risk group on chronic somatic diseases. Addictions are excluded, glucose level is controlled, an important role is played by the correct diet of the pregnant woman. In case of infectious diseases adequate therapy is very important.