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Thrombocytopathy

 
, medical expert
Last reviewed: 20.11.2021
 
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Thrombocytopathy - disorders of hemostasis, due to the qualitative inferiority of blood platelets with a normal number of them. Distinguish between hereditary and acquired. Among the primary hereditary platelet dysfunctions, the most common are atrombia, thrombocytopathy with a defect in the release of thrombocytes, thrombastenia. Secondary hereditary thrombocytopathies are typical of Willebrand's disease of albinism afribinogenemia (Hsizmansky-Pudlak syndrome) of hyperelastic skin syndromes (Ehlers-Danlos), Marfan and other connective tissue dysplasias of many metabolic anomalies. Acquired thrombocytopathies with or without hemorrhagic syndrome are characteristic of many blood diseases (leukemia, hypoplastic and megaloblastic anemia) of uremia of DIC-syndrome of immunopathological diseases (hemorrhagic vasculitis, lupus erythematosus, diffuse glomerulonephritis, etc.) of radiation sickness of medicinal disease when taking salicylates, xanthines, carbenicillin neurocirculatory dysfunction.

The spread of primary hereditary thrombocytopathies in children is not established, but it is undoubtedly the most frequent genetically determined pathology of the hemostasis system. In most cases of so-called family bleeding of an unknown genesis, hereditary thrombocytopathy can be diagnosed. Their frequency in the population reaches 5%.

Causes of thrombocytopathy in children

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Hereditary thrombocytopathies

There are primary hereditary thrombocytopathies (thrombocytopathy with a defect in the release reaction, Glanzmann thrombasthenia, etc.) and secondary ones entering the symptom complex of the underlying disease (eg, thrombocytopathy in Willebrand disease, albinism, Ehlers-Danlo syndrome, etc.).

Acquired thrombocytopathy

They are widely distributed, since the functional state of platelets suffers in the overwhelming majority of severe pathological conditions of newborns (hypoxia, acidosis, infection, shock, etc.).

trusted-source[9], [10], [11], [12], [13], [14], [15], [16], [17], [18], [19]

Medicinal products

Violating the function of platelets can medications that affect the cascade of arachidonic acid, increase the level of cAMP in platelets, inhibit the formation of thrombin, as well as heparin sodium and other drugs: carbenicillin, nitrofuran derivatives, antihistamines, phenobarbital, antioxidants, chlorpromazine, dextrans, povidone, beta-blockers, sulfonamides, cytostatics, B vitamins and other drugs. In newborns, the cause of bleeding is more often carbenicillin, aminophylline, large doses of ascorbic acid, furosemide.

What provokes thrombocytopathy?

Symptoms of thrombocytopathy in children

In the newborn period, hereditary thrombocytopathies manifest clinically under additional effects (hypovitaminosis and other nutritional deficiencies in the mother, perinatal pathology, in particular acidosis, hypoxia, sepsis). Nevertheless, the medicinal acquired thrombocytopathy is predominant. Clinical manifestations of bleeding can be either general (generalized cutaneous hemorrhagic syndrome, bleeding of mucous membranes), and local (hemorrhages in internal organs, as well as intraventricular and other intracranial hemorrhages, hematuria).

In the anamnesis, increased bleeding in the child's relatives. Thrombocytopathy is characterized by positive endothelial tests (tourniquet, pinch, etc.) with a normal number of platelets in the blood and normal parameters of the coagulation system. The time of bleeding is increased.

The diagnosis is established on the basis of the study of the aggregation function of platelets with aggregates (ADP in different doses, collagen, adrenaline, ristocetin).

Symptoms of thrombocytopathy

What tests are needed?

Treatment of thrombocytopathy in children

When thrombocytopathy appoint drugs that increase the functional activity of platelets. Sodium etamzilate in mild cases is prescribed in a dose of 0.05 g 3-4 times a day for 7-10 days; with a more severe course of the disease - intramuscularly or intravenously at a dose of 0.5-1.0 ml of 12.5% solution once a day for 7-10 days. Stimulant effect on the function of platelets (in addition to sodium etamzilate) is calcium pantothenate, aminocaproic acid, carbazochrome, trifosadenin.

Treatment of thrombocytopathies

How are thrombocytopathies prevented in children?

Primary prophylaxis of the disease is not developed, secondary prevention of relapses includes: planned sanation of foci of infection prevention of contacts with patients with infectious diseases (especially acute respiratory viral infections) deworming individual solution of the question of carrying out preventive vaccinations, avoiding insolation, UFO and UHF physical education in the preparatory group, mandatory blood test after any disease.

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