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Ataxy teleangiectasia - Practical hematology of children's age

Table of contents
Practical hematology of children's age
Embryonal hemopoiesis
Morfofunktsionalny characteristic of cells of marrow and peripheral blood
Marrow parenchyma cells
Peripheral blood of children of different age
The system of a hemostasis is normal
Etiology and pathogeny of leukoses
Acute leukoses
Acute leukoses - a preleukosis
Possibilities of a predictive assessment of a course of an acute lymphoblastoid leukosis at children
General principles of treatment of an acute leukosis
Chemotherapeutic drugs
Treatment of an acute lymphoblastoid leukosis
Treatment of myeloid forms of an acute leukosis
Infectious complications and symptomatic therapy of an acute leukosis
Consolidation and maintenance therapy of an acute leukosis
Remission and recurrence of an acute leukosis
Inborn leukosis
Macrofollicular lymphoma
Angioimmunoblastny lymphadenopathy
Leukemoid tests
Infectious lymphocytosis
Infectious mononucleosis
Leukemoid tests of different types
Dysfunctions of granulocytes
Histiocytoses - an eosinophilic granuloma
Malignant histiocytosis
Family erythrophagocytal histiocytosis
Accumulation diseases
Nimann's illness — Peak
Hemorrhagic vasculitis (Shenleyn's illness — Genokh)
Mayokki's purpura
Ataxy teleangiectasia
Entsefalotrigeminalny angiomatosis
Kortiko-meningealny diffusion angiomatosis
Cerebroretinal angiomatosis
Hypertrophic gemangiektaziya
Multiple and huge hemangiomas
Elastic fibrodisplaziya
Hereditary coagulopathies
Hemophilia And
Clinic of hemophilia
Treatment of hemophilia
Cristmas disease (Kristmas's illness)
Hereditary deficit of factors of XI, XII, XIII and I
Hereditary deficit of factors of VII, X, V and II
Deficit K-vitaminozavisimykh of factors of coagulation
Syndrome of the disseminated intravascular coagulation
Clinic and diagnosis of the IDCS
Treatment of the IDCS
Idiopathic Werlhof's disease
Clinic and diagnosis of an idiopathic Werlhof's disease
Treatment of an idiopathic Werlhof's disease
Isoimmune Werlhof's disease
Transimmune Werlhof's disease of newborns
Trombogemolitichesky Werlhof's disease (syndrome Moshkovich)
Hereditary Werlhof's diseases
The anemias connected with blood loss
Chronic posthemorrhagic anemia
Iron deficiency anemias
Clinic and diagnosis of an iron deficiency anemia
Treatment of iron deficiency anemias
Sideroakhrestichesky, sideroblastny anemias
Megaloblastny anemias
Foliyevodefitsitny anemia
Hereditary forms of megaloblastny anemias
Hereditary dizeritropoetichesky anemias
The anemias connected with oppression of proliferation of cells of marrow
Hereditary hypoplastic anemias
Hemolitic anemias
Hemolitic anemias - an ovalocytosis, a hereditary stomatocytosis
Acanthocytosis, piknotsitoz
The hereditary hemolitic anemias connected with disturbance of activity of enzymes of erythrocytes
The hereditary hemolitic anemias connected with disturbance of structure or synthesis of hemoglobin
The acquired immune hemolitic anemias
Isoimmune hemolitic anemias
Treatment of a hemolitic illness of newborns
Autoimmune hemolitic anemias
List of references

Ataxy teleangiectasia (Louis Bar syndrome) - the disease is in detail described in 1941 D. Louis-Bar is characterized by a combination of cerebellar and skin manifestations. At pathoanatomical research define a cerebellum degeneration, an atrophy of its white matter. Multiple dilatation of small vessels of skin, mucous membranes, a meninx and substance of a brain is noted. Some authors consider a disease as a mezenkhialny dysplasia. Now assume that Louis's syndrome - the Bar is the cornerstone disturbance of immune systems. The hypoplasia of lymph nodes, spleens, a thymus is noted. The disease is inherited on autosomal recessively type. Persons man's and female are ill with an identical frequency. Clinical signs appear at early children's age (after 1 year). The first symptoms which attract attention are lacks of coordination of movements. There is unstable and shaky a gait, awkwardness at purposive movements is noted. At more senior children it is possible to reveal disturbance of coordination tests. Further the neurologic symptomatology progresses, cerebellar frustration — an intentsionny tremor, a nystagmus accrue. Patients cease to go independently, lose purposeful coordination of movements. There is a scanning speech, tendon jerks decrease, the hypomyotonia develops. A characteristic symptom of a disease are vascular changes. As a rule, teleangiectasias develop after neurologic disturbances though they can be marked out also at the beginning of a disease, the quantity them gradually increases. Originally teleangiectasias define on a conjunctiva, then they are found on mucous membranes of an oral cavity, a hard and soft palate, lobes of ears, on skin of upper and lower extremities, an upper half of a trunk. On skin a nevus pigmentosus with sites of a depigmentation, a scleroderma and a keratosis appears. Various signs of a dizembriogenez can be noted, children lag behind in intellectual development. At patients the raised susceptibility to infections — the frequent respiratory diseases, otitis, sinusitis, pneumonia accepting long or chronic character is noted. Now it is proved that patients have no immunoglobulin A, and also the level of other classes of immunoglobulins is reduced. Therefore, the raised susceptibility to infections is caused by an immunodeficiency.
The course of a disease steadily progresses. Patients seldom live up to adult age and, as a rule, perish from consecutive infections. Treatment is directed to fight against infections — antibiotics, plasma transfusions, gamma-globulin, fortifying actions. There were messages on transplantation of a thymus and marrow.

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