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Elastic fibrodisplaziya - 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
Immunotherapy
Remission and recurrence of an acute leukosis
Inborn leukosis
Neuroleukosis
Myelosis
Lymphogranulomatosis
Gematosarkoma
Macrofollicular lymphoma
Angioimmunoblastny lymphadenopathy
Leukemoid tests
Infectious lymphocytosis
Infectious mononucleosis
Leukemoid tests of different types
Dysfunctions of granulocytes
Leukopenias
Histiocytoses
Histiocytoses - an eosinophilic granuloma
Malignant histiocytosis
Family erythrophagocytal histiocytosis
Accumulation diseases
Nimann's illness — Peak
Angiopathies
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
Coagulopathies
Hereditary coagulopathies
Hemophilia And
Clinic of hemophilia
Treatment of hemophilia
Angiohemophilia
Cristmas disease (Kristmas's illness)
Hereditary deficit of factors of XI, XII, XIII and I
Dysfibrinogenemias
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
Thrombocytopenia
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
Trobotsitopatiya
Anemias
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

Elastic fibrodisplaziya (Chernogubov's syndrome — Elersa — Danlosa)
The disease is described by Chernogubov (1891), and also E. Ehlers (1899) and F. Danlos (1908). It is characterized by the increased distensibility of skin, her easy vulnerability, looseness of joints. Defect of a structure of collagenic fabric is the cornerstone of a syndrome. The disease has family character. Now six various options of an elastic fibrodisplaziya are described. From them I, II, III options are inherited automno-is prepotent, the IV option — automno-retsessivno, it differs in the heaviest current. The V option is inherited retsessivno (it is linked to X-chromosome) and the VI option — autosomal retsessivno. Clinical symptoms arise at early age. Skin is thin, the sverkhelastichna, a rastyazhima, easily gathers in folds, in the field of natural folds is thinned. Slight injuries conduct to ruptures of skin and walls of vessels which bleed. On skin hemorrhages — from punctulate to large hematomas appear. According to V. M. Yurlova (1979) — Elersa — Danlosa functional inferiority of thrombocytes and genetically determined disturbances of plasma components of blood with deficit VIII, IX or XII factors are the reason of bleeding at Chernogubov's syndrome. Vascular disorders are characterized by development of an aortic aneurysm, multiple aneurisms of small arteries, a varicosity. Ruptures of small aneurisms lead to hemorrhages in different bodies. Skin wounds long heal, leaving hems. Also looseness of joints is noted, such patients are figuratively described as "the rubber person", "person snake". Habitual dislocations of joints can be observed. Besides, in a clinical picture symptoms of defeat of different bodies — damage of eyes, heart diseases, malformations of guts, a bladder, lungs, bone system are noted. These symptoms are changeable.
The forecast of a disease rather favorable, but depends on extent of damage of internals. Patients live up to adult age, keep working capacity. The preventive actions directed to injury prevention are important. There is no special treatment. Ascorbic acid, Retinolum, vitamins of group B have favorable effect.



 
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