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Hypertrophic gemangiektaziya - 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

Hypertrophic gemangiektaziya (Klippel's syndrome — Trenone)
The syndrome is described in 1900 by M. Klippel together with P. Trenaunay. Represents the congenital anomaly of arteriovenous system caused by a vascular dysplasia. At pathoanatomical research define change of superficial and deep veins and arteries of extremities with formation of aneurisms, venous expansions, angiomatous tumors. Clinical symptoms are noted after the birth or on the first year of life. Assume hereditary character of a disease, however genetic aspects are not specified so far. On face skin, mucous membranes of an oral cavity, extremities flat single or multiple angiomas appear. Process has unilateral character more often. Except superficial, deep angiomas meet. The deep angiomatosis of an extremity wears a uniform of a varicosity, cave aneurisms or hemangiomas. The most frequent localization is one of the lower extremities, in particular on the course of a subnodal vein. Profound vascular changes are followed by hypostasis of soft tissues, trophic ulcers. A distinguishing character of the affected extremity — increase in its length. Radiological find lengthening and a thickening of long tubular bones of an extremity. If process affects the lower extremity, at children gait and a bearing because of various length of legs are broken. At Klippel's syndrome — by Trenone other vascular anomalies, stenoses, arteriovenous fistulas can be also observed. Development of cardiovascular insufficiency is possible. Angiomas can be localized in a brain, leading to neurovegetative frustration, lag in psychomotor development. Children live up to adult age, however the forecast of a disease rather serious.
The most effective is radical removal to quinsies — electrocoagulation, surgical excision of affected areas, and then vascular plastics. Surgical treatment has to be carried out to perhaps early terms, before development of vascular insufficiency and bone disturbances.

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