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Entsefalotrigeminalny angiomatosis - 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

Entsefalotrigeminalny angiomatosis (Shturge's syndrome — Weber — Krabbe) - the disease is described by V. A. Sturge (1870) and P. F. Weber (1922), and in 1934 is allocated F. Krabbe in an independent form. This pathology is characterized by a triad of symptoms: the angioma of the person, inborn glaucoma and convulsive attacks caused by a brain choroid angioma. At pathoanatomical research find a widespread angiomatosis of skin, choroidal textures of an eye, a soft meninx, a spinal cord, parenchymatous bodies. The angiomatosis is caused by a neuroectodermal dysplasia, thinner mechanisms of a disease are not studied. The important place is allocated to a role of hereditary factors. A mode of inheritance — autosomal dominatny with incomplete penetrance of a pathological gene, also autosomal recessive inheritance is possible.
Skin angiomas are noted at children from the moment of the birth, in literature they are figuratively described as "the flaring spots". Preferential localization on a face, and is more often unilateral. On skin of the lower extremities and a trunk of an angioma meet seldom. The second characteristic symptom is inborn glaucoma which is usually observed on the party of angiomas.
The neurologic status is characterized by existence of convulsive attacks which develop on the first year of life. Attacks usually have focal character, generalized spasms are less often observed. Pyramidal symptoms — increase of tendon jerks, a hemiparesis on the party opposite to an angioma of the person join convulsive attacks.
At Shturge's syndrome — Weber — Krabbe also other malformations — not fusion of handles of vertebrae, hernias are available. Allocate several options of a disease: with a full triad of symptoms, bisimptomny forms — a combination of an angioma of the person to glaucoma or convulsive attacks and monosimptomny forms. At most of patients moderate decrease in intelligence is noted. The lethal outcome as a result of a hematencephalon is possible. Treatment includes symptomatic, anticonvulsants etc. Ktichesky actions, abstention from a child-bearing in families with the burdened heredity are necessary a profiala.



 
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