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Cerebroretinal 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

Cerebroretinal angiomatosis (Gippel's illness — Lindau, an angioreticulomatosis)
The detailed description of symptoms of a disease N. made Hippel (1904) and A. Lindau (1926). The clinical picture is caused by existence of angiomas in skin, a retina, the central nervous system, mainly in a cerebellum. At pathoanatomical research find considerable capillary ectasia, quite often cystous tumors of internals and a cerebellum. The cerebroretinal angiomatosis is a malformation of vascular system. The disease is inherited on autosomal dominant type, to a thicket males are ill. The first signs appear at children's age. Eye symptoms which are observed even at small children concern to the earliest. At ophthalmologic research define the single angiomas of a retina located on the periphery. Further the disease progresses, large tumors are formed, there can be amotio of a retina. As a rule, defeat has unilateral character. Angiomas are localized in a cerebellum, an oblong, spinal cord, but within several years can not give any symptomatology. Symptoms of defeat of a nervous system remind tumoral process. Note hydrocephaly, signs of a gipertenzionno-likvorny syndrome, focal symptomatology with a lack of coordination of movements and statics, a nystagmus. The main signs arise usually on the party of a tumor. There is a cerebellar ataxy. On face skin, extremities, a stomach, a chest klotka multiple flat angiomas in the form of red specks are noted. On mucous membranes they are localized very seldom. At Gippel's illness — Lindau the possible complication connected with increase of intracranial pressure is infringement of a brainstem and sudden death. The forecast of a disease rather favorable, angiomas of the central nervous system do not tend to growth; most hard the retina angiomatosis proceeds. The symptomatic treatment, is directed to reduction of a gipertenzionno-likvorny syndrome. Apply dehydrational therapy.



 
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