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Clinic of hemophilia - 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

As a rule, if in a clinical picture of hemophilia a hemarthrosis was noted, then with age joint disturbances progress. At modern therapy patients seldom perish from bleedings at children's age; afterwards at them defeat of the device of the movement and a support which extent in many respects defines social suitability of the person prevails.
The clinic of hemophilia is characterized by extraordinary variety of symptoms, but, except above-mentioned, other signs meet smaller frequency. At younger children's age bleedings from urinary tract are very rare, but the frequency and intensity of a hamaturia can increase over the years. Bleedings from a digestive tract are also rare. There can sometimes be bleeding in abdominal organs, a peritoneum, retroperitoneal cellulose, a mesentery with a prelum of the arteries passing in it and a gut gangrenosis. Hemorrhages in a brain or a meninx at hemophilia are extremely rare, but they are fraught because of danger of death or development of crushing organic lesion of a nervous system. The forecast at bleedings in a spinal cord or its covers more favorable (A. M. Abezgauz, 1970).
The children sick with hemophilia, often make impression playful, undisciplined (processes of excitement prevail over braking processes). They usually asthenic constitution and subnutrition. Internals out of bleeding without pathology. The liver and a spleen are, as a rule, not increased.
With increase in life expectancy of patients with hemophilia the frequency of complications increases. Many complications at hemophilia are caused by immune mechanisms. At patients pathological reactions to a transfusion of haemo drugs are often noted. Clinically they are characterized by one or a combination of several symptoms: temperature increase, back pain, skin allergic rash. Terrible complication of hemophilia is development of an inhibitory form of a disease. In blood define a high antiserum capacity, immune inhibitors of factors of coagulation, in particular at hemophilia And — inhibitor of a factor VIII, at a Cristmas disease — a factor of IX. Gravity of situation is caused by the fact that anti-hemophilic drugs lose a therapeutic effectiveness as at introduction by the patient are immediately inactivated by inhibitor. There is a vicious circle: repeated administration of drug leads to increase of an antiserum capacity. According to different authors, inhibitory forms make from 1 to 21% of cases of hemophilia. They are much less often marked out at children of younger age, and their frequency increases after 10 — 12. In diagnosis of ingibigorny forms the clinical assessment of efficiency of the carried-out replacement therapy is on the first place. Absence of effect at the sufficient volume of the entered haemo drugs has to suggest an idea of development of an inhibitory form of hemophilia. Laboratory researches confirm initial judgment. And in diagnosis it is possible to use very simple method: the calcium clotting time of normal plasma is extended at addition to it of a small amount of the plasma containing inhibitor. At addition of plasma of the patient with hemophilia with an uncomplicated current of such phenomenon it is not noted.
Immune disturbances are capable to cause still a number of complications. At hemophilia developing of hemolitic anemia, leukopenia thrombocytopenia is described. The glomerulonephritis and an amyloidosis of kidneys from the outcome can develop in a chronic renal failure.
Some patients have a secondary rhematoid syndrome having the immunocomplex nature. 3. S. Barkagan and L. P. Egorova (1971) allocate the following signs of a secondary rhematoid syndrome: 1. Chronic inflammatory process (often symmetric) in small joints of the brushes and feet which were not surprised earlier hemorrhages; 2. Morning constraint in the joints including affected with a hemorrhagic osteoarthrosis; 3. Change of a current of gemofilichesky arthropathies — pain and constraint in joints. And pain is not weakened at replacement therapy and can even amplify. 4. Progressing of joint changes out of communication with fresh hemorrhages. At laboratory research define substantial increase SOE, a hypergammaglobulinemia, a positive S-reactive protein, increase of level of sialic acids.
At the transfusion hematotherapy which is carried out by the patient with hemophilia there can be a transfer of causative agents of infectious diseases. Now inoculation of a viral hepatitis is most often noted.
Existence of gematomny type of bleeding, especially in combination with the characteristic family anamnesis allows to suspect hemophilia. Approximate laboratory researches following: a normal thrombin and prothrombin time, disturbance of the tests characterizing the internal mechanism of a blood coagulation (kephalin - kaolinic tests etc.).
Differential diagnosis is carried out on the basis of correctional tests.

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