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Treatment 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
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

Treatment is pathogenetic. The basic principle is introduction to an organism of the patient of factors which are absent in his blood; in other words, the carried-out therapy has to be replaceable. Attaching the greatest significance to this principle, it should be noted that treatment has to be complex. In therapy of hemophilia allocate several important points: 1. Actions for a stop of bleeding of the general and local orientation. 2. Treatment of effects of bleeding (hemarthrosis, hematomas etc.). 3. Treatment of complications of hemophilia (inhibitory forms, secondary rhematoid syndrome). 4. Prevention of bleeding at hemophilia: a) the specific measures including preoperative prevention of bleeding; b) the nonspecific, including elements of leaving, the mode, vocational guidance etc.
5. Genoprofilaktika in the families having patients with hemophilia allowing to interrupt further transfer of an illness.
Actions for a bleeding stop first of all are directed to the general increase of coagulability of blood. Replacement therapy is carried out by the haemo drugs containing a factor of VIII. It should be noted that the factor of VIII labilen and does not remain in stored blood, native and dry plasma. The second important property is the short half-life in blood estimated 6 — 8 h that causes need of repeated administrations of haemo drugs within a day for maintenance of necessary therapeutic concentration. The concentrate of a factor of VIII — the cryoprecipitate containing a significant amount of a factor in small volume is most effective. It also contains a factor of XIII and fibrinogen in a large number. Also antigemofnlny plasma, introduction • which in a daily dose of 10 — 20 ml/kg of body weight 3 times in day is effective provides maintenance of a factor of VIII at the level of more than 10%, sufficient at bleedings easy and moderately severe. In urgent situations in the absence of the above-named drugs therapy of the choice is the direct hemotransfusion which can provide haemo static effect. Transfusions of stored blood are a little productive. It should be noted that direct hemotransfusions cannot be the main cure of replacement therapy for hemophilia and to them it is possible to resort only as to a compulsory measure. Administration of anti-hemophilic plasma also cannot provide increase of concentration of a factor of VIII to necessary level at heavy bleedings, injuries, operations as such huge amounts of plasma which will surely lead to the expressed disturbances of a hemodynamics are for this purpose necessary. From these positions cryoprecipitate costs to the first among anti-hemophilic drugs when stopping bleedings at patients with hemophilia. When calculating a necessary dose of drug consider degree of manifestation and localization of gemofilichesky bleedings. Depending on degree of bleeding allocate three groups of bleedings.

  1. Small spontaneous bleedings from mucous membranes of oral and nasal cavities, bleedings .iz wounds, moderate hematomas without prelum of surrounding fabrics and bodies, a moderate hemarthrosis, small surgical interventions (removal of one tooth, except painters). For stopping of bleeding it is necessary to support a level of a factor of VIII within 10 — 20%. The adequate daily dose of cryoprecipitate — 15 — 20 PIECES/kg of body weight or anti-hemophilic plasma of 15 — 20 ml/kg of weight — 1 activity unit is equated to 1 ml of the "average" plasma having a level of a factor of VIII, equal 100%.
  2. Heavy hemarthrosis, extensive hypodermic and intermuscular hematomas, gastrointestinal bleedings, hamaturia, removal of several teeth, band operations, orthopedic interventions. The level of a factor of VIII needs to be supported within 30 — 40%. Use only cryoprecipitate in a dose of 35 — 40 PIECES/kg of body weight a day.
  3. Profuse bleedings from a digestive tract, a gross hematuria, bleedings at big injuries, extensive surgeries, and also a tonsilectomy. The level of a factor of VIII has to exceed 50%. The dose of cryoprecipitate makes 60 — 100 PIECES/kg of weight a day.

The necessary dose of cryoprecipitate can be calculated on a formula, proceeding from a necessary therapeutic level of a factor of VIII (R. A. Rutberg, Yu. N. Andreyev, 1972):
Cryoprecipitate dose =
Frequency of introduction is determined by the level reached after the first infusion, and necessary therapeutic concentration taking into account that in 6 — 8 h concentration of a factor of VIII will decrease twice. At hemophilia And haemo drugs enter 2 — 3 times a day though sometimes happens rather single infusion of cryoprecipitate. All haemo drugs enter intravenously and only struyno. During replacement therapy infusional introduction of the saline solutions, blood substitutes and haemo drugs which are not containing anti-hemophilic globulin is contraindicated as there is a dilution and decrease in concentration of a factor of VIII in blood.
For a local stop of bleeding use 5% solution of aminocapronic acid, process the bleeding surface or holes of the extracted teeth thrombin. Any intramuscular injections which can lead to formation of extensive hematomas are strictly contraindicated to patients with hemophilia.
At renal and gastrointestinal bleedings therapy is carried out by the general principles, using the high doses of cryoprecipitate supporting a level of a factor VIII in blood it is higher than 50%. At renal bleedings use of aminocapronic acid is not recommended as formation of clots can lead to obstruction of urinary tract and an anury. At gastrointestinal bleedings the cryoprecipitate combination to intake of aminocapronic acid in a dose of 0,15 — 0,2 g/kg of weight a day is possible.
Therapy at an acute hemarthrosis has certain features. Stopping of bleeding in a joint is carried out by infusional replacement therapy by cryoprecipitate. Besides, the temporary immobilization of an extremity for 2 — 5 days is necessary for reduction of the acute phenomena. In this period thermal procedures (compresses, wrappings etc.) are shown . It is established that the cold works negatively in this connection this procedure is contraindicated at a hemarthrosis. 3. S. Barkagan (1980) indicates the need of early aspiration of the blood which streamed in a joint then introduction of a hydrocortisone is reasonable. Replacement therapy at a hemarthrosis is carried out within 5 — 10 days, then development of the affected joint is recommended. The technique offered 3. S. Barkagan and N. M. Selivanov, provides the early beginning occupations of physiotherapy exercises — at once after removal of an immovable bandage. It is carried out step by step, with gradual increase in load of a joint, in combination with physioprocedures (from 2nd week). Timely therapy of an acute hemarthrosis — guarantee of successful prevention of chronic osteoarthroses. The great value is attached to constant intensive year-round physiotherapy exercises by special methods. In this plan sets of exercises in water are effective. The physiotherapy exercises are supplemented with methods balneo-, water - and mud cures, physical therapy (an electrophoresis with glucocorticoids). At treatment of chronic gemofilichesky osteoarthroses use a roentgenotherapy, a synovectomy (according to indications).
At formation of hematomas replacement therapy is carried out by the general principles. It is not recommended to punktirovat hematomas in connection with a possibility of infection. Resort to a puncture only in case of a compression syndrome (a prelum of large vessels, nervous trunks etc.). Usually at adequate replacement therapy of a hematoma resolve independently.
In the presence of a wound carry out primary surgical treatment with economical excision of fabrics and its sewing up (under cover of replacement therapy). It creates conditions for primary healing of wounds at patients with hemophilia. Imposing of compressing bandages on a wound is not recommended.
Difficult question is treatment of inhibitory forms of hemophilia. As it was already noted, in these situations replacement infusional therapy yields the return result, strengthening production of inhibitor of a factor of VIII and consequently, worsening a condition of the patient. In urgent situations when stopping bleeding a desperate method is introduction considerably big, than usually, cryoprecipitate doses with calculation what this quantity will be enough also for inhibitor neutralization, and for Hemostatum? eskigo actions (E. Scheibel and soavt., 1985). However afterwards it leads to increase in an antiserum capacity. Use of Prednisolonum and immunodepressants (an imuran, Cyclophosphanum) in most cases was ineffective. Use of a medical plasma exchange for removal of inhibitor from blood is possible. Now the most efficient method of treatment of an inhibitory form of hemophilia is administration of the drug PPSB (a concentrate of factors of II, VII, IX,             X), a number of hereditary coagulopathies applied at therapy of a Cristmas disease and still. Infusions of the drug PPSB should not be combined using aminocapronic acid in connection with a possibility of trombotichesky complications (3. S. Barkagan, 1980).
At development of an infectious disease as a result of a transfusion of haemo drugs carry out treatment according to a nosological form. Prevention of bleeding at hemophilia is a component of complex treatment, and on the importance is on the first place. Replacement therapy by the drugs containing a factor of VIII with the preventive purpose is without fail carried out before surgical interventions, beginning from extraction of teeth and finishing with extensive band operations. The volume of replacement therapy is determined depending on character of surgical intervention.
The general nonspecific measures are of great importance. The correct orientation of parents (and on achievement of the age of reason and the child) about character of a disease, its complications, effects etc. is necessary. Creation is necessary for sick children of the guarding mode, since early age, withdrawal from use of the cutting, pricking, sharp objects, various toys able to put an injury
to the child, it is the best of all to use soft toys. In development of the child it is necessary to limit as much as possible outdoor games, sports activities, the general physical education classes, and also the works connected with physical work. Performance of conditions of limited activity is quite difficult at children of younger age — there is no conscious need of these conditions. Therefore parents have to distract the child from outdoor games, accustoming to reading, board games etc. Becoming is more senior, children learn by own experience in disease severity and already begin to limit themselves. Children and parents have to know about an illness in all subtleties, but at the same time they should explain that the forecast at a disease favorable that observance of necessary measures will allow to grow to the child and to become the full-fledged member of society. Therefore children have to communicate with peers, not feel the lameness, take part in public actions etc. All this promotes normal development of the identity of the child. Also categorically it is impossible to exclude also physical occupations. They promote physical development of the child, strengthen and build muscles which are the best protection of joints. Such atraumatic sport as swimming promoting good development of muscular system is especially shown to patients with hemophilia. For protection of joints it is possible to use porolonovy guards. Already from younger age it is necessary to carry out vocational guidance with the child. The professions connected with brainwork are optimum.
Among the general actions the important place is taken prevention of infectious diseases which can provoke bleeding. Normal development of the child defines normal forming of the general resilience. From the same positions patients with hemophilia can carry out preventive inoculations, excepting the gamma-globulin in high doses causing extensive hematomas. During inoculations it is recommended to press the place of a prick a finger for several minutes. As it was already noted, in general intramuscular injections are not shown to patients with hemophilia therefore in need of treatment concerning any disease it is desirable to appoint medicinal substances inside or intravenously. A number of drugs is contraindicated to patients with hemophilia. Treat them: acetylsalicylic acid, pyrazolon derivatives, indometacin, Brufenum. The specified means break aggregation of thrombocytes and by that promote bleeding. The Euphyllinum, papaverine, furosemide, nitrofuran possess the same, but weaker action. High doses of penicillin and its semi-synthetic analogs which use is undesirable to patients with hemophilia break function of thrombocytes.
At microtraumas the local stop of bleeding can be carried out in house conditions. For this purpose it is necessary to store constantly in the refrigerator haemo static drugs (an absorbable gelatin sponge, thrombin, 5% solution of aminocapronic acid) which process a surface of skin, mucous membranes of an oral cavity, gums. Patients have to consist on accounting in dispensary group of policlinic and the specialized hematologic center; once in two months and consultation of the stomatologist each three months is necessary for them regular survey.



 
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