Literally the hemophilia is a propensity to krovotochivosti and to hemorrhages. Zabolevanie enters into group gemorragicheskih a diathesis (a diathesis - predraspolozhennost, gemorragija - a bleeding) and is hereditary koagulopatiej, i.e. infringement of curling of blood. Bleedings and hemorrhages pri the given disease long, sometimes spontaneous (spontaneous), without vsjakoj the visible reason. The hemophilia is known since very old times. In the Talmud (II century BC) Cases of death of boys from ritual trimming are described. As samostojatelnoe disease a hemophilia has described in 1874 Fordajs, and the term "hemophilia" has offered F.Hopff. Gemofilija in 1828 is connected with the low maintenance, quantitative and qualitative nedostatochnostju any one of factors of curling of blood. Plasma faktory blood curlings have the Roman numbering. The classical hemophilia (to 85 % of all cases) is caused by deficiency antigemofilnogo globulin (AGG) - VII faktora, it call In (illness Kristmasa) is connected A.Gemofilija`s by hemophilia with nedostatkom plasma tromboplastinovogo a component (PTK), or the factor Kristmasa - IX factor, on its share it is necessary to 13 % of all versions of it zabolevanija. The hemophilia With occurs from insufficiency of the predecessor tromboplastina (PPT) - XI factor, its frequency does not exceed 1-2 %. The others of 0,5 % sluchaev hemophilias sjazany with deficiency of other plasma factors of curling krovi: V factor (paragemofidija or gipoproaktselerinemija), VII factor (gipoprokonvertinemija), H the factor (Stewart`s illness - Prauer). Kak both a hemophilia With, and illness Villebranda (angiogemofilija), the specified diseases ne are true hemophilias. At true hemophilias And and In the type of inheritance linked to a floor is observed retsessivnyj. At a hemophilia With it is marked / rautosomno-prepotent type of inheritance. Retsessivnyj, linked to a floor (H-chromosome) the inheritance type is shown that a hemophilia, as a rule, bolejut men, women act only as conductors, illness transmitters. Poskolku marriage and a birth of the child at the man, suffering a hemophilia, and the zhenshchiny-conductor is registered extremely seldom, sons of patients as pravilo, do not inherit this disease, and at daughters of display of a hemophilia in mirovoj to the literature are described only in 60 cases. Disease is inherited from the grandfather to vnuku. Docheri and sisters sick of a hemophilia are conductors of a hemophilia. In connection with etim they can pass disease not only to the sons, but through docherej-conductors and to grandsons, great-grandsons and later posterity man`s pola. Approximately at 30 % of patients it is not possible to track distinctly inheritance zabolevanija, in such cases it is necessary to assume a spontaneous mutation of genes in a RH-chromosome. The heaviest form of a hemophilia is ascertained at the maintenance in plazme blood of the patient VIII or IX factors less than 1 %, the heavy form - from 1 to 2 %, srednej weights - from 2 to 5 %, the easy form - from 5 to 10 % and latentno (is hidden) tekushchaja - over 15 %, but no more than 65-70 %. Chastota hemophilias And by data the CART (the World Organization of Public health services), kolebletsja from 5 to 10, hemophilias In - from 0,5-1 to 5 cases on 100 thousand novorozhdennyh boys. The hemophilia With meets much less often - to 2 % among nasledstvennyh koagulopaty (illnesses of curtailing system of blood). harakternym a clinical symptom of a hemophilia the bleeding coming dazhe after a small trauma is most. Krovotechenija at sick of a hemophilia arise often, easily, they are long and obilny. Bleedings of a measure of effect usually applied to a stop do not give. Chasto there are hematomas (hemorrhage). At newborns it is extensive kefalogematomy (head hematomas), hypodermic and intraskin hemorrhages in oblasti buttocks and promezhnosti, late bleedings from the tied up umbilical cord, krovotechenija during a teething. U sick children 1,5 - 2 years even insignificant traumas are accompanied krovopodtyokami on a forehead, finitenesses, buttocks, and a teething, prikusyvanie jazyka and a mucous membrane of cheeks, injections are accompanied long, sometimes mnogosutochnym, till 2-3 weeks by a bleeding. Often hemorrhages in sustavy are marked. Hypodermic, intermuscular hematomas are extensive, tend to rasprostraneniju, clinically remind tumours, are accompanied by "flowering" (blue colour - sine - violet - brown - golden), resolve during 2 months. The hemorrhage in an eye at a bruise can lead finally slepote. Krovoizlijanie in the spinal channel at a trauma can lead lethal ishodu or to a paralysis. Bleedings from a nose, gums, mucous membranes oral polosti suffer to 75 % of sick children. Gastroenteric and nephritic krovotechenija meet seldom, they can result from traumas or after razlichnyh manipulations. Hemorrhages in a brain and brain obolochki with a lethal (deadly) outcome are possible. At 90-95 % of children approximately with 2 - / r3-year-old age are marked gemartrozy (hemorrhages in joints), especially in krupnye: knee, talocrural, humeral, elbow, luchezapjastnye, tazobedrennye. Otmechajutsja a pain, a swelling, local and general rises in temperature, protective kontraktura (reduction) of muscles. At the first hemorrhages blood gradually rassasyvaetsja, at repeated - the clots postponed on a capsule sustava and a cartilage that leads at first to decrease in its functions, and further - to ankilozu (immovabilities) are formed. Hemorrhages in the bone fabric, leading nekrozu (omertveniju) and dekaltsinatsii (calcium loss) bones are possible. Diagnostics osnovyvaetsja on the basis of inquiry (inheritance of disease among men on materinskoj lines), clinical displays and laboratory data. On most skromnym to data the quantity sick of a hemophilia in the world makes nearby 300000 chelovek. Treatment sick of a hemophilia is spent at gematologa. To such patients kategoricheski are counter-indicative atsetilsalitsilovaja acid (aspirin), brufen, indometatsin, butazolidiny, analginum. Gemofilija concerns rather dangerous diseases. Children`s and youthful years it vsyo still leads to high death rate. The forecast concerning recover neblagoprijatnyj also depends on weight of disease, timeliness and adequacy terapii. The major preventive action - mediko-genetic konsultirovanie marrying. At marriage sick of a hemophilia and the zhenshchiny-conductor of a hemophilia of children to have do not recommend. At the healthy woman, sostojashchej in marriage with sick the hemophilia, on 14 - 16th week of pregnancy a method transabdominalnogo amniotsenteza establishes a floor ploda. Esli the future child - the girl mothers recommend to interrupt pregnancy, poskolku there is a danger of distribution of a hemophilia through the zhenshchinu-conductor. At marriage of the healthy man and the woman-conductor of children to have ne recommend in connection with probability of a birth of the man sick of a hemophilia or the zhenshchiny-conductor. Revealing of women-conductors probably at definition at them v blood VIII or IX factors a quantitative biochemical method. Revealed bolnye prophylactic medical examinations are subject. The guarding mode is necessary for children. Patients gemofiliej concern risk groups on a hepatitis and AIDS (HIV) as the preparations prepared from donor blood are widely applied to them lechenija.

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