{"id":20452,"date":"2022-06-30T12:36:25","date_gmt":"2022-06-30T16:36:25","guid":{"rendered":""},"modified":"2023-01-16T13:09:42","modified_gmt":"2023-01-16T17:09:42","slug":"%d0%bd%d0%be%d1%81%d0%b8%d1%82%d0%b5%d0%bb%d1%8c%d0%bd%d0%b8%d1%86%d1%8b-%d0%b8-%d0%b6%d0%b5%d0%bd%d1%89%d0%b8%d0%bd%d1%8b-%d1%81-%d0%b3%d0%b5%d0%bc%d0%be%d1%84%d0%b8%d0%bb%d0%b8%d0%b5%d0%b9","status":"publish","type":"page","link":"https:\/\/elearning.wfh.org\/ru\/%d1%86%d0%b5%d0%bd%d1%82%d1%80%d1%8b-%d1%8d%d0%bb%d0%b5%d0%ba%d1%82%d1%80%d0%be%d0%bd%d0%bd%d0%be%d0%b3%d0%be-%d0%be%d0%b1%d1%83%d1%87%d0%b5%d0%bd%d0%b8%d1%8f\/%d0%bd%d0%be%d1%81%d0%b8%d1%82%d0%b5%d0%bb%d1%8c%d0%bd%d0%b8%d1%86%d1%8b-%d0%b8-%d0%b6%d0%b5%d0%bd%d1%89%d0%b8%d0%bd%d1%8b-%d1%81-%d0%b3%d0%b5%d0%bc%d0%be%d1%84%d0%b8%d0%bb%d0%b8%d0%b5%d0%b9\/","title":{"rendered":"\u041d\u043e\u0441\u0438\u0442\u0435\u043b\u044c\u043d\u0438\u0446\u044b \u0438 \u0436\u0435\u043d\u0449\u0438\u043d\u044b \u0441 \u0433\u0435\u043c\u043e\u0444\u0438\u043b\u0438\u0435\u0439"},"content":{"rendered":"\t\t
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\n\t\t\t\t\t\u041d\u043e\u0441\u0438\u0442\u0435\u043b\u044c\u043d\u0438\u0446\u044b \u0438 \u0436\u0435\u043d\u0449\u0438\u043d\u044b \u0441 \u0433\u0435\u043c\u043e\u0444\u0438\u043b\u0438\u0435\u0439<\/span>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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\n\t\t\t\t\t\u0418\u041d\u0424\u041e\u0420\u041c\u0410\u0426\u0418\u042f<\/a><\/span>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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\n\t\t\t\t\t\u0420\u0415\u0421\u0423\u0420\u0421\u042b<\/a><\/span>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t
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\u041d\u041e\u0421\u0418\u0422\u0415\u041b\u042c\u041d\u0418\u0426\u042b \u0418 \u0416\u0415\u041d\u0429\u0418\u041d\u042b \u0421 \u0413\u0415\u041c\u041e\u0424\u0418\u041b\u0418\u0415\u0419<\/h2>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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\n\t\t\t\t\t\t\t\t\t\t\t\t\t\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/i><\/span>\n\t\t\t\t\t\t\t\t<\/i><\/span>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/span>\n\t\t\t\t\t\t\t\t\t\t\t\t\u041d\u043e\u0441\u0438\u0442\u0435\u043b\u044c\u043d\u0438\u0446\u044b \u0438 \u0436\u0435\u043d\u0449\u0438\u043d\u044b \u0441 \u0433\u0435\u043c\u043e\u0444\u0438\u043b\u0438\u0435\u0439<\/a>\n\t\t\t\t\t<\/div>\n\t\t\t\t\t

For many years, people believed that only men could have symptoms of\u00a0hemophilia<\/a>\u00a0and that women who \u201ccarry\u201d the hemophilia gene do not experience symptoms themselves.<\/p>\n

We now know that many carriers do experience symptoms of hemophilia. As our knowledge about the disorder has increased, so has our understanding of why and how women can be affected. Some women live with their symptoms for years without being diagnosed or even suspecting they have a bleeding disorder. Through education and awareness-raising, the World Federation of Hemophilia is working to close this gap in care.<\/p>\n

Updated November 2012<\/em><\/h6>\n<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t\t
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Hemophilia is usually\u00a0inherited<\/a>, meaning that it is passed from parent to child through the parent\u2019s genes. Genes are packaged within the body\u2019s cells on structures called chromosomes. The genes involved in hemophilia are located on the \u201cX\u201d chromosome.<\/p>\n

The X chromosome is also called the \u201csex chromosome\u201d because it plays a role in determining whether a person is male or female. Men have one X chromosome, which they inherit from their mother, and one Y chromosome, which they inherit from their father. Women have two X chromosomes: one from each parent.<\/p>\n

If the X chromosome a man inherits from his mother has the altered or changed gene, he will have hemophilia. If a woman inherits a copy of the altered gene from either of her parents, she is said to \u201ccarry\u201d the hemophilia gene and is therefore called a \u201ccarrier\u201d. In other words, she has one normal and one altered copy of the gene.<\/p>\n

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All daughters of a father with hemophilia will inherit his altered copy of the X chromosome.<\/figcaption>
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Carriers have one normal and one altered gene. They have a 50% chance of passing the altered gene on to each child. Boys who inherit the altered gene from their mother will have hemophilia; girls who inherit the altered gene from their mother are carriers.<\/figcaption><\/figure>\n<\/div>\n<\/div>\n

Lyonization and clotting factor levels<\/h3>\n

In each cell in a woman\u2019s body, one of the two X chromosomes is turned off, or \u201csuppressed\u201d. This process is called \u201clyonization\u201d, after Mary Lyon, who first described it. Lyonization is a random process that is not fully understood.<\/p>\n

If the chromosome that\u2019s turned off has the altered gene, that cell will produce clotting factor. If the chromosome with the normal gene is turned off, the cell will not produce clotting factor, or the clotting factor it makes won\u2019t work properly.<\/p>\n

On average, carriers of hemophilia will have about 50% of the normal amount of clotting factor, because about half of their cells will have the \u201cgood\u201d gene turned off. Some carriers have far lower levels of clotting factor because more of the X chromosomes with the normal gene are switched off.<\/p>\n

Updated November 2012<\/em><\/h6>\n<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t\t
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There are three levels of severity of hemophilia<\/a>: mild, moderate, and severe. The severity of hemophilia depends on the amount of clotting factor in the person\u2019s blood.<\/p>\n

Any person (male or female) with 5%\u201340% of the normal amount of clotting factor has\u00a0mild hemophilia<\/strong>.<\/p>\n

A person with between 1%\u20135% of the normal amount of clotting factor has\u00a0moderate hemophilia<\/strong>.<\/p>\n

A person with less than 1% of the normal amount of clotting factor has\u00a0severe hemophilia<\/strong>.<\/p>\n

A woman who has less than 40% of the normal level of clotting factor is no different from a man with the same factor levels\u2014she has hemophilia.<\/p>\n

Some carriers have symptoms of hemophilia even though their clotting factor levels are above 40%. A woman with levels of 40%\u201360% of the normal amount of clotting factor who experiences abnormal bleeding is called a\u00a0symptomatic carrier<\/strong>.<\/p>\n

Updated November 2012<\/em><\/h6>\n<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t\t
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Approximately one third of carriers have clotting factor levels of less than 60% of normal and may experience abnormal bleeding. In most cases, carriers experience symptoms similar to those seen in men with mild hemophilia, as well as some that are specific to women, such as prolonged or heavy menstrual bleeding.<\/p>\n

Symptomatic carriers and women with hemophilia:<\/p>\n