Annually April 17<\/strong> At the initiative of the World Health Organization (WHO) and the World Federation of Hemophilia (WFH), the medical community celebrates World Hemophilia Day.<\/p>\n The main goal of this day is to draw public attention to the problems of people with blood clotting disorders, improve their access to quality medical care, and raise awareness of this rare disease among the population.<\/p>\n The date of the celebration was chosen in honor of the birthday Frank Schnaibel<\/strong> \u2014 the founder of the World Federation of Hemophilia, who dedicated his entire life to protecting the interests of patients with this disease.<\/p>\n In the history of medicine, hemophilia is forever engraved under the name \u00ab"royal disease"\u00bb<\/strong>.<\/p>\n The most famous carrier of this genetic disorder was the British Queen Victoria<\/strong>. Through the dynastic marriages of her children and grandchildren, the disease spread to the royal families of Germany, Spain, and the Russian Empire. In particular, the only son of Emperor Nicholas II, Tsarevich Alexei, suffered from hemophilia. In those days, the disease was a death sentence: there was no cure, and any internal bleeding or a simple bruise after a fall could be fatal for a child.<\/p><\/blockquote>\n Hemophilia is a hereditary disease caused by a mutation in genes that causes a lack of certain proteins in the blood plasma called clotting factors. The disease is transmitted through the X chromosome, so It mostly affects men.<\/strong>, while women are most often only carriers of a "broken" gene.<\/p>\n You can suspect a violation of the hemostasis (blood clotting) system based on the following typical signs:<\/p>\n Today, hemophilia is no longer a death sentence. Modern medicine allows patients to lead full, active lives thanks to replacement therapy with clotting factor concentrates. However, it remains critically important timely laboratory diagnostics<\/strong>.<\/p>\n The main screening tool is coagulogram<\/strong> (comprehensive study of blood clotting functions). In hemophilia, laboratory technicians record an isolated prolongation of the indicator ACTH<\/strong> (activated partial thromboplastin time) with a completely normal platelet count. To definitively confirm the diagnosis, accurate tests<\/a> to determine the activity level of specific factors VIII and IX.<\/p>\n
\n\ud83e\ude78 Why April 17? Interesting facts from history and the "royal trail"\u00ab<\/h3>\n
\n\ud83d\udcca Hemophilia in numbers: types and statistics of the disease<\/h3>\n
\n\n
\n \nType of disease<\/th>\n What factor is missing?<\/th>\n Frequency of occurrence<\/th>\n Features<\/th>\n<\/tr>\n<\/thead>\n \n Hemophilia A<\/strong> (classical)<\/td>\n Factor VIII<\/strong> (8)<\/td>\n 1 case per 5,000 \u2013 10,000 newborn boys<\/td>\n The most common form, which accounts for about 80-85%<\/strong> of all cases in the world.<\/td>\n<\/tr>\n \n Hemophilia B<\/strong> (Christmas disease)<\/td>\n Factor IX<\/strong> (9)<\/td>\n 1 case per 20,000 \u2013 34,000 newborn boys<\/td>\n It is much less common. It is named after the first patient in whom it was studied, Stefan Christmas.<\/td>\n<\/tr>\n \n Hemophilia C<\/strong> (rare form)<\/td>\n Factor XI<\/strong> (11)<\/td>\n Extremely rare<\/td>\n It can occur in both men and women. It is usually mild.<\/td>\n<\/tr>\n<\/tbody>\n<\/table><\/div>\n
\n\ud83d\udd0d Main symptoms and the role of a modern laboratory<\/h3>\n
\n