Hemophilia na ụmụ - congenital ọbara daa ọrịa

Hemophilia na ụmụ - butere n'aka hemorrhagic diathesis. Development of hemophilia e mere site na a congenital erughi ọbara ihe idem ke guzobere plasma thromboplastin, a umi dị mkpa n'ihi na ọbara ngwa ngwa ida ke trauma.

The ọrịa emee predominantly na mmadụ. Ọ na-ebute site ná si na-arịa ọrịa ụmụ ụmụ clinically ike ndinyom. E nwere atọ iche ọrịa a.

1. Hemophilia A - kasị nkịtị ụdị ọrịa ahụ. N'ihi na odida na ọrịa ọbara antihemophilic globulin.

2. Hemophilia B e mere site erughi n'ime ọbara plasma thromboplastin akụrụngwa.

3. Hemophilia e mere site enweghị ọbara plasma thromboplastin ụzọ.

Dị ka a n'ihi nke insufficiency n'ime ọbara nke otu n'ime ihe ndị a na-akwusila ya coagulation. Nke a ọbara anakọtara na a ule tube, a nnọọ ogologo oge na-adịghị clot, na kpụrụ ihe nwayọọ nwayọọ dozie ala.

Nke a na ọrịa e ji gematomny ụdị ọbara ọgbụgba. Isi mkpesa nke ọrịa - a na-agba ọbara ọchịchọ, nke na-egosipụta onwe ya na akpa isua nke ndụ. Na akụkọ ihe mere eme nke hemophilia na ụmụaka na-egosipụta udo agba ọbara, hematoma na mụọ nwa, ruo ogologo oge na-agba ọbara teething, anụ ọnyá. Mgbe e mesịrị na-egosi ọzịza na ịdị nro nke nkwonkwo na obere unan. Adults mgbe ịzụlite na-egbu mgbu nile ọbara ọgbụgba na subcutaneous anụ ahụ, muscle hematoma. Obere ọbara ọgbụgba ọnyá na-echi ọnụ n'ala n'ọnụ ya. Nke a bụ n'ihi na eziokwu na ụkọ plasma thromboplastin beleta ntolite onuogugu site a dị ịrịba ama ntọhapụ nke anụ ahụ.

Kwuru, ọbara ọgbụgba nke mucous membranes na ọnụ, imi, eriri afọ tract, akụrụ, bronchus. E nwere ike ịbụ na-agba ọbara na ụbụrụ ma ọ bụ meninges. Ize ọrịa ndụ retroperitoneal hematoma, na-agba ọbara na-akpata ire, n'olu, akpịrị. Oké ọbara ọgbụgba nwere ike ime ka ọ bụla ọgwụ na ahụ ike mkpachapụ, e.g., mwepụ nke a ezé, ndị dị iche iche na ogwu ogbugba, na karịsịa n'ịwa ahụ. A ikpehe hemorrhagic syndrome bụrụ na nke mmebi bụ obere arịa e mesịrị omume nke ọbara ọgbụgba. Ọ pụrụ ịzụlite n'ime awa ole na ole mgbe ihe ọjọọ.

Ahụkarị mgbaàmà na-erukwa na ụmụ hemophilia na-agba ọbara n'ime nkwonkwo, na-akpọ hemarthroses. Ọtụtụ mgbe ịzụlite hemarthrosises ikpere na n'ikpere aka nkwonkwo. Ha bụ nnọọ na-egbu mgbu, Bilie a ịrị elu okpomọkụ. Ugboro ugboro hemarthrosises-eduga mmepe nke contractures.

Hemophilia - a oké njọ ọbara ọrịa. Karịsịa oké hemophilia na ụmụaka na-eto eto. N'oge gara aga, ọtụtụ ndị ọrịa nwụrụ ọbara ọgbụgba ma ọ bụ hemorrhage na akụkụ ahụ ndị dị ruo mgbe afọ iri abụọ. Nsogbu nke ọrịa na-agụnye oké ụkọ ọbara na ọnya nke nkwonkwo - hemarthrosis na contractures-emepe emepe na hemarthrosis.

Ọ bụrụ na chọpụtara na hemophilia, ọgwụgwọ a ga-rụrụ ke akpa ọnwa nke ndụ nke onye ọrịa. Isi usoro ọgwụgwọ nke ọbara ọgbụgba ihe ngwa ngwa nnọchi ọgwụ. N'ihi hemophilia ọgwụgwọ iji ọbara na ngwaahịa, na nke ọbara coagulation ihe na-debeere ogologo karịa ọhụrụ ọbara. Nke a antihemophilic plasma, cryoprecipitate ma ọ bụ ọbara clotting akpata uche. Na oké ikpe, mgbe ụfọdụ, eji kpọmkwem ọbara si a na-enye onyinye. Na hemophilia B na C na-ndinọ nwa afọ plasma na akọrọ, nakwa dị ka mgbagwoju nkwadebe, nwere ọtụtụ ọbara clotting ihe. Tinyere ihe ndị ọzọ hemostatic mmadụ mee aminocaproic acid na fibrinogen. Hemophilia ọgwụgwọ na-egbochi mmepe nke nsogbu nke ọrịa, budata mma na oge nke ndụ nke onye ọrịa.