Hemophilia A

Hemophilia A cuta ce ta rashin jini da ake gado sakamakon rashin jini mai saka jini VIII. Ba tare da isasshen factor VIII ba, jinin ba zai iya yin daskarewa yadda ya kamata don sarrafa zub da jini ba.

Lokacin da kuka zub da jini, jerin maganganu na faruwa a cikin jiki wanda ke taimakawa yaduwar jini. Wannan tsari shi ake kira coagulation cascade. Ya ƙunshi sunadarai na musamman da ake kira coagulation, ko kuma daskarewa, dalilai. Kuna iya samun damar samun yawan zub da jini idan ɗayan ko fiye daga waɗannan abubuwan sun ɓace ko basa aiki kamar yadda ya kamata.

Dalili na VIII (takwas) shine ɗayan irin wannan matsalar coagulation. Hemophilia A shine sakamakon jiki wanda baya yin isasshen factor VIII.

Hemophilia A ana haifar da shi ne ta hanyar haɗi mai alaƙa da haɗin X, tare da nakasar kwayar halitta da ke jikin X chromosome. Mata suna da kwafi biyu na X chromosome. Don haka idan yanayin kwayar cuta ta VIII akan kwayoyin chromosome daya baya aiki, kwayar halittar da ke dayan chromosome din zata iya yin aikin samarda isasshen factor VIII.

Maza suna da chromosome X guda ɗaya tak. Idan har kwayar halittar VIII ta bata a jikin chromosome na X, zai sami hemophilia A. A saboda wannan dalili, yawancin mutane masu cutar hemophilia A maza ne.

Idan mace tana da nakasasshen factor VIII gene, ana ɗauka ta ɗauka. Wannan yana nufin za'a iya yada kwayar halittar da ta lalace ga 'ya'yanta. Yaran da aka haifa wa irin wadannan matan suna da damar kashi 50% na ciwon hemophilia A. 'Ya'yansu mata suna da damar kashi 50% na zama masu ɗauka. Duk yara maza na mata masu cutar hemophilia suna da nakasar kwayar halitta. Abubuwan haɗari ga hemophilia A sun haɗa da:

• Tarihin iyali na zubar da jini
• Kasancewa namiji

Tsananin bayyanar cututtuka ya bambanta. Tsawan jini shine babban alama. Galibi ana fara ganin sa yayin da aka yi wa jariri kaciya. Sauran matsalolin zub da jini galibi suna bayyana ne lokacin da jariri ya fara rarrafe da tafiya.

Casesananan lamuran na iya zama ba a lura da su har sai daga baya a rayuwa. Bayyanar cututtuka na iya fara faruwa bayan tiyata ko rauni. Zuban jini na ciki na iya faruwa a ko'ina.

Kwayar cutar na iya haɗawa da:

• Zuban jini cikin mahaɗa tare da haɗuwa da zafi da kumburi
• Jini a cikin fitsari ko bayan gida
• Isingaramar
• Maganin ciki da fitsarin jini
• Hancin Hanci
• Tsawon jini daga cuts, cire haƙori, da tiyata
• Zuban jini wanda yake farawa ba tare da dalili ba

Idan kai ne mutun na farko a cikin iyali da ake tsammanin cuta na zub da jini, mai ba ka kiwon lafiya zai yi odar jerin gwaje-gwaje da ake kira nazarin coagulation. Da zarar an gano takamaiman lahani, wasu mutane a cikin danginku za su buƙaci gwaji don gano cutar.

Gwaje-gwajen don gano hemophilia A sun haɗa da:

• Prothrombin lokaci
• Lokacin zuban jini
• Matakan Fibrinogen
• Lokaci na thromboplastin (PTT)
• Sababin magani Sabbin VIII

Jiyya ya haɗa da maye gurbin abin da ya ɓata. Kuna karɓar mahimmancin VIII mai da hankali. Nawa kuke samu ya dogara da:

• Tsananin zubar jini
• Wurin zubar jini
• Nauyin ki da tsayin ki

Moila zazzabin hemophilia mai sauƙi tare da desmopressin (DDAVP). Wannan maganin yana taimakawa sashin sakin jiki na VIII wanda aka adana a cikin rufin jijiyoyin jini.

Don hana rikicin zub da jini, ana iya koyar da mutanen da ke da cutar hemophilia da danginsu don ba da mahimmin abu na VIII a gida a alamun farko na zub da jini. Mutanen da ke da mummunar cuta na cutar na iya buƙatar yin rigakafin rigakafin yau da kullun.

Hakanan za'a iya buƙatar DDAVP ko factor VIII mai da hankali kafin samun hakar haƙori ko tiyata.

Ya kamata ku sami rigakafin cutar hepatitis B Mutanen da ke da hemophilia suna iya kamuwa da cutar hepatitis B saboda suna iya karɓar kayan jini.

Wasu mutanen da ke da cutar hemophilia A suna haɓaka ƙwayoyin cuta don haifar da VIII. Wadannan antibodies ana kiran su masu hanawa. Masu hanawa sun kawo hari kan abin na VIII saboda ya daina aiki. A irin wannan yanayi, ana iya ba da sinadarin daskarewa da mutum ya kira VIIa.

Kuna iya sauƙaƙa damuwar rashin lafiya ta hanyar haɗuwa da ƙungiyar tallafi na haemophilia. Yin tarayya tare da wasu waɗanda suke da masaniya da matsaloli na yau da kullun na iya taimaka muku kada ku ji ku kaɗai.

Tare da magani, yawancin mutane masu cutar hemophilia A suna iya yin rayuwa daidai.

Idan kana da hemophilia A, yakamata kayi bincike akai-akai tare da likitan jini.

Matsaloli na iya haɗawa da:

• Matsalar haɗin gwiwa na dogon lokaci, wanda na iya buƙatar maye gurbin haɗin gwiwa
• Zub da jini a cikin kwakwalwa (zubar jini ta intracerebral)
• Jinin jini saboda magani

Kira mai ba da sabis idan:

• Alamomin cutar zubar jini na ci gaba
• An gano wani dan uwa da hemophilia A
• Kuna da hemophilia A kuma kuna shirin samun yara; kwayoyin shawara akwai

Za'a iya ba da shawarar shawarwarin kwayoyin halitta. Gwaji na iya gano mata da yan matan da ke ɗauke da kwayar cutar hemophilia. Gano mata da yan matan da ke dauke da kwayar cutar hemophilia.

Ana iya yin gwaji yayin daukar ciki a kan jariri a cikin mahaifar uwa.

Dalilin karancin VIII; Classic hemophilia; Rashin jini - hemophilia A

• Jinin jini

Carcao M, Moorehead P, Lillicrap D. Hemophilia A da B. A cikin: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Ka'idoji da Aiki. 7th ed. Philadelphia, PA: Elsevier; 2018: babi na 135.

Scott JP, Ambaliyar VH. Rashin jini (rashin jini). A cikin: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Littafin koyar da ilimin yara. 21st ed. Philadelphia, PA: Elsevier; 2020: babi na 503.