Hemophilia B

Hemophilia B cuta ce ta rashin jini da ake gado sakamakon rashin jini na ɗaukar jini IX. Ba tare da isasshen factor IX ba, jinin ba zai iya yin daskarewa yadda ya kamata don sarrafa 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 abubuwan da ke haifar da daskarewa. 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.

Dalilin IX (tara) shine irin wannan tasirin coagulation. Hemophilia B shine sakamakon jiki wanda baya yin isasshen factor IX. Hemophilia B ana haifar dashi ne ta hanyar haɗi mai alaƙa da haɗin X, tare da nakasar kwayar halitta wacce take akan X chromosome.

Mata suna da kwafi biyu na X chromosome. Idan har kwayar IX akan kwayoyin chromosome daya bata aiki, kwayar halittar akan dayan chromosome din zata iya yin aikin samarda isasshen factor IX.

Maza suna da chromosome X guda ɗaya tak. Idan har kwayar IX bata bace akan kromosome na X, zai sami Hemophilia B. A saboda wannan dalili, yawancin mutane masu cutar hemophilia B maza ne.

Idan mace tana da nakasassun abubuwan IX gene, ana ɗauke ta da ɗaukar hoto. Wannan yana nufin za'a iya yada kwayar halittar da ta lalace ga 'ya'yanta. Yaran da aka haifa ga irin waɗannan matan suna da damar 50% na ciwon hemophilia B. 'Ya'yansu mata suna da damar 50% na zama masu ɗauka.

Duk yara maza na mata masu cutar hemophilia suna da nakasar kwayar halitta.

Abubuwan haɗari ga hemophilia B sun haɗa da:

• Tarihin iyali na zubar da jini
• Kasancewa namiji

Tsananin bayyanar cututtuka na iya 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 B sun haɗa da:

• Lokaci na thromboplastin (PTT)
• Prothrombin lokaci
• Lokacin zuban jini
• Matakan Fibrinogen
• Sinadarin factor IX aiki

Jiyya ya haɗa da maye gurbin abin da ya ɓata. Zaka karɓi nauyin IX mai da hankali. Nawa kuke samu ya dogara da:

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

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

Idan kana da cutar hemophilia mai tsanani, zaka iya buƙatar ɗaukar nauyin IX mai da hankali kafin aikin tiyata ko wasu nau'ikan aikin hakori.

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 hemophilia B suna haɓaka ƙwayoyin cuta don haifar da IX. Wadannan antibodies ana kiran su masu hanawa. Masu hanawa sun kawo hari IX saboda kar yayi 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 mutanen da ke da hemophilia B suna iya yin rayuwa daidai.

Idan kana da hemophilia B, 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)
• Thrombosis saboda magani

Kira mai ba da sabis idan:

• Kwayar cutar rashin jini na ci gaba
• An gano wani dan uwa da hemophilia B
• Idan kuna da hemophilia B, 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.

Ana iya yin gwaji yayin daukar ciki a kan jaririn da ke cikin mahaifar uwa.

Cutar Kirsimeti; Dalilin IX hemophilia; Rashin jini - hemophilia B

• Abubuwan da ke da alaƙa da lahani na haɗin X - yadda yara maza ke shafar su
• X-nasaba da recessive raunin kwayoyin - yadda 'yan mata ke shafar
• X-nasaba da recessive kwayoyin lahani
• Kwayoyin jini
• 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.