Hemophilia B

Hemophilia B ndi matenda obadwa nawo omwe amatuluka chifukwa chosowa magazi othandiza IX. Popanda chinthu chokwanira IX, magazi sangathe kuundana bwino kuti athane ndi magazi.

Mukamatuluka magazi, zochitika zingapo zimachitika mthupi zomwe zimathandiza kuundana kwamagazi. Izi zimatchedwa coagulation cascade. Zimaphatikizapo mapuloteni apadera otchedwa coagulation, kapena clotting factor. Mutha kukhala ndi mwayi wambiri wokhetsa magazi ngati chimodzi kapena zingapo mwazinthuzi zikusowa kapena sizikugwira ntchito momwe ziyenera kukhalira.

Factor IX (zisanu ndi zinayi) ndichimodzi mwazinthu zoterezi. Hemophilia B ndi zotsatira za thupi lomwe silipanga zokwanira IX. Hemophilia B imayambitsidwa ndi mkhalidwe wobwezeretsa wolumikizidwa ndi X, wokhala ndi jini lopunduka lomwe lili pa X chromosome.

Akazi ali ndi makope awiri a X chromosome. Ngati chinthu cha IX pa chromosome imodzi sichigwira ntchito, jini ya chromosome inayo imatha kugwira ntchito yopanga IX yokwanira.

Amuna ali ndi chromosome imodzi yokha ya X. Ngati geni IX ikusowa pa X chromosome yamnyamata, adzakhala ndi Hemophilia B. Pachifukwa ichi, anthu ambiri omwe ali ndi hemophilia B ndi amuna.

Ngati mkazi ali ndi vuto la IX gene, amamuwona ngati wonyamula. Izi zikutanthauza kuti jini lopanda vuto lingaperekedwe kwa ana ake. Anyamata obadwa kwa akazi otere ali ndi mwayi wokhala ndi hemophilia B. 50% ya ana awo aakazi ali ndi mwayi wokhala 50% wonyamula.

Ana onse achimuna a amuna omwe ali ndi hemophilia amakhala ndi jini yolakwika.

Zowopsa za hemophilia B ndizo:

• Mbiri ya banja yakukha magazi
• Kukhala wamwamuna

Kukula kwa zizindikilo kumatha kusiyanasiyana. Kutuluka magazi kwa nthawi yayitali ndiye chizindikiro chachikulu. Kawirikawiri zimawoneka koyamba khanda likadulidwa. Mavuto ena otuluka magazi nthawi zambiri amawonekera khanda likayamba kukwawa ndikuyenda.

Milandu yofewa imatha kuzindikirika mpaka pambuyo pake m'moyo. Zizindikiro zimayamba kuchitika atachitidwa opaleshoni kapena kuvulala. Kutuluka magazi mkati kumatha kuchitika kulikonse.

Zizindikiro zimaphatikizapo:

• Kuthira magazi m'malo olumikizana ndi ululu komanso kutupa
• Magazi mkodzo kapena chopondapo
• Kulalata
• Matenda am'mimba komanso kwamikodzo kutuluka magazi
• Kutulutsa magazi m'mphuno
• Kutuluka magazi kwa nthawi yayitali chifukwa chodulidwa, kuchotsa mano, komanso kuchitidwa opaleshoni
• Kuthira magazi komwe kumayamba popanda chifukwa

Ngati ndinu woyamba m'banja kukhala ndi matenda okayikira otuluka magazi, wothandizira zaumoyo wanu adzaitanitsa mayeso angapo otchedwa coagulation Study. Vutoli likazindikira, anthu ena am'banja mwanu adzafunika kuyesedwa kuti adziwe matendawa.

Kuyesa kuzindikira hemophilia B ndi:

• Nthawi yapadera ya thromboplastin (PTT)
• Nthawi ya Prothrombin
• Nthawi yokhetsa magazi
• Mulingo wa Fibrinogen
• Ntchito ya Serum factor IX

Chithandizochi chimaphatikizapo kuchotsa m'malo mwa chinthu chomwe sichikusunga magazi. Mudzalandira ma factor IX. Zomwe mumapeza zimatengera:

• Kuwonongeka kwa magazi
• Malo otuluka magazi
• Kulemera ndi kutalika kwako

Pofuna kupewa vuto lakutaya magazi, anthu omwe ali ndi hemophilia ndi mabanja awo amatha kuphunzitsidwa kuti azigwiritsa ntchito IX kunyumba nthawi yoyamba kuwonetsedwa. Anthu omwe ali ndi mitundu yayikulu yamatenda angafunikire kulowetsedwa pafupipafupi, kodziteteza.

Ngati muli ndi haemilia wochuluka, mungafunikenso kuganizira za IX musanachite opaleshoni kapena mitundu ina ya mano.

Muyenera kulandira katemera wa hepatitis B. Anthu omwe ali ndi hemophilia amatha kutenga hepatitis B chifukwa amatha kulandira zinthu zamagazi.

Anthu ena omwe ali ndi hemophilia B amapanga ma antibodies kuti apange IX. Ma antibodies awa amatchedwa inhibitors. Ma inhibitors amaukira IX kuti isagwirenso ntchito. Zikatero, chinthu chopangidwa ndi magazi chotseka chotchedwa VIIa chitha kuperekedwa.

Mutha kuchepetsa nkhawa zamankhwala ndikulowa nawo gulu lothandizira hemophilia. Kugawana ndi ena omwe akumana ndi mavuto omwe akukumana nawo kungakuthandizeni kuti musamve nokha.

Ndi chithandizo, anthu ambiri omwe ali ndi hemophilia B amatha kukhala moyo wabwinobwino.

Ngati muli ndi hemophilia B, muyenera kuyezetsa magazi nthawi zonse.

Zovuta zingaphatikizepo:

• Mavuto a nthawi yayitali, omwe angafunike m'malo mwake
• Kutuluka magazi muubongo (kukha magazi m'mimba mwa ubongo)
• Thrombosis chifukwa cha chithandizo

Itanani omwe akukuthandizani ngati:

• Zizindikiro za kutuluka magazi zimayamba
• Wachibale wapezedwa ndi hemophilia B
• Ngati muli ndi hemophilia B, ndipo mukufuna kukhala ndi ana; upangiri wa majini ulipo

Upangiri wa chibadwa ungalimbikitsidwe. Kuyesedwa kumatha kuzindikira azimayi ndi atsikana omwe ali ndi jini la hemophilia.

Kuyesedwa kumatha kuchitika panthawi yapakati pa mwana yemwe ali m'mimba mwa mayi.

Matenda a Khirisimasi; Zinthu IX hemophilia; Matenda a magazi - hemophilia B

• Zolumikizana ndi X zomwe zimalumikizidwa ndi X - momwe anyamata amakhudzidwira
• Zolumikizana ndi X zomwe zimalumikizidwa ndi X - momwe atsikana amakhudzidwira
• Zolumikizana ndi X zomwe zimalumikizidwa kwambiri
• Maselo amwazi
• Kuundana kwamagazi

Carcao M, Moorehead P, Lillicrap D.Hemophilia A ndi B. Mu: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Mfundo Zoyambira ndi Zochita. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2018: mutu 135.

Scott JP, Chigumula VH. Zofooka zobwera chifukwa cha magazi m'masamba (zovuta zamagazi). Mu: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, olemba. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: chap 503.