Matenda a m'magazi A

Hemophilia A ndi matenda obadwa nawo otuluka magazi omwe amayamba chifukwa chosowa magazi VI. Popanda chinthu chokwanira VIII, 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 VIII (eyiti) ndichimodzi mwazinthu zoterezi. Hemophilia A ndizotsatira zakuti thupi silimapanga zokwanira VIII.

Hemophilia A imayambitsidwa ndi mkhalidwe wobwezeretsa wolumikizidwa ndi X, wokhala ndi jini yolakwika yomwe ili pa X chromosome. Akazi ali ndi makope awiri a X chromosome. Chifukwa chake ngati majini a VIII pa chromosome imodzi sagwira ntchito, jini ya chromosome inayo imatha kugwira ntchito yopanga gawo la VIII lokwanira.

Amuna ali ndi chromosome imodzi yokha ya X. Ngati geni ya factor VIII ikusowa pa X chromosome yamnyamata, adzakhala ndi hemophilia A. Pachifukwa ichi, anthu ambiri omwe ali ndi hemophilia A ndi amuna.

Ngati mayi ali ndi vuto VIII jini, amamuwona ngati wonyamula. Izi zikutanthauza kuti jini lopanda vuto lingaperekedwe kwa ana ake. Anyamata obadwa kwa akazi oterewa ali ndi mwayi wokhala ndi hemophilia A. 50% 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 A ndi izi:

• Mbiri ya banja yakukha magazi
• Kukhala wamwamuna

Kukula kwa zizindikilo kumasiyana. Kutuluka magazi kwa nthawi yayitali ndiye chizindikiro chachikulu. Nthawi zambiri 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 zimatha kuphatikiza:

• 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 A kumaphatikizapo:

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

Chithandizochi chimaphatikizapo kuchotsa m'malo mwa chinthu chomwe sichikusunga magazi. Mukalandira zomwe VIII imayang'ana. Zomwe mumapeza zimatengera:

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

Haemophilia wofatsa atha kuchiritsidwa ndi desmopressin (DDAVP). Mankhwalawa amathandiza thupi kumasula chinthu VIII chomwe chimasungidwa mkatikati mwa mitsempha.

Pofuna kupewa vuto lakutaya magazi, anthu omwe ali ndi hemophilia ndi mabanja awo amatha kuphunzitsidwa kuti azigwiritsa ntchito zinthu VIII kunyumba pazizindikiro zoyambirira zotuluka magazi. Anthu omwe ali ndi mitundu yayikulu yamatenda angafunike chithandizo chokhazikika.

DDAVP kapena factor VIII concentrate itha kufunikanso musanachotsere mano kapena opaleshoni.

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 A amapanga ma antibodies ku VIII. Ma antibodies awa amatchedwa inhibitors. The zoletsa kuukira chinthu VIII kuti 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 A amatha kukhala moyo wabwinobwino.

Ngati muli ndi hemophilia A, 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)
• Kuundana kwamagazi chifukwa chothandizidwa

Itanani omwe akukuthandizani ngati:

• Zizindikiro za matenda otuluka magazi zimayamba
• Wachibale wapezedwa ndi hemophilia A
• Muli ndi hemophilia A 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. Dziwani amayi ndi atsikana omwe ali ndi jini la hemophilia.

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

Kusowa kwa Factor VIII; Classic hemophilia; Matenda a magazi - hemophilia A

• 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.