I-hemophilia B

I-Hemophilia B yisifo esopha ifa esidalwa ukuntuleka kwegazi lokuvala igazi i-IX. Ngaphandle kwesici esanele i-IX, igazi alikwazi ukugoqa kahle ukulawula ukopha.

Lapho wopha, uchungechunge lwezinto ezenzekayo emzimbeni olusiza ukwakheka kwamahlule egazi. Le nqubo ibizwa ngokuthi yi-coagulation cascade. Kubandakanya amaprotheni akhethekile abizwa ngokuthi yi-coagulation, noma ama-clotting factor. Ungaba nethuba eliphakeme lokopha ngokweqile uma eyodwa noma eziningi zalezi zinto zilahlekile noma zingasebenzi ngendlela okufanele zenze ngayo.

I-Factor IX (eyisishiyagalolunye) ingesinye seziqu ze-coagulation. IHemophilia B ingumphumela womzimba ongenzi i-factor eyanele ye-IX. I-Hemophilia B ibangelwa isici se-X esixhunywe ku-X esinefa, nesakhi esineziphene esiku-X chromosome.

Abesifazane banamakhophi amabili we-X chromosome. Uma isakhi se-IX se-chromosome eyodwa singasebenzi, isakhi esikolunye i-chromosome singenza umsebenzi wokwenza i-IX eyanele.

Abesilisa bane-X chromosome eyodwa kuphela. Uma isakhi sofuzo i-IX sishoda nge-X chromosome yomfana, uzoba ne-Hemophilia B. Ngalesi sizathu, iningi labantu abane-hemophilia B ngabesilisa.

Uma owesifazane enesici se-IX esinesici esibi, uthathwa njengomthwali. Lokhu kusho ukuthi isakhi sofuzo esingalungile singadluliselwa ezinganeni zakhe. Abafana abazalwa ngabesifazane abanjalo banethuba elingama-50% lokuba ne-hemophilia B. Amadodakazi abo anethuba elingama-50% lokuba othwala.

Zonke izingane zesilisa zamadoda ezine-hemophilia zithwala isakhi sofuzo esingalungile.

Izici eziyingozi ze-hemophilia B zifaka:

• Umlando womndeni wokopha
• Ukuba owesilisa

Ubukhulu bezimpawu bungahluka. Ukopha isikhathi eside kuyisifo esiyinhloko. Kuvame ukubonwa okokuqala lapho usana lusokwa. Ezinye izinkinga zokopha zivame ukuvela lapho usana luqala ukukhasa nokuhamba.

Amacala amancane angahle angabonakali kuze kube kamuva empilweni. Izimpawu zingaqala ukwenzeka ngemuva kokuhlinzwa noma ukulimala. Ukopha kwangaphakathi kungenzeka noma kuphi.

Izimpawu zingafaka:

• Ukopha ezinhlangothini ezinobuhlungu obuhambisanayo nokuvuvukala
• Igazi emchameni noma esitulweni
• Ukulimaza
• Umgudu wamathumbu nokopha komgudu womchamo
• Ukopha ngamakhala
• Ukopha isikhathi eside ngokusikeka, ukukhishwa kwamazinyo nokuhlinzwa
• Ukopha okuqala ngaphandle kwesizathu

Uma ungumuntu wokuqala emndenini onenkinga yokusolwa yokopha, umhlinzeki wakho wezokunakekelwa kwempilo uzo-oda uchungechunge lwezivivinyo ezibizwa nge-coagulation Study. Lapho isici esithile sesitholakele, abanye abantu emndenini wakho bazodinga ukuhlolwa ukuze kutholakale ukuphazamiseka.

Ukuhlolwa kokuthola ukuthi i-hemophilia B ihlanganisa yini:

• Isikhathi esithile se-thromboplastin (PTT)
• Isikhathi se-Prothrombin
• Isikhathi sokopha
• Izinga le-Fibrinogen
• Umsebenzi we-Serum factor IX

Ukwelashwa kubandakanya ukufaka esikhundleni se-clotting factor elahlekile. Uzothola ukugxilwa kwe-factor IX. Ukuthi uthola malini kuncike ku:

• Ukonakala kokopha
• Indawo yokopha
• Isisindo sakho nokuphakama

Ukuvimbela inkinga yokuphuma kwegazi, abantu abane-hemophilia nemindeni yabo bangafundiswa ukunikeza i-factor IX egxila ekhaya lapho kuvela izimpawu zokuqala zokopha. Abantu abanezinhlobo ezinzima zesifo bangadinga ukumnika okujwayelekile, okuvikela.

Uma une-hemophilia enamandla, kungadingeka ukuthi uthathe ukugxila kwe-IX ngaphambi kokuhlinzwa noma izinhlobo ezithile zomsebenzi wamazinyo.

Kufanele uthole umuthi wokugoma i-hepatitis B. Abantu abane-hemophilia maningi amathuba okuthi bathole i-hepatitis B ngoba bangathola imikhiqizo yegazi.

Abanye abantu abane-hemophilia B bakhulisa ama-antibodies ukuze benze i-IX. La ma-antibody abizwa ngokuthi ama-inhibitors. Ama-inhibitors ahlasela i-IX ukuze ingasasebenzi. Ezimweni ezinjalo, kunganikezwa into ejiyile eyenziwe ngumuntu ebizwa nge-VIIa.

Unganciphisa ukucindezela kokugula ngokujoyina iqembu lokwesekwa kwe-hemophilia. Ukwabelana nabanye abanokuhlangenwe nakho okuvamile nezinkinga kungakusiza ungazizwa uwedwa.

Ngokwelashwa, abantu abaningi abane-hemophilia B bayakwazi ukuphila impilo ejwayelekile.

Uma une-hemophilia B, kufanele uhlolwe njalo nge-hematologist.

Izinkinga zingafaka:

• Izinkinga ezihlangene zesikhathi eside, ezingadinga ukushintshwa okuhlanganyelwe
• Ukopha ebuchosheni (i-intracerebral hemorrhage)
• I-thrombosis ngenxa yokwelashwa

Shayela umhlinzeki wakho uma:

• Izimpawu zesifo sokopha ziyakhula
• Ilungu lomndeni litholakale line-hemophilia B
• Uma une-hemophilia B, futhi uhlela ukuba nezingane; ukwelulekwa ngofuzo kuyatholakala

Ukwelulekwa ngofuzo kunganconywa. Ukuhlola kungakhomba abesifazane namantombazane abaphethe isakhi sofuzo i-hemophilia.

Ukuhlola kungenziwa ngesikhathi sokukhulelwa enganeni esesibelethweni sikanina.

Isifo sikaKhisimusi; Isici IX hemophilia; Ukuphazamiseka kwegazi - i-hemophilia B

• Ukukhubazeka kwezakhi zofuzo okuxhunywe ku-X - ukuthi abafana bathinteka kanjani
• Ukukhubazeka kwezakhi zofuzo okuxhunywe ku-X - amantombazane athinteka kanjani
• Iziphazamiso zofuzo ezixhunywe ku-X
• Amaseli egazi
• Amahlule egazi

UCarcao M, Moorehead P, uLillicrap D.Hemophilia A noB B. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 135.

UScott JP, uZamcolo VH. Ukushoda kwegazi lokunqanda isici (ukuphazamiseka kwegazi). Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 503.