Raunin da ke faruwa ne da ciwo a cikin suturar jini
Hanyoyin hemoglobinuria na launi na Paroxysmal (PNH) wani ƙwayar cuta ne na jini. An kira shi ne da farko don bayyanar da zubar da fata mai duhu (hemoglobinuria) da aka samar da safe. Anyi zaton cewa mummunar cututtuka na jini ( jini ) wanda ya haifar da fitsari mai tsafta ya faru a bursts (paroxysms) wanda ya faru a daren. Bincike ya nuna, duk da haka, cewa hawan jini a cikin PNH, wanda cutar ta lalacewa, ta faru a ko'ina cikin rana kuma ba ya faruwa a bursts.
Canje-canje a cikin launi na fitsari yana iya faruwa a kowane lokaci amma yana da mafi ban mamaki a cikin gaggawa na dare.
Ƙara fahimtar halayyar haemoglobinuria maras raiyo ta sake zubar da jini kamar yadda yake da ciwon jini, yaduwar jini a veins (thrombosis), da kuma rashi cikin samar da dukkanin jini ( hematopoiesis ). PNH yana shafi maza da mata na kowane kabila kuma zai iya faruwa a kowane zamani, amma mafi yawancin samuwa a cikin matasa.
Cutar cututtuka
Mutane da yawa da PNH ba su da alamun bayyanar. Kwayoyin cututtuka na PNH sun dogara ne akan ko sassolysis, thrombosis, ko hematopoiesis ba shi da.
- Hemolysis - furotin mai launin cola-colored (hemoglobinuria), anemia hagu
- Thrombosis - inda yaduwar jini ya kayyade bayyanar cututtuka. Idan jini ya kasance a cikin hanta na hanta, akwai jaundice da hanta mai girma. A cikin ciki, jini zai haifar da ciwon ciki; a kai, ciwon kai. Clots a cikin fata fata tashe, mai zafi, red lumps a kan manyan yankunan fata, kamar baya.
- Hematopoiesis marasa talauci - anemia (bai isa ba jini), mai yiwuwa ga cututtuka (ƙananan jini jini), da zubar da jini mai mahimmanci (bai zama cikakke platelets) ba.
Sanin asali
Akwai gwaje gwaje-gwaje da dama da zasu iya taimakawa wajen gano asalin hawan haemoglobinuria maras kyau. Mafi mahimmanci shine gwada jini don gano CD55 da CD59, sunadarai biyu.
Rashin waɗannan sunadarai a kan jinin jini sun tabbatar da ganewar asali na PNH. A lokacin ganewar asali, wasu gwaje-gwaje na jini za ayi ciki har da cikakkiyar ƙwayar jini (CBC), kididdigar reticulocyte, laodate dehydrogenase (LDH), bilirubin, da kuma haptoglobin. Za'a iya ɗaukar samfurin ɓawon kwayar halitta (biopsy) da kuma bincika a karkashin wani microscope.
Jiyya
Jiyya yana dogara ne akan abin da alamun cututtuka suke. Mutane da yawa ana kula da su tare da steroids (prednisone) kowace rana lokacin da yaduwar ya faru kuma ya canza zuwa wasu kwanaki a lokacin gyarawa. Iron da folic acid sun taimaka wajen maye gurbin asarar sakamakon lalataccen halayen. Wasu mutane na iya karɓar hormones na inrogenic don tayar da samfurin jini. A cikin anemia mai tsanani, yaduwar jini zai iya zama dole. Ƙwararrun jini zai iya amfani dashi don rage yawan ciwon jini. Za a iya magance marasa lafiyar hematopoiesis tare da antithymocyte globulin (ATG).
Kwayar kasusuwa (kwayar sautin) zai iya maye gurbin marasa jini a cikin PNH tare da al'ada. Kusan dukkan mutane da PNH zasu buƙaci dashi don su rayu tsawon lokaci. Idan dashi ya yi cikin marigayi a cikin mummunar cutar ba sakamakon ya kasance mai kyau ba saboda mutum yana da lafiya sosai a wancan lokacin.
Drug na farko don PNH Approved
Ranar 16 ga watan Maris, 2007, Cibiyar Abinci da Harkokin Kiyaye ta Amurka (FDA) ta amince da Soliris (eculizumab) don magance PNH. An amince da wannan yarda akan sakamakon binciken da yawa da suka nuna cewa jiyya tare da eculizumab ya haifar da raguwa mai zurfi a cikin nakasa, kuma kwanakin haemoglobinuria a kowane wata sun rage.
Source
> Parker, Charles, Mitsuhiro Omine, Stephen Richards, et al. "Sanin asali da Gudanarwa na Paroxysmal Nocturnal Hemoglobinuria." Blood 106 (2005): 3699 - 3709.