Ingxaki iqhutywe yiNgcembelelo kwiCells Cell
I-Paroxysmal nocturnal hemoglobinuria (i-PNH) igazi elifunyenwe lihlaselwe isifo senxeba. Kwakuqala kuthiwa uphawu lwaso lomnayile omnyama (i-hemoglobinuria) evezwa ekuseni. Kwakucatshangelwa ukuba ukutyhabalalisa ngokwemvelo kwamaseli obomvu (i- hemolysis ) eyabangela ukuba umchamo omnyama uqhutywe kwi-bursts (i-paroxysms) eyenzeka ebusuku. Uphando luye lwabonisa, nangona kunjalo, ukuba i-hemolysis e-PNH, ebangelwa i-defect ye-biochemical, iyenzeka yonke imihla kwaye ayikho ebonakalayo.
Utshintsho lombala we-urine luya kwenzeka nanini na kodwa luyinto ephawulekayo kumchamo wobusuku obugxilile.
Ukuqonda okuphuculweyo kwe-paroxysmal nocturnal hemoglobinuria kuye kwahlengahlengisa i-disorder njengokuba ne-hemolysis, i- blood clots kwiimvini (thrombosis), kunye nokusilela kwimveliso yeentlobo zeeseli zegazi (i- hematopoiesis ). I-PNH ichaphazela bonke abesilisa kunye nabesifazane bazo zonke iintlobo zeemvelaphi kwaye zingenzeka nanini nayiphi na ubudala, kodwa ixhaphaka kakhulu kubantu abadala.
Iimpawu
Abantu abaninzi abane-PNH abanalo iimpawu. Iimpawu ze-PNH zixhomekeke ekubeni i-hemolysis, thrombosis, okanye i-hematopoiesis engenalo ikhoyo.
- I-Hemolysis - umchamo we-cola-color (hemoglobinuria), i-hemolytic anemia
- I-Thrombosis - apho i-clot ivela khona iqinisekisa iimpawu. Ukuba i-clot isesigxeni sesibindi, kunokuthi kube ne-jaundice kunye nesibindi esandisiweyo. Kwisisu, i-clot ibangela iintlungu zomzimba; entloko, intloko. Iingubo zesikhumba zibangele ziphakanyiswe, zibuhlungu, zibomvu iziqhumane kwiindawo ezinkulu zesikhumba, ezifana nasemva.
- I-hematopoiesis engapheliyo-i- anemia (engenayo ngokwaneleyo iiseli zegazi), ukufumana ukutheleleka (ukungabikho okwaneleyo ngamaseli egazi ezimhlophe), kunye negazi elingavamile (iiplatelet ezaneleyo).
Ukuxilongwa
Kukho iimvavanyo ezininzi zelabhoratri ezingancedisa ukuxilongwa kwe-paroxysmal nocturnal hemoglobinuria. Eyona nto ichanekileyo ihlola igazi ukuze ihlole i-CD55 ne-CD59, iiprotheni ezimbini.
Ukungabikho kwezi proteins kwiiseli ezibomvu zegazi kuqinisekisa ukuxilongwa kwe-PNH. Ngexesha lokuxilongwa, ezinye iimvavanyo zegazi ziya kwenziwa kunye nokubalwa kweeseli zegazi (CBC), inani lama-reticulocyte, i-serum lactate dehydrogenase (LDH), i-bilirubin kunye ne-haptoglobin. Isampula somnatha wethambo (i-biopsy) ingathathwa kwaye ihlolwe phantsi kwe-microscope.
Unyango
Utyando lu sekelwe kwiimpawu ezikhoyo. Abantu abaninzi abadala baphathwa nge-steroids (prednisone) imihla ngemihla xa i-hemolysis ivela kwaye yatshintshela kwiintsuku ezongezelelweyo ngexesha lokuxolelwa. I-Iron kunye ne-folic acid supplement help help replaces losses due to hemolysis. Abanye abantu banokufumana i-hormone ye-androgenic ekuvuseleleni ukuveliswa kweeseli zegazi. Kwi-anemicia ephezulu, ukunyelwa igazi kunokufuneka. I-Blood thinners ingasetyenziselwa ukunciphisa ukuphuhliswa kwamacandelo egazi. I-hematopoiesis engapheliyo ingaphathwa nge-antithymocyte globulin (ATG).
Ukufakelwa komnxeba wethambo (stem cell) ukutshintshwa kungathatha iiseli zegazi ezingapheleleyo kwi-PNH eziqhelekileyo. Phantse bonke abantu abanePNH baya kufuna ukutshintshwa ukuze baphile ixesha elide. Ukuba ukufakelwa kwenziwa emva kwexesha le sifo iziphumo azikho kakuhle kuba umntu ugula kakhulu ngeli xesha.
Imithi yokuqala yePNH ivunywe
Ngo-Matshi 16, 2007, i-US Food and Drug Administration (i-FDA) ivume iSoris (eculizumab) kunyango lwe-PNH. Ukuvunyelwa kusekelwe kwimiphumo yezifundo eziliqela ezibonisa unyango nge-eculizumab zenze ukunciphisa ngokuphawulekayo kwe-hemolysis, kwaye iintsuku ze-hemoglobinuria inyanga nganye zinciphile.
Umthombo
> Parker, uCharles, uMitsuhiro Omine, uStephen Richards, et al. "Ukuxilongwa kunye noLawulo lwe-Paroxysmal Nocturnal Hemoglobinuria." Igazi 106 (2005): 3699 - 3709.