Matambudziko Anokonzerwa Nechenyu muDzimba Dzizi
Paroxysmal nocturnal hemoglobinuria (PNH) inopa ropa rakagadziriswa sero disorder. Yaiva pakutanga inonzi chiratidzo chayo chemavara-mavara (hemoglobinuria) yakabudiswa mangwanani. Zvaifungidzirwa kuti kuparara kusina kuchena kwemasero matsvuku eropa ( hemolysis ) iyo yakakonzera kuti mutezi werima wakaitika mu bursts (paroxysms) yakaitika usiku. Kutsvakurudza kwakaratidza, zvisinei, kuti hemolysis muPNH, inokonzerwa nehutachiona hwehupenyu, inowanikwa zuva rose uye haiti kuitika mumashure.
Kushandurwa kwemuti weuti kunogona kuitika panguva ipi zvayo asi kunonyanya kunakidza mumuginha weusiku huripo.
Kunzwisiswa kwakagadziriswa kweparoxysmal nocturnal hemoglobinuria yakashandura chirwere ichi seine hemolysis, ropa rinoputika mune mutsinga (thrombosis), uye kushayikwa mukugadzirwa kwemarudzi ese eropa ( hematopoiesis ). PNH inobata vose varume nemadzimai emarudzi ose uye zvinogona kuitika chero ipi zvayo, asi inonyanya kuwanikwa pakati pevechidiki.
Zviratidzo
Vanhu vazhinji vane PNH havana zviratidzo. Zviratidzo zvePNH zvinotarisa kana hemolysis, thrombosis, kana kushaya hematopoiesis iripo.
- Hemolysis - cola-ruvara urine (hemoglobinuria), hemolytic anemia
- Thrombosis - apo chivharo chinoitika chinoita zviratidzo. Kana chivharo chacho chiri mumuputi wechiropa, panogona kunge kune jaundice uye chiropa chakawedzerwa. Mumuviri, chipfeko chinokonzera marwadzo emimba; mumusoro, musoro. Zvivharo muganda zvinokonzera kukwidza, kunorwadza, mutsvuku mutsvuku pane nzvimbo huru dzeganda, dzakadai sekare.
- Haikwanisi hematopoiesis - anemia (kwete masero matsvuku eropa), kunokonzerwa nehutachiona (husina kukwana masero eropa eropa), nekubuda ropa kusina kukwana (kwete maplatelet akakwana).
Kuziva
Iko kune maverengi emakiritsi anoongorora izvo zvinogona kubatsira kuongororwa kweparoxysmal nocturnal hemoglobinuria. Kunyanya kuedza kuedza ropa kuti ione CD55 neCD59, mapurotini maviri.
Kusabatwa kwemapuroteni aya pamasero matsvuku eropa kunotsigira kuongororwa kwePNH. Panguva yekuongororwa, mamwe maitiro eropa achaitwa, kusanganisira ropa rakazara reyero (CBC), reticulocyte count, serum lactate dehydrogenase (LDH), bilirubin, uye haptoglobin. Mushonga wemapfupa (biopsy) inogona kutorwa uye kuongororwa pasi microscope.
Kurapa
Kurapa kunobva pane zviratidzo zviripo. Vanhu vakawanda vakura vanobatwa ne-steroids (prednisone) zuva rega rega kana hemolysis inowanikwa uye ishanduka kuva mazuva akasiyana panguva yekuregererwa. Iron uye folic acid zvinowedzera kubatsira kutsiva kurasikirwa nekuda kwehemolysis. Vamwe vanhu vanogona kugamuchira hormone ye androgenic kuti ikurudzire kushandiswa kwemasero eropa. Muhosha yakakura, kuwedzerwa ropa kunogona kudiwa. Ropa rakapfava rinogona kushandiswa kuderedza kushandiswa kweropa. Haikwanisi hematopoiesis inogona kubatwa ne antithymocyte globulin (ATG).
Pfupa remasero (stem cell) rinofambisa rinogona kutora masero eropa risina kukwana muPNH nemagetsi. Inenge vanhu vose vane PNH vachapedzisira vachida kubviswa kuitira kuti vararame nguva refu. Kana kuwedzerwa kwaitwa munguva pfupi munguva yechirwere mitsva haisi yakanaka nokuti munhu wacho anorwara panguva iyoyo.
Kutanga Mishonga yePNH Yakagamuchirwa
Musi waMarch 16, 2007, US Food and Drug Administration (FDA) yakagamuchirwa Soliris (eculizumab) mukurapa kwePNH. Chibvumirano chacho chakavakirwa pamigumisiro yezviongorori zvakaratidza kuti kurapa ne eculizumab kwakabudisa kuderedza kunoshamisa kwe hemolysis, uye mazuva e hemoglobinuria mwedzi mumwe nomumwe akaderera.
Chinhu
> Parker, Charles, Mitsuhiro Omine, Stephen Richards, et al. "Kuongororwa uye Kutungamirirwa kweParoxysmal Nocturnal Hemoglobinuria." Ropa 106 (2005): 3699 - 3709.