Chii Alport chirwere?

Alport chirwere chirwere nhaka, iyo inozivikanwa nokuva anotevedzana zvakananga rutapudzo pamusoro renal mashandiro pamwe zvinetso kunzwa kana kunyange chiratidzo. Parizvino, munyika yedu mhando iyi chirwere pakati pavana (zvikurukuru) a contingent anenge 17: 100 000.

The zvikonzero

Maererano nenyanzvi, Alport chirwere kunokonzerwa nokuda abnormalities ari Gene, riri munzvimbo refu ruoko X chromosome mune dzinonzi 21-22q mapazi. Mukuwedzera, kutyora vainzi chinokosha maumbirwo collagen mhando 4 ndiyewo mhaka chirwere. Under collagen zvinonzwisiswa zviri zvesayenzi ane puroteni yakadaro iri zvakananga chezvinonhuwira connective zvinyama izvo chinopa elasticity uye akaramba.

zviratidzo

Alport chirwere rinowanzova kutanga kunoita pacharo akanzwa vana vane makore kubvira mashanu gumi makore uye kunoratidzwa muchimiro hematuria (ropa iri weti kuwana). Kakawanda, chirwere ichi chinowanikwa pavaidira, kureva, unotevera kuongororwa pedyo mazvikokota. Uyezve, Alport chirwere kunoratidza pacharo somunhu anonzi dizembriogeneza stigmas. Uyu pashoma zvarakasiya kuti regai vane basa rinokosha iri kushanda huru mugadziriro muviri. Doctors vanoti epikant (diki crease panguva yomukati kukona ziso), mukuru denga, zvishomanana deformation zvose nzeve nezvimwe zviratidzo. Kuwirirana kusanzwa kuri chiratidzo chirwere chokwadi, uye kusanzwa kunokwanisa kuonekwa kakawanda vakomana. Zvose pamusoro zviratidzo kazhinji iratidzike kuyaruka, apo zvakafanana chisingaperi renal kukundikana anoita pacharo ainzwa chete panguva vayaruka.

diagnosing

Alport chirwere muvana Kazhinji chirwere kwakavakirwa kuvapo zvirwere Data mune dzimwe nhengo dzemhuri mhando iyi. Somuenzaniso, kusimbisa chirwere zvakakwana chienderane vatatu nzira shanu dziri pazasi:

• kunzwa kurasikirwa;
• kufa zviitiko chisingaperi renal kukundikana nehama;
• kusimbisa hematuria mumhuri;
• ziso zvokurwara;
• kuvapo dzakananga kuchinja panguva itsvo biopsy.

mabatiro

Pasina marapiro, vanachiremba vanofanira kutanga kuderedza kuchiita itsvo kukundikana. With chirwere ichi sezvo Alport nechirwere, vana zvaisambobvumirwa muviri, izvozvo prescribes zvinovaka muviri. nzeve Great iri rwakabhadharwa kuna anonzi kunaya noutachiona foci. Kushandiswa pakurapa cytostatics uye siyana zvinodhaka hormone kunosimudzira kuvandudzika. Zvisinei, kazhinji basa iri itsvo nokuisazve sezvo chizivano. Zvinofanira kucherechedzwa kuti kuziva miedzo haematuria pasina nouchapupu kusanzwa rwevanhu zvishoma nani. weboka chirwere fungidziro. Mumamiriro ezvinhu ine renal kukundikana mhando iyi panoonekwa chaizvo kashoma.