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Fibrosis Yekemuviri Inokonzerwa Neitsvo

Pfupiso

Nephrogenic systemic fibrosis chirwere chisingawanzoitika chinoitika kunyanya kune vanhu vane chirwere cheitsvo chakakomba vane kana vasina dialysis. Nephrogenic systemic fibrosis inogona kufanana nezvirwere zve ganda, sekuscleroderma ne scleromyxedema, nekukora nekureruka kuchizivisa munzvimbo huru dze ganda.

Nephrogenic systemic fibrosis inogona kukanganisawo nhengo dzemukati, dzakadai semoyo nemapapu, uye inogona kukonzera kushomeka kwekushanda kwemhasuru nematendon mu majoini (joint contracture).

Kune vamwe vanhu vane chirwere cheitsvo chakakomba, kuiswa pachena kune gadolinium-based contrast agents ekare (boka 1) panguva yekuongororwa kwe magnetic resonance imaging (MRI) nemamwe miedzo yekuongorora kwave kuchizivikanwa sechinhu chinokonzera kukura kwechirwere ichi. Kuziva kwehukama uhu kwaderedza zvikuru huwandu hwe nephrogenic systemic fibrosis. Gadolinium-based contrast agents itsva (boka 2) haina hukama nekuwedzera njodzi ye systemic nephrogenic fibrosis.

Zviratidzo

Nephrogenic systemic fibrosis inogona kutanga mazuva kusvika kumwedzi, kunyange makore, mushure mekubatwa nechirwere chegadolinium-based contrast agent (boka 1). Zvimwe zviratidzo nezvirwere zve nephrogenic systemic fibrosis zvinogona kusanganisira:

  • Kuruma nekukombama kwepande
  • Mapachi akatsvuka kana rima pachiso
  • Kukora nekukomba kwepande, kazhinji pazvibhakera nemaoko uye dzimwe nguva pamuviri, asi zvisingawanzoitika pachiso kana musoro
  • Pande rinogona kunzwa "semuti" uye kuita kutaridzika kweorenji-peel
  • Kupisa, kukwenya kana marwadzo akasimba munzvimbo dzakabatwa
  • Kukora kwepande kunodzivisa kufamba, zvichikonzera kurasikirwa nekufamba kwemashure
  • Zvisingawanzoitika, mavhundutso kana maronda

Mukati vanhu vamwe, kubatanidzwa kwemisungo nemiviri yemuviri kunogona kukonzera:

  • Kushaya simba retsandanyama
  • Kuganhurirwa kwekutambanudza kwemashure kunokonzerwa nekukombama kwemhasuru (contractures) mumaoko, maoko, makumbo nemakumbo
  • Kurwadziwa kwemapfupa, kunyanya mumapfupa echitubu kana mativi
  • Kuderedzwa kwemabasa emukati, kusanganisira moyo, mapapu, diaphragm, gastrointestinal tract kana chiropa
  • Mapachi machena pamusoro pemvere (sclera) meziso

Iyi mamiriro acho kazhinji nguva refu (chronic), asi vamwe vanhu vanogona kuvandudza. Muvanhu vashoma, inogona kukonzera kutadza kwakakomba, kunyange rufu.

Zvinokonzera

Chikonzero chaicho che nephrogenic systemic fibrosis hachizivisike zvizere. Tishu inobatanidza fibrous inoumbwa paganda nemuma tissues anobatanidza, zvichiguma nekukuvadzwa kwetshu mumuviri wose, kazhinji paganda nemuma tissues ari pasi peganda.

Kuonekwa kwezvinhu zvegadolinium-based contrast zvekare (boka 1) panguva yekuongororwa kwe magnetic resonance imaging (MRI) kwakaonekwa sechinhu chinokonzera kukura kwechirwere ichi muvanhu vane chirwere cheitsvo. Njodzi iyi yakawedzera inofungidzirwa kuti ine hukama nekukwanisa kwakaderera kweitsvo kubvisa chinhu chekuenzanisa kubva muropa.

Food and Drug Administration (FDA) inokurudzira kudzivisa zvinhu zvegadolinium-based contrast zvekare (boka 1) muvanhu vane acute kidney injury kana chronic kidney disease.

Pfungwa dzimwe dzinogona kuwedzera njodzi ye nephrogenic systemic fibrosis kana dzakabatana nechirwere cheitsvo uye kuonekwa kwezvinhu zvegadolinium-based contrast zvekare (boka 1), asi hukama hahusi chokwadi. Idzi dzinosanganisira:

  • Kushandiswa kwe erythropoietin (EPO) yakawanda, homoni inokurudzira kugadzirwa kwemasero matsvuku eropa, kazhinji inoshandiswa kurapa anemia
  • Kuongororwa kwevascular kwapfuura
  • Matambudziko ekugwamba kweropa
  • Hutachiona hwakakomba
Zvinhu zvinokonzera njodzi

Ngozi yakanyanya yekunephrogenic systemic fibrosis mushure mekubatwa nemagadolinium-based contrast agents ekare (boka 1) inoitika kune vanhu vane:

  • Hosha yekidney ine mwero kusvika yakakomba
  • Vakawana kusimudzwa kwekidney asi vane basa reitsvo rakashata
  • Vari kugamuchira hemodialysis kana peritoneal dialysis
  • Vane kukuvara kweitsvo kwenguva pfupi
Kudzivirira

Kudzivisa mishonga yekuwedzera ine gadolinium yekare (boka 1) ndiko kuri nyore kudzivirira nephrogenic systemic fibrosis, sezvo mishonga mitsva yekuwedzera ine gadolinium (boka 2) iri yakachengeteka uye haina hukama nekuwedzera njodzi.

Kuongorora

Kuongororwa kwe nephrogenic systemic fibrosis kunoitirwa ne:

  • Kuongororwa kwemuviri kutsvaga zviratidzo nezvikonzero zveronda, uye kuongorora nhoroondo inobvira ye MRI ichishandisa mushonga unotarisa gadolinium kana chirwere cheitsvo chakakura chapo
  • Sampuli yetsinga (biopsy) inotorwa kubva paganda nekumhasuru
  • Kuongororwa kwakawanda sezvinodiwa kunogona kuratidza kuita kwemhasuru nemidziyo yemukati
Kurapa

Hapana mushonga we nephrogenic systemic fibrosis, uye hapana kurapwa kunobudirira nguva dzose mukumirira kana kudzosera kufambira mberi kwechirwere ichi. Nephrogenic systemic fibrosis inoitika zvishoma, zvichiita kuti zviome kuita zvidzidzo zvikuru.Kurapwa kwakati kwakaratidza kubudirira kushoma muvanhu vakawanda vane nephrogenic systemic fibrosis, asi kunodiwa tsvakurudzo yakawanda kuti zvizive kana kurapwa uku kuchibatsira:

  • Hemodialysis. Muvanhu vane chirwere cheitsvo chekusingaperi chakakura vari kugamuchira hemodialysis, kuita hemodialysis nekukurumidza mushure mekugamuchira gadolinium-based contrast agent kunogona kuderedza mukana we nephrogenic systemic fibrosis.
  • Kurapa kwemuviri. Kurapa kwemuviri kunobatsira kukwesha nhengo dzakabatwa kunogona kubatsira kudzikisira kufambira mberi kwezvikwata zvejoini uye kuchengetedza kufamba.
  • Kusimudzirwa kweitsvo. Kune vanhu vakakodzera, kugadziriswa kwemabasa eitsvo nekuda kwekusimudzirwa kweitsvo kunogona kubatsira kugadzirisa nephrogenic systemic fibrosis nekufamba kwenguva.
  • Extracorporeal photopheresis ne ultraviolet A. Kurapwa uku kunosanganisira kubvisa ropa kunze kwemuviri uye kurapa ropa nemushonga unoita kuti rive rakajeka kune chiedza che ultraviolet. Ropa rinobva raburukirwa nechiedza che ultraviolet uye rinodzoserwa mumuviri. Vanhu vakati vakaratidza kugadziriswa mushure mekugamuchira kurapwa uku.

Mishonga iyi iri kuyedzwa, asi haisi kushandiswa ikozvino. Yakaratidza kubatsira vanhu vakati, asi migumisiro inogumira kushandiswa kwayo:

  • Imatinib (Gleevec). Kunyangwe kurapwa uku kuchiratidza vimbiso mukuderedza kuwanda uye kuomarara kwe ganda, kunodiwa tsvakurudzo yakawanda.
  • Pentoxifylline (Pentoxil). Kune kubudirira kushoma nemushonga uyu, unofungidzirwa kuderedza kuwanda uye kunamatira (viscosity) kweropa, kubatsira kuyerera. Kunodiwa tsvakurudzo yakawanda.
  • Sodium thiosulfate. Kubatsira kunobvira kwakaratidzwa uchishandisa mushonga uyu, asi kunodiwa tsvakurudzo yakawanda.
  • High-dose intravenous immune globulin. Kubatsira kunobvira kwakaratidzwa uchishandisa mushonga uyu, asi kunodiwa tsvakurudzo yakawanda.

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