Nephrogenic Systemic Fibrosis

I-nephrogenic systemic fibrosis yisifo esingavamile esihlala kakhulu kubantu abanesifo sokwehluleka kwesibindi esikhulile, kungakhathaliseki ukuthi banokwelashwa nge-dialysis noma cha. I-nephrogenic systemic fibrosis ingafana nezifo zesikhumba, njenge-scleroderma ne-scleromyxedema, lapho ukuqina nokukhanya kuvela ezindaweni ezinkulu zesikhumba.

I-nephrogenic systemic fibrosis ingathinta namanye amalungu omzimba, njengentliziyo nemiphunga, futhi ingabangela ukufinyeka okukhubaza imisipha nemisipha yemisipha ezinhlangothini (ukuncika kwamajozi).

Kubantu abanye abanesifo sesibindi esikhulile, ukubonisana nezikhali ze-gadolinium-based contrast agents (iqembu 1) ngesikhathi sokwenza i-magnetic resonance imaging (MRI) nezinye izifundo zokwenza izithombe kuye kwabonakala njengokuthinta ukuthuthukiswa kwalesi sifo. Ukuqaphela lobu buhlobo kuye kwancishisa kakhulu inani le-nephrogenic systemic fibrosis. Izidakamizwa ezintsha ze-gadolinium-based contrast agents (iqembu 2) azihambisani nokungena engozini enkulu ye-nephrogenic systemic fibrosis.

I-nephrogenic systemic fibrosis ingaqala ezinsukwini ezimbalwa kuya ezinyangeni, ngisho naseminyakeni, ngemva kokubekwa ekuxhumaneni nomkhiqizo wokuhlola osekhulile obunomkhiqizo we-gadolinium (iqembu 1). Amanye ama-sign nama-symptoms e-nephrogenic systemic fibrosis angafaka:

• Ukukhula nokuqina kwesikhumba
• Izicucu ezibomvu noma ezimnyama esikhumbeni
• Ukukhuluphala nokuqina kwesikhumba, ngokuvamile ezingalweni nasemiileni, ngezinye izikhathi emzimbeni, kodwa cishe akukaze kube ebusweni noma ekhanda
• Isikhumba esingase sizizwe "njengomgqomo" futhi sibe nobukhulu obubomvu
• Ukushisa, ukukhahlela noma ubuhlungu obukhulu obukhulu ezindaweni ezihilelekile
• Ukukhuluphala kwesikhumba okuvimba ukunyakaza, okuholela ekwehleni kokuguquguquka kwamajozi
• Ngokungavamile, ama-blisters noma ama-ulcers

Kwabanye abantu, ukuhileleka kwemisipha nezingxenye zomzimba kungabangela:

• Ubuthakathaka bemisipha
• Ukulinganiselwa kokunyakaza kwamajozi okubangelwa ukuqina kwemisipha (ama-contractures) ezingalweni, ezandleni, ezinyaweni nasezinyaweni
• Ubuhlungu lwamathambo, ikakhulukazi emathanjeni noma eziphahleni
• Ukusebenza okunciphisiwe kwezingxenye zomzimba yangaphakathi, kufaka phakathi inhliziyo, amaphaphu, i-diaphragm, umgudu wokugaya ukudla noma isibindi
• Ama-plaque aphuzi ebusweni obumhlophe (sclera) amehlo

Isimo ngokuvamile sihlala isikhathi eside (esingapheli), kodwa abanye abantu bangase bathuthuke. Kubantu abambalwa, kungabangela ukukhubazeka okukhulu, ngisho nokufa.

Imbangela eqondile ye-nephrogenic systemic fibrosis ayiqondakali ngokuphelele. I-fibrous connective tissue yakha oluswini nakwi-connective tissues, okuholela ekwenzakaleni kwezicubu emzimbeni wonke, ikakhulu oluswini nakwi-subcutaneous tissues.

Ukubonisana ne-gadolinium-based contrast agents endala (iqembu 1) ngesikhathi se-magnetic resonance imaging (MRI) kuye kwabonakala njengokuthinta ukuthuthukiswa kwalesi sifo kubantu abanesifo sezinso. Lo mongo wokwanda kuthiwa uxhumekile ekubeni izinso zinekhono eliphansi lokususa i-contrast agent egazini.

I-Food and Drug Administration (FDA) iphakamisa ukugwema i-gadolinium-based contrast agents endala (iqembu 1) kubantu abanesifo sezinso esibuhlungu noma i-chronic kidney disease.

Ezinye izimo zingandisa ingozi ye-nephrogenic systemic fibrosis uma zidibene nesifo sezinso esikhoyo nokubonisana ne-gadolinium-based contrast agents endala (iqembu 1), kodwa uxhumano alucaci. Lezi zihlanganisa:

• Ukusetshenziswa kwe-high-dose erythropoietin (EPO), i-hormone ekhuthaza ukukhiqizwa kwamaseli ebomvu egazi, evame ukusetshenziswa ekwelapheni i-anemia
• Ukuhlinzwa kwesicubu segagasi samuva nje
• Izinkinga zokugqwala kwegazi
• Ukutheleleka okukhulu

Ingozi enkulu kunazo zonke ye-nephrogenic systemic fibrosis ngemva kokubekwa ezingozini ze-gadolinium-based contrast agents (iqembu 1) kwenzeka kubantu abalandelayo:

• Abane-kidney disease ephakathi nendawo
• Abake baba nokuhlinzwa kwe-kidney kodwa banokusebenza okungathandeki kwe-renal
• Abathola i-hemodialysis noma i-peritoneal dialysis
• Abane-acute kidney injury

Ukugwema ukusetshenziswa kwezinto ezikhuthazayo ezikhuluma i-gadolinium ezindala (iqembu 1) kubalulekile ekuvinjeni i-nephrogenic systemic fibrosis, njengoba izinto ezikhuthazayo ezikhuluma i-gadolinium ezintsha (iqembu 2) ziphephile futhi azixhunyaniswanga nenkinga eyenzeka ngokwanda.

Ukuxilongwa kwe-nephrogenic systemic fibrosis kwenziwa ngokusebenzisa:

• Uvivinyo lomzimba lokuqaphela izimpawu nezibonakaliso zesifo, kanye nokulinganisa umlando onokwenzeka we-MRI usebenzisa i-gadolinium-based contrast agent uma kukhona isifo esithuthukile sezinso
• Isibonelo sethusi (i-biopsy) esithathwe oluswini nakemisipha
• Ezinye izivivinyo ezidingekayo ezingabonisa ukuhileleka kwemisipha namalungu angaphakathi