Nephrogenic Systemic Fibrosis

Ko te nephrogenic systemic fibrosis he mate onge e puta ana te nuinga ki te hunga e pangia ana e te ngoikore o te whatukuhu i te taumata teitei, ahakoa kei te mahi rongoā toto ranei. Ka rite pea te nephrogenic systemic fibrosis ki ētahi mate kiri, pēnei i te scleroderma me te scleromyxedema, me te whākōkī me te pōuri e whanake ana i ngā wāhi nui o te kiri.

Ka pā ano te nephrogenic systemic fibrosis ki ngā whekau o roto, pēnei i te ngākau me ngā pūkahukahu, ā, ka taea e ia te whakaiti i te roa o ngā uaua me ngā uaua i roto i ngā hononga (hononga hononga).

Mō ētahi o te hunga e pangia ana e te mate whatukuhu i te taumata teitei, ko te whakamahinga o ngā kaiwhakaatu whakapae-pūmua gadolinium tawhito (rōpū 1) i te wā o te whakaahua pūmanawa maikuku (MRI) me ētahi atu rangahau whakaahua kua kitea he take whakaoho mō te whanaketanga o tēnei mate. Kua whakaitihia te maha o ngā nephrogenic systemic fibrosis i te kitenga o tēnei hononga. Kāore ngā kaiwhakaatu whakapae-pūmua gadolinium hou (rōpū 2) e hono atu ki te tūponotanga nui ake o te nephrogenic systemic fibrosis.

Ka taea te tīmata o te nephrogenic systemic fibrosis i ngā rā, ngā marama, ā, tae noa ki ngā tau, i muri i te whakamahinga o te rongoā whakaatu-whakaahua e whakamahia ana te gadolinium (rōpū 1). Ko ētahi o ngā tohu me ngā pūāhua o te nephrogenic systemic fibrosis ka uru ki: \n\n- Te pupuhi me te pūmau o te kiri\n- Ngā wahi whero, kē, pango rānei o te kiri\n- Te māngō me te pakari o te kiri, te nuinga i ngā ringa me ngā waewae, ā, i etahi wā i te tinana, engari kaore noa i te mata, i te ūtū rānei\n- Te kiri ka rongo "rākau" ka puta he ahua o te kiri kāreti\n- Te tahuna, te ngau, te mamae nui rānei i ngā wāhi e pā ana\n- Te māngō o te kiri e ārai ana i te nekehanga, ka ngaro ai te kōpū o ngā hononga\n- Ko ētahi wā, ngā pupuhi, ngā whekau rānei\n\nI ētahi tāngata, ko te whakauru o ngā uaua me ngā kōpū o te tinana ka pā: \n\n- Te ngoikore o ngā uaua\n- Te whakaiti i te nekehanga o ngā hononga e puta mai ana i te pūmau o ngā uaua (ngā whakaheke) i ngā ringa, ngā ringaringa, ngā waewae me ngā waewae\n- Te mamae o ngā wheua, ina koa i ngā wheua huha, i ngā iwi rānei\n- Te whakaiti i te mahi o ngā kōpū o roto, tae atu ki te ngakau, te manawa, te diaphram, te pūnaha kai, te ate rānei\n- Ngā papa kowhai i te mata ma (sclera) o ngā kanohi\n\nKo te āhuatanga he roa te wā (mate pūmau), engari ka pai ake pea ētahi tāngata. I ētahi wā, ka taea e ia te whakaeke i te ngoikore nui, tae noa ki te mate.

Kaore he uauatanga

Kāore i tino mōhiotia te take tino o te nephrogenic systemic fibrosis. Ka hanga te kiko honohono fibrous i roto i te kiri me ngā kiko honohono, ka hua ai te tohe o te kiko puta noa i te tinana, te nuinga o te kiri me ngā kiko subcutaneous.

Kua kitea te whakamahinga o ngā kaiwhakaatu rerekē-pūtaiao-gadolinium tawhito (rōpū 1) i te wā o te whakaahua whakaata pūtaiao (MRI) hei kaiwhakaoho mō te whanaketanga o tēnei mate i roto i te hunga e pangia ana e te mate whatukuhu. E whakaarohia ana ko te nui ake o te tūponotanga e hono ana ki te kaha iti o ngā whatukuhu ki te tango i te kaiwhakaatu rerekē mai i te toto.

Ka tūtohu te Food and Drug Administration (FDA) kia kaua e whakamahia ngā kaiwhakaatu rerekē-pūtaiao-gadolinium tawhito (rōpū 1) i roto i te hunga e pangia ana e te whara whatukuhu koi, te mate whatukuhu roa rānei.

Ka taea e ētahi atu āhuatanga te whakanui ake i te tūponotanga o te nephrogenic systemic fibrosis ina honoa ki te mate whatukuhu kei reira, me te whakamahinga o ngā kaiwhakaatu rerekē-pūtaiao-gadolinium tawhito (rōpū 1), engari kāore i te tino mōhiotia te hononga. Kei roto i ēnei:

• Te whakamahinga o te erythropoietin (EPO) nui, he homoni e whakatairanga ana i te hanga o ngā pūtau whero toto, te nuinga o te wā e whakamahia ana hei whakaora i te mate toto iti
• Te pokanga toto hou
• Ngā raruraru toto-whakakino
• Te mate kino

Ko te tino morearea o te nephrogenic systemic fibrosis i muri i te whakamahinga o ngā kaiwhakaatu whakaaturanga konupūmā tawhito (rōpū 1) ka puta ki ngā tāngata e:

• He mate whatukuhu o te taumata waenga ki te kino
• Kua whakawhiti whatukuhu, engari he ngoikore te mahi o te whatukuhu
• Kei te whakamahi i te hemodialysis, te peritoneal dialysis rānei
• He whara whatukuhu pakari

Kaore he uauatanga

Ko te whakakorenga i ngā kaiwhakaatu whakawhāiti-konupūmā tawhito (rōpū 1) te mea nui hei aukatinga i te nephrogenic systemic fibrosis, i te mea he ātete ake ngā kaiwhakaatu whakawhāiti-konupūmā hou (rōpū 2) ā, kāore e hono atu ki te tūponotanga kē.

Ko te taatai o te nephrogenic systemic fibrosis ka mahia me:

• Arotake tinana mo nga tohu me nga tohu o te mate, me te aromatawai mo te hitori pea o te MRI ma te whakamahi i te taonga whakaatu-i-te-gadolinium ina he mate whatukuhu teitei
• Tauira o te kiko (biopsy) i tangohia mai i te kiri me te uaua
• Etahi atu whakamatautau kia rite ai ka taea te tohu i te urupare o nga uaua me nga whekau o roto

Kāore he rongoā mō te nephrogenic systemic fibrosis, ā, kāore he rongoā e angitu tonu ana ki te whakamutu, ki te whakahuri rānei i te whanake o te mate. Ko te nephrogenic systemic fibrosis te tino iti te puta, nā reira ka uaua te mahi i ngā rangahau nui.

Kua whakaatu etahi rongoā i te angitu iti ki ētahi tangata e pangia ana e te nephrogenic systemic fibrosis, engari he nui ake te rangahau e hiahiatia ana kia taea ai te whakatau mena ka āwhina ēnei rongoā:

• Hemodialysis. Ki ngā tāngata e pangia ana e te mate whatukuhu kino roa e whiwhi ana i te hemodialysis, ko te mahi hemodialysis i muri tonu i te whiwhi i te taonga whakaatu-pūtau-gadolinium ka taea te whakaiti i te tūponotanga o te nephrogenic systemic fibrosis.
• Whakaora tinana. Ko te whakaora tinana e āwhina ana ki te whakawhāiti i ngā waewae e pā ana ka āwhina pea ki te whakaiti i te whanake o ngā hononga hono, ā, kia mau tonu te nekehanga.
• Nehua whatukuhu. Ki ngā tāngata he hunga e tika ana, ko te whakapai ake o te mahi whatukuhu nā te nehua whatukuhu ka āwhina pea ki te whakapai ake i te nephrogenic systemic fibrosis i te wā.
• Extracorporeal photopheresis me te ultraviolet A. Ko tēnei rongoā e whakauru ana i te tango i te toto ki waho o te tinana me te whakaora i te toto ki te rongoā e whakapakari ana ki te mārama ultraviolet. Katahi ka whakatārewatia te toto ki te mārama ultraviolet, ā, ka whakahokia ki te tinana. Kua whakaatu etahi tangata i te whakapainga i muri i te whiwhi i tēnei rongoā.

He whakamātautau ēnei rongoā, engari kāore i te whakamahia inaianei. Kua whakaaturia e rātou te āwhina ki ētahi tangata, engari ka whakatauritea te whakamahinga o rātou e ngā pānga taha:

• Imatinib (Gleevec). Ahakoa ka whakaatu tēnei rongoā i ētahi tumanako ki te whakaiti i te māngere me te pūngāwerewere o te kiri, he nui ake te rangahau e hiahiatia ana.
• Pentoxifylline (Pentoxil). He iti te angitu me tēnei rongoā, e whakaarohia ana e whakaiti ana i te māngere me te piri (viscosity) o te toto, ā, e āwhina ana i te rere toto. He nui ake te rangahau e hiahiatia ana.
• Sodium thiosulfate. Kua whakaaturia te painga pea mā te whakamahi i tēnei rongoā, engari he nui ake te rangahau e hiahiatia ana.
• High-dose intravenous immune globulin. Kua whakaaturia te painga pea mā te whakamahi i tēnei rongoā, engari he nui ake te rangahau e hiahiatia ana.

Kaore he uara (NaN)