Uri kusangana nematambudziko neitsvo here? Ngatikurukurei nezveFanconi Syndrome!

Unogara uchinzwa nyota isingafungidzike here? Kana kuti kana ari mwana mudiki, wamboona kurwadziwa mumuviri wake uye mapfupa asina simba here? Dzimwe nguva, shure kwezviratidzo izvi, panogona kunge paine chirwere chatisati tanzwa zvakawanda nezvacho, asi zvakakosha kuziva nezvacho. Chimwe chezvirwere zvakadaro iFanconi Syndrome. Nhasi, tichataura nezvazvo zviri nyore, nenzira yaunogona kunzwisisa.

Chii chinonzi Fanconi Syndrome?

Zvichitaurwa zviri nyore, chirwere cheFanconi chirwere chinoitika kana nzira dzakaoma dzemigero muitsvo dzedu, kunyanya proximal tubules, dzisingashande zvakanaka. Zvino funga nezvazvo nenzira iyi: itsvo dzedu dzakaita se super filter system mumuviri wedu. Dzinosefa ropa uye dzinobvisa tsvina seweti. Dzinotorawo zvinovaka muviri zvakakosha, zvakaita se electrolytes ne glucose.

Zvisinei, muitsvo dzemunhu ane Fanconi syndrome, aya anonzi ma proximal tubules haashande nemazvo. Chinoitika ndechekuti zvinhu zvakakosha mumuviri hazvidzokeri mumuviri uye zvinoburitswa muweti. Nemamwe mashoko, zvinhu zvinokosha zvinoraswa.

Pakati pezvinhu zvakakosha zvinoburitswa mumuviri nenzira iyi, zvinotevera zvinonyanya kuwanikwa:

• Fosforasi
• Glucose
• Potassium
• Bicarbonate
• Asidhi yeUric
• Amino acids

Zvinhu izvi zvinodiwa pakuita kwese kwese mumuviri wedu. Saka, kana izvi zvapera, matambudziko anotanga kumuka.

Ndiani anogona kuwana chirwere cheFanconi?

Ichi chirwere chinogona kukanganisa chero munhu. Pane nzira mbiri huru dzachinogona kuitika nadzo.

1. Inherited Fanconi Syndrome: Ichi chirwere chinokonzerwa nemajini, zvichireva kuti chinotorwa kubva kuna amai kana baba.

2. Acquired Fanconi Syndrome: Chirwere ichi chinogona kuitika pane imwe nguva muhupenyu, nekuda kwezvimwe zvikonzero.

Ndezvipi zviratidzo zveFanconi syndrome?

Zviratidzo zvinogonawo kusiyana zvishoma zvichienderana nekuti ndezvokuzvarwa nazvo here kana kuti zvakawanikwa.

Zviratidzo zvechirwere cheFanconi chekuzvarwa nacho:

• Kuita weti kakawanda: Kubuda weti yakawanda kupfuura yakajairika.
• Kushaya mvura mumuviri: Kushaya mvura mumuviri.
• Nyota inogara iripo (Polydipsia): Kunzwa sekuti hausi kuwana mvura yakakwana zvisinei nekuti unonwa mvura yakawanda sei.
• Kurwadziwa kwemapfupa: Unogona kunzwa kurwadziwa mumuviri wako, kunyanya mumapfupa ako.
• Kushaya simba kwetsandanyama.
• Mapfupa asina simba: Mapfupa anogona kupwanyika uye kutyoka zviri nyore.
• Kutyoka kwemapfupa: Kunyangwe kudonha kudiki kunogona kukonzera kuti pfupa rityoke.
• Kureba kudiki: Ungave mupfupi pane vamwe vezera rako.

Zviratidzo zveFanconi syndrome zvinotevera:

• Kushaya simba kwetsandanyama.
• Kushomeka kwephosphate muropa (Hypophosphatemia): Izvi zvinogona kukonzera matambudziko emapfupa.
• Kuderera kwepotassium muropa (Hypokalemia): Izvi zvinogonawo kukanganisa kurova kwemoyo.
• Hyperaminoaciduria ndiko kuvapo kwemaamino acids akawanda muweti.
• Kuwedzera kweacidity mumuviri (Metabolic acidosis): Izvi zvinogona kukonzera kuneta uye kuoma kufema.
• Kuita weti kakawanda.
• Kushaya mvura mumuviri.
• Nyota nguva dzose.

Iye zvino wava kuona kuti zvimwe zvezviratidzo izvi zvakafanana, saka zvakanaka kutsvaga mazano echiremba kuti uzive zviri kuitika.

Chii chinokonzera chirwere cheFanconi?

Panogona kunge paine zvikonzero zvakawanda. Ngatizviparadzanisei muzvikamu zviviri.

Zvikonzero zvekuzvarwa kweFanconi syndrome:

Izvi zvinowanzova zvirwere zvemajini.

• Cystinosis: Izvi zvinokonzerwa nekuunganidzwa kweamino acid cystine mumuviri. Inogona kukanganisa nhengo dzakawanda dzemuviri, kusanganisira itsvo, maziso, tsandanyama, mwoyo, neuropi. Ndiyo inonyanya kukonzera chirwere chekuzvarwa nacho cheFanconi.
• Lowe syndrome: Ichi chirwere chisingawanzoitiki chemajini chakabatana neX chromosome. Chinokanganisa maziso, itsvo, neuropi. Zviratidzo zvinowanzoonekwa pakuzvarwa.
• Chirwere chaWilson: Muchirwere ichi, muviri haugone kubvisa mhangura nemazvo. Kana mhangura yaungana, inogona kukuvadza chiropa, uropi, itsvo, nemaziso.
• Kusashivirira fructose kubva muchibereko: Izvi zvinokonzerwa nekushaikwa kweenzyme inonzi Aldolase B, iyo inokonzera kuderera kweshuga muropa (hypoglycemia) kana munhu achidya shuga yemuchero (fructose) ne sucrose, izvo zvinogona kukanganisa chiropa.
• Chirwere chemazino: Ichi chirwere cheitsvo chisingawanzoitiki. Chinogona kukonzera mapuroteni muweti, kuwedzera kwecalcium muweti, kuiswa kwecalcium muitsvo (Nephrocalcinosis), matombo eitsvo, uye pakupedzisira kutadza kushanda kweitsvo (Chronic kidney disease). Chinowanzoitika kuvanhurume.
• Glycogenosis: Ichi chirwere chemajini chinokonzerwa nekusashanda zvakanaka kweprotein inonzi GLUT2, iyo inotakura shuga. Inozivikanwawo seFanconi Bickel syndrome.
• Chirwere chetyrosinemia cherudzi rwekutanga: Ichi chikanganiso mukushandiswa kweamino acid tyrosine mumasero, izvo zvinogona kukanganisa chiropa, tsinga, neitsvo, zvichikonzera Fanconi syndrome.

Zvikonzero zvekutanga kweFanconi syndrome:

• Mimwe mishonga:Dambudziko iri rinogona kuitika semhedzisiro yemimwe mishonga, yakadai semishonga inorwisa mabhakitiriya, mishonga yeHIV/AIDS, uye mishonga yechemotherapy, izvo zvinogona kukuvadza itsvo.
• Kuisirwa itsvo: Izvi zvinogona kuitika nekuda kwemishonga inoshandiswa mushure mekuisirwa itsvo, kukuvara kweitsvo panguva yekuvhiyiwa, kana kurambwa kweitsvo yakaisirwa.
• Multiple myeloma: Iyi ikenza yemasero eplasma muropa. Puroteni isina kujairika inogadzirwa nemasero aya inogona kukanganisa itsvo, zvichikonzera Fanconi syndrome.
• AL amyloidosis (primary amyloidosis): Muchiitiko ichi, puroteni iri mumasero eplasma inova isina kujairika uye inokanganisa nhengo dzakasiyana-siyana, kusanganisira itsvo.
• Chiratidzo cheLight chain proximal tubulopathy (LCPT): Mumamiriro ezvinhu aya, mapuroteni asina kujairika anochengetwawo muitsvo.
• Chepfu yemutobvu: Kunyanya kusangana nemutobvu ndicho chimwe chikonzero. Mapendi ekare, mamwe mabhatiri, uye mimwe mishonga yechivanhu zvinogona kunge zvine mutobvu, saka chenjera.
• Kusangana neToluene: Toluene ikemikari inowanikwa mumatadza, pendi, uye mvura yekuchenesa simbi. Kufema izvi (semuenzaniso, kunhuhwirira kwegum) kunogona kukonzera Fanconi syndrome.
• Mimwe mishonga yemishonga: Mimwe mishonga yemishonga ine aristolochic acid yakawanikwawo ichibatanidzwa neizvi. Saka, hazvikurudzirwi kushandisa zvinhu zvakadaro pasina kurairwa nachiremba.

Ndeipi mishonga chaiyo inokonzera Fanconi syndrome?

Kazhinji, mhando dzemishonga idzi dzakaonekwa sedzinonyanya kukonzera Fanconi syndrome:

• Cisplatin `(Cisplatin)`
• Ifosfamide
• Tenofovir
• Asidhi yeValproic `(asidhi yeValproic)`
• Mishonga inorwisa mabhakitiriya yeAminoglycoside, yakadai seGentamicin
• Deferasirox `(Deferasirox)`

Havasi vese vanoshandisa mushonga uyu vachawana mushonga uyu, asi pane njodzi. Saka kana chiremba akakunyorera mushonga uyu, vachakutarisa.

Fanconi syndrome inotapukira here?

Kwete. Ichi chirwere hachitapurirwi. Hachiparadzirwi kubva kumunhu mumwe kuenda kune mumwe kuburikidza nekubatana kwepedyo.

Fanconi syndrome inoonekwa sei?

Chiremba achakubvunza nezvezviratidzo zvako nemishonga yauri kutora. Zvadaro vachakuongororwa muviri. Vanogonawo kuita mimwe miedzo yekusimbisa chirwere chacho. Unogona zvakare kutumirwa kuna chiremba ane ruzivo rwezvirwere zveitsvo (Nephrologist).

Ndedzipi bvunzo dziri kuitwa pakuita izvi?

Kunyanya kuongororwa kweropa neropa kunoitwa.

• Kuongororwa kweweti / Kuongororwa kweweti:Vachatora weti yenyu votarisa kana ine huwandu hwakawanda hwezvinhu zvakaita seglucose, amino acids, uye phosphate. Kana izvi zvakakwirira, chiratidzo cheFanconi syndrome.
• Kuongororwa kweropa: Kuongororwa kweropa kunoongororawo kuti phosphate, bicarbonate, uye potassium hazvina kukwana here. Kudzikira kweizvi chiratidzo chechirwere ichi.

Chiremba anoita chirwere ichi zvichibva paruzivo rwakawanikwa kubva muongororo idzi.

Ko Fanconi syndrome inogona kurapwa here?

Izvi zvinoenderana nechikonzero chechirwere.

• Matambudziko emumajini anokonzera Fanconi syndrome anowanzo kuve akaoma kurapa zvachose. Zvisinei, kuchinja zvekudya nekurapwa kunogona kubatsira kudzora zviratidzo uye kuvandudza hupenyu.
• Kana chikonzero cheFanconi syndrome chikawanikwa uye chikarapwa, itsvo dzinogona kupora dzimwe nguva. Zvisinei, izvi hazviwanzovimbiswi. Zvisinei, zviratidzo zvinogona kugadziriswa uye kukuvara kweitsvo, tsandanyama, nemapfupa kunogona kuderedzwa.

Fanconi syndrome inorapwa sei?

Nzira yekurapa inosiyanawo zvichienderana nechikonzero uye kuoma kwechirwere.

1. Kurapa chikonzero chiripo: Chiremba achatanga arapa chirwere chiripo chakakonzera Fanconi syndrome. Semuenzaniso, kana chikakonzerwa nemushonga, mushonga wacho unogona kumira kana kuti dosi yacho inogona kuderedzwa.

2. Kuzadza muviri: Muviri unozadzwa nezvinovaka muviri zvakakosha (maelectrolyte, mvura) zvinorasika kuburikidza neweti. Izvi zvinogona kuitwa kuburikidza nekuchinja kudya, mishonga yekuwedzera inonwiwa, kana kuisirwa mutsinga (IV).

3. Kudzora acidity yemuviri (Metabolic acidosis): Sezvo vanhu vazhinji vachisangana nedambudziko iri, zvinhu zvakaita se sodium bicarbonate zvinogona kupihwa kuti pave ne pH yeropa (pH scale).

4. Kune avo vane phosphate shoma: Sezvo phosphate shoma ichideredza simba remapfupa, mishonga yekuwedzera yephosphate nevhitamini D zvinogona kupihwa.

5. Kudya kwakakosha kwezvirwere zvekuzvarwa nazvo: Kana mwana akazvarwa aine Fanconi syndrome, angangoda kudya kwakagadzirwa nenzira yakasarudzika. Semuenzaniso, angangoda kuderedza kudya kune fructose, galactose, kana tyrosine. Izvi zvinoenderana nemamiriro ezvinhu emajini.

Chinhu chinonyanya kukosha kutevedzera mirairo yachiremba. Kana ukaedza kuita zvinhu wega, mamiriro acho ezvinhu anogona kutowedzera kuipa.

Ndichakurumidza sei kupora mushure mekurapwa?

Izvi zvinogonawo kusiyana zvakanyanya zvichienderana nechikonzero chacho. Dzimwe nyaya dzeFanconi syndrome dzinozobuda gare gare dzinogona kupera mukati memazuva mashoma kana mavhiki. Zvisinei, dzimwe nyaya dzekuzvarwa nadzo uye dzinotanga mushure menguva dzinogona kutora nguva refu. Saka, zvakakosha kuva nemwoyo murefu uye kutora kurapwa.

Ko Fanconi syndrome inogona kudzivirirwa here?

Hapana chatingaita kudzivirira zvirwere zvemajini zviripo pakuzvarwa. Zvisinei, pane zvimwe zvatinogona kuita kuti tizvidzivirire kubva pakuva nechirwere cheFanconi gare gare :

• Dzivisa kubatwa nemutobvu. Mutobvu unogona kuwanikwa mupendi yekare yemba, matoyi, uye mapaipi emvura ane mutobvu.
• Taura nachiremba usati washandisa mishonga yemishonga kana zvimwe zvinovaka muviri. Zvimwe zvacho zvinogona kukuvadza itsvo.
• Taura nachiremba wako nezvenjodzi dzemishonga chero ipi zvayo (semuenzaniso mishonga inorwisa mabhakitiriya, mishonga yekurwisa kenza) yaanokupa. Kana mushonga wacho uchidikanwa, chiremba achatarisirawo itsvo dzako.

Chii chaungatarisira kana uine Fanconi syndrome?

Nhasi uno, vanachiremba nevaongorori vanoziva zvakawanda nezvechirwere cheFanconi uye maitiro ekurapa. Mishonga mitsva yabatsira vanhu vazhinji kurarama hupenyu hwakajairika.

• Congenital Fanconi syndrome: Zviratidzo zvinowanzoonekwa achiri mudiki. Kana izvi zvichikonzerwa ne cystinosis, mwana anogona kunge aine matambudziko ekukura uye kuwedzera huremu. Kukundikana kweitsvo kunogona kuitika nekukurumidza. Dzimwe nhengo, dzakadai semaziso, chiropa, nemapfupa, dzinogonawo kukanganiswa.
• Fanconi syndrome inotanga kuitika: Kana chikonzero chacho chaonekwa uye charapwa, itsvo dzinogona kupora. Zvisinei, dzimwe nguva kukuvara kweitsvo kunogona kugara kwenguva refu.

Unogona kurarama kwenguva yakareba sei uine Fanconi syndrome?

Hazvigoneki kutaura kuti izvi zvichatora nguva yakareba sei. Kana ukashanda zvichienderana nekurapwa kwakakodzera uye chirongwa chekurapa, unogona kurarama hupenyu hwakajairika. Zvisinei, kana itsvo dzako dzikakundikana, hupenyu hwako hunogona kudzikira. Muzviitiko zvezvirwere zvekuzvarwa nazvo, hupenyu hunotarisirwa hunosiyana zvichienderana nerudzi rwechirwere chemajini.

Ndinozvitarisira sei?

Chiremba wako achagadzira hurongwa hwekurapa hwakakunakira. Izvi zvinogona kusanganisira kutora mishonga yekuwedzera, kuchinja zvekudya, uye kuchinja mararamiro ako. Zvakakosha kutevedzera mirairo yachiremba wako nemazvo. Iva nechokwadi chekuti waongororwa nenguva uye wanwa mishonga yako sezvakarairwa.

Ndinofanira kuona chiremba rini?

Kana uine zviratidzo zveFanconi syndrome, kana chimwe chezvirwere zvatakurukura zvinogona kukonzera chirwere ichi, enda kuna chiremba nekukasika. Kuonekwa kwechirwere ichi nekukurumidza kuri nyore kurapa uye kunogona kuderedza matambudziko.

Mibvunzo ipi yaunofanira kubvunza chiremba?

• Unoziva sei kana ndine Fanconi syndrome?
• Ndedzipi mhando dzebvunzo dzinoitwa kuti dzionekwe sedziri kuitika?
• Chinhu chandakazvarwa nacho here kana kuti chimwe chinhu chandakazokura nacho gare gare?
• Chii chinokonzera chirwere changu cheFanconi?
• Ndoona nyanzvi here?
• Ndezvipi zvimwe zvinovaka muviri zvandinofanira kudya?
• Ndeipi njodzi yangu yekutadza kushanda kweitsvo?
• Ndichada kuisirwa itsvo here?
• Ndinofanira kuongororwa majini here?
• Ndinofanira kuuya kuzoongorora mamiriro angu kangani?
• Kune mapoka ekutsigira vanhu vane Fanconi syndrome here?

Ndeupi musiyano uripo pakati peFanconi Syndrome neFanconi Anemia?

Izvi zvinovhiringidza vanhu vazhinji. Fanconi syndrome neFanconi anemia zvirwere zviviri zvakasiyana zvachose.

• Fanconi syndrome idambudziko apo itsvo hadzikwanise kunwisa zvinhu zvinodiwa nemuviri.
• Fanconi anemia chirwere chisingawanzoitiki, chinogara nhaka chinokanganisa mupfupa. Mumamiriro ezvinhu aya, mupfupa haugone kugadzira masero eropa ane hutano. Izvi zvinowedzerawo njodzi yekubatwa neleukemia nedzimwe mhando dzekenza.

Saka, unogona kuona kuti vaviri ava vakasiyana sei.

Pakupedzisira, zvinhu zvekurangarira (Mharidzo Yekuenda Kumba)

Fanconi Syndrome chirwere chisingawanzoitiki chinokanganisa itsvo, asi zvakakosha kuziva nezvazvo. Chinokonzera kuti muviri urasikirwe nezvinovaka muviri zvinokosha kuburikidza neweti. Chinogona kuvapo pakuzvarwa kana kukura mune zvimwe zvikonzero.

Unganzwa kutya uye kunetseka paunonzwa nezvechirwere ichi. Zvisinei, nekurapwa kwazvino uno, vanhu vazhinji vanokwanisa kurarama hupenyu hwakajairika. Kana uine mibvunzo nezveizvi, taura nachiremba. Iye anogona kupindura mibvunzo yako, kukutumira kuna nyanzvi kana zvichidikanwa, kana kupa ruzivo nezvemapoka ekutsigira. Usatya, uye tevera mazano ekurapa akakodzera.

Fanconi Syndrome, Chirwere cheItsvo, Zvirwere zveMajini, Maelectrolyte, Matambudziko eWeti, Hutano hweVana, Migumisiro yeMishonga