Chirwere cheGamstorp (Hyperkalemic Periodic Paralysis)

Zvemukati

• Chii chinonzi chirwere cheGamstorp?
• Ndezvipi zviratidzo zvechirwere cheGamstorp?
• Kuoma mitezo
• Myotonia
• Ndezvipi zvikonzero zvechirwere cheGamstorp?
• Ndiani ari panjodzi yechirwere cheGamstorp?
• Chirwere cheGamstorp chinoonekwa sei?
• Kugadzirira kuona chiremba wako
• Ndeapi marapirwo eGamstorp chirwere?
• Mishonga
• Mishonga yekumba
• Kutsungirira chirwere cheGamstorp
• Ndeupi mutarisiro wenguva refu?

Chii chinonzi chirwere cheGamstorp?

Chirwere cheGamstorp chinonyanya kushomeka chimiro chemajini chinokonzeresa kuti uve nezvikamu zvekushaya simba kwemhasuru kana kuora muviri kwenguva pfupi Chirwere chinozivikanwa nemazita mazhinji, kusanganisira hyperkalemic periodic kuoma mitezo.

Icho chirwere chakagarwa nhaka, uye zvinokwanisika kuti vanhu vatakure uye vapfuure jeni vasina kana kumboona zviratidzo. Mumwe muvanhu mazana maviri nemakumi mashanu ane chirwere ichi.

Kunyangwe pasina mushonga weGamstorp chirwere, vanhu vazhinji vanayo vanogona kurarama zvakajairika, zvinoshanda hupenyu.

Vanachiremba vanoziva zvakawanda zvezvikonzero zveakaoma mitezo zvikamu uye zvinowanzo kubatsira kubatsira kudzikamisa mhedzisiro yechirwere nekutungamira vanhu vane chirwere ichi kuti vadzivise zvimwe zvinozivikanwa zvinokonzeresa.

Ndezvipi zviratidzo zvechirwere cheGamstorp?

Chirwere cheGamstorp chinokonzeresa zviratidzo, kusanganisira:

• kushaya simba kwakasimba kwemitezo
• kuoma mitezo
• kurova kwemoyo zvisina kujairika
• kusvetuka kurova kwemoyo
• kuomarara kwemhasuru
• kusagadzikana kusingaperi
• kusafamba

Kuoma mitezo

Zvikamu zvakafa mitezo zvipfupi uye zvinogona kupera mushure memaminetsi mashoma. Kunyangwe iwe uine chikamu chakareba, iwe unofanirwa kupora zvakakwana mukati maawa maviri ezviratidzo kutanga.

Zvisinei, zvikamu zvinowanzoitika kamwe kamwe. Unogona kuona kuti hauna yambiro yakakwana yekutsvaga nzvimbo yakachengeteka yekumirira chikamu. Neichi chikonzero, kukuvara kubva pakuwa kwakajairika.

Zvikamu zvinowanzotanga muhucheche kana paudiki hudiki. Kune vanhu vazhinji, kuwanda kwezvikamu kunowedzera kuburikidza nemakore ekuyaruka uye kusvika pakati pavo makumi maviri.

Sezvaunosvika kuma30s ako, kurwiswa kunozoita kushoma. Kune vamwe vanhu, vanonyangarika zvachose.

Myotonia

Imwe yezviratidzo zveGamstorp chirwere ndeye myotonia.

Kana iwe uine ichi chiratidzo, mamwe emapoka ako emhasuru anogona kuve akaomarara kwechinguva uye kunonetsa kufamba. Izvi zvinogona kurwadza kwazvo. Nekudaro, vamwe vanhu havanzwe chero kusagadzikana panguva yechiitiko.

Nekuda kwekusagadzikana nguva dzose, mhasuru dzinokanganiswa ne myotonia dzinowanzo tarisa dzakatsanangurwa uye dzakasimba, asi iwe unogona kuwana iwe unogona chete kushandisa zvishoma kana kusatomboita simba uchishandisa aya mhasuru.

Myotonia inokonzeresa kukuvara kusingaperi muzviitiko zvakawanda. Vamwe vanhu vane chirwere cheGamstorp vanozoshandisa mawiricheya nekuda kwekudzikira kwetsoka dzemakumbo avo.

Kurapa kunowanzo dzivirira kana kudzoreredza mberi kushomeka kwemhasuru.

Ndezvipi zvikonzero zvechirwere cheGamstorp?

Chirwere cheGamstorp mhedzisiro yeshanduko, kana shanduko, mune geni inonzi SCN4A. Iri geni rinobatsira kuburitsa migero yesodium, kana kuvhurika kuduku-duku kuburikidza nesodium inofamba nemumasero ako.

Magetsi magetsi anoburitswa neakasiyana mamoduleti esodiyamu uye potasium achipfuura nemumasero membrane anodzora mhasuru kufamba.

Muchirwere cheGamstorp, migero iyi ine zvisakanaka zvinoitika zvinoita kuti potasiamu iungane kudivi rimwe remukono wesero uye iwedzere muropa.

Izvi zvinodzivirira magetsi anodikanwa kubva mukuumbika uye anoita kuti iwe utadze kutamisa tsandanyama yakakanganiswa.

Ndiani ari panjodzi yechirwere cheGamstorp?

Chirwere cheGamstorp chirwere chakagarwa nhaka, uye chinotyisa zvikuru. Izvi zvinoreva kuti iwe unongofanirwa kuve nekopi imwe chete yeiyo mutated geni kuti uwedzere chirwere.

Pane mukana we50 muzana iwe uine geni kana mumwe wevabereki vako ari mutakuri. Nekudaro, vamwe vanhu vane iyo jeni havatombogadzira zviratidzo.

Chirwere cheGamstorp chinoonekwa sei?

Kuti uongorore chirwere cheGamstorp, chiremba wako anotanga kutonga kusagadzikana kweadrenal senge chirwere cheAdisonison, icho chinoitika kana gland rako readrenal risingabudise zvakakwana mahormone cortisol uye aldosterone.

Vachaedza zvakare kubvisa zvirwere zveitsvo zvinogona kukonzera zvisina kujairika potasiamu mazinga.

Kana vachinge vadzora kusagadzikana kweadrenal uye zvirwere zveitsvo zvakagarwa nhaka, chiremba wako anogona kusimbisa kana chiri chirwere cheGamstorp kuburikidza nekuongororwa kweropa, kuongororwa kweDNA, kana nekuongorora yako serum electrolyte uye potasiamu mazinga.

Kuti uongorore aya mazinga, chiremba wako anogona kunge iwe uchiita bvunzo dzinosanganisira kurovedza muviri zvine hunyanzvi zvichiteverwa nekuzorora kuti uone masero e potassium.

Kugadzirira kuona chiremba wako

Kana iwe uchifunga kuti ungave uine chirwere cheGamstorp, zvinogona kubatsira kuchengetedza diary yekutarisa ako esimba mazinga mukati mezuva rega rega. Iwe unofanirwa kuchengeta zvinyorwa nezvezvaunoita uye kudya kwemazuva iwayo kuti ubatsire kuona zvaunokonzeresa.

Iwe unofanirwa zvakare kuunza chero ruzivo iwe rwaunogona kuunganidza nezve kana iwe kana uine mhuri nhoroondo yechirwere.

Ndeapi marapirwo eGamstorp chirwere?

Iyo kurapwa kunoenderana nekuomarara uye kuwanda kwezvikamu zvako. Mishonga uye zvinowedzera zvinoshanda zvakanaka kune vanhu vazhinji vane chirwere ichi. Kudzivirira zvimwe zvinokonzeresa zvinoshanda zvakanaka kune vamwe.

Mishonga

Vanhu vazhinji vanofanirwa kuvimba nemishonga kudzora kurwara kwakaoma mutezo. Imwe yemishonga inowanzo nyorerwa acetazolamide (Diamox), iyo inowanzo shandiswa kubata pfari.

Chiremba wako anogona kuraira diuretics kudzora iyo potasiamu mazinga muropa.

Vanhu vane myotonia semhedzisiro yechirwere vanogona kurapwa vachishandisa madosi akaderera emishonga senge mexiletine (Mexitil) kana paroxetine (Paxil), iyo inobatsira kudzikamisa mhasuru dzakakomba dzemhasuru.

Mishonga yekumba

Vanhu vanoona zvinyoro kana zvisingawanzoitika zvikamu dzimwe nguva vanogona kudzora kurwara nekuoma mutezo pasina kushandisa mushonga.

Iwe unogona kuwedzera zvicherwa zvinowedzera, senge calcium gluconate, kune chinwiwa chinotapira kuti umise chikamu chakapfava.

Kunwa girazi remvura inononoka kana kuyamwa pachidimbu chewiti yakaoma pazviratidzo zvekutanga zveyakafa mitezo kunogona kubatsira futi.

Kutsungirira chirwere cheGamstorp

Kudya kwe potassium-kupfuma kana kunyange mamwe maitiro anogona kukonzera zvikamu. Potassium yakawandisa muropa inozokonzeresa kushomeka kwemhasuru kunyangwe muvanhu vasina chirwere cheGamstorp.

Nekudaro, avo vane chirwere vanogona kuita shanduko diki kwazvo mumazinga e potassium ayo asingakanganise mumwe munhu asina chirwere cheGamstorp.

Zvinowanzoitika zvinosanganisira:

• michero yakawanda mu potassium, yakadai semabhanana, apricots, uye mazambiringa akaomeswa
• potassium-yakawanda miriwo, senge sipinashi, mbatatisi, broccoli, uye kolifulawa
• nyemba, bhinzi, uye nzungu
• doro
• nguva refu yekuzorora kana kusaita basa
• kutora nguva refu usina kudya
• kutonhora kwakanyanya
• kupisa kwakanyanya

Haasi munhu wese ane chirwere cheGamstorp achave nezvakakonzera zvakafanana. Taura nachiremba wako, uye edza kurekodha zviitiko zvako uye kudya mune dhayari kuti ubatsire kuona zvaunokonzeresa.

Ndeupi mutarisiro wenguva refu?

Nekuti chirwere cheGamstorp inhaka, haugone kuchidzivirira. Nekudaro, iwe unogona kuyera mukati mhedzisiro yemamiriro acho nekunyatso gadzirisa ako njodzi njodzi. Kukwegura kunoderedza kuwanda kwezvikamu.

Izvo zvakakosha kuti utaure nachiremba wako nezve zvekudya uye zviitiko zvinogona kunge zvichikonzera zvikamu zvako. Kudzivisa zvinokonzeresa izvo zvinokonzeresa zvikamu zveakaoma mutezo kunogona kudzikamisa mhedzisiro yechirwere.