Matenda opatsirana nthawi ndi nthawi

Hyperkalemic periodic paralysis (hyperPP) ndimatenda omwe amachititsa kuti nthawi zina minofu ifooke ndipo nthawi zina imakhala potaziyamu wokwanira kuposa magazi. Dzina lachipatala la potaziyamu yayikulu ndi hyperkalemia.

HyperPP ndi amodzi mwamagulu amtundu wamatenda omwe amakhala ndi ziwalo za hypokalemic periodic komanso ziwalo za thyrotoxic periodic.

HyperPP ndiyobadwa. Izi zikutanthauza kuti imakhalapo pakubadwa. Nthawi zambiri, imafalikira kudzera m'mabanja (obadwa nawo) ngati vuto lalikulu la autosomal. Mwanjira ina, kholo limodzi lokha liyenera kupatsira mwana wawo zakabadwa kuti zikhozeke.

Nthawi zina, vutoli limatha kukhala chifukwa cha vuto la chibadwa lomwe silinatengere.

Amakhulupirira kuti vutoli limakhudzana ndi zovuta momwe thupi limayang'anira magawo a sodium ndi potaziyamu m'maselo.

Zowopsa zimaphatikizaponso kukhala ndi achibale ena omwe ali ndi ziwalo nthawi ndi nthawi. Zimakhudza amuna ndi akazi mofanana.

Zizindikiro zake zimaphatikizapo kufooka kwa kufooka kwa minofu kapena kutayika kwa minofu (ziwalo) zomwe zimabwera ndikumapita. Pali mphamvu yabwinobwino yamphamvu pakati pamisempha.

Kuukira kumayambira ali mwana. Nthawi zambiri ziwopsezo zimachitika zimasiyanasiyana. Anthu ena amaukiridwa kangapo patsiku. Nthawi zambiri amakhala osakwanira kusowa chithandizo. Anthu ena agwirizana ndi myotonia, momwe sangathe kupumula minofu yawo akaigwiritsa ntchito.

Kufooka kapena kufooka:

• Kawirikawiri amapezeka pamapewa, kumbuyo, ndi m'chiuno
• Zitha kuphatikizanso mikono ndi miyendo, koma sizimakhudza minofu ya maso ndi minofu yomwe imathandizira kupuma ndi kumeza
• Amakonda kupezeka atapumula pambuyo pa zochitika kapena zolimbitsa thupi
• Zitha kuchitika pakudzuka
• Zimapezeka ndi kutseka
• Nthawi zambiri zimatenga mphindi 15 mpaka ola limodzi, koma zimatha mpaka tsiku lonse

Zoyambitsa zitha kuphatikiza:

• Kudya chakudya cham'madzi ambiri
• Pumulani mutatha kuchita masewera olimbitsa thupi
• Kuwonetseredwa kuzizira
• Kudya chakudya
  
• Kudya zakudya zokhala ndi potaziyamu kapena kumwa mankhwala omwe ali ndi potaziyamu
• Kupsinjika
  

Wothandizira zaumoyo atha kukayikira hyperPP kutengera mbiri ya banja lavutoli. Zina mwazomwe zimayambitsa matendawa ndi kufooka kwa minofu komwe kumabwera ndikubwera ndi zotsatira zabwinobwino za mayeso a potaziyamu.

Pakati pakuukiridwa, kuyezetsa thupi sikuwonetsa zachilendo. Pakati komanso pakachitika, mulingo wa potaziyamu umatha kukhala wabwinobwino kapena wokwera.

Pakati pa kuukira, kusinthasintha kwa minofu kumachepetsedwa kapena kulibe. Ndipo minofu imayenda molumala m'malo mokhala olimba. Magulu am'miyendo pafupi ndi thupi, monga mapewa ndi chiuno, amatenga nawo mbali pafupipafupi kuposa mikono ndi miyendo.

Mayeso omwe angachitike ndi awa:

• Electrocardiogram (ECG), yomwe imatha kukhala yachilendo panthawi yamawonekedwe
• Electromyography (EMG), yomwe nthawi zambiri imakhala yachilendo pakati pa ziwopsezo ndi zosazolowereka mukamayesedwa
• Minofu biopsy, yomwe imatha kuwonetsa zodetsa nkhawa

Mayesero ena atha kulamulidwa kuti athetse zifukwa zina.

Cholinga cha chithandizo ndikuthetsa zizindikiro ndikupewa kuukira kwina.

Kuukira sikumakhala koopsa mokwanira kufuna chithandizo chadzidzidzi. Koma kugunda kwamtima kosazolowereka (mtima arrhythmias) kumathanso kuchitika pakuwukira, komwe kumafunikira chithandizo chadzidzidzi. Kufooka kwa minofu kumatha kukulirakulira ndikumenyedwa mobwerezabwereza, chifukwa chake chithandizo chopewa ziwopsezo chikuyenera kuchitika mwachangu.

Glucose kapena chakudya china (shuga) chomwe chimaperekedwa panthawi yamavuto chitha kuchepetsa kuopsa kwa zizindikirazo. Calcium kapena diuretics (mapiritsi amadzi) angafunike kuperekedwa kudzera mumitsempha kuti athetse kuukira kwadzidzidzi.

Nthawi zina, ziwopsezo zimatha pambuyo pake paokha. Koma kuukira mobwerezabwereza kumatha kufooketsa minofu mpaka kalekale.

HyperPP imayankha bwino kuchipatala. Chithandizo chitha kuteteza, ndipo mwina chitha kusintha, kufooka kwapang'onopang'ono kwa minofu.

Mavuto azaumoyo omwe atha kukhala chifukwa cha hyperPP ndi awa:

• Miyala ya impso (mankhwala omwe amagwiritsidwa ntchito pochiza vutoli)
• Kugunda kwamtima kosasintha
• Kufooka kwa minofu komwe kumangowonjezereka pang'onopang'ono

Itanani omwe akukuthandizani ngati inu kapena mwana wanu muli ndi kufooka kwa minofu komwe kumabwera ndikudutsa, makamaka ngati muli ndi abale anu omwe amakhala ndi ziwalo nthawi ndi nthawi.

Pitani kuchipinda chodzidzimutsa kapena itanani nambala yadzidzidzi yakomweko (monga 911) ngati mwakomoka kapena mukuvutika kupuma, kuyankhula, kapena kumeza.

Mankhwala acetazolamide ndi thiazides amateteza kuukira nthawi zambiri. Potaziyamu wochepa, chakudya chambiri chazakudya, komanso kuchita masewera olimbitsa thupi mopepuka kungathandize kupewa ziwopsezo. Kupewa kusala kudya, kutopetsa, kapena kuzizira kumathandizanso.

Nthawi ziwalo - hyperkalemic; Banja lodziwika bwino lofa ziwalo nthawi ndi nthawi; HyperKPP; HyperPP; Matenda a Gamstorp; Kuuma kwa potaziyamu nthawi zina

• Kulephera kwa minofu

Amato AA. Kusokonezeka kwa mafupa am'mafupa. Mu: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, olemba. Neurology ya Bradley mu Kuchita Zachipatala. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2016: chap 110.

Kerchner GA, Ptácek LJ. Channelopathies: zovuta zamagetsi zamagetsi zamanjenje ndi zamagetsi zamanjenje. Mu: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SK, olemba. Neurology ya Bradley mu Kuchita Zachipatala. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2016: chap 99.

Moxley RT, Heatwole C. Channelopathies: zovuta za myotonic komanso ziwalo zina. Mu: Swaiman KF, Ashwal S, Ferriero DM, et al, olemba. Swaiman's Pediatric Neurology: Mfundo ndi Zochita. Lachisanu ndi chimodzi. Philadelphia, PA: Elsevier; 2017: mutu 151.