Ukukhubazeka ngezikhathi ezithile

I-Hyperkalemic periodic paralysis (hyperPP) yisifo esidala ukuthi ngezikhathi ezithile sibe nobuthakathaka bemisipha futhi kwesinye isikhathi sibe sezingeni eliphakeme kunelejwayelekile le-potassium egazini. Igama lezokwelapha lezinga eliphakeme le-potassium yi-hyperkalemia.

I-HyperPP ingenye yeqembu lokuphazamiseka kofuzo okubandakanya ukukhubazeka kwe-hypokalemic periodic kanye ne-thyrotoxic periodic paralysis.

IHyperPP izalwa naso. Lokhu kusho ukuthi ikhona ngesikhathi sokuzalwa. Ezimweni eziningi, idluliselwa emindenini (ezuzwe njengefa) njengesifo esivelele se-autosomal. Ngamanye amagama, umzali oyedwa kuphela odinga ukudlulisa isakhi sofuzo esihlobene nalesi simo enganeni yakhe ukuze ingane ithinteke.

Ngezikhathi ezithile, lesi simo singaba umphumela wenkinga yezofuzo engazuzwa njengefa.

Kukholakala ukuthi lesi sifo sihlobene nezinkinga ngendlela umzimba olawula ngayo amazinga e-sodium ne-potassium kumaseli.

Izici zobungozi zifaka ukuba namanye amalungu omndeni anokukhubazeka ngezikhathi ezithile. Ithinta abesilisa nabesifazane ngokulinganayo.

Izimpawu zifaka phakathi ukuhlaselwa ubuthakathaka bemisipha noma ukulahleka kokunyakaza kwemisipha (ukukhubazeka) okuzayo kudlule. Kukhona amandla emisipha ejwayelekile phakathi kokuhlaselwa.

Ukuhlaselwa kuvame ukuqala ebuntwaneni. Ukuhlaselwa kwenzeka kaningi kangakanani kuyehluka. Abanye abantu banokuhlaselwa okuningana ngosuku. Imvamisa ayinzima ngokwanele ukuthi ingadinga ukwelashwa. Abanye abantu baye bahlobanisa i-myotonia, lapho bengakwazi khona ukukhulula ngokushesha imisipha yabo ngemuva kokuyisebenzisa.

Ubuthakathaka noma ukukhubazeka:

• Okuvame kakhulu ukwenzeka emahlombe, emuva nasesinqeni
• Kungabandakanya izingalo nemilenze, kepha akuthinti imisipha yamehlo nemisipha esiza ngokuphefumula nokugwinya
• Okuvame ukwenzeka ngesikhathi sokuphumula ngemuva komsebenzi noma ukuzivocavoca umzimba
• Kungenzeka kwenzeke lapho uphaphama
• Kuvela futhi kuvaliwe
• Imvamisa ihlala imizuzu eyi-15 kuya ehoreni eli-1, kepha ingahlala usuku lonke

Izimbangela zingafaka:

• Ukudla ukudla okuphezulu kwe-carbohydrate
• Phumula emva kokuzivocavoca umzimba
• Ukuchayeka emakhazeni
• Ukweqa ukudla
  
• Ukudla ukudla okunothe nge-potassium noma ukuthatha imithi equkethe i-potassium
• Ukucindezeleka
  

Umhlinzeki wezokunakekelwa kwempilo angasola i-hyperPP ngokuya ngomlando womndeni walesi sifo. Eminye imikhondo yalesi sifo yizimpawu zobuthakathaka bemisipha eziza zihambe nemiphumela ejwayelekile noma ephezulu yokuhlolwa kwe-potassium.

Phakathi kokuhlaselwa, ukuhlolwa komzimba akubonisi lutho olungajwayelekile. Ngesikhathi naphakathi kokuhlaselwa, izinga legazi le-potassium lingajwayelekile noma liphezulu.

Ngesikhathi sokuhlaselwa, izingqondo zemisipha ziyancipha noma azikho. Futhi imisipha iyaxhuga kunokuba ihlale iqina. Amaqembu emisipha aseduze komzimba, njengamahlombe nezinqulu, abandakanyeka kaningi kunezingalo nemilenze.

Ukuhlolwa okungenziwa kufaka phakathi:

• I-Electrocardiogram (ECG), engahle ingajwayelekile ngesikhathi sokuhlaselwa
• I-Electromyography (EMG), ejwayelekile ejwayelekile phakathi kokuhlaselwa nokungajwayelekile ngesikhathi sokuhlaselwa
• Imisipha biopsy, engakhombisa ukungajwayelekile

Olunye uvivinyo lunga-oda ukukhipha ezinye izimbangela.

Umgomo wokwelashwa ukukhulula izimpawu nokuvikela okunye ukuhlaselwa.

Ukuhlaselwa akuvamile ukuba kubi ngokwanele ukudinga ukwelashwa okuphuthumayo. Kepha ukushaya kwenhliziyo okungajwayelekile (i-heart arrhythmias) nakho kungenzeka ngesikhathi sokuhlaselwa, okudingeka ukwelashwa okuphuthumayo. Ubuthakathaka bemisipha bungaba buhlungu kakhulu ngokuhlaselwa okuphindaphindiwe, ngakho-ke ukwelashwa ukuvimbela ukuhlaselwa kufanele kwenzeke ngokushesha okukhulu.

I-glucose noma amanye ama-carbohydrate (ushukela) anikezwa ngesikhathi sokuhlaselwa anganciphisa ubunzima bezimpawu. I-calcium noma isisu (amaphilisi amanzi) kungadingeka ukuthi unikezwe ngomthambo ukunqanda ukuhlaselwa okungazelelwe.

Kwesinye isikhathi, ukuhlaselwa kuyanyamalala kamuva empilweni bebodwa. Kodwa ukuhlaselwa okuphindaphindiwe kungaholela ekubeni buthakathaka kwemisipha unomphela.

I-HyperPP iphendula kahle ekwelashweni. Ukwelashwa kungavimbela, futhi kungaphindisela emuva, ubuthakathaka bemisipha obuqhubekayo.

Izinkinga zempilo ezingaba ngenxa ye-hyperPP zifaka:

• Amatshe ezinso (umphumela ohlangothini womuthi osetshenziselwa ukwelapha lesi simo)
• Ukushaya kwenhliziyo okungajwayelekile
• Ubuthakathaka bemisipha obuqhubeka buba bucayi

Shayela umhlinzeki wakho uma wena noma ingane yakho unobuthakathaka bemisipha obufikayo buqhubeke, ikakhulukazi uma unamalungu omndeni anokukhubazeka ngezikhathi ezithile.

Iya egumbini labezimo eziphuthumayo noma ushayele inombolo yendawo ephuthumayo (efana ne-911) uma uquleka noma unenkinga yokuphefumula, ukukhuluma, noma ukugwinya.

Imithi i-acetazolamide ne-thiazides ivimbela ukuhlaselwa ezimweni eziningi. I-potassium ephansi, ukudla okuphezulu kwe-carbohydrate, nokuvivinya umzimba okulula kungasiza ukuvimbela ukuhlaselwa. Ukugwema ukuzila ukudla, ukusebenza kanzima, noma amazinga okushisa abandayo nakho kungasiza.

Ukukhubazeka ngezikhathi ezithile - i-hyperkalemic; Ukukhubazeka okujwayelekile kwe-hyperkalemic periodic; IHyperKPP; HyperPP; Isifo seGamstorp; Ukukhubazeka okwenzeka ngezikhathi ezithile nge-potassium

• I-atrophy yemisipha

Amato AA. Ukuphazamiseka kwemisipha yamathambo. Ku: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, ama-eds. I-Neurology kaBradley ekwenziweni kwemitholampilo. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2016: isahluko 110.

UKerchner GA, uPtácek LJ. I-Channelopathies: iziphazamiso ze-episodic nezikagesi zesistimu yezinzwa. Ku: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SK, ama-eds. I-Neurology kaBradley ekwenziweni kwemitholampilo. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2016: isahluko 99.

Moxley RT, Heatwole C. Channelopathies: ukuphazamiseka kwe-myotonic nokukhubazeka ngezikhathi ezithile. Ku: Swaiman KF, Ashwal S, Ferriero DM, et al, abahleli. I-Swaiman's Pediatric Neurology: Izimiso kanye Nokuzijwayeza. Umhlaka 6. IPhiladelphia, PA: Elsevier; I-2017: isahluko 151.