Isifo seBartter

Isifo seBartter liqela leemeko ezinqabileyo ezichaphazela izintso.

Zintlanu iziphene zofuzo ezaziwa ukuba zinxulunyaniswa neBartter's syndrome. Le meko ikhoyo ekuzalweni (kokuzalwa).

Imeko ibangelwa sisiphene kwizintso ukukwazi ukubuyisela kwakhona isodiyam. Abantu abachaphazeleke yi-Bartter's syndrome baphulukana nesodium eninzi kumchamo. Oku kubangela ukunyuka kwinqanaba le-hormone aldosterone, kwaye kwenza izintso zisuse i-potassium eninzi kakhulu emzimbeni. Oku kwaziwa ngokuba kukumosha i-potassium.

Le meko ikwakhokelela ekulinganisweni kweasidi engaqhelekanga egazini elibizwa ngokuba yihypokalemic alkalosis, ebangela ikhalsiyam eninzi kumchamo.

Esi sifo sihlala sivela ebuntwaneni. Iimpawu zibandakanya:

• Ukuqunjelwa
• Izinga lokufumana ubunzima liphantsi kakhulu kunelabanye abantwana abakwiminyaka efanayo kunye nesini (ukukhula kokungaphumeleli)
• Ukufuna ukuchama rhoqo kunesiqhelo (ukuhamba rhoqo)
• Uxinzelelo lwegazi oluphantsi
• Amatye eentso
• Ukuxinana kwezihlunu kunye nobuthathaka

I-Bartter syndrome ihlala ikrokrelwa xa uvavanyo lwegazi lufumana inqanaba eliphantsi le-potassium egazini. Ngokungafaniyo nezinye iintlobo zesifo sezintso, le meko ayibangeli uxinzelelo lwegazi oluphezulu. Kukho ukutyekela kuxinzelelo lwegazi olusezantsi. Iimvavanyo zaselebhu zingabonisa:

• Amanqanaba aphezulu e-potassium, i-calcium, kunye ne-chloride kumchamo
• Amanqanaba aphezulu amahomoni, i-renin kunye ne-aldosterone, egazini
• I-chloride yegazi ephantsi
• I-alkalosis yeMetabolic

Ezi mpawu kunye neempawu zinokwenzeka nakwabantu abathatha ii-diuretics ezininzi (iipilisi zamanzi) okanye i-laxatives. Uvavanyo lomchamo lunokwenziwa ukukhupha ezinye izizathu.

I-ultrasound yezintso inokwenziwa.

Isifo seBartter siphathwa ngokutya ukutya okunotye nge potassium okanye ukuthatha izongezo zepotassium.

Abantu abaninzi bafuna ityiwa kunye nezongezo zemagniziyam.Unyango lunokufuneka oluthintela amandla ezintso okususa i-potassium. Amanqanaba aphezulu e-nonsteroidal anti-inflammatory drug (NSAIDs) nawo anokusetyenziswa.

Iintsana ezinokukhula okungathethekiyo kunokukhula ngokwesiqhelo ngonyango. Ixesha elingaphezulu, abanye abantu abanemeko baya kuphuhlisa ukusilela kwezintso.

Fowunela umboneleli wakho wezempilo ukuba umntwana wakho ngu:

• Ukuba necramps zemisipha
• Ayikhuli kakuhle
• Ukuchama rhoqo

Ukuchithwa kwePotassium; Ityhefu yokuchitha ityuwa

• Uvavanyo lwenqanaba leAldosterone

Dixon BP. Ukuhamba ngendlela engaqhelekanga kwezothutho: I-Bartter syndrome. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahluko 549.1

IGuay-Woodford LM. Ilifa nephropathies kunye nokukhula ngendlela engaqhelekanga kwendlela yokuchama. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 119.

Intaba DB. Ukuphazamiseka kwemali eseleyo ye potassium. Ku: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, ii-eds. Brenner kunye noMphathi weZintso. Ngomhla we-11. IPhiladelphia, PA: Elsevier; 2020: isahluko 17.