Isifo seBartter

IBartter syndrome iyiqembu lezimo ezingavamile ezithinta izinso.

Kunokukhubazeka kwezakhi zofuzo ezinhlanu okwaziwa ukuthi kuhlotshaniswa ne-Bartter syndrome. Isimo sikhona ngesikhathi sokuzalwa (esibelethweni).

Isimo sibangelwa ukukhubazeka emandleni ezinso zokubuyisela kabusha i-sodium. Abantu abathintekayo yi-Bartter syndrome balahlekelwa yi-sodium eningi kakhulu ngomchamo. Lokhu kubangela ukukhuphuka kwezinga le-hormone aldosterone, futhi kwenza izinso zisuse i-potassium eningi emzimbeni. Lokhu kwaziwa njengokumosha i-potassium.

Lesi simo siphinde siholele ekulinganisweni kwe-asidi okungajwayelekile egazini elibizwa nge-hypokalemic alkalosis, elibanga i-calcium eningi emchameni.

Lesi sifo sivame ukuvela ebuntwaneni. Izimpawu zifaka:

• Ukuqunjelwa
• Izinga lokuzuza kwesisindo liphansi kakhulu kunezinye izingane ezineminyaka efanayo nobulili (ukwehluleka ukukhula)
• Ukudinga ukuvuthwa kaningi kunokujwayelekile (imvamisa ye-urinary)
• Umfutho wegazi ophansi
• Amatshe ezinso
• Ukuqunjelwa kwemisipha nobuthakathaka

I-Bartter syndrome ivame ukusolwa lapho ukuhlolwa kwegazi kuthola izinga eliphansi le-potassium egazini. Ngokungafani nezinye izinhlobo zesifo sezinso, lesi simo asibangeli umfutho wegazi ophezulu. Kukhona ukuthambekela ekubhekaneni nomfutho wegazi ophansi. Ukuhlolwa kwelabhoratri kungakhombisa:

• Izinga eliphakeme le-potassium, i-calcium, ne-chloride emchameni
• Izinga eliphakeme lamahomoni, i-renin ne-aldosterone, egazini
• I-chloride ephansi yegazi
• I-alkalosis yomzimba

Lezi zimpawu nezimpawu ezifanayo zingenzeka nakubantu abaphuza isisu (amaphilisi amanzi) amaningi noma ama-laxatives. Ukuhlolwa komchamo kungenziwa ukukhipha ezinye izimbangela.

I-ultrasound yezinso ingenziwa.

I-Bartter syndrome ilashwa ngokudla ukudla okunothe nge-potassium noma ngokuthatha izithako ze-potassium.

Abantu abaningi badinga nosawoti kanye nezengezo ze-magnesium.Kungadingeka umuthi ovimba ikhono lezinso lokususa i-potassium. Imithamo ephezulu yezidakamizwa ezingekho ukuvuvukala (ama-NSAID) nayo ingasetshenziswa.

Izinsana ezinokwehluleka okukhulu ukukhula zingakhula ngokujwayelekile ngokwelashwa. Ngokuhamba kwesikhathi, abanye abantu abanesimo bazokhula ukwehluleka kwezinso.

Shayela umhlinzeki wakho wezokunakekelwa kwezempilo uma ingane yakho:

• Ukuba namajaqamba emisipha
• Ukungakhuli kahle
• Ukuchama kaningi

Ukuchitha i-potassium; I-nephropathy yokuchitha usawoti

• Ukuhlolwa kwezinga le-Aldosterone

I-Dixon BP. Ukungajwayelekile okuthuthwa kwamathambo okuhambahamba: I-Bartter syndrome. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 549.1

IGuay-Woodford LM. I-nephropathies yefa kanye nokungajwayelekile kokukhula komgudu womchamo. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 119.

Intaba DB. Ukuphazamiseka kwebhalansi ye-potassium. Ku: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, ama-eds. IBrenner neRector's The Kidney. Umhla ka-11. IPhiladelphia, PA: Elsevier; 2020: isahluko 17.