Bartter mob

Bartter mob yog ib pab pawg tsawg heev uas cuam tshuam rau lub raum.

Muaj tsib lub teeb meem kab mob uas paub tias yuav txuam nrog Bartter syndrome. Tus mob muaj nyob rau thaum yug (yug los yug).

Tus mob yog tshwm sim los ntawm kev puas tsuaj nyob rau hauv lub raum muaj peev xwm reabsorb sodium. Cov neeg cuam tshuam los ntawm Bartter syndrome plam sodium ntau dhau los ntawm kev tso zis. Qhov no ua rau muaj kev nce qib hauv qib aldosterone, thiab ua rau lub raum tshem cov poov tshuaj ntau hauv lub cev. Qhov no paub li poov tshuaj khib nyiab.

Qhov xwm txheej tseem yuav ua rau cov kua qaub txav tsis zoo hauv cov ntshav hu ua hypokalemic alkalosis, uas ua rau muaj cov calcium ntau hauv cov zis.

Tus kabmob no feem ntau tshwm sim thaum menyuam yaus. Cov tsos mob muaj xws li:

• Cem quav
• Tus nqi ntawm qhov kev nce phaus yog qis dua li ntawm lwm cov menyuam yaus uas muaj hnub nyoog zoo sib xws thiab sib deev (kev loj hlob tsis)
• Xav tso zis ntau dua li niaj zaus (tso zis ib zaug)
• Cov ntshav siab qis
• Raum pob zeb
• Cov leeg mob thiab tsis muaj zog

Bartter syndrome feem ntau xav tias tsam lub sijhawm kuaj ntshav muaj cov poov tshuaj muaj ntshav ntau tsawg. Tsis zoo li lwm hom mob raum, cov mob no tsis ua rau muaj ntshav siab. Muaj tus yam ntxwv ua kom ntshav siab. Kuaj ntshav yuav qhia pom:

• Theem siab ntawm cov poov tshuaj, calcium, thiab chloride hauv zis
• Cov theem ntawm cov tshuaj hormones, renin thiab aldosterone, hauv cov ntshav
• Cov tshuaj muaj ntshav tsawg
• Kev mob plab zom mov

Cov kev qhia thiab cov tsos mob zoo li no tuaj yeem tshwm sim hauv cov neeg uas noj tshuaj ntau dhau (dej tshuaj) lossis tshuaj laxatives. Kuaj cov zis tuaj yeem ua kom paub lwm yam teeb meem.

Ua ultrasound ntawm ob lub raum yuav ua tau.

Tus mob Bartter yog kho tau los ntawm kev noj zaub mov ntau ntxiv potassium los yog noj tshuaj ntxiv.

Coob tus neeg kuj xav tau cov tshuaj ntsev thiab magnesium.Cov tshuaj yuav tsum muaj uas yog txhaws cov raum lub raum pov tseg cov poov tshuaj. Kev siv tshuaj ntau dua cov tshuaj nonsteroidal anti-inflammatory (NSAIDs) kuj tseem siv tau.

Cov menyuam mos uas muaj mob loj hlob tsis zoo tuaj yeem loj hlob nrog kev kho mob. Ntev mus, qee cov neeg muaj tus mob yuav ua lub raum tsis ua haujlwm.

Hu koj tus kws kho mob yog tias koj tus menyuam yog:

• Muaj cov leeg mob ntswj
• Tsis loj hlob zoo
• Tso zis ntau zaus

Poov tshuaj wasting; Ntsev-wasting nephropathy

• Kev xeem qib Aldosterone

Dixon BP. Inherited tubular thauj txawv txav: Bartter mob. Hauv: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Phau Ntawv ntawm PediatricsCov. 21st ed. Philadelphia, PA: Elsevier; 2020: cha 549.1

Guay-Woodford LM. Cov kab mob muaj keeb nephropathies thiab kev loj hlob tsis zoo ntawm cov mob txeeb zig. Hauv: Goldman L, Schafer AI, eds. Goldman-Cecil TshuajCov. 26th ed. Philadelphia, PA: Elsevier; 2020: chap 119.

Pem Roob DB. Kev ua kom tsis haum cov poov tshuaj sib npaug. Hauv: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. Brenner thiab Rector's RaumCov. 11th ed. Philadelphia, PA: Elsevier; 2020: chap 17.