Cystinuria

Cystinuria yog ib qho mob uas tsis tshua pom muaj pob zeb los ntawm cov amino acid hu ua cysteine ​​nyob rau lub raum, zis thiab lub zais zis. Cystine yog tsim thaum ob lub molecules ntawm ib qho amino acid hu ua cysteine ​​yog khi ua ke. Tus mob yog kis los ntawm tsev neeg.

Txhawm rau kom muaj cov tsos mob ntawm cystinuria, koj yuav tsum txais cov caj ces xeeb ntxwv los ntawm ob leeg niam txiv. Koj cov menyuam puav leej yuav tau txais keeb kwm ntawm cov xeeb leej xeeb ntxwv los ntawm koj.

Cystinuria yog tshwm sim los ntawm cystine ntau nyob rau hauv cov zis. Nquag, feem ntau cystine yaj thiab rov qab mus rau cov ntshav tom qab nkag mus rau lub raum. Cov neeg uas muaj cystinuria muaj keeb caj ces uas cuam tshuam nrog txoj kev ua no. Raws li qhov tshwm sim, cystine tsim ntau ntxiv hauv cov zis thiab tsim muaju lossis pob zeb. Cov muaju no yuav lo rau hauv raum, raum, lossis zais zis.

Kwv yees li ntawm ib ntawm txhua 7000 tus neeg muaj mob cystinuria. Cystine pob zeb feem ntau yog rau cov neeg hluas hnub nyoog qis dua 40 xyoo. Tsawg dua li 3% ntawm cov mob txeeb zig yog cov pob zeb cystine.

Cov tsos mob muaj xws li:

• Cov ntshav hauv cov zis
• Flank mob lossis mob hauv sab lossis nraub qaum. Mob yog feem ntau rau ntawm ib sab. Nws tsis tshua muaj qhov hnov ​​ntawm ob tog. Kev hnov ​​mob feem ntau mob hnyav. Nws yuav mob heev ntxiv rau ib hnub. Koj yuav hnov ​​mob hauv lub plab mog, puab tais, ntawm caj pas, lossis ntawm nruab nrab plab thiab sab nraub qaum.

Tus mob feem ntau tau kuaj tom qab kuaj mob ntawm lub raum. Kev kuaj cov pob zeb tom qab lawv tau tshem tawm qhia tau hais tias lawv ua los ntawm cystine.

Tsis zoo li cov pob zeb muaj calcium, cov pob zeb cystine tsis zoo ua cov duab xoo hluav taws xob.

Kev ntsuam xyuas uas yuav ua los tshawb xyuas cov pob zeb no thiab kuaj xyuas tus mob muaj xws li:

• Kev khaws zis 24 xuab moos
• Kev thaij duab hauv plab hauv plab, lossis ultrasound
• Tso cov tshuaj pyelogram (IVP)
• Mob Lub Hlaus

Lub hom phiaj ntawm kev kho mob yog ua kom daws tau cov tsos mob thiab tiv thaiv ntau pob zeb los ntawm kev sib sau. Tus neeg muaj mob hnyav tej zaum yuav tau mus tom tsev kho mob.

Txoj kev kho yog hais txog haus dej kom ntau, tshwj xeeb yog dej, kom tso zis ntau ntau. Koj yuav tsum haus tsawg kawg 6 mus rau 8 khob nyob rau ib hnub. Koj yuav tsum haus dej thaum hmo ntuj thiab kom koj sawv ib hmos yam tsawg ib zaug txhawm rau tso zis.

Muaj qee kis, yuav tau haus dej raws txoj leeg ntshav (los ntawm IV).

Ua kom cov zis ntau alkaline yuav pab yaj lub cystine muaju. Qhov no yuav ua tau nrog kev siv cov potassium citrate lossis sodium bicarbonate. Noj cov ntsev tsawg kuj tseem tuaj yeem txo muaj cov cystine tso tawm thiab cov pob zeb tsim.

Koj tuaj yeem xav tau cov tshuaj pleev xim kom tswj tau qhov mob nyob rau hauv lub raum lossis zais zis thaum koj dhau pob zeb. Cov pob zeb me me (ntawm 5 hli lossis tsawg dua 5 hli) feem ntau kis tau cov zis ntawm lawv tus kheej. Cov pob zeb loj dua (ntau dua 5 hli) yuav xav tau kev kho mob ntxiv. Qee lub pob zeb loj tuaj yeem yuav tsum tau muab tshem tawm uas siv cov txheej txheem xws li:

• Extracorporeal shock wave lithotripsy (ESWL): Lub suab nrov tau dhau los ntawm lub cev thiab tau tsom rau cov pob zeb zom lawv mus rau hauv cov khoom me me, passable. ESWL tej zaum yuav tsis ua haujlwm zoo rau cystine pob zeb vim lawv tau sib zog heev dua li piv nrog lwm hom pob zeb.
• Percutaneous nephrostolithotomy lossis nephrolithotomy: Lub raj me me tso rau ntawm qhov flank ncaj qha mus rau lub raum. Lub tsom iav raj yog tom qab ntawd dhau los ntawm lub raj ua kom tawg lub pob zeb hauv qab lub zeem muag ncaj qha.
• Kev siv tshuab ntsuas yob thiab cov khoom siv roj carbon fiber ntau: lub laser siv los zom cov pob zeb thiab tuaj yeem siv los kho cov pob zeb uas tsis loj dhau.

Cystinuria yog ib tug mob ntev, tag lub neej. Pob zeb feem ntau rov qab los. Txawm li cas los xij, qhov xwm txheej tsis tshua pom muaj tshwm sim los ntawm lub raum tsis ua haujlwm. Nws tsis cuam tshuam lwm yam kabmob.

Teeb meem yuav suav nrog:

• Lub qhov ncauj raug mob los ntawm pob zeb
• Raum raug mob los ntawm pob zeb
• Mob raum
• Mob raum
• Txoj hlab raum
• Mob txeeb zig

Hu rau koj tus kws kho mob yog tias koj muaj cov tsos mob ntawm lub tso mob tawm ntawm txoj hlab zis.

Muaj tshuaj uas tau noj tau yog li cystine tsis ua lub pob zeb. Nug koj tus kws kho mob txog cov tshuaj no thiab cov kev mob tshwm sim.

Ib tug neeg uas muaj keeb kwm muaj pob zeb nyob hauv lub tso zis yuav tsum haus dej kom ntau mus rau cov zis kom ntau. Qhov no tso cai rau cov pob zeb thiab lub teeb ci tawm hauv lub cev ua ntej lawv loj txaus kom ua rau cov tsos mob. Txo koj cov ntsev los yog ntsev yuav pab koj kom zoo.

Pob zeb - cystine; Cystine pob zeb

• Lub raum pob zeb thiab lithotripsy - kev tshem tawm
• Raum pob zeb - kev saib xyuas tus kheej
• Lub raum pob zeb - dab tsi yuav tsum nug koj tus kws kho mob
• Cov kab mob tso zis ntau dhau - tso tawm

• Poj niam tso zis tso zis
• Cov txiv neej tso zis
• Cystinuria
• Tus mob Nerolrolithiasis

Txwj Laug JS. Cov mob ntsws tso zis. 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 562.

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.

Lipkin ME, Ferrandino MN, Preminger GM. Soj ntsuam thiab kho mob kev kho mob ntawm urinary lithiasis. Hauv: Wein AJ, Kavoussi LR, Partin AW, Peters CA, eds. Campbell-Walsh UrologyCov. 11th ed. Philadelphia, PA: Elsevier; 2016: chap 52.

Sakhaee K, Moe OW. Urolithiasis. 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: cha 38.