ICystinuria

ICystinuria yisimo esingajwayelekile lapho amatshe enziwe nge-amino acid ebizwa nge-cysteine ​​form ezinso, ureter, nasesinyeni. ICystine yakhiwa lapho ama-molecule amabili e-amino acid okuthiwa i-cysteine ​​ahlanganiswa ndawonye. Isimo sidluliselwa ngemindeni.

Ukuze ube nezimpawu ze-cystinuria, kufanele uzuze ifa lesakhi esingalungile kubo bobabili abazali. Izingane zakho zizophinda zithole ikhophi yofuzo olungalungile kuwe.

ICystinuria ibangelwa i-cystine eningi kakhulu emchameni. Imvamisa, i-cystine eningi iyancibilika ibuyele egazini ngemuva kokungena ezinso. Abantu abane-cystinuria banokukhubazeka kofuzo okuphazamisa le nqubo. Ngenxa yalokhu, i-cystine iyakheka emchameni futhi yakhe amakristalu noma amatshe. Lezi zinhlayiya zingase zinamathele ezinso, ama-ureters, noma isinye.

Cishe oyedwa kubantu abangu-7000 bane-cystinuria. Amatshe e-cystine ajwayelekile kakhulu kubantu abadala abangaphansi kweminyaka engama-40. Amatshe angaphansi kuka-3% wamatshe okugaya umchamo ngamatshe e-cystine.

Izimpawu zifaka:

• Igazi emchameni
• Ubuhlungu beFlank noma ubuhlungu ohlangothini noma emuva. Ubuhlungu buvame kakhulu ohlangothini olulodwa. Akuvamile ukuzwakala ezinhlangothini zombili. Ubuhlungu buvame ukuba nzima. Kungaba kubi ngokuhamba kwezinsuku. Ungase futhi uzwe ubuhlungu esikhaleni, emgodini, esithweni sangasese, noma phakathi kwesisu esingaphezulu nangemuva.

Isimo sitholakala kakhulu ngemuva kwesiqephu samatshe ezinso. Ukuhlola amatshe ngemuva kokuba esusiwe kukhombisa ukuthi enziwe nge-cystine.

Ngokungafani namatshe aqukethe i-calcium, amatshe e-cystine awabonakali kahle kuma-x-ray asobala.

Ukuhlolwa okungenziwa ukuthola la matshe nokuxilonga isimo kufaka:

• Ukuqoqwa komchamo wamahora angama-24
• Iskena se-CT esiswini, noma i-ultrasound
• I-pyelogram efakwa emithanjeni (IVP)
• Ukuhlolwa komchamo

Inhloso yokwelashwa ukuqeda izimpawu nokuvimbela amatshe amaningi ekwakhekeni. Umuntu onezimpawu ezinzima angadinga ukungena esibhedlela.

Ukwelashwa kubandakanya ukuphuza uketshezi oluningi, ikakhulukazi amanzi, ukukhiqiza umchamo omkhulu. Kufanele uphuze okungenani izingilazi eziyisithupha kuya kweziyisishiyagalombili ngosuku. Kufanele uphuze namanzi ebusuku ukuze uvuke ebusuku okungenani kanye ukuze udlulise umchamo.

Kwezinye izimo, uketshezi kungadingeka ukuthi lunikezwe nge-vein (nge-IV).

Ukwenza umchamo ube ne-alkaline engaphezulu kungasiza ukuncibilikisa amakristalu e-cystine. Lokhu kungenziwa ngokusetshenziswa kwe-potassium citrate noma i-sodium bicarbonate. Ukudla usawoti omncane nakho kunganciphisa ukukhishwa kwe-cystine nokwakhiwa kwamatshe.

Ungadinga ukuqeda izinhlungu ukulawula izinhlungu endaweni yezinso noma yesinye lapho udlula amatshe. Amatshe amancane (ka-5 mm noma ngaphansi kuka-5 mm) avame ukudlula emchameni ngokwawo. Amatshe amakhulu (angaphezu kwama-5 mm) angadinga ukwelashwa okwengeziwe. Amanye amatshe amakhulu angadinga ukususwa kusetshenziswa izinqubo ezifana nalezi:

• I-extracorporeal wave wave lithotripsy (ESWL): Amagagasi omsindo adluliselwa emzimbeni futhi agxile ematsheni ukuwahlephula abe izingcezu ezincane, ezidlulisekayo. I-ESWL kungenzeka ingasebenzi kahle ngamatshe e-cystine ngoba anzima kakhulu uma kuqhathaniswa nezinye izinhlobo zamatshe.
• I-nephrostolithotomy noma i-nephrolithotomy ye-Percutaneous: Ishubhu encane ibekwa ohlangothini ngqo ezinso. Isibonakude sidluliswa ngeshubhu ukuze sihlukanise itshe ngaphansi kombono oqondile.
• I-Ureteroscopy ne-laser lithotripsy: I-laser isetshenziselwa ukuqhekeza amatshe futhi ingasetshenziselwa ukwelapha amatshe angekho amakhulu kakhulu.

ICystinuria yisifo esingelapheki, sempilo yonke. Amatshe avame ukubuya. Kodwa-ke, lesi simo asivamile ukuholela ekuhlulekeni kwezinso. Akuthinti ezinye izitho.

Izinkinga zingafaka:

• Ukulimala kwesinye etsheni
• Ukulimala kwezinso etsheni
• Ukutheleleka kwezinso
• Isifo sezinso esingalapheki
• Ukuvinjelwa kwesibeletho
• Ukutheleleka ngomgudu womchamo

Shayela umhlinzeki wakho wezokunakekelwa kwezempilo uma unezimpawu zamatshe e-urinary tract.

Kunemithi engathathwa ngakho i-cystine ayakhi itshe. Buza umhlinzeki wakho mayelana nale mithi nemiphumela yako emibi.

Noma yimuphi umuntu onomlando owaziwayo wamatshe asemgodini wokuchama kufanele aphuze uketshezi oluningi ukukhiqiza njalo inani eliphezulu lomchamo. Lokhu kuvumela amatshe namakristalu ukuthi ashiye umzimba ngaphambi kokuba abe makhulu ngokwanele ukudala izimpawu. Ukwehlisa ukudla kwakho usawoti noma i-sodium kuzosiza futhi.

Amatshe - i-cystine; Amatshe e-cystine

• Izinso zamatshe kanye ne-lithotripsy - ukukhishwa
• Izinso zezinso - ukuzinakekela
• Izinso zamatshe - okufanele ubuze udokotela wakho
• Izinqubo zomchamo ezinamandla - ukukhipha

• Umgudu womchamo wabesifazane
• Umgudu womchamo wabesilisa
• ICystinuria
• I-Nephrolithiasis

Umdala JS. I-lithiasis ye-Urinary. 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 562.

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.

I-Lipkin ME, iFerrandino MN, i-Preminger GM. Ukuhlolwa nokuphathwa kwezokwelapha kwe-lithiasis ye-urinary. Ku: Wein AJ, Kavoussi LR, Partin AW, Peters CA, ama-eds. ICampbell-Walsh Urology. Umhla ka-11. IPhiladelphia, PA: Elsevier; 2016: isahluko 52.

USakhaee K, Moe OW. I-Urolithiasis. 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 38.