ICystinuria

ICystinuria yimeko enqabileyo apho amatye enziwe ngeamino acid ebizwa ngokuba yicysteine ​​form kwizintso, ureter, kunye bladder. ICystine yenziwa xa iimolekyuli ezimbini zeamino acid ebizwa ngokuba yicysteine ​​zibotshelelwe kunye. Imeko idluliselwa kwiintsapho.

Ukuze ube neempawu ze-cystinuria, kuya kufuneka ulidle ilifa i-gene engalunganga kubazali bobabini. Abantwana bakho baya kubudla ilifa ikopi yohlobo olungalunganga kuwe.

I-cystinuria ibangelwa yi-cystine eninzi kumchamo. Ngokwesiqhelo, uninzi lwecystine luyanyibilika kwaye lubuyele egazini emva kokungena kwizintso. Abantu abane-cystinuria banesiphene semfuza esiphazamisa le nkqubo. Ngenxa yoko, icystine iyakha kumchamo kwaye yenze iikristali okanye amatye. Ezi kristali zinokubambeka kwizintso, ureters, okanye isinyi.

Phantse umntu omnye kuma-7000 abantu bane-cystinuria. Amatye e-cystine aqheleke kakhulu kubantu abadala abancinci abangaphantsi kweminyaka engama-40. Ngaphantsi kwe-3% yamatye e-urinary amatye e-cystine.

Iimpawu zibandakanya:

• Igazi kumchamo
• Intlungu yeflank okanye iintlungu ecaleni okanye ngasemva. Iintlungu zihlala zihlala kwicala elinye. Kunqabile ukuziva kumacala omabini. Ubuhlungu buhlala bunzima. Inokuba mandundu ngokuhamba kweentsuku. Unokuziva ubuhlungu kwi-pelvis, kwi-groin, kwizitho zangasese, okanye phakathi kwesisu esingaphezulu kunye nasemva.

Imeko ihlala ichongwa emva kwesiqendu samatye ezintso. Ukuvavanya amatye emva kokuba esusiwe kubonisa ukuba enziwe ngecystine.

Ngokungafaniyo namatye ane-calcium, amatye e-cystine akabonakali kakuhle kwi-x-ray ecacileyo.

Uvavanyo olunokwenziwa ukufumana la matye kunye nokuchonga imeko kubandakanya:

• Ukuqokelela umchamo iiyure ezingama-24
• Ukuskena kwe-CT esiswini, okanye i-ultrasound
• Ipyramogram efakwa ngaphakathi (IVP)
• Uhlalutyo lomchamo

Injongo yonyango kukhulula iimpawu kunye nokuthintela amatye amaninzi ekwenzeni. Umntu oneempawu ezinzima usenokufuna ukuya esibhedlele.

Unyango lubandakanya ukusela amanzi amaninzi, ngakumbi amanzi, ukuvelisa umchamo omkhulu. Kufuneka usele iiglasi ezi-6 ukuya kwezi-8 ngosuku. Kufuneka usele amanzi nasebusuku ukuze uvuke ebusuku nokuba kube kanye ukhupha umchamo.

Ngamanye amaxesha ulwelo lunokufuna ukunikezelwa nge-vein (nge-IV).

Ukwenza umchamo ube nealkaline ngakumbi kunokunceda ukuncibilikisa iikristale zecystine. Oku kunokwenziwa kusetyenziswa i-potassium citrate okanye isodium bicarbonate. Ukutya ityuwa encinci kunokunciphisa ukukhutshwa kwecystine kunye nokwenziwa kwamatye.

Ungadinga ukuphelisa iintlungu ukulawula intlungu kwizintso okanye kwindawo yesinyi xa udlula amatye. Amatye amancinci (ama-5 mm okanye ngaphantsi kwe-5 mm) ahlala edlula kumchamo ngokwawo. Amatye amakhulu (ngaphezulu kwe-5 mm) anokufuna unyango olongezelelekileyo. Amanye amatye amakhulu anokufuna ukuba asuswe kusetyenziswa iinkqubo ezinje:

• I-extracorporeal wave wave lithotripsy (ESWL): Amagagasi esandi adluliselwa emzimbeni kwaye ajolise ematyeni ukuze aqhekeke abe ngamaqhekeza amancinci, anokugqitha. I-ESWL ayinakusebenza kakuhle kumatye e-cystine kuba anzima kakhulu xa kuthelekiswa nezinye iintlobo zamatye.
• I-Percutaneous nephrostolithotomy okanye i-nephrolithotomy: Ityhubhu encinci ibekwa ngasecaleni ngqo kwizintso. Iteleskopu emva koko idluliswa ngombhobho ukuze iqhekeze ilitye phantsi kwembono ethe ngqo.
• I-Ureteroscopy kunye ne-laser lithotripsy: I-laser isetyenziselwa ukuqhekeza amatye kwaye inokusetyenziselwa ukunyanga amatye angenkulwanga kakhulu.

ICystinuria yimeko engapheliyo, yobomi bonke. Amatye ahlala ebuya. Nangona kunjalo, imeko enqabileyo ibangela ukusilela kwezintso. Ayichaphazeli amanye amalungu.

Iingxaki zinokubandakanya:

• Ukulimala kwesinyi kwilitye
• Ukwenzakala kwezintso elityeni
• Usulelo lwezintso
• Isifo sezintso esinganyangekiyo
• Ukuphazamiseka kwethambo
• Usulelo lwe-Urinary tract

Tsalela umnxeba umboneleli wakho ukuba unempawu zamatye e-urinary.

Kukho amayeza anokuthathwa ukuze i-cystine ayenzi litye. Buza umboneleli wakho malunga nala mayeza kunye neziphumo ebezingalindelekanga.

Nawuphi na umntu onembali eyaziwayo yamatye kwindawo yokuchama kufuneka asele ulwelo oluninzi ukuvelisa rhoqo umchamo omkhulu. Oku kuvumela amatye kunye neekristali ukuba ziwushiye umzimba ngaphambi kokuba zikhulu ngokwaneleyo ukuba zibangele iimpawu. Ukunciphisa ukutya kwakho ityuwa okanye isodiyam kuya kunceda ngokunjalo.

Amatye - icystine; Amatye ecystine

• Izintso kunye ne-lithotripsy - ukukhutshwa
• Izintso zamatye - ukuzinyamekela
• Izintso zamatye - yintoni oza kuyibuza ugqirha wakho
• Iinkqubo ezisebenzayo zokuchama-ukukhupha

• Iphecana lomchamo labasetyhini
• Indoda yokuchama
• ICystinuria
• I-Nephrolithiasis

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