Cystinuria

Cystinuria ke boemo bo sa tloaelehang moo majoe a entsoeng ka amino acid e bitsoang cysteine ​​a bōpang liphio, ureter le senya. Cystine e thehoa ha limolek'hule tse peli tsa amino acid tse bitsoang cysteine ​​li hokahane. Boemo bona bo fetisoa ke malapa.

Ho ba le matšoao a cystinuria, o tlameha ho rua lefutso le fosahetseng ho batsoali ka bobeli. Bana ba hau le bona ba tla rua khophi ea lefutso le fosahetseng ho uena.

Cystinuria e bakoa ke cystine e ngata haholo morong. Ka tloaelo, cystine e ngata e qhala ebe e khutlela maling kamora ho kena liphio. Batho ba nang le cystinuria ba na le bofokoli ba lefutso bo sitisang ts'ebetso ena. Ka lebaka leo, cystine e ba teng ka har'a moroto ebe e etsa likristale kapa majoe. Makhalase ana a ka 'na a khomarela liphio, li-ureters kapa senya.

Hoo e ka bang motho a le mong ho batho ba 7000 o na le cystinuria. Majoe a cystine a atile haholo ho bacha ba ka tlase ho lilemo tse 40. Majoe a ka tlase ho 3% a majoe a ho ntša metsi ke majoe a cystine.

Matšoao a kenyelletsa:

• Mali ka morong
• Bohloko ba mahlakore kapa bohloko lehlakoreng kapa mokokotlong. Hangata bohloko bo lehlakoreng le le leng. Ke ka seoelo e utluoang ka mahlakore ka bobeli. Bohloko hangata bo ba matla. E kanna ea mpefala ha matsatsi a ntse a feta. U kanna oa utloa bohloko mahalapeng, mabitleng, liphateng tsa botona kapa botšehali, kapa lipakeng tsa mpa le mokokotlo.

Boemo bona bo fumanoa hangata kamora ketsahalo ea majoe a liphio. Ho leka majoe kamora hore a tlosoe ho bontša hore a entsoe ka cystine.

Ho fapana le majoe a nang le calcium, majoe a cystine ha a hlahe hantle mahlaseling a hlakileng a x-ray.

Liteko tse ka etsoang ho fumana majoe ana le ho fumana boemo bo kenyelletsa:

• Pokello ea moroto oa lihora tse 24
• Mpa ea ka mpeng ea CT, kapa ultrasound
• Pyelogram e kenang ka hare (IVP)
• Ho hlahloba urinal

Morero oa kalafo ke ho imolla matšoao le ho thibela majoe a mangata ho theha. Motho ea nang le matšoao a matla a ka hloka ho kena sepetlele.

Kalafo e kenyelletsa ho noa maro a mangata, haholo metsi, ho hlahisa moroto o mongata. U lokela ho noa bonyane likhalase tse 6 ho isa ho tse 8 ka letsatsi. U lokela ho noa metsi le bosiu e le hore u tsohe bosiu bonyane hanngoe ho feta moroto.

Maemong a mang, maro a ka hloka ho fanoa ka mothapo (ka IV).

Ho etsa hore moroto o be le alkaline e ngata ho ka thusa ho qhala likristale tsa cystine. Sena se ka etsoa ka tšebeliso ea potassium citrate kapa sodium bicarbonate. Ho ja letsoai le lenyane ho ka fokotsa tlhahiso ea cystine le sebopeho sa majoe.

U kanna ua hloka litheko tse kokobetsang bohloko ho laola bohloko ba liphio kapa sebaka sa senya ha u feta majoe. Majoe a manyane (a 5 mm kapa a ka tlase ho 5 mm) hangata a feta ka moroto ka botsona. Majoe a maholo (a fetang 5 mm) a ka hloka kalafo e eketsehileng. Majoe a mang a maholo a ka hloka ho tlosoa ka mekhoa e kang:

• Extracorporeal shock wave lithotripsy (ESWL): Maqhubu a molumo a feta 'meleng' me a shebane le majoe ho a roba likotoana tse nyane tse fetisang. ESWL e kanna ea se sebetse hantle bakeng sa majoe a cystine hobane a thata haholo ha a bapisoa le mefuta e meng ea majoe.
• Percutaneous nephrostolithotomy kapa nephrolithotomy: Phala e nyane e beoa ka lehlakoreng ka kotloloho ho liphio. Joale sebonela-hōle se fetisoa ka har'a tube ho arola lejoe ka pono e tobileng.
• Ureteroscopy le laser lithotripsy: Laser e sebelisoa ho roba majoe mme e ka sebelisoa ho alafa majoe a seng maholo haholo.

Cystinuria ke boemo bo sa foleng, ba bophelo bohle. Majoe a khutla hangata. Leha ho le joalo, boemo bona hangata bo baka ho hloleha ha liphio. Ha e ame litho tse ling tsa mmele.

Mathata a ka kenyelletsa:

• Kotsi ea senya e tsoang lejoeng
• Kotsi ea liphio lejoeng
• Tšoaetso ea liphio
• Lefu le sa foleng la liphio
• Tšitiso ea mali
• Tšoaetso ea pampitšana ea moroto

Bitsa mofani oa tlhokomelo ea bophelo haeba u e-na le matšoao a majoe a mosese.

Ho na le meriana e ka nooang kahoo cystine e sa etse lejoe. Botsa mofani oa hau ka meriana ena le litla-morao tsa eona.

Motho ofe kapa ofe ea nang le nalane e tsebahalang ea majoe a pampitšaneng ea ho ntša metsi o lokela ho noa maro a mangata ho hlahisa moroto khafetsa. Sena se lumella majoe le likristale ho tsoa 'meleng pele li ka ba kholo ho lekana ho baka matšoao. Ho fokotsa ho noa letsoai kapa sodium le hona ho tla thusa.

Majoe - cystine; Majoe a cystine

• Majoe a liphio le lithotripsy - ho tsoa
• Liphio tsa liphio - ho itlhokomela
• Liphio tsa liphio - seo u lokelang ho se botsa ngaka ea hau
• Mekhoa ea ho ntša metsi ka makhetlo a mangata - ho tsoa

• Mosali pampitšana ea mosese
• Mokhoa oa banna oa ho ntša metsi
• Cystinuria
• Nephrolithiasis

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Guay-Woodford LM. Lefa la nephropathies le kholo e sa tloaelehang ea nts'etsopele ea pampitšana ea ho ntša metsi. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 26th. Philadelphia, PA: Elsevier; 2020: khaolo ea 119.

Lipkin ME, Ferrandino MN, Preminger GM. Tlhatlhobo le taolo ea bongaka ea lithiasis ea ho ntša metsi. Ka: Wein AJ, Kavoussi LR, Partin AW, Peters CA, bahlophisi. Urology ea Campbell-Walsh. 11th ed. Philadelphia, PA: Elsevier; 2016: khaolo ea 52.

Sakhaee K, Moe OW. Urolithiasis. Ka: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, li-eds. Brenner le Rector ea Liphio. 11th ed. Philadelphia, PA: Elsevier; 2020: khaolo ea 38.