Cystinuria

ʻO Cystinuria kahi ʻano maʻamau kahi i hana ʻia ai nā pōhaku mai kahi amino acid i kapa ʻia he cysteine ​​form i loko o ka puʻuwai, ureter, a me ka ʻai. Hoʻokumu ʻia ʻo Cystine ke hoʻopaʻa ʻia nā mole ʻelua o ka amino acid i kapa ʻia ʻo cysteine. Ua hala ke ʻano i loko o nā ʻohana.

I loaʻa nā ʻōuli o cystinuria, pono ʻoe e hoʻoilina i ka ʻāpana hewa mai nā mākua ʻelua. E hoʻoilina ʻia kāu mau keiki i kahi kope o ka ʻāpana hewa.

Hoʻokumu ʻia ʻo Cystinuria e ka nui o ka cystine i ka mimi. ʻO ka maʻamau, hoʻoheheʻe ka hapa nui o ka cystine a hoʻi i ke kahe o ke koko ma hope o ke komo ʻana i nā puʻupaʻa. Loaʻa i ka poʻe me cystinuria kahi kīnā kīnā ʻole e hoʻopilikia i kēia kaʻina hana. ʻO ka hopena, kūkulu ka cystine i ka mimi a hana i nā aniani a i ʻole nā ​​pōhaku. Kū paʻa paha kēia mau pōaka i nā puʻupaʻa, nā ureter, a i ʻole ka ʻaʻa.

Ma kahi o hoʻokahi o kēlā me kēia 7000 poʻe i loaʻa ka cystinuria. ʻO nā pōhaku Cystine ka mea maʻamau i ka poʻe ʻōpio ma lalo o 40 mau makahiki. Ma lalo o 3% o nā pōhaku o ka mimi he pōhaku cystine.

ʻO nā hōʻailona me:

• Koko i ka mimi
• Paʻi ʻeha a ʻeha paha ma ka ʻaoʻao a i ʻole ke kua. ʻO ka ʻeha ka pinepine ma kekahi ʻaoʻao. ʻAʻohe ʻike iki ʻia ma nā ʻaoʻao ʻelua. ʻEha pinepine ka ʻeha. E māhuahua paha ia i nā lā. Hiki paha iā ʻoe ke ʻeha i ka pelvis, ʻūhā, nā genital, a ma waena paha o ka ʻōpū luna a me ke kua.

Hoʻomaopopo pinepine ʻia ke ʻano ma hope o kahi hanana o nā pōhaku kōhaka. ʻO ka hoʻāʻo ʻana i nā pōhaku ma hope o ka hemo ʻana e hōʻike ana ua hana ʻia lākou i ka cystine.

ʻAʻole like me nā pōhaku i loaʻa i ka calcium, ʻaʻole hōʻike maikaʻi nā pōhaku cystine i nā kukuna x maʻamau.

Nā hoʻāʻo e hiki ke hana ʻia e ʻike i kēia mau pōhaku a kuhikuhi i ke ʻano me nā:

• Hōʻiliʻili mimi 24 hola
• Scanner CT ʻōpū, a i ʻole ultrasound
• ʻO ka pyelogram Intravenous (IVP)
• Urinalysis

ʻO ka pahuhopu o ka mālama ʻana e hōʻoluʻolu i nā ʻōuli a pale i nā pōhaku hou aʻe mai ka hana ʻana. Pono paha kahi kanaka me nā ʻōuli koʻikoʻi e hele i ka haukapila.

Hoʻopilikia ka mālama ʻana i ka inu ʻana i ka nui o ka wai, keu hoʻi i ka wai, e hana i ka mimi he nui. E inu ʻoe ma ka liʻiliʻi 6 a 8 mau aniani i kēlā me kēia lā. E inu ʻoe i ka wai i ka pō a ala ʻoe i ka pō ma ka liʻiliʻi i hoʻokahi manawa e hele i ka mimi.

I kekahi mau hihia, pono e hāʻawi ʻia nā wai i loko o kahi aa (e IV).

ʻO ka hana ʻana i ka mimi ʻoi aku ka alkaline i hiki ke kōkua i ka hemo ʻana i nā aniani cystine. Hana ʻia kēia me ka hoʻohana ʻana i ka potassium citrate a i ʻole sodium bicarbonate. ʻO ka ʻai ʻana i ka paʻakai liʻiliʻi hiki ke hōʻemi i ka hoʻokuʻu ʻana o ka cystine a me ka hana pōhaku.

Hiki paha iā ʻoe ke hōʻoluʻolu i ka ʻeha e kaohi i ka ʻeha ma ka puʻupaʻa a i ʻole ka wahi o ke kolo ke hala ʻoe i nā pōhaku. ʻO nā pōhaku liʻiliʻi (o 5 mm a i ʻole emi o 5 mm) hele pinepine i ka mimi ma kā lākou iho. ʻO nā pōhaku ʻoi aku ka nui (ʻoi aku ma mua o 5 mm) pono paha i nā hoʻoponopono hou aʻe. Pono paha e hemo i kekahi mau pōhaku nunui e hoʻohana ana i nā kaʻina hana e like me:

• Extracorporeal shock shock lithotripsy (ESWL): Holo ʻia nā nalu kani i ke kino a kau ʻia i nā pōhaku e haki iā lākou i nā ʻāpana liʻiliʻi, passable. ʻAʻole holo maikaʻi paha ʻo ESWL no nā pōhaku cystine no ka mea paʻakikī loa lākou ke hoʻohālikelike ʻia me nā ʻano pōhaku ʻē aʻe.
• Percutaneous nephrostolithotomy a i ʻole nephrolithotomy: Hoʻokomo ʻia kahi paipu liʻiliʻi ma o ka ʻaoʻao i loko pono o ka puʻupaʻa. Lawe ʻia kahi teleskopa ma o ka paipu e ʻāpana i ka pōhaku ma lalo o ka ʻike pololei.
• Ureteroscopy a me ka laser lithotripsy: Hoʻohana ʻia ka laser e wāwahi i nā pōhaku a hiki ke hoʻohana ʻia e mālama i nā pōhaku ʻaʻole nui loa.

ʻO Cystinuria kahi manawa lōʻihi, ola holoʻokoʻa. Hoʻi pinepine nā pōhaku. Eia nō naʻe, paʻakikī ke ʻano o ka hopena i ka holo pono ʻole ʻana o ka hakuʻala. ʻAʻole ia e pili i nā ʻokana ʻē aʻe.

Komo pū nā pilikia:

• ʻO ka hōʻino ʻia o ka puhaka mai ka pōhaku
• ʻO ka ʻeha pūhaka mai ka pōhaku
• Maʻi kīnā
• Maʻi maʻi ʻōniʻoniʻo
• Pākana Ureteral
• Maʻi ʻaʻai Urinary

Kāhea i kāu mea mālama ola inā loaʻa nā hōʻailona o nā pōhaku urinary tract.

Aia kekahi mau lāʻau i hiki ke lawe ʻia no laila ʻaʻole hana ka cystine i pōhaku. E noi i kāu mea hāʻawi e pili ana i kēia mau lāʻau a me ko lākou hopena.

ʻO kēlā me kēia kanaka me kahi mōʻaukala i ʻike ʻia o nā pōhaku i loko o ka mimi e pono e inu i ka nui o nā wai e hana mau i ka nui o ka mimi. ʻAe kēia i nā pōhaku a me nā aniani e haʻalele i ke kino ma mua o ka nui ʻana e hiki ai i nā hōʻailona. ʻO ka hōʻemi ʻana i kāu lawe ʻana i ka paʻakai a sodium paha e kōkua pū kekahi.

Pohaku - cystine; Pōhaku Cystine

• Nā pōhaku hakuʻala a me ka lithotripsy - hoʻokuʻu
• Nā pōhaku hakuʻala - mālama pono iā ʻoe iho
• Nā pōhaku hakuʻala - he aha e nīnau aku ai i kāu kauka
• Nā hanana urinary Percutaneous - hoʻokuʻu

• ʻĀpana mimi wahine
• ʻĀpana mimi kāne
• Cystinuria
• Neikolithiasis

ʻO Elder JS. Lithiasis mimi. I: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 562.

ʻO Guay-Woodford LM. Nā nephropathies hoʻoilina a me nā hanana kūpono ʻole o ke kahe urinary. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 119.

Lipkin ME, Ferrandino MN, Preminger GM. Ka loiloi a me ka hoʻokele olakino o urinary lithiasis. I: Wein AJ, Kavoussi LR, Partin AW, Peters CA, eds. Campbell-Walsh Urology. 11th hua. Piladelapia, PA: Elsevier; 2016: chap 52.

Sakhaee K, Moe OW. Urolithiasis. I: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. ʻO Brenner a me Rector's The Kidney. 11th hua. Piladelapia, PA: Elsevier; 2020: chap 38.