ʻO nā mucopolysaccharides ʻakika

ʻO ka mucopolysaccharides ʻakika kahi hōʻike e ana i ka nui o nā mucopolysaccharides i hoʻokuʻu ʻia i loko o ka mimi i loko o hoʻokahi mahele a i ʻole 24 mau hola.

ʻO nā Mucopolysaccharides he mau kaulahao lōʻihi o nā mole kō i ke kino. ʻIke pinepine ʻia lākou i ka mucus a me ka wai a puni nā hono.

No ka hoʻāʻo 24-hola, pono ʻoe i ka mimi i loko o kahi ʻeke kūikawā a i ʻole ipu i kēlā me kēia manawa ke hoʻohana ʻoe i ka lua. ʻO ka pinepine, hāʻawi ʻia ʻoe i ʻelua pahu. E mimi pololei ʻoe i loko o ka ipu kūikawā liʻiliʻi a laila hoʻoili i kēlā mimi i loko o kahi pahu nui ʻē aʻe.

• I ka lā 1, mimiki i loko o ka lua ke ala ʻoe i ke kakahiaka.
• Ma hope o ka mimiki mua, mimiki i loko o ka pahu kūikawā i kēlā me kēia manawa ke hoʻohana ʻoe i ka lua no 24 mau hola e hiki mai ana. Hoʻololi i ka mimi i loko o ka pahu nui a mālama i ka ipu nui ma kahi anuanu a i loko o ka pahu hau. E mālama paʻa i kēia ipu.
• I ka lā 2, mimi hou i loko o ka ipu i ke kakahiaka ke ala hou ʻoe a hoʻolilo i kēia mimi i ka ipu nui.
• E lepili i ka ipu nui me kou inoa, ka lā, ka manawa o ka pau ʻana, a hoʻihoʻi e like me ke kuhikuhi.

No kahi pēpē:

E holoi pono i ka wahi a puni ka urethra (ka lua e kahe ai ka mimi). E wehe i kahi ʻeke hōʻiliʻili mimi (kahi ʻeke palekiko me kahi pepa lina ma kekahi wēlau).

• No nā kāne, kau i ka peni holoʻokoʻa i loko o ka ʻeke a hoʻopili i ka pepa lina i ka ʻili.
• No nā wahine, e hoʻokau i ka ʻeke ma luna o nā pelu ʻelua o ka ʻili ma nā ʻaoʻao ʻelua o ka ʻōpū (labia). E kau i kahi diaper ma luna o ka pēpē (ma luna o ka ʻeke).

E hōʻoia pinepine i ka pēpē, a hoʻololi i ka ʻeke ma hope o ka mimiki ʻana o ke keiki. Hoʻopau i ka mimi mai ka ʻeke i loko o ka ipu i hāʻawi ʻia e kāu mea mālama ola.

Hiki i nā pēpē hana ke hoʻoneʻe i ka ʻeke, e hele ai ka mimi i loko o ka diaper. Pono ʻoe i mau ʻeke hōʻiliʻili keu.

Ke pau, e lepili i ka ipu a hoʻihoʻi e like me ka mea i haʻi ʻia iā ʻoe.

ʻAʻole pono kahi hoʻomākaukau kūikawā.

Pili ka hōʻike i ka urination maʻamau wale nō, a ʻaʻohe pilikia.

Hana ʻia kēia hōʻike e kuhikuhi i kahi hui laha ʻole o nā maʻi kūlohelohe i kapa ʻia mucopolysaccharidoses (MPS). Hoʻopili kēia, Hurler, Scheie, a me Hurler / Scheie syndromes (MPS I), Hunter syndrome (MPS II), Sanfilippo syndrome (MPS III), Morquio syndrome (MPS IV), Maroteaux-Lamy syndrome (MPS VI), a me Sly (MPS VII).

ʻO ka hapanui o nā manawa, hana ʻia kēia hoʻokolohua i nā pēpē i loaʻa paha kahi hōʻailona a i ʻole ka moʻolelo ʻohana o kekahi o kēia mau maʻi.

Kūlike nā pae maʻamau me ka makahiki a mai ka lab a lab. E kamaʻilio i kāu mea hāʻawi e pili ana i ke ʻano o kāu mau hopena hōʻike.

Hiki ke kūlike nā pae kiʻekiʻe Abnormally me kekahi ʻano o mucopolysaccharidosis. Pono nā ho'āʻo hou aʻe e hoʻoholo i ke ʻano kikoʻī o ka mucopolysaccharidosis.

AMP; Dermatan sulfate - mimi; Urine heparan sulfate; Urine dermatan sulfate; Heparan sulfate - mimi

Kumar V, Abbas AK, Aster JC. Nā maʻi genetic. I: Kumar V, Abbas AK, Aster JC, eds. ʻO Robbins a me Cotran Pathologic Basis o ka maʻi. 9th ed. Piladelapia, PA: Elsevier Saunders; 2015: chap 5.

ʻO Spranger JW. ʻO Mucopolysaccharidoses. 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 107.

Turnpenny PD, Ellard S. Nā hewa o ka metabolism. I loko o: Turnpenny PD, Ellard S, eds. Nā Emperor o nā Genetics Lapaʻau. 15th hua. Piladelapia, PA: Elsevier; 2017: chap 18.