ʻO Mucopolysaccharides

ʻO nā mucopolysaccharides he mau kaulahao lōʻihi o nā mole kō i loaʻa i loko o ke kino, pinepine i ka mucus a me nā wai e pili ana i nā hono. Kāhea ʻia lākou he glycosaminoglycans.

Ke hiki ʻole i ke kino ke uhaʻi i nā mucopolysaccharides, hiki i kahi ʻano i kapa ʻia mucopolysaccharidoses (MPS). Pili ʻo MPS i kahi hui o nā maʻi hoʻoilina o ka metabolism. ʻAʻohe o ka poʻe me MPS, a lawa paha, o kahi mea (enzyme) e pono ai e wāwahi i nā kaulahao mole kō.

Loaʻa nā ʻano o MPS:

• MPS I (Hurler Syndrome; Hurler-Scheie maʻi pale; Scheie maʻi pale)
• MPS II (Hunter syndrome)
• MPS III (Sanfilippo syndrome)
• MPS IV (Morquio maʻi pale)

ʻO Glycosaminoglycans; GAG

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 Pyeritz RE. Nā maʻi i hoʻoilina ʻia no nā mea pili. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 244.

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.