Tus Sau: Clyde Lopez
Hnub Kev Tsim: 19 Lub Xya Hli Ntuj 2021
Hloov Hnub: 1 Lub Rau Hlis Ntuj 2025
Anonim
Mucopolysaccharide Storage Disease Type I: Hurler, Hurler-Scheie, and Scheie syndromes
Daim Duab: Mucopolysaccharide Storage Disease Type I: Hurler, Hurler-Scheie, and Scheie syndromes

Mucopolysaccharides yog cov hlua ntev ntawm cov suab thaj molecules uas pom thoob plaws lub cev, feem ntau hauv hnoos qeev thiab hauv cov dej nyob ib ncig ntawm cov pob qij txha. Lawv feem ntau hu ua glycosaminoglycans.

Thaum lub cev tsis tuaj yeem rhuav tshem cov mucopolysaccharides, ib qho mob hu ua mucopolysaccharidoses (MPS) tshwm sim. MPS hais txog ib pawg neeg muaj keeb ntawm kev sib faib ntawm cov metabolism. Cov neeg uas muaj MPS tsis muaj ib yam khoom (enzyme) uas yuav tsum tau muab kom tshem tau cov zom cov suab thaj suab thaj.

Cov ntaub ntawv ntawm MPS suav nrog:

  • MPS I (Hurler syndrome; Hurler-Scheie syndrome; Scheie syndrome)
  • MPS II (Tus Kab Mob Saib Xyuas Kev Yos Hav Zoov)
  • MPS III (Sanfilippo syndrome)
  • MPS IV (Morquio syndrome)

Glycosaminoglycans; GAG

Kumar V, Abbas AK, Aster JC. Kev mob caj ces tsis sib xws. Hauv: Kumar V, Abbas AK, Aster JC, eds. Robbins thiab Cotran Pathologic Cov Hauv Paus Ntawm Kab MobCov. 9le ed. Philadelphia, PA: Elsevier Saunders; 2015: chap 5.

Pyeritz RE. Inherited kab mob ntawm cov nqaij mos. Hauv: Goldman L, Schafer AI, eds. Goldman-Cecil TshuajCov. 26th ed. Philadelphia, PA: Elsevier; 2020: chap 244.


Spranger JW. Mucopolysaccharidoses. Hauv: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Phau Ntawv ntawm PediatricsCov. 21st ed. Philadelphia, PA: Elsevier; 2020: chap 107.

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