Umbhali: Clyde Lopez
Umhla Wokudalwa: 19 Eyekhala 2021
Hlaziya Umhla: 1 Eyekhala 2025
Anonim
Mucopolysaccharide Storage Disease Type I: Hurler, Hurler-Scheie, and Scheie syndromes
Ividiyo: Mucopolysaccharide Storage Disease Type I: Hurler, Hurler-Scheie, and Scheie syndromes

I-Mucopolysaccharides yimixokelelwane emide yeemolekyuli zeswekile ezifumaneka kuwo wonke umzimba, zihlala zikwimicus nakulwelo olujikeleze amalungu. Ngokuqhelekileyo zibizwa ngokuba yi-glycosaminoglycans.

Xa umzimba ungakwazi ukwahlula i-mucopolysaccharides, imeko ebizwa ngokuba yi-mucopolysaccharidoses (MPS) iyenzeka. I-MPS ibhekisa kwiqela lokuphazamiseka kwilifa lemetabolism. Abantu abanee-MPS abanazo, okanye ezoneleyo, zezinto (i-enzyme) ezifunekayo ukwaphula imixokelelwane yemolekyuli yeswekile.

Iifom ze-MPS zibandakanya:

  • I-MPS I (i-Hurler syndrome; i-Hurler-Scheie syndrome; i-Scheie syndrome)
  • I-MPS II (isifo seHunter)
  • I-MPS III (iSanfilippo syndrome)
  • I-MPS IV (iMorquio syndrome)

IiGlycosaminoglycans; IGAG

UKumar V, u-Abbas AK, uAster JC. Ukuphazamiseka kwemfuza. Ku: Kumar V, Abbas AK, Aster JC, ii-eds. Robbins kunye neCotran Pathologic Isiseko seZifo. Umhla we-9. IPhiladelphia, PA: Elsevier Saunders; 2015: isahluko 5.

UPyeritz RE. Izifo ezizuze njengelifa zezihlunu ezinxibelelanayo. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 244.


Isitshisi iJW. Iimpawu zeMucopolysaccharidoses. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahl. 107.

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