Iicidi ze-mucopolysaccharides

I-acid mucopolysaccharides luvavanyo olulinganisa inani le-mucopolysaccharides ekhutshwe kumchamo nokuba kukwisiqendu esinye okanye ngaphezulu kweeyure ezingama-24.

I-Mucopolysaccharides yimixokelelwane ende yeemolekyuli zeswekile emzimbeni. Zifumaneka rhoqo kwi-mucus nakulwelo olujikeleze amalungu.

Kuvavanyo lweeyure ezingama-24, kufuneka uchame ubhaka okanye isikhongozeli ngalo lonke ixesha usebenzisa igumbi lokuhlambela. Rhoqo, uya kunikwa izikhongozeli ezibini. Uya kuchama ngqo kwisikhongozeli esincinci esincinci kwaye emva koko udlulisele umchamo kwesinye isitya esikhulu.

• Ngomhla woku-1, chamela kwindlu yangasese xa uvuka kusasa.
• Emva kokuchama okokuqala, chamela kwisitya esikhethekileyo ngalo lonke ixesha usebenzisa igumbi lokuhlambela iiyure ezingama-24 ezizayo. Dlulisa umchamo kwisitya esikhulu kwaye ugcine isitya esikhulu kwindawo epholileyo okanye kwifriji. Gcina esi sikhongozeli sivalekile.
• Ngomhla wesi-2, chamela kwisitya kusasa kwakhona xa uvuka kwaye ugqithisela lo mchamo kwisitya esikhulu.
• Bhala ileyibhile enkulu ngegama lakho, umhla, ixesha lokugqitywa, kwaye uyibuyise njengoko kuyalelwe.

Yosana:

Geza kakuhle indawo ejikeleze i-urethra (umngxuma ophuma kuwo umchamo). Vula ingxowa yokuqokelela umchamo (ibhegi yeplastiki enephepha lokuncamathela kwelinye icala).

• Kumadoda, beka yonke incanca engxoweni kwaye uncamathisele iphepha lokuncamathelisa ulusu.
• Kwababhinqileyo, beka ibhegi ngaphezulu kwemiphetho emibini yolusu ngapha nangapha kwelungu lobufazi (ilebhu). Beka ilweyile emntwaneni (ngaphezulu kwengxowa).

Jonga usana rhoqo, kwaye utshintshe ibhegi emva kokuba usana luchamile. Khuphela umchamo engxoweni kwisitya esinikezwe ngumboneleli wakho wezempilo.

Iintsana ezisebenzayo zinokuhambisa ingxowa, zibangele umchamo ungene kwi-diaper. Unokufuna iibhegi zokuqokelela ezongezelelweyo.

Xa ugqibile, bhala isitya uze usibuyisele njengoko uxelelwe.

Akukho lungiselelo lukhethekileyo luyafuneka.

Uvavanyo lubandakanya kuphela ukuchama okuqhelekileyo, kwaye akukho kungonwabi.

Olu vavanyo lwenziwa ukufumanisa iqela elinqabileyo lokuphazamiseka kwemfuza elibizwa ngokuba yi-mucopolysaccharidoses (MPS). Oku kubandakanya, i-Hurler, Scheie, kunye ne-Hurler / Scheie syndromes (MPS I), Hunter syndrome (MPS II), Sanfilippo syndrome (MPS III), Morquio syndrome (MPS IV), Maroteaux-Lamy syndrome (MPS VI), kunye neSly syndrome (MPS VII).

Uninzi lwexesha, olu vavanyo lwenziwa kwiintsana ezinokuba neempawu okanye imbali yosapho yenye yezi ngxaki.

Amanqanaba aqhelekileyo ahluka ngokobudala kunye nokusuka kwelebhu ukuya kwelebhu. Thetha nomboneleli wakho malunga nentsingiselo yeziphumo zakho zovavanyo.

Amanqanaba aphezulu ngokungaqhelekanga anokuhambelana nohlobo lwe-mucopolysaccharidosis. Olunye uvavanyo luyafuneka ukumisela uhlobo oluthile lwe-mucopolysaccharidosis.

AMP; I-Dermatan sulfate - umchamo; Umchamo heparan sulphate; Umchamo wedermatan sulphate; I-Heparan sulphate - umchamo

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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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