Ukuphazamiseka Kwezomsoco neMetabolism

-Delile

• Isebenza kanjani imetabolism yakho?
• Kuyini ukuphazamiseka komzimba?
• Yini ebangela ukuphazamiseka komzimba?
• Izinhlobo zokuphazamiseka komzimba
• Isifo sikaGaucher
• I-glucose galactose malabsorption
• I-hemochromatosis eyifa
• Isifo se-maple syrup urine (MSUD)
• I-Phenylketonuria (PKU)
• Umbono

Isebenza kanjani imetabolism yakho?

I-Metabolism inqubo yamakhemikhali umzimba wakho oyisebenzisayo ukuguqula ukudla okudlayo kube uphethiloli okugcina uphila.

Ukudla okunomsoco (ukudla) kunamaprotheni, ama-carbohydrate, namafutha. Lezi zinto zihlukaniswa ngama-enzyme ohlelweni lwakho lokugaya ukudla, bese ziyiswa kumaseli lapho zingasetshenziswa njengophethiloli. Umzimba wakho usebenzisa lezi zinto ngokushesha, noma uzigcina esibindini, emafutheni omzimba nasezicutshini zemisipha ukuze zisetshenziswe kamuva.

Kuyini ukuphazamiseka komzimba?

Isifo se-metabolic senzeka lapho inqubo ye-metabolism yehluleka futhi idale ukuthi umzimba ube nezinto eziningi kakhulu noma ezincane kakhulu ezidingekayo ukuze uhlale uphilile.

Imizimba yethu izwela kakhulu emaphutheni asemetabolism. Umzimba kufanele ube nama-amino acid nezinhlobo eziningi zamaprotheni ukwenza yonke imisebenzi yawo. Isibonelo, ubuchopho budinga i-calcium, i-potassium, ne-sodium ukuze ikhiqize imizwa kagesi, nama-lipids (amafutha namafutha) ukugcina isimiso sezinzwa esinempilo.

Ukuphazamiseka komzimba kungathatha izinhlobo eziningi. Lokhu kufaka:

• i-enzyme noma i-vitamin elahlekile edingekayo ekuphenduleni okubalulekile kwamakhemikhali
• ukusabela okungajwayelekile kwamakhemikhali okuvimbela izinqubo ze-metabolic
• isifo esibindini, amanyikwe, izindlala ze-endocrine, noma ezinye izitho ezibandakanyeka emzimbeni
• ukuntuleka kokudla okunempilo

Yini ebangela ukuphazamiseka komzimba?

Ungaba nesifo se-metabolism uma izitho ezithile - ngokwesibonelo, amanyikwe noma isibindi - ziyeka ukusebenza kahle. Lezi zinhlobo zokuphazamiseka zingaba ngumphumela wezakhi zofuzo, ukuntuleka kwehomoni ethile noma i-enzyme, ukudla ukudla okuningi kakhulu, noma ezinye izici eziningi.

Kunamakhulu ezinkinga zofuzo zomzimba ezibangelwa ukuguqulwa kohlobo olulodwa. Lokhu kuguquka kungadluliselwa ezizukulwaneni zemindeni. Ngokusho kwe, izinhlanga ezithile noma izinhlanga ezithile kungenzeka ukuthi zidlulise izakhi zofuzo eziguquliwe ngezifo ezithile ezingakazalwa. Okuvame kakhulu kulokhu:

• i-sickle cell anemia kuma-Afrika aseMelika
• i-cystic fibrosis kubantu befa laseYurophu
• isifo se-maple syrup umchamo emiphakathini yamaMennonite
• Isifo sikaGaucher kubantu abangamaJuda abavela eMpumalanga Yurophu
• i-hemochromatosis kwabaseCaucasus e-United States

Izinhlobo zokuphazamiseka komzimba

Isifo sikashukela yisona sifo esivame kakhulu se-metabolic. Kunezinhlobo ezimbili zesifo sikashukela:

• Thayipha i-1, imbangela yayo engaziwa, yize kungaba khona isici sofuzo.
• Uhlobo 2, olungatholwa, noma olungabangelwa izici zofuzo.

Ngokwe-American Diabetes Association, izingane nabantu abadala abayizigidi ezingama-30.3, noma cishe amaphesenti angu-9.4 wabantu base-U.S. Banesifo sikashukela.

Kuhlobo lwesifo sikashukela sohlobo 1, ama-T cell ahlasela futhi abulale amaseli e-beta emanyikwe, amaseli akhiqiza i-insulin. Ngokuhamba kwesikhathi, ukuntuleka kwe-insulin kungadala:

• ukulimala kwemizwa nezinso
• ukukhubazeka kwamehlo
• ingozi eyengeziwe yesifo senhliziyo nesemithambo yegazi

Kutholwe amakhulu amaphutha azalwa nawo ku-metabolism (IEM), futhi iningi lawo lingajwayelekile kakhulu. Kodwa-ke, kulinganiselwa ukuthi i-IEM ngokuhlanganyela ithinta u-1 kuzo zonke izinsana eziyi-1 000. Eziningi zalezi zinkinga zingelashwa kuphela ngokunciphisa ukudla okudliwayo noma izinto umzimba ongenakuzicubungula.

Izinhlobo ezivame kakhulu zokuphazamiseka kokudla okunempilo nezomzimba zifaka:

Isifo sikaGaucher

Lesi simo senza ukungakwazi ukwephula uhlobo oluthile lwamafutha, aqoqana esibindini, ebunzimeni nasemnkantsheni wethambo. Lokhu kwehluleka kungadala ubuhlungu, ukulimala kwamathambo, ngisho nokufa. Iphathwa nge-enzyme therapy yokwelapha.

I-glucose galactose malabsorption

Lokhu kuyiphutha ekuhanjisweni kwe-glucose ne-galactose enqamula ulwelwesi lwesisu okuholela kuhudo olunzima nokuphela kwamanzi emzimbeni. Izimpawu zilawulwa ngokususa i-lactose, i-sucrose, ne-glucose ekudleni.

I-hemochromatosis eyifa

Kulesi simo, i-iron eyeqile ifakwa ezingxenyeni eziningana, futhi ingadala:

• i-cirrhosis yesibindi
• umdlavuza wesibindi
• isifo sikashukela
• Isifo senhliziyo

Ilashwa ngokususa igazi emzimbeni (phlebotomy) njalo.

Isifo se-maple syrup urine (MSUD)

I-MSUD iphazamisa imetabolism yama-amino acid athile, edala ukonakala okusheshayo kwama-neurons. Uma ingalashwa, ibangela ukufa ezinyangeni ezimbalwa zokuqala izelwe. Ukwelashwa kubandakanya ukufaka umkhawulo ekudleni kokudla kwama-amino acid we-branched-chain.

I-Phenylketonuria (PKU)

I-PKU ibangela ukwehluleka ukukhiqiza i-enzyme, i-phenylalanine hydroxylase, okuholela ekulimaleni komzimba, ukubambezeleka kwengqondo, nokuma okungajwayelekile. Kulashwa ngokunciphisa ukusetshenziswa kokudla kwezinhlobo ezithile zamaprotheni.

Umbono

Ukuphazamiseka kwemetabolism kuyinkimbinkimbi kakhulu futhi akuvamile. Noma kunjalo, bayindaba yocwaningo oluqhubekayo, olusiza nososayensi ukuthi baqonde kangcono izimbangela zezinkinga ezivame kakhulu njenge-lactose, i-sucrose, ne-glucose ukungabekezelelani, nokugcwala kwamaprotheni athile.

Uma unesifo se-metabolic, ungasebenzisana nodokotela wakho ukuthola uhlelo lokwelashwa olulungele wena.