Mathata a Phepo e nepahetseng le Metabolism

Litaba

• Metabolism ea hau e sebetsa joang?
• Bothata ba metabolism ke eng?
• Ke eng e bakang mathata a metabolism?
• Mefuta ea mathata a metabolism
• Lefu la Gaucher
• Glucose galactose malabsorption
• Lefutso hemochromatosis
• Lefu la moroto oa moroto (MSUD)
• Phenylketonuria (PKU)
• Ponahalo

Metabolism ea hau e sebetsa joang?

Metabolism ke ts'ebetso ea 'mele eo' mele oa hau o e sebelisang ho fetola lijo tseo o li jang mafura a u phelisang.

Phepo e nepahetseng (lijo) e na le liprotheine, lik'habohaedreite le mafura. Lintho tsena li senyeha ke li-enzyme tsamaisong ea hau ea lijo, ebe li isoa liseleng moo li ka sebelisoang e le mafura. 'Mele oa hau o sebelisa lintho tsena hanghang, kapa oa li boloka sebeteng, mafura a' mele le mesifa ea mesifa hore e tle e sebelisoe hamorao.

Bothata ba metabolism ke eng?

Boloetse ba ts'oaetso ea metabolism bo hlaha ha ts'ebetso ea metabolism e hloleha mme e etsa hore 'mele o be le lintho tsa bohlokoa haholo kapa tse fokolang haholo tse hlokahalang ho lula o phetse hantle.

'Mele ea rona e ela hloko liphoso tsa metabolism. 'Mele o tlameha ho ba le li-amino acid le mefuta e mengata ea liprotheine ho etsa mesebetsi eohle ea eona. Mohlala, boko bo hloka calcium, potasiamo, le sodium ho hlahisa tšusumetso ea motlakase, le lipids (mafura le oli) ho boloka tsamaiso ea methapo e phetseng hantle.

Mathata a metabolism a ka hlaha ka mefuta e mengata. Sena se kenyelletsa:

• enzyme kapa vithamine e haellang e hlokahalang bakeng sa tšebetso ea bohlokoa ea lik'hemik'hale
• liphetoho tse sa tloaelehang tsa lik'hemik'hale tse sitisang ts'ebetso ea metabolism
• lefu le sebeteng, manyeme, litšoelesa tsa endocrine, kapa litho tse ling tse amehang metabolism
• khaello ea phepo e nepahetseng

Ke eng e bakang mathata a metabolism?

O ka ba le bothata ba ts'oaetso ea mmele haeba litho tse ling - ka mohlala, manyeme kapa sebete - li emisa ho sebetsa hantle. Mefuta ena ea mathata e ka bakoa ke liphatsa tsa lefutso, khaello ea hormone e itseng kapa enzyme, ho ja lijo tse ngata haholo, kapa mabaka a mang a mangata.

Ho na le mathata a makholo a liphatsa tsa lefutso a bakoang ke liphetoho tsa lefutso le le leng. Liphetoho tsena li ka fetisoa ka meloko ea malapa. Ho ea ka, merabe e meng kapa merabe e meng e na le monyetla oa ho fetisa liphatsa tsa lefutso tse fetotsoeng bakeng sa mathata a itseng a tlhaho. Tse tloaelehileng haholo ho tsena ke:

• sickle cell anemia ho Maafrika a Amerika
• cystic fibrosis bathong ba lefa la Europe
• Mafu a moroto a maple sirapo metseng ea Mamennone
• Lefu la Gaucher bathong ba Bajuda ba tsoang Europe Bochabela
• hemochromatosis ho batho ba Caucasus ba United States

Mefuta ea mathata a metabolism

Lefu la tsoekere ke lefu le atileng haholo la ts'oaetso. Ho na le mefuta e 'meli ea lefu la tsoekere:

• Type 1, eo sesosa sa eona se sa tsejoeng, leha ho ka ba le lebaka la lefutso.
• Mofuta oa 2, o ka fumanoang, kapa o ka bakoang ke liphatsa tsa lefutso.

Ho ea ka American Diabetes Association, bana le batho ba baholo ba limilione tse 30,3, kapa liperesente tse ka bang 9.4 tsa baahi ba U.S. ba na le lefu la tsoekere.

Mofuteng oa lefu la tsoekere oa mofuta oa 1, lisele tsa T li hlasela le ho bolaea lisele tsa beta manyeme, lisele tse hlahisang insulin. Ha nako e ntse e feta, khaello ea insulin e ka baka:

• tšenyo ea methapo le liphio
• ho holofala mahlo
• kotsi e kholo ea lefu la pelo le vascular

Ho fumanoe makholo a makholo a liphoso tsa tlhaho ea metabolism (IEM), 'me boholo ba ona bo sa tloaelehang haholo. Leha ho le joalo, ho hakanngoa hore IEM ka kakaretso e ama ngoana a le mong ho ba bang le ba bang ba 1000. Bongata ba mathata ana a ka phekoloa feela ka ho fokotsa ho ja lijo tsa ntho kapa lintho tseo 'mele o ke keng oa li sebetsana.

Mefuta e tloaelehileng ea mathata a phepo e nepahetseng le a ts'oaetso e kenyelletsa:

Lefu la Gaucher

Boemo bona bo baka ho se khone ho senya mofuta o itseng oa mafura, a bokellanang sebeteng, sebeteng le mokong oa masapo. Ho se khonehe hona ho ka baka bohloko, tšenyo ea masapo, esita le lefu. E alafshoa ka kalafo e nchafatsang ea enzyme.

Glucose galactose malabsorption

Hona ke bofokoli ba ho tsamaisa tsoekere le galactose ho parola mpa ea mpa ho lebisang ho letšollo le ho hloka metsi. Matšoao a laoloa ka ho tlosa lactose, sucrose le glucose lijong.

Lefutso hemochromatosis

Boemong bona, tšepe e fetelletseng e kenngoa likarolong tse 'maloa,' me e ka baka:

• ho thatafala ha sebete
• Kankere ea sebete
• lefu la tsoekere
• lefu la pelo

E alafshoa ka ho tlosa mali 'meleng (phlebotomy) khafetsa.

Lefu la moroto oa moroto (MSUD)

MSUD e senya metabolism ea li-amino acid tse itseng, e baka ho senyeha kapele ha methapo ea kutlo. Haeba e sa phekoloe, e baka lefu nakong ea likhoeli tse 'maloa tsa pele ka mor'a ho hlaha. Kalafo e kenyelletsa ho fokotsa phepo ea phepo ea li-amino acid tse nang le li-branched.

Phenylketonuria (PKU)

PKU e baka ho hloleha ho hlahisa enzyme, phenylalanine hydroxylase, e hlahisang tšenyo ea setho, ho holofala kelellong le boemo bo sa tloaelehang. E alafshoa ka ho fokotsa phepo ea lijo ea mefuta e itseng ea protheine.

Ponahalo

Mathata a metabolism a rarahane haholo ebile ha a fumanehe hangata. Leha ho le joalo, ke taba ea lipatlisiso tse tsoelang pele, tse thusang bo-ramahlale ho utloisisa hantle lisosa tsa mathata a atileng haholo joalo ka lactose, sucrose le ho hloka mamello ha tsoekere, le ho tlallana ha liprotheine tse itseng.

Haeba u na le bothata ba ts'oaetso, u ka sebetsa le ngaka ea hau ho fumana moralo oa kalafo o u loketseng.