Maple phoov zis mob ntshav

Maple phoov zis mob ntshav (MSUD) yog qhov tsis txaus ntseeg uas lub cev tsis tuaj yeem rhuav qee qhov ntawm cov protein. Cov zis ntawm cov neeg muaj tus mob no tuaj yeem hnov ​​ntxhiab zoo li maple phoov.

Maple phoov kua txiv kab mob (MSUD) yog tau txais cov muaj mob, uas txhais tau tias nws kis dhau los ntawm cov tsev neeg. Nws tshwm sim los ntawm qhov tsis xws luag hauv 1 ntawm 3 noob. Cov neeg muaj tus mob no tsis tuaj yeem tsoo cov amino acids leucine, isoleucine, thiab valine. Qhov no ua rau kom tsim tau cov tshuaj hauv cov ntshav no.

Hauv daim foos hnyav tshaj plaws, MSUD tuaj yeem ua rau lub hlwb puas thaum lub sijhawm kev ntxhov siab ntawm lub cev (xws li kis mob, ua npaws lossis tsis noj mov ntev heev).

Qee hom ntawm MSUD yog mob me lossis los thiab mus. Txawm hais tias qhov kev mob hnyav tshaj plaws, kev pheej rov qab ua dua dhau los ntawm kev ntxhov siab rau lub cev tuaj yeem ua rau lub hlwb tsis zoo thiab qib siab ntawm leucine tsim.

Cov tsos mob ntawm tus mob no suav nrog:

• Coma
• Noj pub nyuaj
• Qaug zog
• Qaug dab peg
• Zis uas muaj ntxhiab tsw qab xws li kua txiv kab ntxwv
• Ntuav

Cov kev sim no yuav ua tau los xyuas qhov tsis txaus siab no:

• Kev kuaj ntshav amino acid
• Tso zis kuaj cov organic acid
• Kev tshuaj ntsuam caj ces

Yuav muaj cov cim ntawm ketosis (tsim tsa ntawm ketones, ib qho los ntawm cov khoom ntawm cov roj hlawv) thiab ntau cov kua qaub hauv cov ntshav (acidosis).

Thaum kuaj pom tus mob thiab thaum sijhawm mob, kev kho mob cuam tshuam nrog kev noj zaub mov tsis muaj protein ntau. Cov kua ua kua, piam thaj, thiab qee zaum muaj cov rog tau muab los ntawm txoj hlab ntshav (IV). Kev lim ntshav hauv koj lub plab lossis txoj hlab ntshav tuaj yeem ua kom txo qis ntawm cov tshuaj tsis xwm yeem hauv koj cov ntshav.

Kev kho mob ntev yuav tsum muaj kev noj haus tshwj xeeb. Rau cov menyuam mos, cov khoom noj muaj xws li cov mis mos uas muaj cov amino acids leucine tsawg, isoleucine, thiab valine. Cov neeg muaj tus mob no yuav tsum nyob nrog noj cov zaub mov muaj amino acids rau lub neej.

Nws yog ib qho tseem ceeb heev uas ib txwm ua raws li cov zaub mov no txhawm rau tiv thaiv kev puas siab puas ntsws (puas hlwb) puas. Qhov no yuav tsum tau kuaj ntshav tas li thiab saib xyuas los ntawm tus kws qhia noj zaub mov thiab kws kho mob, nrog rau kev koom tes los ntawm niam txiv ntawm cov menyuam muaj tus mob no.

Tus kab mob no tuaj yeem tsim kev phom sij tau yog tias tsis kho.

Txawm hais tias muaj kev noj zaub mov zoo, muaj kev ntxhov siab thiab muaj mob kuj tseem tuaj yeem ua rau muaj qee cov amino acids. Kev tuag yuav tshwm sim thaum ntu no. Nrog rau kev noj zaub mov kom nruj, cov menyuam yaus tau loj tiav neeg laus thiab tuaj yeem nyob zoo.

Cov teeb meem no tuaj yeem tshwm sim:

• Neurological puas tsuaj
• Coma
• Kev Tuag
• Kev mob hlwb

Hu koj tus kws kho mob yog tias koj muaj tsev neeg muaj keeb kwm ntawm MSUD thiab tab tom npaj yuav pib tsev neeg. Hu rau koj tus kws kho mob sai li sai tau yog tias koj muaj ib tus menyuam mos uas muaj tsos mob ntawm Maple syrup tso zis mob.

Kev tawm tswv yim rau caj ces tau hais tawm rau cov neeg uas xav kom muaj menyuam thiab leej twg muaj keeb kwm keeb kwm ntawm Maple phoov zis mob ntshav qab zib. Ntau lub xeev tam sim no kuaj xyuas txhua tus menyuam mos yug nrog kuaj ntshav rau MSUD.

Yog tias kuaj ntsuas pom tias koj tus me nyuam yuav muaj MSUD, qhov kev kuaj ntshav ntxiv rau cov amino acid yuav tsum ua tam sim ntawd kom paub meej tias yog tus kab mob.

MSUD

Gallagher RC, Enns GM, Cowan TM, Mendelsohn B, Packman S. Aminoacidemias thiab organic acidemias. Hauv: Swaiman KF, Ashwal S, Ferriero DM, li al, eds. Swaiman's Pediatric Neurology: Cov Ntsiab Cai thiab Kev Coj UaCov. 6 tus ed. Philadelphia, PA: Elsevier; 2017: chap 37.

Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. Kev ua tsis taus ntawm cov metabolism hauv cov amino acids. 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: cha 103.

Merritt JL, Gallagher RC. Cov kab mob tsis txaus siab ntawm cov carbohydrate, ammonia, amino acid, thiab cov organic metabolism. Hauv: Gleason CA, Juul SE, eds. Avery Cov Kab Mob ntawm Cov Menyuam MosCov. 10 tus ed. Philadelphia, PA: Elsevier; 2018: chap 22.