Aminoaciduria

Aminoaciduria yog ib qho txawv txav ntawm cov amino acids nyob rau hauv cov zis. Cov amino acids yog qhov ua kom muaj cov protein hauv lub cev.

Qhov yuav kuaj tau cov zis kom huv. Qhov no feem ntau yuav ua hauv koj tus kws kho mob lub chaw haujlwm lossis chaw kuaj mob.

Feem ntau, koj tsis tas yuav ua cov kauj ruam tshwj xeeb ua ntej qhov kev kuaj no. Nco ntsoov tias koj tus kws kho mob paub txog txhua yam tshuaj koj nyuam qhuav siv. Yog tias ntsuas qhov tshuaj no rau cov menyuam mos uas noj niam mis, ua kom tus kws kho mob paub txog hom tshuaj twg uas tus niam mis noj.

Qhov kev kuaj ntshav tsuas yog tso zis ib txwm muaj xwb.

Qhov kev kuaj no yog ua los ntsuas cov amino acid qib hauv cov zis. Muaj ntau ntau hom amino acids. Nws yog ib qho muaj rau txhua yam yuav pom nyob hauv cov zis. Kev nce qib ntawm ib tus neeg cov amino acids tuaj yeem ua lub cim ntawm qhov teeb meem nrog cov metabolism.

Tus nqi tshwj xeeb yog ntsuas hauv mmol / mol creatinine. Cov nqi hauv qab no sawv cev ib txwm muaj hauv 24 teev tso zis rau cov laus.

Alanine: 9 txog 98

Arginine: 0 txog 8

Asparagine: 10 txog 65

Aspartic acid: 5 txog 50

Citrulline: 1 txog 22

Cystine: 2 txog 12

Glutamic acid: 0 txog 21

Glutamine: 11 txog 42

Glycine: 17 txog 146

Keebkwm: 49 txog 413

Isoleucine: 30 txog 186

Leucine: 1 txog 9

Lysine: 2 txog 16

Methionine: 2 txog 53

Ornithine: 1 txog 5

Phenylalanine: 1 txog 5

Proline: 3 txog 13

Pab Xim: 0 txog 9

Taurine: 18 txog 89

Threonine: 13 txog 587

Tyrosine: 3 txog 14

Valine: 3 txog 36

Cov kab ke tus nqi yuav txawv me ntsis ntawm ntau lub chaw soj nstuam. Tham nrog koj tus kws kho mob txog cov ntsiab lus ntawm koj cov kev kuaj sim.

Cov piv txwv saum toj no qhia cov kev ntsuas sib xws rau cov txiaj ntsig rau cov kev xeem no. Qee lub chaw soj nstuam siv cov kev ntsuas sib txawv los yog sim cov qauv sib txawv.

Ua kom tag nrho cov zis amino acids yuav yog vim:

• Alkaptonuria
• Tus kab mob Canavan
• Cystinosis
• Cystathioninuria
• Fructose intolerance
• Galactosemia
• Kab mob Hartnup
• Homocystinuria
• Hyperammonemia
• Hyperparathyroidism
• Maple phoov zis mob ntshav
• Methylmalonic acidemia
• Ntau myeloma
• Ornithine transcarbamylase tsis muaj peev xwm
• Osteomalacia
• Propionic acidemia
• Cov yob
• Tyrosinemia Hom 1
• Tyrosinemia hom 2
• Kab mob siab
• Wilson kab mob

Kev soj ntsuam cov menyuam mos rau cov qib amino ntau dua tuaj yeem pab daws teeb meem nrog cov metabolism. Kev kho thaum ntxov rau cov mob no tuaj yeem tiv thaiv cov teeb meem tom ntej.

Cov amino acids - cov zis; Zis amino acids

• Zis cov qauv
• Kuaj cov ntshav Aminoaciduria

Dietzen DJ. Cov amino acids, peptides, thiab cov protein. Hauv: Rifai N, ed. Tietz Phau Ntawv Sau Txog Kev Siv Science Thiab Kev Kuaj Mob MolecularCov. 6 tus ed. St Louis, MO: Elsevier; 2018: chap 28.

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.

Riley RS, McPherson RA. Kev kuaj mob ntawm cov zis. Hauv: McPherson RA, Pincus MR, eds. Henry's Kev Kuaj Mob thiab Kev Tswj Xyuas los ntawm Laboratory Txoj KevCov. 23rd ed. St Louis, MO: Elsevier; 2017: chap 28.