{"id":13036,"date":"2025-07-22T08:25:02","date_gmt":"2025-07-22T12:25:02","guid":{"rendered":"https:\/\/www.rigicon.com\/glossary-term\/amino-aciduria\/"},"modified":"2025-07-22T08:25:02","modified_gmt":"2025-07-22T12:25:02","slug":"amino-aciduria","status":"publish","type":"glossary-term","link":"https:\/\/www.rigicon.com\/hi\/glossary-term\/amino-aciduria\/","title":{"rendered":"\u090f\u092e\u093f\u0928\u094b \u090f\u0938\u093f\u0921\u0941\u0930\u093f\u092f\u093e (Amino 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\u0938\u0902\u0915\u0947\u0924 \u0926\u0947\u0924\u093e \u0939\u0948\u0964<sup>1<\/sup> \u092f\u0939 \u0938\u094d\u0925\u093f\u0924\u093f \u0924\u092c \u0939\u094b\u0924\u0940 \u0939\u0948 \u091c\u092c \u092e\u0942\u0924\u094d\u0930 \u092e\u0947\u0902 \u0905\u0938\u093e\u092e\u093e\u0928\u094d\u092f \u0930\u0942\u092a \u0938\u0947 \u0909\u091a\u094d\u091a \u092e\u093e\u0924\u094d\u0930\u093e \u092e\u0947\u0902 \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u0939\u094b\u0924\u0947 \u0939\u0948\u0902, \u091c\u094b \u0936\u0930\u0940\u0930 \u092e\u0947\u0902 \u092a\u094d\u0930\u094b\u091f\u0940\u0928 \u0915\u0947 \u0932\u093f\u090f \u0928\u093f\u0930\u094d\u092e\u093e\u0923 \u0916\u0902\u0921 \u0939\u094b\u0924\u0947 \u0939\u0948\u0902\u0964<sup>2<\/sup> \u090f\u0915 \u0938\u094d\u0935\u0938\u094d\u0925 \u0917\u0941\u0930\u094d\u0926\u0947 \u092e\u0947\u0902, \u0917\u094d\u0932\u094b\u092e\u0947\u0930\u0941\u0932\u0940 \u0930\u0915\u094d\u0924 \u0938\u0947 \u0938\u092d\u0940 \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u0915\u094b \u092b\u093c\u093f\u0932\u094d\u091f\u0930 \u0915\u0930\u0924\u093e \u0939\u0948, \u0914\u0930 \u0917\u0941\u0930\u094d\u0926\u0947 \u0915\u0940 \u0928\u0932\u093f\u0915\u093e\u090f\u0902 \u092b\u093c\u093f\u0932\u094d\u091f\u0930 \u0915\u093f\u090f \u0917\u090f 95% \u0938\u0947 \u0905\u0927\u093f\u0915 \u0915\u094b \u092a\u0941\u0928: \u0905\u0935\u0936\u094b\u0937\u093f\u0924 \u0915\u0930 \u0932\u0947\u0924\u0940 \u0939\u0948\u0902\u0964 \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u0935\u093e\u092a\u0938 \u0930\u0915\u094d\u0924 \u092e\u0947\u0902\u0964<sup>3<\/sup><\/p>\n\n <p>\u0905\u092e\u0940\u0928\u094b-\u090f\u0938\u093f\u0921\u094d\u092f\u0942\u0930\u093f\u092f\u093e \u0915\u094b \u0905\u0902\u0924\u0930\u094d\u0928\u093f\u0939\u093f\u0924 \u0924\u0902\u0924\u094d\u0930 \u0915\u0947 \u0906\u0927\u093e\u0930 \u092a\u0930 \u0926\u094b \u092e\u0941\u0916\u094d\u092f \u092a\u094d\u0930\u0915\u093e\u0930\u094b\u0902 \u092e\u0947\u0902 \u0935\u0930\u094d\u0917\u0940\u0915\u0943\u0924 \u0915\u093f\u092f\u093e \u091c\u093e \u0938\u0915\u0924\u093e \u0939\u0948:<\/p>\n\n <p>1. <strong>\u0905\u0924\u093f\u092a\u094d\u0930\u0935\u093e\u0939 \u0905\u092e\u0940\u0928\u094b-\u090f\u0938\u093f\u0921\u0941\u0930\u093f\u092f\u093e<\/strong>: \u0924\u092c \u0939\u094b\u0924\u093e \u0939\u0948 \u091c\u092c \u0930\u0915\u094d\u0924 \u092a\u094d\u0932\u093e\u091c\u094d\u092e\u093e \u092e\u0947\u0902 \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u0915\u0940 \u0905\u0938\u093e\u092e\u093e\u0928\u094d\u092f \u0930\u0942\u092a \u0938\u0947 \u0909\u091a\u094d\u091a \u0938\u093e\u0902\u0926\u094d\u0930\u0924\u093e \u0935\u0943\u0915\u094d\u0915 \u0928\u0932\u093f\u0915\u093e\u0913\u0902 \u0915\u0940 \u0905\u0935\u0936\u094b\u0937\u0923 \u0915\u094d\u0937\u092e\u0924\u093e \u0915\u094b \u092a\u094d\u0930\u092d\u093e\u0935\u093f\u0924 \u0915\u0930\u0924\u0940 \u0939\u0948, \u091c\u093f\u0938\u0915\u0947 \u092a\u0930\u093f\u0923\u093e\u092e\u0938\u094d\u0935\u0930\u0942\u092a \u092e\u0942\u0924\u094d\u0930 \u092e\u0947\u0902 \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u0915\u0940 \u0909\u091a\u094d\u091a \u0938\u093e\u0902\u0926\u094d\u0930\u0924\u093e \u0939\u094b\u0924\u0940 \u0939\u0948\u0964<sup>4<\/sup> \u092f\u0939 \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u091a\u092f\u093e\u092a\u091a\u092f \u0915\u0947 \u091c\u0928\u094d\u092e\u091c\u093e\u0924 \u0935\u093f\u0915\u093e\u0930\u094b\u0902 \u0915\u0947 \u0915\u093e\u0930\u0923 \u0939\u094b \u0938\u0915\u0924\u093e \u0939\u0948, \u091c\u0948\u0938\u0947 \u0915\u093f \u092b\u0947\u0928\u093f\u0932\u0915\u0947\u091f\u094b\u0928\u0941\u0930\u093f\u092f\u093e, \u092f\u093e \u092f\u0915\u0943\u0924 \u0915\u0947 \u0932\u093f\u090f \u092e\u093e\u0927\u094d\u092f\u092e\u093f\u0915 \u0939\u094b \u0938\u0915\u0924\u093e \u0939\u0948 \u0930\u094b\u0917.<sup>3<\/sup><\/p>\n\n <p>2. <strong>\u0930\u0940\u0928\u0932 \u0905\u092e\u0940\u0928\u094b-\u090f\u0938\u093f\u0921\u0941\u0930\u093f\u092f\u093e<\/strong>: \u0924\u092c \u0939\u094b\u0924\u093e \u0939\u0948 \u091c\u092c \u0935\u0943\u0915\u094d\u0915 \u0928\u0932\u093f\u0915\u093e\u090f\u0902 \u092b\u093c\u093f\u0932\u094d\u091f\u0930 \u0915\u093f\u090f \u0917\u090f \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u0915\u094b \u0935\u093e\u092a\u0938 \u0930\u0915\u094d\u0924 \u092e\u0947\u0902 \u0905\u0935\u0936\u094b\u0937\u093f\u0924 \u0915\u0930\u0928\u0947 \u092e\u0947\u0902 \u0905\u0938\u092e\u0930\u094d\u0925 \u0939\u094b\u0924\u0940 \u0939\u0948\u0902, \u091c\u093f\u0938\u0938\u0947 \u092e\u0942\u0924\u094d\u0930 \u092e\u0947\u0902 \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u0915\u0940 \u0909\u091a\u094d\u091a \u0938\u093e\u0902\u0926\u094d\u0930\u0924\u093e \u0939\u094b \u091c\u093e\u0924\u0940 \u0939\u0948\u0964<sup>5<\/sup> \u092f\u0939 \u0935\u0943\u0915\u094d\u0915 \u0928\u0932\u093f\u0915\u093e \u092e\u0947\u0902 \u092a\u0930\u093f\u0935\u0939\u0928 \u092a\u094d\u0930\u094b\u091f\u0940\u0928 \u092e\u0947\u0902 \u0926\u094b\u0937 \u0915\u0947 \u0915\u093e\u0930\u0923 \u0939\u094b \u0938\u0915\u0924\u093e \u0939\u0948, \u091c\u0948\u0938\u093e \u0915\u093f \u0939\u093e\u0930\u094d\u091f\u0928\u092a \u0930\u094b\u0917 \u092e\u0947\u0902 \u0939\u094b\u0924\u093e \u0939\u0948, \u092f\u093e \u0917\u0941\u0930\u094d\u0926\u0947 \u0915\u0940 \u0928\u0932\u093f\u0915\u093e \u092e\u0947\u0902 \u0915\u094d\u0937\u0924\u093f \u0915\u0947 \u0915\u093e\u0930\u0923 \u0939\u094b \u0938\u0915\u0924\u093e \u0939\u0948, \u091c\u0948\u0938\u093e \u0915\u093f \u092b\u0948\u0902\u0915\u094b\u0928\u0940 \u092e\u0947\u0902 \u0939\u094b\u0924\u093e \u0939\u0948\u0964 \u0938\u093f\u0902\u0921\u094d\u0930\u094b\u092e.<sup>4<\/sup><\/p>\n\n <p>\u0935\u0902\u0936\u093e\u0928\u0941\u0917\u0924 \u0905\u092e\u0940\u0928\u094b\u090f\u0938\u093f\u0921\u094d\u092f\u0942\u0930\u093f\u092f\u093e \u0917\u0941\u0930\u094d\u0926\u0947 (\u092a\u0941\u0928\u0930\u094d\u0905\u0935\u0936\u094b\u0937\u0923) \u0914\u0930 \u0915\u0908 \u092e\u093e\u092e\u0932\u094b\u0902 \u092e\u0947\u0902 \u091b\u094b\u091f\u0940 \u0906\u0902\u0924 \u0915\u0947 \u0909\u092a\u0915\u0932\u093e (\u0905\u0935\u0936\u094b\u0937\u0923) \u0915\u0947 \u092e\u093e\u0927\u094d\u092f\u092e \u0938\u0947 \u0926\u094b\u0937\u092a\u0942\u0930\u094d\u0923 \u0905\u092e\u0940\u0928\u094b-\u090f\u0938\u093f\u0921 \u092a\u0930\u093f\u0935\u0939\u0928 \u0915\u0947 \u0915\u093e\u0930\u0923 \u0939\u094b\u0924\u093e \u0939\u0948\u0964<sup>6<\/sup> \u092f\u0947 \u0935\u093f\u0915\u093e\u0930 \u0935\u093f\u0936\u093f\u0937\u094d\u091f \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u092f\u093e \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u0915\u0947 \u0938\u092e\u0942\u0939\u094b\u0902 \u0915\u0947 \u091a\u092f\u093e\u092a\u091a\u092f \u0915\u094b \u092a\u094d\u0930\u092d\u093e\u0935\u093f\u0924 \u0915\u0930 \u0938\u0915\u0924\u0947 \u0939\u0948\u0902, \u091c\u093f\u0938\u0938\u0947 \u0930\u0915\u094d\u0924 \u092e\u0947\u0902 \u0909\u0928\u0915\u093e \u0938\u0902\u091a\u092f \u0939\u094b\u0924\u093e \u0939\u0948 \u0914\u0930 \u092c\u093e\u0926 \u092e\u0947\u0902 \u092e\u0942\u0924\u094d\u0930 \u092e\u0947\u0902 \u0909\u0924\u094d\u0938\u0930\u094d\u091c\u0928 \u0939\u094b\u0924\u093e \u0939\u0948\u0964<sup>7<\/sup><\/p>\n<p>\u090f\u092e\u093f\u0928\u094b \u090f\u0938\u093f\u0921\u0941\u0930\u093f\u092f\u093e \u092e\u0947\u0902 \u0935\u0943\u0915\u094d\u0915 \u0928\u0932\u093f\u0915\u093e\u0913\u0902 \u092e\u0947\u0902 \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u0915\u0947 \u092a\u0941\u0928\u0930\u094d\u0905\u0935\u0936\u094b\u0937\u0923 \u092e\u0947\u0902 \u0926\u094b\u0937 \u0939\u094b\u0924\u093e \u0939\u0948\u0964 \u0938\u093f\u0938\u094d\u091f\u093f\u0928\u0941\u0930\u093f\u092f\u093e \u091c\u0948\u0938\u0940 \u0938\u094d\u0925\u093f\u0924\u093f\u092f\u093e\u0902 \u092e\u0942\u0924\u094d\u0930 \u092a\u0925\u0930\u0940 \u0928\u093f\u0930\u094d\u092e\u093e\u0923 \u0915\u093e \u0915\u093e\u0930\u0923 \u092c\u0928 \u0938\u0915\u0924\u0940 \u0939\u0948\u0902, \u091c\u094b \u092e\u0942\u0924\u094d\u0930 \u0930\u094b\u0917 \u0935\u093f\u091c\u094d\u091e\u093e\u0928 \u092e\u0947\u0902 \u0928\u0948\u0926\u093e\u0928\u093f\u0915 \u0930\u0942\u092a \u0938\u0947 \u092e\u0939\u0924\u094d\u0935\u092a\u0942\u0930\u094d\u0923 \u0939\u0948\u0964<\/p>","gt-synonyms":"\u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 - \u092e\u0942\u0924\u094d\u0930, \u092e\u0942\u0924\u094d\u0930 \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921, \u0939\u093e\u0907\u092a\u0930\u090f\u092e\u093f\u0928\u094b\u090f\u0938\u093f\u0921\u094d\u092f\u0942\u0930\u093f\u092f\u093e, \u090f\u092e\u093f\u0928\u094b\u090f\u0938\u093f\u0921\u094b\u092a\u0948\u0925\u0940, \u0930\u0940\u0928\u0932 \u090f\u092e\u093f\u0928\u094b\u090f\u0938\u093f\u0921\u094d\u092f\u0942\u0930\u093f\u092f\u093e, \u0905\u0924\u093f\u092a\u094d\u0930\u0935\u093e\u0939 \u090f\u092e\u093f\u0928\u094b\u090f\u0938\u093f\u0921\u094d\u092f\u0942\u0930\u093f\u092f\u093e, \u0938\u093e\u092e\u093e\u0928\u094d\u092f\u0940\u0915\u0943\u0924 \u0905\u092e\u0940\u0928\u094b\u090f\u0938\u093f\u0921\u094d\u092f\u0942\u0930\u093f\u092f\u093e","gt-citation":"[1] Aliu E, Kanungo S, Arnold GL. Amino acid disorders. Ann Transl Med. 2018 Dec;6(24):471. DOI: 10.21037\/atm.2018.12.12\n\n[2] \"Aminoaciduria\". Mosby's Medical Dictionary (9th ed.). Mosby. 2013. p. 79. ISBN 9780323112581.\n\n[3] Sch\u00fcck, O (1984). \"Amino acids excretion. Physiology and pathophysiology\". Examination of Kidney Function. Translated by Cort, JH. Dordrecht: Springer Netherlands. p. 252. ISBN 9789400956605.\n\n[4] Crook, Martin Andrew (2012). \"Chapter 27: Inborn errors of metabolism\". Clinical biochemistry and metabolic medicine (8th ed.). London: Hodder Arnold. pp. 371\u2013383. ISBN 9781444144154.\n\n[5] Mundt, LA; Shanahan, K (2011). \"Chapter 7: Urinary and metabolic diseases and related urinalysis findings. Aminoacidurias\". Graff's textbook of routine urinalysis and body fluids (2nd ed.). Philadelphia: Wolters Kluwer\/Lippincott Williams & Wilkins Health. pp. 203\u2013210. ISBN 9781582558752.\n\n[6] \"Aminoaciduria\". ScienceDirect Topics. Retrieved from Kidney International, 2015. https:\/\/www.sciencedirect.com\/topics\/medicine-and-dentistry\/aminoaciduria\n\n[7] Ziegler SG, Ferreira CR. Inborn errors of amino acid metabolism. Transl Sci Rare Dis. 2023;6(1-2):37-55. DOI: 10.3233\/TRD-230018","gt-clinical-context":"<p>\u0905\u092e\u0940\u0928\u094b-\u090f\u0938\u093f\u0921\u094d\u092f\u0942\u0930\u093f\u092f\u093e \u091a\u093f\u0915\u093f\u0924\u094d\u0938\u0915\u0940\u092f \u0930\u0942\u092a \u0938\u0947 \u092e\u0939\u0924\u094d\u0935\u092a\u0942\u0930\u094d\u0923 \u0939\u0948 \u0915\u094d\u092f\u094b\u0902\u0915\u093f \u092f\u0939 \u0905\u0915\u094d\u0938\u0930 \u0935\u093f\u092d\u093f\u0928\u094d\u0928 \u091a\u092f\u093e\u092a\u091a\u092f \u0935\u093f\u0915\u093e\u0930\u094b\u0902 \u0915\u0947 \u0932\u093f\u090f \u092c\u093e\u092f\u094b\u092e\u093e\u0930\u094d\u0915\u0930 \u0915\u0947 \u0930\u0942\u092a \u092e\u0947\u0902 \u0915\u093e\u0930\u094d\u092f \u0915\u0930\u0924\u093e \u0939\u0948, \u0935\u093f\u0936\u0947\u0937 \u0930\u0942\u092a \u0938\u0947 \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u091a\u092f\u093e\u092a\u091a\u092f \u0915\u0940 \u091c\u0928\u094d\u092e\u091c\u093e\u0924 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\u0935\u093f\u0915\u093e\u0938 \u092e\u0947\u0902 \u0935\u093f\u092b\u0932\u0924\u093e, \u092f\u093e \u091a\u092f\u093e\u092a\u091a\u092f \u0938\u0902\u092c\u0902\u0927\u0940 \u0935\u093f\u0915\u093e\u0930\u094b\u0902 \u0915\u093e \u092a\u093e\u0930\u093f\u0935\u093e\u0930\u093f\u0915 \u0907\u0924\u093f\u0939\u093e\u0938 \u0936\u093e\u092e\u093f\u0932 \u0939\u0948\u0964<sup>5<\/sup><\/p>\n\n <p>3. <strong>\u0928\u0948\u0926\u093e\u0928\u093f\u0915 \u200b\u200b\u092a\u094d\u0930\u092c\u0902\u0927\u0928<\/strong>: \u0915\u0908 \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u0935\u093f\u0915\u093e\u0930\u094b\u0902 \u0915\u0947 \u0932\u093f\u090f, \u0939\u093e\u0928\u093f\u0915\u093e\u0930\u0915 \u0905\u092e\u0940\u0928\u094b \u090f\u0938\u093f\u0921 \u0915\u093e \u0906\u0939\u093e\u0930 \u092a\u094d\u0930\u0924\u093f\u092c\u0902\u0927 \u0909\u092a\u091a\u093e\u0930 \u0915\u093e \u092e\u0941\u0916\u094d\u092f \u0906\u0927\u093e\u0930 \u0939\u0948\u0964<sup>1<\/sup> \u0907\u0938 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\u0938\u0915\u0924\u093e \u0939\u0948\u0964<sup>2<\/sup><\/p>\n<p>\u092e\u0942\u0924\u094d\u0930 \u0930\u094b\u0917 \u0935\u093f\u091c\u094d\u091e\u093e\u0928 \u092e\u0947\u0902, \u0938\u093f\u0938\u094d\u091f\u093f\u0928\u0941\u0930\u093f\u092f\u093e \u0938\u092c\u0938\u0947 \u0906\u092e \u090f\u092e\u093f\u0928\u094b \u090f\u0938\u093f\u0921\u0941\u0930\u093f\u092f\u093e \u0939\u0948 \u091c\u094b \u092c\u093e\u0930-\u092c\u093e\u0930 \u092e\u0942\u0924\u094d\u0930 \u092a\u0925\u0930\u0940 \u0915\u093e \u0915\u093e\u0930\u0923 \u092c\u0928\u0924\u0940 \u0939\u0948\u0964 EAU \u092a\u0925\u0930\u0940 \u0926\u093f\u0936\u093e\u0928\u093f\u0930\u094d\u0926\u0947\u0936\u094b\u0902 \u0915\u0947 \u0905\u0928\u0941\u0938\u093e\u0930, 24-\u0918\u0902\u091f\u0947 \u092e\u0942\u0924\u094d\u0930 \u0935\u093f\u0936\u094d\u0932\u0947\u0937\u0923 \u0914\u0930 \u0906\u0939\u093e\u0930 \u092a\u094d\u0930\u092c\u0902\u0927\u0928 \u0906\u0935\u0936\u094d\u092f\u0915 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