Growth hormone studies in lysinuric protein intolerance.
Our two patients with lysinuric protein intolerance, a 14-year-old boy and his 12-year-old sister, showed growth retardation and their bone ages were retarded. Growth hormone secretion responded to glucagon-propranolol and showed a good response to arginine. However, growth hormone showed little response to insulin. After the oral administration of arginine hydrochloride, growth hormone showed a good response to insulin and glucagon-propranolol, and gain in height and weight accelerated. This result may suggest that an adequate supply of arginine is effective in improving the growth retardation in lysinuric protein intolerance.
D000293:Adolescent; D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications* / Q000188:drug therapy / Q000378:metabolism; D000599:Amino Acids, Diamino / Q000652:urine; D001120:Arginine / Q000494:pharmacology / Q000627:therapeutic use; D002648:Child; D004044:Dietary Proteins / Q000378:metabolism; D005260:Female; D006130:Growth Disorders / Q000150:complications* / Q000188:drug therapy / Q000378:metabolism; D013006:Growth Hormone / Q000378:metabolism*; D006801:Humans; D008239:Lysine / Q000652:urine; D008297:Male
D000599:Amino Acids, Diamino; D004044:Dietary Proteins; D013006:Growth Hormone; D001120:Arginine; D008239:Lysine