disease has basis in dysfunction of disease has feature HPRT1 Self-injurious behavior Lesch-Nyhan syndrome DOID:1919 GARD:0007226 HG-PRT deficiency HPRT complete deficiency HPRT deficiency HPRT deficiency grade IV HPRT deficiency, complete HPRT deficiency, neurologic variant Hprt1 deficiency ICD10:E79.1 ICD9:277.2 LNS Lesch - Nyhan syndrome Lesch Nyhan disease Lesch Nyhan syndrome Lesch-Nyhan syndrome Lesch-Nyhan syndrome (LNS) is the most severe form of hypoxanthine-guanine phosphoribosyltransferase (HPRT) deficiency, a hereditary disorder of purine metabolism, and is associated with uric acid overproduction (UAO), neurological troubles, and behavioral problems. Lesch-Nyhan syndrome, neurologic variant MESH:D007926 MONDO:0010298 MedDRA:10057589 NCIT:C61255 OMIM:300322 Orphanet:510 SCTID:10406007 UMLS:C0023374 UMLS:CN205196 X-linked hyperuricemia X-linked hyperuricemia (disorder) [ambiguous] complete hypoxanthine-guanine phosphoribosyltransferase deficiency deficiency of IMP pyrophosphorylase hypoxanthine guanine phospho-ribosyltransferase 1 deficiency hypoxanthine guanine phosphoribosyltransferase 1 deficiency hypoxanthine guanine phosphoribosyltransferase complete deficiency hypoxanthine guanine phosphoribosyltransferase deficiency hypoxanthine guanine phosphoribosyltransferase deficiency, grade IV hypoxanthine-guanine phosphoribosyltransferase deficiency hypoxanthine-guanine phosphoribosyltransferase deficiency (disorder) [ambiguous] hypoxanthine-guanine-phosphoribosyltransferase deficiency hypoxanthine-guanine-phosphoribosyltransferase deficiency (& [Lesch - Nyhan syndrome]) hypoxanthine-guanine phosphoribosyltransferase deficiency