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TH converts tyrosine into L\u2010DOPA, which is the direct precursor of catecholamine biosynthesis. TH deficiency causes a neurological disease with primary extrapyramidal signs and a variable response to L\u2010DOPA. We report three patients in China who were diagnosed with Tyrosine hydroxylase (TH) deficiency by genetic testing and clinical manifestations. After L\u2010DOPA treatment, their condition had sustained improvement.<\/jats:p>","DOI":"10.1016\/j.ijdevneu.2019.08.002","type":"journal-article","created":{"date-parts":[[2019,8,13]],"date-time":"2019-08-13T15:32:12Z","timestamp":1565710332000},"page":"28-32","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Levodopa is effective in the treatment of three Chinese Tyrosine hydroxylase (TH) deficiency children"],"prefix":"10.1002","volume":"78","author":[{"given":"Mei","family":"Hou","sequence":"first","affiliation":[{"name":"Pediatric Department of the Qingdao Women &amp; Children Hospital No. 6 Tongfu Road Shandong 266000 PR China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chengqing","family":"Yang","sequence":"additional","affiliation":[{"name":"Pediatric Department of the Affiliated Hospital of Qingdao University No. 16 Jiangsu Road Shandong 266000 PR China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jingfei","family":"Hu","sequence":"additional","affiliation":[{"name":"Pediatric Department of the Qingdao Women &amp; Children Hospital No. 6 Tongfu Road Shandong 266000 PR China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ya","family":"Guo","sequence":"additional","affiliation":[{"name":"Pediatric Department of the Affiliated Hospital of Qingdao University No. 16 Jiangsu Road Shandong 266000 PR China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peipei","family":"Liu","sequence":"additional","affiliation":[{"name":"Pediatric Department of the Affiliated Hospital of Qingdao University No. 16 Jiangsu Road Shandong 266000 PR China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yedan","family":"Liu","sequence":"additional","affiliation":[{"name":"Pediatric Department of the Affiliated Hospital of Qingdao University No. 16 Jiangsu Road Shandong 266000 PR China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jie","family":"Song","sequence":"additional","affiliation":[{"name":"Pediatric Department of the Affiliated Hospital of Qingdao University No. 16 Jiangsu Road Shandong 266000 PR China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Wei","sequence":"additional","affiliation":[{"name":"Kangso Medical Inspection Co., Ltd No. 65 Haidian District, Xingshikou Road Yiyuan Cultural Creative Industry Base C District No. 10, Floor 2, 201\u2010203 Beijing 100195 PR China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zongbo","family":"Chen","sequence":"additional","affiliation":[{"name":"Pediatric Department of the Affiliated Hospital of Qingdao University No. 16 Jiangsu Road Shandong 266000 PR China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,8,13]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13312-017-1055-9"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.10095"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004390050756"},{"issue":"6","key":"e_1_2_8_5_1","doi-asserted-by":"crossref","first-page":"S56","DOI":"10.1002\/ana.10632","article-title":"Tyrosine hydroxylase deficiency causes progressive encephalopathy and dopa\u2010nonresponsive dystonia","volume":"54","author":"Hoffmann G.F.","year":"2003","journal-title":"Ann. 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