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ABSTRACT Objective: To determine the incidence of congenital hypothyroidism (CH) over a 10-year period at the Reference Service in Neonatal Screening of the state of Rio Grande do Sul (RSNS-RS). Subjects and methods: Historical cohort study including all newborns screened for CH by the RSNS-RS from January 2008 until December 2017. Data of all newborns with neonatal TSH (neoTSH; heel prick test) values ≥ 9 mIU/L were collected. According to neoTSH values, the newborns were allocated into two groups: Group 1 (G1), comprising newborns with neoTSH ≥ 9 mIU/L and serum TSH (sTSH) < 10 mIU/L, and Group 2 (G2), comprising those with neoTSH ≥ 9 mIU/L and sTSH ≥ 10 mIU/L. Results: Of 1,043,565 newborns screened, 829 (0.08%) had neoTSH values ≥ 9 mIU/L. Of these, 284 (39.3%) had sTSH values < 10 mIU/L and were allocated to the G1 group, while 439 (60.7%) had sTSH ≥ 10 mIU/L and were allocated to the G2 group, and 106 (12.7%) were considered missing data. The overall incidence of CH was 42.1 per 100,000 newborns screened (95% confidence interval [CI] 38.5-45.7/100,000) or 1:2377 screened newborns. The sensibility and specificity of neoTSH ≥ 9 mIU/L were 97% and 11%; of neoTSH 12.6 mUI/L, 73% and 85% respectively. Conclusion: In this population, the incidence of permanent and transitory CH was 1:2377 screened newborns. The neoTSH cutoff value adopted during the study period showed excellent sensibility, which matters for a screening test. Objective (CH 10year year RSNSRS. RSNSRS RSNS RS . (RSNS-RS) methods 200 2017 (neoTSH test mIUL mIU L collected groups G1, G , (G1) (sTSH G2, (G2) Results 1043565 043 565 1,043,56 82 0.08% 008 0 08 (0.08% these 28 39.3% 393 39 3 (39.3% group 43 60.7% 607 60 7 (60.7% 12.7% 127 12 (12.7% data 421 42 42. 100000 100 000 100,00 95% 95 (95 CI [CI 38.545.7/100,000 385457100000 38.5 45.7/100,000 38 5 45 38.5-45.7/100,000 12377 2377 1:237 97 11% 11 126 6 12. mUIL mUI mUI/L 73 85 respectively Conclusion population (RSNS-RS 20 201 (G1 (G2 104356 04 56 1,043,5 8 0.08 00 (0.08 39.3 (39.3 4 60.7 (60.7 12.7 (12.7 10000 100,0 (9 545 38.545.7/100,00 38545710000 385 38. 457100000 45.7/100,00 38.5-45.7/100,00 1237 237 1:23 (G 10435 1,043, 0.0 (0.0 39. (39. 60. (60. (12. 1000 100, ( 54 38.545.7/100,0 3854571000 45710000 45.7/100,0 38.5-45.7/100,0 123 23 1:2 1043 1,043 0. (0. (39 (60 (12 38.545.7/100, 385457100 4571000 45.7/100, 38.5-45.7/100, 1: 104 1,04 (0 (3 (6 (1 38.545.7/100 38545710 457100 45.7/100 38.5-45.7/100 1,0 38.545.7/10 3854571 45710 45.7/10 38.5-45.7/10 1, 38.545.7/1 385457 4571 45.7/1 38.5-45.7/1 38.545.7/ 38545 457 45.7/ 38.5-45.7/ 38.545.7 3854 45.7 38.5-45.7 38.545. 45. 38.5-45. 38.545 38.5-45 38.54 38.5-4 38.5-