Jamie E Anderson1, Melissa A Vanover2, Payam Saadai2, Rebecca A Stark2, Jacob T Stephenson2, Shinjiro Hirose2. 1. Division of Pediatric General, Thoracic, and Fetal Surgery, University of California, Davis Medical Center, 2215 Stockton Boulevard, OP512, Sacramento, CA, 95817, USA. jeanderson@ucdavis.edu. 2. Division of Pediatric General, Thoracic, and Fetal Surgery, University of California, Davis Medical Center, 2215 Stockton Boulevard, OP512, Sacramento, CA, 95817, USA.
Abstract
PURPOSE: This study seeks to update current epidemiology of Hirschsprung disease (HD) in California. METHODS: Using data from the California Office of Statewide Health Planning and Development Linked Birth (1995-2012) and Patient Discharge Databases (1995-2013), patients from either dataset with an ICD-9 diagnosis code of HD (751.3) or procedure code of Soave (48.41), Duhamel (48.65), or Swenson/other pull-through (48.49) were included. Patients > age 18 during their first admission were excluded. RESULTS: Of 9.3 million births, 2,464 patients were identified. Incidence was 2.2 cases/10,000 live births, with rates peaking at 2.9/10,000 births in 2002. Incidence was highest among African American (4.1/10,000) and Asian/Pacific Islander (2.5/10,000) births. Most were male (n = 1652, 67.1%). Sixty patients (2.4%) had Down syndrome. The median gestational age at birth was 38 weeks 6 days (interquartile range [IQR] 37 weeks 1 day-40 weeks 1 day). Mortality during the first year of life was 1.7%. Median age at death was 14.5 days (IQR 0-113 days). CONCLUSION: This is one of the largest population-based studies of HD. In California, the incidence of HD is stable, risk is highest among African American children, and the mortality rate is < 2%.
PURPOSE: This study seeks to update current epidemiology of Hirschsprung disease (HD) in California. METHODS: Using data from the California Office of Statewide Health Planning and Development Linked Birth (1995-2012) and Patient Discharge Databases (1995-2013), patients from either dataset with an ICD-9 diagnosis code of HD (751.3) or procedure code of Soave (48.41), Duhamel (48.65), or Swenson/other pull-through (48.49) were included. Patients > age 18 during their first admission were excluded. RESULTS: Of 9.3 million births, 2,464 patients were identified. Incidence was 2.2 cases/10,000 live births, with rates peaking at 2.9/10,000 births in 2002. Incidence was highest among African American (4.1/10,000) and Asian/Pacific Islander (2.5/10,000) births. Most were male (n = 1652, 67.1%). Sixty patients (2.4%) had Down syndrome. The median gestational age at birth was 38 weeks 6 days (interquartile range [IQR] 37 weeks 1 day-40 weeks 1 day). Mortality during the first year of life was 1.7%. Median age at death was 14.5 days (IQR 0-113 days). CONCLUSION: This is one of the largest population-based studies of HD. In California, the incidence of HD is stable, risk is highest among African American children, and the mortality rate is < 2%.
Authors: Earl C Downey; Elizabeth Hughes; Angelica R Putnam; Henry J Baskin; Michael D Rollins Journal: J Pediatr Surg Date: 2014-10-30 Impact factor: 2.545
Authors: Monica K Lieng; James P Marcin; Parul Dayal; Daniel J Tancredi; Morgan B Swanson; Sarah C Haynes; Patrick S Romano; Ilana S Sigal; Jennifer L Rosenthal Journal: J Pediatr Date: 2021-05-14 Impact factor: 4.406