Epidemiology of Hirschsprung disease in California from 1995 to 2013

Jamie Anderson, Melissa A. Vanover, Payam Saadai, Rebecca Stark, Jacob T. Stephenson, Shinjiro Hirose

Research output: Contribution to journalArticle

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%.

Original languageEnglish (US)
Pages (from-to)1299-1303
Number of pages5
JournalPediatric Surgery International
Volume34
Issue number12
DOIs
StatePublished - Dec 1 2018

Fingerprint

Hirschsprung Disease
Epidemiology
Parturition
African Americans
Incidence
Child Mortality
Health Planning
Mortality
Patient Discharge
Birth Rate
Live Birth
International Classification of Diseases
Down Syndrome
Gestational Age
Databases
Population

Keywords

  • California
  • Epidemiology
  • Hirschsprung disease
  • Incidence

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

Cite this

Epidemiology of Hirschsprung disease in California from 1995 to 2013. / Anderson, Jamie; Vanover, Melissa A.; Saadai, Payam; Stark, Rebecca; Stephenson, Jacob T.; Hirose, Shinjiro.

In: Pediatric Surgery International, Vol. 34, No. 12, 01.12.2018, p. 1299-1303.

Research output: Contribution to journalArticle

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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{\%}.",
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