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Published on: November 30, 2010
The epidemiology of infantile hypertrophic pyloric stenosis
R Schechter1, C P Torfs, T F Bateson
1California Department of Health Services, Berkeley 94704, USA.
Insights
Infantile hypertrophic pyloric stenosis (IHPS) is most common in White and Hispanic infants. Environmental factors likely play a role, and a link with Smith-Lemli-Opitz syndrome warrants further investigation.
Area of Science:
- Pediatric Surgery
- Medical Genetics
- Epidemiology
Background:
- Infantile hypertrophic pyloric stenosis (IHPS) is a congenital anomaly affecting the gastric outlet.
- Understanding the epidemiology and associated conditions of IHPS is crucial for diagnosis and management.
Purpose of the Study:
- To analyze the demographic characteristics and associated birth defects in infants with IHPS.
- To investigate the incidence, timing of diagnosis, and potential etiological factors of IHPS.
Main Methods:
- Retrospective analysis of IHPS cases from the California Birth Defects Monitoring Program (CBDMP) database (1983-1988).
- Comparison of IHPS cases with the general birth cohort regarding demographics and co-occurring birth defects.
- Calculation of cumulative incidence rates across different racial/ethnic groups, maternal ages, and birth ranks.
Main Results:
- A cumulative incidence of 1.9 per 1000 livebirths for IHPS was observed.
- Higher incidence rates were noted in White (2.4) and Hispanic (1.8) infants compared to Black (0.7) and Asian (0.6) infants.
- Diagnosis typically occurred between 3-12 weeks postpartum, with delayed diagnosis in premature infants. Concordance in monozygous twins was low (0.25-0.44), suggesting environmental influences. A 157-fold increased incidence of Smith-Lemli-Opitz syndrome (SLO) was found in IHPS infants.
Conclusions:
- IHPS affects diverse racial and ethnic groups, with higher prevalence in White and Hispanic populations.
- The low concordance in monozygous twins highlights the significant role of environmental factors in IHPS etiology.
- The strong association between IHPS and SLO necessitates further research and vigilant monitoring of infants with suspected SLO for pyloric stenosis.
Abstract:
Infants with infantile hypertrophic pyloric stenosis (IHPS) born from 1983 to 1988 and recorded in the California Birth Defects Monitoring Program (CBDMP) database were compared with their birth cohort by demographic characteristics and selected associated birth defects. We identified 1963 cases of IHPS for a cumulative incidence of 1.9 per 1000 livebirths. The cumulative incidence per 1000 livebirths was 2.4 in White, 1.8 in Hispanic, 0.7 in Black, and 0.6 in Asian infants. Between weeks 3-12 after birth, 1871 (95%) IHPS cases were diagnosed. Premature infants were diagnosed with IHPS later than term or post-term infants. The incidence of IHPS declined for those born to maternal age groups of > or = 25 years and, independently, for successive birth ranks. The probandwise concordance rate for IHPS in monozygous twins was less than unity (0.25-0.44), although higher than the concordance for dizygous twins (0.05-0.10). The incidence of Smith-Lemli-Opitz syndrome (SLO) diagnosed in infants with IHPS (3 of 1963) was 157-fold higher than the incidence of SLO diagnosed in the CBDMP population. IHPS occurs in all of the largest racial and ethnic groups in California, most frequently in White and Hispanic infants. Pyloric stenosis presents only within a brief phase of development, which may be delayed in premature infants. A predominant discordance of disease state in monozygous twins implies an aetiological role for undetermined environmental factors. The association between SLO, caused by deficient cholesterol synthesis, and IHPS deserves additional study. Infants with suspected SLO require close observation for the onset of IHPS.
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