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Hirschsprung's disease: associated abnormalities and demography
E T Ryan1, J L Ecker, N A Christakis
1Children's Hospital Medical Center, Boston, MA 02115.
Insights
Children with Hirschsprung disease are less likely to be firstborn. Hirschsprung disease is associated with other abnormalities, but not linked to advanced maternal age.
Area of Science:
- Pediatric Gastroenterology
- Medical Genetics
- Developmental Biology
Background:
- Hirschsprung disease is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
- Understanding the demographic and associated factors of Hirschsprung disease is crucial for diagnosis and management.
Purpose of the Study:
- To analyze the demographic parameters of patients diagnosed with Hirschsprung disease.
- To identify associated conditions and risk factors in a pediatric population.
Main Methods:
- Retrospective review of 179 patients with histologically confirmed Hirschsprung disease.
- Data collected from 1961 to 1986 at Children's Hospital Medical Center of Boston.
- Statistical analysis to determine demographic associations and co-occurring conditions.
Main Results:
- Patients with Hirschsprung disease were significantly less likely to be firstborn (P < .01).
- 22% of affected children had associated abnormalities (neurological, cardiovascular, urological, gastrointestinal).
- No association found between Hirschsprung disease and increased maternal age; 7% were born prematurely.
Conclusions:
- Birth order may be a relevant demographic factor in Hirschsprung disease.
- Associated anomalies suggest a potential link to neurocristopathies.
- Further research into the etiology and developmental origins of Hirschsprung disease is warranted.
Abstract:
We here examine the demographic parameters of patients with Hirschsprung's disease. The study population includes all patients with histologically confirmed disease treated at the Children's Hospital Medical Center of Boston over the 25-year period extending from 1961 through the first quarter of 1986. There were 179 cases. Overall, children with Hirschsprung's disease were found less likely to be first born (P less than .01). This relationship was seen to persist irrespective of maternal age, maternal race, or type of disease. The implications of this finding are discussed. Overall, 22% of these children had one or more associated abnormalities involving the neurological, cardiovascular, urological, and gastrointestinal systems. Many of the disorders appeared to be related to neurocrestopathies. Frequent associations included Down's syndrome, defects in cardiac septation, tetralogy of Fallot, and Dandy-Walker syndrome. These conditions occurred more frequently than would have been predicted through chance alone. This study found no association between an increased maternal age and the occurrence of Hirschsprung's disease. This study also found that approximately 7% of the affected children had been born prematurely.