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- W4385294598 abstract "Given the lack of data to help caregivers in the follow-up of Hirschsprung disease (HD), this study aimed to compare the functional outcomes of isolated Hirschsprung disease (I-HD) to syndrome-associated Hirschsprung disease (SA-HD) at 1, 3, 5, and 10 years.A retrospective chart review of patients diagnosed with HD between January 1990 and May 2021 at our pediatric center was performed to collect data on patient characteristics, investigations, and treatments. Ninety-five patients were identified, of whom 76 were included in the study. SA-HD is defined as a syndrome known to be associated with HD or cognitive impairment.Patient characteristics were comparable between groups (P > 0.05). There were 52 patients with I-HD and 24 with SA-HD. The patients median age was 9 days at diagnosis and 1.5 month at surgery. SA-HD patients became bowel continent at a significantly older age (mean age 8.43y vs 4.94 years, p=0.0471) and received more bowel continence medications. At 5y, SA-HD patients requiring ≥ 2 medications for bowel continence represented 54.5% vs. 11.1% of I-HD patients (p=0.009). Lastly, SA-HD patients had urinary incontinence at a significantly older age (p=0.0136, 5y).Clinicians should be aware that SA-HD patients are more prone to bladder dysfunction and became bowel continent at an older age than I-HD patients. They need more and prolonged bowel management medications, and other important complications need to be addressed in patient care. These results should prompt a longer follow-up period for these patients, especially in SA-HD." @default.
- W4385294598 created "2023-07-28" @default.
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- W4385294598 date "2023-07-27" @default.
- W4385294598 modified "2023-09-25" @default.
- W4385294598 title "Short and long-term outcomes in Hirschsprung disease: Are the syndrome-associated patients really doing worse?" @default.
- W4385294598 doi "https://doi.org/10.1097/mpg.0000000000003896" @default.
- W4385294598 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/37496148" @default.
- W4385294598 hasPublicationYear "2023" @default.
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