Hirschsprung disease in the premature newborn: A population based study and 40-year single center experience

Downey, Earl C.; Hughes, Elizabeth M.; Putnam, Angelica R.; Baskin, H. Jack; Rollins, Michael D.

doi:10.1016/j.jpedsurg.2014.10.013

Cited by 23 publications

(18 citation statements)

References 15 publications

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“…Downey et al studied preterm patients with HSCR and concluded that they had more associated anomalies than term patients with HSCR. 28 The rate of associated malformations reported in this study (34.5%) is fairly high compared with other studies. This could be explained by our broad definition of malformation: at least 1 diagnosis in any of the registers, which could cause an overrepresentation of the patients with associated malformations.…”

Section: Figurecontrasting

confidence: 42%

Maternal Risk Factors and Perinatal Characteristics for Hirschsprung Disease

Granström¹,

Svenningsson²,

Hagel

et al. 2016

Pediatrics

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Section: Figurecontrasting

confidence: 42%

Maternal Risk Factors and Perinatal Characteristics for Hirschsprung Disease

Granström¹,

Svenningsson²,

Hagel

et al. 2016

Pediatrics

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“…In this study, a quarter of infants with HD passed meconium within 24 hours of birth and nearly half of infants within 48 hours of birth. Two recent studies1 13 also reported that around 40% of infants with HD, including preterm infants,13 passed meconium within 48 hours of birth. Current National Institute for Health and Care Excellence (NICE) guidance on ‘Constipation in Children and Young People’ incorporates ‘delayed passage of meconium beyond 48 hours in term infants’ as a red flag for urgent specialist referral to exclude HD 39.…”

Section: Discussionmentioning

confidence: 94%

“…around 40% of infants with HD, including preterm infants, 13 passed meconium within 48 hours of birth. Current National Institute for Health and Care Excellence (NICE) guidance on 'Constipation in Children and Young People' incorporates 'delayed passage of meconium beyond 48 hours in term infants' as a red flag for urgent specialist referral to exclude HD.…”

Section: Discussionmentioning

confidence: 99%

“…Some infants fail to decompress adequately with rectal washouts and require a stoma to achieve satisfactory colonic decompression, and some are deemed unsuitable from the outset. The majority of previous studies examining the early management of HD comprise retrospective case series,13 14 voluntary reporting surveys with variable methods of case ascertainment,1 7 15 surveys of intended practice11 16 17 or meta-analyses and systematic reviews of retrospective case series 18–20. To date, there are very few prospective, population-based observational studies of HD anywhere in the world1 7 and none that provide representative data in a cohort of children born during a short time period.…”

Section: Introductionmentioning

confidence: 99%

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Hirschsprung's disease in the UK and Ireland: incidence and anomalies

et al. 2017

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ObjectivesTo describe clinical characteristics and preoperative management of a national cohort of infants with Hirschsprung's disease (HD).DesignPopulation-based cohort study of all live-born infants with HD born in the UK and Ireland from October 2010 to September 2012.SettingAll 28 paediatric surgical centres in the UK and Ireland.Participants305 infants presenting before 6 months of age with histologically proven HD.Main outcome measuresIncidence, clinical characteristics including gestational age, birth weight, gender, associated anomalies; age and clinical features at presentation; and use of rectal washouts or stoma.ResultsThe incidence of HD in the UK and Ireland was 1.8 per 10 000 live births (95% CI 1.5 to 1.9). Male to female ratio was 3.3:1. An associated anomaly was identified in 23% (69), with 15% (47) having a recognisable syndrome. The proportion of infants who presented and were diagnosed in the neonatal period was 91.5% (279) and 83.9% (256), respectively. 23.9% (73) and 44.2% (135) passed meconium within 24 and 48 hours of birth. 81% (246) first presented to a hospital without tertiary paediatric surgical services, necessitating interhospital transfer. Initial colonic decompression was by rectal washouts in 86.2% (263) and by defunctioning stoma in 12.8% (39). Subsequently, 27.4% (72) of infants failed management with rectal washouts and required a delayed stoma, resulting in 36.4% (111) of infants having a stoma.ConclusionsIn this population-based cohort, presentation outside the neonatal period was rare. Nearly half of the infants with HD passed meconium within 48 hours of birth and over one third were managed with a stoma.

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“…While previously thought to be rare in premature infants, recent analyses indicate that the incidence of HSCR is similar for pre-term and term births[5–7]. The incidence of HAEC reported in the literature is highly variable, with reports as high as 60%, although an incidence of 25–35% is generally quoted in current series[8, 9].…”

Section: Established Conceptsmentioning

confidence: 99%

Established and emerging concepts in Hirschsprung’s-associated enterocolitis

Gosain

2016

Pediatr Surg Int

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Hirschsprung’s disease (HSCR) is a common cause of neonatal bowel obstruction and the approach to diagnosis and surgical treatment is well defined and accepted. Hirschsprung’s-associated enterocolitis (HAEC) remains a frequent cause of pre-operative and post-operative morbidity and mortality, with unchanged treatment guidelines over multiple decades. Recent advances in our understanding of the genetics underlying HSCR have allowed the development of animal models, some of which recapitulate the HAEC phenotype. These animal models, along with recent translational studies, have implicated multiple facets of mucosal immunity and microbiome dysbiosis in the development of HAEC. Here, we will review the established epidemiology, modes of diagnosis and treatment of HAEC. Furthermore, we will explore emerging concepts in the pathogenesis of this disease; including animal models, alterations in mucosal immunity, dysbiosis of the intestinal microbiome, specific genetic susceptibility, and novel treatment modalities.

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Hirschsprung disease in the premature newborn: A population based study and 40-year single center experience

Cited by 23 publications

References 15 publications

Maternal Risk Factors and Perinatal Characteristics for Hirschsprung Disease

Maternal Risk Factors and Perinatal Characteristics for Hirschsprung Disease

Hirschsprung's disease in the UK and Ireland: incidence and anomalies

Established and emerging concepts in Hirschsprung’s-associated enterocolitis

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