A Practical Guide for the Diagnosis of Primary Enteric Nervous System Disorders

Schäppi, Michela; Staiano, Annamaria; Milla, Peter J.; Smith, Virpi V.; Dias, Jcp; Heuschkel, Robert; Husby, Steffen; Mearin, M. Luisa; Papadopoulou, Anthie; Ruemmele, Frank M; Vandenplas, Yvan; Koletzko, S.

doi:10.1097/mpg.0b013e3182a8bb50

Cited by 81 publications

(78 citation statements)

References 76 publications

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“…Some infants may not have had prenatal screening and serious congenital anomalies that would have excluded them from the study may not be diagnosed until days or weeks after randomization. Even in cases of excellent prenatal care, some conditions (e.g., Hirschsprung’s disease) cannot be diagnosed until the postnatal period [30, 31]. The best way to handle this will be to follow all randomized participants to the primary endpoint of full enteral feeds and analyze the data on the basis of intention-to-treat.…”

Section: Methodsmentioning

confidence: 99%

Glycerin suppositories used prophylactically in premature infants (SUPP) trial: a study protocol for a pilot randomized controlled trial

Livingston

Zequeira

Blinder

et al. 2015

Pilot Feasibility Stud

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BackgroundFeeding is a significant challenge for premature infants in the neonatal intensive care unit (NICU). These patients are often treated with glycerin suppositories to stimulate the passage of meconium and prevent feeding intolerance. Unfortunately, the evidence for this practice is inconclusive.Methods/designThis protocol is for an external pilot study that will assess the feasibility of a superiority, placebo-controlled, parallel-design, multicenter randomized controlled trial. Participants are premature infants treated in a level 3 NICU with a gestational age 24 to 32 weeks and/or birth weight of 500 to 1500 g. Thirty participants will be recruited as part of this external pilot study. Participants will be randomized to glycerin suppository (250 mg) or placebo starting 48 to 72 h after birth and continuing once daily until meconium evacuation is complete or for a maximum of 12 days. The placebo consists of a 250-mg glycerin suppository placed in the diaper rather than the rectum. Study treatments are administered by the charge nurse on duty who is not otherwise involved in patient care. All other clinicians and research personnel will remain blinded. Outcomes for the pilot study are percentage of eligible participants randomized, percentage of infants reaching full enteral feeds, cost, and treatment-related adverse events (rectal bleeding, rectal perforation, and anal fissure).DiscussionThis external pilot study will assess the feasibility of a multicenter randomized controlled trial of glycerin suppositories in premature infants. The subsequent multicenter trial will have sufficient power to determine whether this treatment strategy is associated with decreased time to full enteral feeds.Trial registrationClinicalTrials.gov: NCT02153606

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Section: Methodsmentioning

confidence: 99%

Glycerin suppositories used prophylactically in premature infants (SUPP) trial: a study protocol for a pilot randomized controlled trial

Livingston

Zequeira

Blinder

et al. 2015

Pilot Feasibility Stud

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“…These disorders include achalasia (Castell, 2013), gastroparesis (Camilleri et al, 2013), chronic intestinal pseudoobstruction syndrome (CIPO) (Di Lorenzo and Youssef, 2010; Schappi et al, 2013), slow transit constipation, and irritable bowel syndrome (IBS) (Camilleri, 2013; Heuckeroth, 2014; Knowles et al, 2010; Panza et al, 2012; Wood, 2013) (Table 2). …”

Section: Non-hscr Motility Disordersmentioning

confidence: 99%

Gene-environment interactions and the enteric nervous system: Neural plasticity and Hirschsprung disease prevention

Heuckeroth

Schäfer

2016

Developmental Biology

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Intestinal function is primarily controlled by an intrinsic nervous system of the bowel called the enteric nervous system (ENS). The cells of the ENS are neural crest derivatives that migrate into and through the bowel during early stages of organogenesis before differentiating into a wide variety of neurons and glia. Although genetic factors critically underlie ENS development, it is now clear that many non-genetic factors may influence the number of enteric neurons, types of enteric neurons, and ratio of neurons to glia. These non-genetic influences include dietary nutrients and medicines that may impact ENS structure and function before or after birth. This review summarizes current data about gene-environment interactions that affect ENS development and suggests that these factors may contribute to human intestinal motility disorders like Hirschsprung disease or irritable bowel syndrome.

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“…Hirschsprung's disease is a unique lower gastrointestinal tract anomaly in which there is an absence of enteric neurons from the myenteric and submucosal plexuses 5. Hirschsprung's disease has been linked to many different medical conditions, including trisomy 21, multiple endocrine neoplasia type 2 and Waardenburg syndrome, to name only a few 6…”

Section: Introductionmentioning

confidence: 99%

Müllerian anomaly in a woman with Hirschsprung's disease

Davis

Hoover

Kauffman³

2017

BMJ Case Reports

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Müllerian anomalies are usually isolated or found in association with abnormalities of the renal tract. Rarely, they are encountered with coexisting Ggastrointestinal anomalies. Similarly, Hirschsprung's disease may coexist with additional somatic abnormalities, yet only a single case report has reported a müllerian anomaly coexisting with Hirschsprung's. A 35-year-old woman with long-standing dysmenorrhoea and infertility and history of Hirschsprung's disease and some components of VATER syndrome (vertebral anomalies, anal atresia, tracheoesophageal fistula, renal anomalies) underwent operative laparoscopic exploration for pelvic pain and was found to have an incidental finding of a unilateral segmentally hypoplastic uterine horn (variation of an ASRM (American Society for Reproductive Medicine) type 2C müllerian anomaly). The genetics of müllerian anomalies and Hirschsprung's disease have been extensively studied with certain loci and candidate genes theorised as being causative. An established familial relationship and heritability exists with both syndromes; however, no genetic foci have been found to connect müllerian abnormalities and Hirschsprung's disease.

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A Practical Guide for the Diagnosis of Primary Enteric Nervous System Disorders

Cited by 81 publications

References 76 publications

Glycerin suppositories used prophylactically in premature infants (SUPP) trial: a study protocol for a pilot randomized controlled trial

Glycerin suppositories used prophylactically in premature infants (SUPP) trial: a study protocol for a pilot randomized controlled trial

Gene-environment interactions and the enteric nervous system: Neural plasticity and Hirschsprung disease prevention

Müllerian anomaly in a woman with Hirschsprung's disease

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