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Paediatric and adult colonic manometry: A tool to help unravel the pathophysiology of constipation

World Journal of Gastroenterology 2010年第41期16卷 5162-5172页

作者： Philip G Dinning Marc A Benninga bridget r southwell S Mark Scott Department of Gastroenterology St George Hospital University of New South Wales Kogarah Sydney 2217 Australia Department of Pediatric Gastroenterology and Nutrition Emma Children's Hospital/AMC Amsterdam 1105 AZ The Netherlands Murdoch Childrens research Institute Royal Children's Hospital Parkville Melbourne 3052 Australia Department of Paediatrics University of Melbourne Melbourne 3052 Australia Queen Mary University London Barts and the London School of Medicine and Dentistry London E11BB United Kingdom

Colonic motility subserves large bowel functions, including absorption, storage, propulsion and defaecation. Co-lonic motor dysfunction remains the leading hypothesis to explain symptom generation in chronic constipation, a heterogeneous condition which is extremely prevalent in the general population, and has huge socioeconomic impact and individual suffering. Physiological testing plays a crucial role in patient management, as it is now accepted that symptom-based assessment, although im-portant, is unsatisfactory as the sole means of directing therapy. Colonic manometry provides a direct method for studying motor activities of the large bowel, and this review provides a contemporary understanding of how this technique has enhanced our knowledge of normalcolonic motor physiology, as well as helping to elucidate pathophysiological mechanisms underlying constipation. Methodological approaches, including available catheter types, placement technique and recording protocols, are covered, along with a detailed description of re-corded colonic motor activities. This review also criti-cally examines the role of colonic manometry in current clinical practice, and how manometric assessment may aid diagnosis, classification and guide therapeutic inter-vention in the constipated individual. Most importantly, this review considers both adult and paediatric patients. Limitations of the procedure and a look to the future are also addressed.

关键词： Colon Constipation Manometry Paediatric Adult

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Multiple endocrine neoplasia 2B: Differential increase in enteric nerve subgroups in muscle and mucosa

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World Journal of Gastrointestinal Pathophysiology 2017年第3期8卷 142-149页

作者： John M Hutson Pam J Farmer Cristal J Peck Chung W Chow bridget r southwell Surgical research/Gastrointestinal Motility Laboratory Murdoch Children’s Research Institute Departments of Urology and Anatomical Pathology The Royal Children’s HospitalParkville Victoria Department of Paediatrics University of Melbourne

Multiple endocrine neoplasia 2B(MEN2B) is a rare syndrome caused by an activating mutation of the rET gene, leading to enteric gangliomatosis. This child presented with constipation at 1-mo old, was diagnosed with MEN2 B by rectal biopsy at 4 mo, had thyroidectomy at 9 mo and a colectomy at 4 years. We studied the extent of neuronal and nerve fibre proliferation and which classes of enteric nerves are affected by examining the colon with multiple neuronal antibodies. resected transverse colon was fixed, frozen, sectioned and processed for fluorescence immunohistochemistry labelling with antibodies against TUJ1, Hu, ChAT, NOS, VIP, SP and CGrP and cKit. Control transverse colon was from the normal margin of Hirschsprung(HSCr) colon(4-year-old) and a child with familial adenomatous polyposis(FAP, 12 year). Myenteric ganglia were increased in size to as wide as the circular muscle. There was a large increase in nerve cells and nerve fibres. ChAT-, NOS-, VIP-and SP-immunoreactive nerve fibres all increased in the myenteric ganglia. NOS-Ir nerves preferentially increased in the muscle, while VIP and SP increased in submucosal ganglia and mucosal nerve fibres. The density of ICC was normal. rET overactivation in MEN2B lead to a large increase in intrinsic nerve fibres in the myenteric and submucosal ganglia, with a relative increase in NOS-Ir nerve fibres in the circular muscle and VIP and SP in the submucosal ganglia and mucosa. The changes were associated with severe constipation resulting in colectomy at 4 years.

关键词： Enteric nervous system rET Neuroganglioma MEN2B

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