Knowles Charles H, De Giorgio Roberto, Kapur Raj P, Bruder Elisabeth, Farrugia Gianrico, Geboes Karel, Gershon Michael D, Hutson John, Lindberg Greger, Martin Joanne E, Meier-Ruge William A, Milla Peter J, Smith Virpi V, Vandervinden Jean Marie, Veress Béla, Wedel Thilo
Neurogastroenterology Group, Centres for Academic Surgery and Pathology, Institute of Cellular and Molecular Science, Barts and the London School of Medicine and Dentistry, Queen Mary University London, London, Whitechapel, UK.
Acta Neuropathol. 2009 Aug;118(2):271-301. doi: 10.1007/s00401-009-0527-y. Epub 2009 Apr 10.
The term gastrointestinal neuromuscular disease describes a clinically heterogeneous group of disorders of children and adults in which symptoms are presumed or proven to arise as a result of neuromuscular, including interstitial cell of Cajal, dysfunction. Such disorders commonly have impaired motor activity, i.e. slowed or obstructed transit with radiological evidence of transient or persistent visceral dilatation. Whilst sensorimotor abnormalities have been demonstrated by a variety of methods in these conditions, standards for histopathological reporting remain relatively neglected. Significant differences in methodologies and expertise continue to confound the reliable delineation of normality and specificity of particular pathological changes for disease. Such issues require urgent clarification to standardize acquisition and handling of tissue specimens, interpretation of findings and make informed decisions on risk-benefit of full-thickness tissue biopsy of bowel or other diagnostic procedures. Such information will also allow increased certainty of diagnosis, facilitating factual discussion between patients and caregivers, as well as giving prognostic and therapeutic information. The following report, produced by an international working group, using established consensus methodology, presents proposed guidelines on histological techniques and reporting for adult and paediatric gastrointestinal neuromuscular pathology. The report addresses the main areas of histopathological practice as confronted by the pathologist, including suction rectal biopsy and full-thickness tissue obtained with diagnostic or therapeutic intent. For each, indications, safe acquisition of tissue, histological techniques, reporting and referral recommendations are presented.
胃肠神经肌肉疾病这一术语描述了儿童和成人临床上一组异质性疾病，其症状被推测或证实是由神经肌肉功能障碍（包括Cajal间质细胞功能障碍）引起的。这类疾病通常存在运动活动受损，即通过影像学证据显示有短暂或持续性内脏扩张，导致转运减慢或受阻。虽然在这些情况下已通过多种方法证实了感觉运动异常，但组织病理学报告的标准仍相对被忽视。方法和专业知识的显著差异继续混淆了疾病正常状态和特定病理变化特异性的可靠界定。这些问题需要紧急澄清，以规范组织标本的采集和处理、结果解读，并就肠道全层组织活检或其他诊断程序的风险效益做出明智决策。此类信息还将提高诊断的确定性，促进患者与护理人员之间的实际讨论，并提供预后和治疗信息。以下报告由一个国际工作组采用既定的共识方法编写，提出了关于成人和儿童胃肠神经肌肉病理学组织学技术和报告的拟议指南。该报告涉及病理学家面临的组织病理学实践的主要领域，包括直肠吸引活检以及出于诊断或治疗目的获取的全层组织。针对每一项，均给出了适应证、安全获取组织的方法、组织学技术、报告内容及转诊建议。
