神经纤维直径能否有效用于提高先天性巨结肠症患者术中活检相比的无神经节细胞段长度判断的准确性？

Can the diameter of nerve fibers be effectively utilized to enhance the accuracy of determining the length of the aganglionic segment compared to intraoperative biopsy in patients with Hirschsprung's disease?

机构信息

Pediatric Disease Research Center, Guilan University of Medical Science, Rasht, Iran.

Medical school, Tehran University of Medical Science, Tehran, Iran.

出版信息

BMC Res Notes. 2024 Aug 6;17(1):220. doi: 10.1186/s13104-024-06873-x.

DOI:10.1186/s13104-024-06873-x

PMID:39107802

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11305011/

Abstract

OBJECTIVE

The aim of this study is to investigate the accuracy of utilizing neural fiber trunk diameter in accurately diagnosing the length of the aganglionic segment in patients definitively diagnosed with Hirschsprung's disease.

RESULTS

In this study, 40 patients (19 males, 21 females; mean age 2.5 ± 2.2646 years) were assessed for Hirschsprung's disease. Constipation was the main symptom (75%), followed by abdominal issues. All underwent contrast enema and rectal suction biopsy for diagnosis, followed by surgery (predominantly Soave and Swensen techniques). Majority (85%) had rectosigmoid involvement. Neural fiber diameter was measured, with 52.5% ≤40 μm and 47.5% >40 μm. Statistical analysis showed 40% sensitivity(CI:95%) and 47% specificity(CI:95%) with a cutoff of 40.5 μm. Cohen's kappa index for aganglionic segment size was 0.7.

摘要

目的

本研究旨在探讨利用神经纤维干直径准确诊断明确诊断为先天性巨结肠病患者无神经节细胞段长度的准确性。

结果

本研究共评估了 40 例（男 19 例，女 21 例；平均年龄 2.5±2.2646 岁）先天性巨结肠病患者。便秘是主要症状（75%），其次是腹部问题。所有患者均接受对比灌肠和直肠抽吸活检进行诊断，然后进行手术（主要为 Soave 和 Swensen 技术）。大多数（85%）患者存在直肠乙状结肠受累。测量神经纤维直径，其中 52.5%≤40μm，47.5%>40μm。统计分析显示，截断值为 40.5μm 时，敏感性为 40%（95%CI），特异性为 47%（95%CI）。无神经节细胞段大小的 Cohen's kappa 指数为 0.7。