Miyazaki E, Ohshiro K, Puri P
Children's Research Centre, Our Lady's Hospital for Sick Children, Dublin 12, Ireland.
Pediatr Surg Int. 1998 Sep;13(7):464-7. doi: 10.1007/s003830050375.
The purpose of this study was to investigate the specificity and sensitivity of NADPH-diaphorase (NADPH-D) staining in suction rectal biopsies (SRB) to determine whether it can be used as a diagnostic test for Hirschsprung's disease (HD) and related disorders. We studied SRB material in 80 patients suspected of having such disorders taken at 3, 5, and 7 cm above the pectinate line. Eight-micron sections were stained with hematoxylin and eosin, acetylcholinesterase histochemistry, and NADPH-D histochemistry. Normal biopsy specimens demonstrated strong NADPH-D reactivity in the submucosal ganglia and a large number of NADPH-D-positive fibers in the muscularis mucosae (MM). In contrast, there were no NADPH-D-positive fibers in the MM in patients with HD and hypertrophic nerve trunks stained weakly. Patients with hypoganglionosis (HYPG) demonstrated only a few NADPH-D-positive fibers in the MM and scant submucosal ganglia. Our results show that it is possible to diagnose HD and HYPG in mucosal rectal biopsies containing MM only and stained by NADPH-D histochemistry. As there is no background staining in NADPH-D histochemistry, it is easy to detect NADPH-D-positive fibers. NADPH-D histochemical staining may be an important additional technique for diagnosing HD and related disorders.
本研究的目的是调查直肠吸引活检（SRB）中烟酰胺腺嘌呤二核苷酸磷酸 - 黄递酶（NADPH - D）染色的特异性和敏感性，以确定其是否可作为先天性巨结肠症（HD）及相关疾病的诊断检测方法。我们研究了80例疑似患有此类疾病患者的SRB材料，取材于齿状线以上3厘米、5厘米和7厘米处。8微米厚的切片用苏木精和伊红、乙酰胆碱酯酶组织化学以及NADPH - D组织化学进行染色。正常活检标本在黏膜下神经节显示出强烈的NADPH - D反应性，在黏膜肌层（MM）中有大量NADPH - D阳性纤维。相比之下，HD患者的MM中没有NADPH - D阳性纤维，肥大的神经干染色较弱。神经节减少症（HYPG）患者的MM中仅显示少数NADPH - D阳性纤维，黏膜下神经节稀少。我们的结果表明，仅通过含有MM且经NADPH - D组织化学染色的直肠黏膜活检就有可能诊断HD和HYPG。由于NADPH - D组织化学中没有背景染色，很容易检测到NADPH - D阳性纤维。NADPH - D组织化学染色可能是诊断HD及相关疾病的一项重要辅助技术。
