■先天性巨结肠病(ADHD)的相关疾病表现出类似于先天性巨结肠病的症状,主要以肠梗阻为特征,肠道扩张,和慢性便秘.ADHD患者淀粉样蛋白并发症的发生很少。在这份报告中,我们介绍了一例以肠溃疡为首发表现的ADHD,随后的病理检查显示结肠粘膜中存在淀粉样蛋白沉积物。
■一名男性患者,20岁,表现为持续三年的反复腹胀和肠梗阻。多次结肠镜检查显示存在复发性结肠溃疡,病理检查表明结肠粘膜层中存在淀粉样蛋白沉积物。腹部CT扫描提示结肠扩张。经过多学科协商,进行了结肠次全切除,术后病理证实肌间神经丛神经节细胞减少和缺失。考虑到患者的症状和术后病理结果,诊断为ADHD.患者术后症状缓解,出院,随访1年,病情稳定。
■我们的研究强调了ADHD与结肠溃疡复发的初始表现之间的潜在关联。伴有淀粉样蛋白在肠粘膜中沉积。这一发现提示了ADHD的可能致病机制,并为其诊断提供了新的视角。
UNASSIGNED: Allied disorders of Hirschsprung\'s disease (ADHD) exhibit symptoms akin to those of Hirschsprung\'s disease, primarily characterized by intestinal obstruction, bowel dilatation, and chronic constipation. The occurrence of amyloid complications in patients with ADHD is infrequent. In this report, we present a
case of ADHD with intestinal ulcers as the initial gastrointestinal manifestation, and subsequent pathological examination revealed the presence of amyloid deposits in the colonic mucosa.
UNASSIGNED: A male patient, aged 20, exhibited recurring abdominal distension and intestinal obstruction for a duration of three years. Multiple colonoscopies revealed the presence of recurrent colonic ulcers, with pathological examination indicating the existence of amyloid deposits within the mucosal layer of the colon. Abdominal CT scans suggested colonic dilatation. Following a multidisciplinary consultation, a subtotal resection of the colon was performed, and subsequent postoperative pathology confirmed a decrease and absence of myenteric plexus ganglion cells. Considering the patient\'s symptoms and the findings from the postoperative pathology, a diagnosis of ADHD was made. The patient\'s symptoms resolved postoperatively and he was discharged from the hospital and followed up for 1 year in stable condition.
UNASSIGNED: Our study highlights the potential association between ADHD and the initial presentation of recurrent colonic ulcers, accompanied by amyloid deposition in the intestinal mucosa. This finding suggests a possible pathogenic mechanism for ADHD and offers a novel perspective on its diagnosis.