Uchida Keiichi, Otake Kohei, Inoue Mikihiro, Koike Yuhki, Matsushita Kohei, Araki Toshimitsu, Okita Yoshiki, Tanaka Koji, Uchida Katsunori, Yodoya Noriko, Iwamoto Shotaro, Arai Katsuhiro, Kusunoki Masato
Department of Gastrointestinal and Pediatric Surgery, Mie University Graduate School of Medicine, Tsu, Mie, Japan;
Department of Pathology, Mie University Graduate School of Medicine, Tsu, Mie, Japan;
Intractable Rare Dis Res. 2012 Feb;1(1):35-9. doi: 10.5582/irdr.2012.v1.1.35.
Lymphocytic intestinal leiomyositis is a rare entity, which causes chronic intestinal pseudo-obstruction (CIPO) in children. We present the first case of a boy who had pure red cell anemia 1 year before onset. Prolonged ileus developed after gastroenteritis and the patient was diagnosed using a biopsy of the intestinal wall. Findings from the present case indicate that there are three important factors for accurate diagnosis: history of enteritis, positive serum smooth muscle antibody, and lymphocyte infiltration with muscle destruction in the muscularis propria in the intestinal wall. Earlier diagnosis and induction of immunosuppressive therapy may be essential for a better outcome.
淋巴细胞性肠道平滑肌瘤病是一种罕见的疾病，可导致儿童慢性假性肠梗阻（CIPO）。我们报告首例发病前1年患有纯红细胞贫血的男孩。该患儿在患肠胃炎后出现了长时间的肠梗阻，并通过肠壁活检确诊。本病例的研究结果表明，准确诊断有三个重要因素：肠炎病史、血清平滑肌抗体阳性以及肠壁固有肌层中伴有肌肉破坏的淋巴细胞浸润。早期诊断并采用免疫抑制治疗可能是取得更好疗效的关键。
