Fukunaga Shuhei, Takedatsu Hidetoshi, Mitsuyama Keiichi, Torimura Takuji
Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine.
Department of Gastroenterology and Medicine, Fukuoka University Faculty of Medicine.
Kurume Med J. 2018 Feb 26;64(1.2):25-27. doi: 10.2739/kurumemedj.MS00014. Epub 2017 Jul 3.
Concomitant association of histologically proven inflammatory bowel disease (IBD) and neurofibromatosis type 1 (NF1) is a rare finding documented in only 5 adult clinical cases. A 34-year-old woman with known neurofibromatosis was admitted to our department with a 6-month history of intractable bloody diarrhea and abdominal pain. After a thorough clinical examination and paraclinical assessments, including colonoscopy and biopsy, ulcerative colitis (UC) was confirmed as the cause of gastrointestinal bleeding. NF1 is considered an autosomal dominant condition caused by mutations in the NF1 gene, which is located on chromosome 17q11.2 [1]. A wide variety of NF1 mutations have been found in patients with NF1, but no frequently recurring mutation has been identified. Since the pathogenesis of IBD is also associated with genetic make-up, these two entities may be associated with a genetic factor.
经组织学证实的炎症性肠病(IBD)与1型神经纤维瘤病(NF1)并存是一种罕见的情况,仅有5例成人临床病例记录在案。一名患有神经纤维瘤病的34岁女性因顽固性血性腹泻和腹痛6个月入住我科。经过全面的临床检查和辅助检查评估,包括结肠镜检查和活检,确诊溃疡性结肠炎(UC)为胃肠道出血的病因。NF1被认为是一种常染色体显性疾病,由位于17q11.2染色体上的NF1基因突变引起[1]。在NF1患者中发现了多种NF1突变,但尚未确定常见的复发性突变。由于IBD的发病机制也与基因组成有关,这两种疾病可能与遗传因素有关。