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遗传性出血性毛细血管扩张症中发生的青少年息肉病。

Juvenile polyposis occurring in hereditary hemorrhagic telangiectasia.

作者信息

Inoue S, Matsumoto T, Iida M, Hoshika K, Shimizu M, Hisamoto N, Kihara T

机构信息

Department of Medicine, Kawasaki Medical School, Kurashiki-City, Japan.

出版信息

Am J Med Sci. 1999 Jan;317(1):59-62. doi: 10.1097/00000441-199901000-00010.

Abstract

Various extraintestinal manifestations may occur in juvenile polyposis, but hereditary hemorrhagic telangiectasia has rarely been reported in this type of polyposis. The authors treated a 14-year-old girl with rectal bleeding and anemia who had multiple polyps of the colorectum. Large polyps were removed and histologically diagnosed as juvenile polyps. She was later diagnosed as having telangiectasia of the skin, and arteriovenous malformations in the lung and in the liver, all of which were compatible with the diagnosis of hereditary hemorrhagic telangiectasia. At age 32, she had multiple ulcers in the ileum and in the colon. The coexistence of juvenile polyposis and hereditary hemorrhagic telangiectasia may be a clue for the understanding of the histopathogenesis of juvenile polyposis.

摘要

幼年性息肉病可能出现各种肠道外表现,但遗传性出血性毛细血管扩张症在这类息肉病中鲜有报道。作者治疗了一名14岁患有直肠出血和贫血的女孩,她的结直肠有多个息肉。大的息肉被切除,组织学诊断为幼年性息肉。她后来被诊断患有皮肤毛细血管扩张症,以及肺部和肝脏的动静脉畸形,所有这些都符合遗传性出血性毛细血管扩张症的诊断。32岁时,她的回肠和结肠出现多处溃疡。幼年性息肉病与遗传性出血性毛细血管扩张症的共存可能是理解幼年性息肉病组织发病机制的一个线索。

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