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Life-threatening gastrointestinal haemorrhage in Von Recklinghausen's disease.

作者信息

Hanly A M, Coffey J C, Breathnach O, Calvert R, Murphy M N, Cross K S

机构信息

Department of Surgery, Waterford Regional Hospital, Waterford, Ireland.

出版信息

Surgeon. 2007 Jun;5(3):186-9. doi: 10.1016/s1479-666x(07)80047-3.

Abstract

Neurofibromatosis Type I (NF-1), also known as Von Recklinghausen's disease, is a common disorder, but gastrointestinal manifestations are rare and can be associated with severe complications and malignancy. We describe a case of multiple intestinal tumours, which presented as major per-rectal bleeding and was diagnosed by laparotomy. Presenting symptoms of this condition are usually non-specific, but the risk of malignancy and perforation should allow for a high index of suspicion in patients with NF-1 presenting with gastrointestinal symptoms. We present this case as a reminder that blood loss from the bowel in Von Recklinghausen's disease may be life-threatening, requiring immediate surgery to control haemorrhage.

摘要

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