A case of Peutz-Jeghers syndrome presenting as multiple intussusception of small bowel – A rare surgical emergency

N. M. Srinivas, Mohammed Muzamil Pasha, Hanim Mohammed Ridwan
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Abstract

Peutz-Jeghers syndrome (PJS) is an autosomal dominant genetic disorder characterised by melanin pigment spots on the oral mucosa, lips, nasal alae, palm and soles, as well as hamartomatous polyps in the alimentary canal. Polyps are often a cause of intussusception in the affected patients. Cancers of the gastrointestinal system, uterus, and breast are common in patients with PJS. Long-term follow-up is required to prevent intussusception in children and cancer in adults. We report a classical case of PJS presenting with multiple intussusception of the small bowel in a 20-year-old male patient.
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一例表现为多发性小肠肠套叠的 Peutz-Jeghers 综合征--罕见的外科急症
Peutz-Jeghers 综合征(PJS)是一种常染色体显性遗传疾病,其特征是口腔粘膜、嘴唇、鼻翼、手掌和足底出现黑色素沉着斑,以及消化道出现肉瘤状息肉。息肉通常是导致患者肠梗阻的原因之一。胃肠道系统、子宫和乳腺癌在 PJS 患者中很常见。为预防儿童肠套叠和成人癌症,需要进行长期随访。我们报告了一例典型的 PJS 病例,患者为一名 20 岁男性,表现为多发性小肠肠套叠。
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