Magnetic resonance enteroclysis for Peutz-Jeghers syndrome
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چکیده
A 21-year-old male reporting melena over one week is presented. Laboratory testing revealed severe anemia (Hgb 7 g/dL). Hyperpigmentation spots were present in the patient’s bucal mucosa and lower lip that arose during infancy. Three years ago, these lesions had raised the clinical suspicion of Peutz-Jeghers syndrome (PJS) and the patient had undergone an upper gastrointestinal (GI) endoscopy and barium study of the small bowel, however no lesions were demonstrated. An urgent upper GI endoscopy was performed de monstrating a largely distended stomach, full of non-hemorrhagic content, suggestive of high intestinal obstruction. Two gastric polyps of ~1.5 cm diameter and multiple small duodenal polyps were also revealed. A second upper GI endoscopy with a PENTAX HD i-scan colonoscope (Pentax EC-3890Li, Pentax, Japan) revealed a large polyp nearly obstructing the enteric lumen after the fourth part of the duodenum (Fig. 1). Histology a
منابع مشابه
Peutz-Jeghers syndrome: a new understanding.
Peutz-Jeghers syndrome is an autosomal dominant inherited disorder characterized by hamartomatous polyps in the small bowel and mucocutaneous pigmentation. Patients with Peutz-Jeghers syndrome often present as surgical emergencies with complications of the polyps, such as intussusception, bowel obstruction and bleeding. Furthermore, repeated operations may be needed in some patients, which may ...
متن کامل[Peutz-Jeghers syndrome].
Peutz-Jeghers syndrome is a rare, autosomal dominant inherited disorder, which is characterized by mucocutaneous pigmentations, gastrointestinal polyposis and an increased risk of cancer. It is caused by germline mutations in the LKB1 tumour suppressor gene, as a result of which hamartomatous polyps can develop already at an early age, which may cause various complications, including abdominal ...
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