博文

目前显示的是 九月, 2015的博文

新英格兰:骨硬化症和锥形瓶畸形

一位 8 岁男性患儿出现右腿新发急性疼痛。X 线示无移位性骨折(图 A)和股骨远端骨干倒置管形,即常说的锥形瓶畸形(图 B)。患儿即往有神经母细胞瘤病史7年余(患儿 6 月龄大时确认为神经母细胞瘤),神经母细胞瘤进行性发展,5年前经环磷酰胺和每月一次双膦酸盐化疗后神经母细胞瘤缓解。双膦酸盐治疗停止 18 月后因为担心骨硬化症而中止治疗。实验室检查,X 线检查和遗传学检查未有任何遗传或其他原因所致的获得性骨疾病发现,如原发性骨髓疾病,重金属中毒,骨代谢疾病以及其他发育不良等。 骨硬化症和正常骨(图B)之间的分界线表明某段时间骨转换率增加,从而引起骨生长增快。该发现更可能与双膦酸盐治疗更一致,而与遗传性因素关系不大。患者仍有发生在骨硬化和正常骨之间发生骨折的风险,因此建议患儿避免剧烈运动并随诊。18 个月后患儿未出现骨折和新的症状。 Osteopetrosis and Erlenmeyer-Flask Deformity An 8-year-old boy presented with new-onset acute pain in his right leg. Radiographs showed a nondisplaced fracture (Panel A) and undertubulation of the distal femoral diaphysis — a condition known as Erlenmeyer-flask deformity (Panel B). He had a history of neuroblastoma, which was diagnosed when he was 6 months of age; the disease progressed after standard chemotherapy but had been in remission for the 5 years before this presentation, after treatment with cyclophosphamide and monthly intravenous bisphosphonate therapy. Bisphosphonate therapy was discontinued after 18 months be

新英格兰:因十二指肠蹼引起的新生儿胃积气

一个 4 个月大的女性患儿拒食并伴有非胆汁性呕吐。患儿 1  月时曾因为轻度呕吐而住院治疗,期间患儿可以哺乳。但是 1 周患儿出现呕吐并拒食。体检发现患儿一般症状尚可,腹部膨胀,触之柔软。实验室检查示低氯性代谢性碱中毒;X 线示胃积气(图 A 和 B 箭头所示)。患儿给予鼻胃管留置,静脉补液治疗。上消化道 X 线造影检查示造影剂可通过胃到达小肠;后续 X 线显示积气和「双气泡」,提示十二指肠梗阻(图 C)。术中确认十二指肠梗阻因十二指肠蹼引起,患儿行十二指肠吻合术,术后患儿症状逐渐减轻。 胃积气比较罕见,可能因缺血或者感染导致黏膜破坏,从而气体渗透到胃壁而引起。新生儿胃积气与坏死性小肠结肠炎有关,但是十二指肠梗阻,幽门狭窄或乳源性肠胃结石(lactobezoar:酪蛋白质凝块能导致乳酸胃石,是奶和粘液在胃内形成的凝块,好发于1岁以内的婴儿,尤其是1个月以内的新生儿)导致的严重梗阻也可能会引起胃积气。 Gastric Pneumatosis A 4-month-old girl presented with poor feeding and nonbilious emesis. At 1 month of age, she had been hospitalized briefly for emesis but was able to feed until 1 week before presentation. She appeared well, and her abdomen was soft but distended. Laboratory investigations revealed a hypochloremic metabolic alkalosis, and radiographs showed gastric pneumatosis (Panels A and B, arrows). A nasogastric tube was placed, and intravenous administration of fluids was started. Radiography of the upper gastrointestinal tract with contrast revealed contrast medium passing into