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儿童和青少年嗜铬细胞瘤。

Pheochromocytoma in children and adolescents.

机构信息

Department of Endorinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India.

出版信息

Indian J Pediatr. 2009 Nov;76(11):1151-3. doi: 10.1007/s12098-009-0287-7. Epub 2010 Jan 14.

Abstract

Eleven subjects aged <20 yr with histologically proven pheochromocytoma between 1987 and 2006 were analyzed. Family history was present in 18%. In 2 patients, pheochromocytoma was part of VHL and in one it was associated with MEN 2. Twenty four hour urine VMA level was elevated in 100% and metanephrine level in 73%. CT/ MRI were showing the tumor in all. Prazosin extended release tablets (maximum 30 mg/day) were used in 73% and doxazosin (maximum 12 mg/ day) in 27%. Intraoperative BP fluctuations were seen in 27%. All were biochemically cured after surgery. Preoperative á blockade with extended release prazosin and doxazosin were effective in controlling perioperative BP fluctuations. Hence these drugs can be used in children and adolescents without fear of postoperative hypotension.

摘要

分析了 1987 年至 2006 年间经组织学证实的 11 例年龄<20 岁的嗜铬细胞瘤患者。18%的患者有家族史。在 2 例患者中,嗜铬细胞瘤是 VHL 的一部分,在 1 例患者中,它与 MEN 2 有关。24 小时尿 VMA 水平升高 100%,间甲肾上腺素水平升高 73%。CT/MRI 均显示肿瘤存在。73%的患者使用了缓释特拉唑嗪片(最大剂量 30mg/天),27%的患者使用了多沙唑嗪(最大剂量 12mg/天)。27%的患者术中出现血压波动。所有患者术后均生化治愈。术前使用缓释特拉唑嗪和多沙唑嗪进行 á 阻断可有效控制围手术期血压波动。因此,这些药物可在儿童和青少年中使用,而不必担心术后低血压。

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