• 主题
高级搜索  >
历史搜索 清空历史
首页> 美国卫生研究院文献>Journal of the Endocrine Society >MON-055 A Rare Case of a Macroadenoma Causing Cushing Syndrome in a Pediatric Patient
【2h】

MON-055 A Rare Case of a Macroadenoma Causing Cushing Syndrome in a Pediatric Patient

机译：Mon-055罕见的颈部罕见的致癌患者在儿科患者中导致缓冲综合征

代理获取
本网站仅为用户提供外文OA文献查询和代理获取服务，本网站没有原文。下单后我们将采用程序或人工为您竭诚获取高质量的原文，但由于OA文献来源多样且变更频繁，仍可能出现获取不到、文献不完整或与标题不符等情况，如果获取不到我们将提供退款服务。请知悉。
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Background: Pediatric Cushing Syndrome, a rare diagnosis, is almost always caused by pituitary microadenomas less than 5 mm in children older than 6 years old. We report on a 9-year-old girl with a 2.3 cm pituitary macroadenoma, whose ACTH and urinary 24-hour free cortisol were the highest recorded at our institution. Clinical Case: A 9-year-old pre-pubertal female presented with six months of frontal headaches, rapid weight gain, and hirsutism. Two months prior she developed fatigue and proximal muscle weakness and pain. On physical exam, she had plethoric round facies with acne and hirsutism, dorsal fat pad, central adiposity, and violet colored abdominal striae. Her pubertal development was tanner stage 3 for breast and 2 for pubic hair. BMI was 95th percentile and height was 40th percentile, previously 75th and 50th percentile respectively one year prior. 24 hour urinary free cortisol was 40,650 mcg/day [normal:100 mcg/day]. A 48 hour high dose dexamethasone suppression test was done as it is the most accurate in pediatric patients over 40 kg, morning cortisol was 100 mcg/dL [normal: 5-20 mcg/dL], ACTH 868 pg/ml [normal: 9-57 pg/ml], 24 Urinary Free Cortisol was 15,878 mcg/day [normal: 100 mcg/day]. A MRI Pituitary/Sella revealed a 2.3 cm pituitary macroadenoma superiorly displacing and flattening the optic chiasm, invading into the right cavernous sinus. She was referred to Neurosurgery, who did a partial transphenoidal resection, pathology consistent with ACTH producing tumor. Post-operatively she developed central diabetes insipidus and adrenal insufficiency for which she received desmopressin and oral hydrocortisone respectively. Her laboratory values eight months since surgery show normalization of ACTH and cortisol levels. The patient’s general health has improved, headaches have resolved, strength has returned, and her hirsutism is reduced. Her BMI remains elevated at 88% but is declining and growth velocity is increasing back to her pre-disease level. Conclusion: Cushing Syndrome is exceedingly rare in pediatric aged patients and pituitary macroadenomas are atypical in this population.This is a unique case of an ACTH producing macroadenoma in a Pediatric patient, which has seldom been reported in the literature, and should be considered in patients with similar presenting symptoms.
机译：背景：儿科缓冲综合征，罕见的诊断，几乎总是由垂体微观细胞造成的，在6岁6岁以上的儿童少于5毫米。我们报告了一个9岁的女孩，垂体垂体癌瘤，其acth和尿24小时免费皮质醇是我们机构的最高记录。临床案例：一位9岁的前普别特女性患有六个月的正面头痛，重量快，重量增益和流氓主义。在她开发疲劳和近端肌肉无力和疼痛的两个月。在体育考试中，她有充满粉刺和流氓，背部脂肪垫，中央肥胖和紫罗兰色腹部腹部的近圆形。她的青春期发育是乳腺和2的乳腺第3阶段为阴毛。 BMI为95百分位数，高度为40百分位数，以前75th和50百分位数分别前一年。 24小时尿液式皮质醇为40,650 mcg / day [正常：100 mcg / day]。 48小时高剂量的地塞米松抑制试验，因为它是40公斤40公斤的儿科患者中最准确的，早晨皮质醇为100 mcg / dl [正常：5-20 mcg / dl]，ACTH 868 pg / ml [正常：9 -57 pg / ml]，24个尿液式皮质醇为15,878 mcg / day [正常：100 mcg / day]。 MRI垂体/溶液揭示了2.3厘米垂体的脑垂体瘤优势，光学赤字，侵入右侧海绵窦。她被称为神经外科，他做了部分转骨切除术，与产生肿瘤的acth一致的病理学。操作后，她开发了中枢性糖尿病胰岛素和肾上腺功能不全，她分别接受了去氨加压素和口服氢化胞质。她的实验室价值8个月以来，手术显示acth和皮质醇水平的正常化。患者的一般健康状况有所改善，头痛已经解决了，力量已经归还，她的流氓主义减少了。她的BMI保持升高为88％，但下降，增长速度正在增加疾病预级别。结论：在儿科老年患者和垂体癌症中，缓解综合征非常罕见。这一人口中是非典型的。这是一种独特的案例，在文献中很少报告的儿科患者中的acroadenoma是一种独特的acth，应该在患者中考虑具有类似的呈现症状。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
代理获取

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号