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Pneumocystis Carinii Pneumonia in Ectopic Adrenocorticotrophic Hormone Syndrome: A Case Report and Literature Review
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摘要:
目的 探讨异位促肾上腺皮质激素(adrenocorticotropic hormone, ACTH)综合征合并肺孢子菌肺炎(pneumocystis carinii pneumonia, PCP)患者的临床特征和治疗。 方法 回顾性分析北京协和医院诊治的1例异位ACTH综合征合并PCP患者的临床资料, 并复习相关文献。 结果 患者女性, 52岁, 以向心性肥胖、乏力起病, 血ACTH、总皮质醇显著升高, 诊断异位ACTH综合征, 右肺类癌为分泌异位ACTH的病灶。合并PCP的症状出现于手术切除异位ACTH病灶、血ACTH及总皮质醇明显下降后, 主要症状为发热、咳嗽、气短, 经磺胺、外源性激素治疗后恢复。 结论 PCP是异位ACTH综合征的严重机会性感染类型, 高的血皮质醇水平预示着更高的肺孢子菌感染率, 异位ACTH综合征合并PCP患者的症状出现在开始治疗、血皮质醇水平下降、免疫功能重建时, 尽早明确病原学、预防性治疗对改善异位ACTH综合征合并PCP预后有益。 -
关键词:
- 异位促肾上腺皮质激素综合征 /
- 肺孢子菌肺炎
Abstract:Objective To analyze the clinical features and therapy of patients with ectopic adrenocorticotrophic hormone (ACTH) syndrome and pneumocystis carinii pneumonia (PCP). Methods A retrospective study was conducted on one patient of ectopic ACTH syndrome with PCP diagnosed and treated in Peking Union Medical College Hospital. Relevant literature was reviewed. Results The patient was a 52-year-old woman presented with central obesity and weakness. Laboratory investigations revealed marked elevations of plasma ACTH and cortisol levels. A diagnosis of ectopic ACTH syndrome based on a carcinoid tumor in her right lung was made. The symptoms of complicated PCP occurred shortly after the resection of the tumor that caused ACTH syndrome and the reduction of plasma ACTH and cortisol levels, which were mainly fever, nonproductive cough, and shortness of breath. The patient recovered after the treatment of sulfamethoxazole and glucocorticoid. Conclusions PCP is a severe opportunistic infection in ectopic ACTH syndrome. Patients with higher cortisol level may at a higher risk of pneumocystis carinii infection. Clinical symptoms of PCP may be present after initiation of treatment, reduction of cortisol level, and recovery of immune functions. Early identification of etiological mechanism and prophylactic treatment for PCP may improve the prognosis of ACTH patients with PCP. -
表 1 异位ACTH综合征合并PCP患者临床特征
病例 文献来源 性别 年龄
(岁)ACTH
(pg/ml)*UFC
(μg)皮质醇
(μg/dl)*PCP症状
距治疗时间发病时
皮质醇*
(μg/dl)预防 转归** 合并感染 1 本例 女 52 198.0 3826.3 ＞75.0 3 d 29.1 磺胺 恢复 肺炎克雷伯杆菌 2 Gabalec等[3] 女 60 250.0 4940.9 114.2 4 d 44.3 *** 恢复 巨细胞病毒 3 Gabalec等[3] 男 20 300.0 430.7 ＞50.0 4 d 15.4 - 恢复 - 4 Arlt等[4] 男 36 1118.0 3297.1 79.0 10 d 34.6 - 死亡 - 5 Oosterhuis等[5] 女 62 296.0 18 637.6 70.5 数天 - - 恢复 - 6 Oosterhuis等[5] 女 57 318.0 106 685.9 85.9 2 d - 磺胺 恢复 - 7 Kim等[6] 女 60 460.0 2509.0 80.0 3 d 16.2 - 死亡 大肠杆菌 8 Keenan等[7] 女 26 204.5 11237.5 66.9 2周 10.9~14.5 - 恢复 - 9 Bakker等[8] 男 56 315.0 18 654.2 197.5 4 d 36.2 - 死亡 单纯疱疹病毒、金黄色
葡萄球菌、曲霉菌10 McOuillen等[9] 女 73 194.0 528.0 102.8 18 d 86.5 - 死亡 - 11 Sieber等[10] 男 66 431.4 - 116.0 未治疗 - - 死亡 曲霉菌、巨细胞病毒 12 Graham等[11] 男 50 - - 117.0 - - - 死亡 粘质沙门氏菌 13 Fulkerson等[12] 女 38 - - - 2 d - - 死亡 李斯特菌 14 Natale等[13] 女 24 - - - 1月 - - 恢复 奴卡菌 ACTH：促肾上腺皮质激素；PCP：肺孢子菌肺炎；UFC：尿游离皮质醇；*数据采用文献原文中的单位，与法定计量单位的换算关系：ACTH 1 pg/ml= 0.22 pmol/L，皮质醇1 μg/dl=27.6 nmol/L；* *定义为该次PCP转归；* * *文献中未提及或数据不确切 
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