Shakir K M, Jasser M Z, Yoshihashi A K, Drake A J, Eisold J F
Department of Internal Medicine, National Naval Medical Center, Bethesda, MD 20889-5600, USA.
Mayo Clin Proc. 1996 Dec;71(12):1145-9. doi: 10.4065/71.12.1145.
To characterize a disorder of episodes of flushing and increased levels of 5-hydroxyindoleacetic acid (5-HIAA) in men with secondary hypogonadism who respond to testosterone therapy.
We present detailed case reports of three male patients who had flushing, secondary hypogonadism, and increased urinary 5-HIAA levels and describe their clinical and laboratory features before and after treatment with testosterone. In addition, six male patients with hypogonadism (three with primary and three with secondary hypogonadism) without flushing were assessed.
The three patients with flushing and secondary hypogonadism (serum total testosterone 5.45 +/- 0.63 nmol/L, free testosterone 89.3 +/- 7.0 pmol/L, follicle-stimulating hormone 3.85 +/- 0.58 IU/L, and luteinizing hormone 4.41 +/- 0.98 IU/L) had increased urinary 5-HIAA levels (98.5 +/- 12.2 micromol/24 h) but normal blood serotonin levels (9.66 +/- 1.58 micromol/L). During a pentagastrin-calcium stimulation test, serum calcitonin and blood serotonin values were normal in patients with secondary hypogonadism and flushing. Detailed investigation showed no evidence of a carcinoid tumor. Urinary 5-HIAA levels became normal (16.6 +/- 1.73 micromol/24 h) after treatment with testosterone. When testosterone therapy was discontinued in two patients, flushing and increased urinary 5-HIAA levels recurred. Furthermore, flushing and the elevated urinary 5-HIAA values resolved when testosterone treatment was reinitiated. The six patients with hypogonadism without flushing had normal urinary 5-HIAA levels (14.9 +/- 3.31 micromol/24 h).
Male patients with flushing and increased urinary 5-HIAA levels should undergo assessment for hypogonadism after screening for carcinoid tumor. If hypogonadism is diagnosed, resolution of flushing and normalization of 5-HIAA may be achieved with testosterone treatment. We suggest that pseudocarcinoid syndrome associated with hypogonadism be the descriptive label used for this combination of clinical features.
对继发性性腺功能减退且对睾酮治疗有反应的男性患者中出现的潮红发作及5-羟吲哚乙酸（5-HIAA）水平升高的病症进行特征描述。
我们呈现了3例患有潮红、继发性性腺功能减退且尿5-HIAA水平升高的男性患者的详细病例报告，并描述了他们在接受睾酮治疗前后的临床和实验室特征。此外，对6例无潮红的性腺功能减退男性患者（3例原发性和3例继发性性腺功能减退）进行了评估。
3例患有潮红和继发性性腺功能减退的患者（血清总睾酮5.45±0.63 nmol/L，游离睾酮89.3±7.0 pmol/L，促卵泡激素3.85±0.58 IU/L，促黄体生成素4.41±0.98 IU/L）尿5-HIAA水平升高（98.5±12.2 μmol/24 h），但血血清素水平正常（9.66±1.58 μmol/L）。在五肽胃泌素-钙刺激试验中，继发性性腺功能减退且有潮红的患者血清降钙素和血血清素值正常。详细检查未发现类癌肿瘤的证据。睾酮治疗后尿5-HIAA水平恢复正常（16.6±1.73 μmol/24 h）。当2例患者停用睾酮治疗时，潮红和尿5-HIAA水平再次升高。此外，重新开始睾酮治疗时，潮红和升高的尿5-HIAA值消失。6例无潮红的性腺功能减退患者尿5-HIAA水平正常（14.9±3.31 μmol/24 h）。
有潮红且尿5-HIAA水平升高的男性患者在筛查类癌肿瘤后应进行性腺功能减退评估。如果诊断为性腺功能减退，睾酮治疗可能使潮红消退且5-HIAA恢复正常。我们建议将与性腺功能减退相关的假性类癌综合征作为用于描述这种临床特征组合的标签。
