Vistelle R, Grulet H, Gibold C, Chaufour-Higel B, Delemer B, Fay R, Delisle M J, Caron J
Endocrinology Unit, Laboratory of Pharmacology, Hôpital de la Maison Blanche, Reims, France.
Clin Endocrinol (Oxf). 1991 Feb;34(2):133-8. doi: 10.1111/j.1365-2265.1991.tb00283.x.
Increases in urinary, plasma and tumour adrenaline have been previously observed in MEN II patients with phaeochromocytoma. However, the sensitivity of adrenaline for early detection of adrenal medullary disease has not been accurately evaluated. Twenty-five patients with medullary thyroid carcinoma (MTC) histologically confirmed but without clinical or biological evidence of phaeochromocytoma have been studied. Medullary adrenal status was evaluated by adrenal CT-scan. MIBG scintigraphy, determination of urinary VMA, metanephrines and total catecholamine levels, measurement of nyctohemeral plasma adrenaline or noradrenaline concentrations (every 2 h during 24 h) and clonidine suppression test. Four of the 25 patients had evidence of adrenal medullary disease in view of the coexistence of CT-scan, MIBG scintigraphy and plasma adrenaline abnormalities. Moderate adrenal enlargement (unilateral, n = 3; bilateral, n = 1) was observed on scans together with a high adrenal MIBG uptake (bilateral, n = 4). Among the urinary parameters studied, a minor MN increase was observed in only one of the four patients. Plasma adrenaline levels were significantly (P less than 0.01) different from those of the other 21 patients (mean + SD 115 + 110 pmol/l). This plasma adrenaline increase is reproducible and not suppressed by clonidine. Unilateral adrenalectomy performed in one patient confirmed a phaeochromocytoma and induced normalization of plasma adrenaline levels. In contrast, the plasma noradrenaline levels of the four patients were not statistically different from those of the other 21 patients. These data suggest that persistent high plasma adrenaline levels may be selectively increased in MTC patients together with a moderate adrenal CT-scan enlargement and a high adrenal MIBG uptake, despite a normal urinary excretion of total catecholamines and catecholamines metabolites.(ABSTRACT TRUNCATED AT 250 WORDS)
此前在患有嗜铬细胞瘤的MEN II患者中观察到尿、血浆和肿瘤肾上腺素水平升高。然而,肾上腺素对肾上腺髓质疾病早期检测的敏感性尚未得到准确评估。对25例经组织学确诊为甲状腺髓样癌(MTC)但无嗜铬细胞瘤临床或生物学证据的患者进行了研究。通过肾上腺CT扫描评估肾上腺髓质状态。进行间碘苄胍(MIBG)闪烁显像、测定尿香草扁桃酸(VMA)、甲氧基肾上腺素和总儿茶酚胺水平、测量昼夜血浆肾上腺素或去甲肾上腺素浓度(24小时内每2小时一次)以及可乐定抑制试验。25例患者中有4例鉴于CT扫描、MIBG闪烁显像和血浆肾上腺素异常并存而有肾上腺髓质疾病的证据。扫描显示有中度肾上腺增大(单侧,n = 3;双侧,n = 1),同时肾上腺MIBG摄取较高(双侧,n = 4)。在所研究的尿参数中,仅4例患者中的1例观察到甲氧基肾上腺素略有升高。这4例患者的血浆肾上腺素水平与其他21例患者的血浆肾上腺素水平有显著差异(P小于0.01)(均值±标准差为115±110 pmol/L)。这种血浆肾上腺素升高是可重复的,且不受可乐定抑制。对1例患者进行单侧肾上腺切除术证实为嗜铬细胞瘤,并使血浆肾上腺素水平恢复正常。相比之下,这4例患者的血浆去甲肾上腺素水平与其他21例患者的血浆去甲肾上腺素水平无统计学差异。这些数据表明,尽管总儿茶酚胺及其代谢产物的尿排泄正常,但MTC患者可能会选择性地出现持续高血浆肾上腺素水平,同时伴有中度肾上腺CT扫描增大和高肾上腺MIBG摄取。(摘要截取自250字)