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地塞米松抑制试验联合唾液皮质醇测定在成人重型β地中海贫血中的应用

Low-dose Synachten test with measurement of salivary cortisol in adult patients with β-thalassemia major.

机构信息

Neuroendocrine Research Laboratory, Istituto Auxologico Italiano IRCCS, Milan, Italy.

Division of Endocrine and Metabolic Diseases, Ospedale San Luca, Istituto Auxologico Italiano IRCCS, Milan, Italy.

出版信息

Endocrine. 2018 May;60(2):348-354. doi: 10.1007/s12020-018-1562-z. Epub 2018 Mar 23.

Abstract

PURPOSE

Beta-thalassemia major is a severe, congenital hematological disorder and, if untreated, leads to early mortality. Progress in therapeutical strategies improved clinical outcomes and life expectancy; however, increased survival led to the development of new disorders, including endocrinopathies. Little is known on the possible impairment of adrenocortical function, a potentially life-threatening condition, in long-term thalassaemic survivors. We therefore decided to assess adrenal reserve and the value of salivary cortisol during ACTH stimulation in the diagnosis of adrenocortical insufficiency in adult patients with β-thalassemia major.

METHODS

Cross-sectional study including 72 adults with β-thalassemia major. Patients were tested with 1 µg ACTH for serum and salivary cortisol.

RESULTS

Subnormal serum cortisol responses to ACTH stimulation (i.e., <500 nmol/l) were registered in 15 out of 72 patients. Salivary cortisol increased in parallel with serum cortisol and a clear-cut positive correlation was detected at each timepoint. Moreover, peak salivary cortisol values after ACTH stimulation were significantly lower in patients with impaired adrenal reserve (513.6 ± 52.33 vs. 914.1 ± 44.04 nmol/l p < 0.0001).

CONCLUSIONS

Our results attest to the need for testing for adrenal insufficiency among adult thalassaemic patients, as up to 20% presented impaired adrenal reserve. Salivary and serum cortisol levels during stimulation with ACTH were closely correlated and the use of salivary cortisol sampling during ACTH testing may represent a surrogate to serum cortisol in these patients.

摘要

目的

重型β地中海贫血是一种严重的先天性血液疾病,如果不进行治疗,会导致早期死亡。治疗策略的进步改善了临床结果和预期寿命;然而,生存时间的延长导致了新的疾病的发展,包括内分泌疾病。对于长期生存的β地中海贫血患者,其肾上腺皮质功能(一种潜在的危及生命的疾病)可能受到损害,目前人们对此知之甚少。因此,我们决定评估长期生存的β地中海贫血患者的肾上腺储备,并在 ACTH 刺激下检测唾液皮质醇在诊断肾上腺皮质功能不全方面的价值。

方法

这项横断面研究纳入了 72 名β地中海贫血成年患者。患者接受了 1μg ACTH 刺激的血清和唾液皮质醇检测。

结果

在 72 名患者中,有 15 名患者的血清皮质醇对 ACTH 刺激的反应异常(即<500nmol/l)。唾液皮质醇与血清皮质醇平行增加,在每个时间点都检测到明显的正相关。此外,在肾上腺储备受损的患者中,ACTH 刺激后的唾液皮质醇峰值明显较低(513.6±52.33 vs. 914.1±44.04nmol/l,p<0.0001)。

结论

我们的研究结果表明,需要对成年地中海贫血患者进行肾上腺功能不全的检测,因为多达 20%的患者存在肾上腺储备受损。ACTH 刺激时唾液和血清皮质醇水平密切相关,在这些患者中,使用 ACTH 检测时的唾液皮质醇采样可以作为血清皮质醇的替代物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b66e/5893656/c5d61947cfb6/12020_2018_1562_Fig1_HTML.jpg

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