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.

Low-dose Synachten test with measurement of salivary cortisol in adult patients with β-thalassemia major / A.G. Ambrogio, L. Danesi, M. Baldini, R. Radin, E. Cassinerio, G. Graziadei, N. Mirra, E. D’Angelo, A. Marcon, M. Mancarella, A. Orsatti, F. Bonetti, M. Scacchi, M.D. Cappellini, L. Persani, F. Pecori Giraldi. - In: ENDOCRINE. - ISSN 1355-008X. - 60:2(2018 May 01), pp. 348-354. [10.1007/s12020-018-1562-z]

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

A.G. Ambrogio;R. Radin;G. Graziadei;A. Marcon;M. Mancarella;A. Orsatti;F. Bonetti;M. Scacchi;M.D. Cappellini;L. Persani;F. Pecori Giraldi
2018

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.
adrenocortical insufficiency; Cortisol-binding globulin; low-dose ACTH stimulation test; salivary cortisol; Serum cortisol; β-thalassemia major; endocrinology, diabetes and metabolism;
Settore MED/09 - Medicina Interna
Settore MED/13 - Endocrinologia
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/580273
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