Circadian Plasma Cortisol Measurements Reflect Severity of Hypercortisolemia in Children with Different Etiologies of Endogenous Cushing Syndrome

Tirosh, Amit; Lodish, Maya; Lyssikatos, Charalampos; Belyavskaya, Elena; Papadakis, Georgios Z.; Stratakis, Constantine A.

doi:10.1159/000464463

Cited by 3 publications

(1 citation statement)

References 13 publications

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“…Our prior study has specifically included midnight cortisol as one factor that is linked to disease severity . Additionally, the ratio of midnight to morning cortisol levels has also been reported to indicate the extent of hypercortisolemia . Previous studies in CS patients have noted the difficulty in distinguishing between cardiovascular remodelling caused by increased cortisol and metabolic disease .…”

Section: Discussionmentioning

confidence: 99%

Aortic pulse wave velocity in children with Cushing syndrome: A window into a marker of early cardiovascular disease

Blain

Sinaii

Zeltser

et al. 2019

Endocrino Diabet & Metabol

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Summary Objective To investigate early signs of cardiovascular arterial remodelling in paediatric patients with Cushing syndrome (CS) in comparison with normative values from healthy children. Study Design The metrics used to assess cardiac health were from thoracic aorta and carotid MRI. Scans were performed on 18 children with CS (mean: 12.5 ± 3.1 years, range: 6.0‐16.8 years, 10 female). Pulse wave velocity (PWV), aortic distensibility (AD) and carotid intima‐media thickness (cIMT), well‐validated measurements of cardiac compromise, were measured from the images and compared to normative age‐matched values where available. Results Patients with CS had significantly higher PWV compared to age‐adjusted normal median control values (4.0 ± 0.7 m/s vs. 3.4 ± 0.2 m/s, respectively, P = 0.0115). PWV was positively correlated with midnight plasma cortisol ( r = 0.56, P = 0.02). Internal and common cIMT were negatively correlated with ascending AD ( r = −0.75, P = 0.0022, r = −0.69, P = 0.0068, respectively). Conclusion Pulse wave velocity data indicate that paediatric patients with CS have early evidence of cardiovascular remodelling. The results suggest the opportunity for monitoring as these changes begin in childhood.

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Section: Discussionmentioning

confidence: 99%

Aortic pulse wave velocity in children with Cushing syndrome: A window into a marker of early cardiovascular disease

Blain

Sinaii

Zeltser

et al. 2019

Endocrino Diabet & Metabol

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Cushing Disease: Diagnosis and Treatment

Tatsi

Stratakis

2019

Pituitary Disorders of Childhood

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The clinical characteristics and pathogenic variants of primary pigmented nodular adrenocortical disease in 210 patients: a systematic review

Sun,

Ding,

et al. 2024

Front. Endocrinol.

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AimsPrimary pigmented nodular adrenocortical disease (PPNAD), as a rare kind of Cushing’s syndrome, is frequently misdiagnosed. To get a better understanding of the disease, we analyzed the clinical characteristics and pathogenic variants of PPNAD.MethodsDatabases were searched, and the pathogenic variants and clinical manifestations of patients were summarized from the relevant articles.ResultsA total of 210 patients in 86 articles were enrolled with a median age of 22 and a female-to-male ratio of 2:1. Sixty-six (31.43%) patients were combined with Carney complex (CNC) and 94.29% were combined with osteoporosis/osteopenia. Among 151 patients who underwent genetic testing, 87.42% (132/151) had pathogenic variants. Six gene mutations (PRKAR1A, PDE11A, PRKACA, CTNNB1, PDE8B, and ARMC5) were detected in the patients. The most common mutation was PKAR1A, accounting for 79.47% (120/151). There was a significant correlation between PRKAR1A pathogenic variant and spotty skin pigmentation in CNC concurrent with PPNAD (p < 0.05). Among pregnant patients with PPNAD, those without surgical treatment and with bilateral adrenalectomy suffered from a high-risk perinatal period. However, patients with unilateral adrenalectomy presented a safe perinatal period.ConclusionsFor young patients with Cushing’s syndrome, especially female patients with spotty skin pigmentation and osteoporosis/osteopenia, PPNAD should be considered. Unilateral adrenal resection may be considered as an option for women with fertility needs. In view of the difficulty of PPNAD diagnosis, genetic testing before surgery might be a reasonable option. Patients with PPNAD with spotty skin pigmentation should consider the PRKAR1A pathogenic variant and pay attention to CNC.Systematic review registrationhttps://www.crd.york.ac.uk/prospero, identifier CRD42023416988.

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Circadian Plasma Cortisol Measurements Reflect Severity of Hypercortisolemia in Children with Different Etiologies of Endogenous Cushing Syndrome

Cited by 3 publications

References 13 publications

Aortic pulse wave velocity in children with Cushing syndrome: A window into a marker of early cardiovascular disease

Aortic pulse wave velocity in children with Cushing syndrome: A window into a marker of early cardiovascular disease

Cushing Disease: Diagnosis and Treatment

The clinical characteristics and pathogenic variants of primary pigmented nodular adrenocortical disease in 210 patients: a systematic review

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