Publications by authors named "William F. Young"

Background: Primary aldosteronism can be treated medically but there is no standardised method to evaluate treatment outcomes. We aimed to develop criteria for assessing the outcomes of targeted medical treatment of primary aldosteronism, analyse outcomes across an international cohort, and identify factors associated with a complete treatment response.

Methods: An international panel of 31 primary aldosteronism experts used the Delphi method to reach consensus on the definition of complete, partial, or absent biochemical and clinical outcomes of medical treatment of primary aldosteronism.

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Purpose: To evaluate the impact of corticosteroid premedication on the performance of adrenal vein sampling (AVS) in patients with primary aldosteronism (PA) and allergy to iodinated contrast media (ICM).

Materials And Methods: Patients who underwent AVS for PA, between September 1990 and October 2023, were retrospectively identified. Patients with ICM allergy who received corticosteroid pre-medication were matched 1:1 with patients without contrast allergy.

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Context: Data on giant pheochromocytomas (PHEO), defined based on size ≥ 10 cm, are scarce.

Objective: to compare presentation, management, and outcomes of patients with giant vs non-giant PHEOs.

Design: retrospective cohort study, 2000-2023.

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Article Synopsis
  • This study examines the link between clinical and imaging features with hormonal subtypes, growth, and surgical treatment of incidental adrenal cortical adenomas (ACAs) in a cohort of patients from 2000 to 2016.
  • Out of 1516 patients, various hormonal subtypes were identified, with factors like age, sex, and tumor size influencing these subtypes, while imaging measures like Hounsfield units (HU) were not significant predictors.
  • The findings indicate younger age, larger tumor size, and increased growth were associated with a higher likelihood of needing adrenalectomy, especially for patients with mild autonomous cortisol secretion (MACS) and nonfunctioning adenomas (NFAs).
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Background: Protein-truncating germline pathogenic variants in the N- and C-terminal exons (2, 9, and 10) of the MEN1 gene may be associated with aggressive pancreatic neuroendocrine tumors. However, the impact of these variants on parathyroid disease is poorly understood. We sought to investigate the effects of genotype and surgical approach on clinical phenotype and postoperative outcomes in patients with multiple endocrine neoplasia type 1 (MEN1)-related primary hyperparathyroidism.

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Primary aldosteronism is a prevalent but underdiagnosed cause of hypertension, contributing to increased cardiovascular and cerebrovascular events and end-organ damage independent of blood pressure. Prompt diagnosis and treatment with targeted surgical or medical therapy reduce the risk of complications and improve prognosis. This review outlines a practical approach to diagnosis and management of primary aldosteronism for global practitioners.

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Objective: Glucocorticoid withdrawal syndrome (GWS) is a scarcely studied phenomenon that complicates the recovery following surgical remission of hypercortisolism. We aimed to characterize the presence and trajectory of glucocorticoid withdrawal symptoms in the postoperative period and to determine presurgical predictors of GWS severity.

Design: Longitudinal observational study.

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Objective: Prospective data on determinants of muscle strength impairment and quality of life in patients with various subtypes and severity of endogenous hypercortisolism are lacking.

Design: Single-center cross-sectional study, 2019 to 2022.

Methods: Patients with Cushing syndrome (CS) and mild autonomous cortisol secretion (MACS) were assessed with clinical and biochemical severity scores, muscle function (nondominant hand grip strength and sit-to-stand test), and quality of life (Short Form-36 [SF36] and CushingQoL).

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Background: Paragangliomas are rarely found in the mediastinum, where they account for a small proportion of mediastinal masses. This study aimed to better characterize the presenting features and relevant aspects in optimizing the diagnosis and treatment of mediastinal paragangliomas.

Methods: A computer-assisted search of electronic health records was performed to identify adult patients (≥18 years) who underwent evaluation for a primary mediastinal paraganglioma at Mayo Clinic between January 2000 and April 2022.

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Objective: Adrenal schwannomas and juxta-adrenal schwannomas are rare tumours. We aimed to summarise their clinical, biochemical and imaging characteristics.

Design: Single-centre retrospective study of eligible patients between 1995 and 2022.

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Objective: The objective of this study was to evaluate the role of adrenal venous sampling (AVS) in guiding the management of patients with corticotropin (ACTH)-independent glucocorticoid secretory autonomy and bilateral adrenal masses.

Design And Patients: A cohort with 25 patients underwent AVS and surgical management.

Measurements: Cortisol was measured from the adrenal veins (AVs) and inferior vena cava (IVC).

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Article Synopsis
  • Bilateral idiopathic hyperaldosteronism (IHA) accounts for 60% of primary aldosteronism cases, with medical management being the main treatment, but unilateral adrenalectomy is debated as a palliative option.* -
  • A study reviewed 24 patients who had undergone unilateral adrenalectomy for IHA, showing most had preoperative hypokalemia and required multiple antihypertensives that decreased post-surgery, with no significant complications.* -
  • The results suggest that unilateral adrenalectomy can improve blood pressure control and potassium levels in select patients with bilateral hyperaldosteronism, but more extensive studies are needed to clarify its benefits.*
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Intrapericardial paragangliomas are rare, highly vascular tumors that frequently adhere to adjacent structures and blood vessels, making surgical resection challenging. In this case series, we discuss the role of multimodality imaging and preoperative embolization in the management of 3 patients presenting with intrapericardial paragangliomas. ().

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Objective: Benign adrenal cysts are rare lesions of the adrenal glands. Limited data are available to guide management. We aimed to describe the presentation and outcomes of patients with benign adrenal cysts.

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Article Synopsis
  • Urinary bladder paraganglioma (UBPGL) is a rare tumor, with a study analyzing 110 patients revealing a median diagnosis age of 50 years and a median tumor size of 2 cm.
  • Only 34% of patients were diagnosed prior to surgery, highlighting a need for improved diagnostic methods, and 25% required additional treatments post-surgery.
  • The study found that younger age, larger tumor size, and higher catecholamine levels were linked to a greater risk of metastases, and patients with synchronous metastases had significantly higher disease-specific mortality.
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Context: Due to its rare incidence, molecular features of primary aldosteronism (PA) in young adults are largely unknown. Recently developed targeted mutational analysis identified aldosterone-driver somatic mutations in aldosterone-producing lesions, including aldosterone-producing adenomas (APAs), aldosterone-producing nodules (APNs), and aldosterone-producing micronodules, formerly known as aldosterone-producing cell clusters.

Objective: To investigate histologic and genetic characteristics of lateralized PA in young adults.

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Objective: Cystic adrenal mass is a rare imaging presentation of pheochromocytoma. We aimed to describe the clinical, biochemical and imaging characteristics of patients with cystic pheochromocytoma.

Design: Single-centre, retrospective study, 2000-2020.

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Background: Benign adrenal tumors are commonly discovered on cross-sectional imaging. Mild autonomous cortisol secretion (MACS) is regularly diagnosed, but its effect on cardiometabolic disease in affected persons is ill defined.

Objective: To determine cardiometabolic disease burden and steroid excretion in persons with benign adrenal tumors with and without MACS.

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Context: The detection and management of concomitant pheochromocytoma (PHEO) and primary aldosteronism (PA) is not well understood.

Objective: To investigate varying presentations and outcomes of cases with coexisting PHEO and PA to provide an approach to its diagnosis and management.

Methods: We conducted a retrospective case series of adult patients with concomitant PHEO and PA at Mayo Clinic from 2000-2020 and an additional review of cases before 2000 and from the medical literature.

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Article Synopsis
  • Autonomous cortisol secretion (ACS) occurs in up to 50% of patients with adrenal adenomas, and clinical guidelines suggest measuring serum DHEA-S and ACTH levels for diagnosis.
  • A study analyzed 468 adults with adrenal adenomas from 2000 to 2020, finding that lower levels of DHEA-S and ACTH correlated with ACS diagnosis.
  • The combination of DHEA-S <40 mcg/dL and ACTH <10 pg/mL offered the best accuracy for diagnosing ACS, achieving 92% specificity and 87% positive predictive value (PPV).
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