Highlights► The adrenal glands from fgfr2IIIb knock-out mice are hypoplastic. ► Steroidogenic enzymes are reduced in fgfr2IIIb knock-out mice. ► Dlk1, rather than Shh, signalling is affected in the adrenal cortex of fgfr2IIIb knock-out mice. ► FGF1 is proposed as the ligand for FGFR2IIIb in the adrenal cortex.
Preliminary experience is presented of an objective radiological scoring system for selecting patients with PA for AVS. PST provides little, if any, useful additional information. A significant minority of patients with PA exhibit evidence of cortisol cosecretion, which may have implications for perioperative management.
SummaryContext In patients with primary aldosteronism (PA), adrenalectomy is potentially curative for those correctly identified as having unilateral excessive aldosterone production. It has been suggested that a recently developed and published clinical prediction score (CPS) may correctly identify some patients as having unilateral disease, without recourse to adrenal venous sampling.Objective We have applied the CPS to a large cohort of PA patients with defined and documented outcomes. We also incorporated a minor modification to the CPS and a radiological grading score (RGS) into our analysis to assess whether its performance could be augmented. Results A total of 75 patients with a robust diagnosis following bilateral adrenal venous cannulation and/or strictly defined surgical outcome were analysed. Applying the CPS to this group of patients produced a sensitivity of 38Á8% and a specificity of 88Á5% of correctly identifying unilateral aldosterone production. Using a suggested modification to the CPS, in which different levels of hypokalaemia were given different weightings, the sensitivity rose to 40Á8%, with an identical specificity. Using the RGS alone improved sensitivity to 91Á7%, but specificity was reduced to 62Á5%. Conclusion Applying the recently developed CPS to this cohort of patients, it was not possible to reproduce the 100% specificity reported in the original publication. Using the modified score or incorporating the RGS did not improve its performance. In this cohort, we were unable to show superiority of the CPS over an imaging-based strategy. CPS may have a role in guiding clinical decision-making, especially in those whose adrenal venous sampling (AVS) has been unsuccessful.
Context
In primary aldosteronism, cosecretion of cortisol may alter cortisol-derived adrenal venous sampling indices.
Objective
To identify whether cortisol cosecretion in primary aldosteronism alters adrenal venous sampling parameters and interpretation.
Design
Retrospective case–control study
Setting
A tertiary referral center
Patients
144 adult patients with primary aldosteronism who had undergone both adrenocorticotropic hormone-stimulated adrenal venous sampling and dexamethasone suppression testing between 2004 and 2018.
Main Outcome Measures
Adrenal venous sampling indices including adrenal vein aldosterone/cortisol ratios and the selectivity, lateralization, and contralateral suppression indices.
Results
21 (14.6%) patients had evidence of cortisol cosecretion (defined as a failure to suppress cortisol to ≤50 nmol/L post dexamethasone). Patients with evidence of cortisol cosecretion had a higher inferior vena cava cortisol concentration (P = .01) than those without. No difference was observed between the groups in terms of selectivity index, lateralization index, lateralization of aldosterone excess, or adrenal vein cannulation rate.
Conclusions
Cortisol cosecretion alters some parameters in adrenocorticotrophic hormone-stimulated adrenal venous sampling but does not result in alterations in patient management.
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