Assessing treatment adherence is crucial to determine adequacy of mineralocorticoid therapy

Pofi, Riccardo; Bonaventura, Ilaria; Duffy, Joanne R.; Maunsell, Zoë; Shine, Brian; Isidori, Andrea M.; Tomlinson, Jeremy

doi:10.1530/ec-23-0059

Cited by 3 publications

(1 citation statement)

References 53 publications

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“…However, it is thought that PRA lacks accuracy due to glucocorticoid-mediated changes in angiotensinogen levels and a resultant upward shift of PRA, hence a move towards using PRC ( 7 ). Whilst other studies have looked at using PRC ( 8 , 9 ), there are no reports directly comparing the use of PRC and PRA for monitoring of patients with adrenal insufficiency.…”

Section: Discussionmentioning

confidence: 99%

Performance of renin assays in selecting fludrocortisone dose in children with adrenal disorders

Morris,

Whatmore,

Hamilton

et al. 2024

Endocrine Connections

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Children with salt-wasting adrenal insufficiency are managed with glucocorticoid and mineralocorticoid replacement. Measurement of renin activity or concentration alongside blood electrolyte levels are used to monitor the adequacy of mineralocorticoid replacement. Our unit changed from using renin activity to renin concentration and carried out a service review to assess whether this influenced decision-making for fludrocortisone dosing. 50 measurements of plasma renin activity and 50 of renin concentration were analysed on separate cohorts before and after the assay change, with values standardised to multiples of the upper limit of normal (MoU) to allow comparison between assays. We were more likely to increase the fludrocortisone dose for a raised renin concentration than a raised renin activity. The renin concentration MoU was more strongly related to plasma sodium (negatively) and 17α-hydroxyprogesterone (17αOHP) (positively) than the renin activity MoU. Using a MoU cut-off of 1.5, a decision to increase the dose of fludrocortisone was more likely to be made when using the renin concentration assay compared to the activity assay. Using a cut-off of 40nmol/L for 17αOHP, a decision not to change the fludrocortisone dose when 17αOHP was <40 was more likely when using the renin concentration assay. For both assays, a plasma sodium <140mmol/L was more likely to lead to a fludrocortisone dose increase, and most likely for the renin concentration assay. Overall, the decision to adjust fludrocortisone dose in this cohort of children with adrenal insufficiency was better supported by the biochemical parameters when based on renin concentration results and clinical status.

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Section: Discussionmentioning

confidence: 99%

Performance of renin assays in selecting fludrocortisone dose in children with adrenal disorders

Morris,

Whatmore,

Hamilton

et al. 2024

Endocrine Connections

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Long‐term health consequences of congenital adrenal hyperplasia

Pofi,

Ji,

Krone

et al. 2023

Clinical Endocrinology

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Congenital adrenal hyperplasia (CAH) caused by 21‐hydroxylase deficiency accounts for 95% of all CAH cases and is one of the most common inborn metabolic conditions. The introduction of life‐saving glucocorticoid replacement therapy 70 years ago has changed the perception of CAH from a paediatric disorder into a lifelong, chronic condition affecting patients of all age groups. Alongside health problems that can develop during the time of paediatric care, there is an emerging body of evidence suggesting an increased risk of developing co‐morbidities during adult life in patients with CAH. The mechanisms that drive the negative long‐term outcomes associated with CAH are complex and involve supraphysiological replacement therapies (glucocorticoids and mineralocorticoids), excess adrenal androgens both in the intrauterine and postnatal life, elevated steroid precursors and adrenocorticotropic hormone levels. Alongside a review of mortality outcome, we discuss issues that need to be addressed when caring for the CAH patient including female and male fertility, cardio‐metabolic morbidity, bone health and other important long‐term outcomes of CAH.

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The multiple faces of autoimmune Addison’s disease in children

Capalbo,

Esposito,

Gaeta

et al. 2024

Front. Endocrinol.

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Primary adrenal insufficiency (PAI) is a rare medical condition, characterized by a deficiency in adrenal hormones. Although rare, PAI is a life-threatening disease requiring prompt recognition and treatment. However, symptoms of PAI are often non-specific and diagnosis can be challenging, causing frequent diagnostic delays. In adults, autoimmunity is the most common cause of PAI in industrialized countries, whereas in children, the most frequent etiology is represented by congenital defects of steroidogenesis and, in particular, by congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. A few recent case series from different countries have reported that autoimmunity is the second most common etiology of PAI in the pediatric age group. However, data on autoimmune PAI in children are still scant and the exact epidemiology, clinical manifestations, and long-term outcomes of this condition have yet to be defined. The scope of this review is to summarize the current knowledge on the etiology, presentation, and treatment of autoimmune PAI in childhood and to increase physicians’ awareness of the signs that should raise an early suspicion of this condition.

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Assessing treatment adherence is crucial to determine adequacy of mineralocorticoid therapy

Cited by 3 publications

References 53 publications

Performance of renin assays in selecting fludrocortisone dose in children with adrenal disorders

Performance of renin assays in selecting fludrocortisone dose in children with adrenal disorders

Long‐term health consequences of congenital adrenal hyperplasia

The multiple faces of autoimmune Addison’s disease in children

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