The Adrenal Cortex and Its Disorders

Hughes, Claire; Man, Elim; Achermann, John C.

doi:10.1002/9781119152712.ch9

Cited by 4 publications

(4 citation statements)

References 50 publications

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“…Another potential limitation was that our study included MC samples taken up to 9 a.m. Cortisol secretion reaches morning peak at approximately 8 a.m., and therefore MC testing is usually conducted at this time. 19,20,26 Due to some individuals having been screened with MC past this 8 a.m. peak, it is possible that our study overtested individuals who had an MC <240 nmol/L with a confirmatory ACTH stimulation test.…”

Section: Discussionmentioning

confidence: 99%

“…9 Since cortisol secretion follows a diurnal pattern with a morning peak at approximately 8 a.m., the morning cortisol (MC) test is commonly used for this purpose. 19 Selecting an accurate threshold value for diagnosing AI in children is challenging, considering variability related to sex and pubertal status. 20 One review suggests that for children, an MC level of <83 nmol/L is indicative of AI, whereas ≥500 nmol/L rules it out.…”

Section: Introductionmentioning

confidence: 99%

“…The potential need for routine AI surveillance has been recommended by Valentin et al., after identifying an AI prevalence of 43% in an adult kidney transplant cohort 9 . Since cortisol secretion follows a diurnal pattern with a morning peak at approximately 8 a.m., the morning cortisol (MC) test is commonly used for this purpose 19 . Selecting an accurate threshold value for diagnosing AI in children is challenging, considering variability related to sex and pubertal status 20 .…”

Section: Introductionmentioning

confidence: 99%

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Adrenal insufficiency in pediatric kidney transplantation recipients

Chae,

Ahmed,

Bone

et al. 2024

Pediatric Transplantation

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BackgroundImmunosuppression of pediatric kidney transplant (PKT) recipients often includes corticosteroids. Prolonged corticosteroid exposure has been associated with secondary adrenal insufficiency (AI); however, little is known about its impact on PKT recipients.MethodsThis was a retrospective cohort review of PKT recipients to evaluate AI prevalence, risk factors, and adverse effects. AI risk was assessed using morning cortisol (MC) and diagnosis confirmed by an ACTH stimulation test. Potential risk factors and adverse effects were tested for associations with MC levels and AI diagnosis.ResultsFifty‐one patients (60.8% male, age 7.4 (IQR 3.8, 13.1) years; 1 patient counted twice for repeat transplant) were included. Patients at risk for AI (MC < 240 nmol/L) underwent definitive ACTH stimulation testing, confirming AI in 13/51 (25.5%) patients. Identified risk factors for AI included current prednisone dosage (p = .001), 6‐month prednisone exposure (p = .02), daily prednisone administration (p = .002), and rejection episodes since transplant (p = .001). MC level (2.5 years (IQR 1.1, 5.1) post‐transplant) was associated with current prednisone dosage (p < .001), 6‐month prednisone exposure (p = .001), daily prednisone administration (p = .006), rejection episodes since transplant (p = .003), greater number of medications (β = −16.3, p < .001), 6‐month hospitalization days (β = −3.3, p = .013), creatinine variability (β = −2.4, p = .025), and occurrence of acute kidney injury (β = −70.6, p = .01).ConclusionGreater corticosteroid exposure was associated with a lower MC level and confirmatory diagnosis of AI noted with an ACTH stimulation test. Adverse clinical findings with AI included greater medical complexity and kidney function lability. These data support systematic clinical surveillance for AI in PKT recipients treated with corticosteroids.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Adrenal insufficiency in pediatric kidney transplantation recipients

Chae,

Ahmed,

Bone

et al. 2024

Pediatric Transplantation

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“…About 100 cases of ACTH-independent CS have been reported in association with bilateral nodules of 2–4 mm diameter with microadenomatous hyperplasia with brown-black colour on cut section due to lipofuscin pigment, presenting with typical features of CS, of these, 50% were younger than 15 years of age 3. PPNAD is associated with mutation of PRKAR1A (PPNAD1), PDE11A (PPNAD2) or PDE8B (PPNAD3) genes and is distinguished from ACTH-independent macronodular adrenal hyperplasia (AIMAH), another rare cause of endogenous CS, by intranodular adrenal tissue atrophy 4…”

Section: Introductionmentioning

confidence: 99%

Primary pigmented nodular adrenal disease presenting as hypertensive crisis

2022

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We present a case of a young girl who presented with hypertensive crisis and recent onset weight gain with hirsutism. On evaluation for Cushing syndrome (CS), her cortisol concentration was high, showed a paradoxical cortisol rise on dexamethasone suppression and the adrenocorticotropic hormone (ACTH) was low. Adrenal imaging showed normal adrenal morphology. Genetic diagnosis of primary pigmented nodular adrenal disease (PPNAD) was made. She was operated for bilateral adrenalectomy and histopathology confirmed the diagnosis of PPNAD. Our case highlights the rare aetiology of PPNAD as a cause of CS resulting in a hypertensive crisis. To the best of our knowledge, this is the youngest case of ACTH independent CS presenting as hypertensive encephalopathy.

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Two siblings with non-classic P450scc deficiency resulted from a novel mutation inCYP11A1gene misdiagnosed as familial glucocorticoid deficiency

Duong

Nguyen

et al. 2022

BMJ Case Rep

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P450scc deficiency due toCYP11A1gene mutations is a rare cause of primary adrenal insufficiency (PAI) in children. We reported two young siblings with PAI presented with hyperpigmentation. They were referred to our clinic with a diagnosis of familial glucocorticoid deficiency (FGD), another rare cause of PAI. However, further hormonal evaluation revealed increased plasma renin activity, which was not congruent with the diagnosis of FGD. Genetic analysis showed the compound heterozygous mutations in exon 8 of theCYP11A1gene, including a missense mutation, R466W (c1396C>T), and a nonsense mutation, R439X (c1315C>T). A case responded well to hydrocortisone, while another case received prednisolone due to her unresponsiveness to hydrocortisone. To correctly diagnose P450scc deficiency, physicians should be alerted with the similarity between this disease and FGD because of their predominant glucocorticoid deficiency. Long-acting glucocorticoids may be used with caution to reach treatment goals.

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The Adrenal Cortex and Its Disorders

Cited by 4 publications

References 50 publications

Adrenal insufficiency in pediatric kidney transplantation recipients

Adrenal insufficiency in pediatric kidney transplantation recipients

Primary pigmented nodular adrenal disease presenting as hypertensive crisis

Two siblings with non-classic P450scc deficiency resulted from a novel mutation inCYP11A1gene misdiagnosed as familial glucocorticoid deficiency

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