Adrenal cortex and its disorders

Miller, Walter L.; Flück, Christa E.

doi:10.1016/b978-1-4557-4858-7.00022-6

Cited by 28 publications

(25 citation statements)

References 247 publications

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“…Long duration of glucocorticoid therapy and high steroid doses are risk factors for the development adrenal insufficiency [ 10 , 14 ]. Consistently, in our study higher glucocorticoid doses and prolonged duration of the therapy associated with low serum morning cortisol levels.…”

Section: Discussionmentioning

confidence: 99%

“…Adrenal suppression is a condition in which adrenal glands do not produce adequate amounts of cortisol in response to physiological stress. It is caused by the suppression of the hypothalamic-pituitary-adrenal (HPA) axis by the circulating exogenous glucocorticoids and may lead into adrenal crisis or even death [ 10 ].…”

Section: Introductionmentioning

confidence: 99%

“…Risk factors for the development of adrenal suppression are not yet fully understood. However, high glucocorticoid dose and long duration of the therapy seem to associate with the increased risk [ 10 ] but intestinal absorption and genetic factors related to glucocorticoid sensitivity are likely to play an additional role [ 13 ]. Indeed, it is recommended that all children who have received pharmacologic doses of systemic glucocorticoid therapy for more than two weeks should be assessed for adrenal insufficiency [ 14 ].…”

Section: Introductionmentioning

confidence: 99%

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Screening for adrenal suppression in children with inflammatory bowel disease discontinuing glucocorticoid therapy

Sidoroff

Kolho

2014

BMC Gastroenterol

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BackgroundPharmacological doses of corticoids may result in adrenal suppression but with individual sensitivity. In paediatric inflammatory bowel disease (IBD), glucocorticoids are needed in the majority of the patients but there are less studies related to tapering off the drugs. The objective of this study was to estimate the frequency of adrenal insufficiency in children with IBD that were at the end of their systemic glucocorticoid therapy course.MethodsThe study was a retrospective case series of 59 consecutive paediatric IBD patients (median age 14.1 years; Crohn’s disease n = 22, ulcerative colitis n = 26, unclassified colitis n = 11) that were on oral prednisolone therapy about to be discontinued. The study patients were treated in a tertiary university hospital setting. Serum morning cortisol was measured with Immulite 2000 cortisol kit. Values < 20 nmol/l are undetectable and indicate adrenal suppression, values > 69 nmol/l are considered to represent normal basal secretion.ResultsThe morning cortisol was below the reference range in 20% of the patients and undetectable in 10%. Low cortisol levels associated with higher daily glucocorticoid doses (median 7.2 mg/m2 vs. 3.0 mg/m2 in patients with normal cortisol levels, p < 0.05) and with the long duration of the treatment (median 11 months vs. 4 months, p < 0.05). Patients with undetectable cortisol levels recovered within few weeks (median 5.6 weeks).ConclusionsIn paediatric IBD prolonged courses of glucocorticoids are frequent due to the steroid-dependent nature of the disease in a considerable proportion of patients. Adrenal suppression may occur in at least one fifth of the patients despite slowly tapering off the glucocorticoids. Notably, this is based on a set of serum cortisol measurements by request of experienced clinicians. All paediatric IBD patients receiving conventional doses of oral glucocorticoids should be subjected to screening for adrenal suppression when anticipated discontinuation of the drug.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Screening for adrenal suppression in children with inflammatory bowel disease discontinuing glucocorticoid therapy

Sidoroff

Kolho

2014

BMC Gastroenterol

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“…Very rarely, drug abuse and environmental toxins may play a role and should be considered in unsolved cases ( 2 ). Timely investigation of the girl with virilization at puberty is essential, particularly if associated with a sudden onset of symptoms and/or a rapid progression because this should raise immediate concern of an androgen-secreting tumor of the ovaries or adrenal cortex ( 3-6 ).…”

Section: Introductionmentioning

confidence: 99%

“…In addition, rarer forms of DSD affecting gonadal development and/or sex steroid synthesis and action need to be considered. CAH is in most cases caused by 21-hydroxylase deficiency resulting from autosomal recessive variants of the CYP21A2 gene, which has a prevalence of its classic form of 1 in 15,000 births worldwide ( 3 , 9 ). Numerous genetic variants lead to glucocorticosteroid deficiency and adrenal androgen excess ( 3 , 9 ).…”

Section: Introductionmentioning

confidence: 99%

Approach to the Virilizing Girl at Puberty

Santi

Graf

Zeino

et al. 2020

The Journal of Clinical Endocrinology &Amp; Metabolism

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Virilization is the medical term for describing a female who develops characteristics associated with male hormones (androgens) at any age, or when a newborn girl shows signs of prenatal male hormone exposure at birth. In girls, androgen levels are low during pregnancy and childhood. A first physiologic rise of adrenal androgens is observed at the age of 6-8 years and reflects functional activation of the zona reticularis of the adrenal cortex at adrenarche manifesting clinically with first pubic and axillary hairs. Early adrenarche is known as ‘premature adrenarche’. It is mostly idiopathic and of uncertain pathologic relevance but requires the exclusion of other causes of androgen excess (e.g. non-classic congenital adrenal hyperplasia) that might exacerbate clinically into virilization. The second modest physiologic increase of circulating androgens occurs then during pubertal development which reflects the activation of ovarian steroidogenesis contributing to the peripheral androgen pool. However, at puberty initiation (and beyond) ovarian steroidogenesis is normally devoted to estrogen production for the development of secondary female bodily characteristics (e.g. breast development). Serum total testosterone in a young adult woman is therefore about 10-20-fold lower than in a young man, while midcycle estradiol is about 10-20-fold higher. But if androgen production starts too early, progresses rapidly and in marked excess (usually more than 3-5 times above normal), females will manifest with signs of virilization such as masculine habitus, deepening of the voice, severe acne, excessive facial and (male typical) body hair, clitoromegaly and increased muscle development. Several medical conditions may cause virilization in girls and women including androgen-producing tumors of the ovaries or adrenal cortex, (non-)classical congenital adrenal hyperplasia (CAH) and, more rarely, other disorders (also referred to as differences) of sex development (DSD). The purpose of this article is to describe the clinical approach to the girl with virilization at puberty, focussing on diagnostic challenges. The review is written from the perspective of the case of an 11.5-year-old girl who was referred to our clinic for progressive, rapid onset clitoromegaly, and was then diagnosed with a complex genetic form of DSD that led to abnormal testosterone production from a dysgenetic gonad at onset of puberty. Her genetic workup revealed a unique translocation of an abnormal duplicated Y-chromosome to a deleted chromosome 9, including the Doublesex and Mab-3 Related Transcription factor 1 (DMRT1) gene.

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Challenges in the diagnosis and management of disorders of sex development

Kutney

Konczal

Kaminski

et al. 2016

Birth Defects Research Pt C

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Disorders of sex development (DSD) represent a spectrum of uncommon but very complex disorders with medical, psychosexual, and family implications for those affected by them. The diagnosis and management of these disorders requires a coordinated team of multiple specialists. Following an international conference in Chicago in 2005, a consensus statement was created and presented, which has resulted in a new paradigm in the nomenclature, classification, and management of DSDs. Since that time, many improvements have been forthcoming, most notably in the area of molecular genetic technologies. These developments have advanced our understanding of the specific etiologies underlying many of these conditions. In this article, we present an overview of the physiology of sex development, a few clinical vignettes highlighting specific pathologic conditions, discussions regarding the evaluation and management of these disorders, and some thoughts on future directions in this field. Birth Defects Research (Part C) 108:293–308, 2016. © 2016 Wiley Periodicals, Inc.

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Adrenal cortex and its disorders

Cited by 28 publications

References 247 publications

Screening for adrenal suppression in children with inflammatory bowel disease discontinuing glucocorticoid therapy

Screening for adrenal suppression in children with inflammatory bowel disease discontinuing glucocorticoid therapy

Approach to the Virilizing Girl at Puberty

Challenges in the diagnosis and management of disorders of sex development

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