Cushing's Syndrome in Children and Adolescents -- Presentation, Diagnosis, and Therapy

Ma, Magiakou; Mastorakos, George; Eh, Oldfield; Mt, Gomez; Jl, Doppman; Gb, Cutler; Lk, Nieman; Gp, Chrousos

doi:10.1056/nejm199409083311002

Cited by 395 publications

(330 citation statements)

References 27 publications

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“…Here, as in other studies, bone age was within the normal range in 83% of cases [24]; although androgens accelerate bone maturation, hypercortisolaemia delays it [38,11]. Another difference is that pituitary MRI identified a lesion in all our 16 CD patients, in contrast with other studies where up to 30-50% of children with CD have normal pituitary imaging [24,3,1,18].…”

Section: Discussioncontrasting

confidence: 52%

“…Another difference is that pituitary MRI identified a lesion in all our 16 CD patients, in contrast with other studies where up to 30-50% of children with CD have normal pituitary imaging [24,3,1,18]. This increase in identification may be due to improvements in MRI technology over time.…”

Section: Discussioncontrasting

confidence: 48%

“…In the current study, within the whole group, growth retardation was present in 36%, hirsutism in 56% and hypertension in 50%. Weight gain was the most frequent presenting complaint, and changes in school performance and mood were commoner in our series (43%) compared to previous studies with a prevalence of 19% [24].…”

Section: Discussionmentioning

confidence: 44%

“…In a previous series of 72 children with CD, growth retardation was present in 82%, hypertension in 46% and striae in 40% [2]. In another cohort of 59 children with CS, growth retardation was found in 83%, hirsutism in 78%, striae in 61% and hypertension in 47% [24]. In the current study, within the whole group, growth retardation was present in 36%, hirsutism in 56% and hypertension in 50%.…”

Section: Discussionmentioning

confidence: 48%

“…The biochemical confirmation of hypercortisolism requires a two-stage approach: initially the diagnosis needs to be confirmed, followed by further biochemical tests and imaging to localize the origin of this excessive secretion. In children, weight gain and growth retardation are considered the most common presenting features [24]. Additionally, most adrenocortical tumours in children present with virilisation or precocious puberty in addition to the features of CS [30].…”

Section: Introductionmentioning

confidence: 99%

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Management of Cushing syndrome in children and adolescents: experience of a single tertiary centre

et al. 2016

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What is Known:-Cushing syndrome is an extremely rare entity in the paediatric and adolescent age groups, so not many cohort studies have been published in this population.-Several tests can be employed to firstly diagnose hypercortisolaemia and secondly, identify the source of origin of it. The efficacy and safety of these tests in children is still uncertain. What is New:-This study includes cases due to the different aetiologies of endogenous hypercortisolaemia (pituitary, adrenal and ectopic hypercortisolaemia) allowing us to compare the differences in presentation, diagnosis, management and long-term outcome between the groups.-There is a difference in the prevalence of Cushing syndrome symptoms and in the performance of the tests in our cohort compared to previously published studies in the literature. ABSTRACT:The diagnosis and management of paediatric Cushing syndrome (CS) is highly challenging. This study aims to characterise its presentation, diagnosis, management and outcome by a retrospective case review of 30 patients (14 females) followed at a single tertiary paediatric endocrinology centre over a 30-year period.At presentation, median age was 8.9 years (0.2-15.5) and the commonest manifestations were weight gain (23/30), hirsutism (17/30), acne (15/30) and hypertension (15/30). Growth retardation was present in 11/30. Median BMI was +2.1SDS (-6.5 -+4.6). Urinary Free Cortisol (UFC) was abnormal in 17/18 (94%), midnight cortisol in 27/27 (100%) and Low Dose Dexamethasone Suppression (LDDS) test in 20/20 (100%). High Dose Dexamethasone Suppression (HDDS) test was abnormal in: 6/6 (100%) of adrenal tumours, 1/10 (10%) of Cushing Disease (CD) and 1/2 (50%) of ectopic tumours. Bilateral Inferior Petrosal Sinus Sampling (IPSS) identified 5 CD cases and 1 ectopic tumour.All patients underwent surgery and subsequently required cortisol replacement. Final diagnoses were 16 CD, 11 adrenal disease, 2 ectopic ACTH-secreting lesions and 1 case of unidentified aetiology. One year post-diagnosis, median BMI was 0.5SDS (-2.5 -+3.7), hypertension was present in 4/14 (28%) and 43% (12/30) of individuals were off hydrocortisone. Conclusion:The prevalence of the clinical manifestations differs from that reported in other series.Screening tests were highly sensitive, with UFC, midnight cortisol and LDDS performing well. One year post-treatment, BMI and BP normalized in the majority of patients, and almost half of them were able to discontinue replacement hydrocortisone.

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Section: Discussioncontrasting

confidence: 52%

Section: Discussioncontrasting

confidence: 48%

Section: Discussionmentioning

confidence: 44%

Section: Discussionmentioning

confidence: 48%

Section: Introductionmentioning

confidence: 99%

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Management of Cushing syndrome in children and adolescents: experience of a single tertiary centre

et al. 2016

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The Diagnosis of Cushing's Syndrome

Boscaro¹,

Barzon²,

Sonino³

2000

Arch Intern Med

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In the last 3 decades, there have been several advances in understanding the pathogenesis of Cushing's syndrome and in testing for the diagnosis and differential diagnosis of its various forms. Advanced diagnostic techniques provide useful tools in discovering ectopic adrenocorticotropic hormone sources. However, the occurrence of unusual clinical presentations, laboratory shortcomings, and exogenous compound interference may lead to wrong conclusions. This article reviews the atypical presentations of hypercortisolism and some laboratory shortcomings that may confuse the diagnosis of Cushing's syndrome. Comments and suggestions are given with the aim of helping the clinician avoid diagnostic mistakes.

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The Adrenal Cortex and its Disorders

Miller¹

2005

Clinical Pediatric Endocrinology

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Cushing's Syndrome in Children and Adolescents -- Presentation, Diagnosis, and Therapy

Cited by 395 publications

References 27 publications

Management of Cushing syndrome in children and adolescents: experience of a single tertiary centre

Management of Cushing syndrome in children and adolescents: experience of a single tertiary centre

The Diagnosis of Cushing's Syndrome

The Adrenal Cortex and its Disorders

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