Work-up and management of paediatric Cushing's syndrome

Savage, Martin O.; Chan, Li F.; Grossman, Ashley; Storr, Helen L

doi:10.1097/med.0b013e328305082f

Cited by 43 publications

(39 citation statements)

References 47 publications

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“…Although this study confirms the predominance of CD in this age group, previous studies [31,25] have reported higher prevalence rates of pituitary CS.…”

Section: Discussionsupporting

confidence: 68%

“…The accuracy of HDDST has been questioned in several studies in adults [31,32,3,19,29] and in children it has been found to lack specificity, in that patients with ectopic CS may also show a degree of cortisol suppression on HDDST [28]. One of our patients with ectopic ACTH demonstrated a 75% reduction in serum cortisol, illustrating the fallibility of the HDDST.…”

Section: Discussionmentioning

confidence: 70%

“…ACTH-independent CS (15% of cases in adults) is due to increased autonomous production of cortisol from an adrenal tumour (adenoma or carcinoma) or hyperplasia [22,27]. In ACTH-dependent CS, the excess ACTH is produced either by the pituitary gland [Cushing's disease (CD), 75-80% in children] [31,25] or by other tumours (ectopic CS, less common in the pediatric population than the 15% estimate in adults) [31] or rarely, due to tumour production of excess corticotrophin-releasing hormone (CRH). 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.…”

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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“…Although this study confirms the predominance of CD in this age group, previous studies [31,25] have reported higher prevalence rates of pituitary CS.…”

Section: Discussionsupporting

confidence: 68%

Section: Discussionmentioning

confidence: 70%

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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“…Fakt ten powinien nasunąć podejrzenie organicznego podłoża zaburzeń. Brak skuteczności leczenia antydepresyjnego jest typowym zjawiskiem w zespołach depresyjnych spowodowanych hiperkortyzolemią [3,5,7].…”

Section: Dyskusjaunclassified

“…Takie opóźnienie jest częstym zjawiskiem, według danych z piśmiennictwa w endogennym zespole Cushinga rozpoznanie zwykle jest opóź-nione o 2-3 lata w stosunku do czasu wystąpienia pierwszych objawów [2,5,7,8]. U opisanej pacjentki część objawów, jak bóle głowy i nadciśnienie tęt-nicze, przez długi czas uznawano za psychogenne, a narastającą otyłość i wtórny brak miesiączki wią-zano z wpływem leków antydepresyjnych.…”

Section: Dyskusjaunclassified

Psychiatric disorders as predominant symptom of hypercortisolism in 17 year-old girl with primary pigmented nodular adrenocortical disease

Kądziela¹,

Kucharská²,

Grela³

2014

Pediatr. Endocrinol.

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55 Kądziela K. i inni: Zaburzenia psychiczne jako dominujący objaw hiperkortyzolemii u 17-letniej pacjentki z pierwotną pigmentową chorobą guzkową nadnerczy STRESZCZENIE/ABSTRACTEndogenny zespół Cushinga u dzieci i młodzieży spowodowany chorobą nadnerczy stanowi tylko 15-20% przypadków endogennej hiperkortyzolemii. Rzadkość występowania, powolne narastanie objawów i różna manifestacja kliniczna utrudniają diagnostykę i wydłużają czas ustalenia rozpoznania. Przedstawiamy przypadek 17-letniej pacjentki z endogenną hiperkortyzolemią, u której wczesnym i dominującym objawem były zaburzenia lękowo-depresyjne. Od ujawnienia się choroby do postawienia rozpoznania upłynęło około 3 lat. W tym czasie była nieskutecznie leczona lekiem antydepresyjnym. W momencie rozpoczęcia diagnostyki endokrynologicznej stwierdzono pełny obraz zespołu Cushinga z powikłaniami w postaci cukrzycy i osteopenii. U pacjentki rozpoznano pierwotną pigmentową chorobę guzkową nadnerczy (PPNAD). Wykonano dwuetapową, całkowitą adrenalektomię, co doprowadziło do ustąpienia u niej wszystkich objawów zespołu Cushinga, w tym także zaburzeń psychicznych. Endokrynol. Ped. 13/2014;1(46):55-60.Endogenous Cushing's syndrome in children and adolescents as a result of adrenal glands disease happens in only 15-20% of cases of endogenous hypercortisolism. Rarity of the disease, the slow progress of symptoms and variable clinical manifestation usually delays the diagnosis. We present a case of 17-year-old girl with endogenous hypercortisolism, in whome the early and predominant symptoms were anxiety-depressive disorders. From the onset of disease to the final diagnosis passed about three years. During this time the patient was treated ineffectively with antidepressant drug. At the begining of endocrinological investigations typical clinical and biochemical findings of Cushing's syndrome were revealed with complications of hypercortisolism: diabetes and osteopenia. In a patient primary pigmented nodular adrenocortical disease (PPNAD) was diagnosed. Two-step, total adrenalectomy was performed, which led to withdrawal of all symptoms of Cushing's syndrome, including psychiatric disorders. Pediatr. Endocrinol. 13/2014;1(46):55-60. Zaburzenia psychiczne jako dominujący objaw hiperkortyzolemii u 17-letniej pacjentki z pierwotną pigmentową chorobą guzkową nadnerczy Psychiatric disorders as predominant symptom of hypercortisolism in 17 year-old girl with primary pigmented nodular adrenocortical disease

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Cutaneous Manifestations of Endocrine Disease

Hogan¹

2011

Harper's Textbook of Pediatric Dermatology

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Work-up and management of paediatric Cushing's syndrome

Abstract: Early diagnosis and treatment of Cushing's syndrome is vital for long-term outcome. The overall prognosis for Cushing's syndrome is good but challenges remain to ensure normal postcure growth and body composition.

Cited by 43 publications

References 47 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

Psychiatric disorders as predominant symptom of hypercortisolism in 17 year-old girl with primary pigmented nodular adrenocortical disease

Cutaneous Manifestations of Endocrine Disease

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