2012
DOI: 10.1136/bcr-2012-006685
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Cushing syndrome associated with an adrenal tumour

Abstract: SummaryCushing syndrome (CS) in children is a rare disorder that is most frequently caused by an adrenal tumour or a pituitary corticotrophinsecreting adenoma. The management is challenging and requires an individualised approach and multidisciplinary care. We present the case of a 23-month-old female child with a history of excessive weight gain, growth failure, hirsutism, acne and behavioural difficulties. Investigations revealed elevated serum midnight cortisol and 24 h urinary free cortisol. Overnight dexa… Show more

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Cited by 5 publications
(3 citation statements)
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“…Hypersensitivity to metyrapone or any of the excipientsHow should metyrapone be used in special populations?Paediatric patientsNo specific dosage adjustment required (limited data; 21 case reports[8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23], including 1 case of rectal administration[21]); adjust based on cortisol levels and tolerabilityElderly patients (≥ 65 years) No specific dosage adjustment required (limited data) [11 case reports[16,[24][25][26]]What other special warnings and precautions pertain to the use of metyrapone?Excipients b Delayed allergic reactions may occurAssay methods Measure plasma/serum and urine cortisol levels using a reliable assay without cross-reactivity with steroids precursors (e.g. a specific immuno-assay or a liquid chromatography-mass spectrometry method) to allow accurate dose adjustment Supervision Monitor and instruct patients on the signs and symptoms of hypocortisolism; manage hypocortisolism with temporary exogenous steroid replacement therapy and/or dose modification (reduction or interruption), as necessary…”
mentioning
confidence: 99%
“…Hypersensitivity to metyrapone or any of the excipientsHow should metyrapone be used in special populations?Paediatric patientsNo specific dosage adjustment required (limited data; 21 case reports[8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23], including 1 case of rectal administration[21]); adjust based on cortisol levels and tolerabilityElderly patients (≥ 65 years) No specific dosage adjustment required (limited data) [11 case reports[16,[24][25][26]]What other special warnings and precautions pertain to the use of metyrapone?Excipients b Delayed allergic reactions may occurAssay methods Measure plasma/serum and urine cortisol levels using a reliable assay without cross-reactivity with steroids precursors (e.g. a specific immuno-assay or a liquid chromatography-mass spectrometry method) to allow accurate dose adjustment Supervision Monitor and instruct patients on the signs and symptoms of hypocortisolism; manage hypocortisolism with temporary exogenous steroid replacement therapy and/or dose modification (reduction or interruption), as necessary…”
mentioning
confidence: 99%
“…The rarity of ACTs in children can cause difficulties with diagnosis. Most of the available scientific papers describe individual cases observed for only a few years (Table 2) [8,[13][14][15].…”
Section: Discussionmentioning
confidence: 99%
“…The majority of cases present with a combination of clinical features of Cushing's syndrome and hyperandrogenism with or without pulmonary metastasis. [1][2][3] Hypertension may not be always present. 4 Diagnosis depends on clinical manifestations, demonstration of elevated hormone levels and localization of tumour(s).…”
Section: Introductionmentioning
confidence: 99%