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Review
. 2023 Dec 12:14:1329082.
doi: 10.3389/fendo.2023.1329082. eCollection 2023.

Adrenal Cushing's syndrome in children

Valentina Guarnotta  1 , Fabrizio Emanuele  1 , Riccardo Salzillo  1 , Carla Giordano  1
Affiliations
Review

Adrenal Cushing's syndrome in children

Valentina Guarnotta et al. Front Endocrinol (Lausanne). .

Abstract

Adrenal Cushing's syndrome is a rare cause of endogenous hypercortisolism in neonatal and early childhood stages. The most common causes of adrenal CS are hyperfunctioning adrenal tumours, adenoma or carcinoma. Rarer causes are primary bilateral macronodular adrenal hyperplasia (PBAMH), primary pigmented adrenocortical disease (PPNAD) and McCune Albright syndrome. The diagnosis represents a challenge for clinicians. In cases of clinical suspicion, confirmatory tests of hypercortisolism should be performed, similarly to those performed in adults. Radiological imaging should be always combined with biochemical confirmatory tests, for the differential diagnosis of adrenal CS causes. Treatment strategies for adrenal CS include surgery and in specific cases medical drugs. An adequate treatment is associated to an improvement of growth, bone health, reproduction and body composition from childhood into and during adult life. After cure, lifelong glucocorticoid replacement therapy and endocrine follow-up are required, notably in patients with Carney's complex disease.

Keywords: adrenal hyperplasia; adrenal tumors; childhood; hypercortisolism; pediatric.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision.

Figures

Figure 1
Figure 1
Symptoms and signs of paediatric adrenal Cushing’s syndrome.
Figure 2
Figure 2
Diagnostic work-up of adrenal Cushing’s syndrome.
See this image and copyright information in PMC

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