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Leka S.,Aglaia Kyriakou Children S Hospital | Kousta E.,Aglaia Kyriakou Children S Hospital | Anyfandakis K.,Aglaia Kyriakou Children S Hospital | Dolianiti M.,Aglaia Kyriakou Children S Hospital | And 4 more authors.
Journal of Pediatric Endocrinology and Metabolism | Year: 2011

Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of Cushing syndrome in children, often occurring in association with Carney complex. We report a case of Cushing syndrome due to isolated non-familial PPNAD. The child presented with typical clinical characteristics, growth retardation and obesity. Liddle's test was positive but micronodular appearance was not evident on CT scan and MRI; selective venous sampling revealed higher cortisol concentrations in the right adrenal vein. The patient underwent a laparoscopic right adrenalectomy. Postoperatively, hypercortisolism signs disappeared but after the second year a slight increase in urinary cortisol was noted and the patient developed osteopenia. Although significant catch-up growth occurred postoperatively, height did not normalize over the next 2 years. When she entered puberty, treatment with a luteinizing-hormone-releasing hormone agonist was initiated and growth hormone was added. Almost 5 years later a left adrenalectomy was also performed. Thereafter, complete disease remission was observed, the patient's growth accelerated and her osteopenia reversed. © 2011 by Walter de Gruyter Berlin New York 2011.

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