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  • Title: Primary pigmented nodular adrenocortical disease: a case report in a 7-year-old girl.
    Author: Leka S, Kousta E, Anyfandakis K, Dolianiti M, Vakaki M, Linos D, Chrousos GP, Papathanasiou A.
    Journal: J Pediatr Endocrinol Metab; 2011; 24(3-4):197-202. PubMed ID: 21648292.
    Abstract:
    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.
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