@article{3090677,
    title = "Primary pigmented nodular adrenocortical disease: A case report in a 7-year-old girl",
    author = "Leka, S. and Kousta, E. and Anyfandakis, K. and Dolianiti, M. and Vakaki, M. and Linos, D. and Chrousos, G.P. and Papathanasiou, A.",
    journal = "Journal of Pediatric Endocrinology and Metabolism",
    year = "2011",
    volume = "24",
    number = "3-4",
    pages = "197-202",
    doi = "10.1515/JPEM.2011.033",
    keywords = "dexamethasone;  fludrocortisone;  hydrocortisone, adrenal disease;  adrenalectomy;  article;  case report;  child;  child growth;  computer assisted tomography;  Cushing syndrome;  female;  histopathology;  human;  hypercortisolism;  laboratory test;  laparoscopic surgery;  low drug dose;  nuclear magnetic resonance imaging;  osteopenia;  physical examination;  primary pigmented nodular adrenocortical disease;  school child, Adrenal Cortex Diseases;  Adrenalectomy;  Bone Diseases, Metabolic;  Child;  Cushing Syndrome;  Female;  Gonadotropin-Releasing Hormone;  Growth Hormone;  Hormone Replacement Therapy;  Humans;  Hydrocortisone;  Hyperpigmentation;  Remission Induction",
    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. © 2011 by Walter de Gruyter Berlin New York 2011."
}