title: Primary pigmented nodular adrenocortical disease: a case report in a 7-year-old girl.
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.
authors.affiliation: Department of Pediatric Endocrinology, Aglaia Kyriakou Children's Hospital, Athens, Greece.
authors.forename: Sofia
authors.initials: S
authors.lastname: Leka
authors.forename: Eleni
authors.initials: E
authors.lastname: Kousta
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Anyfandakis
authors.forename: Maria
authors.initials: M
authors.lastname: Dolianiti
authors.forename: Marina
authors.initials: M
authors.lastname: Vakaki
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Linos
authors.forename: George P
authors.initials: GP
authors.lastname: Chrousos
authors.forename: Asteroula
authors.initials: A
authors.lastname: Papathanasiou
mesh_terms: D000303:Adrenal Cortex Diseases / Q000097:blood / Q000473:pathology* / Q000628:therapy; D000315:Adrenalectomy; D001851:Bone Diseases, Metabolic / Q000188:drug therapy; D002648:Child; D003480:Cushing Syndrome / Q000097:blood / Q000473:pathology* / Q000628:therapy; D005260:Female; D007987:Gonadotropin-Releasing Hormone / Q000819:agonists; D013006:Growth Hormone / Q000627:therapeutic use; D020249:Hormone Replacement Therapy; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D017495:Hyperpigmentation / Q000473:pathology; D012074:Remission Induction
chemical_list: D007987:Gonadotropin-Releasing Hormone; D013006:Growth Hormone; D006854:Hydrocortisone