Τίτλος:
Primary pigmented nodular adrenocortical disease: A case report in a 7-year-old girl
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
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.
Συγγραφείς:
Leka, S.
Kousta, E.
Anyfandakis, K.
Dolianiti, M.
Vakaki, M.
Linos, D.
Chrousos, G.P.
Papathanasiou, A.
Περιοδικό:
Journal of Pediatric Endocrinology and Metabolism
Λέξεις-κλειδιά:
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
DOI:
10.1515/JPEM.2011.033
