Primary pigmented nodular adrenocortical disease: a case report in a 7-year-old girl.
J Pediatr Endocrinol Metab
; 24(3-4): 197-202, 2011.
Article
em En
| MEDLINE
| ID: mdl-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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Base de dados:
MEDLINE
Assunto principal:
Doenças do Córtex Suprarrenal
/
Síndrome de Cushing
Limite:
Child
/
Female
/
Humans
Idioma:
En
Ano de publicação:
2011
Tipo de documento:
Article