title: Causes and consequences of abandoning one-stage bilateral adrenalectomy recommended in primary pigmented nodular adrenocortical disease--case presentation.
abstract: We present a 7-year-old girl with a 2-year history of decelerated growth rate and cushingoidal obesity, upon admission presenting with fixed hypertension. Cyclic hypercortisolemia with inhibited baseline and post-CRH stimulation ACTH level pointed to primary adrenal hypercortisolemia. Ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) showed normal adrenal glands. 131J-labeled cholesterol scintiscan showed a weak but slightly more expressed tracer uptake in the left adrenal gland. Cushing syndrome concomitant with isolated primary pigmented nodular adrenocortical disease (PPNAD) was diagnosed. After hypotensive pretreatment, a left adrenalectomy was performed, resulting in normalization of corticoadrenal function, blood pressure, Cushing features and growth rate. Histopathology confirmed PPNAD. In the course of infection, corticoadrenal function showed absence of adrenal reserve, and adrenal crisis. Hydrocortisone (HC) therapy, followed by HC supplementation was introduced. Four years later, a contralateral adrenalectomy was performed and total HC supplementation was introduced. Causes and consequences of abandoning one-stage bilateral adrenalectomy recommended in PPNAD are reviewed.
authors.affiliation: Department of Pediatric and Adolescent Endocrinology, Polish-American Children's Hospital, Collegium Medicum, Jagiellonian University, Krakow, Poland. endodim@cm-uj.krakow.pl
authors.forename: Malgorzata
authors.initials: M
authors.lastname: Kumorowicz-Czoch
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Dolezal-Oltarzewska
authors.forename: Dorota
authors.initials: D
authors.lastname: Roztoczynska
authors.forename: Malgorzata
authors.initials: M
authors.lastname: Chrupek
authors.forename: Andrzej Igor
authors.initials: AI
authors.lastname: Prokurat
authors.forename: Grazyna
authors.initials: G
authors.lastname: Drabik
authors.forename: Jerzy
authors.initials: J
authors.lastname: Starzyk
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000503:physiopathology / Q000601:surgery*; D000315:Adrenalectomy / Q000009:adverse effects*; D002648:Child; D003480:Cushing Syndrome / Q000188:drug therapy / Q000209:etiology; D005260:Female; D006130:Growth Disorders / Q000209:etiology; D020249:Hormone Replacement Therapy; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D010858:Pigmentation; D017410:Practice Guidelines as Topic; D012086:Reoperation / Q000009:adverse effects*; D016896:Treatment Outcome
chemical_list: D006854:Hydrocortisone