title: Primary pigmented nodular adrenocortical disease: literature review and case report of a 6-year-old boy.
abstract: Cushing's syndrome is rare in childhood and is usually caused by a pituitary adenoma. Primary hyperfunction of adrenal glands is less frequent, particularly primary pigmented nodular adrenocortical disease (PPNAD). It occurs usually in children and adolescents, with female preponderance, while Cushing's disease has increased frequency in prepubertal males. A case of a 6-year-old boy is presented with isolated non-familiar PPNAD. The clinical pattern involved Cushingoid appearance, hypertension, virilization and depressive mood. Laboratory analyses showed loss of circadian rhythm of cortisol, undetectable adrenocorticotropic hormone (ACTH) level, impaired fasting glucose, polycythemia and elevated white blood count (WBC). Radiology investigation revealed a slightly enlarged medial branch of the left adrenal gland and a normal right one, so a unilateral adrenalectomy was performed. Pathohistology described multiple dark brownish pigmented nodules of various sizes confined to the cortex. Contralateral adrenalectomy was done 3 months later. Follow-up of 3 years was uneventful, except for one adrenal crisis during an intercurrent respiratory illness.
authors.affiliation: Paediatrician-Endocrinologist, Faculty of Medicine, University Paediatric Clinic, Novi Sad.
authors.forename: Dragan
authors.initials: D
authors.lastname: Katanić
authors.affiliation: University of Novi Sad, Faculty of Medicine, Novi Sad.
authors.forename: Dejan
authors.initials: D
authors.lastname: Kafka
authors.affiliation: University of Novi Sad, Faculty of Medicine, Novi Sad.
authors.forename: Mirjana
authors.initials: M
authors.lastname: Živojinov
authors.affiliation: University of Novi Sad, Faculty of Medicine, Novi Sad.
authors.forename: Jovan
authors.initials: J
authors.lastname: Vlaški
authors.affiliation: University of Novi Sad, Faculty of Medicine, Novi Sad.
authors.forename: Zorana
authors.initials: Z
authors.lastname: Budakov
authors.affiliation: University of Novi Sad, Faculty of Medicine, Novi Sad.
authors.forename: Marija
authors.initials: M
authors.lastname: Knežević Pogančev
authors.affiliation: University of Novi Sad, Faculty of Medicine, Novi Sad.
authors.forename: Ivana
authors.initials: I
authors.lastname: Vorgučin
authors.affiliation: University of Novi Sad, Faculty of Medicine, Novi Sad.
authors.forename: Tomislav
authors.initials: T
authors.lastname: Ćuk
mesh_terms: D000303:Adrenal Cortex Diseases / Q000473:pathology* / Q000601:surgery; D000315:Adrenalectomy; D002648:Child; D003480:Cushing Syndrome / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008297:Male; D010859:Pigmentation Disorders / Q000473:pathology* / Q000601:surgery; D011379:Prognosis
keywords: Cushing’s; adrenal; child; primary pigmented nodular adrenocortical disease (PPNAD)