title: Familial Cushing's syndrome due to nodular adrenocortical dysplasia is an inherited disease of immunological origin.
abstract: Circulating immunoglobulins may be involved in the pathogenesis of familial Cushing's syndrome due to nodular adrenocortical dysplasia. The disease may also be part of a complex including cutaneous and cardiac myxomas, spotty pigmentation of the skin, and other signs of endocrine overactivity. In the present study of two sisters treated by complete adrenalectomy for Cushing's syndrome due to nodular adrenocortical dysplasia, and of their first-degree relatives, no evidence of associated disorders was present. However, the serum of both girls and of their mother contained immunoglobulins capable of stimulating adrenal DNA synthesis and cortisol production in vitro. The study supports the theory that familial Cushing's syndrome due to adrenocortical dysplasia is an inherited disease of immunological origin.
authors.affiliation: Department of Endocrinology, University Hospital, Utrecht, The Netherlands.
authors.forename: F
authors.initials: F
authors.lastname: Teding van Berkhout
authors.forename: R J
authors.initials: RJ
authors.lastname: Croughs
authors.forename: N M
authors.initials: NM
authors.lastname: Wulffraat
authors.forename: H A
authors.initials: HA
authors.lastname: Drexhage
mesh_terms: D000302:Adrenal Cortex / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D000303:Adrenal Cortex Diseases / Q000150:complications* / Q000276:immunology / Q000378:metabolism; D003480:Cushing Syndrome / Q000209:etiology* / Q000235:genetics / Q000276:immunology; D004247:DNA / Q000096:biosynthesis; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism; D008297:Male; D012712:Serum Globulins / Q000378:metabolism / Q000494:pharmacology
chemical_list: D012712:Serum Globulins; D004247:DNA; D006854:Hydrocortisone