Familial Cushing's syndrome due to nodular adrenocortical dysplasia. A putative receptor-antibody disease?
Two sisters aged 13 and 19 years suffering from familial Cushing's syndrome due to nodular adrenocortical dysplasia are described. Pituitary adrenocortical function tests indicated the presence of adrenal autonomy. Adrenal scintigraphy showed bilateral symmetrical uptake indicating the bilateral character of the autonomous process. Complete adrenalectomy was performed in both girls. The adrenals were of about normal weight showing numerous dark brown pigmented nodules and small perivascular lymphocytic infiltrates. Serum immunoglobulin preparations obtained from both girls stimulated adrenocortical cell growth in a cytochemical bioassay system. It is proposed that circulating growth factors may be involved in the pathogenesis of the disease.
Teding van Berkhout
F
F
Croughs
R J
RJ
Kater
L
L
Schuurman
H J
HJ
Gmelig Meyling
F J
FJ
Kooyman
C D
CD
van der Gaag
R D
RD
Jolink
D
D
Drexhage
H A
HA
D000293:Adolescent; D000303:Adrenal Cortex Diseases / Q000150:complications* / Q000276:immunology / Q000473:pathology; D000311:Adrenal Glands / Q000473:pathology; D000315:Adrenalectomy; D000328:Adult; D003480:Cushing Syndrome / Q000209:etiology / Q000235:genetics* / Q000276:immunology; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D010912:Pituitary-Adrenal Function Tests
D007136:Immunoglobulins