Familial Cushing's Syndrome Due to Primary Pigmented Nodular Adrenocortical Disease

Carney complex, a familial multiple neoplasia and lentiginosis syndrome. Analysis of 11 kindreds and linkage to the short arm of chromosome 2.

“…Close to 200 patients have been reported from diverse ethnic groups, since the initial description of the complex (1,16,17,64,(69)(70)(71)(72). In the late 1980's, investigators reported the presence of "adrenal-stimulating immunoglobulins" leading to corticotropin-independent adrenocortical nodular hyperplasia in affected subjects and suggested that "Carney complex was an inherited disease of immunological origin" (52,73). In the present study of 11 families with Carney complex from the United States and Canada, we found no evidence of autoimmune disease or immune dysfunction.…”

“…Each sample was given a two-digit number that reflected the order of its collection (for example, CAR107.03 represents the third specimen collected from the CAR.107 family). Clinical information for most of these families has been reported before (5,10,16,(47)(48)(49)(50)(51)(52)(53)(54).…”

“…1]), because conclusive information could not be obtained about Figure 1. Pedigrees of the 11 families included in the study (clinical information in 9 of the 11 kindreds was reported previously: CAR01 [47], CAR02 [48], CAR101 [5,49], CAR102 [36,50], CAR103 [51], CAR104 [52], CAR105 [53,54], CAR107 [10], and CAR108 [16]). Open squares and circles represent unaffected male and female patients, respectively.…”

Випадок Хвороби Іценка-Кушинга (Історична Мініатюра)

“…У числі останніх часто траплявся синдром Кушінга на ґрунті двобічної пігментної вузлової гіперплазії кори надниркових залоз. Для цієї форми синдрому Кушінга іноді характерний тривалий хронічний циклічний перебіг зі схильністю до ремісій, патоге-нетично її можна розглядати як первинно наднир-кову [11][12][13][14]. Зацікавленість Карні долею Мінні Г. підштовхнуло також його власне спостереження аналогічного хворого у клініці Мейо [12].…”

An unusual presentation of Carney complex with diffuse primary pigmented nodular adrenocortical disease on one adrenal gland and a nonpigmented adrenocortical adenoma and focal primary pigmented nodular adrenocortical disease on the other

“…The patient's weight was 55 kg and her blood presadrenal glands are involved and feature small brownblack nodules separated by atrophic adrenal cortex. Microscopically, the nodules consist of large cortical cells with eosinophilic cytoplasm and lipofuscin pigment, and internodular cortical atrophy [3,4,6].…”