120 PRIMARY PIGMENTED NODULAR ADRENOCORTICAL DISEASE. RARE CAUSE OF THE CUSHING SYNDROME

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Primary pigmented nodular adrenocortical disease.

Primary pigmented nodular adrenocortical disease (PPNAD) is a rare adrenal tumour causing corticotrophin-independent Cushing's syndrome. It occurs mainly in children and young adults. The histological examination is characterised by small pigmented micronodules on the adrenal cortex. The diagnosis is most often seen in patients with Carney Complex, but it can also occur in isolation. We report ...

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[Primary pigmented nodular adrenocortical dysplasia. A rare cause of Cushing's syndrome].

HISTORY AND CLINICAL FINDINGS A 29-year-old woman was found to have arterial hypertension (175/115 mm Hg). The 24-hour profile showed no diurnal cortisol variations with normal concentrations and 24-hour urinary cortisol was normal. 14 months later there was definite hypercortisolism with discrete Cushing signs and no amenorrhoea. She also had signs of depression. INVESTIGATIONS Routine labor...

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Cushing’s Syndrome Due To Primary Pigmented Nodular Adrenocortical Disease

A 12.5-year-old girl with diabetes mellitus type 1 presented with stunted growth and an increase in body weight. Also, her blood-sugar levels were difficult to manage. An adrenocorticotropin-(ACTH)-independent form of Cushing's syndrome was diagnosed. During the dexamethasone-suppression test, a paradoxical increase in urinary-free cortisol excretion was observed, which is a clear indication of...

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Primary pigmented nodular adrenocortical disease and Cushing's syndrome.

Primary pigmented nodular adrenocortical disease (PPNAD) is a form of bilateral adrenocortical hyperplasia that is often associated with corticotrophin (ACTH)-independent Cushing's syndrome (CS) and is characterized by small to normal-sized adrenal glands containing multiple small cortical pigmented nodules (1,2). PPNAD may occur in an isolated form or associated with a multiple neoplasia syndr...

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Pigmented Nodular Adrenocortical Disease

Carney complex (CNC) is a multiple endocrine neoplasia (MEN) syndrome associated with other, non-endocrine manifestations such as lentigines, cardiac myxomas and schwannomas. Primary pigmented nodular adrenocortical disease (PPNAD), leading to corticotrophin-independent Cushing’s syndrome is the most frequent endocrine lesion in CNC. The complex has been mapped to 2p16 and 17q22-24, although ad...

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ژورنال

عنوان ژورنال: Pediatric Research

سال: 1985

ISSN: 0031-3998,1530-0447

DOI: 10.1203/00006450-198506000-00140