Familial Cushing's syndrome due to primary pigmented nodular adrenocortical disease: reinvestigation 50 years later

Article Abstract:

The family of a young woman treated in 1939 for irregularities of her adrenal gland is reinvestigated. She was seen when, at the age of 13, she stopped growing, had an excessive weight gain and developed hirsutism, becoming excessively hairy. She had only an initial menstrual cycle at the age of 16, and was finally admitted to a hospital at the age of 17. At that time she was hirsute, had a full round face (moon face) and a conspicuous fatty deposit, a buffalo-hump, on her upper back. Freckles covered her face, neck, and shoulders. The overall appearance was of Cushing's syndrome, which results from excessive adrenal hormone production. Because of this suspicion, an exploratory operation was carried out. The adrenal glands were found to be excessively small and had an unusual appearance. Following surgery the patient began to have irregular menstruation, and she ultimately had two children. Following the delivery of her second child she lost her hirsutism and her excess body weight. When re-examined in 1988 she retained none of the symptoms of Cushing's syndrome. The patient's family tree is documented and her younger daughter shows signs of the disorder which were confirmed by laboratory findings and surgery in which her adrenal glands were removed. A tumor in the area between the nasal passage and the throat was also identified in the daughter and removed. The elder daughter shows no sign of the condition. The diagnosis in these cases was primary pigmented nodular adrenocortical disease, an hereditary condition that is passed as an autosomal dominant condition to children, meaning that a child of an affected parent of either sex has a fifty-fifty chance of inheriting the condition.

Diagnosis, Genetic aspects, Cushing syndrome, Pigmentation disorders, Endocrine gland diseases, Endocrine diseases, column

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Association of hypokalemia, aldosteronism, and renal cysts

Article Abstract:

Aging is associated with the development of renal cysts, sac-like structures containing fluid or solids in the kidneys. One factor that may contribute to kidney cyst formation is long-term hypokalemia, or abnormally low amounts of potassium. Studies have demonstrated that chronic hypokalemia causes increases in tissue size and cyst development in the kidney. The formation of renal cysts was examined in 55 patients with primary aldosteronism, a condition resulting from disorders in the adrenal gland and characterized by abnormally high levels of the hormone aldosterone, which leads to retention of sodium and increased loss of potassium in the urine. Twenty-four of the 55 patients had cysts, with more frequent cyst development in patients with adrenal tumors than in patients with adrenal hyperplasia, the overgrowth of normal cells. Cyst development in the kidneys was correlated with low blood potassium levels, high aldosterone levels in the blood and urine, and high blood levels of the hormone renin, which is involved in increasing aldosterone levels. Prolonged hypokalemia resulted in scarring of kidney tissue, and removal of the adrenal tumor led to decreased cyst formation. Hypokalemia, aldosteronism, and kidney cyst formation also occurred in two patients with primary renal potassium wasting, the deterioration of the kidney tissue associated with loss of potassium. These results demonstrate that hypokalemia is associated with increased cyst formation in the kidneys. (Consumer Summary produced by Reliance Medical Information, Inc.)

Causes of, Complications and side effects, Physiological aspects, Hyperaldosteronism, Hypokalemia, Kidney, Cystic, Cystic kidney

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The incidentally discovered adrenal mass

Article Abstract:

A case study of a 68-year old woman who was found to have a left adrenal mass, 2.8 cm in diameter, on abdominal computed tomography that was ordered to evaluate right lower abdominal discomfort is reported. The method of evaluating the woman, whose physical examination was unremarkable, is presented.

Case studies, Disease/Disorder overview, Case study

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