Treatment of pituitary-dependent Cushing's syndrome: long-term results of unilateral adrenalectomy followed by external pituitary irradiation compared to transsphenoidal pituitary surgery.

Background: The preferred treatment of Cushing's disease (CD) nowadays is transsphenoidal pituitary surgery (TPS). Prior to TPS, patients at the Leiden University Medical Centre were treated by unilateral adrenalectomy followed by external pituitary irradiation (UAPI). We report on long-term results of both UAPI and TPS and compare remission, relapse rates, and complications.

Long-term results of total adrenalectomy for Cushing's disease.

The objective of this study was to present the long-term results of total adrenalectomy for Cushing's disease. Forty-four patients undergoing total adrenalectomy for Cushing's disease between 1953 and 1989 at Leiden University Medical Center, The Netherlands, were studied retrospectively. Remission was achieved in 42 patients (95%), with a mean duration of 19 years.

Evaluation of the effects of radiotherapy on macroprolactinomas using the decline rate of serum prolactin levels as a dynamic parameter.

The effect of radiotherapy (24- > 50 Gy) on serum prolactin levels was studied in 28 patients with macroprolactinomas. All had been treated with surgery and 21 had also received bromocriptine interim therapy. Serum prolactin levels decreased in a log-linear fashion with time, both in the patients who never received bromocriptine and those who were treated with this drug.

Molecular basis of congenital adrenal hyperplasia due to 3 beta-hydroxysteroid dehydrogenase deficiency.

Congenital adrenal hyperplasia is the most frequent cause of adrenal insufficiency and ambiguous genitalia in newborn children. In contrast to congenital adrenal hyperplasia due to 21-hydroxylase and 11 beta-hydroxylase deficiencies, which impair steroid formation in the adrenal cortex, exclusively, classical 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD) deficiency affects steroid biosynthesis in the gonads as well as in the adrenals. The structures of the highly homologous type I and II 3 beta-HSD genes have been analyzed in three male pseudohermaphrodite 3 beta-HSD deficient patients from unrelated families in order to elucidate the molecular basis of classical 3 beta-HSD deficiency from patients exhibiting various degrees of severity of salt losing.

Semiquantitative assessment of hirsutism in Dutch women.

Many doctors frequently encounter hirsute patients. Quantification of hair growth may be useful for diagnosis and follow-up. To establish the reference range for distribution and density of hair in females, and to determine the regions yielding the best discrimination between normal and hirsute women, we studied the distribution and density of terminal hair on 12 body regions assessed on a scale of 0-4.

Relationship between hunger and plasma cholecystokinin during weight reduction with a very low calorie diet.

Based on a large number of studies in various species, cholecystokinin (CCK) is considered to function as an important regulator of satiety. The present study was undertaken to determine the relationship between hunger score and basal and postprandial plasma CCK secretion, in six obese subjects before and after weight loss with modified fasting. Modified fasting (modifast, 240 kcal/day) for ten weeks induced a mean weight loss of 23 kg.

Expression of P-glycoprotein in relation to clinical manifestation, treatment and prognosis of adrenocortical cancer.

The presence of P-glycoprotein, associated with multiple drug resistance and present in the normal adrenal cortex, was studied in 15 cases of adrenocortical carcinoma. P-glycoprotein was found in eight tumours; no correlation was found with clinical presentation, steroid production or histological index. 10 patients received mitotane.

A case of recurrent adrenocortical carcinoma, with observations on long-term o,p'-DDD therapy and complications.

This report describes a patient with a recurring, one stemline-aneuploid, adrenocortical carcinoma. The condition showed a number of unusual characteristics over a period of 22 yr. It changed from a biochemically functioning, low-grade malignant tumour into a non-functioning malignancy with pronounced mitotic activity, accompanied by an ovarian carcinosarcoma 1 yr before death.

Adult adrenoleukodystrophy: the clinical spectrum in a large Dutch family.

A large family with adrenoleukodystrophy is described and the case histories of two clinically symptomatic and related male patients are presented. Clinical, biochemical and genetic screening of their family demonstrated two clinically affected males, one biochemically affected male and five carrier females. Two women were symptomatic; one suffered an acute exacerbation.

The distribution of o,p'-DDD (mitotane) among serum lipoproteins in normo- and hypertriglyceridemia.

We found that the distribution of the lipophilic chemotherapeutic agent o,p'-DDD (mitotane) among serum (lipo)proteins was altered in hypertriglyceridemia, with relatively more o,p'-DDD accumulating in the chylomicron and very-low-density lipoprotein (VLDL) fraction. Intralipid, an artificial chylomicron emulsion, or isolated VLDL could extract o,p'-DDD from the other serum (lipo)proteins. There was an inverse relationship between the relative amount of o,p'-DDD found in the fraction exhibiting a density of less than 1.

Mitotane increases the blood levels of hormone-binding proteins.

In 3 patients with adrenocortical carcinoma the effects of long-term mitotane therapy on the serum levels of three hormone-binding globulins and vitamin D-binding protein were studied. Within the first month of treatment cortisol-binding globulin increased two to three times, in close correlation with sex hormone-binding globulin. The rises in thyroxine-binding globulin and vitamin D-binding protein were considerably less.

Prolonged bleeding time due to mitotane therapy.

After finding prolonged bleeding times in 2 patients treated with mitotane, we prospectively studied 7 patients with adrenocortical cancer on mitotane therapy. Before and 1 and 2 or more weeks after starting mitotane we determined the platelet counts, bleeding times and global coagulation parameters. All patients had a normal bleeding time before treatment.

Large pituitary adenomas with extension into the nasopharynx. Report of three cases with a review of the literature.

Three cases of pituitary adenoma with extension into the nasopharynx and nasal cavity are reported. The occurrence of this rare tumor underscores the need to consider a pituitary tumor whenever a patient presents with rhinologic complaints and destruction of the sellar floor. Epistaxis, although exceptional, may be the first manifestation of a pituitary tumor.

Bromocriptine therapy for non-functioning pituitary adenoma.

Over a 6-year period, 25 patients with non-functioning pituitary adenomas were treated with bromocriptine, while 18 other patients with this condition underwent surgery as the first therapeutic modality. The medically treated group, consisting of those patients without alarming eye symptoms, was analysed in retrospect. Eight of these patients had previously undergone surgery and radiotherapy.

Plasma growth hormone profiles and sleep: a study of 13 treated acromegalics.

The plasma growth hormone profiles and sleep patterns of 13 treated male acromegalic patients, aged 29-67 years, were studied. All patients had undergone selective pituitary transsphenoidal surgery 4-9 years previously; five patients had also undergone supplemental pituitary irradiation. Blood samples were taken at 20-min intervals; the sleep patterns were obtained by EEG.

Bromocriptine-induced trigeminal neuralgia attacks in a patient with a pituitary tumor.

We describe a patient who suffered from ophthalmic trigeminal neuralgia as an isolated symptom of a noninvasive pituitary tumor. Bromocriptine appeared to provoke the attacks within hours. This provocation could be prevented by domperidone.

Mutually gratifying heterosexual relationship with micropenis of husband.

Three adult patients with micropenis are described: two genetic females, reared as boys, with congenital adrenocortical hyperplasia (CAH), and one male with anorchia. The patients had a male gender identity/role. All three had established a satisfying heterosexual relationship.

Magnetic resonance imaging of the adrenal glands.

Although CT should be used as the initial procedure, MRI potentially can identify most adrenal masses without the hazard of ionizing radiation or the injection of iodinated contrast material.

Computed tomography in untreated adults with virilizing congenital adrenal cortical hyperplasia.

Thirteen adult patients with biochemically proven congenital adrenal hyperplasia (CAH) were examined by computed tomography (CT). Six patients had never received glucocorticoid therapy. In three of those six patients, CT revealed a tumorous transformation in one of the hyperplastic adrenal glands.