Static lung volumes in patients with Cushing's disease.

OBJECTIVE: Numerous clinical manifestations have been described in association with Cushing's syndrome. There are no eligible data on pulmonary function tests in Cushing's disease (CD). We aimed to asses pulmonary function tests including spirometry in a series of patients with active CD. MATERIALS AND METHODS: This cross-sectional study comprised 10 patients with Cushing's disease (F/M, 9/1). The forced expiratory volume in 1st second (FEV1), the forced vital capacity (FVC), the FEV1/FVC ratio and the forced expiratory flow over the middle half of the FVC (FEF 25-75%) values and predicted values were determined by spirometry. RESULTS: Mean age, height, weight, body mass index were 36.7+/-12.6 yrs (range 22-63 years), 156.9+/-8.4 cm, 74.1+/-10.7 kg, 29.6+/-3.8 kg/m(2), respectively. Spirometric abnormalities (impairment of FEV1, FVC, FEV1/FVC and FEF 25-75 values) were not detected, and there were no significant differences compared to reference values. Disease duration and cortisol concentrations by HDDSTs were negatively correlated with predicted FEV1/FVC values and the percentage of predicted FEV1 ratios, respectively. DISCUSSION: The lung volume and ventilatory performance by spirometry were not disturbed in patients with endogenous hypercostisolism due to Cushing's disease.

Clinical laboratory findings and results of therapy in 55 patients with Cushing's syndrome.

In this study, 55 patients with Cushing's syndrome (CS) (50 female, 5 male; mean age 34 +/- 12.3 yr) who attended our clinics between the years 1983 and 2000 were retrospectively evaluated for clinical and laboratory features and modalities and results of therapy, due to a few similar studies over the last ten years. Cushing's disease was diagnosed in 39 patients (71%), adrenal adenoma in 13 patients (23.6%) and adrenal carcinoma in 3 patients (5.5%). Centripedal obesity, moon face, hypertension, hirsutism and purplish stria were the most frequent findings. Loss of normal serum F circadian rhythm was found in all patients with CS. The overnight 1 mg oral dexamethasone suppression test and low-dose dexamethasone suppression test (LDDST) yielded 100% and 100% diagnostic sensitivity for CS, respectively. Sensitivity and specivity of the high-dose dexamethasone suppression test (HDDST) in distinguishing Cushing's disease was found to be 82% and 100%, respectively. All of the patients with adrenal CS were not suppressed with HDDST. Sellar CT and/or MRI accurately identified the tumor in 58% of these patients. Recurrence was observed in 3 (11%) of the 28 patients with Cushing's disease, treated by transsphenoidal adenomectomy. Recurrence was diagnosed 1.5, 3 and 6 yr after the operation in these 3 patients. One patient had residue tumor. In our case series, bilateral adrenalectomy plus pituitary irradiation achieved the highest remission rate (100%) in Cushing's disease. In 2 out of 4 patients (50%) treated by left adrenalectomy associated with pituitary irradiation, recurrence was observed. Panhypopituitarism due to tumor apoplexy was observed in one of the patients with Cushing's disease. All of the patients with adrenal CS, the tumor was accurately localized with imaging methods before the operation. The appropriate operative procedure resulted in complete remission in patients with adrenal adenoma. Consequently, Cushing's disease was the most common form of CS. The overnight 1 mg oral DST and 24-h urine free F excretion (UFC) as screening tests, 2-day LDDST as diagnostic test and 2-day HDDST as differential diagnostic test were good studies. More successful outcomes have been achieved in treatment of Cushing's disease with the development of pituitary surgery in the recent years, as well as in our case series. Surgery is also curative for adrenal adenoma patients. Survival remains poor among carcinoma patients.

Relationship between adrenal cortex pathology in Cushing's syndrome and its response to the dexamethasone suppression test.

Cushing's syndrome is a severely disabling condition which can cause death if left untreated. Endogenous Cushing's syndrome can be ACTH-dependent or ACTH-independent. The ACTH-dependent type is more common and is usually caused by diffuse hyperplasia on the adrenal cortex. This study investigated the response to low- and high-dose dexamethasone suppression testing of 30 adrenalectomized patients with Cushing's syndrome, average age 37.3 +/- 9.7 years. Twenty-four (79.3%) patients were female, and six (20.7%) were male. Bilateral adrenalectomy was performed in 14 (48.2%) patients and unilateral adrenalectomy (nine and seven right adrenalectomy) in 16 (51.8%). Two of the bilateral adrenalectomies were applied via endoscopic surgical approach. In the histopathological evaluation, diffuse hyperplasia was diagnosed in 13 (44.8%) patients and nodular hyperplasia in eight (26.6%), three macronodular and five micronodular hyperplasia. Adrenal cell adenoma was diagnosed in nine (28.6%) patients. Classic dexamethasone suppression testing was performed on all patients. Plasma levels of cortisol were not significantly decreased after low-dose testing, but plasma levels of cortisol were significantly decreased after high-dose testing in the diffuse hyperplasia group. In summary, due to the pathological changes of the adrenal cortex, dexamethasone suppression testing can differentiate between the two types of Cushing's syndrome.

Pituitary macroadenoma in Addison's disease.

Long-standing primary failure of pituitary-dependent endocrine glands may lead to hyperplasia of the pituitary cells. These changes in the pituitary gland may be correlated with the severity and duration of target-endocrine gland insufficiency. Production of adrenocorticotrophic hormone by the pituitary tumour and modest hyperprolactinaemia may develop due to adrenocortical insufficiency, but production of prolactin by the pituitary tumour due to primary adrenal insufficiency is rare. A case study is presented, with primary adrenal insufficiency associated with hyperprolactinaemia and pituitary macroadenoma (most probably prolactinoma). Plasma levels of prolactin were found to decrease after glucocorticoid, mineralocorticoid and bromocriptine therapy.

Clinical and laboratory evaluation of 40 patients with Sheehan's syndrome.

Forty patients with typical obstetric history of Sheehan's syndrome were reviewed retrospectively. Together with baseline laboratory values, insulin hypoglycemia test was evaluated in 15 patients, thyrotropin-releasing hormone (TRH) in 27 and luteinizing hormone-releasing hormone (LH-RH) in 7 patients. Baseline hormone values suggested secondary hypothyroidism, hypogonadotropic hypogonadism and hypocortisolemia. According to the results of the anterior pituitary stimulation tests, one patient (6.6%) showed normal cortisol response and one patient (6.6%) showed normal growth hormone response to hypoglycemia. Nine patients (33.3%) who were clinically and biochemically hypothyroid demonstrated adequate TSH response to TRH. None of the patients showed normal prolactin response to TRH. Four out of seven amenorrheic patients (57.1%) had adequate follicle-stimulating hormone and/or LH responses to LH-RH. It has been concluded that isolated anterior pituitary hormone deficiencies may occur in patients with Sheehan's syndrome. Prolactin response to TRH seems the most sensitive screening test for detecting Sheehan's syndrome in patients with typical obstetric history.

Human lymphocyte antigens (HLA) and Graves' disease in Turkey.

To evaluate the association of HLA types with Turkish patients with Graves' disease, HLA typing, clinical findings, and thyroid antibodies were correlated. The HLA types, clinical findings (ophthalmopathy and age at onset), and thyroid stimulating hormone (TSH) receptor (TRAb) and antithyroid microsomal antibodies (MAb) were analyzed. Seventy Turkish patients with Graves' disease and 306 control subjects were assessed. Serological HLA typing was performed in HLA A, B, C, DR, and DQ loci. There was a significantly increased prevalence of HLA B8, B49, DR3, DR4, and DR10 in Graves' disease. The association of Graves' disease with HLA DR3 was found to be less strong than previously described. The HLA DR4 antigen may contribute to the predisposition of Graves' disease in Turkey. The results suggest that HLA B7, B13, DR7, DQw2, and DQw3 may confer a protective effect for Graves' disease in Turkey. Patients carrying HLA B12, B18, and B44 haplotypes had a tendency to develop the disease at a later age. The difference from the other studies may be the result of the selection of the controls; in part, of the variability in serological typing reagents; and, also, of the rather weak HLA associations with the disease.