[Trans-sphenoidal surgery of pituitary adenomas and other forms of pituitary pathology].
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Biomedical subjects
Publications and source records attributed to B Merola.
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Twenty-six consecutive patients with ACTH-dependent Cushing syndrome were subjected to simultaneous, bilateral inferior petrosal sinus sampling for ACTH assay before and after ACTH-releasing hormone (CRH) stimulation. The baseline ACTH inferior petrosal sinus/periphery (IPS/P) ratio was > or = 2 in 12 of 26 patients (46%), whereas the CRH-stimulated IPS/P ratio was > or = 3 in 19 of 26 patients (73%). A pituitary adenoma, ACTH-secreting at immunostaining, was surgically proved in all of the 19 patients who had an ACTH IPS/P ratio > or = 2 basally or > or = 3 after the CRH test but also in three other patients who did not have such ratios. The value of the basal IPS/P ratio and the complete lack of ACTH increase after CRH led to the diagnosis of an ectopic ACTH syndrome in four patients: a bronchial carcinoid was found in three patients, and the site of the tumor was still unknown in the other. In conclusion, the CRH test improved the diagnostic accuracy of inferior petrosal sinus sampling from 61.5% (12 pituitary, 4 ectopic) to 92.0% (19 pituitary, 4 ectopic). Thus it should be performed during the diagnostic process.
The aim of the current study was to evaluate the effectiveness of a long-term treatment with sodium valproate in 19 patients with Cushing's disease. Before therapy beginning, the patients were subjected to acute test with 600 mg sodium valproate. Then, they were subjected to a 3-month therapy with sodium valproate at the dose of 600 mg/day before surgery (presurgical study). The 7 patients not surgically cured were subjected again to a 3-month therapy with sodium valproate at the dose of 600 mg/day after surgery (postsurgical study). Circulating ACTH and cortisol and urinary free cortisol levels were evaluated before and monthly after the beginning of the therapy. A decrease of plasma ACTH and serum cortisol levels greater than 50% of baseline was considered as positive response to acute test whereas the normalization of plasma ACTH, serum cortisol and urinary free cortisol levels and the clinical remission were considered as positive response to the long-term treatment. At acute test, 8 patients were considered responders and 11 patients non-responders. In no patient plasma ACTH, serum cortisol and urinary free cortisol were normalized during the long-term treatment. Urinary free cortisol levels significantly decreased (483.2 +/- 33.8 vs 699.4 +/- 67.0 micrograms/24 h), whereas plasma ACTH (302.8 +/- 17.7 vs 183.3 +/- 25.0 ng/l) and serum cortisol (466.5 +/- 23.2 vs 356.7 +/- 19.6 micrograms/l) significantly increased during sodium valproate administration in the 19 patients enrolled in the presurgical study. Plasma ACTH (247.7 +/- 22.3 vs 168.6 +/- 15.0 ng/l), serum cortisol (387.4 +/- 35.8 vs 282.0 +/- 16.0 micrograms/l) and urinary free cortisol (370.9 +/- 70.6 vs 261.3 +/- 37.8 micrograms/24 h) levels significantly increased in the 7 patients enrolled in the postsurgical study. No patient had clinical remission of Cushing's disease. In conclusion, the current study showed that long-term therapy with sodium valproate is not useful in the therapeutic management of Cushing's disease neither as alternative nor as adjunctive therapy to surgery.
It is known that women suffering from temporal lobe epilepsy may frequently present reproductive endocrine disorders (REDs). We hypothesized that a high occurrence of REDs could be found also in primary generalized epilepsy (PGE), and therefore investigated the hormonal and ovarian echographic profiles in 20 PGE female patients of reproductive age. Fourteen reported normal menstrual cycles, while 6 complained of longstanding menstrual irregularities. All but three patients were receiving antiepileptic drug (AED) therapy. In all subjects, the basal levels of gonadotropins, prolactin, and gonadal steroids were assayed. The response of luteinizing hormone (LH) to gonadotropin-releasing hormone was also investigated and ovarian ultrasonographic findings were evaluated. In five of six patients with menstrual problems (25% of the group), a well-defined RED was diagnosed (polycystic ovarian disease in three cases and hypothalamic ovarian failure in two). The 14 patients with normal menstrual cycles showed an elevation of mean basal follicle-stimulating hormone and prolactin, and a blunting of mean LH response. Our results suggest that a high occurrence of REDs may be found also in PGE. We hypothesize that a neurotransmitter dysfunction might be the common pathogenetic mechanism resulting in both REDs and PGE. The hormonal alterations observed in the patients with normal menstrual cycles seem to support our hypothesis. Previous data seem to rule out a possible AED effect accounting for the hormonal findings observed in our series. However, further studies are needed to confirm our preliminary results.
In recent years it has been demonstrated that both GH deficiency and excess include in their advanced clinical manifestations an impaired cardiovascular function, which may reduce life expectancy. This observation has allowed the investigation of the role played by the GH/IGF-I axis on cardiac structure and function. In particular, several recent experimental and clinical studies support the evidence implicating GH and/or IGF-I in the regulation of heart development. Acromegalic cardiomyopathy is characterized by myocardial hypertrophy with interstitial fibrosis, lymphomononuclear infiltration and areas of monocyte necrosis which often result in increased right and left ventricular mass and concentric hypertrophy. Conversely, patients with childhood or adulthood-onset GH deficiency (GHD) have a reduced left ventricular mass and ejection fraction and the indexes of left ventricular systolic function remained markedly depressed during exercise. In addition, a significant increase in the thickness of the vascular intima-media wall and a higher number of atheromatous plaques have been reported. These abnormalities of the cardiovascular system are partially reversed after normalization of GH and IGF-I levels, by octreotide in acromegaly or after GH replacement therapy in GHD patients. The evidence that GH is able to increase cardiac mass suggested its use in the treatment of idiopathic dilated cardiomyopathy. In a recent study on such patients, the administration of rhGH was demonstrated to increase myocardial mass and to reduce the size of the left ventricular chamber, resulting in an improvement in hemodynamics, myocardial energy metabolism and clinical status. These promising results might open a new field for GH treatment.
A comparative study of 90 post-menopausal osteoporotic women, has been carried out. The patients were divided in 3 homogeneous groups. According to 3 different schemes: Group A = 30 patients received carbocalcitonin i.m. 40 UMRC/day/1 month and 40 UMRC/alternating days/2 months followed by 1 month of wash-out for 11 months; Group B = 30 patients received carbolcitonin nasal-spray 80 UMRC/day/1 month and 80 UMRC/alternating day/2 months followed by 1 month of wash-out for 11 months; Group C = 30 patients received ipriflavone x os (600 mg/day/3 months followed by 1 month of wash-out for 3 times). BMC significantly was increased in all Groups after 7 and 11 months. In conclusion, carbocalcitonin and ipriflavone seem to show a similar improving densitometric effect in post-menopausal osteoporosis.
The beta-endorphin-like-immunoreactivity (beta-ELI) has been evaluated both in plasma and in cerebrospinal fluid (CSF) in 30 patients during trans-sphenoidal surgery. Blood and liquoral samples were collected in five conditions: (1) "reference", (2) "pain", (3) "analgesia", (4) "end", and (5) "24th hour". A significant rise of both plasma and liquoral beta-ELI levels (p less than 0.00001 and p less than 0.08, respectively) when compared to basal ones occurred following the painful stimulation due to the divarication of the nasal mucosa by speculum. A significant decrease (p less than 0.01) was noticed for plasma concentrations at the third sample followed by a new significant increase at the end of the operation, (p less than 0.05 when compared to the third sample and p less than 0.01 when compared to the reference sample). In CSF, beta-ELI levels decreased at the third sample (p less than 0.01 when compared to the painful levels) and at the end of surgery (p less than 0.01, p less than 0.01 and p less than 0.05 vs first, second and third samples, respectively). Twenty-four hours after surgery either plasma and liquoral beta-ELI levels decreased (p less than 0.05). The modifications of the opiatergic system after acute painful stimuli should be, hence, characterized by an early rise followed by a progressive decrease of beta-ELI concentrations. The increase of plasma beta-ELI levels, at the end of surgery, could be due to pituitary manipulation with massive release in the peripheral blood.
We describe the association of cerebellar ataxia and hypogonadism in four female patients. Clinical, neurophysiological, neuroradiological and endocrinological features are reported. Post-mortem findings in one case are also described. We show in our patients that, beside the cerebellum, both the supratentorial white matter and the peripheral nerve are involved. We suggest that this association can be considered as a "multiple system atrophy". Furthermore, since hypogonadism is hypergonadotropic in three patients and hypogonadotropic in one, the endocrine pattern appears to be heterogeneous.
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The relationship between growth hormone deficiency (GHd) and ventilatory function is not well understood. We studied 7 patients with GHd since childhood who had been adequately treated with replacement therapy until cartilage fusion. Together with 7 well-matched (age and body-type) healthy control subjects, they underwent spirometry including determination of residual volume, and lung diffusing capacity. Also recorded were maximal respiratory muscle pressure during inspiration (PImax) and expiration (PEmax). Patients with GHd showed a significant reduction in total lung capacity and vital capacity while residual volume and lung diffusing capacity remained unchanged. All patients had a significant reduction of both PImax and PEmax. Previously treated adult subjects with GHd present a persistent decrease in lung mobilizing volumes associated with reduced respiratory muscle strength. These alterations may have implications in the management of GHd in adult patients.