1,721,016 research outputs found
Hypophysitis and Granulomatous Pituitary Lesions in Systemic Diseases
No full textIn clinical practice, expansive sellar lesions are largely represented by primary diseases of the pituitary gland, mostly pituitary tumors, which can be managed by endocrinologists and/or multidisciplinary pituitary teams. Hypophysitis has long been considered as a rare disease, and is classified on the basis of pathological findings, and as primary or secondary. Nowadays, an enlarging spectrum of pituitary and/or hypothalamic inflammatory, granulomatous, or even neoplastic disorders can be recognized, which may be part of systemic conditions and require appropriate multidisciplinary management involving a variety of specialists. For example, hypophysitis is being increasingly recognized in the setting of systemic IgG4-related disorders or as side effects of immune checkpoints inhibitors in oncological patients. Lymphocytic hypophysitis may sometimes be part of autoimmune polyendocrine syndromes. Significant knowledge has been added in the last decade in the pathogenesis and management of histiocytic disorders, which may infiltrate the pituitary and/or the hypothalamus Infective causes of pituitary inflammation should not be underestimated and are increasingly recognized in immunocompromised patients. Based on updated literature on these topics, this chapter aims to place pituitary inflammatory, granulomatous, and histiocytic disorders in their systemic context and stimulate multidisciplinary collaboration in order to optimize their diagnosis and clinical management
Carney Complex in clinical practice
No full textCarney complex (CNC) is a rare autosomal dominantly inherited syndrome with near-to-complete penetrance characterized by multiple endocrine and nonendocrine neoplasia. Morbidities are mainly associated with cardiac myxoma and hormonal overactivity.
Although genetically heterogeneous, most CNC cases are a consequence of germline inactivating mutations in the gene encoding the cAMP-dependent protein kinase A type I-alpha regulatory subunit (PRKAR1A).
Here, we present the case of a patient with Cushing’s syndrome due to primary pigmented nodular adrenocortical disease (PPNAD) and an asymptomatic IGF-1 and GH excess, with a positive family history for adrenal cortisol-secreting adenoma. DNA mutation analysis revealed a heterozygous splice site mutation in PRKAR1A with possible deleterious effects on protein function.
After medical treatment with steroidogenesis inhibitors, left monolateral laparoscopic adrenalectomy was performed, achieving a recovery of cortisol-related signs and symptoms. After substitutive glucocorticoid discontinuation, the pituitary-adrenal function was consistent with autonomous cortisol secretion without overt hypercortisolism. Treatment with somatostatin analogues was efficacious in normalizing IGF-1 levels
Prevalence of hypertension in acromegalic patients: clinical measurement versus 24-hour ambulatory blood pressure monitoring.
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Recurrence of GH-secreting pituitary adenomas during puberty in children with germline AIP mutations: a clinical challenge
No full textHPLC determination of urinary catecholamines as a screening test for pheochromocytoma in a patient with medullary carcinoma of the thyroid. Gi
No full textLong-term follow-up results of postoperative radiation therapy for Cushing disease.
No full textAbstract Objectives Radiotherapy is currently used in patients with residual or recurrent pituitary adenomas after surgery. However, there is little information of longterm outcome of patients with Cushing’s disease following radiotherapy. We assessed the long-term efficacy and toxicity of conventional radiotherapy in the control of Cushing’s disease after unsuccessful transsphenoidal surgery. Patients and Methods Forty patients with Cushing’s disease were treated with conventional external beam radiotherapy at our Institution between 1988 and 2002. The median age was 38. All patients received radiotherapy following unsuccessful surgery or at tumour recurrence to a dose of 45–50 Gy in 25–28 fractions. The persistence of active disease after surgery was diagnosed by the increased high plasma cortisol levels, high 24 h urinary cortisol levels and absence of cortisol suppression after administration of dexamethasone. Results The 5 and 10 year local tumour control was 93% and the 5 and 10 year survival was 97 and 95%. Normalization of plasma cortisol was seen in 28% of patients at 1 year, 73% at 3 years, 78% at 5 years and 84% at 10 years. The average timing to remission was 24 months. The most common side effect was hypopituitarism that increased progressively during the follow- up, being present in 62% and in 76% of patients at 5 and 10 years after RT. There were no other serious complications as radiation induced optic neuropathy or second tumours. Conclusion Radiotherapy is effective in the long-term tumour- and hormone hypersecretion control of ACTHsecreting pituitary adenomas, however with a high prevalence of hypopituitarism. At the moment, it remains an important treatment option after failure of surgery. Keywords Radiotherapy ACTH-secreting pituitary adenoma Cushing’s disease Hypopitu
Fenofibrate has differential effects on cell proliferation and GH secretion in GH3 cells
No full textEvaluation of the adequacy of levothyroxine replacement therapy in patients with central hypothyroidism.
No full textAs there are few data on the evaluation of the adequacy of levothyroxine (L-T4) therapy in patients with central hypothyroidism (CH), a prospective study was performed to assess the accuracy of various parameters in the follow-up of 37 CH patients. Total and free thyroid hormones, TSH, and a series of clinical and biochemical indexes of peripheral thyroid hormone action have been evaluated off and on L-T4 therapy. Samples were taken before the daily administration of L-T4. In all patients off therapy, clinical hypothyroidism and low levels of free T4 (FT4) were observed, whereas values of FT3, total T4, and total T3 were below the normal range in 73%, 57%, and 19% of cases, respectively. Most of the indexes of thyroid hormone action were significantly modified after L-T4 withdrawal and exhibited significant correlation with free thyroid hormone levels. During L-T4 replacement therapy, 32 patients had circulating levels of FT4 and FT3 and indexes within the normal range with a mean L-T4 daily dose of 1.5 +/- 0.3 microg/kg BW. Despite normal serum FT4, 3 patients had borderline high values of FT3 and a clear elevation of serum-soluble interleukin-2 receptor concentrations, suggesting overtreatment. Low or borderline low FT4/FT3 levels indicated undertreatment in 2 patients. The clinical parameters lack the required specificity for the diagnosis or follow-up of CH patients. The L-T4 daily dose should be established, taking into account the weight, the age, and the presence of other hormone deficiencies or pharmacological treatment of CH patients. In conclusion, our results indicate that the diagnosis of CH is reached at best by measuring TSH and FT4 concentrations. In the evaluation of the adequacy of L-T4 replacement therapy, both FT4 and FT3 serum levels together with some biochemical indexes of thyroid hormone action are all necessary to a more accurate disclosure of over- or undertreated patients
Effects of 17-beta estradiolo, progesterone and Tamoxifen on in vitro proliferation of human pituitary adenomas: correlation with specific cellular receptors
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