Pituitary最新文献

Purpose: A recent update of consensus guidelines for the management of Cushing's disease (CD) included indications for medical therapy. However, there is limited evidence regarding their implementation in clinical practice. This study aimed to evaluate current medical therapy approaches by expert pituitary centers through an audit conducted to validate the criteria of Pituitary Tumors Centers of Excellence (PTCOEs) and provide an initial standard of medical care for CD.

Methods: Based on the activities of nine international PTCOEs between 2018 and 2020, we evaluated patients under medical treatment and their biochemical control rates.

Results: The median number of active patients with CD per center was 117 (35-279), with a median number of 10 new patients with CD managed annually in the endocrinology units of PTCOEs (4-42). The median percentage of patients with CD receiving medical treatment was 13.3% (4.8-82.9). Ketoconazole was the most frequently used drug, with a median rate of usage of 26.5% (5-66.7) of those receiving medical therapy. The median rates of metyrapone and pasireotide use were 17.2% (0-50) and 9.3% (0-51.7), respectively. For cabergoline and osilodrostat, therapy, the median rates of use were 2.8% (0-33.3), and 1.7% (0-25), respectively. Combination therapy was reported to be utilized in 13.6% (0-45.5) of medically treated patients. Mifepristone was used in a single center, representing 1.1% of its medically treated patients. Overall, the median control rate in patients with CD receiving medical treatment was 75% (10-100).

Conclusion: Adrenal steroidogenesis inhibitors were the most commonly used medications amongst the centers. Despite the use of combination therapy, up to 25% of patients did not achieve disease control even in PTCOEs, highlighting the need for either more efficient combination therapies or novel therapeutic options.

Sheehan syndrome (SS) is postpartum pituitary necrosis leading to severe hypopituitarism. Severe bleeding during delivery and postpartum period results in ischemic necrosis of the enlarged pituitary gland during pregnancy. The improved obstetrical care decreased the incidence of SS significantly, however SS should always be kept in mind in the etiologies of hypopitutarism in women which can be easily recognized by medical history of the patient. The nonspecific signs and symptoms of hypopituitarism result in significant delay in diagnosis and treatment. The diagnostic delay makes the patients to expose hypopituitarism without essential replacement therapies leading to increased morbidity and mortality of the patients. Awareness of physicians about SS is critical for the diagnosis of the disease. In this review, the epidemiology, pathophysiology, clinical manifestations and treatment of SS are discussed in the light of recent studies.

Purpose: Pituitary adenomas, despite their histologically benign nature, can severely impact patients' quality of life due to hormone hypersecretion. Invasion of the medial wall of the cavernous sinus (MWCS) by these tumors complicates surgical outcomes, lowering biochemical remission rates and increasing recurrence. This study aims to share our institutional experience with the selective resection of the MWCS in endoscopic pituitary surgery.

Methods: This prospective study included patients diagnosed with pituitary adenomas who underwent endoscopic endonasal surgery at Mayo Clinic, Jacksonville. Inclusion criteria encompassed confirmed pituitary adenomas, selective MWCS resection, and availability of histopathological data. Patient demographics, tumor characteristics, surgical outcomes, and postoperative complications were analyzed using descriptive statistics.

Results: Twenty-six cases met the inclusion criteria. Functional adenomas and macroadenomas constituted 80.8% of cases. Recurrent adenomas represented 19.2% of cases. Tumor invasion of the MWCS was confirmed in 76.9% of cases and patients with acromegaly had the highest rate of confirmed invasion of the MW (88.8%). Initial disease control was achieved in 88.5% of the patients, and disease remission at last follow up was observed in those with acromegaly, Cushing's disease and prolactinomas in 87.5%, 83.3% and 66.6% of cases, respectively. Complete resection was achieved in 5/5 nonfunctioning adenomas. There were no carotid injuries, cranial deficits or fatalities observed.

Conclusion: Resection of the MWCS is an effective strategy for improving surgical outcomes in pituitary adenomas with potential invasion into this area, especially in patients with functional and/or recurrent adenomas. The procedure demonstrates a positive balance of efficacy and safety, when performed by teams with high level of experience in endoscopic skull base surgery and in selected patients.

Background: Arginine infusion stimulates copeptin secretion, a surrogate marker of arginine vasopressin (AVP), thereby serving as a diagnostic test in the differential diagnosis of suspected AVP deficiency (AVP-D). Yet, the precise mechanism underlying the stimulatory effect of arginine on the vasopressinergic system remains elusive. Arginine plays a significant role in the urea cycle and increases the production of urea. An increase in plasma urea concentration raises blood osmolality, thereby possibly stimulating AVP release. We therefore hypothesized that the stimulatory effect of arginine on AVP may involve an increase in plasma urea levels.

Methods: This analysis combined data from two prospective diagnostic studies. In total, 30 healthy adults (HA), 69 patients with AVP-D, and 89 patients with primary polydipsia (PP) underwent the arginine stimulation test. Infusion of arginine (L--arginine--hydrochloride 21%) at a dose of 0.5 g/kg body weight diluted in 500 mL of 0.9% normal saline was administered over 30 min. Blood was collected at baseline and 60, 90, and 120 min to analyze plasma copeptin and urea. The main objective was to investigate urea dynamics in response to arginine administration and its effect on copeptin release.

Results: Plasma urea levels at baseline were comparable and increased 60 min after arginine infusion with a median (IQR) change of + 1.1 mmol/L (+ 0.8, + 1.5) in HA, + 1.4 mmol/L (+ 1.1, + 1.7) in patients with AVP-D and + 1.3 mmol/L (+ 0.9, + 1.5) in patients with PP. Concurrently, plasma copeptin levels substantially increased 60 min from baseline in HA (median change + 5.3 pmol/L (+ 3.2, + 8.8)) and in patients with PP (median change + 2.4 pmol/L (+ 1.2, + 3.8)), but remained stable in patients with AVP-D (median change + 0.3 pmol/L (+ 0.1, + 0.6)). Plasma urea and copeptin levels correlated the most in HA, with a Spearman's rho of 0.41 at baseline. Patients with AVP-D and PP showed only weak correlations of plasma urea and copeptin, with a correlation coefficient between 0.01 and 0.28.

Conclusion: We demonstrate a slight increase in plasma urea levels in response to arginine, but plasma urea and copeptin levels were weakly correlated. Based on these findings, the stimulatory effect of arginine on AVP cannot be explained primarily by increasing urea levels.

Purpose: Prolonged length of stay (PLOS) can lead to resource misallocation and higher complication risks. However, there is no consensus on defining PLOS for endoscopic transsphenoidal pituitary surgery (ETPS). Therefore, we investigated the impact of varying PLOS definitions on factors associated with PLOS in patients undergoing ETPS.

Methods: We conducted a retrospective review of patients with pituitary adenomas who underwent ETPS at our institution from 2012 to 2023. Patients were divided into non-PLOS and PLOS groups based on varying definitions of PLOS: > median, > 4 days, > 75th percentile, and > 90th percentile. Bivariate statistical analyses were conducted using Fisher's exact test, chi-square test, and t-tests. Univariate and multivariate logistic regression identified significant predictors for each PLOS definition.

Results: Our cohort (n = 808) had a mean age of 54.37 ± 16.06 years, 50.43% male, and a median LOS of 3 days. The 75th and 90th percentiles of LOS were 4 and 6 days, respectively. The way PLOS was defined influenced associated factors identified. Preoperative KPS score, non-private insurance, and non-home discharge disposition were associated with PLOS across all definitions used (p < 0.05). Increased preoperative tumor volumes and postoperative hyponatremia were associated with PLOS only when defined by the 75th and 90th percentiles (p < 0.05). Non-White race and low income were significantly associated with PLOS > median while intraoperative CSF leak was a significant predictor for PLOS > 90th percentile (p < 0.05).

Conclusion: Our study highlights the variability in predictors of PLOS based on its definition and emphasizes the role of non-clinical factors on LOS.

Purpose: To evaluate the effect of osilodrostat and hypercortisolism control on blood pressure (BP) and glycemic control in patients with Cushing's disease.

Methods: Pooled analysis of two Phase III osilodrostat studies (LINC 3 and LINC 4), both comprising a 48-week core phase and an optional open-label extension. Changes from baseline in systolic and diastolic BP (SBP and DBP), fasting plasma glucose (FPG), and glycated hemoglobin (HbA_1c) were evaluated during osilodrostat treatment in patients with/without hypertension or diabetes at baseline.

Results: Of 210 patients, 82.9% met criteria for hypertension and 40.0% for diabetes at baseline. In patients with hypertension, reductions in mean SBP/DBP were observed from week (W)12 to W72, and 49.1%/58.5% of patients with high SBP/DBP (> 130/>90 mmHg) at baseline had normotensive levels at W72. Antihypertensive medication dose was reduced/stopped in 26.8% of patients, and the proportion taking antihypertensive medication decreased from 54.3% at baseline to 47.3% at W72. In patients with diabetes, mean FPG and HbA_1c decreased from W12 to W72, and 33.3%/61.5% with high FPG/HbA_1c (≥ 100 mg/dL/≥6.5%) at baseline had normal levels at W72. Antihyperglycemic medication dose was reduced/stopped in 35.7% of patients, and the proportion taking antihyperglycemic medication decreased from 21.9% at baseline to 17.1% at W72; improvements in SBP/DBP and FPG/HbA_1c were correlated with improvement in mean urinary free cortisol but not weight change. BP/glycemic parameters generally remained normal in patients without hypertension/diabetes at baseline.

Conclusions: Patients with Cushing's disease and comorbid hypertension/diabetes receiving osilodrostat had rapid and sustained improvements in SBP/DBP and glycemic control, respectively.

Acromegaly-induced diabetes presents unique features due to the direct effects of excess growth hormone (GH) and insulin-like growth factor 1 (IGF-) on glucose metabolism, especially insulin resistance in association to low body fat content and water retention. Increased cardiovascular risk is much higher when acromegaly is complicated with diabetes, thus requiring a holistic management that addresses also these specific characteristics which differ from those of classical type 2 diabetes.The optimal management of diabetes in acromegaly requires not only an effective control of carbohydrate disturbances per se, but also the concurrent control of GH hypersecretion as it will directly impact on glucose control. If surgical treatment is not effective to normalize GH and IGF-1 levels, pharmacologic therapy for acromegaly must consider the metabolic effects that the different drugs may induce, as some of them may worsen carbohydrate metabolism. When treating acromegaly-induced diabetes, a comprehensive approach is essential, incorporating medications that may also protect against acromegaly associated comorbidities. Metformin remains the first-line therapy due to its ability to reduce hepatic glucose production enhance insulin sensitivity and its cost effectiveness. The newer drug classes, such as glucagon-like peptide-1 receptor agonists and sodium-glucose cotransporter-2 inhibitors, offer benefits similar to those seen in type 2 diabetes, but the unique metabolic profile of acromegaly-including an enhanced ketogenic state and the effects of incretins on GH secretion-have to be considered as it may influence outcomes. Understanding the distinct pathophysiology of acromegaly-induced diabetes and the benefits of these newer drug classes for the patient with acromegaly is crucial for optimizing treatment outcomes and improving the quality of life.

Introduction: Empty sella is characterized by a flattened profile of the pituitary gland that represents in most cases only a radiological incidental finding. When endocrine, ophthalmic, and neurological symptoms occur, this condition is described as empty sella syndrome.

Materials and methods: We searched MEDLINE (PubMed database) with the data filter 2024-2009 using the keywords listed above. The articles met the following inclusion criteria: 1) written in English; 2) published between 1 January 2009, and 31 December 2023; 3) original studies and case series on the epidemiology, characteristics, and treatment of empty sella and empty sella syndrome. Exclusion criteria included: articles written in languages other than English and single case report.

Results: The incidence of empty sella is about 12%, when it comes to neuroimaging, while in clinical practice it can reach 35%. Endocrine pituitary disorders, defined as at least one hormone deficit, were reported in 19% to 40% of patients. Magnetic nuclear imaging (MRI) is confirmed to be the gold standard for radiological diagnosis of empty sella. If no alterations are detected it is suggested a careful revaluation at 24-36 months, in relation to the low risk of progression to PES syndrome.

Conclusions: Empty sella is, in most cases, a radiological finding without clinical manifestations. In people carrying empty sella hormonal pituitary evaluation should be assessed at the moment of the diagnosis, along with a careful imaging using MRI.