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Beverly M K Biller - One of the best experts on this subject based on the ideXlab platform.
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hypertension in Acromegaly in relationship to biochemical control and mortality global acrostudy outcomes
Frontiers in Endocrinology, 2020Co-Authors: Greisa Vila, Sebastian J C M M Neggers, Aart Jan Van Der Lely, Beverly M K Biller, Anton Luger, Susan M Webb, Srinivas Rao Valluri, Judith HeyhadaviAbstract:Context: Hypertension is a major cardiovascular risk factor related to increased mortality in Acromegaly. Surgical cure of Acromegaly is associated with improvement in blood pressure levels, however little is known about the effect of pegvisomant (PEGV) treatment in patients with hypertension. This analysis evaluates outcomes in patients with hypertension and Acromegaly included in ACROSTUDY. Methods: ACROSTUDY is a global non-interventional surveillance study of long-term treatment with PEGV, monitoring its safety and efficacy. The cohort was retrospectively divided in two subgroups: patients with and without hypertension. Stepwise logistic regression and Kaplan-Meyer analyses were performed for testing predictors of mortality. Results: The total cohort included 2090 patients with Acromegaly treated with PEGV who were followed for a median of 6.8 years (range up to 12.1 yrs). At ACROSTUDY start, there were 1344 patients with hypertension (52.3% males). This subgroup was older, had a higher BMI, and higher prevalence of diabetes, hyperlipidemia, and cardiovascular disease (CVD) when compared to patients without hypertension. During ACROSTUDY, 68 deaths were reported in the hypertension cohort, vs 10 in the cohort without hypertension. Both CVD (p<0.0001) and anterior pituitary deficiencies (p=0.0105) at study entry independently predicted mortality in patients with Acromegaly and hypertension; Kaplan-Meier analysis confirmed that CVD significantly impairs survival. Conclusions: Hypertension is common in patients with Acromegaly and significantly increases mortality, especially when there is concomitant CVD. These data suggest that treatment goals should extend beyond IGF-I normalization, and include optimisation of substitution of pituitary deficiencies and scrutinous screening and treatment of CVD.
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A Pituitary Society update to Acromegaly management guidelines
Pituitary, 2020Co-Authors: Maria Fleseriu, Andrea Giustina, Beverly M K Biller, Christian J Strasburger, Pamela U. Freda, Monica R. Gadelha, Laurence Katznelson, Mark E. Molitch, Susan L. Samson, A. J. LelyAbstract:Guidelines and consensus statements ensure that physicians managing Acromegaly patients have access to current information on evidence-based treatments to optimize outcomes. Given significant novel recent advances in understanding Acromegaly natural history and individualized therapies, the Pituitary Society invited Acromegaly experts to critically review the current literature in the context of Endocrine Society guidelines and Acromegaly Consensus Group statements. This update focuses on how recent key advances affect treatment decision-making and outcomes, and also highlights the likely role of recently FDA-approved therapies as well as novel combination therapies within the treatment armamentarium.
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bone microarchitecture and estimated bone strength in men with active Acromegaly
European Journal of Endocrinology, 2017Co-Authors: Paula Pb Silva, Pouneh K Fazeli, Elizabeth A Lawson, Karen K Miller, Fatemeh G Amlashi, Karen J Pulaskiliebert, Anu V Gerweck, Lisa B Nachtigall, Beverly M K Biller, Anne KlibanskiAbstract:CONTEXT Both Acromegaly and adult growth hormone deficiency (GHD) are associated with increased fracture risk. Sufficient data are lacking regarding cortical bone microarchitecture and bone strength, as assessed by microfinite element analysis (µFEA). OBJECTIVE To elucidate both cortical and trabecular bone microarchitecture and estimated bone strength in men with active Acromegaly or GHD compared to healthy controls. DESIGN AND SUBJECTS Cross-sectional study at a clinical research center, including 48 men (16 with Acromegaly, 16 with GHD and 16 healthy controls). OUTCOME MEASURES Areal bone mineral density (aBMD), cortical and trabecular bone microarchitecture and estimated bone strength (µFEA) at the radius and tibia. RESULTS aBMD was not different between the 3 groups at any skeletal site. At the radius, patients with Acromegaly had greater cortical area (P < 0.0001), cortical thickness (P = 0.0038), cortical pore volume (P < 0.0001) and cortical porosity (P = 0.0008), but lower trabecular bone density (P = 0.0010) compared to controls. At the tibia, patients with Acromegaly had lower trabecular bone density (P = 0.0082), but no differences in cortical bone microstructure. Compressive strength and failure load did not significantly differ between groups. These findings persisted after excluding patients with hypogonadism. Bone microarchitecture was not deficient in patients with GHD. CONCLUSIONS Both cortical and trabecular microarchitecture are altered in men with Acromegaly. Our data indicate that GH excess is associated with distinct effects in cortical vs trabecular bone compartments. Our observations also affirm the limitations of aBMD testing in the evaluation of patients with Acromegaly.
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pegvisomant a growth hormone receptor antagonist used in the treatment of Acromegaly
Pituitary, 2017Co-Authors: Nicholas A Tritos, Beverly M K BillerAbstract:To review published data on pegvisomant and its therapeutic role in Acromegaly. Electronic searches of the published literature were conducted using the keywords: Acromegaly, growth hormone (GH) receptor (antagonist), pegvisomant, therapy. Relevant articles (n = 141) were retrieved and considered for inclusion in this manuscript. Pegvisomant is a genetically engineered, recombinant growth hormone receptor antagonist, which is effective in normalizing serum insulin-like growth factor 1 (IGF-1) levels in the majority of patients with Acromegaly and ameliorating symptoms and signs associated with GH excess. Pegvisomant does not have direct antiproliferative effects on the underlying somatotroph pituitary adenoma, which is the etiology of GH excess in the vast majority of patients with Acromegaly. Therefore, patients receiving pegvisomant monotherapy require regular pituitary imaging in order to monitor for possible increase in tumor size. Adverse events in patients on pegvisomant therapy include skin rashes, lipohypertrophy at injection sites, and idiosyncratic liver toxicity (generally asymptomatic transaminitis that is reversible upon drug discontinuation), thus necessitating regular patient monitoring. Pegvisomant is an effective therapeutic agent in patients with Acromegaly who are not in remission after undergoing pituitary surgery. It mitigates excess GH action, as demonstrated by IGF-1 normalization, but has no direct effects on pituitary tumors causing Acromegaly. Regular surveillance for possible tumor growth and adverse effects (hepatotoxicity, skin manifestations) is warranted.
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growth hormone deficiency is associated with decreased quality of life in patients with prior Acromegaly
The Journal of Clinical Endocrinology and Metabolism, 2009Co-Authors: Tamara Wexler, Lisa B Nachtigall, Beverly M K Biller, Lindsay Gunnell, Zehra B Omer, Karen Kuhlthau, Catherine Beauregard, Gwenda J Graham, Andrea L Utz, Jay S LoefflerAbstract:Context: Both GH deficiency (GHD) and GH excess are associated with a decreased quality of life. However, it is unknown whether patients with GHD after treatment for Acromegaly have a poorer quality of life than those with normal GH levels after cure of Acromegaly. Objective: The aim of the study was to determine whether patients with GHD and prior Acromegaly have a poorer quality of life than those with GH sufficiency after cure of Acromegaly. Design and Setting: We conducted a cross-sectional study in a General Clinical Research Center. Study Participants: Forty-five patients with prior Acromegaly participated: 26 with GHD and 19 with GH sufficiency. Intervention: There were no interventions. Main Outcome Measures: We evaluated quality of life, as measured by 1) the Quality of Life Adult Growth Hormone Deficiency Assessment (QoL-AGHDA); 2) the Short-Form Health Survey (SF-36); and 3) the Symptom Questionnaire. Results: Mean scores on all subscales of all questionnaires, except for the anger/hostility and anxiety subscales of the Symptom Questionnaire, showed significantly impaired quality of life in the GH-deficient group compared with the GH-sufficient group. Peak GH levels after GHRH-arginine stimulation levels were inversely associated with QoL-AGHDA scale scores (R = −0.53; P = 0.0005) and the Symptom Questionnaire Depression subscale scores (R = −0.35; P = 0.031) and positively associated with most SF-36 subscale scores. Conclusions: Our data are the first to demonstrate a reduced quality of life in patients who develop GHD after cure of Acromegaly compared to those who are GH sufficient. Further studies are warranted to determine whether GH replacement would improve quality of life for patients with GHD after cure from Acromegaly.
Catharina Larsson - One of the best experts on this subject based on the ideXlab platform.
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a kindred with a variant of multiple endocrine neoplasia type 1 demonstrating frequent expression of pituitary tumors but not linked to the multiple endocrine neoplasia type 1 locus at chromosome region 11q13
The Journal of Clinical Endocrinology and Metabolism, 1997Co-Authors: John L Stock, Maria R Warth, Bin Tean Teh, James A Coderre, Judith H Overdorf, Gerhard Baumann, Raymond L Hintz, M L Hartman, Bernd R Seizinger, Catharina LarssonAbstract:Acromegaly is uncommon in kindreds with multiple endocrine neoplasia type 1 (MEN1), whereas primary hyperparathyroidism (PHP) has the highest penetrance of any endocrinopathy. We report an unusual MEN1 kindred with frequent expression of pituitary tumors and a low penetrance of PHP. Four members were found to have disease: PHP in generation I, Acromegaly (2 cases) in generation II, and hyperprolactinemia associated with a pituitary tumor in generation III. There was no evidence for PHP in 1 patient with Acromegaly (age 60 yr), the patient with hyperprolactinemia and the pituitary tumor (age 22 yr), and 1 asymptomatic obligate carrier (age 50 yr). Screening of 26 members revealed the possible diagnosis of PHP in 1 family member in generation II and possible early Acromegaly in 2 members of generation III with elevated serum concentrations of insulin-like growth factor I and insulin-like growth factor-binding protein-3 but normal patterns of pulsatile GH release. Although the predisposing genetic defect in ...
Andrea Giustina - One of the best experts on this subject based on the ideXlab platform.
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A Pituitary Society update to Acromegaly management guidelines
Pituitary, 2020Co-Authors: Maria Fleseriu, Andrea Giustina, Beverly M K Biller, Christian J Strasburger, Pamela U. Freda, Monica R. Gadelha, Laurence Katznelson, Mark E. Molitch, Susan L. Samson, A. J. LelyAbstract:Guidelines and consensus statements ensure that physicians managing Acromegaly patients have access to current information on evidence-based treatments to optimize outcomes. Given significant novel recent advances in understanding Acromegaly natural history and individualized therapies, the Pituitary Society invited Acromegaly experts to critically review the current literature in the context of Endocrine Society guidelines and Acromegaly Consensus Group statements. This update focuses on how recent key advances affect treatment decision-making and outcomes, and also highlights the likely role of recently FDA-approved therapies as well as novel combination therapies within the treatment armamentarium.
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Multidisciplinary management of Acromegaly: A consensus
Reviews in Endocrine and Metabolic Disorders, 2020Co-Authors: Andrea Giustina, Albert Beckers, Marek Bolanowski, Garni Barkhoudarian, Anat Ben-shlomo, Nienke Biermasz, Beverly Biller, Cesar Boguszewski, Jens Bollerslev, Vivien BonertAbstract:The 13th Acromegaly Consensus Conference was held in November 2019 in Fort Lauderdale, Florida, and comprised Acromegaly experts including endocrinologists and neurosurgeons who considered optimal approaches for multidisciplinary Acromegaly management. Focused discussions reviewed techniques, results, and side effects of surgery, radiotherapy, and medical therapy, and how advances in technology and novel techniques have changed the way these modalities are used alone or in combination. Effects of treatment on patient outcomes were considered, along with strategies for optimizing and personalizing therapeutic approaches. Expert consensus recommendations emphasize how best to implement available treatment options as part of a multidisciplinary approach at Pituitary Tumor Centers of Excellence.
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bone turnover bone mineral density and fracture risk in Acromegaly a meta analysis
The Journal of Clinical Endocrinology and Metabolism, 2015Co-Authors: Gherardo Mazziotti, Filippo Maffezzoni, Elena Iagioli, Maurizio Spinello, Vincenza Serra, Roberto Maroldi, Irene Floriani, Andrea GiustinaAbstract:Context: GH excess causes an increase in bone turnover, but the consequences in terms of skeletal fragility have long been uncertain due to the heterogeneity of studies dealing with this topic. Objective: We conducted a meta-analysis of studies examining the effects of Acromegaly on bone turnover, bone mineral density (BMD), and fractures. Furthermore, we evaluated the effects of sex, gonadal status, and activity of disease on skeletal end-points in Acromegaly. Data Sources: We conducted MEDLINE and EMBASE systematic searches up to December 31, 2013. Study Eligibility Criteria: Studies conducted in patients with Acromegaly and reporting at least one determinant of skeletal fragility. Data Extraction and Analysis: Study design, patient characteristics, interventions, and outcomes were independently extracted by two authors. We calculated the standardized mean difference (SMD) of bone turnover and BMD differences, whereas fractures were presented as relative frequencies in Acromegaly and odds ratios between...
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investigational drugs targeting somatostatin receptors for treatment of Acromegaly and neuroendocrine tumors
Expert Opinion on Investigational Drugs, 2014Co-Authors: Andrea Giustina, Gherardo Mazziotti, Filippo Maffezzoni, Vito Amoroso, Alfredo BerrutiAbstract:Introduction: Octreotide long-acting release (LAR) and lanreotide Autogel (ATG) are the two somatostatin analogs currently approved for treatment of Acromegaly and neuroendocrine tumors (NETs). The strength of these drugs has been their specificity for somatostatin receptor subtype 2. However, this peculiarity may become a weakness in some patients with tumors harboring somatostatin receptors different from the subtype 2. Another clinically relevant aspect related to the use of octreotide LAR and lanreotide ATG is the burden of injectable drug regimen that may adversely impact the quality of life of patients with Acromegaly and NETs.Areas covered: The authors review the recently published evidence on novel drugs targeting somatostatin receptors developed for treating Acromegaly and NETs. Within this article, the authors discuss: i) the pharmacology of somatostatin and traditional somatostatin analogs; ii) the efficacy and safety of multireceptor-targeted somatostatin analogs in Acromegaly and NETs; iii) t...
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use of pegvisomant in Acromegaly an italian society of endocrinology guideline
Journal of Endocrinological Investigation, 2014Co-Authors: Andrea Giustina, L De Marinis, Salvatore Cannavo, F Bogazzi, S Grottoli, Maria Rosaria Ambrosio, Beck P Peccoz, E De Menis, Rosario PivonelloAbstract:Acromegaly management is a significant challenge for endocrinologists. The Acromegaly Consensus Group developed several statements on the management of Acromegaly and specifically on its medical treatment [1–3]. Acromegaly is a quite rare condition generally caused by a growth hormone (GH)-secreting pituitary adenoma [4]. Delayed diagnosis leads to prevalent presentation of the disease at the stage of macroadenoma (two-thirds of patients) and frequent persistence of active disease after surgery which remains in many patients the primary treatment option [5]. However, active Acromegaly is potentially a life threatening condition due its severe systemic complications [6, 7] Therefore, elevated GH and insulin-like growth factor (IGF)-1 levels need to be strictly controlled after failure of surgery with medical or radiation treatments [8]. Furthermore, criteria for disease control may not be fulfilled in a considerable proportion of patients undergoing medical treatment with somatostatin receptor ligands (SRLs) after unsuccessful surgery [9, 10]. Accordingly, some Acromegaly patients require the administration of GH antagonist Pegvisomant [11]. Pegvisomant has been introduced in clinical practice more than a decade ago as a medical therapy of Acromegaly. However, specific guidelines for Pegvisomant use in Acromegaly are lacking. Therefore, the Italian Society of Endocrinology constituted a task force with the objective of assessing the published literature and the clinical experience with Pegvisomant. This group involved endocrinologists recognized experts in the field of Acromegaly management and their understanding of the data reported so far worldwide as well as their recommendations for Pegvisomant use in clinical practice are presented here. Biochemical and clinical results of Pegvisomant, indications, treatment modalities, combination therapies, safety and regulatory and cost/efficacy issues were evaluated. Evidences were graded with GRADE system [1–3, 12, 13] based on the quality of evidence as very low quality (VLQ; expert opinion with one or a small number of small uncontrolled studies in support), low quality (LQ; large series of small uncontrolled studies), moderate quality (MQ; one or a small number of large uncontrolled studies or meta-analyses), or high quality (HQ; controlled studies or large series of large uncontrolled studies with sufficiently long follow-up). Recommendations were defined discretionary (DR) if based on VLQ-LQ evidence, or strong (SR) if supported by MQ-HQ evidence.
John L Stock - One of the best experts on this subject based on the ideXlab platform.
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a kindred with a variant of multiple endocrine neoplasia type 1 demonstrating frequent expression of pituitary tumors but not linked to the multiple endocrine neoplasia type 1 locus at chromosome region 11q13
The Journal of Clinical Endocrinology and Metabolism, 1997Co-Authors: John L Stock, Maria R Warth, Bin Tean Teh, James A Coderre, Judith H Overdorf, Gerhard Baumann, Raymond L Hintz, M L Hartman, Bernd R Seizinger, Catharina LarssonAbstract:Acromegaly is uncommon in kindreds with multiple endocrine neoplasia type 1 (MEN1), whereas primary hyperparathyroidism (PHP) has the highest penetrance of any endocrinopathy. We report an unusual MEN1 kindred with frequent expression of pituitary tumors and a low penetrance of PHP. Four members were found to have disease: PHP in generation I, Acromegaly (2 cases) in generation II, and hyperprolactinemia associated with a pituitary tumor in generation III. There was no evidence for PHP in 1 patient with Acromegaly (age 60 yr), the patient with hyperprolactinemia and the pituitary tumor (age 22 yr), and 1 asymptomatic obligate carrier (age 50 yr). Screening of 26 members revealed the possible diagnosis of PHP in 1 family member in generation II and possible early Acromegaly in 2 members of generation III with elevated serum concentrations of insulin-like growth factor I and insulin-like growth factor-binding protein-3 but normal patterns of pulsatile GH release. Although the predisposing genetic defect in ...
Gherardo Mazziotti - One of the best experts on this subject based on the ideXlab platform.
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treatment of acromegalic osteopathy in real life clinical practice the baac bone active drugs in Acromegaly study
The Journal of Clinical Endocrinology and Metabolism, 2020Co-Authors: Gherardo Mazziotti, Filippo Maffezzoni, Claudia Battista, Sabrina Chiloiro, Emanuele Ferrante, Nunzia Prencipe, Ludovica F S Grasso, Federico Gatto, Roberto Olivetti, Maura ArosioAbstract:BACKGROUND Vertebral fractures (VFs) are a frequent complication of Acromegaly, but no studies have been so far published on effectiveness of antiosteoporotic drugs in this clinical setting. OBJECTIVE To evaluate whether in real-life clinical practice bone active drugs may reduce the risk of VFs in patients with active or controlled Acromegaly. STUDY DESIGN Retrospective, longitudinal study including 9 tertiary care endocrine units. PATIENTS AND METHODS Two hundred and forty-eight patients with Acromegaly (104 males; mean age 56.00 ± 13.60 years) were evaluated for prevalent and incident VFs by quantitative morphometric approach. Bone active agents were used in 52 patients (20.97%) and the median period of follow-up was 48 months (range 12-132). RESULTS During the follow-up, 65 patients (26.21%) developed incident VFs in relationship with pre-existing VFs (odds ratio [OR] 3.75; P < .001), duration of active Acromegaly (OR 1.01; P = .04), active Acromegaly at the study entry (OR 2.48; P = .007), and treated hypoadrenalism (OR 2.50; P = .005). In the entire population, treatment with bone active drugs did not have a significant effect on incident VFs (P = .82). However, in a sensitive analysis restricted to patients with active Acromegaly at study entry (111 cases), treatment with bone active drugs was associated with a lower risk of incident VFs (OR 0.11; P = .004), independently of prevalent VFs (OR 7.65; P < .001) and treated hypoadrenalism (OR 3.86; P = .007). CONCLUSIONS Bone active drugs may prevent VFs in patients with active Acromegaly.
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bone turnover bone mineral density and fracture risk in Acromegaly a meta analysis
The Journal of Clinical Endocrinology and Metabolism, 2015Co-Authors: Gherardo Mazziotti, Filippo Maffezzoni, Elena Iagioli, Maurizio Spinello, Vincenza Serra, Roberto Maroldi, Irene Floriani, Andrea GiustinaAbstract:Context: GH excess causes an increase in bone turnover, but the consequences in terms of skeletal fragility have long been uncertain due to the heterogeneity of studies dealing with this topic. Objective: We conducted a meta-analysis of studies examining the effects of Acromegaly on bone turnover, bone mineral density (BMD), and fractures. Furthermore, we evaluated the effects of sex, gonadal status, and activity of disease on skeletal end-points in Acromegaly. Data Sources: We conducted MEDLINE and EMBASE systematic searches up to December 31, 2013. Study Eligibility Criteria: Studies conducted in patients with Acromegaly and reporting at least one determinant of skeletal fragility. Data Extraction and Analysis: Study design, patient characteristics, interventions, and outcomes were independently extracted by two authors. We calculated the standardized mean difference (SMD) of bone turnover and BMD differences, whereas fractures were presented as relative frequencies in Acromegaly and odds ratios between...
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investigational drugs targeting somatostatin receptors for treatment of Acromegaly and neuroendocrine tumors
Expert Opinion on Investigational Drugs, 2014Co-Authors: Andrea Giustina, Gherardo Mazziotti, Filippo Maffezzoni, Vito Amoroso, Alfredo BerrutiAbstract:Introduction: Octreotide long-acting release (LAR) and lanreotide Autogel (ATG) are the two somatostatin analogs currently approved for treatment of Acromegaly and neuroendocrine tumors (NETs). The strength of these drugs has been their specificity for somatostatin receptor subtype 2. However, this peculiarity may become a weakness in some patients with tumors harboring somatostatin receptors different from the subtype 2. Another clinically relevant aspect related to the use of octreotide LAR and lanreotide ATG is the burden of injectable drug regimen that may adversely impact the quality of life of patients with Acromegaly and NETs.Areas covered: The authors review the recently published evidence on novel drugs targeting somatostatin receptors developed for treating Acromegaly and NETs. Within this article, the authors discuss: i) the pharmacology of somatostatin and traditional somatostatin analogs; ii) the efficacy and safety of multireceptor-targeted somatostatin analogs in Acromegaly and NETs; iii) t...
