The effectiveness of acromegaly treatment according to the Registry of patients with pituitary tumors in the Republic of Tatarstan

2020 ◽  
Vol 49 ◽  
Author(s):  
G. R. Vagapova ◽  
B. Yu. Pashaev ◽  
R. R. Ashimova ◽  
A. R. Khafizov ◽  
Iu. V. Mokhova

Background: Acromegaly is a severe progressive neuroendocrine disease caused by chronic elevated concentrations of growth hormone and insulin-like growth factor 1 in individuals with completed physiological growth and leading to early disability and mortality. The Acromegaly Registry with its regular updates makes it possible to merge and systematize all the available information on the diseases, to assess the efficacy of treatment algorithms and to choose an optimal diagnostic and treatment strategy in a given territory.Aim: To evaluate the efficacy of various treatments for acromegaly based on the analysis of the regional Registry of patients with pituitary tumors in the Republic of Tatarstan.Materials and methods: The study was based on data from 217 patients with somatotropin producing pituitary adenomas entered in the Registry of patients with pituitary tumors in the Republic of Tatarstan as per January 2021. One hundred and eighty one (181 patients) with sufficient data to assess the remission rates and the disease course were included into the analysis.Results: Endonasal endoscopic adenomectomy as a first-line treatment has been performed in 93% (169/181) of the patients, with 34% of the cases (57/169) being in the full postoperative remission. The efficacy of surgical treatment depended on the tumor size, invasion grade and aggressiveness. A 1st generation long-acting somatostatin analog (octreotide) has been prescribed to 50% (91/181) of the patients (11 patients as first-line therapy and 80 patients after adenomectomy), with biochemical remission rate of 56% (51/91). Radiological treatment has been completed in 11,6% (21/181) of the patients. The complete remission rate after all treatment modalities (surgical, medical, and radiation therapy) was 60% (108/181).Conclusion: The effectiveness of treatment for acromegaly in the Republic of Tatarstan is comparable to that in the all-Russian and international registries. Nevertheless, 40% of the patients with acromegaly in the region do not have their disease biochemically controlled indicating the need to optimize drug treatment.

2020 ◽  
Vol 66 (1) ◽  
pp. 93-103
Author(s):  
Zhanna E. Belaya ◽  
Olga O. Golounina ◽  
Liudmila Y. Rozhinskaya ◽  
Galina A. Melnichenko ◽  
Michail А. Isakov ◽  
...  

BACKGROUND: The registry is the main source of information about patients with acromegaly for assessing the quality of medical care, effectiveness of treatment, determining the compliance of real clinical practice with existing standards and patient management protocols. AIMS: To evaluate epidemiological, demographic and clinical characteristics of acromegaly in Russian Federation and effectiveness of treatment modalities. MATERIALS AND METHODS: The object of the study was the database of the united Russian registry of patients with pituitary tumors with specific analysis of patients with acromegaly only. We analyzed the data of 4114 patients with acromegaly stored on the online system in February 2019. RESULTS: Based on the data 32% of patients had complete clinical and laboratory remission of acromegaly; the percentage of patients with no remission was 68%, among them 22.5% had significant improvements in clinical symptoms and a decrease in growth hormone (GH) and insulin-like growth factor-1 (IGF-1) without IGF-1 normalization. The average age of patients at the onset of the disease was 42.7 years and at diagnosis 45.8 years. The ratio of men to women was 1:2.6. In patients with acromegaly hypopituitarism was registered in 14.7% of cases and among them hypothyroidism (66%) and hypogonadism (52%) were registered more often. Among other complications the leading were diabetes mellitus (15.7%) and acromegalic arthropathy (15%). The proportion of patients receiving neurosurgical treatment increased from 35.7% to 49.6% in 20122019; the portion of patients undergoing radiation therapy decreased significantly from 17.7% in 2012 to 0.8% in 2019. Remission was achieved in 40.47% after neurosurgery and 28.95% after medical treatment as a first line therapy p0.01. The number of patients receiving medical treatment at the time of the study was 1209. Among them 51% of patients treated with long-acting lanreotide and 24% receiving long-acting octreotide achieved remission (p0.0001) CONCLUSIONS: The remission rate of acromegaly remains suboptimal despite increased surgical activity, which corresponds to global trends. Long-acting lanreotide was significantly superior versus long-acting octreotide in the rate of acromegaly remission, which does not correspond with clinical trials.


2021 ◽  
Vol 40 (10) ◽  
pp. 637-645
Author(s):  
Maria Chiara Pellegrin ◽  
Alessandro Occhipinti ◽  
Benedetta Bossini ◽  
Stefania Norbedo ◽  
Elena faleschini ◽  
...  

Hyperthyroidism is a rare clinical entity in paediatric age. In most cases the etiology is autoimmune and goiter represents the typical presentation. Ophthalmopathy is rare and can precede the diagnosis of autoimmune hyperthyroidism. Thyrotoxicosis caused by the hyperthyroid phase of Hashimoto’s thyroiditis must be carefully distinguished from Graves’ disease (GD), since the first condition has a better prognosis for spontaneous remission. Three treatment options are currently available for the management of paediatric GD. First-line therapy is antithyroid drugs, while thyroidectomy and radioiodine are considered on relapse. A lower remission rate and a higher risk of adverse events are observed after the first course of methimazole in childhood with respect to adulthood. Children and adolescents may require a prolonged antithyroid treatment, but an overall consensus regarding the optimal regimen is lacking.


2009 ◽  
Vol 29 (02) ◽  
pp. 171-176 ◽  
Author(s):  
G. Janssen ◽  
A. Borkhardt ◽  
H. J. Laws

SummaryApproximately 70% of children have the acute form of immune thrombocytopenia (ITP), which is defined by recovery within six months of presentation with or without treatment. Chronic ITP is to be reserved for patients with platelets < 100 000/μl for more than twelve months and exclusion of other diagnosis like systemic lupus erythematosus or bone marrow failures. In children, the chance of spontaneous recovery is 52% after diagnosis of chronic ITP. The Intercontinental Childhood ITP Study group recommends that children without bleeding may not require therapy regardless of their platelet count. Whereas in patients with bleeding symptoms first line therapy is defined and includes steroids or immunoglobuline, second line therapy in refractory patients with significant hemorrhagic problems is unclear. Guidelines recommend splenectomy, but for more than 50 years patients and physicians look for pharmacological alternatives. It may be that rituximab is a promising option which has been proven to be effective with few adverse effects. Till now the treatment has focused on immunomodulation. Research has now focused on stimulating platelet production. In this review we discuss old and new therapy modalities for children with cITP.


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e20014-e20014 ◽  
Author(s):  
Aikaterini Bilitou ◽  
Zhongyun Zhao ◽  
Beth L. Barber ◽  
Genevieve Sian Clapton ◽  
Deborah Saltman

e20014 Background: Since 2011, two therapies that provide novel approaches to the treatment of advanced (unresectable or metastatic) melanoma have been introduced to the market: ipilimumab for second line and vemurafenib for BRAF mutation-positive melanoma. It is not known how the new drugs influence treatment; this study investigated current treatment patterns in advanced melanoma. Methods: A clinician-validated, web-based survey was administered between August and November 2012 to clinicians who treat advanced melanoma in France, Germany, Italy, Spain, and the UK. Respondents were asked about their treatment of patients in the previous 12 months, including treatment modalities and pharmaceutical therapies used, and factors that affect treatment choice. Results: 150 oncologists and dermatologists completed the survey, 30 in each country. Pharmaceutical therapy was more commonly used than other treatment modalities and varied by stage of disease. A high proportion of patients with late stage of disease were treated with pharmaceutical therapy in Germany (85% in M1B) and France (83% in M1C). Among the countries, 51% (Italy) to 87% (France) of respondents test BRAF mutation status. In patients with wild-type BRAF tumors, dacarbazine, which has not been shown to provide any overall survival benefit, was the most commonly used drug for first-line therapy in all countries. In mutated BRAF tumors, vemurafenib was the most commonly used drug in first line therapy in 3 of 5 countries. There was no standard of care in second line for either patients with wild-type or mutated-BRAFtumors; therapies used included fotemustine, temozolomide, interferon, paclitaxel, and ipilimumab. Conclusions: Treatment options for patients with advanced melanoma are limited, particularly for patients with wild-type BRAF disease. In second line, there does not appear to be an established standard of care: a range of treatments are used, including several not indicated for melanoma.


2021 ◽  
Vol 184 (1) ◽  
pp. 1-8
Author(s):  
Frédéric Illouz ◽  
Philippe Chanson ◽  
Emmanuel Sonnet ◽  
Thierry Brue ◽  
Amandine Ferriere ◽  
...  

Objective Somatostatin receptor ligands (SRL) are useful to control central hyperthyroidism in patients with thyrotropin-secreting pituitary adenoma (TSH pituitary adenoma). The aim of this study was to describe the frequency of thyrotropin deficiency (TSH deficiency) in patients with TSH pituitary adenoma treated by SRL. Design Retrospective study. Methods Patients with central hyperthyroidism due to TSH pituitary adenoma treated by short or long-acting SRL were retrospectively included. TSH deficiency was defined by a low FT4 associated with non-elevated TSH concentrations during SRL therapy. We analysed the frequency of TSH deficiency and the characteristics of patients with or without TSH deficiency. Results Forty-six patients were included. SRL were used as the first-line therapy in 21 of 46 patients (46%). Central hyperthyroidism was controlled in 36 of 46 patients (78%). TSH deficiency appeared in 7 of 46 patients (15%) after a median time of 4 weeks (4–7) and for a median duration of 3 months (2.5–3). The TSH deficiency occurred after one to three injections of long-acting SRL used as first-line therapy in 6/7 cases. There were no differences in terms of clinical and hormonal features, size of adenomas or doses of SRL between patients with or without TSH deficiency. Conclusions SRL can induce TSH deficiency in patients with central hyperthyroidism due to TSH pituitary adenoma. Thyrotropic function should be assessed before the first three injections of SRL in order to track TSH deficiency and reduce the frequency of injections when control of thyrotoxicosis rather than tumour reduction is the aim of the treatment.


Author(s):  
Kyungwon Kim ◽  
Yae Won Park ◽  
Daham Kim ◽  
Sung Soo Ahn ◽  
Ju Hyung Moon ◽  
...  

Abstract Context Dopamine agonists (DAs) are the first-line therapy for prolactinomas. Although pituitary tumors often do not completely disappear, discontinuing DAs in patients with no visible tumor on magnetic resonance imaging is advised. Objective To analyze biochemical remission after cabergoline (CAB) withdrawal in patients with visible remnant pituitary tumors. Design Retrospective cohort study. Setting Severance Hospital. Subjects We identified 734 patients with prolactinomas undergoing CAB therapy for at least 12 months from 2005 to 2018. We selected 44 patients with prolactinomas who discontinued CAB with normal prolactin levels; they were receiving a minimal CAB dose but had visible remnant tumors. Results Median age at diagnosis was 32 (18-58) years, and most patients were women (95.45%). Median treatment duration was 32 (12-120) months. Of 44 patients, 33 continued to have normoprolactinemia, but 11 patients developed hyperprolactinemia after drug withdrawal within 26 (12-97) months. Age, sex, maximal and remnant tumor size, and treatment duration were similar between the groups. The initial prolactin level and chances of cavernous sinus (CS) invasion were higher in the recurrence group. CS invasion at diagnosis was associated with an increased recurrence rate. Although treatment response did not correlate with the initial and final signal intensity assessments, a significant decrease in T2 intensity ratio after 6 months of CAB therapy was observed in the remission group (P = .043). Conclusion In patients with visible tumors, the presence of CS invasion at diagnosis may be an unfavorable predictor for biochemical remission after CAB discontinuation.


2020 ◽  
Vol 7 (12) ◽  
pp. 1781
Author(s):  
Pravin Kahale ◽  
Pijush Kanti Biswas ◽  
Sunil George ◽  
Sree Ranga P. C. ◽  
Pankaj Singh ◽  
...  

Background: The treatment modalities of resistant hypertension (RH) remain a clinical challenge, often requiring secondary/add-on drugs with first-line therapy to control blood pressure (BP). This study was conducted to explore and understand the preferences and practices of Indian physicians towards the use of vasodilator (especially di-hydralazine) in the management of RH.Methods: This was a cross-sectional, observational, web-based physician survey. The study included cardiologist, nephrologist and consultant physicians from different geographical regions of India. A web-based physician survey questionnaire (PSQ) was created in google forms and the link was circulated to the physicians. Responses obtained were analysed.Results: A total of 457 physicians participated in this survey. In majority of the physicians, vasodilators were the treatment choice as secondary or add-on drugs with first line therapy to control BP in RH; especially hydralazine/di-hydralazine preferred the most. Majority of the physicians preferred to combine vasodilator with beta blocker and diuretic in patients with uncontrolled and RH. Cardiac failure, followed by chronic kidney disease (CKD), diabetes, dyslipidaemia, hypertensive emergency and angina were the common patient profile in RH in which majority physicians prescribed vasodilator (di-hydralazine). Majority of the physicians rated vasodilator di-hydralazine as “good-very good” in terms of efficacy, safety, tolerability, patient compliance and patient satisfaction in RH.Conclusions: Overall, vasodilators (hydrazinophthalazine derivatives) are preferred as add-on drugs along with first-line drugs in RH. Physician’s opinion towards the use of di-hydralazine was positive. Di-hydralazine may be preferred as an add-on therapeutic option to control BP in RH, however randomized clinical trials are needed for recommendation in cardio-renal medicine.


Blood ◽  
2009 ◽  
Vol 114 (16) ◽  
pp. 3382-3391 ◽  
Author(s):  
Barbara F. Eichhorst ◽  
Raymonde Busch ◽  
Stephan Stilgenbauer ◽  
Martina Stauch ◽  
Manuela A. Bergmann ◽  
...  

Abstract Although chronic lymphocytic leukemia (CLL) is a disease of elderly patients, subjects older than 65 years are heavily underrepresented in clinical trials. The German CLL study group (GCLLSG) initiated a multicenter phase III trial for CLL patients older than 65 years comparing first-line therapy with fludarabine with chlorambucil. A total of 193 patients with a median age of 70 years were randomized to receive fludarabine (25 mg/m2 for 5 days intravenously, every 28 days, for 6 courses) or chlorambucil (0.4 mg/kg body weight [BW] with an increase to 0.8 mg/kg, every 15 days, for 12 months). Fludarabine resulted in a significantly higher overall and complete remission rate (72% vs 51%, P = .003; 7% vs 0%, P = .011). Time to treatment failure was significantly shorter in the chlorambucil arm (11 vs 18 months; P = .004), but no difference in progression-free survival time was observed (19 months with fludarabine, 18 months with chlorambucil; P = .7). Moreover, fludarabine did not increase the overall survival time (46 months in the fludarabine vs 64 months in the chlorambucil arm; P = .15). Taken together, the results suggest that in elderly CLL patients the first-line therapy with fludarabine alone does not result in a major clinical benefit compared with chlorambucil. This trial is registered with www.isrctn.org under identifier ISRCTN 36294212.


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