Treatment and outcomes of POEMS syndrome: changes in the past 20 years.
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ABSTRACT:
SUBMITTER: Yu YY
PROVIDER: S-EPMC8364551 | biostudies-literature | 2021 Aug
REPOSITORIES: biostudies-literature
Publications
Treatment and outcomes of POEMS syndrome: changes in the past 20 years.
Yu Yan-Ying YY Gao Xue-Min XM Zhao Hao H Cai Hao H Feng Jun J Zhang Lu L Cao Xin-Xin XX Zhou Daobin D Li Jian J
Blood cancer journal 20210814 8
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Project description:BackgroundFor decades, open surgical repair was the only available method to treat pectus excavatum (PE). In 1998 Donald Nuss described first time a minimally invasive repair of pectus excavatum (MIRPE), and today MIRPE is performed with increasing frequency worldwide. However, despite its minimally invasive approach, with the widespread use of MIRPE the character and number of complications have increased. 20 years ago, non-surgical measures such as vacuum bell therapy (VBT) were introduced as a useful complement for specific treatment of PE patients. However, until today there are no existing validated guidelines concerning VBT.MethodsThe study includes the results of an online survey submitted to the members of Chest Wall International Group (CWIG), a selective review of the English spoken current literature with focus on VBT and an analysis of our own previous studies concerning VBT.ResultsSeventy-two percent of the CWIG members practicing in 47 different institutions confirmed to use VBT for PE patients. Furthermore, within the last 10 to 15 years an increasing number of studies were identified reporting on successful use of VBT for PE. However, a recently published study stated that the effect of VBT is predominantly because of thickening of the chest wall by increasing pre-sternal adipose tissue. There was one study comparing VBT to MIRPE but no randomized and/or prospective studies comparing conservative treatment vs. surgical repair or conservative treatment vs. no specific therapy. Variables predictive of an excellent outcome could be identified. Especially in younger PE patients, VBT is reported with increasing frequency in the survey as well as in the literature. However, validated guidelines concerning VBT are still not available.ConclusionsNon-operative treatment of PE with VBT proved to be safe and a potential alternative to surgical repair in carefully selected PE patients. Patient's age at diagnosis and severity of the PE represents relevant variables to decide which kind of therapy might be successful to correct PE. Especially in PE patients under the age of 10 years, VBT seems to represent the first step of specific therapy.
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Project description:OBJECTIVES:A bibliometric approach using network analysis was applied to identify the development and research trends for moxibustion. This study also examined the network hub of moxibustion research by investigating the collaborative work of organizations and authors. METHODS:Academic articles on moxibustion research published from 2000 to 2019 were retrieved from the Web of Science database. Extracted records were analyzed according to publication year, research area, journal title, country, organization, and authors. The VOSviewer program was utilized to visualize the trends in moxibustion research and to explore the influential organizations and authors. RESULTS:Analyses of 1146 original and review articles written in English demonstrated that the number of publications related to moxibustion research has increased consistently over the last 20 years. China issued the most articles in this field, and the most represented research area was integrative complementary medicine. A network analysis based on the co-occurrence and publication year of keywords identified the relevant characteristics and trends of moxibustion research. By assessing the total link strength of organizations and authors, influential organizations and authors who have contributed to moxibustion research were identified. CONCLUSIONS:The current study examined research on moxibustion using bibliometric analysis and identified a time-based development of moxibustion research and a global network hub of moxibustion research.
| S-EPMC7288119 | biostudies-literature
Project description:ObjectivePolyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome is a rare multisystem disease associated with a plasma-cell dyscrasia. Although pachymeningeal involvement has occasionally been described, MRI of the central nervous system (CNS) has not yet been extensively investigated.MethodsWe retrospectively evaluated CNS MRI in Europe's largest single-center cohort of POEMS syndrome. Of 77 patients who have been formally diagnosed with POEMS, 41 had MRI brain and 29 had MRI spine. A control group of 33 patients with chronic inflammatory demyelinating polyneuropathy (CIDP) was used as this is the major differential diagnosis. Of these CIDP patients, 12 underwent both MRI brain and spine, 7 had solely MRI brain and 14 had MRI spine.ResultsIn 41 POEMS patients with MRI brain, we identified frequent smooth, diffuse meningeal thickening of the cerebral convexities and falx (n = 29, 71%), of which 4 had meningeal collections. 17 (41%) had vascular abnormalities including white-matter disease, of which 4 had established infarcts. Of 29 patients with MRI spine, 17 (59%) had thickening of the brachial and lumbosacral plexus. Conversely in 19 CIDP patients with MRI brain, none had meningeal thickening (p < 0.0001); however, 8 (42%) had vascular abnormalities (p = 0.85). Of 26 patients with MRI spine, 9 (35%) had brachial or lumbosacral plexus thickening (p = 0.06).ConclusionsIn contrast to CIDP, POEMS patients frequently have pachymeningeal thickening. Vascular abnormalities and plexus thickening were also common but not statistically different to CIDP.
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Project description:Patient-reported outcome measures engage patients in disease severity measurement and the metrics reported can be meaningful to their lives. The Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes syndrome (POEMS) is a complex multisystem disorder with disabling neuropathy which is distinct from other acquired inflammatory neuropathies. No current POEMS-specific validated disability scales exist. To address this, we have produced a Rasch-built overall disability scale (RODS) specific to POEMS. A 146-item preliminary questionnaire containing relevant activity and participation items for neuropathic disability was applied to 49 clinically stable patients with POEMS from the UK national POEMS cohort. A total of 123 items not fulfilling Rasch model expectations were sequentially removed. The final 23-item POEMS-RODS fulfilled Rasch model expectations and showed acceptable test-retest reliability. The 23-item POEMS-RODS is a disease-specific patient-reported outcome measure able to detect activity limitations within the range of ability demonstrated by the UK POEMS cohort. Larger international studies are needed to confirm the broader applicability and responsiveness of this scale in other countries.
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Project description:BackgroundThe epidemiology of pathogenic bacteria varies according to the socioeconomic status and antimicrobial resistance status. However, longitudinal epidemiological studies to evaluate the changes in species distribution and antimicrobial susceptibility of pathogenic bacteria nationwide are lacking. We retrospectively investigated the nationwide trends in species distribution and antimicrobial susceptibility of pathogenic bacteria over the last 20 years in Korea.MethodsFrom 1997 to 2016, annual cumulative antimicrobial susceptibility and species distribution data were collected from 12 university hospitals in five provinces and four metropolitan cities in South Korea.ResultsThe prevalence of Staphylococcus aureus was the highest (13.1%) until 2012 but decreased to 10.3% in 2016, consistent with the decrease in oxacillin resistance from 76.1% in 2008 to 62.5% in 2016. While the cefotaxime resistance of Escherichia coli increased from 9.0% in 1997 to 34.2% in 2016, E. coli became the most common species since 2013, accounting for 14.5% of all isolates in 2016. Pseudomonas aeruginosa and Acinetobacter baumannii rose to third and fifth places in 2008 and 2010, respectively, while imipenem resistance increased from 13.9% to 30.8% and 0.7% to 73.5% during the study period, respectively. Streptococcus agalactiae became the most common pathogenic streptococcal species in 2016, as the prevalence of Streptococcus pneumoniae decreased since 2010. During the same period, pneumococcal penicillin susceptibility decreased to 79.0%, and levofloxacin susceptibility of S. agalactiae decreased to 77.1% in 2016.ConclusionThe epidemiology of pathogenic bacteria has changed significantly over the past 20 years according to trends in antimicrobial resistance in Korea. Efforts to confine antimicrobial resistance would change the epidemiology of pathogenic bacteria and, consequently, the diagnosis and treatment of infectious diseases.
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Project description:POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy, presence of monoclonal proteins (M-protein), and skin changes. Even though the pathophysiology is poorly understood, recent studies suggest that both clonal and polyclonal plasmacytosis leading to the production of pro-inflammatory cytokines and angiogenic mediators play the central role. These mediators including vascular endothelial growth factor (VEGF) are the driving forces of the syndrome. The diagnosis of POEMS is not always straight forward and often the diagnosis is delayed. It is based on fulfilling mandatory criteria of polyradiculoneuropathy and monoclonal protein and the presence of one major criterion (Castleman disease, sclerotic bone lesions, or elevated VEGF), and at least one minor criterion. Due to the presence of neuropathy, it can be confused with chronic inflammatory demyelinating polyradiculopathy (CIDP), and if thrombocytosis and splenomegaly are present, it can be confused with myeloproliferative neoplasms. Due to the rarity of the syndrome, clear guidelines for treatment are still lacking. Immediate treatment targeting the underlying plasma cell proliferation results in a dramatic response in most patients. The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients. For patients with localized bony disease, radiotherapy has proven to be very effective. Lenalidomide and dexamethasone is a proven therapy in patients ineligible for ASCT. In this review article, we tackle the diagnostic approach and discuss the latest treatment modalities of this rare debilitating disease.
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Project description:POEMS syndrome is a rare clonal plasma cell disease. Patients with POEMS syndrome are at risk of developing pulmonary hypertension, but the data on its incidence and impact on outcome are limited. We reviewed records of 154 POEMS syndrome patients with complete duplex echocardiography data for estimation of pulmonary artery systolic pressure (sPAP) at the time of diagnosis. Forty-two (27%) of 154 patients with pulmonary hypertension (estimated sPAP ≥50 mmHg) were identified. Median age was 46 years (range 31-71 years). Patients with pulmonary hypertension were more likely to have peripheral edema (P=0.04), ascites (P=0.02), pleural effusion (P=0.005), and have longer time from onset to diagnosis (P=0.004) when compared with those without pulmonary hypertension. Restrictive abnormalities and decreased diffusion capacity of carbon monoxide were observed in 83% and 96% patients with pulmonary hypertension, compared with 50% and 72% in patients without pulmonary hypertension, respectively. Reversibility of pulmonary hypertension was observed after treatment of POEMS syndrome. After median follow of 32 months, survival of patients with pulmonary hypertension was worse than those without (median overall survival 54 months vs. median not reached, P=0.021). In conclusion, pulmonary hypertension is a common feature of POEMS syndrome, and is associated with signs of extravascular volume overload. Although active treatment of POEMS syndrome can reverse pulmonary hypertension, survival of these patients is worse than those without pulmonary hypertension.
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Project description:BackgroundThe results of several large randomized controlled trials (RCTs) have changed the clinical practice of bariatric surgery. However, the characteristics of global RCTs of bariatric surgery have not been reported internationally and whether there was research waste in these RCTs is unknown.MethodsSearch ClinicalTrials.gov for bariatric surgery RCTs registered between January 2000 and December 2022 with the keywords 'Roux-en-Y gastric-bypass' and 'Sleeve Gastrectomy'. The above analysis was conducted in January 2023.ResultsA total of 326 RCTs were included in this study. The number of RCTs registered for sleeve gastrectomy and gastric bypass surgery increased year by year globally. Europe has always accounted for the largest proportion, Asia has gradually increased, and North America has decreased. A total of 171 RCTs were included in the analysis of waste, of which 74 (43.8%) were published. Of the 74 published RCTs, 37 (37/74, 50.0%) were judged to be adequately reported and 36 (36/74, 48.6%) were judged to have avoidable design defects. In the end, 143 RCTs (143/171, 83.6%) had at least one research waste. Body weight change as the primary endpoint (OR: 0.266, 95% CI: 0.103-0.687, P =0.006) and enrolment greater than 100 (OR: 0.349, 95% CI: 0.146-0.832, P =0.018) were independent protective factors for research waste.ConclusionsThis study for the first time describes the characteristic changes of the mainstream RCT of bariatric surgery globally in the last 20 years and identifies a high research waste burden and predictive factor in this area, which provides reference evidence for carrying out bariatric surgery RCTs more rationally.
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Project description:Full-length immunoglobulin high-throughput sequencing in POEMS syndrome
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Project description:Tyrosine kinases are implicated in tumorigenesis and progression, and have emerged as major targets for drug discovery. Tyrosine kinase inhibitors (TKIs) inhibit corresponding kinases from phosphorylating tyrosine residues of their substrates and then block the activation of downstream signaling pathways. Over the past 20 years, multiple robust and well-tolerated TKIs with single or multiple targets including EGFR, ALK, ROS1, HER2, NTRK, VEGFR, RET, MET, MEK, FGFR, PDGFR, and KIT have been developed, contributing to the realization of precision cancer medicine based on individual patient's genetic alteration features. TKIs have dramatically improved patients' survival and quality of life, and shifted treatment paradigm of various solid tumors. In this article, we summarized the developing history of TKIs for treatment of solid tumors, aiming to provide up-to-date evidence for clinical decision-making and insight for future studies.
| S-EPMC7590700 | biostudies-literature