scholarly journals When the Good Syndrome Goes Bad: A Systematic Literature Review

2021 ◽  
Vol 12 ◽  
Author(s):  
Yiyun Shi ◽  
Chen Wang

BackgroundGood syndrome is a rare adult-onset immunodeficiency characterized by thymoma and hypogammaglobulinemia. Its clinical manifestations are highly heterogeneous, ranging from various infections to autoimmunity.ObjectiveThis study was to summarize patient characteristics, identify prognostic factors and define clinical subgroups of Good syndrome.MethodsA systematic literature review was conducted to include patients with Good syndrome identified in PubMed, Embase and Cochrane databases between January 2010 and November 2020. Logistic and Cox regressions were used to identify prognostic factors impacting outcomes. Clinical subgroups were defined by multiple correspondence analysis and unsupervised hierarchical clustering. A decision tree was constructed to characterize the subgroup placement of cases.ResultsOf 162 patients included in the current study, the median age at diagnosis was 58 years and 51% were male. Type AB was the most common histological subtype of thymoma, and infections as well as concurrent autoimmune disorders were identified in 92.6% and 51.2% patients, respectively. Laboratory workup showed typical findings of combined immunodeficiency. Thymoma status (odds ratio [OR] 4.157, confidence interval [CI] 1.219-14.177, p = 0.023), infections related to cellular immunity defects (OR 3.324, 95% CI 1.100-10.046, p = 0.033), infections of sinopulmonary tract (OR 14.351, 95% CI 2.525-81.576, p = 0.003), central nerve system (OR 6.403, 95% CI 1.205-34.027, p = 0.029) as well as bloodstream (OR 6.917, 95% CI 1.519-31.505, p = 0.012) were independent prognostic factors. The 10-year overall survival was 53.7%. Cluster analysis revealed three clinical subgroups with distinct characteristics and prognosis (cluster 1, infections related to cellular immunity defects; cluster 2, infections related to other immunity defects; cluster 3, infections related to humoral and phagocytic immunity defects). A decision tree using infection types (related to humoral and cellular immunity defects) could place patients into corresponding clusters with an overall correct prediction of 72.2%.ConclusionsInfection type and site were the main prognostic factors impacting survival of patients with Good syndrome. We identified three subgroups within Good syndrome associated with distinct clinical features, which may facilitate the study of underlying pathogenesis as well as development of targeted therapy.

2018 ◽  
Vol 109 ◽  
pp. 307-327 ◽  
Author(s):  
Ming-Xiang Zou ◽  
Guo-Hua Lv ◽  
Qian-Shi Zhang ◽  
Shao-Fu Wang ◽  
Jing Li ◽  
...  

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1863-1863 ◽  
Author(s):  
Kevin B Knopf ◽  
Mei Sheng Duh ◽  
Marie-Hélène Lafeuille ◽  
Jonathan Gravel ◽  
Patrick Lefebvre ◽  
...  

Abstract Abstract 1863 Introduction: Bortezomib is approved for the treatment of MM, and bortezomib-based therapies are a cornerstone of care for both first-line and relapsed and/or refractory MM. As bortezomib is administered for a finite course, as opposed to treatment being continued to disease progression, patients may remain sensitive to bortezomib-based therapy at relapse. A number of prospective and retrospective studies in patients with relapsed and/or refractory MM have demonstrated the efficacy of subsequent retreatment with bortezomib-containing regimens. We report a systematic literature review and meta-analysis to assess the efficacy and safety of bortezomib-based retreatment in relapsed and/or refractory MM. Methods: Using PubMed and major hematology/oncology conference proceedings, a systematic literature review was performed to identify studies from January 2005 to May 2012 of bortezomib-based therapy in patients with relapsed and/or refractory MM. All studies of bortezomib-based retreatment in MM patients with prior exposure to bortezomib were systematically selected. Treatments were classified as bortezomib ± dexamethasone (dex) or bortezomib-based combinations. The proportion of bortezomib-refractory patients was identified for each study where available. Additional prognostic factors, including number of prior therapies, proportion of patients with prior autologous stem cell transplant (ASCT), and time since last bortezomib, were extracted and used in weighted stratified analyses of time to progression (TTP), progression-free survival (PFS), and overall survival (OS). Random-effect pooled estimates (taking into account heterogeneity across studies) were calculated for overall response rate (ORR; partial response or better) and rates of common adverse events (AEs). A meta-regression was conducted to explore the interactive effects of prognostic factors on ORR. Results: Twenty-three studies (N=1051 patients) were identified. Bortezomib was administered IV in all studies. Retreatment comprised bortezomib ± dex in 5 studies and combination therapy in 18. Bortezomib-refractory patients were included in 11 studies (10–100% of patients), 6 studies included only relapsed patients, and data were not available for 6 studies. Across studies in which data were available, the pooled, weighted average ORR was 39% (95% CI: 31–47) and the pooled, weighted average median TTP, PFS, and OS were 7.5, 5.8, and 16.6 months, respectively. Data from stratified univariate analyses are shown in the table. Outcomes appeared generally consistent across groups while patients with fewer prior therapies (≤4) and relapsed (but not refractory) patients achieved higher ORRs of 43% (95% CI: 31–55) and 57% (95% CI: 45–69), respectively. Random-effects meta-regression analysis controlling for number of prior therapies and % prior ASCT confirmed that, compared to refractory patients, relapsed patients were associated with a higher ORR by 28–41 percentage points. In studies of bortezomib ± dex in patients with a median age >65 years and with a lower % of patients receiving prior ASCT (≤30%), retreatment remained active (pooled average ORR of 51%, median TTP and OS of 8.4 and 19.2 months). The most common grade 3/4 AEs included thrombocytopenia (35%), neutropenia (15%), anemia (14%), pneumonia (10%), and peripheral neuropathy (3%). Conclusions: Based on these findings, retreatment with bortezomib alone or in combination is efficacious and well tolerated in relapsed patients, with an ORR of 57% and median TTP and OS of 8.5 and 19.7 months. In an era of new and emerging treatment options for relapsed and/or refractory MM, these data indicate bortezomib retreatment remains a highly effective therapeutic option in previously treated patients. Disclosures: Knopf: Millennium Pharmaceuticals, Inc.: Consultancy. Duh:Millennium Pharmaceuticals, Inc.: Research Funding. Lafeuille:Millennium Pharmaceuticals, Inc.: Research Funding. Gravel:Millennium Pharmaceuticals, Inc.: Research Funding. Lefebvre:Millennium Pharmaceuticals, Inc.: Research Funding. Niculescu:Millennium Pharmaceuticals, Inc.: Employment. Ba-Mancini:Millennium Pharmaceuticals, Inc.: Employment. Ma:Millennium Pharmaceuticals, Inc.: Employment. Shi:Millennium Pharmaceuticals, Inc.: Employment. Comenzo:Millennium Pharmaceuticals, Inc.: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1408.3-1409
Author(s):  
T. Ito ◽  
S. Fukui ◽  
T. Kanie ◽  
T. Nakai ◽  
G. Kidoguchi ◽  
...  

Background:Coronary periarteritis is one of the clinical manifestations of IgG4-related disease. It can cause serious conditions such as angina and ruptured aneurysms. Therefore, it is important to recognize the clinical and radiological characteristics, which was little known.Objectives:We report four patients with IgG4-related coronary periarteritis with a systematic literature review.Methods:We identified four patients with IgG4-related coronary periarteritis at the St. Luke’s International Hospital in Tokyo, Japan from 2014 to 2020. A systematic literature review was conducted for English articles on IgG4-related coronary periarteritis cases with a full text or abstract available. We summarized patient demographics, IgG and IgG4 titers, the site and morphological type of coronary lesion, and other organ involvements.Results:Our 4 cases and 38 cases identified by the literature review were assessed. Coronary artery lesions were detected by a coronary CT in all but two cases. Wall thickening was the most common type of the lesion. Moreover, there were 32 (76.1%) patients with other organ involvements. The commonest other lesion was peri-aortitis in 21 (50.0%) patients. In cases with peri-aortitis, IgG and IgG4 titers were significantly higher than those without peri-aortitis (IgG4; 1540 [705.0, 2570.0] vs 246.0 [160.0, 536.3]; p = 0.001, IgG; 3596.5 [2838.3, 4260.0] vs 1779.0 [1288.3, 1992.8]; p =0.040). In addition, 15 (71.4%) patients of them had three or more IgG4 related organ involvements.Conclusion:Coronary CT was a useful imaging modality for the diagnosis of IgG4-related coronary periarteritis, and wall thickening was the most common lesion. Moreover, about half cases coexisted with peri-aortitis. Peri-aortitis and other organ involvements should be screened in those with higher IgG and IgG4.Table 1.Characteristics of our cases and the literature review cases.RCA: right coronary artery, LAD: left anterior descending artery, LCx: left circumflex arteryDisclosure of Interests:None declared


2020 ◽  
Vol 7 (1) ◽  
pp. 63
Author(s):  
Solikin Solikin

Abstrak: Penelitian dengan melakukan tinjauan literatur sistematis (Sistematic Literatur Review-SLR) dilakukan untuk mempelajari berbagai teknik identifikasi penyakit pada daun dengan citra digital  sebagai tahapan untuk mendapatkan pemahaman mengenai teknik identifikasi penyakit pada daun mangga dengan citra digital. Produksi Mangga di Indonesia dari tahun 2014 – 2018 secara fluktuatif selalu mengalami peningkatan dan di tahun 2018 produksi mangga di Indonesia mencapai 2.624.783 ton, proses budidaya tanaman mangga tidak selamanya dapat terlepas dari serangan penyakit. Penyakit pada tanaman mangga disebabkan oleh jamur atau bakteri yang biasanya menyerang pada bagian akar, batang, kulit batang, ranting atau buah mangga. Jenis penyakit pada tanaman mangga adalah : Penyakit mangga (Jamur Gloesoporium), Penyakit Diplodia, Cendawan jelaga, Bercak karat merah, Kudis buah, Penyakit Blendok. Penyakit pada mangga memiliki berbagai gejala dan kadang sulit didiagnosis oleh petani dan untuk itu diperlukan keahlian untuk mendiagnosis penyakit pada tanaman mangga dan bagaimana cara penanggulangannya yang biasanya keahlian tersebut terdapat pada ahli patologi tanaman professional. Sehingga dibutuhkan suatu Teknologi IT dengan Sistem Cerdas yang dirancang untuk dapat mengidentifikasi secara otomatis penyakit tanaman mangga dan cara penanggulangannya berdasarkan gejala visual dengan menggunakan metode citra digital. Metode literatur review yang digunakan yaitu Compare, Contrast, Criticize, Synthesize dan Summarize. Metode Citra Digital yang dapat digunakan dalam identifikasi penyakit pada daun mangga adalah tahapan Image Acquisition, Preprocessing , Segmentation, Ekstraksi Fitur, Seleksi Fitur. Metode Klasifikasi yang dapat digunakan adalah SVM, Artificial Neural Network, Decision Tree, Convolutional Neural Network.   Kata kunci: citra digital, daun, penyakit mangga, tinjauan literatur sistematis     Abstract: Research by conducting a systematic literature review (Systematic Literature Review-SLR) was conducted to study various techniques of disease identification in leaves with digital images as a stage to gain an understanding of the techniques for disease identification on mango leaves with digital images. Mango production in Indonesia from 2014 - 2018 fluctuations has always increased and in 2018 mango production in Indonesia reached 2,624,783 tons, the process of mango cultivation is not always free from disease. Diseases of mango plants are caused by fungi or bacteria that usually attack the roots, stems, bark, twigs or mangoes. Types of diseases in mango plants are: Mango disease (Gloesoporium Fungus), Diplodia disease, sooty fungus, red rust spots, fruit scabies, Blendok disease. Diseases of mangoes have a variety of symptoms and are sometimes difficult to diagnose by farmers and expertise is needed to diagnose diseases on mango plants and how to overcome them which are usually found in professional plant pathologists. So that we need an IT Technology with an Intelligent System that is designed to be able to automatically identify mango plant diseases and how to overcome them based on visual symptoms using digital image methods. The literature review method used is Compare, Contrast, Criticize, Synthesize and Summarize. Digital image methods that can be used in the identification of diseases on mango leaves are the stages of Image Acquisition, Preprocessing, Segmentation, Feature Extraction, Feature Selection. Classification methods that can be used are SVM, Artificial Neural Network, Decision Tree, Convolutional Neural Network.


2021 ◽  
Author(s):  
Jailma Januário da Silva ◽  
Norton Trevisan Roman

In this article, we present a systematic literature review, carried out from February to March 2020, on the application of a machine learning technique to predict student dropout in higher education institutions. Besides describing the protocol followed during our research, which includes the research questions, searched databases and query strings, along with criteria for inclusion and exclusion of articles, we also present our main results, in terms of the attributes used by current research on this theme, along with adopted approaches, specific algorithms, and evalution metrics. The Decision Tree technique is the most used for the construction of models, and accuracy and recall and precision being the most used metric for evaluating models.


2011 ◽  
Vol 71 (3) ◽  
pp. 319-326 ◽  
Author(s):  
Zoe Ash ◽  
Cécile Gaujoux-Viala ◽  
Laure Gossec ◽  
Elizabeth MA Hensor ◽  
Oliver FitzGerald ◽  
...  

ObjectivesTo review the available evidence for the efficacy and safety of non-steroidal anti-inflammatory drugs (NSAIDs), synthetic and biological drug therapies for the different clinical manifestations of psoriatic arthritis (PsA) in order to provide data for the development of treatment recommendations by the European League Against Rheumatism (EULAR) taskforce.MethodsA systematic literature review (SLR) of available treatments for PsA was performed using the largest electronic databases (MEDLINE, EMBASE and COCHRANE) by two working groups formed within the EULAR taskforce. This comprised a comprehensive sample of rheumatologists, dermatologists, epidemiologists and patients. The available evidence was reviewed for NSAIDs, synthetic disease modifying antirheumatic drugs (DMARDs), local and systemic corticosteroids and biologic drugs. All articles and abstracts published between 1962 and January 2010 were reviewed and considered and a meta-analysis of data on biological therapies was performed.ResultsWhile little data are available on NSAIDs, glucocorticoids and synthetic DMARDs, the available evidence suggests an acceptable efficacy and safety profile of both NSAIDs and synthetic DMARDs (methotrexate, cyclosporine A, sulfasalazine and leflunomide) in PsA. More evidence is available (level 1B) supporting the efficacy of anti-tumour necrosis factor (anti-TNF) agents (adalimumab, etanercept, golimumab and infliximab) in treating the signs and symptoms of PsA as well as reducing radiographic progression. Registry data show no new safety concerns, although the numbers studied to date are relatively small.ConclusionsThis SLR reveals some evidence to support the use of NSAIDs and synthetic DMARDs and good evidence for the efficacy of anti-TNF therapy in PsA.


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