bluuebunny/crossref_metadata_2025_split huggingface dataset:
Left ventricle diastolic dysfunction in idiopathic inflammatory myopathies: A meta-analysis and systematic review
Authors: Zhang Yiwen , Yang Xiaoqian , Qin Li , Luo Qiang , Wang Han 
Published: October 2021

ABSTRACT

Objectives
Recent studies have confirmed that heart failure is one of the most important causes of death in patients with idiopathic inflammatory myopathy (IIM). Left ventricle diastolic dysfunction (LVDD) is closely associated with heart failure. Our aim is to determine if the prevalence of LVDD is increased in IIM patients.

Methods
We performed a time- and language-restricted literature search to identify studies conducted to compare the echocardiographic parameters in IIM patients and controls. Mean differences were used to calculate the effect sizes of the echocardiographic parameters.

Results
A total of 13 studies met the inclusion criteria and comprised a total of 227 juvenile dermatomyositis (JDM) patients, 391 adult IIM patients, and 550 controls. The adult IIM patients had lower mitral annular early diastolic velocity (eʹ) and peak of early diastolic flow velocity/peak of late diastolic flow velocity (E/A) ratio compared to controls. The mean left atrial dimension and E/eʹ ratio was higher in adult IIM patients as compared to controls. Similarly, in JDM patients, the decreased eʹ was also observed.

Conclusion
Patients with IIM were more likely to have echocardiographic parameters indicative of diastolic dysfunction. The early heart assessments should be performed in IIM patients.
Ultrasonography of IgG4-related dacryoadenitis and sialadenitis: Imaging features and clinical usefulness
Authors: Komori Takahiro , Inoue Dai , Izumozaki Akira , Sugiura Takumi , Terada Kanako , Yoneda Norihide , Toshima Fumihito , Yoshida Kotaro , Kitao Azusa , Kozaka Kazuto , Takahira Masayuki , Kawano Mitsuhiro , Kobayashi Satoshi , Gabata Toshifumi 
Published: October 2021

ABSTRACT

Objectives
To clarify the ultrasonographic features of immunoglobulin G4 (IgG4)-related dacryoadenitis and sialadenitis (IgG4-DS) and their usefulness in clinical diagnostic sessions.

Methods
By re-evaluating 96 consecutive patients with IgG4-related disease, we identified 54 patients (male:female = 37:17; median age, 69.5 years) who underwent lacrimal or submandibular gland (LG or SG, respectively) ultrasonography and computed tomography (CT). Their clinical and ultrasonographic features were retrospectively analysed. Radio-pathological correlations were also examined in LG (23 cases) and SG lesions (20 cases). Additionally, the diagnostic accuracy of CT for LG/SG lesions was evaluated.

Results
Abnormal ultrasonographic findings were detected in 33 (LGs) and 38 (SGs) patients, and most of them were observed bilaterally. All lesions were well demarcated and demonstrated diffuse low-echoic areas (rocky pattern) or multiple low-echoic nodules surrounded by high-echoic linear shadows (cobblestone pattern) corresponding to intra-lobular inflammation and inter-lobular fibrosis. Moreover, 42% (LGs; 14/33) and 42% (SGs; 16/38) patients had glandular lesions without clinical symptoms associated with the affected glands. The diagnostic accuracy of CT was ∼80% for LG and 55% for SG.

Conclusions
Ultrasonographic findings in IgG4-DS included diffuse or nodular low-echoic areas with linear high-echoic structures corresponding to inflamed lobules and inter-lobular fibrosis. These findings can help detect IgG4-DS.
A pooled analysis of serious infections and herpes zoster-related disease in Asian patients with rheumatoid arthritis treated with peficitinib (ASP015K) over a median of 3 years
Authors: Tanaka Yoshiya , Takeuchi Tsutomu , Kato Daisuke , Kaneko Yuichiro , Fukuda Musashi , Izutsu Hiroyuki , Rokuda Mitsuhiro 
Published: October 2021

ABSTRACTObjectiveTo analyse serious infection (SI) and herpes zoster-related disease (HZD) during long-term treatment of rheumatoid arthritis with the oral Janus kinase inhibitor, peficitinib (ASP015K).MethodsThis was a post hoc analysis of pooled data from one Phase 2b study and two Phase 3 studies and final data from a long-term extension study of peficitinib in Asian rheumatoid arthritis patients. Two pooled datasets were analysed (Phase 3 studies and Phase 2/3 studies). Univariate and multivariate Cox regression analyses explored relationships between exposure-adjusted incidence rate of SI and HZD, peficitinib dose, and baseline factors.ResultsTotal peficitinib exposure for 1052 patients receiving once-daily peficitinib in the pooled Phase 2/3 Asian studies was 2998.9 patient-years. Exposure-adjusted incidence rates (95% confidence interval) of SI and HZD were 2.7 (2.2, 3.4) and 6.9 (6.0, 8.0) per 100 patient-years, respectively, in pooled Phase 2/3 studies. Advanced age was prognostic for SI and HZD, while baseline prednisolone dose was prognostic for SI. There was no temporal relationship between either adverse event and prolonged peficitinib administration.ConclusionsAs expected in this peficitinib-treated population, older patients had increased risk of SI and HZD, and those receiving higher prednisolone doses had increased risk of SI.
Otitis media with ANCA-associated vasculitis: A retrospective study of 30 patients
Authors: Tabei Akifumi , Sakairi Toru , Ohishi Yuko , Watanabe Mitsuharu , Nakasatomi Masao , Hamatani Hiroko , Ikeuchi Hidekazu , Kaneko Yoriaki , Hiromura Keiju 
Published: October 2021

ABSTRACT

Objectives
Otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) is a new category of otitis media in which cases of otitis media due to ANCA-associated vasculitis (AAV) are classified, regardless of ANCA variant or ANCA serotype. We aimed to describe the clinical features and course of patients with OMAAV and identify factors associated with hearing outcomes.

Methods
We retrospectively analysed 30 patients with OMAAV, classified based on the criteria proposed by the Japan Otological Society in 2016.

Results
Single-positive myeloperoxidase-ANCA, single-positive proteinase 3-ANCA, double-positive ANCA, and double-negative ANCA were identified in 47%, 33%, 7%, and 13% of the patients, respectively. All patients subjected to audiometry showed hearing impairments, and 85% were affected bilaterally. Mixed- and sensorineural-type hearing impairments were identified in 80% and 20% of impaired ears, respectively. Hypertrophic pachymeningitis (HPM) was identified in 37% of the patients. Immunosuppressive therapy was administered to 93% of patients, and the median air conduction hearing levels at pre- and post-treatment were 66.1 dB and 43.4 dB, respectively, indicating significant hearing improvements. HPM and a long interval between disease onset and treatment initiation were significantly correlated with poor hearing prognosis.

Conclusions
OMAAV develops under any type of ANCA-serology and typically causes mixed or sensorineural bilateral hearing loss. The early initiation of immunosuppressive therapy and the absence of HPM were associated with good hearing outcomes.
Epidemiology and clinical characteristics of systemic sclerosis overlap syndrome (SSc-OS), and the factors significantly associated with SSc-OS in Thai patients with systemic sclerosis
Authors: Jantarat Apichart , Muangchan Chayawee 
Published: October 2021

ABSTRACT

Objective
To investigate the epidemiology and characteristics of systemic sclerosis (SSc) overlap syndrome (SSc-OS).

Methods
This study included patients enrolled in the Siriraj Systemic Sclerosis Cohort registry during November 2013 to September 2019. SSc-OS was defined as SSc patients who also met criteria for rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), dermatomyositis (DM), polymyositis (PM), Sjogren, and/or antiphospholipid antibody syndrome. Baseline and 1-year characteristics were compared between SSc and SSc-OS.

Results
185 patients (age 50.3 ± 11.4 years, 85.4% female, disease duration 2.7 years, 75.1% diffuse cutaneous subset, 75.6% anti-Scl-70 positivity) were included. The incidence and prevalence rate of SSc-OS was 3.2/100 patient-years and 17.8%. Regarding SSc-OS, 12.4%, 2.2%, 1.1%, 1.6%, and 0.5% of patients were classified as SSc-RA, SSc-SLE, SSc-PM, SSc-RA-SLE, and SSc-SLE-PM. SSc-OS had a higher prevalence of limited cutaneous subset (lcSSc), usual interstitial pneumonia, finger contractures, ESR &gt;20 mm/hr., globulin &gt;3.5 g/dL, rheumatoid factor, anti-citrullinated peptide antibody, and antiphospholipid antibodies. LcSSc subset (OR: 11.3, 95%CI: 2.0-62.6) and globulin &gt;3.5 g/dL (OR: 6.2, 95%CI: 1.6–23.6) were associated with SSc-OS.

Conclusion
SSc-OS is associated with the lcSSc subset. RA is the most common overlap syndrome. LcSSc patients with globulin &gt;3.5 g/dL are associated with SSc-OS.
BrainApp: Using near-patient sensing through a mobile app and machine learning in brain tumour patients
Authors: Anwar Nur Aizaan , Williams Matthew 
Published: October 2021

Abstract

Aims
1. To assess the feasibility, acceptability, and performance of a mobile app, 'BRIAN', developed by The Brain Tumour Charity (BTC), in collecting data on quality of life (QOL), activity and sleep, for predicting disease progression in adult brain tumour patients.
2. To generate a prospectively collected dataset of patient measures obtained through mobile devices in brain tumour patients and healthy volunteers.
3. To assess compliance and performance of micro-challenges (hand coordination, visual memory, speech and facial features) in study participants using a mobile application.
4. To assess differences and systematic variation in micro-challenge performance between healthy volunteers and brain tumour patients.
5. To assess factors associated with micro-challenge performance in brain tumour patients, the relationship between micro-challenges and standard measures of QoL and disease progression.
6. To assess the diagnostic performance of different machine learning models in detecting brain tumour progression.

Method
This abstract describes the protocol for a multi-centre observational non-randomised phase II trial for adult brain tumour patients and healthy volunteers in the UK. Participants will use the BRIAN mobile app, developed by BTC to help individuals cope with a brain tumour, and share their journey with both researchers and clinicians. Participants will be required to enter information on their medical background, mood, and QOL; have the option to link fitness trackers to the app; as well as perform mini-games which assess speech, coordination, facial features and reaction time. Patients will have their brain imaging and histopathology report submitted to the sponsor. We will then investigate the correlation and temporal relation of these multimodal data with conventional measures of disease progression. We will use traditional statistical methods initially (i.e. descriptive statistics and multilevel modelling), which will then inform the development of a machine learning model in predicting brain tumour progression.

Results
The BRIAN app is currently being beta-tested by healthy volunteers from the Computational Oncology Lab, Imperial College London. We are in the process of obtaining ethical approval for the trial.

Conclusion
This study may enable the development of a tool that allows us to detect earlier signs of disease progression, and so offer earlier treatment and preservation of quality of life; and hence the best course of action. Such a tool would also be non-invasive, cheap, quick, and can be used by patients in the comfort of their own homes.
GlioCova: Treatment and hospital admissions for patients with GBM in England
Authors: Mauricaite Radvile , Calvez Kerlann Le , Williams Matthew 
Published: October 2021

Abstract

Aims
Data on the treatment and outcomes of patients with primary brain tumours in England is sparse. The GlioCova project uses linked national data from England to explore the incidence, treatment, outcomes, and treatment costs of all adult brain tumour patients in all 50,000 patients in England from 2013 – 2018. Here we present initial results from patients with glioblastoma (GBM).

Method
We used a linked dataset from the national cancer registration system in England including all adult patients diagnosed with a malignant or benign brain tumour between 2013 and 2018 (51,775 patients in total).
Glioblastoma patients were selected based on ICD-10 codes (C70, C71, C72), morphology codes (9440, 9441, 9442), and grade (G4, G3, GX and NA) from the national cancer registry. We extracted data on treatment (radiotherapy, chemotherapy, brain surgery or biopsy) and measured how many patients who had adjuvant Temozolomide completed 6 cycles.

Results
We identified 15,294 glioblastoma patients. Most had glioblastoma morphology (14,924), followed by gliosarcoma (264) and giant cell glioblastoma (106). Almost all had a cranial tumour (C71) while 17 had a tumour originating in the spinal cord, cranial nerves or other part of central nervous system (C72). Median age was 66 (IQR=17) and 60% were male. 51.9% (7,935) underwent surgery; an additional 18.2% (2,784) had a biopsy; 3,701 (24.2%) out of 15,294 patients received radiotherapy (only) and 316 (2.1%) received chemotherapy (only). 5,520 (36.1%) received both radiotherapy and chemo. Out of 4,101 GBM patients receiving temozolomide after radiotherapy, only 1,535 (37.4%) completed 6 cycles. The 7,935 GBM patients who had surgery had a median length of stay in hospital of 5 days (IQR=6) while those that had a biopsy had a median of 3 days (IQR=6).

Conclusion
We have presented a description of treatment of all GBM patients in England over a five-year period. This is the first time we have been able to understand detailed treatment patterns at a national scale, and significantly extends previous analyses. Further work will look at patient safety indicators, variation across centres and costs of treatment.

Acknowledgements
We would like to thank the GlioCova Expert Advisory Group for their input and discussion.
This work uses data provided by patients and collected by the NHS as part of their care and support.
Efficacy and Safety of CyberKnife Stereotactic Radiosurgery in Acromegaly
Authors: Seguna Desiree , Akker Scott , Ahlquist James , Pal Aparna , Brooke Antonia , Lewis Rachel , Plowman P N , Evanson Jane , Panday Pratistha , Drake William Martyn 
Published: October 2021

Abstract

Aims
Objective: Active acromegaly is associated with increased mortality. While surgery is the mainstay of treatment, it is not always curative. In selected cases, CyberKnife stereotactic radiosurgery (CK SRS) can be used as adjuvant treatment in patients with persistent disease.

Method
Methodology: Biochemical response was measured using serum IGF-1 levels, calculated as a percentage of the upper limit of normal (% ULN). Levels were recorded prior to treatment, at 6-12 months post-treatment and at the most recent follow-up. Anterior pituitary hormone deficits were assessed before and after treatment. Tumour size was followed-up using MRI.

Results
10 patients (7 male, mean age 36 yrs [+/- 12.6, SD]) with acromegaly were treated with CK SRS. 9 were treated following failure to attain biochemical remission with TSS. 1 had primary CK SRS. 2 had previous conventional fractionated external beam radiotherapy.
Median tumour diameter was 6 mm (IQR 5.2-10.5 mm), with cavernous sinus invasion in 2 cases. The dose was 20-24Gy/1#. 4 patients were on dopamine agonist, 4 on somatostatin analogue and 2 on pegvisomant. Mean follow-up 31.6 months (+/- 13.5 months, SD).
Median IGF-1 % ULN was 146% pre-treatment (IQR 126.5-208.5), 109% at 6-12 months (IQR 76.5-131%) and 71% (IQR 59-91%) at last follow-up. Mean radiological follow-up 16.6 months (+/- 15.9 months, SD). No cases showed tumour enlargement.
One patient developed secondary hypothyroidism. Side-effects: headache (7 patients), blurred vision (1 patient), fatigue/nausea (1 patient). No new visual fields defects, cranial nerve palsies, cerebrovascular events or secondary tumours.

Conclusion
Conclusions: CK SRS appears safe and effective in selected patients with acromegaly, when there is failure to attain biochemical cure with surgery and in patients intolerant or resistant to medical treatment.
Cranial Meningiomas Requiring Cranioplasty
Authors: Norrington Max , Millward Christopher , Doherty John , Mustafa Mohammad , Humphries Thomas , Gillespie Conor , Richardson George , Islim Abdurrahman , Jenkinson Michael , Brodbelt Andrew 
Published: October 2021

Abstract

Aims
Bone infiltration in association with intracranial meningioma (4.5% of cases) and primary intraosseous meningioma (2%) are rare. Management can be challenging, as cranial vault reconstruction may be required. This study aimed to examine the surgical techniques used and outcomes in this patient population.

Method
A single-centre, retrospective cohort study was conducted between January 2010 and September 2020. All adult patients who required cranial reconstruction due to bone involvement of their meningioma were included. Patient demographics, tumour characteristics, operative details, complications, and outcomes were examined. Statistical analyses were performed using SPSS v24.0.

Results
There were 30 patients (17 female; 56.7%), median age 54 yrs (range 28-86 yrs), of whom 25 (83.3%) had bone infiltration, and 5 (16.7%) had primary intraosseous meningioma. Only 10 patients had a Simpson I or II resection. Twenty-eight had 'on-table' primary cranioplasties. Materials used were titanium (n=13; 43.3%), acrylic (n=10; 33.3%), PMMA (n=5; 16.7%), and hydroxyapatite (n=2; 6.7%). There were 9 (mostly minor) surgical complications and only one wound infection. Twelve patients had WHO grade II tumours, and 14 required radiotherapy. Ten patients (33.3%) had re-operation for recurrent tumour, with a median time to progression of 41 months. At 6 months, 24 patients had a performance score less than 2.

Conclusion
On-table cranioplasty provides a lower risk surgical option for patients with high risk meningiomas.
An audit on the diagnosis of primary CNS lymphoma
Authors: Joe Dorothy , Yin Lucia , Kassam Shireen , Cikurel Katia , Lavrador Jose-Pedro , Vergani Francesco , Gullan Richard , Bhangoo Ranjeev , Ashkan Keyoumars , Finnerty Gerald 
Published: October 2021

Abstract

Aims
Primary central nervous system lymphoma (PCNSL) is a rare form of non–Hodgkin lymphoma with exclusive manifestations in the central nervous system, leptomeninges and eyes. It forms around 5% of all primary brain tumours. It is an aggressive tumour which has a poor prognosis if left untreated. It is imperative that diagnosis is made timely so treatment can be started promptly. Therefore, we performed an audit looking into the speed of diagnostic process of PCNSL in our tertiary Neuro–oncology Unit.

Method
Single-centre retrospective review of PCNSL cases referred to a tertiary Neuro–Oncology Unit over a six month period from June to November 2020.

Results
A total of 1309 cases were discussed in the Neuro–oncology MDT meeting over the study period. Fourteen cases (6 male, 8 female; median age [range] 66 [59–83] years) were identified as highly likely PCNSL. Neuroimaging suggested PCNSL as the likely diagnosis in twelve patients. Twelve patients were started on steroids after CT or MRI brain scans. Nine patients had a surgical target and proceeded to have diagnostic brain biopsy. Two patients had different working diagnoses and three patients were deemed unsuitable for brain surgery. One patient required repeat brain biopsy. A tissue diagnosis was made in twelve patients. One patient deteriorated rapidly and one patient had a brain lesion that was deemed too high risk for surgery. The median time between neuroimaging and biopsy was 25 days. The median time taken from first investigation to the pathological confirmation of PCNSL was 36 days (range 6–86 days).

Conclusion
The chief reason for delay in diagnosis of PCNSL was that patients were started on steroids before diagnostic investigations were completed. Steroids caused the brain lesions to become smaller or disappear. Accordingly, time was needed to allow withdrawal of steroids before diagnostic investigations could be repeated. Diagnostic delays may have been exacerbated by logistical issues associated with COVID–19. We propose that there needs to be greater awareness of how early introduction of steroids can markedly delay the diagnosis of PCNSL.
US-Focused Conceptual Health Care Decision-Analytic Models Examining the Value of Pivmecillinam Relative to Current Standard-of-Care Agents Among Adult Patients With Uncomplicated Urinary Tract Infections due to Enterobacterales
Authors: Lodise Thomas P , Henriksen Anne Santerre , Hadley Thomas , Patel Nimish 
Published: October 2021

Abstract

Background
Pivmecillinam is approved for the treatment of adults with uncomplicated urinary tract infection (uUTI) in Canada and Europe and is pending United States (US) Food and Drug Administration submission for consideration for approval. US-focused health care decision-analytics were developed to define the value of an agent like pivmecillinam relative to current standard-of-care (SOC) agents among adult patients with Enterobacterales uUTIs based on its improved microbiologic activity against common Enterobacterales.

Methods
The model population was 100 theoretical adult outpatients with Enterobacterales uUTIs under 4 different uUTI first-line empiric treatment scenarios (ie, pivmecillinam, nitrofurantoin, trimethoprim-sulfamethoxazole [SXT], or fluoroquinolones). The total mean uUTI-related 30-day costs, including inappropriate treatment costs, were calculated for each regimen. The range of pivmecillinam regimen costs that conferred cost savings relative to the current SOC agents based on its potentially improved microbiologic activity against common Enterobacterales was determined.

Results
The 30-day uUTI-related costs associated with nitrofurantoin, SXT, and fluoroquinolones were $655.61, $687.57, and $659.69, respectively. The pivmecillinam neutral regimen cost thresholds that resulted in the same uUTI-related 30-day per-patient costs for nitrofurantoin, SXT, and fluoroquinolones were $83.50, $115.45, and $87.58, respectively. The overall antimicrobial susceptibility improvement required with pivmecillinam fixed at $200/regimen, for it to be cost savings relative to SOC agents, was 28%.

Conclusions
The analyses suggests that an agent like pivmecillinam, if approved in the US, has the potential to reduce the economic burden associated with inappropriate treatment of adult outpatients with uUTIs, especially in patients at high risk for an Enterobacterales uUTI that is resistant to SOC agents.