231 Outcomes of Cochlear Implantation in Patients with Waardenburg Syndrome: A Systematic Review and Narrative Synthesis

2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
A Lovett ◽  
M Eastwood ◽  
C Metcalfe ◽  
J Muzaffar ◽  
P Monksfield ◽  
...  

Abstract Aim This systematic review aims to establish outcomes of cochlear implantation (CI) in patients with a diagnosis of Waardenburg syndrome (WS). Method A systematic review and narrative synthesis were undertaken. Databases searched: Medline, PubMed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. No limits placed on language or year of publication. A review conducted in accordance with the PRISMA statement. Results Searches identified 160 abstracts and 157 full texts. Of these, 22 studies met inclusion criteria reporting outcomes in 191 patients and at least 209 implants. Hearing outcomes of those receiving cochlear implantation were generally good. Five studies included genetic analysis of one or more of the participants. A total of 10 intra- or post-operative complications were reported. The methodological quality of included studies was modest, mainly comprising case reports and non-controlled case series with small cohort size. All studies were OCEBM grade III-IV. Conclusions Hearing outcomes following CI in Waardenburg syndrome are generally good with the majority of patients experiencing improvement in audiometry, speech perception, and speech intelligibility. Cochlear implant teams must use their clinical expertise to assess individual patients’ needs in order to perform cochlear implantation at the optimum age and provide thorough rehabilitation to maximise implantation benefits.

2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
C M Lam ◽  
H L Cornwall ◽  
A Chaudhry ◽  
J Muzaffar ◽  
M Bance ◽  
...  

Abstract Introduction In otosclerosis, spongiotic bone replaces normal bone in the ossicular chain. Focal deposits may also be found within the cochlea leading to sensorineural hearing loss refractory to conventional treatment. Cochlear implantation can play an important role in the management of otosclerosis in these patients. Our study objective is to establish hearing outcomes following cochlear implantation in patients with otosclerosis. Method Systematic review and narrative synthesis. Databases searched: Medline, PubMed, Embase, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Review conducted in accordance with the PRISMA statement. Results Searches identified 68 studies meeting the inclusion criteria and reporting outcomes in a minimum of 481 patients with at least 516 implants. The methodological quality of included studies was modest, predominantly consisting of case reports and non-controlled case series with small numbers of patients. Significant heterogeneity existed in terms of outcomes and methods of reporting, which precluded a meta-analysis. Access to good rehabilitation support is essential to achieving the good hearing outcomes and Patient Reported Outcome Measures that can be expected by 12 months post-implantation in most cases. There was significant association between the radiological severity of otosclerosis and an increase in surgical and post-operative complications. Post-operative facial nerve stimulation can occur and may require deactivation of electrodes and subsequent hearing detriment. Conclusions Hearing outcomes are typically good, but patients should be counselled on associated surgical complications which may compromise hearing. Modern diagnostic techniques may help to identify potentially difficult cases to aid operative planning and patient counselling.


Author(s):  
Mansour Tobaiqy ◽  
Mohammed Qashqary ◽  
Shrooq Al-Dahery ◽  
Alaa Mujallad ◽  
Almonther Abdullah Hershan ◽  
...  

AbstractBackgroundSARS-CoV-2 is the cause of the COVID-19 that has been declared a global pandemic by the WHO in 2020. The COVID-19 treatment guidelines vary in each country, and yet there is no approved therapeutic for COVID-19.Aims of the studythis review aimed to report any evidence of therapeutics used for the management of COVID-19 patients in clinical practice since the emergence of the virus.MethodsA systematic review protocol was developed based on PRISMA Statement. Articles for review were selected from electronic databases (Embase, Medline and Google Scholar). Readily accessible peer-reviewed full articles in English published from December 1 st, 2019 to March 26 th, 2020 were included. The search terms included combinations of: COVID, SARS-COV-2, glucocorticoids, convalescent plasma, antiviral, antibacterial. There were no restrictions on the type of study design eligible for inclusion.ResultsAs of March 26, 2020, of the initial manuscripts identified (n=449) articles. Forty-one studies were included, of which clinical trials (n=3), (case reports n=7), case series (n=10), retrospective (n=11) and prospective (n=10) observational studies. Thirty-six studies were conducted in China (88%).The most common mentioned and reported medicine in this systematic review was corticosteroids (n=25), followed by Lopinavir (n=21) and oseltamivir (n=16).ConclusionsThis is the first systematic review up to date related to the therapeutics used in COVID-19 patients. Only forty-one research articles on COVID-19 and therapeutics were found eligible to be included, most conducted in China, corticosteroid therapy was found to be the most used medicine in these studies.


VASA ◽  
2010 ◽  
Vol 39 (1) ◽  
pp. 43-53 ◽  
Author(s):  
Grotenhermen

Background: To investigate the hypothesis that cases of arteritis similar to thromboangiitis obliterans (TAO) and associated with the use of cannabis were caused by cannabis or THC (dronabinol), or that cannabis use is a co-factor of TAO. Patients and methods: A systematic review on case reports and the literature on so-called cannabis arteritis, TAO, and cardiovascular effects of cannabinoids was conducted. Results: Fifteen reports with 57 cases of an arteritis associated with the use of cannabis and two additional case series of TAO, in which some patients also used cannabis, were identified. Clinical and pathological features of cannabis-associated arteritis do not differ from TAO and the major risk factor of TAO, tobacco use, was present in most, if not in all of these cases. The proposed pathophysiological mechanisms for the development of an arteritis by cannabis use are not substantiated. Conclusions: The hypothesis of cannabis being a causative factor or co-factor of TAO or an arteritis similar to TAO is not supported by the available evidence. The use of the term “cannabis arteritis” should be avoided until or unless more convincing scientific support is forthcoming.


2019 ◽  
Vol 24 (5) ◽  
pp. 558-571 ◽  
Author(s):  
Kartik Bhatia ◽  
Hans Kortman ◽  
Christopher Blair ◽  
Geoffrey Parker ◽  
David Brunacci ◽  
...  

OBJECTIVEThe role of mechanical thrombectomy in pediatric acute ischemic stroke is uncertain, despite extensive evidence of benefit in adults. The existing literature consists of several recent small single-arm cohort studies, as well as multiple prior small case series and case reports. Published reports of pediatric cases have increased markedly since 2015, after the publication of the positive trials in adults. The recent AHA/ASA Scientific Statement on this issue was informed predominantly by pre-2015 case reports and identified several knowledge gaps, including how young a child may undergo thrombectomy. A repeat systematic review and meta-analysis is warranted to help guide therapeutic decisions and address gaps in knowledge.METHODSUsing PRISMA-IPD guidelines, the authors performed a systematic review of the literature from 1999 to April 2019 and individual patient data meta-analysis, with 2 independent reviewers. An additional series of 3 cases in adolescent males from one of the authors’ centers was also included. The primary outcomes were the rate of good long-term (mRS score 0–2 at final follow-up) and short-term (reduction in NIHSS score by ≥ 8 points or NIHSS score 0–1 at up to 24 hours post-thrombectomy) neurological outcomes following mechanical thrombectomy for acute ischemic stroke in patients < 18 years of age. The secondary outcome was the rate of successful angiographic recanalization (mTICI score 2b/3).RESULTSThe authors’ review yielded 113 cases of mechanical thrombectomy in 110 pediatric patients. Although complete follow-up data are not available for all patients, 87 of 96 (90.6%) had good long-term neurological outcomes (mRS score 0–2), 55 of 79 (69.6%) had good short-term neurological outcomes, and 86 of 98 (87.8%) had successful angiographic recanalization (mTICI score 2b/3). Death occurred in 2 patients and symptomatic intracranial hemorrhage in 1 patient. Sixteen published thrombectomy cases were identified in children < 5 years of age.CONCLUSIONSMechanical thrombectomy may be considered for acute ischemic stroke due to large vessel occlusion (ICA terminus, M1, basilar artery) in patients aged 1–18 years (Level C evidence; Class IIb recommendation). The existing evidence base is likely affected by selection and publication bias. A prospective multinational registry is recommended as the next investigative step.


BMJ Open ◽  
2020 ◽  
Vol 10 (12) ◽  
pp. e042350
Author(s):  
Maximilian Sohn ◽  
Ayman Agha ◽  
Igors Iesalnieks ◽  
Anna Tiefes ◽  
Alfred Hochrein ◽  
...  

IntroductionAcute diverticulitis of the sigmoid colon is increasingly treated by a non-operative approach. The need for colectomy after recovery from a flare of acute diverticulitis of the left colon, complicated diverticular abscess is still controversial. The primary aim of this study is to assess the risk of interval emergency surgery by systematic review and meta-analysis.Methods and analysisThe systematic review and meta-analysis will be conducted in accordance to the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols statement. PubMed/MEDLINE, Cochrane Central Register of Controlled Trials and EMBASE will be screened for the predefined searching term: (Diverticulitis OR Diverticulum) AND (Abscess OR pelvic abscess OR pericolic abscess OR intraabdominal abscess) AND (surgery OR operation OR sigmoidectomy OR drainage OR percutaneous drainage OR conservative therapy OR watchful waiting). All studies published in an English or German-speaking peer-reviewed journal will be suitable for this analysis. Case reports, case series of less than five patients, studies without follow-up information, systematic and non-systematic reviews and meta-analyses will be excluded. Primary endpoint is the rate of interval emergency surgery. Using the Review Manager Software (Review Manager/RevMan, V.5.3, Copenhagen, The Nordic Cochrane Centre, The Cochrane Collaboration, 2012) meta-analysis will be pooled using the Mantel-Haenszel method for random effects. The Risk of Bias in Non-randomized Studies of Interventions tool will be used to assess methodological quality of non-randomised studies. Risk of bias in randomised studies will be assessed using the Cochrane developed RoB 2-tool.Ethics and disseminationAs no new data are being collected, ethical approval is exempt for this study. This systematic review is to provide a new insight on the need for surgical treatment after a first attack of acute diverticulitis, complicated by intra-abdominal or pelvic abscesses. The results of this study will be presented at national and international meetings and published in a peer-reviewed journal.PROSPERO registration numberCRD42020164813.


Author(s):  
Anna K. Dietl ◽  
Matthias W. Beckmann ◽  
Konrad Aumann

Abstract Purpose Villoglandular adenocarcinoma (VGA) of the uterine cervix has been classified as a rare subtype of cervical adenocarcinoma with good prognosis. A conservative surgical approach is considered feasible. The main risk factor is the presence of other histologic types of cancer. In this largest systematic review to date, we assess oncological outcomes associated with conservative therapy compared to those associated with invasive management in the treatment of stage Ia and Ib1 VGA. Methods Case series and case reports identified by searching the PubMed database were eligible for inclusion in this review (stage Ia–Ib1). Results A total of 271 patients were included in our literature review. 54 (20%) patients were treated by “conservative management” (conization, simple hysterectomy, and trachelectomy) and 217 (80%) by “invasive management” (radical hysterectomy ± radiation, hysterectomy, and radiation). Recurrences of disease (RODs) were found in the conservative group in two (4%) cases and in the invasive group in nine (4%) cases. There was no significant difference in disease-free survival (DFS) according to conservative or invasive treatment (p = 0.75). The histology of VGA may be complex with underlying usual adenocarcinoma (UAC) combined with VGA. Conclusion The excellent prognosis of pure VGA and the young age of the patients may justify the management of this tumor using a less radical procedure. The histological diagnosis of VGA is a challenge, and pretreatment should not be based solely on a simple punch biopsy but rather a conization with wide tumor-free margins.


Author(s):  
Julien Favresse ◽  
Jean-Louis Bayart ◽  
Damien Gruson ◽  
Sergio Bernardini ◽  
Aldo Clerico ◽  
...  

Abstract Cardiac troponins (cTn) are the preferred biomarkers for the evaluation of myocardial injury and play a key role in the diagnosis of acute myocardial infarction (MI). Pre-analytical or analytical issues and interferences affecting troponin T and I assays are therefore of major concern given the risk of misdiagnosis. False positive troponin results have been related to various interferences including anti-troponin antibodies, heterophilic antibodies, or elevated alkaline phosphatase level. On the other hand, false negative results have been reported in the case of a large biotin intake. These interferences are characterized with erroneous but reproducible troponin results. Of interest, non-reproducible results have also been reported in the literature. In other words, if the sample is reanalyzed a second time, a significant difference in troponin results will be observed. These interferences have been named “fliers” or “outliers”. Compared to the biotin interference that received major attention in the literature, troponin outliers are also able to induce harmful clinical consequences for the patient. Moreover, the prevalence of outliers in recent studies was found to be higher (0.28–0.57%) compared to the biotin interference. The aim of this systematic review is to warn clinicians about these non-reproducible results that may alter their clinical judgment. Four case reports that occurred in the Clinique of Saint-Luc Bouge are presented to attest this point. Moreover, we aimed at identifying the nature of these non-reproducible troponin results, determining their occurrence, and describing the best way for their identification.


Author(s):  
Amaan Javed

Background: The current ongoing COVID-19 pandemic has compelled us to scrutinize major outbreaks in the past two decades, severe acute respiratory syndrome (SARS), in 2002, and Middle East respiratory syndrome (MERS), in 2012. We aimed to assess the associated neurological manifestations with SARS CoV-2 infection. Methods: In this systematic review, a search was carried out by key-electronic databases, controlled vocabulary, and indexing of trials to evaluate the available pertinent studies which included both medical subject headings (MeSH) and advance electronic databases comprising of PubMed, Embase, Scopus, Cochrane Central Register of Controlled Trials (CENTRAL). Peer-reviewed studies published in English and Spanish were considered which reported data on the neurological associations of individuals with suspected or laboratory-confirmed SARS-CoV-2 infection. Outcomes were nervous signs or symptoms; symptom severity; and diagnoses. Findings: Our search identified 45 relevant studies, with 21 case reports, 3 case series, 9 observational studies, 1 retrospective study, 9 retrospective reviews, and 2 prospective reviews. This systematic review revealed that most commonly reported neuronal presentations involved headache, nausea, vomiting and muscular symptoms like fibromyalgia. Anosmia and ageusia, defects in clarity or sharpness of vision (error in visual acuity), and pain may occur in parallel. Notable afflictions in the form of anxiety, anger, confusion, post-traumatic stress symptoms, and post-intensive care syndrome were observed in individuals who were kept in quarantine and those with long-stay admissions in healthcare settings. SARS CoV-2 infection may result in cognitive impairment. Patients with more severe infection exhibited uncommon manifestations, such as acute cerebrovascular diseases (intracerebral haemorrhage, stroke), rhabdomyolysis, encephalopathy, Guillain-Barré syndrome. Interpretation: SARS-CoV-2 patients experience neuronal presentations varying with the progression of the infection. Healthcare professionals should be acquainted with the divergent neurological symptoms and to curb misdiagnosis and limit long term sequelae. Health-care planners and policymakers must prepare for this eventuality, while the ongoing studies increase our knowledge base on acute and chronic neurological associations of this pathogen.


2018 ◽  
Vol 79 (05) ◽  
pp. 408-415 ◽  
Author(s):  
Jan Victor ◽  
Thibault Poujade ◽  
Nadine Hollevoet ◽  
Gregoire Chick

Aims To identify the frequency of reports of sporadic schwannomatosis, the types of patients affected, and the nerves affected. Patients and Methods We identified all case reports and case series that reported on patients with sporadic schwannomatosis according to established criteria. Results The initial search yielded 1,597 studies, of which 15 were included. A total of 38 of 55 individuals met the inclusion criteria. The mean age of the patients was 48 years; 41% were male. Thirty-three patients had peripheral nerve tumors, and 17 had spinal tumors. Twelve had tumors in both locations. Tumor distribution was unilateral in 25 of 30 cases (83.3%) and segmental (limited to one limb or five or fewer contiguous segments of the spine) in 28 of 38 cases (73.7%). Conclusion This systematic review quantified the number of individuals who meet the criteria for sporadic schwannomatosis and better described this population to facilitate the classification of neurofibromatosis in regard to the 2005 consensus statement. Unilateral or segmental distribution of nerve tumors are key aspects when dealing with multiple noncutaneous schwannomas without involvement of the vestibular nerve.


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