Various case reports have described sudden sensorineural hearing loss (SSNHL) in patients with the 2019 novel coronavirus disease (COVID-19) caused by the severe acute respiratory syndrome ...coronavirus 2 (SARS-CoV-2). Our aim was to determine the incidence of COVID-19 in patients with SSNHL.
All consecutive patients with audiometric confirmed SSNHL between November 2020 and March 2021 in a Dutch large inner city teaching hospital were included. All patients were tested for COVID-19 by polymerase-chain-reaction (PCR) and awaited the results in quarantine.
Out of 25 patients, zero (0%) tested positive for COVID-19. Two patients had previously tested positive for COVID-19: at three and eight months prior to the onset of hearing loss.
This is the largest series to date investigating COVID-19 in SSNHL patients. In this series there is no apparent relationship between SSNHL and COVID-19.
In the Lancet, Guy N Rutty and colleagues6 report a prospective study about the value of PMCTA in natural sudden death. For this post-mortem imaging study, the primary outcome for large prospective ...series was the accuracy of cause of death, if identifiable on PMCTA compared with the autopsy (which they class as gold standard).
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
Abstract Objective The fenestrated Anaconda endograft (Vascutek, Renfrewshire, Scotland) was introduced in 2010 and showed promising short-term results with high technical success and low morbidity ...rates. The aim of this study was to present the midterm results, with a minimum of 12 months follow-up, for all patients treated with the fenestrated Anaconda endograft in The Netherlands. Methods Patients treated with the fenestrated Anaconda endograft between May 2011 and February 2015 were included. Follow-up consisted of computed tomography angiography at 1 month and 1 year, and duplex ultrasound yearly thereafter with additional computed tomography angiography if indicated using a standard protocol. Results A total of 60 patients were included; 48 patients (80.0%) were treated for juxtarenal aneurysms, and 12 (20.0%) were short-neck infrarenal aneurysms. Mean aneurysm size was 64 ± 9 mm. A total of 140 fenestrations were incorporated. Median follow-up was 16.4 months (interquartile range, 11.9-27.4). The 30-day mortality was 3.4% (n = 2). Kaplan-Meier estimates for 1-year, 2-year, and 3-year survival were 91.4%, 89.5%, and 86.3%, respectively, without aneurysm-related mortality during follow-up. Main body primary and secondary endograft patencies were 98.3% and 100%, respectively. Target vessel primary and secondary patencies were 95.0% and 98.6%, respectively. Early type IA endoleaks occurred in seven patients (11.7%) and spontaneously resolved in all patients. At 1-year follow-up 4 (6.7%) type II endoleaks persisted. One patient experienced aneurysm rupture because of a late type III endoleak attributable to a dislodged renal stent and subsequently underwent successful conversion to open surgery. Conclusions The fenestrated Anaconda is a viable treatment option for complex abdominal aortic aneurysms. Acceptable mortality and morbidity and low reintervention rates contribute to good midterm results. Occurrence of early type I endoleak was relatively common, but these resolved spontaneously in all patients.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Dysostosis multiplex is a progressive skeletal disease in patients with mucopolysaccharidosis type I (MPS I). One of its key features is thoracolumbar kyphosis and no guideline for surgical treatment ...is available yet. Therefore, we investigated the natural course of kyphosis and initiated an international consensus procedure with the aim to develop the first clinical practice guideline for the management of kyphosis in MPS I patients. Sequential radiographs (WKZ/UMC Utrecht and Amsterdam UMC, the Netherlands Royal Manchester Children’s Hospital, United Kingdom San Gerardo Hospital, Italy) were obtained and measurements included the kyphotic angle (modified Cobb angle (CA) and posterior angle (PA), both developed by us for measurement of kyphosis in dysplastic vertebrae) and spondylolisthesis. A modified Delphi procedure was used to develop statements with an international expert panel including 10 spinal/orthopedic surgeons, 6 metabolic pediatricians and 3 physiotherapists, all experienced in MPS I. The procedure consisted of 3 written rounds and a face-to-face meeting. In the natural history study 44 patients (233 radiographs) were included. Intra- and inter-rater reliability was ≥ 0.9 for all measurements. The median modified CA was larger in Hurler patients (38°) compared to non-Hurler patients (9°) and an abnormal modified CA was present at a younger age. A modeled trend showed a progression of the modified CA of 1.8°/year for Hurler patients. The modified CA and the PA correlated strongly. Spondylolisthesis was present in 34 patients. During the modified Delphi procedure 16 statements on decision for surgery and goals of treatment were developed. Consensus was reached on all statements. In conclusion, we present an extensive radiographic assessment of thoracolumbar kyphosis in MPS I patients showing kyphosis is progressive over time. These natural history data may be important for future studies. In addition, we present the first clinical practice guideline for management of kyphosis in MPS I patients.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Objective: We studied the interaction between valproate (VPA) and ethosuximide (ESM) in diminishing the incidence of absence-like spike-wave discharges (SWDs) in the EEG of WAG/Rij rats.
Methods: ...VPA, ESM, their combination and saline were evaluated in 16 rats. The doses of VPA ranged from 0 to 280
mg/kg and the doses of ESM ranged from 0 to 40
mg/kg. For the drug combination, a fixed weight ratio of 7/1 VPA/ESM was used. The incidence of SWDs in the EEG was determined for the period of 15–75
min after injection and compared to the incidence of SWDs prior to injection. The sigmoid-
E
max equation was fitted to the data. Isobolic analysis, on 50% effect, was used to assess the character of the drug interaction.
Results: The parameters for diminishing the incidence of the SWDs were: VPA: ED
50: 121
mg/kg; ESM: ED
50: 21.5
mg/kg; VPA/ESM: ED
50: 112/16
mg/kg. Isobolic analysis showed that a higher drug load was needed of the combination than of the individual drugs to achieve a 50% reduction of SWDs: factor 1.67;
P=0.012.
Conclusion: The interaction between valproate and ethosuximide was shown to be infra-additive in diminishing the incidence of SWDs in WAG/Rij rats.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
In mid September 2008, clinical signs of bluetongue (particularly coronitis) were observed in cows on three different farms in eastern Netherlands (Luttenberg, Heeten, and Barchem), two of which had ...been vaccinated with an inactivated BTV-8 vaccine (during May-June 2008). Bluetongue virus (BTV) infection was also detected on a fourth farm (Oldenzaal) in the same area while testing for export. BTV RNA was subsequently identified by real time RT-PCR targeting genome-segment (Seg-) 10, in blood samples from each farm. The virus was isolated from the Heeten sample (IAH “dsRNA virus reference collection” dsRNA-VRC isolate number NET2008/05) and typed as BTV-6 by RT-PCR targeting Seg-2. Sequencing confirmed the virus type, showing an identical Seg-2 sequence to that of the South African BTV-6 live-vaccine-strain. Although most of the other genome segments also showed very high levels of identity to the BTV-6 vaccine (99.7 to 100%), Seg-10 showed greatest identity (98.4%) to the BTV-2 vaccine (RSAvvv2/02), indicating that NET2008/05 had acquired a different Seg-10 by reassortment. Although Seg-7 from NET2008/05 was also most closely related to the BTV-6 vaccine (99.7/100% nt/aa identity), the Seg-7 sequence derived from the blood sample of the same animal (NET2008/06) was identical to that of the Netherlands BTV-8 (NET2006/04 and NET2007/01). This indicates that the blood contained two different Seg-7 sequences, one of which (from the BTV-6 vaccine) was selected during virus isolation in cell-culture. The predominance of the BTV-8 Seg-7 in the blood sample suggests that the virus was in the process of reassorting with the northern field strain of BTV-8. Two genome segments of the virus showed significant differences from the BTV-6 vaccine, indicating that they had been acquired by reassortment event with BTV-8, and another unknown parental-strain. However, the route by which BTV-6 and BTV-8 entered northern Europe was not established.
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DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Abstract
Background
Accurate risk prediction is needed in order to provide personalized healthcare for chronic kidney disease (CKD) patients. An overload of prognosis studies is being published, ...ranging from individual biomarker studies to full prediction studies. We aim to systematically appraise published prognosis studies investigating multiple biomarkers and their role in risk predictions. Our primary objective was to investigate if the prognostic models that are reported in the literature were of sufficient quality and to externally validate them.
Methods
We undertook a systematic review and appraised the quality of studies reporting multivariable prognosis models for end-stage renal disease (ESRD), cardiovascular (CV) events and mortality in CKD patients. We subsequently externally validated these models in a randomized trial that included patients from a broad CKD population.
Results
We identified 91 papers describing 36 multivariable models for prognosis of ESRD, 50 for CV events, 46 for mortality and 17 for a composite outcome. Most studies were deemed of moderate quality. Moreover, they often adopted different definitions for the primary outcome and rarely reported full model equations (21% of the included studies). External validation was performed in the Multifactorial Approach and Superior Treatment Efficacy in Renal Patients with the Aid of Nurse Practitioners trial (n = 788, with 160 events for ESRD, 79 for CV and 102 for mortality). The 24 models that reported full model equations showed a great variability in their performance, although calibration remained fairly adequate for most models, except when predicting mortality (calibration slope >1.5).
Conclusions
This review shows that there is an abundance of multivariable prognosis models for the CKD population. Most studies were considered of moderate quality, and they were reported and analysed in such a manner that their results cannot directly be used in follow-up research or in clinical practice.
The effects of single or multiple concordant HPV infections at various anatomical sites on type-specific HPV seropositivity are currently unknown. In this cross-sectional study we assessed whether ...high-risk HPV infections at various anatomical sites (i.e., anal canal, penile shaft, and oral cavity), as well as concordant infections at multiple anatomical sites, were associated with type-specific seropositivity in HIV-positive and HIV-negative MSM. MSM aged ≥ 18 years were recruited in Amsterdam, the Netherlands (2010-2011). Baseline anal, penile, and oral samples were analyzed for HPV DNA and genotyped using a highly sensitive PCR and reverse line blot assay. Virus-like particle (VLP) based multiplex immunoassay was used to asses HPV-specific serum antibodies against L1 VLPs. The associations between HPV infections and type-specific seropositivity of seven high-risk HPV types (7-hrHPV: types 16, 18, 31, 33, 45, 52, 58) were estimated using logistic regression analyses with generalized estimating equations. We found that 86% of 306 HIV-positive MSM and 62% of 441 HIV-negative MSM were seropositive for at least one 7-hrHPV type. 69% of HIV-positive and 41% of HIV-negative MSM were infected with at least one 7-hrHPV type at the anus, penis, or oral cavity. In multivariable analyses, 7-hrHPV seropositivity was associated with type-specific anal (and not penile) 7-hrHPV infection, and did not significantly increase with a higher number of infected anatomical sites. Oral 7-hrHPV infection showed a positive, albeit non-significant, association with seropositivity. In conclusion, seropositivity among MSM appears to be largely associated with anal HPV infection, irrespective of additionally infected anatomical sites.
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DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Radiation therapy for head and neck cancer frequently leads to salivary gland damage and subsequent xerostomia. The radiation response of the parotid glands of rats, mice, and patients critically ...depends on dose to parotid gland stem cells, mainly located in the gland's main ducts (stem cell rich SCR region). Therefore, this double-blind randomized controlled trial aimed to test the hypothesis that parotid gland stem cell sparing radiation therapy preserves parotid gland function better than currently used whole parotid gland sparing radiation therapy.
Patients with head and neck cancer (n = 102) treated with definitive radiation therapy were randomized between standard parotid-sparing and stem cell sparing (SCS) techniques. The primary endpoint was >75% reduction in parotid gland saliva production compared with pretreatment production (FLOW12M). Secondary endpoints were several aspects of xerostomia 12 months after treatment.
Fifty-four patients were assigned to the standard arm and 48 to the SCS arm. Only dose to the SCR regions (contralateral 16 and 11 Gy P = .004 and ipsilateral 26 and 16 Gy P = .001 in the standard and SCS arm, respectively) and pretreatment patient-rated daytime xerostomia (35% and 13% P = .01 in the standard and SCS arm, respectively) differed significantly between the arms. In the SCS arm, 1 patient (2.8%) experienced FLOW12M compared with 2 (4.9%) in the standard arm (P = 1.00). However, a trend toward better relative parotid gland salivary function in favor of SCS radiation therapy was shown. Moreover, multivariable analysis showed that mean contralateral SCR region dose was the strongest dosimetric predictor for moderate-to-severe patient-rated daytime xerostomia and grade ≥2 physician-rated xerostomia, the latter including reported alteration in diet.
No significantly better parotid function was observed in SCS radiation therapy. However, additional multivariable analysis showed that dose to the SCR region was more predictive of the development of parotid gland function–related xerostomia endpoints than dose to the entire parotid gland.
Simultaneous analysis of multiple genes using next‐generation sequencing (NGS) technology has become widely available. Copy‐number variations (CNVs) in disease‐associated genes have emerged as a ...cause for several hereditary disorders. CNVs are, however, not routinely detected using NGS analysis. The aim of this study was to assess the diagnostic yield and the prevalence of CNVs using our panel of Hereditary Thoracic Aortic Disease (H‐TAD)‐associated genes. Eight hundred ten patients suspected of H‐TAD were analyzed by targeted NGS analysis of 21 H‐TAD associated genes. In addition, the eXome hidden Markov model (XHMM; an algorithm to identify CNVs in targeted NGS data) was used to detect CNVs in these genes. A pathogenic or likely pathogenic variant was found in 66 of 810 patients (8.1%). Of these 66 pathogenic or likely pathogenic variants, six (9.1%) were CNVs not detectable by routine NGS analysis. These CNVs were four intragenic (multi‐)exon deletions in MYLK, TGFB2, SMAD3, and PRKG1, respectively. In addition, a large duplication including NOTCH1 and a large deletion encompassing SCARF2 were detected. As confirmed by additional analyses, both CNVs indicated larger chromosomal abnormalities, which could explain the phenotype in both patients. Given the clinical relevance of the identification of a genetic cause, CNV analysis using a method such as XHMM should be incorporated into the clinical diagnostic care for H‐TAD patients.
The aim of this study was to assess the diagnostic yield and the prevalence of copy number variants in patients suspected of hereditary thoracic aortic disease (H‐TAD). A pathogenic or likely pathogenic variant was found in 66 of 810 patients (8.1%) and six out of these 66 likely pathogenic or pathogenic variants (9.1%) were copy number variants. Given the clinical relevance of identification of a genetic cause, copy number variant analysis should be incorporated into the clinical diagnostics of patients suspected of hereditary thoracic aortic disease.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
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