Jervell and Lange-Nielsen syndrome (Online Mendelian Inheritance in Man 220400) is a rare autosomal recessive cardioauditory ion channel disorder that affects 1/200,000 to 1/1,000,000 children. It is character...
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Σάββατο 1 Απριλίου 2017
Clinical and molecular findings in a Moroccan family with Jervell and Lange-Nielsen syndrome: a case report
Benefits of subspecialty adherence after asthma hospitalization and patient perceived barriers to care
Source:Annals of Allergy, Asthma & Immunology
Author(s): Neema Izadi, Jonathan S. Tam
BackgroundComparative studies have demonstrated that asthma education to pediatric patients decreases average hospital usage and that allergy specialists provide stronger asthma education and more improved outcomes.ObjectiveTo evaluate the real-world benefits of allergy subspecialty involvement outside inpatient consultation and the impediments for patients in establishing allergy subspecialty care.MethodsThe study population was composed mostly of minority children 0 to 18 years old seen at a large university-affiliated stand-alone children's hospital who had a hospital discharge diagnosis of asthma from 2009 to 2013. The retrospective portion of the study compared all variables pertaining to asthma, teaching, and discharge reconciliation for the following subgroups: patients recommended to allergy and immunology (AI) follow-up who adhered to the appointment (adherent), patients recommended to AI follow-up who did not adhere (nonadherent), and patients not recommended to AI follow-up (non-referred). In the phone interview portion of the study, the nonadherent patients were contacted to identify barriers to AI follow-up.ResultsOf the referred sample, the adherent group had significantly fewer visits to the pediatric intensive care unit, days in the pediatric intensive care unit, and days in the hospital. Providing more specific hospital discharge instructions increased AI follow-up and hospital teaching given on the baseline admission decreased hospital visits. Phone interviews showed that nonadherent patients most commonly missed follow-up because the parents believed it unnecessary because their child showed acute improvement or from advice from their primary care physician.ConclusionThese results showed improvement in outcomes for patients who attended AI follow-up and specifically identified key barriers that could be addressed in a standardized form to prevent nonadherence in the future.
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Effect of alfacalcidol on the pulmonary function of adult asthmatic patients: A randomized trial
Source:Annals of Allergy, Asthma & Immunology
Author(s): Amani M. Ali, Samah Selim, Maggie M. Abbassi, Nirmeen A. Sabry
BackgroundDespite the use of alfacalcidol in the management of corticosteroid-induced osteoporosis, it has never been considered an adjunct treatment for asthma management. It can target vitamin D deficiency, a possible risk factor for asthma, and, hence, improve pulmonary function of patients with asthma.ObjectiveTo explore the effect of alfacalcidol administration on pulmonary function and study the pattern of vitamin D deficiency in adults with asthma in Egypt.MethodsSerum 25-hydroxyvitamin D was measured in 115 adults: 33 healthy subjects and 82 patients with asthma. Then, patients with asthma were randomized to receive standard asthma treatment only (n = 39) or receive it in addition to 1 μg of alfacalcidol daily for 4 months (n = 43). Randomization was stratified by the stage of asthma severity. Spirometry and measurement of 25-hydroxyvitamin were performed at baseline and end of follow-up.ResultsVitamin D deficiency was more common in patients with asthma (57.3%) than in healthy subjects (21.2%; P < .001). In patients with asthma, alfacalcidol significantly improved forced expiratory volume in the first second and forced vital capacity (P < .001 for the 2 tests). Moreover, more patients in the intervention arm showed improvement in asthma severity stage (P = .04). A nonsignificant difference was observed in improvement of forced expiratory volume in the first second between patients with vitamin D deficiency and those without deficiency in the intervention group (P > .05).ConclusionAlfacalcidol supplementation improved the pulmonary function and severity stage of adult patients with asthma regardless of deficiency.Trial RegistrationClinicalTrials.gov Identifier: NCT02747381.
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Mimicker of hereditary angioedema: Idiopathic systemic capillary leak syndrome successfully treated with intravenous immunoglobulin
Source:Annals of Allergy, Asthma & Immunology
Author(s): Xiao Chloe Wan, Andrew Lai, Tejaswi Kompala, Rosa Ten
http://ift.tt/2nsFgR2
Dual training in allergy/immunology and rheumatology
Source:Annals of Allergy, Asthma & Immunology
Author(s): Bharat Kumar, Svjetlana Dolovcak, Scott A. Vogelgesang
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Broader concepts of quality of life measurement, encompassing validation
Abstract
Background
Developing and managing measures of Quality of Life (QoL) requires attention to a range of broader concepts, in addition to meeting validation requirements.
Objectives
The aim of this review is to describe development and experience in Cardiff of these concepts and to inform users of Cardiff quality of life tools of aspects of their origin, for the benefit of developers of novel QoL measures or other patient reported outcome measures.
Methods
Publications from the Cardiff team over the last three decades are used to illustrate descriptions of concepts involved in developing and managing QoL measures.
Results
The concepts are grouped into three main themes: A) Design of tools: measurement ability turns ideas into science, QoL measurement based on patient experience, the need for tools to be clinically practical and useful with meaningful scores, different ages need tailored tools. B) Practical management of tools: enabling ease of access, maintenance of single version, translation validity, enabling access to post publication experience and further validation. C) Promoting wider understanding of QoL: examples include educate thinking with disease severity definition; heighten awareness of broader burden, family impact and the time dimension.
Conclusion
The development and management of QoL and other outcome measures involves attention to a wide range of other issues, in addition to meeting validation requirements.
This article is protected by copyright. All rights reserved.
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Diagnostic accuracy of confocal microscopy imaging versus punch biopsy for diagnosing and subtyping basal cell carcinoma
Abstract
Background
In vivo reflectance confocal microscopy (RCM) is a promising non-invasive skin imaging technique that could facilitate early diagnosis of basal cell carcinoma (BCC) instead of routine punch biopsies. However, the clinical value and utility of RCM versus a punch biopsy in diagnosing and subtyping BCC is unknown.
Objective
To assess diagnostic accuracy of RCM versus punch biopsy for diagnosing and subtyping clinically suspected primary BCC.
Methods
A prospective, consecutive cohort of 100 patients with clinically suspected BCC were included at two tertiary hospitals in Amsterdam, the Netherlands, between February 3, 2015 and October 2, 2015. Patients were randomized between two test-treatment pathways: diagnosing and subtyping using RCM imaging followed by direct surgical excision (RCM one-stop-shop) or planned excision based upon the histological diagnosis and subtype of punch biopsy (standard care). The primary outcome was the agreement between the index tests (RCM versus punch biopsy) and reference standard (excision specimen) in correctly diagnosing BCC. The secondary outcome the agreement between the index tests and reference standard in correctly identifying the most aggressive BCC subtypes.
Results
Sensitivity to detect BCC was similar for RCM and punch biopsy (100% versus 93.94%), but a punch biopsy was more specific than RCM (79% versus 38%). RCM expert evaluation for diagnosing BCC had a sensitivity of 100% and a specificity of 75%. The agreement between RCM and excision specimen in identifying the most aggressive BCC subtype ranged from 50% to 85% versus 77% by a punch biopsy.
Conclusion
RCM and punch biopsy have comparable diagnostic accuracy to diagnose and subtype BCC depending on RCM experience. Although experienced RCM users could accurately diagnose BCC at a distance, we found an important difference in subtyping BCC. Future RCM studies need to focus on diagnostic accuracy, reliability and specific criteria to improve BCC subtype differentiation.
This article is protected by copyright. All rights reserved.
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The Effect of Body Weight on the Efficacy and Safety of Ixekizumab: Results from an Integrated Database of 3 Randomized, Controlled Phase 3 Studies of Patients with Moderate-to-Severe Plaque Psoriasis
Abstract
Background
There is concern that increased body weight may impact efficacy of some therapies used to treat psoriasis.
Objective
To evaluate the effect of body weight on response to ixekizumab treatment in moderate-to-severe psoriasis patients.
Methods
Patients were characterized under 3 body weight categories (<80 kg, 80 to 100 kg, ≥100 kg) in this preplanned subgroup analysis from an integrated database of 3 multicenter, randomized, double-blind, controlled Phase 3 clinical studies (UNCOVER -1, UNCOVER-2, and UNCOVER-3). In the first 12 weeks of each study, patients were randomly assigned to receive subcutaneous placebo, 80-mg ixekizumab every 2 weeks (IXE Q2W) or every 4 weeks (IXE Q4W) after a starting dose of 160-mg ixekizumab, or 50-mg etanercept twice weekly (UNCOVER-2 and UNCOVER-3 only).
Results
This analysis included 3855 patients with baseline body weight in the IXE Q4W (N=1159), IXE Q2W (N=1168), placebo (N=789), and etanercept (N=739) groups. Distribution of patients across body weight categories was similar between treatment groups. Baseline demographics and patients characteristics were generally consistent across treatment groups within each body weight category. Across all body weight categories, a significantly higher percent of patients treated with IXE Q2W or IXE Q4W than with placebo or etanercept achieved PASI75, PASI90, or PASI100 at Week 12. No meaningful differences in PASI75 response rates were observed across body weight categories. Some numerical differences in PASI 90 and PASI100 response rates were observed between body weight categories with IXE Q2W providing numerically higher response rates than IXE Q4W. The incidence of treatment-emergent adverse events was similar in the treatment groups and across body weight categories.
Conclusion
Ixekizumab was efficacious in the treatment of moderate-to-severe psoriasis regardless of body weight. The safety profile of ixekizumab was also similar across body weight categories and no safety signals were identified specific to any of the body weight categories.
This article is protected by copyright. All rights reserved.
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The Icatibant Outcome Survey: experience of hereditary angioedema management from six European countries
Abstract
Background
Hereditary angioedema (HAE) due to C1 INH deficiency (C1-INH-HAE) is a rare, potentially fatal, bradykinin-mediated disease. Icatibant is a bradykinin B2 receptor antagonist originally approved in 2008 in the European Union and 2011 in the United States, as an acute therapy option for HAE attacks in adults.
Objective
To compare demographics, disease characteristics and treatment outcomes of icatibant-treated HAE attacks in patients with C1-INH-HAE enrolled in the Icatibant Outcome Survey across six European countries: Austria, France, Germany, Italy, Spain and the United Kingdom.
Methods
The Icatibant Outcome Survey (IOS; Shire, Zug, Switzerland [NCT01034969]) is an international observational study monitoring the safety and effectiveness of icatibant. Descriptive, retrospective analyses compared IOS country data derived during July 2009 – April 2015.
Results
Overall, 481 patients with C1-INH-HAE provided demographic data. A significant difference across countries in age at onset (P = 0.003) and baseline attack frequency (P < 0.001) was found though no significant differences were found with respect to gender (majority female; P = 0.109), age at diagnosis (P = 0.182) or delay in diagnosis (P=0.059). Icatibant was used to treat 1893 attacks in 325 patients with majority self-administration in all countries. Overall, significant differences (all P< 0.001) were found across countries in time to treatment (median 1.8 hours; median range: 0.0 [Germany-Austria] to 4.4 [France] hours), time to resolution (median 6.5 hours; median range: 3 [Germany-Austria] to 12 [France] hours) and attack duration (median 10.5 hours; median range: 3.1 [Germany-Austria] to 18.5 [France] hours.
Conclusion
These data form the first European cross-country comparison of disease characteristics and icatibant use in patients with C1-INH-HAE who are enrolled in IOS. International variation in icatibant practice and treatment outcomes across the six European countries assessed highlight the need to further investigate the range of country-specific parameters driving regional variations in icatibant use.
This article is protected by copyright. All rights reserved.
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Topical treatment of psoriasis: questionnaire results on topical therapy accessibility and influence of body surface area on usage
Abstract
Background
Topical treatment of mild to moderate psoriasis is first line treatment and exhibits varying degrees of success across patient groups. Key factors influencing treatment success are physician topical treatment choice (high efficacy, low adverse events) and strict patient adherence. Currently, no formalized, international consensus guidelines exist to direct optimal topical treatment, although many countries have national guidelines.
Objective
To describe and analyze cross-regional variations in the use and access of psoriasis topical therapies.
Methods
The study was conducted as an observational cross-sectional study. A survey was distributed to dermatologists from the International Psoriasis Council (IPC) to assess topical therapy accessibility in 26 countries and to understand how body surface area (BSA) categories guide clinical decisions on topical use.
Results
Variation in the availability of tars, topical retinoids, dithranol and balneotherapy was reported. The vast majority of respondents (100% and 88,4%) used topical therapy as first line monotherapy in situations with BSA<3% and BSA between 3% and 10%, respectively. However, with disease severity increasing to BSA>10%, the number of respondents who prescribe topical therapy decreased considerably. In addition, combination therapy of a topical drug and a systemic drug was frequently reported when BSA measured >10%.
Conclusion
This physician survey provides new evidence on topical access and the influence of disease severity on topical usage in an effort to improve treatment strategies on a global level.
This article is protected by copyright. All rights reserved.
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Effects of music intervention on anxiety and pain reduction in ambulatory maxillofacial and otorhinolaryngology surgery: a descriptive survey of 27 cases
Abstract
Purpose
The aim of this study is to determine patients' opinion regarding listening to music before an ambulatory maxillofacial surgery and effects on anxiety and pain reduction.
Methods
This study was conducted on outpatients having a maxillofacial surgery between December 2015 and April 2016 at Poissy/Saint-Germain-en-Laye hospital (France). Patients listened with headphones to an easy-listening music in the operation theater before the first ambulation. A questionnaire including a visual analog scale (VAS) for pain and anxiety was given to participants. The primary endpoint was to determine patients' opinion regarding listening to music before surgery. Secondary endpoints were to determine VAS pain mean, VAS anxiety mean before surgery, VAS anxiety mean after surgery, and if patients wanted to listen to their own playlist. We decided to compare VAS anxiety and pain mean between patients who accepted to listen to music (ALM) and who refused to listen to music (RLM).
Results
Nineteen patients ALM and 8 patients RLM to music. 78.9% of patients considered that listening to music before surgery decreased their anxiety. In patients who ALM, the mean (standard deviation, SD) of VAS pain after surgery was 3.42 (1.95), the mean (SD) of VAS anxiety before surgery was 3.1 (2.3), and the mean (SD) of VAS anxiety was 1.21 (0.85). There was a statistically significantly difference of the VAS anxiety mean (SD) before surgery between patients who ALM 3.10 (2.30) and who RLM 6.12 (1.88) (p = 0.005). There was a statistically significantly difference of the VAS anxiety mean (SD) after surgery between patients who ALM 1.21 (0.85) and who RLM 2.62 (1.30) (p = 0.009). Fifty percent of the patients wanted to choose their own music.
Conclusion
Music seems to reduce anxiety before maxillofacial surgery. An interventional randomized study is needed to demonstrate the positive impact of music on anxiety before maxillofacial surgery.
http://ift.tt/2nrEDaD
Morphological characteristics of external auditory canal in congenital aural stenosis patients
ObjectiveTo investigate characteristics of congenital aural stenosis (CAS) patients' external auditory canal (EAC) (position, length, orientation, etc.) and compare them with normal EAC.
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Clinical and audiological characteristics of 1000Hz audiometric notch patients
Among the hearing loss patients, we can confirm that the hearing loss of the specific frequency decreases, such as the 2000Hz notch in otosclerosis and the 4000Hz notch (c5-dip) in noise-induced hearing loss. The 1000Hz notch (c3-dip), however, is rarely studied. We fortuitously encountered a group of patients with a 1kHz hearing loss and report it with a review of the literature.
http://ift.tt/2ns2pmQ
Important Announcement
<span class="paragraphSection"><span style="font-style:italic;">International Immunology</span> is the official journal of the Japanese Society for Immunology (JSI) and was launched in partnership with Oxford University Press in 1989 with Professor Tomio Tada as its inaugural Editor-in-Chief. Through Professor Tada's efforts <span style="font-style:italic;">International Immunology</span> firmly established itself as an internationally renowned, first rate immunology journal.</span>
http://ift.tt/2ov1Fzb
Mesenchymal stem cell-derived extracellular vesicles: a glimmer of hope in treating Alzheimer’s disease
<span class="paragraphSection"><div class="boxTitle">Abstract</div>One of the pathological hallmarks of Alzheimer's disease (AD) is the presence of extracellular plaques resulting from the accumulation of beta-amyloid peptide (Aβ). To date, a definitive cure for this disease is still lacking as the currently approved drugs used are mainly symptomatic treatments. The revolutionary discovery of extracellular vesicles (EVs) has shed new light on the development of disease-modifying treatments for AD, owing to their potential in delivering the therapeutic agents to the brain. The feasibility of harnessing EVs for clinical applications is highly dependent on the donor cell, which determines the intrinsic properties of EVs. The merit of mesenchymal stem cells (MSCs) as therapeutic delivery vehicles, and the proven therapeutic effects of the EVs derived from these cells, make researchers esteem MSCs as ideal producers of EVs. Therefore, MSC-derived EVs (MSC-EVs) emerge to be an appealing therapeutic delivery approach for the treatment of AD. Here, we discuss perspectives on the therapeutic strategies using MSC-EVs to treat AD and the associated challenges in clinical application.</span>
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Inflammatory and anti-inflammatory states of adipose tissue in transgenic mice bearing a single TCR
<span class="paragraphSection"><div class="boxTitle">Abstract</div>Obesity is accompanied by chronic, low-grade inflammation in adipose tissue, which is associated with insulin resistance and consequent multiple metabolic diseases. In addition to M1 macrophage infiltration, multiple involvements of adipose tissue T lymphocytes in the progression of inflammation have been highlighted recently. Here, we isolated a specific Vα5/Vβ8.2 TCR-bearing T cell that accumulated in obese adipose tissue of mice, and generated transgenic mice expressing this TCR. Under lean conditions with a normal chow diet, CD4<sup>+</sup>FoxP3<sup>+</sup> T<sub>reg</sub> cells and M2 macrophages increased in adipose tissue with ageing in wild-type mice, but not in transgenic mice. However, both mice exhibited no obvious adipose tissue inflammation such as the formation of crown-like structures (CLSs) of infiltrating macrophages. When fed a high-fat diet, the proportion of adipose tissue T<sub>reg</sub> cells was markedly small at a similar level in transgenic and wild-type mice. Both types of mice exhibited comparable inflammatory states in adipose tissue, including vast formation of macrophage CLSs, accompanied by insulin resistance. Together, our findings suggest that the absence of an increase in T<sub>reg</sub> cells and M2 macrophages is not sufficient to initiate inflammatory macrophage infiltration in lean adipose tissue and also provide a new view about the involvement of T cells in promoting obesity-associated inflammation.</span>
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The spleen is the site where mast cells are induced in the development of food allergy
<span class="paragraphSection"><div class="boxTitle">Abstract</div>It has been reported that splenic immune responses play pivotal roles in the development of allergic diseases; however, the precise role of the spleen remains unclear. Herein, we demonstrated a novel role of the spleen in the pathogenesis of food allergy (FA). We found that mast cells (MCs) developed from progenitor cells present in spleen during an antigen-specific T-cell response <span style="font-style:italic;">in vitro</span>. In a T<sub>h</sub>2 response-mediated FA model, significant expansion of MCs was also observed in spleen. The incidence of allergic diarrhea was profoundly reduced in splenectomized mice, whereas adoptive transfer of <span style="font-style:italic;">in vitro</span>-induced splenic MCs into these mice restored allergic symptoms, suggesting that the splenic MCs functioned as the pathogenic cells in the development of FA. The <span style="font-style:italic;">in vitro</span>-generated MCs required not only IL-3 but also IFN-γ, and treatment of FA-induced mice with anti-IFN-γ antibody suppressed expansion of MCs in spleen as well as diarrhea development, highlighting that IFN-γ in the spleen orchestrated the development of FA, which was followed by a T<sub>h</sub>2 response in the local lesion. Overall, we propose that the role of the spleen in the development of FA is to provide a unique site where antigen-specific T cells induce development of pathogenic MCs.</span>
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Rejuvenation of mucosal immunosenescence by adipose tissue-derived mesenchymal stem cells
<span class="paragraphSection"><div class="boxTitle">Abstract</div>Age-associated alterations in the mucosal immune system are generally termed mucosal immunosenescence. The major change seen in the aged mucosa is a failure to elicit an antigen-specific secretory IgA (SIgA) antibody response, which is a central player for host defense from various pathogens at mucosal surfaces. In this regard, it would be a first priority to compensate for mucosal dysregulation in the elderly in order to maintain their health in aging. We have successfully established antigen-specific SIgA antibody responses in aged (2 years old) mice, which provide protective immunity from <span style="font-style:italic;">Streptococcus pneumoniae</span> and influenza virus infections, by using a new adjuvant system consisting of a plasmid encoding Flt3 ligand (pFL) and CpG ODN. In order to explore possible use of current mucosal vaccine strategies for the elderly, we have adoptively transferred adipose tissue-derived mesenchymal stem cells (AMSCs) to aged mice prior to mucosal vaccination. This immune therapy successfully resulted in protective antigen-specific antibody responses in the intestinal mucosa of aged mice that were comparable to those seen in young adult mice. In this regard, we postulate that adoptively transferred AMSCs could augment dendritic cell functions in aged mice. The potential cellular and molecular mechanisms whereby AMSCs restore mucosal immunity in immunosenescence are discussed in this short review. A stem cell transfer system could be an attractive and effective immunologic intervention strategy to reverse mucosal immunosenescence.</span>
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Zuckerkandl’s tubercle and its relationship to the recurrent laryngeal nerve: A cadaveric dissection and meta-analysis
Zuckerkandl's tubercle (ZT), when present, is an anatomical landmark by which surrounding structures such as the recurrent laryngeal nerve (RLN) can be identified intraoperatively. This study aimed to investigate the prevalence and anatomical characteristics of Zuckerkandl's tubercle by combining cadaveric dissection with a meta-analysis.
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Effect of alfacalcidol on the pulmonary function of adult asthmatic patients: A randomized trial
Despite the use of alfacalcidol in the management of corticosteroid-induced osteoporosis, it has never been considered an adjunct treatment for asthma management. It can target vitamin D deficiency, a possible risk factor for asthma, and, hence, improve pulmonary function of patients with asthma.
http://ift.tt/2ov6SHi
Dual training in allergy/immunology and rheumatology
The specialties of allergy/immunology and rheumatology (AI&R) have been closely associated with one another because of their heavy emphasis on clinical immunology. In fact, there has been a long tradition of physicians being trained in both fields, whether through pursuing 2 fellowships separately or through dual certification pathways established jointly by their respective certifying boards. However, the posttraining careers of these dual-trained physicians have not yet been characterized.
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Mimicker of hereditary angioedema: Idiopathic systemic capillary leak syndrome successfully treated with intravenous immunoglobulin
Idiopathic systemic capillary leak syndrome (ISCLS) is a rare, potentially life-threatening disease characterized by episodic attacks of systemic capillary hyperpermeability and vascular collapse accompanied by hypoalbuminemia, hemoconcentration, and edema.1–3 We describe the case of a 46-year-old man with recurrent abdominal pain and facial edema, long believed to have recurrent angioedema, who was ultimately diagnosed with ISCLS.
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Benefits of subspecialty adherence after asthma hospitalization and patient perceived barriers to care
Comparative studies have demonstrated that asthma education to pediatric patients decreases average hospital usage and that allergy specialists provide stronger asthma education and more improved outcomes.
http://ift.tt/2onICti
Clinical and audiological characteristics of 1000Hz audiometric notch patients
Source:American Journal of Otolaryngology
Author(s): Hyun-Min Lee, Seok-Hyun Kim, Jae-Hwan Jung, Se-Joon Oh, Soo-Keun Kong, Il-Woo Lee
PurposeAmong the hearing loss patients, we can confirm that the hearing loss of the specific frequency decreases, such as the 2000Hz notch in otosclerosis and the 4000Hz notch (c5-dip) in noise-induced hearing loss. The 1000Hz notch (c3-dip), however, is rarely studied. We fortuitously encountered a group of patients with a 1kHz hearing loss and report it with a review of the literature.MethodsOtological history, audiogram, diagnosis, occupation, and history of noise exposure were reviewed from charts and telephone interview, and compared between c3-dip and c5-dip patients (n=98).ResultsThirty-one patients (mean age: 46.2years) demonstrated 1kHz hearing loss; these included 11 males. The pure-tone threshold was 37.97dB at 1kHz and the average threshold was 22.38dB at other frequencies. In the c3-dip group, tinnitus was the most common complaint, while sudden sensorineural hearing loss and idiopathic tinnitus (n=8 each) were the most common diagnoses. Female patients and unilateral cases were more common in the c3-dip than in the c5-dip group, and ear fullness was more common in the c3-dip group than in the c5-dip group. The duration of occupation-related noise exposure was longer in the c5 group, and head or ear trauma was more frequent in the c3-dip group.ConclusionWe have defined a new clinical entity of 1kHz hearing loss in patients, defined as the c3-dip, which was clinically and audiologically distinct from the c5-dip. Further study is needed to clarify this new entity of hearing loss.
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Morphological characteristics of external auditory canal in congenital aural stenosis patients
Source:American Journal of Otolaryngology
Author(s): Dongming Yin, Chenlong Li, Keguang Chen, Juan Hong, Jieying Li, Lin Yang, Tianyu Zhang, Peidong Dai
ObjectiveTo investigate characteristics of congenital aural stenosis (CAS) patients' external auditory canal (EAC) (position, length, orientation, etc.) and compare them with normal EAC.MethodsCT images of normal people and CAS patient were utilized. We obtained coordinates of EAC landmarks. Then the Matlab program could calculate some anatomic parameters about EAC, including distances from central point of tympanic annulus (CA), central point of osseous EAC opening (CO), central point of cartilaginous EAC inside opening (CCi), central point of cartilaginous EAC outside opening (CCo) to the Frankfurt horizontal plane (Pfrkt), the median sagittal plane (Psag), the coronal plane (Pcor); orientations of EAC bendings; straight and arc lengths of EAC.ResultsDistances from CA, CO, CCi and CCo to Pfrkt were all shorter in CAS group than control group (p<0.05). The straight and arc lengths of cartilaginous EAC in CAS group were shorter than control group (p<0.05). Straight and arc lengths of EAC in CAS group were shorter than those in control group (p<0.05). The proportion of one bending in cartilaginous EAC in control group was significantly lower than CAS group (p<0.05). Orientations of EAC bendings in CAS group differed from those in control group (p<0.05).ConclusionIn addition to smaller diameters, compared with normal EAC, the position of CAS patients' osseous EAC was higher compared with the normal. The majority of CAS patients have a bending and downward slanting cartilaginous EAC. Orientations of EAC bending in CAS patients were different from normal. Besides, the length of CAS patients' cartilaginous EAC was shorter. However, there were no significant differences between CAS patients and normal people in length of osseous EAC. These differences in anatomic parameters could provide the basis for optimizing the meatoplasty.
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Scales used to rate adult patients’ psycho-emotional status in tooth extraction procedures a systematic review
The aim of this study was to review scales used to assess anxiety, stress, and pain in dental patients undergoing a tooth extraction procedure and to propose a novel psycho-emotional rating scale based on the relevant literature and our own experience. An electronic literature search was conducted of the National Library of Medicine database MEDLINE (Ovid) and EMBASE databases between January 2005 and April 2016. Sequential screening at the title/abstract and full-text levels was performed. The review included all human prospective or retrospective follow-up studies and clinical trials, cohort studies, case–control studies, and case series that demonstrated at least one scale used to measure tooth extraction anxiety, stress, or pain.
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A three-dimensional planned osteotomy on the zygomatic arch for reduction malarplasty
During a reduction malarplasty, precise sectioning of the zygomatic arch according to the plan formulated in the diagnostic stage is very important, because differences in the locations of the osteotomies in the left and right zygomatic arch will result in facial asymmetry, and arch osteotomies that are placed at locations other than those specified during planning elicit unwanted results. A method for the precise planning and sectioning of the zygomatic arch involving the use of computed tomography (CT) and a viewer program is presented herein.
http://ift.tt/2oM0WZG
The role of wide local excision for the treatment of severe hidradenitis suppurativa (Hurley grade III): Retrospective analysis of 74 patients
Effective medical treatment for patients with severe hidradenitis suppurativa (HS) is limited.
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Injection speed of spinal anaesthesia for Caesarean delivery in Asian women and the incidence of hypotension: A randomised controlled trial
The purpose of this investigation was to determine if a slower speed of spinal anaesthesia injection would reduce the incidence of hypotension.
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Factors influencing postoperative pain following discharge in pediatric ambulatory surgery patients
To identify demographic, intraoperative, and parental factors that influence the postoperative pain experience in ambulatory surgery pediatric patients. We also monitored postoperative maladaptive behavior changes (PMBCs) to investigate the relationship between pain and PMBCs.
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Expression and splicing of Ikaros family members in murine and human thymocytes
Publication date: July 2017
Source:Molecular Immunology, Volume 87
Author(s): Julie L. Mitchell, Amara Seng, Thomas M. Yankee
The Ikaros family of transcription factors includes five highly homologous members that can homodimerize or heterodimerize in any combination. Dimerization is essential for their ability to bind DNA and function as transcription factors. Previous studies showed that eliminating the function of the entire family blocks lymphocyte development while deletion of individual family members has relatively minor defects. These data indicate that multiple family members function during T cell development, so we examined the changes in expression of each family member as thymocytes progressed from the CD4−CD8− double negative (DN) to the CD4+CD8+ double positive (DP) developmental stage. Further, we compared the expression of each family member in murine and human thymocytes. In both species, Ikaros and Aiolos mRNA levels increased as thymocytes progressed through the DN to DP transition, but the corresponding increases in protein levels were only observed in mice. Further, Ikaros and Aiolos underwent extensive alternative splicing in mice, whereas only Ikaros was extensively spliced in humans. Helios mRNA and protein levels decreased during murine T cell development, but increased during human T cell development. These differences in the expression and splicing of Ikaros family members between human and murine thymocytes strongly suggest that the Ikaros family of transcription factors regulates murine and human T cell development differently, although the similarities across Ikaros family members may allow different proteins to fulfill similar functions.
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Corrigendum to “MiR-146a modulates macrophage polarization in systemic juvenile idiopathic arthritis by targeting INHBA” [Mol. Immunol. 77 (2016) 205–212]
Publication date: Available online 31 March 2017
Source:Molecular Immunology
Author(s): Dan Li, Mingyue Duan, Yuan Feng, Lingling Geng, Xiaoqing Li, Wanggang Zhang
http://ift.tt/2oni1MQ
Attenuation of cortical activity triggering descending pain inhibition in chronic low back pain patients: a functional magnetic resonance imaging study
Abstract
Purpose
A considerable portion of chronic low back pain (cLBP) patients lack anatomical abnormality, resist conventional therapeutic interventions, and their symptoms are often complicated with psychological and social factors. Such patients have been reported to show cerebral abnormalities both in anatomy and function by neuroimaging studies. Here we examined differences in cerebral reactivity to a simulated low back pain stimulus between cLBP patients and healthy controls by functional magnetic resonance imaging (fMRI), and their behavioral correlates from a psychophysical questionnaire.
Methods
Eleven cLBP patients and 13 healthy subjects (HS) were enrolled in this study. After psychophysical evaluation on-going pain with McGill Pain Questionnaire Short Form (MPQ), they underwent whole-brain fMRI in a 3-Tesla MRI scanner while receiving three blocks of 30-s mechanical pain stimuli at the left low back with a 30-s rest in between, followed by a three-dimensional anatomical imaging. Functional images were analyzed with a multi-subject general linear model for blood oxygenation level-dependent (BOLD) signal changes associated with pain. Individual BOLD signal amplitudes at activated clusters were examined for correlation with psychophysical variables. Two in the cLBP and five data sets in the HS groups were excluded from analysis because of deficient or artifactual data or mismatch in age.
Results
The HS group showed LBP-related activation at the right insular cortex, right dorsolateral prefrontal cortex (DLPFC), left anterior cingulate cortex (ACC), and left precuneus; and deactivation in a large area over the parietal and occipital cortices, including the bilateral superior parietal cortex. On the other hand, the cLBP group did not show any significant activation at those cortical areas, but showed similar deactivation at the bilateral superior parietal cortex and part of the premotor area. An HS > cLBP contrast revealed significantly less activity at the ACC and DLPFC in the cLBP group, which was negatively correlated with higher MPQ scores.
Conclusions
The cLBP patients showed attenuated reactivity to pain at the ACC and DLPFC, known cortical areas mediating affective component, and top-down modulation, of pain. The present results might be associated with possible dysfunction of the descending pain inhibitory system in patients with chronic low back pain, which might possibly play a role in chronification of pain.
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The effects of clinical factors on airway outcomes of mandibular distraction osteogenesis in children with Pierre Robin sequence
Publication date: Available online 31 March 2017
Source:International Journal of Oral and Maxillofacial Surgery
Author(s): X.C. Ren, Z.W. Gao, Y.F. Li, Y. Liu, B. Ye, S.S. Zhu
Mandibular distraction osteogenesis (MDO) is an effective treatment for tongue-based airway obstruction in children with severe Pierre Robin sequence. An investigation was performed to determine whether certain clinical factors influence the airway outcomes of MDO. A literature search of several databases was performed to identify studies providing individual patient data. Data extracted from the studies included patient sex, age at distraction, disease type, experience of any previous surgery on the airway, length of distraction, pre- and postoperative blood oxygen saturation nadir, and osteotomy design. Non-parametric tests and multivariate logistic regression analysis were conducted to investigate the potential interaction between these clinical factors and the efficacy of surgery. Five studies met the inclusion criteria, with data available for 73 individual patients. The results of the statistical analysis revealed that few of the factors investigated influenced the surgical efficacy in children with Pierre Robin sequence; the effect of the length of distraction was regarded as uncertain because of the limited amount of individual data available. In conclusion, no influencing factors were found, and according to this analysis, mandibular distraction may be a widely effective procedure. However, more well-designed studies and more individual data are needed to strengthen the results of this meta-analysis.
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Islanded facial artery musculomucosal flap for tongue reconstruction: reply to the comment
Source:International Journal of Oral and Maxillofacial Surgery
Author(s): S.T. Joseph
http://ift.tt/2nHAPUB
Recurrent dislocation: scientific evidence and management following a systematic review
Publication date: Available online 31 March 2017
Source:International Journal of Oral and Maxillofacial Surgery
Author(s): A.R. Melo, E.D. Pereira Júnior, L.A. de M. Santos, B.C. do E. Vasconcelos
Recurrent mandibular dislocation is a rare condition that can have a negative impact on quality of life. Different surgical techniques are employed in the treatment of this condition, and the demand for maximum healthcare quality has contributed to the implementation of evidence-based clinical practice. The objective of this study was to determine the level of scientific evidence in articles reporting open surgical treatment for recurrent mandibular dislocation. A comprehensive search strategy was conducted to locate relevant articles in the PubMed and Web of Science databases on open surgical treatment for recurrent mandibular dislocation published between January 1974 and August 2014. These were classified into one of the five established levels/sublevels of evidence: the level of evidence was determined based on the classification proposed by the Oxford Centre for Evidence-Based Medicine. One hundred and fourteen articles were identified, 91 of which were excluded based on the eligibility criteria. Thus, 23 articles were selected for inclusion in the review. All of the selected articles were rated as level 4 (low quality) regarding the level of evidence. The present review revealed that articles on open surgical treatment for recurrent mandibular dislocation exhibit a low level of scientific evidence. Thus, further studies on this topic with greater methodological rigour are needed.
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Paediatric oral submucous fibrosis – The neglected pre-malignancy of childhood
Publication date: June 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 97
Author(s): Chandala Chitguppi, Tripti Brar
Oral submucous fibrosis in children is one of the devastating consequences of arecanut addiction in children. Arecanut is the fourth commonest psychoactive agents used worldwide. However, regrettably, unlike other addictions like tobacco and alcohol, its use in children has become socially acceptable atleast in a few countries. Consequently, children as young as two years have been noticed to be addicted to arecanuts. It has been a menace in most Asian countries and with increasing population migration, this has picked up even in the west. In order to understand the social factors leading to arecanut addiction in children and prognostic outcomes of paediatric oral submucous fibrosis, this review has been undertaken.
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Intraoperative and postoperative hyperkalaemia after total parathyroidectomy following exposure to cinacalcet in sixteen patients for renal hyperparathyroidism
Abstract
Our clinical observation suggested that patients with prior treatment with cinacalcet and undergoing parathyroidectomy for renal hyperparathyroidism had greater acute hyperkalaemia in the intraoperative and immediate postoperative period
81.25% of the cinacalcet patients recorded an intraoperative or 24 hour postoperative potassium value >6mmol/L compared to 1.67% in the controls (P<0.001)
Cinacalcet patients had greater median percentage rise of potassium from preoperative value (33.33%) compared to control group (11.54%) (P<0.001)
Cinacalcet patients demonstrated greater degree of hypocalcaemia and an increased requirement for intravenous calcium in the postoperative period
The mechanism for this observation/association is unknown, however awareness of the phenomenon is imperative for surgeons, anaesthetists and renal physicians.
This article is protected by copyright. All rights reserved.
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Hearing Optimization in Neurofibromatosis type 2: A Systematic Review
Abstract
Background
It is common for patients with Neurofibromatosis type 2 to develop bilateral profound hearing loss hearing loss and this is one of the main determinants of quality of life in this patient group.
Objectives
The aim of this systematic review was to review the current literature regarding hearing outcomes of treatments for vestibular schwannomas in neurofibromatosis type 2 including conservative and medical management, radiotherapy, hearing preservation surgery and auditory implantation in order to determine the most effective way of preserving or rehabilitating hearing
Search Strategy: A MESH search in PubMed using search terms (("Neurofibromatosis 2"[Mesh]) AND "Neuroma, Acoustic"[Mesh]) AND "Hearing Loss"[Mesh] was performed. A search using keywords was also performed. Studies with adequate hearing outcome data were included. With the exception of the cochlear implant studies (cohort size was very small) case studies were excluded.
Evaluation Method: The GRADE system was used to assess quality of publication. Formal statistical analysis of data was not performed because of very heterogenous data reporting.
Results
Conservative management offers the best chance of hearing preservation in stable tumours. The use of Bevacizumab probably improves the likelihood of hearing preservation in growing tumours in the short term and is probably more effective than hearing preservation surgery and radiotherapy in preserving hearing. Of the hearing preservation interventions, hearing preservation surgery probably offers better hearing preservation rates than radiotherapy for small tumours but recurrence rates for hearing preservation surgery were high. For patients with profound hearing loss, cochlear implantation provides significantly better auditory outcomes than auditory brainstem implantation. Patients with untreated stable tumours are likely to achieve the best outcomes from cochlear implantation. Those who have had their tumours treated with surgery or radiotherapy do not gain as much benefit from cochlear implantation than those with untreated tumours.
Conclusions
This review summarises the current literature related to hearing preservation/rehabilitation in patients with NF2. Whilst it provides indicative data, the quality of the data was low and should be interpreted with care. It is also important to consider that the management of vestibular schwannomas in NF2 is complex and decision making is determined by many factors, not just the need to preserve hearing.
This article is protected by copyright. All rights reserved.
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Sixty-Three Patients Based Survey - Can Sinonasal Outcome Test-22 Be a Suitable Evaluation Method for Septoplasty and Turbinectomy?
Abstract
In this study we evaluated the patient-perceived benefit of surgery for nasal obstruction.
Data were collected prospectively from both sexes of all ages who underwent elective surgery for nasal obstruction. All patients completed Sinonasal Outcome Test-22 (SNOT-22) questionnaire pre- and postoperatively.
During the study period 63 subjects underwent surgery for nasal obstruction (septoplasty and/or turbinectomy).
The median total SNOT-22 score was 35 and 14 pre- and postoperatively, respectively. The median difference between post- and preoperative scores was -15.
We did not find significant correlation between changes in SNOT-22 scores postoperatively and age, gender, follow-up time, operation type or survey method.
We can carefully conclude that SNOT-22 can be a useful instrument for patients undergoing operation for nasal obstruction.
This article is protected by copyright. All rights reserved.
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Thoracic spondylolisthesis and spinal cord compression in diffuse idiopathic skeletal hyperostosis: a case report
Diffuse idiopathic skeletal hyperostosis has long been regarded as a benign asymptomatic clinical entity with an innocuous clinical course. Neurological complications are rare in diffuse idiopathic skeletal hy...
http://ift.tt/2okVJeD
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