British Rhinological Society 16th Annual Meeting Jersey, 19 May 2017

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Applying behavioral economics to psoriasis treatment: interventions to improve patient adherence to biologics

Abstract

Zschocke et al¹ identified factors associated with adherence to biologics for psoriasis. The physician-patient relationship influences adherence considerably.¹ Specific interventions may increase adherence to biologics.

This article is protected by copyright. All rights reserved.

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Hand, foot and mouth disease in adult patients

Abstract

We read with great interest the paper by Balestri et al¹ "atypical presentation of enterovirus infection in adults: outbreak of Hand, Foot, Mouth and Scalp disease in Northern Italy" which confirms the adult involvement in hand, foot and mouth disease (HFMD).

This article is protected by copyright. All rights reserved.

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Cutaneous leishmaniasis by Leishmania infantum: behind granulomatous lesions of unknown etiology

Abstract

Background

Cutaneous leishmaniasis (CL) is underestimated in Spain as in other European countries due to the polymorphism of its clinical manifestations and histopathological features discouraging doctors from suspecting leishmaniasis. Mucosal manifestations (ML) are misdiagnosed due to the fact that they often mimic cancer.

Objectives

Given that leishmaniasis may be masked as different granulomatous diseases in Leishmania infantum endemic areas, the aim of this study is to verify this misdiagnosing and contribute to the improvement of CL/ML diagnosis.

Methodology

A retrospective study involving formalin-fixed paraffin-embedded tissue biopsies with histopathological features of granulomatous lesions of unknown origin (GLUO) detected 17 patients. This study included 13 patients with CL that were used as positive controls, 9 patients with other confirmed diseases used as negative controls and 7 patients with histological features suggestive of CL or ML without confirmation. Molecular analysis was blindly performed using two different PCR techniques.

Results

The PCR detected 15 CL cases in which the diagnosis was neither clinically nor histologically suspected. Leishmaniasis was confirmed in 7 suspected patients in whom the classical techniques failed to detect the parasite. L. infantum was identified in all cases. A systematic review of CL cases in GLUO patients from European countries identified 45 reported cases.

Conclusions

In L. infantum endemic areas, a high percentage of GLUO are due to Leishmania infection. The main consequences are delayed diagnosis and underestimation of the real incidence. PCR performed on paraffin-embedded tissue proved to be a reliable tool for diagnosis of CL/ML and must be performed routinely in any granulomatous dermatitis, even when the morphological features are no stereotypical of leishmaniasis.

This article is protected by copyright. All rights reserved.

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Eruptive keratoacanthomas arising in the setting of lichenoid toxicity after PD1 inhibition with nivolumab

Abstract

We were fascinated to read the observations of Bandino et al. who described two patients who developed keratoacanthomas and squamous cell carcinoma in association with bullous pemphigoid-like lesions after treatment with PD-1 inhibitors, nivolumab or pembrolizumab, in a recent issue of the Journal. Herein we report two patients with no prior history of skin cancer who developed eruptive keratoacanthomas in the setting of lichenoid skin toxicity after starting nivolumab.

This article is protected by copyright. All rights reserved.

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« Malignant » Rosacea as a sign of systemic marginal zone lymphoma

Abstract

Rosacea is a common facial dermatosis for which differential diagnoses have to be considered in the case of atypical features or treatment failure. We report 3 cases of systemic marginal zone lymphomas (MZL), with skin involvement simulating rosacea. A 76-year-old man presented a rhinophyma for one year (Fig 1).

This article is protected by copyright. All rights reserved.

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Oral administration of geranylgeranylacetone to protect vestibular hair cells

We recently reported that the heat shock response played a major role in the protection of hair cells against stress. Oral administration of the heat shock inducer, geranylgeranylacetone (GGA) protected hair cells against intense noise. In our present study, we investigated the effect of GGA on vestibular hair cell death induced by an aminoglycoside.

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Patient interest in and familiarity with anti-aging therapies: A survey of the general dermatology clinic population

Summary

Background

The appearance of aging skin is a common complaint among dermatology patients. There is an expanding market for anti-aging therapies, but little information is available regarding which patients utilize these treatments and patient preferences regarding treatment.

Aims

To describe the patient population utilizing anti-aging therapies, assess patient familiarity with treatment options, and learn where treatment information is most often obtained.

Patients/Methods

Three hundred patients were surveyed in the University of Michigan General Dermatology Clinic.

Results

Fifty-three percent of the general dermatology patient population has used an anti-aging treatment in the past; 66% reported interest in the future use. Interest is high among all genders, ages, and incomes. Most subjects obtained treatment information from magazines, but subjects were more likely to pursue treatment if information was obtained from a dermatologist.

Conclusion

Demographics of anti-aging therapy are changing, and a wide variety of patients pursue treatment. Patients are largely unfamiliar with most treatment options and are more likely to pursue treatment after receiving treatment information from a dermatologist. The information presented in this study is helpful to both dermatologists and marketers of anti-aging products.

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Microbiome of peri-implantitis affected and healthy dental sites in patients with a history of chronic periodontitis

Publication date: November 2017
Source:Archives of Oral Biology, Volume 83
Author(s): Danae Apatzidou, David F. Lappin, Graham Hamilton, Christos A. Papadopoulos, Antonis Konstantinidis, Marcello P. Riggio
ObjectiveTo determine the composition of the microbiome of peri-implantitis sites and corresponding dental sites in subjects with a history of chronic periodontitis.DesignClinical and radiographic examination assessed the periodontal/peri-implant disease status. Plaque samples were collected from one diseased implant with peri-implantitis, functional for at least two years and healthy sites in ten non-smokers who had received periodontal treatment prior to implant placement. Following DNA extraction, the bacteria present in each sample were determined by high-throughput sequencing of V3-V4 region of the 16S rRNA gene using the Illumina MiSeq platform. OTUs were picked using QIIME. Differences between dental and implant sites were determined using linear discriminant analysis, effect size and diversity analyses were conducted using PAST v3.02.ResultsThe microbiomes of healthy samples were more diverse than those found in disease, although disease was associated with a higher abundance of taxa relative to health. The genera Actinobacillus and Streptococcus were most closely associated with health, whereas Prevotella and Porphyromonas were most discriminative for disease. Synergistetes were highly associated with peri-implantitis.ConclusionIn patients with a history of periodontitis, putative periodontal pathogens prevailed in the microbiome of diseased implants. Diseased implants and corresponding healthy sites appear to have distinct microbiological ecosystems.



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Anti-PD-1 Antibody Therapy Induces Hashimoto's Disease with an Increase in Peripheral Blood Follicular Helper T Cells

Thyroid , Vol. 0, No. 0.


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Prevalence and Characterization of Thyroid Hemiagenesis in Japan: The Fukushima Health Management Survey

Thyroid Aug 2017, Vol. 27, No. 8: 1011-1016.


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Future Meetings

Thyroid Aug 2017, Vol. 27, No. 8: 1107-1107.


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First Israeli National Iodine Survey Demonstrates Iodine Deficiency Among School-Aged Children and Pregnant Women

Thyroid Aug 2017, Vol. 27, No. 8: 1083-1091.


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Issue Information

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Issue Information

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Corrigendum

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Acute generalized exanthematous pustulosis and polyarthritis associated with a novel CARD14 mutation

Abstract

Acute generalised exanthematous pustulosis (AGEP) is a rare toxicoderma characterised by an acute onset rash, with many sterile pustules on the surface, high fever and increased acute phase reactants. We report the case of a patient who presented to the dermatology department with an AGEP and polyarthritis, in which a novel CARD14 mutation was identified. The pathophysiological mechanism of AGEP remains unclear, although mutations in the IL36RN gene have been identified in a small subset of AGEP patients. Similarly, mutations in the CARD14 gene have been linked to pustular types of psoriasis and familiar cases of pityriasis rubra pilaris; however, there are no reports associating mutations in the CARD14 gene with AGEP.

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Prevalence and risk factors of hand eczema in hospital-based nurses in northern China

Abstract

Background/Objectives

This study aimed to evaluate the prevalence of hand eczema among nurses in northern China and to analyse associated risk factors.

Methods

A retrospective self-reported questionnaire was designed and distributed to 1322 nurses who were selected from three regional hospitals. Risk factors related to hand eczema in nurses were analysed using multivariable logistic regression.

Results

Hand eczema was present in 183/934 respondents (20%) and was not significantly associated with gender or workplace (P > 0.05). Factors such as the use of moisturiser (odds ratio [OR] 0.6; 95% confidence interval [CI] 0.3–1.0) and not wearing impervious gloves (OR 0.5; 95% CI 0.3–0.7) were associated with lower odds of developing hand eczema. A personal (OR 5.7; 95% CI 3.9–8.2) and family history (OR 2.3; 95% CI 1.5–3.5) of atopy, washing hands more than 20 times per day (OR 1.6; 95% CI 1.1–2.4), and more years working as a nurse (OR 1.1; 95% CI 1.0–1.1) were associated with higher odds of developing hand eczema.

Conclusions

A personal and familial history of atopy, the prolonged use of impervious gloves, washing hands more than 20 times per day and more years in work as a nurse are all risk factors for increased hand eczema in nurses.

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Drug eruption caused by enzalutamide: A case and literature review of androgen receptor inhibitor-related drug eruptions

Abstract

Enzalutamide is an androgen receptor inhibitor. We report a new cutaneous eruption to this drug and review cases of drug eruptions caused by androgen receptor inhibitors.

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The bacterial species associated with aspirated foreign bodies in children

Publication date: Available online 2 August 2017
Source:Auris Nasus Larynx
Author(s): Maayan Gruber, Graeme van Der Meer, Benjamin Ling, Colin Barber, Nikki Mills, Michel Neeff, Lesley Salkeld, Murali Mahadevan
ObjectiveInhaled foreign bodies in children are common and may be complicated by secondary airway tract infection. The inhaled foreign body may act as carrier of infectious material and the aim of this study was to explore the bacterial species associated with aspirated foreign bodies in a cohort of children.MethodsRetrospective case series of 34 patients who underwent rigid laryngobronchoscopy because of foreign body aspiration. Each patient had a sample taken from tracheobronchial secretions during the procedure.ResultsThe average patient age was 31.2 months and the average hospital stay was 2.5 days. Of the foreign bodies 24 (71%) were organic in nature and 10 (29%) were non-organic. Twenty eight (82.3%) patients had mixed oropharyngeal flora organisms growth. Fifteen (44%) samples were positive for organisms other than oropharyngeal flora with the most common cultured organisms being: Streptococcus pneumonia (4/12%), Haemophilus influenza (4/12%), Moraxella catarrhalis (4/12%). Four samples (12%) grew a fungus; Candida albicans was cultured in 3 patients and Aspergillus glaucus was identified in one sample. Of the non-oropharyngeal organisms 7(47%) demonstrated antibiotic resistance with four having resistance to amoxycillin, two resistant to penicillin and one resistant to cotrimoxazole.ConclusionSome children who present with aspirated foreign body may be complicated with secondary airway infection. Antibacterial treatment might be considered in some of these cases. The regimen of antibiotics should aim to cover oropharyngeal flora, S. pneumonia, H. influenza and Moraxella catarrhalis.



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The Otology Questionnaire Amsterdam: a generic patient reported outcome measure about the severity and impact of ear complaints. A cross-sectional study on the development of this questionnaire

Abstract

Objective

Development of the Otology Questionnaire Amsterdam (OQUA); a patient reported outcome measure (PROM), measuring the severity and impact of ear complaints of patients visiting an ENT surgeon.

Design

Multicenter, cross-sectional study.Phase 1: qualitative research. In depth interviews (N=16) to identify relevant types of ear complaints and to formulate items. Pilot study of the first and second draft of the OQUA (N=32, N=39). Phase 2: quantitative research. Field-testing of the OQUA (N=352). Item reduction based on inter-item correlation, factor analysis and expert opinion.

Setting

Two secondary and two tertiary ENT clinics.

Participants

Patients over the age of sixteen visiting an ENT surgeon with an ear complaint.

Main outcome measures

Phase 1: meaning units and frequency of selected descriptions. Phase 2:inter-item correlation, factor loading and Cronbach's Alpha (α).

Results

Phase 1: eight relevant types of ear complaints were identified: earache, pressure in ear, hearing loss, tinnitus, otorrhoea, itch, dizziness and loss of taste. Phase 2: factor analysis generated a factor 'impact' (α = 0.913). The current version of the OQUA consists of 34 items, covers eight types of ear complaints and consists of two constructs: complaints and impact.

Conclusion

The OQUA is a generic, otologic PROM designed to evaluate the severity of ear complaints and their impact on patients lives.

This article is protected by copyright. All rights reserved.

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Granulomas in the gastrointestinal tract: deciphering the Pandora’s box

Abstract

Granulomas are organised collection of activated histiocytes induced by a persistent antigen stimulus. A wide variety of antigens encountered by the gastrointestinal tract are of this nature and hence the resulting granulomatous inflammation represents a tissue reaction pattern. The potential causes can be broadly classified as infections or non-infectious immune reactions. There is also a group where a cause is never identified. Granulomas may be of varying morphological appearance, most commonly epithelioid, foreign body type, suppurative and necrotizing. This may provide a clue as to the aetiology; however, in most cases, the cause requires further inquiry. Pathologists may need to cut deeper levels to look for foreign material and apply special stains to look for microorganisms. Pathologists also need to be certain that the process is a true granuloma and not a mimic. The site of occurrence in the gastrointestinal tract and the clinical setting is often paramount in establishing the aetiology. For instance, infections are more likely the cause in developing countries or when there is immunosuppression. Similarly, granulomas in the stomach are usually due to Crohn's disease; however, it is only rarely the cause of granulomas isolated to the appendix.

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Lytic eosinophils produce extracellular DNA traps as well as free eosinophil granules

Publication date: Available online 3 August 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Carl Persson, Shigeharu Ueki




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Reply

Publication date: Available online 3 August 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Anne M. Fitzpatrick, Daniel J. Jackson, David Mauger, Stanley J. Szefler




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Reply

Publication date: Available online 3 August 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Elien Gevaert, Shida Yousefi, Claus Bachert, Hans-Uwe Simon




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Nonatopic persistent asthma in children, a missed phenotype of asthma?

Publication date: Available online 3 August 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Giorgio Longo, Ester Conversano, Elisa Panontin, Giovanna Ventura, Alessandro Ventura




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Diagnosis and management of anaphylaxis in precision medicine

Publication date: August 2017
Source:Journal of Allergy and Clinical Immunology, Volume 140, Issue 2
Author(s): Mariana Castells
Anaphylaxis is the most severe and frightening of the allergic reactions, placing patients at high risk and demanding prompt recognition and immediate management by health care providers. Yet because its symptoms imitate those of other diseases, such as asthma and urticaria, current data suggest that its diagnosis is often missed, with underuse of tryptase measurement; its treatment is delayed, with little use of epinephrine; and its underlying cause or causes are poorly investigated. Deaths from anaphylaxis are difficult to investigate because of miscoding. Surprisingly, patients treated with new and powerful chemotherapy agents and humanized mAbs present with nonclassical symptoms of anaphylaxis, and patients may present with unrecognized clonal mast cell disorders with KIT mutations may present as Hymenoptera-induced or idiopathic anaphylaxis. The goal of this review is to recognize the presentations of anaphylaxis with the description of its current phenotypes, to provide new insight and understanding of its mechanisms and causes through its endotypes, and to address its biomarkers for broad clinical use. Ultimately, the aim is to empower allergists and heath care providers with new tools that can help alleviate patients' symptoms, preventing and protecting them against anaphylaxis.



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TCRαβ+ and CD19+ cell depleted Haploidentical and Mismatched Hematopoietic Stem Cell Transplantation in Primary Immune Deficiency

Publication date: Available online 3 August 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Ravi M. Shah, Reem Elfeky, Zohreh Nademi, Waseem Qasim, Persis Amrolia, Robert Chiesa, Kanchan Rao, Giovanna Lucchini, Juliana M.F. Silva, Austen Worth, Dawn Barge, David Ryan, Jane Conn, Andrew J. Cant, Roderick Skinner, Intan Juliana Abd Hamid, Terence Flood, Mario Abinun, Sophie Hambleton, Andrew R. Gennery, Paul Veys, Mary Slatter
BackgroundAllogeneic hematopoietic stem cell transplantation (HSCT) is used as a therapeutic approach for primary immunodeficiencies (PIDs). The best outcomes have been achieved using HLA-matched donors, but when a matched donor is not available, a haploidentical or mismatched unrelated donor (mMUD) can be useful. Various strategies are used to mitigate the risk of graft versus host disease (GvHD) and rejection associated with such transplants.ObjectiveWe sought to evaluate the outcomes of haploidentical or mMUD HSCT after depleting GvHD causing TCRαβCD3+ cells from the graft.MethodsCD3+TCRαβ+/CD19⁺ depleted grafts were given in conditioned (except three) children with PIDs. Treosulfan (busulfan in one), fludarabine, thiotepa and ATG or alemtuzumab conditioning was used in 77% cases, and all but four received GvHD prophylaxis.Results25 patients with 12 types of PIDs received 26 HSCTs. Three were transplanted for refractory GvHD developing after first cord transplantation. At 20.8 months (5 month- 3.3 years) median follow up, 21/25 patients survived and were cured of underlying immunodeficiency. Overall and Event Free Survival at 3 years was 83.9% and 80.4% respectively. Cumulative incidence (CI) of Grade II-IV acute GvHD was 22±8.7%. No case of visceral or chronic GvHD was seen. CI of graft failure, CMV or/and adeno viral infections and transplant related mortality at 1 year were 4.2±4.1%, 58.8±9.8% and 16.1±7.4% respectively. Patients going into transplant with systemic viral infections had poor survival in comparison to those with absent or resolved infection (33.3% vs.100%).ConclusionCD3+TCRαβ⁺ and CD19⁺ depleted haploidentical or mMUD HSCT is a practical and viable alternative for children with a range of PIDs.

Teaser

CD3+TCRαβ⁺ and CD19+ cell depleted haploidentical or mMUD HSCT has reproducible outcomes in children with a range of PIDs resulting in good immune reconstitution. Viral infections before or after HSCT are major contributors to mortality and morbidity.


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Diagnosis and management of anaphylaxis in precision medicine

Publication date: August 2017
Source:Journal of Allergy and Clinical Immunology, Volume 140, Issue 2





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The pathophysiology of anaphylaxis

Publication date: August 2017
Source:Journal of Allergy and Clinical Immunology, Volume 140, Issue 2
Author(s): Laurent L. Reber, Joseph D. Hernandez, Stephen J. Galli
Anaphylaxis is a severe systemic hypersensitivity reaction that is rapid in onset; characterized by life-threatening airway, breathing, and/or circulatory problems; and usually associated with skin and mucosal changes. Because it can be triggered in some persons by minute amounts of antigen (eg, certain foods or single insect stings), anaphylaxis can be considered the most aberrant example of an imbalance between the cost and benefit of an immune response. This review will describe current understanding of the immunopathogenesis and pathophysiology of anaphylaxis, focusing on the roles of IgE and IgG antibodies, immune effector cells, and mediators thought to contribute to examples of the disorder. Evidence from studies of anaphylaxis in human subjects will be discussed, as well as insights gained from analyses of animal models, including mice genetically deficient in the antibodies, antibody receptors, effector cells, or mediators implicated in anaphylaxis and mice that have been "humanized" for some of these elements. We also review possible host factors that might influence the occurrence or severity of anaphylaxis. Finally, we will speculate about anaphylaxis from an evolutionary perspective and argue that, in the context of severe envenomation by arthropods or reptiles, anaphylaxis might even provide a survival advantage.



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Mast cell activation syndromes

Publication date: August 2017
Source:Journal of Allergy and Clinical Immunology, Volume 140, Issue 2
Author(s): Cem Akin
Mast cell activation is common and possibly necessary for maintenance of survival. Disordered mast cell activation occurs when mast cells are pathologically overproduced or if their activation is out of proportion to the perceived threat to homeostasis. Mast cell activation syndrome refers to a group of disorders with diverse causes presenting with episodic multisystem symptoms as the result of mast cell mediator release. Despite introduction of diagnostic criteria and some advances in treatment in the last decade, many areas of mast cell activation syndrome are in need of research. This article reviews our current knowledge about the various types of mast cell activation disorders, their treatment, and areas of uncertainty in need of future investigation.



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The Editors' Choice

Publication date: August 2017
Source:Journal of Allergy and Clinical Immunology, Volume 140, Issue 2
Author(s): Cezmi A. Akdis, Zuhair K. Ballas




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News Beyond Our Pages

Publication date: August 2017
Source:Journal of Allergy and Clinical Immunology, Volume 140, Issue 2
Author(s): Marc E. Rothenberg, Jean Bousquet




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Bilateral blindness secondary to optic nerve ischemia from severe amlodipine overdose: a case report

Calcium channel blockers are commonly prescribed medications; calcium channel blocker overdose is becoming increasingly prevalent. The typical presentation of a calcium channel blocker overdose is hypotension ...

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Vitiligo and Emerging Skin Repigmentation Options

Skin diseases have enormous social and emotional burdens. For vitiligo, a condition with no drug for repigmentation approved by the US Food and Drug Administration, new options are on the horizon.
Medscape Medical News

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Safety and efficacy of topical timolol treatment of infantile haemangioma: A prospective trial

Abstract

Topical timolol therapy is considered a relatively "safer" alternative for the treatment of infantile haemangiomas (IH); however, sufficient supportive pharmacokinetic data does not exist. Most efficacy studies have not evaluated systemic absorption.^1-5 To our knowledge, this is the first prospective trial to assess the clinical response of proliferating IH to topical timolol maleate 0.5% gel-forming solution and to determine if systemic absorption occurred.Twenty-six subjects enrolled in this trial approved by the UCSD IRB.

This article is protected by copyright. All rights reserved.

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TNF antagonist induced lupus: a critically appraised topic

Abstract

TNF antagonist induced lupus is diagnosed upon a temporal relationship of TNF antagonist therapy and the development of four of the 11 American college of rheumatology (ACR) diagnostic criteria for lupus.

We critically appraise the literature on TNF antagonist induced lupus, focusing on the diagnostic criteria, dermatological manifestations, management and outcome.

Eighty cases of TNF antagonist induced lupus that met the ACR criteria were included and analysed. Infliximab was the most commonly reported agent (58.8%, n=47). Cutaneous manifestations were the most common presentation (67.5%, n=54,), specifically malar rash (26.3%, n=21) and photosensitivity (23.4%, n=19). Lupus symptoms resolved in all cases (n=80), withdrawal of the TNF antagonist was sufficient in 18.8% of cases (n=15), additional immunosuppression was required in the remaining cases. Four patients were treated with a second, different TNF antagonist, with no recurrence of symptoms. TNF antagonist induced lupus appears to have a good prognosis and treatment should be tailored to each individual case. As cutaneous features are the most common manifestation it is important that dermatologists are aware of the ACR diagnostic criteria of lupus to ensure accurate diagnoses of TNF antagonist induced lupus.

This article is protected by copyright. All rights reserved.

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Time, PASI and DLQI of psoriasis patients who “drop out” of clinical trials on etanercept due to “lack of efficacy” - a pooled analysis from ten clinical trials

SUMMARY

Background

Patient-reported outcomes in psoriasis studies are assessed at specific study time points. If a treatment has not become effective by a certain time point, it may increase the likelihood of patients being dissatisfied and leaving a clinical study.

Objectives

Generate evidence concerning the number of patients dropping out of etanercept trials over time including PASI and DLQI data.

Methods

Data from psoriasis patients in ten trials with etanercept were pooled. Analyses were performed for a) patients who dropped out due to 'lack of efficacy' b) patients who continued the trial. Psoriasis Area Severity Index (PASI) and Dermatology Life Quality Index (DLQI) data were summarized for different time points. Distribution of dropouts over time, PASI and DLQI and the proportion of patients dropping out with given treatment responses, were calculated.

Results

Of 6119 patients 128 dropped out due to 'lack of efficacy'(or synonym). The highest increase of patients dropping out happened between Day 75 and 85 (46% to 73%). The lowest PASI of patients dropping out was 6.3 within 120 days. Dropouts who achieved ≥ PASI 75 were rare.

Conclusion

A critical time when many patients might have lost their willingness to wait for their treatment with etanercept to show a better effect appeared to be at around Day 80. Most of the patients dropping out showed little improvement, stable disease or worsening of their psoriasis.

This article is protected by copyright. All rights reserved.

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Prognostic importance of a second invasive primary melanoma according to tumor stage

Abstract

It is well known that a considerable proportion of the Caucasian population in many countries are diagnosed with multiple melanomas over time¹. Despite this, most analyses of melanoma survival, including that of the American Joint Committee on Cancer (AJCC), consider only one tumor for every patient and disregard the potential effect of multiple melanomas on outcomes and this is reflected in the lack of specific management guidelines for patients with multiple melanomas beyond those dictated by the clinicopathological features of each tumour^2-4.

Recently we and others have shown that patients with multiple primary invasive melanomas have an increased risk of melanoma death compared to patients with a single melanoma^5,6.

This article is protected by copyright. All rights reserved.

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Neurotrophin Gene Therapy in Deafened Ears with Cochlear Implants: Long-term Effects on Nerve Survival and Functional Measures

Abstract

Because cochlear implants function by stimulating the auditory nerve, it is assumed that the condition of the nerve plays an important role in the efficacy of the prosthesis. Thus, considerable research has been devoted to methods of preserving the nerve following deafness. Neurotrophins have been identified as a potential contributor to neural health, but most of the research to date has been done in young animals and for short periods (less than 3 to 6 months) after the onset of treatment. The first objective of the current experiment was to examine the effects of a neurotrophin gene therapy delivery method on spiral ganglion neuron (SGN) preservation and function in the long term (5 to 14 months) in mature guinea pigs with cochlear implants. The second objective was to examine several potential non-invasive monitors of auditory nerve health following the neurotrophin gene therapy procedure. Eighteen mature adult male guinea pigs were deafened by cochlear perfusion of neomycin and then one ear was inoculated with an adeno-associated viral vector with an Nft3-gene insert (AAV.Ntf3) and implanted with a cochlear implant electrode array. Five control animals were deafened and inoculated with an empty AAV and implanted. Data from 43 other guinea pig ears from this and previous experiments were used for comparison: 24 animals implanted in a hearing ear, nine animals deafened and implanted with no inoculation, and ten normal-hearing non-implanted ears. After 4 to 21 months of psychophysical and electrophysiological testing, the animals were prepared for histological examination of SGN densities and inner hair cell (IHC) survival. Seventy-eight percent of the ears deafened and inoculated with AAV.Ntf3 showed better SGN survival than the 14 deafened-control ears. The degree of SGN preservation following the gene therapy procedure was variable across animals and across cochlear turns. Slopes of psychophysical multipulse integration (MPI) functions were predictive of SGN density, but only in animals with preserved IHCs. MPI was not affected by the AAV.Ntf3 treatment, but there was a minor improvement in temporal integration (TI). AAV.Ntf3 treatment had significant effects on ECAP and EABR amplitude growth func-tion (AGF) slopes; the reduction in slope in deafened ears was ameliorated by the AAV.Ntf3 treatment. Slopes of the ECAP and EABR AGFs were predictive of SGN density in a broad area near and just apical to the implant. The highest ensemble spontaneous activity (ESA) values were seen in animals with surviving IHCs, but AAV.Ntf3 treatment in deafened ears resulted in slightly higher ESA values compared to deafened untreated ears. Overall, a combination of the psychophysical and electrophysiological measures can be useful for monitoring the health of the implanted cochlea in guinea pigs. These measures should be applicable for assessing cochlear health in human subjects.

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Local IL-25 contributes to Th2-biased inflammatory profiles in nasal polyps

Summary

Background

IL-25 has been proposed to play a key role in the pathogenesis of chronic rhinosinusitis with nasal polyps (CRSwNP). This study aims to evaluate the association of IL-25 with the Th2-biased inflammatory profiles in CRSwNP.

Methods

Nasal polyp (NP) tissues and control uncinate process tissues were collected from 92 patients with CRSwNP, 20 patients with chronic rhinosinusitis without nasal polyps (CRSsNP), and 16 normal control subjects. IL-25 expression was examined using immunohistochemistry and immunofluorescence staining, flow cytometry, RT-qPCR, and ELISA. The inflammatory profiles and clinical characteristics of 2 NP subtypes (IL-25^high and IL-25^low) were evaluated, and the effects of IL-25 on Th2 cytokine production in cultured dispersed polyp cells were examined in vitro.

Results

The mRNA and protein levels of IL-25 were significantly increased in the polyp tissues compared with the control uncinate process tissues. The IL-25^high subtype showed greater computed tomography scores, endoscopic scores, and Th2 response. Exposure to IL-25 activated type 2 innate lymphoid cells and Th2 cells in NP simultaneously which further increased Th2 cytokine production in vitro.

Conclusions

Local IL-25 plays a crucial role in promoting Th2-biased inflammatory profiles in NP, and may serve as a promising therapeutic target in CRSwNP patients.

This article is protected by copyright. All rights reserved.

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Challenges in the implementation of EAACI Guidelines on Allergen Immunotherapy: A global perspective on the regulation of allergen products

Abstract

Regulatory approaches for allergen immunotherapy (AIT) products and the availability of high quality AIT products are inherently linked to each other. While allergen products are available in many countries across the globe, their regulation is very heterogeneous. First, we describe the regulatory systems applicable for AIT products in the European Union (EU) and in the United States (US). For Europe, a depiction of the different types of relevant procedures, as well as the committees involved is provided and the fundamental role of national agencies of the EU member states in this complex and unique network is highlighted. Furthermore, the regulatory agencies from Australia, Canada, Japan, Russia, and Switzerland provided information on the system implemented in their countries for the regulation of allergen products. While AIT products are commonly classified as biological medicinal products, they are made available by varying types of procedures, most commonly by either obtaining a marketing authorisation or by being distributed as named patient products. Exemptions from marketing authorisations in exceptional cases, as well as import of allergen products from other countries, are additional tools applied by countries to ensure availability of needed AIT products. Several challenges for AIT products are apparent from this analysis and will require further consideration.

This article is protected by copyright. All rights reserved.

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Assessment of eosinophilic airway inflammation as a contribution to the diagnosis of occupational asthma

Abstract

Background

Ascertaining the presence of asthma through the assessment of nonspecific bronchial hyperresponsiveness (NSBH) is a key step in the diagnosis of occupational asthma (OA). We aimed at investigating whether indices of airway inflammation including fractional exhaled nitric oxide (FeNO) and sputum eosinophils would be useful adjuncts to the measurement of NSBH in diagnosing OA defined as a positive specific inhalation challenge (SIC).

Methods

The study included 240 consecutive subjects with a suspicion of OA who completed a SIC, of whom 133 showed a positive response. The sensitivity, specificity, and predictive values of NSBH, and FeNO, as well as sputum eosinophil counts assessed at baseline of the SIC were determined.

Results

A concentration of histamine inducing a 20% decline in FEV₁ (PC₂₀) ≤16 mg/mL showed a sensitivity of 87% and a specificity of 36%. A FeNO level ≥25 ppb and a sputum eosinophil count ≥ 2% provided lower sensitivity rates (47% and 39%, respectively) than the PC20 value. Eight of the 17 subjects without baseline NSBH despite a positive SIC showed a sputum eosinophil count ≥2%, a FeNO level ≥25 ppb or both outcomes. Combining either a PC₂₀ value ≤16mg/mL or a FeNO ≥25 ppb increased the sensitivity to 91%. Using either a PC₂₀ ≤16mg/mL or a sputum eosinophil count ≥1% increased the sensitivity to 94%.

Conclusion

Adding the assessment of FeNO level and sputum eosinophils to NSBH improves the identification of subjects who may have OA and require further objective testing before excluding the possibility of OA.

This article is protected by copyright. All rights reserved.

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Neurotrophin Gene Therapy in Deafened Ears with Cochlear Implants: Long-term Effects on Nerve Survival and Functional Measures

Abstract

Because cochlear implants function by stimulating the auditory nerve, it is assumed that the condition of the nerve plays an important role in the efficacy of the prosthesis. Thus, considerable research has been devoted to methods of preserving the nerve following deafness. Neurotrophins have been identified as a potential contributor to neural health, but most of the research to date has been done in young animals and for short periods (less than 3 to 6 months) after the onset of treatment. The first objective of the current experiment was to examine the effects of a neurotrophin gene therapy delivery method on spiral ganglion neuron (SGN) preservation and function in the long term (5 to 14 months) in mature guinea pigs with cochlear implants. The second objective was to examine several potential non-invasive monitors of auditory nerve health following the neurotrophin gene therapy procedure. Eighteen mature adult male guinea pigs were deafened by cochlear perfusion of neomycin and then one ear was inoculated with an adeno-associated viral vector with an Nft3-gene insert (AAV.Ntf3) and implanted with a cochlear implant electrode array. Five control animals were deafened and inoculated with an empty AAV and implanted. Data from 43 other guinea pig ears from this and previous experiments were used for comparison: 24 animals implanted in a hearing ear, nine animals deafened and implanted with no inoculation, and ten normal-hearing non-implanted ears. After 4 to 21 months of psychophysical and electrophysiological testing, the animals were prepared for histological examination of SGN densities and inner hair cell (IHC) survival. Seventy-eight percent of the ears deafened and inoculated with AAV.Ntf3 showed better SGN survival than the 14 deafened-control ears. The degree of SGN preservation following the gene therapy procedure was variable across animals and across cochlear turns. Slopes of psychophysical multipulse integration (MPI) functions were predictive of SGN density, but only in animals with preserved IHCs. MPI was not affected by the AAV.Ntf3 treatment, but there was a minor improvement in temporal integration (TI). AAV.Ntf3 treatment had significant effects on ECAP and EABR amplitude growth func-tion (AGF) slopes; the reduction in slope in deafened ears was ameliorated by the AAV.Ntf3 treatment. Slopes of the ECAP and EABR AGFs were predictive of SGN density in a broad area near and just apical to the implant. The highest ensemble spontaneous activity (ESA) values were seen in animals with surviving IHCs, but AAV.Ntf3 treatment in deafened ears resulted in slightly higher ESA values compared to deafened untreated ears. Overall, a combination of the psychophysical and electrophysiological measures can be useful for monitoring the health of the implanted cochlea in guinea pigs. These measures should be applicable for assessing cochlear health in human subjects.

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Transoral Robotic Surgery

To the Editor Hatten et al hypothesize 2 potential roles of transoral robotic surgery-assisted endoscopy (TORS-AE) for deintensification in the treatment of patients presenting with metastatic carcinoma of the neck from an unknown primary site (CUP).

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Transoral Robotic Surgery—Reply

In Reply We appreciate Drs Garden and Morrison's interest in our recently published article reviewing the surgical experience of transoral robotic surgery-assisted endoscopy (TORS-AE) for carcinoma of unknown primary (CUP).

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Development and Validation of a Consumer Questionnaire to Detect Ear Disease

This study develops and validates a consumer questionnaire for the self-assessment of risk for ear diseases associated with hearing loss.

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Focus Groups to Develop a Cochlear Implant Quality-of-Life Instrument

This study used focus groups consisting of adult cochlear implant users to identify content areas and domains for developing the item bank for a cochlear implant–specific quality-of-life instrument.

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Factors to Consider When Deciding on the Type of Free-Flap Reconstruction of Head and Neck Soft Tissue Defects

Objectives: The aim of the study was to evaluate the functional and aesthetic outcomes on donor and recipient sites and to determine the effects of technical factors including flap thickness and vessel diameters measured by ultrasonography as well as the size of the defect and postoperative volume reduction of the flaps measured by magnetic resonance imaging (MRI). Methods: In patients who had undergone soft tissue reconstructive surgery between March 2013 and March 2016 using 55 anterolateral thigh flaps (ALTFs), 30 radial forearm flaps (RFFs), and 18 latissimus dorsi flaps (LDFs), color Doppler ultrasonography was performed to measure the thickness of the flap at the site of the perforator. Preoperative color Doppler ultrasound examinations of the blood vessel diameters of donor and recipient sites were carried out. Results: 97.1% of flaps showed complete survival and 2.9% complete failure (2 ALTFs and 1 LDF). The difference in flap volume of ALTFs, RFFs, and LDFs between MRI 1 (3-6 weeks) and MRI 2 (6-18 months) was 27.6, 17.9, and 36.1%, respectively. Conclusion: Proper selection of the flap is important for the optimization of the aesthetic and functional outcomes. Ultrasound, the surgeon's experience and the extension and nature of the defect play a key role in the selection of the flap.
ORL 2017;79:230-238

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Toxidermie à l’amoxicilline épargnant une plaque d’érythème migrant : phénomène de Renbök et revue de la littérature

Publication date: Available online 3 August 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): M. Dumas, L. Blum, E. Begon
IntroductionLe phénomène de Renbök désigne le respect, par une seconde dermatose, d'une zone de peau touchée précédemment par une première dermatose. Nous présentons le premier cas de phénomène de Renbök au cours d'un érythème migrant (EM), épargné par une toxidermie, et faisons une revue exhaustive de la littérature.ObservationUne patiente de 60 ans était traitée par amoxicilline pour un EM du genou droit. Sept jours plus tard, elle développait une toxidermie à type d'exanthème maculopapuleux généralisé, respectant avec une nette délimitation la plaque d'EM. Le diagnostic de phénomène de Renbök était posé.DiscussionLe phénomène de Renbök a été décrit en 1981 par Cochran et al., qui avaient constaté le respect, par une toxidermie, d'un site antérieurement irradié pour tumeur. Depuis, une quarantaine de cas ont été rapportés. La plupart décrivent des plaques de psoriasis anciennes épargnées lors d'une pelade. La principale hypothèse physiopathologique proposée est l'existence d'un état réfractaire sur une zone topographique donnée, où une modification de l'environnement cytokinique induit par la première dermatose empêcherait la seconde de se développer.ConclusionLe phénomène de Renbök est l'inverse du phénomène de Köbner. Nous décrivons le premier cas au cours d'une phase primaire de borréliose compliquée de toxidermie aux aminopénicillines. Ce phénomène clinique spectaculaire et rare de non-chevauchement entre deux dermatoses est une source d'enseignements sur la physiopathologie de la réponse inflammatoire cutanée.IntroductionRenbök phenomenon describes the inhibition of a lesion when a different one appears. We describe the first case of Renbök phenomenon occurring in a context of erythema migrans (EM) spared by an amoxicillin-induced skin rash and we also present a literature review.Case reportA 60-year-old patient was treated with amoxicillin for EM on the right knee and subsequently developed generalized erythema as a result of an antibiotic-induced skin rash, with sparing of the area previously affected by EM. Renbök phenomenon was diagnosed.DiscussionIn 1981, Cochran et al. first described a maculopapular drug reaction, which spared the sites of previous X irradiation for a tumor. Since then, nearly 40 cases have been reported, mostly describing patient with alopecia areata of the scalp with hair growth within plaques of psoriasis. One of the mechanisms suggested is a role played by cytokine cross-regulation in competition among distinct immune responses.ConclusionWe report the first case of Renbök phenomenon involving EM spared by a drug reaction. This phenomenon provides an insight into inflammatory response competition within a single patient.



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Frequency of self-reported drug allergy

Publication date: Available online 2 August 2017
Source:Annals of Allergy, Asthma & Immunology
Author(s): Bernardo Sousa-Pinto, João Almeida Fonseca, Eva Rebelo Gomes
BackgroundPatients reporting drug allergy are treated with second-line therapies, with possible negative clinical and health consequences.ObjectiveTo assess the prevalence of self-reported drug allergy.MethodsWe performed a systematic review of observational studies assessing the prevalence of self-reported drug allergy. We searched 4 electronic databases. From selected studies, we extracted data on self-reported drug allergy prevalence, study design, participants' demographic characteristics, reported clinical manifestations, and suspected culprit drugs. We performed a random-effects meta-analysis followed by a meta-regression.ResultsFifty-three studies were included in the systematic review, assessing a total of 126,306 participants, of whom 8.3% (range across studies 0.7–38.5%) self-reported drug allergy. Cutaneous manifestations were reported by 68.2% of participants, and anaphylactic or systemic reactions were reported by 10.8%. Antibiotics, nonsteroidal anti-inflammatory drugs, and anesthetics were the most frequently reported culprit drug classes. The frequency of self-reported drug allergy was higher in female (11.4%) than in male (7.2%) patients, adults (10.0%) than in children (5.1%), and in studies in the medical setting (15.9% in inpatients, 11.4% in outpatients) than in the general population (5.9%). The meta-analysis rendered a pooled prevalence of 7.9% (95% confidence interval 6.4–9.6), and the meta-regression identified study region, participants' age group, and study setting as factors associated with significant heterogeneity. Confirmation tests (including skin, in vitro, and drug provocation tests) were performed in only 3 studies.ConclusionThe prevalence of self-reported drug allergy is highly variable and is higher in female patients, adults, and inpatients. To overcome this variability, further studies using confirmation tests are needed.



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A Study of Epacadostat + Pembrolizumab in Head and Neck Cancer Patients, Who Failed Prior PD-1/PD-L1 Therapy

Condition:   Head and Neck Cancer
Interventions:   Drug: Pembrolizumab;   Drug: epacadostat
Sponsor:   University of Chicago
Not yet recruiting - verified August 2017

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Salvage Brachytherapy and Hyperthermia for Recurrent H&N-tumours

Condition:   Locally Recurrent Head and Neck Cancer
Interventions:   Radiation: Brachytherapy;   Other: Hyperthermia
Sponsor:   University of Erlangen-Nürnberg Medical School
Recruiting - verified July 2017

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Nivolumab With or Without Tadalafil in Treating Patients With Recurrent Head and Neck Squamous Cell Carcinoma That Can Be Removed by Surgery

Conditions:   Lip, Oral Cavity and Pharynx;   Larynx
Interventions:   Biological: Nivolumab;   Drug: Tadalafil;   Procedure: Therapeutic Conventional Surgery
Sponsors:   Sidney Kimmel Cancer Center at Thomas Jefferson University;   Bristol-Myers Squibb
Not yet recruiting - verified July 2017

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89Zr-Df-IAB22M2C PET/CT in Patients With Selected, Metastatic Solid Malignancies or Hodgkin's Lymphoma

Conditions:   Positron-Emission Tomography;   Immunomodulation;   Metastatic Solid Malignancies;   Hodgkin Lymphoma
Intervention:   Drug: 89Zr-Df-IAB22M2C Infusion
Sponsor:   ImaginAb, Inc.
Recruiting - verified August 2017

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Bilateral pneumothoraces following acupuncture

A 50-year-old woman was brought to the emergency department with shortness of breath and chest tightness following acupuncture to her upper back for a chronically painful left shoulder. She had symptoms of respiratory distress and chest X-ray revealed bilateral pneumothoraces. Symptoms resolved after insertion of bilateral Seldinger chest drains. She was admitted to the Cardiothoracic Surgery ward, chest drains were removed on the second and third days and the patient was discharged from hospital after 3 days. Clinicians and acupuncturists should be aware of this adverse event following acupuncture.

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Poland syndrome: a case report

Description

Poland syndrome (PS) is a rare chest wall developmental anomaly characterised by ipsilateral agenesis/hypoplasia of the sternocostal head of pectoralis major, hypoplasia of nipple or breast, absence of subcutaneous fat, multiple rib abnormalities, elevated and rotated scapula (Sprengel deformity) and ipsilateral digit abnormalities (brachydactyly, syndactyly). These findings vary and all are rarely found in a single individual.

Our case involves an 8-year-old boy with no medical history who presented for evaluation of chronic rhinitis. There was no breathing or cardiac complaints on review of systems. Physical examination showed chest asymmetry with right anterior chest wall depression and flattening of the right pectoral region with displaced nipple (figure 1). Abduction of the shoulders showed absence of the sternocostal head of pectoralis major. Hand examination did not show any signs of ipsilateral digital abnormality.

Figure 1

Chest asymmetry with right anterior chest wall depression and...

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POEMS syndrome in a patient with rheumatoid arthritis

Multiple myeloma has been reported to be associated with rheumatoid arthritis (RA). POEMS syndrome is a rare variant of multiple myeloma and is characterised by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. We report the case of a 67-year-old patient with RA who developed numbness and tingling in both legs due to polyneuropathy. CT showed a massive right pleural effusion and a sclerotic lesion in the right ninth rib. Histopathological examination of the rib revealed IgA lambda-type plasmacytoma. Serum vascular endothelial growth factor was extremely high at 5530 pg/mL. We made a diagnosis of POEMS syndrome. A literature search of the PubMed database identified only two documented cases of POEMS syndrome in patients with RA. Neuropathies are reportedly more frequent in patients with RA than in the general population. Rheumatologists should consider POEMS syndrome in patients with RA and neurological symptoms.

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Acute interstitial nephritis and DRESS syndrome without eosinophilia associated with cefepime

Description

The drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) is a potentially life-threatening drug-induced reaction characterised by a severe skin eruption, fever, haematological abnormalities (eosinophilia or atypical lymphocytes) and internal organ involvement (liver is the most common). These manifestations are thought to be a type IV hypersensitivity with a delayed onset of usually 2–6 weeks after the initiation of drug therapy.1 2 While the pathogenesis of this condition is not well understood, mechanisms such as detoxification defects leading to reactive metabolite formation and subsequent immunological reactions, slow acetylation and reactivation of Epstein-Barr virus and human herpesvirus 6 and 7 have been proposed.2 The most common drugs associated with DRESS syndrome are the aromatic anticonvulsants (phenytoin, phenobarbital, carbamazepine) and sulfonamides.3

A 62-year-old man with poorly controlled diabetes and subacute osteomyelitis presented to the hospital with fatigue, fever and anorexia. Prior...

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Flexion deformity of the finger caused by tophaceous gout of the flexor tendon

Description

A 53-year-old, right-handed man was referred to orthopaedic clinic with a 1-year history of progressive 'trigger-like' symptoms and eventual locking of the right ring and middle fingers. There was no history of trauma or joint problems. Medical history included stable chronic kidney disease and well-controlled hypertension. On examination of the right hand, there was limited passive mobility with complete flexion of the ring and middle fingers by 100° at the proximal interphalangeal joint, and additionally the ring finger by 45° at the distal interphalangeal joint. The left hand was unaffected. Furthermore, in the context of a raised serum uric acid concentration of 767 μmol/L 3 years previously, two palpable, non-tender nodules were found on the left olecranon.

Surgical exploration of the right ring finger found multiple small, white speckled deposits infiltrating the flexor tendon from the first annular pulley (A1) to just beyond the A3 pulley (

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Atypical presentation of false asystole detection in implantable loop recorder

Description

A 57-year-old male patient was admitted for evaluation of pacemaker explantation 7 years after implantation because of paroxysmal complete atrioventricular (AV) block due to presumed lyme carditis. In view of a stable ventricular pacing rate of <1%, complete removal of the system without reimplantation was considered. An electrophysiological study revealed normal AV conduction and normal AVBCL (atrioventricular block cycle length). Therefore, pacemaker removal and lead extraction were scheduled, the pacemaker was set to a VVI 30/min mode and a newer generation implantable loop recorder (ILR, Medtronic Reveal LINQ, Medtronic, Dublin, Ireland) was implanted for remote monitoring of the patient's heart rhythm. At a regular outpatient visit 2 weeks later, the ILR indicated an episode of asystole over 7.8 s (figure 1) during the daytime 1 day after ILR implantation. The patient did not report any symptoms at that time. Pacemaker interrogation showed appropriate function. The episode was hence considered erroneous asystole...

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A garden of parathyroid adenomas

Despite improvements in parathyroidectomy success rates, patients with persistent primary hyperparathyroidism (PHPT) after initial surgery continue to challenge clinicians. Some of the challenges are due to ectopic parathyroid adenomas, including thymic, intrathyroidal, carotid sheath and mediastinal glands, and others are because of supernumerary glands. While uncommon in PHPT, multigland disease is also an important consideration in patients requiring reoperative surgery for persistent disease. For this reason, localisation studies including sestamibi, ultrasound, CT scan and venous sampling for abnormal glands may be an essential component of the preoperative workup. In this report, we describe an unusual case of a patient who required a total of four operations to cure PHPT arising from seven parathyroid adenomas.

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Descended right superior parathyroid adenoma mimicking as inferior adenoma in primary hyperparathyroidism

Description

A 22-year-old woman presented with generalised bony pains over past 2 years. Clinical examination was unremarkable, and blood biochemistry revealed parathyroid hormone (PTH)-dependent hypercalcaemia, suggestive of primary hyperparathyroidism (albumin-corrected calcium –11 mg/dL (normal range 8.3–10.4); PTH –256 pg/mL (normal range 8–50)). Her creatinine was 0.8 mg/dL (normal range 0.6–1.2) and 25-OH vitamin D level was 31 ng/mL (30–75). She had low bone mass at distal end of radius (Z score of –2.8) and there was no evidence of renal stones. On localisation, both parathyroid scinitigraphy and ultrasound of neck localised the lesion to right inferior gland (figure 1A,B). There were no features of multiple endocrine neoplasia type 1.

Figure 1

(A)Parathyroid scintigraphy displaying an adenoma at right side. (B) Ultrasound neck showing right-sided adenoma.

She underwent focused right inferior parathyroidectomy, without intraoperative PTH (IOPTH) assay. Postoperatively,however, she had persistent hyperparathyroidism, and biopsy from the excised lesion revealed...

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Opaque intraocular lens for correction of intractable diplopia and torticollis

Description

A 72-year-old woman was referred to our hospital for treatment of persistent diplopia and bilateral large angle of esotropia (>50 prismatic dioptres): She had a 12-year previous history of double vision attributed to double paralysis of the VI nerve after a traffic accident. In order to avoid double vision, she permanently misplaced her head in torticollis by turning it all the way to the left. The left eye was the fixator and the view of the right eye was occluded by the nose (figure 1). Due to this, the patient suffered cervical contractures and experienced poor quality of life.

Figure 1

Torticollis and large bilateral esotropia.

On examination, best-corrected visual acuities were 20/70 in the right eye and 20/30 in the left eye. Orthoptic assessment revealed a complete paralysis of both VI cranial nerves with total absence of abduction. The...

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Dialysis-associated steal syndrome with limb ischaemia

Description

A 62-year-old man with end-stage renal failure on dialysis was referred to our hospital with worsening pain and ulceration in his right arm. He was started on haemodialysis 6 months ago, once his newly created brachiocephalic fistula was matured. Previously, he had been on peritoneal dialysis for over 2 years and was transitioned to haemodialysis after multiple catheter-site infections and peritonitis. His medical history was significant for poorly controlled hypertension and hypercholesterolaemia. On physical examination, necrosis of the skin and subcutaneous tissue localised to the right forearm and hand was evident (figure 1), along with dry gangrene of the fourth digit (figure 2). Right radial and ulnar artery pulses were absent. The arm distal to the fistula was cool, with decreased capillary refill and decreased sensation. Continuous handheld Doppler revealed exceptionally low radial and ulnar artery pulse signals and absence of flow at the digital...

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Challenging airway in a paediatric patient impaled by a freezer pop stick

Description

As experts of the human airway, it is our job as anaesthesiologists to control and secure the airway in the safest way possible for the patient. The perioperative anaesthetic plan always includes an airway component, ranging from bag-mask ventilation to endotracheal intubation. Routine surgical procedures may warrant a certain standardised airway strategy that may vary among institutions. However, there exist situations that are less common, in which standard airway practice is unclear. If the injury is suspected to be in a highly sensitive area and securing an airway may compromise or cause further injury, radiographic imaging may be warranted.1

Figure 1 depicts a paediatric patient impaled in the soft palate of the left oropharynx by a freezer pop stick that the patient had been previously enjoying and accidentally fell onto. (left of image). The CT scan portion of the figure is a contrast CT...

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All That Wheezes…

Foreword. In this Journal feature, information about a real patient is presented in stages (boldface type) to an expert clinician, who responds to the information, sharing his or her reasoning with the reader (regular type). The authors' commentary follows. Stage. A 20-year-old woman presented to…

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Limited effectiveness with a 10-day bismuth-containing quadruple therapy (Pylera®) in third-line recue treatment for Helicobacter pylori infection. A real-life multicenter study

Abstract

Background

Helicobacter pylori antibiotic resistance is an increasing problem worldwide. Pylera^® may be an option as salvage therapy.

Aim

To assess the effectiveness, safety, and tolerance of Pylera^® as a third-line in clinical practice.

Materials and Methods

This was a multicenter, observational, prospective database study in four Spanish hospitals. Consecutive H. pylori-infected individuals treated with Pylera^® and a proton-pump inhibitor (PPI) were invited to participate if they had failed to respond to PPI-clarithromycin-amoxicillin as first-line and to levofloxacin-amoxicillin-PPI as second-line therapy. Eradication was tested 4-8 weeks after Pylera^® using a C¹³-urea breath test. Treatment-related adverse effects (TRAEs) were assessed through a questionnaire and by reviewing databases. A questionnaire on patient satisfaction was completed in the last visit.

Results

Of 103 subjects fulfilling the selection criteria, 101 were included in the intention-to-treat (ITT) analysis and 97 in the per-protocol (PP) analysis. A 10 day course was prescribed in all patients. Esomeprazole 40 mg b.i.d. was the most used PPI regimen (ITT=94.1%). Ninety-seven individuals (ITT=96.04%) completed more than 90% of the treatment. Overall eradication rates were ITT=80.2% (95% confidence interval [CI]: 72.3%-88.1%) and PP=84.4% (95% CI: 76.8%-91.8%). One or more TRAEs were experienced by 67.3% (95% CI: 57.7%-75.7%), all mild or moderate. TRAEs and the number of pills were the main complaints.

Conclusion

In an area of high antibiotic resistance to H. pylori, 10-day Pylera^® plus double-dose PPI emerged as an alternative as third-line therapy, although not achieving optimal eradication rates. TRAEs were common but were neither severe nor did they condition compliance.

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Effect on Helicobacter pylori eradication therapy against gastric cancer in Japan

Abstract

Background

In Japan, there have been approximately 50 000 deaths from gastric cancer annually for over 40 years with little variation. It has been reported that most gastric cancers in Japan are caused by Helicobacter pylori infection. H. pylori eradication therapy was approved for patients with chronic gastritis by the Japanese national health insurance scheme in February 2013 for patients with an endoscopic diagnosis of chronic gastritis is positive for H. pylori. We examined the effect on gastric cancer death rate 4 years after expansion of health insurance coverage.

Aim

We conducted an epidemiological study and analyzed trends in prescription for H. pylori eradication therapy. We used the electronic medical claims database from Hokkaido, Japan to evaluate the impact of expansion of national health insurance coverage for H. pylori eradication therapy on deaths from gastric cancer.

Methods

Data on deaths from gastric cancer were obtained from the Japanese Ministry of Health, Labour and Welfare and the Cancer Statistics in Japan (2015). Analysis of electronic claims records was performed using the National Database, mainly focusing on Hokkaido. Prescriptions for H. pylori eradication therapy and the number of patients treated for gastric cancer were also extracted from the Hokkaido database.

Results

Approximately 1.5 million prescriptions for H. pylori eradication therapy were written annually. Gastric cancer deaths fell each year: 48 427 in 2013, 47 903 in 2014, 46 659 in 2015, and 45 509 in 2016, showing a significant decrease after expansion of insurance coverage for H. pylori eradication therapy (P<.0001).

Conclusions

Prescriptions for H. pylori eradication therapy increased markedly after approval of the gastritis indication by the national health insurance scheme and was associated with a significant decrease in gastric cancer deaths.

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Donor-Cell Origin High-Risk Myelodysplastic Syndrome Synchronous with an Intracranial Meningioma-Like Tumor, 8 Years after Allogeneic Hematopoietic Stem Cell Transplantation for Chronic Lymphocytic Leukemia

Secondary neoplasias are well known consequences of radiotherapy or chemotherapy for a primary cancer. In this report, we describe two rare secondary neoplasias occurring in the same patient: a meningioma-like intracranial tumor and high-risk myelodysplastic syndrome (MDS) of donor-cells origin, both diagnosed simultaneously, 8 years after an allogeneic hematopoietic stem cell transplantation (allo-HSCT) for chronic lymphocytic leukemia (CLL). Due to an engraftment failure during the first allo-HSCT of a matched related donor for CLL treatment, the salvage treatment was a second allo-HSCT. At the moment of meningioma-like tumor diagnosis, the patient was pancytopenic due to high-risk MDS, so it was decided to postpone a surgical intervention until hematological improvement. For the high-risk MDS of donor-cells origin the chosen treatment was induction with intensive chemotherapy. Due to refractory disease, the patient was treated with 5-azacitidine and donor-lymphocytes infusion with no response and, finally, a third allo-HSCT of a matched unrelated donor was performed. The patient died 6 months after the third allo-HSCT, in cytogenetic remission but without hematological recovery, due to an intracranial hemorrhage with origin in the meningioma-like tumor.

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“How I treat” Autoimmune Diseases: State of the art on the management of rare rheumatic diseases and ANCA-associated systemic idiopathic vasculitis

Publication date: Available online 2 August 2017
Source:Autoimmunity Reviews
Author(s): Dario Roccatello
This Special Issue of Autoimmunity Reviews constitutes summaries of presentations at the 20th International Meeting on Immune Pathology and Orphan Diseases, held in Torino, Italy, 25-28th January 2017. As such, these presentations represent the state of the art on the pathophysiology of autoimmune diseases as well as the most recent insights into the management of these pathologic conditions. The latter includes both the optimal use of established drugs and approaches as well as novel knowledge on the means and consequences of targeted blocking of molecules or cellular mechanisms.The 2nd Turin Congress on systemic idiopathic vasculitis concluded the works of the International Meeting on Immune Pathology and Orphan Diseases.This Satellite Congress was mainly addressed to the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitis: advances on induction therapy and maintenance treatment. Guidelines and recommendations were critically discussed, reviewing available evidence and providing experts' insights. New intensive therapeutic approaches had been also reported.



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Antineutrophil Cytoplasmic Antibody-Associated Vasculitides and IgG4-Related Disease: A new overlap syndrome

Publication date: Available online 2 August 2017
Source:Autoimmunity Reviews
Author(s): François-Xavier Danlos, Giovanni Maria Rossi, Daniel Blockmans, Giacomo Emmi, Andreas Kronbichler, Stéphane Durupt, Claire Maynard, Luminita Luca, Cyril Garrouste, Bertrand Lioger, Rachel Mourot-Cottet, Robin Dhote, Jean-Benoit Arlet, Thomas Hanslik, Philippe Rouvier, Mikael Ebbo, Xavier Puéchal, Dominique Nochy, Agnès Carlotti, Luc Mouthon, Loïc Guillevin, Augusto Vaglio, Benjamin Terrier
ObjectiveAtypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap.MethodsWe conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria.ResultsEighteen patients were included (median age 55.5 years, 13 men). AAV and IgG4-RD were diagnosed concomitantly in 13/18 (72%) patients; AAV preceded IgG4-RD in 3/18 (17%) while IgG4-RD preceded AAV in 2/18 (11%). AAV diagnoses included granulomatosis with polyangiitis in 14 (78%), microscopic polyangiitis in 3 (17%), and eosinophilic granulomatosis with polyangiitis in one case. IgG4-RD diagnosis included definite IgG4-RD in 5 (28%) cases, probable IgG4-RD in 5 (28%) and possible IgG4-RD in 8 (44%). IgG4-RD manifestations were chronic periaortitis in 9/18 (50%) patients, orbital mass and tubulointerstitial nephritis in 4 (22%) cases, prevertebral fibrosis in 3 (17%), pachymeningitis and autoimmune pancreatitis in 2 (11%) cases. Patients required median number of 2 (range 0–4) lines of immunosuppressants in combination with glucocorticoids. During the follow-up (median 49,8 months, range 17,25–108 months), AAV manifestations relapsed in 10/18 (56%) cases and IgG4-RD lesions in 5/18 (28%). When used, mainly for relapses, rituximab showed response in all cases.ConclusionAAV and IgG4-RD may overlap. Clinicians should consider that atypical manifestations during AAV could be related to IgG4-RD rather than to refractory granulomatous or vasculitic lesions.



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Evaluation of a xenogeneic vascular endothelial growth factor-2 vaccine in two preclinical metastatic tumor models in mice

Abstract

In this study, a xenogeneic DNA vaccine encoding for human vascular endothelial growth factor receptor-2 (hVEGFR-2) was evaluated in two murine tumor models, the B16-F10 melanoma and the EO771 breast carcinoma model. The vaccine was administered by intradermal injection followed by electroporation. The immunogenicity and the biological efficacy of the vaccine were tested in (1) a prophylactic setting, (2) a therapeutic setting, and (3) a therapeutic setting combined with surgical removal of the primary tumor. The tumor growth, survival, and development of an immune response were followed. The cellular immune response was measured by a bioluminescence-based cytotoxicity assay with vascular endothelial growth factor-2 (VEGFR-2)-expressing target cells. Humoral immune responses were quantified by enzyme-linked immunosorbent assay (ELISA). Ex vivo bioluminescence imaging and immunohistological observation of organs were used to detect (micro)metastases. A cellular and humoral immune response was present in prophylactically and therapeutically vaccinated mice, in both tumor models. Nevertheless, survival in prophylactically vaccinated mice was only moderately increased, and no beneficial effect on survival in therapeutically vaccinated mice could be demonstrated. An influx of CD3+ cells and a slight decrease in VEGFR-2 were noticed in the tumors of vaccinated mice. Unexpectedly, the vaccine caused an increased quantity of early micrometastases in the liver. Lung metastases were not increased by the vaccine. These early liver micrometastases did however not grow into macroscopic metastases in either control or vaccinated mice when allowed to develop further after surgical removal of the primary tumor.

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Swollen Optic Disc and Sinusitis

Purpose: To present a case of optic disc swelling caused by sinusitis. Methods: Ocular symptoms were investigated using computed tomography imaging of the facial bones to detect the relationship between the sinus inflammation and the optic nerve. Results: A particular configuration of the optic nerve was detected. Optic nerve course through the inflamed sphenoidal sinus is a condition associated with a greater risk of inflammation. Conclusion: Sinusitis is a rare but treatable cause of optic neuritis. The choice of the correct radiological investigation to be done to set up a proper treatment of the sinus pathological condition is also essential for the resolution of ocular symptoms.
Case Rep Ophthalmol 2017;8:421–424

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Primary Corneoscleral Cyst in a Pediatric Patient

Purpose: Primary corneoscleral cyst is a rare disease occurring in the pediatric age group. We report a case of corneoscleral cyst with visual diminution. Methods: We conducted a case report. Results: A 7-year-old girl presented with corneal opacity in the left eye. Examination revealed a corneoscleral cyst. The corneal part of the cyst involved visual axis. Surgical excision with a scleral graft was performed, leading to an improvement in visual acuity. No recurrences were observed until the last follow-up at 18 months. Conclusions: Corneoscleral cyst should be considered in the differential diagnosis of cystic ocular surface disorders in the pediatric age group.
Case Rep Ophthalmol 2017;8:425–428

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Corneal Neovascularization with Associated Lipid Keratopathy in a Patient with Obstructive Sleep Apnea-Hypopnea Syndrome Using a Continuous Positive Airway Pressure Machine

Objective: To report a case of corneal neovascularization with secondary lipid keratopathy in a patient treated with continuous positive airway pressure (CPAP) for obstructive sleep apnea-hypopnea syndrome (OSAHS). Case Report: A 49-year-old male had been diagnosed with obstructive sleep apnea syndrome 10 years ago and has been treated with the application of a CPAP machine during night sleep ever since. For the past year, the patient had been complaining for ocular irritation and excessive tearing of the left eye on awakening. Slit-lamp biomicroscopy revealed the presence of neovascularization and lipid exudation in the inferior third of the cornea of the left eye. Ocular patching during night sleep resulted in recession of the reported symptoms and shrinkage of the neovascularization, while the area of lipid exudation ceased to enlarge. Conclusion: To the best of our knowledge, this is the first report of corneal neovascularization in a patient using a CPAP machine for OSAHS.
Case Rep Ophthalmol 2017;8:416–420

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Vestibuläre Störungen

http://ift.tt/2vwcZ4B

Zur Verwendung des Begriffs „Radiochirurgie“ in wissenschaftlichen Publikationen

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Gründe für die Bezeichnungen „Radiochirurgie“ und „Gammaknife“

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Papuloerythroderma of Ofuji presenting as a paraneoplastic phenomenon in myelodysplastic syndrome

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Patients’ and doctors’ preferences for disclosing dermatology results

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Australian trainee dermatologists’ opinions on skin of colour education: Does it reflect changing demographics of the population?

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The bacterial species associated with aspirated foreign bodies in children

Inhaled foreign bodies in children are common and may be complicated by secondary airway tract infection. The inhaled foreign body may act as carrier of infectious material and the aim of this study was to explore the bacterial species associated with aspirated foreign bodies in a cohort of children.

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Retinoic acid induction of CD1d expression primes chronic lymphocytic leukemia B cells for killing by CD8+ invariant natural killer T cells

Publication date: Available online 3 August 2017
Source:Clinical Immunology
Author(s): Yasmeen G. Ghnewa, Vincent P. O'Reilly, Elisabeth Vandenberghe, Paul V. Browne, Anthony M. McElligott, Derek G. Doherty
Invariant natural killer T (iNKT) cells are cytotoxic T cells that respond to glycolipid antigens presented by CD1d. Therapeutic activation of iNKT cells with α-galactosylceramide (α-GalCer) can prevent and reverse tumor growth in mice and clinical trials involving α-GalCer-stimulated iNKT cells are ongoing in humans. B cells express CD1d, however, we show that CD1d expression is reduced on B cells from patients with chronic lymphocytic leukemia (CLL). B cells from CLL patients pulsed with α-GalCer failed to stimulate cytolytic degranulation by iNKT cell lines, but could present the more potent glycolipid analogue, 7DW8-5. Retinoic acid receptor-α (RAR-α) agonists induced CD1d expression by CLL B cells, restoring their ability to present α-GalCer to CD8α⁺ iNKT cells, resulting in cytolytic degranulation. Thus, RAR-α agonists can augment the anti-tumor activities of iNKT cells against CLL cells in vitro. Their inclusion in iNKT cell-based therapies may benefit patients with CLL.



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Adjuvant formulations for virus-like particle (VLP) based vaccines

Publication date: Available online 3 August 2017
Source:Clinical Immunology
Author(s): Velasco Cimica, Jose M. Galarza
The development of virus-like particle (VLP) technology has had an enormous impact on modern vaccinology. In order to optimize the efficacy and safety of VLP-based vaccines, adjuvants are included in most vaccine formulations. To date, most licensed VLP-based vaccines utilize the classic aluminum adjuvant compositions. Certain challenging pathogens and weak immune responder subjects may require further optimization of the adjuvant formulation to maximize the magnitude and duration of the protective immunity. Indeed, novel classes of adjuvants such as liposomes, agonists of pathogen recognition receptors, polymeric particles, emulsions, cytokines and bacterial toxins, can be used to optimize the immunostimulatory activity of a VLP-based vaccine.This review describes the current advances in adjuvant technology for VLP-based vaccines directed at viral diseases, and discusses the basic principles for designing adjuvant formulations for enhancing the vaccine immunogenicity.

Graphical abstract

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Maternal T and B cell engraftment in two cases of X-linked severe combined immunodeficiency with IgG1 gammopathy

Publication date: Available online 3 August 2017
Source:Clinical Immunology
Author(s): Tsubasa Okano, Takuro Nishikawa, Eri Watanabe, Takashi Watanabe, Takehiro Takashima, Tzu-Wen Yeh, Motoi Yamashita, Mari Tanaka-Kubota, Satoshi Miyamoto, Noriko Mitsuiki, Masatoshi Takagi, Yoshifumi Kawano, Yoshiki Mochizuki, Kohsuke Imai, Hirokazu Kanegane, Tomohiro Morio
X-linked severe combined immunodeficiency (X-SCID), caused by defects in the common gamma chain, is typically characterized by T and NK cell defects with the presence of B cells. T cell dysfunction and impaired class-switch recombination of B cells mean that patients typically have defects in class-switched immunoglobulins (IgG, IgA, and IgE) with detectable IgM. Here, we describe two patients with X-SCID with IgG1 gammopathy, in whom we identified maternal T and B cell engraftment. Exclusively, maternal B cells were found among the IgD⁻CD27⁺ class-switched memory B cells, whereas the patients' B cells remained naïve. In vitro stimulation with CD40L+IL-21 revealed that peripheral blood cells from both patients produced only IgG1. Class-switched maternal B cells had restricted receptor repertoires with various constant regions and few somatic hypermutations. In conclusion, engrafted maternal B cells underwent class-switch recombination and produced immunoglobulin, causing hypergammaglobulinemia in patients with X-SCID.



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Non-pseudomonal ecthyma gangrenosum caused by methicillin-resistant Staphylococcus aureus (MRSA) in a chronic alcoholic patient

We report for the first time a case of non-pseudomonal ecthyma gangrenosum (EG) caused by methicillin-resistant Staphylococcus aureus in a chronic alcoholic without an associated immunocompromised state or disease. EG is an infection of the skin, commonly associated with Pseudomonas aeruginosa bacteraemia, though other organisms are also known to be causally related. It usually occurs in patients who are terminally ill and the immunocompromised. In our case, chronic alcoholism with suppression of innate immunity may have led to the development of the condition.

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Missed aortic valve endocarditis resulting in complete atrioventricular block and redo mechanical valve replacement

Infective endocarditis is a rare disease associated with high morbidity and mortality. As a result, early diagnosis and prompt antibiotic treatment with or without surgical intervention is crucial in the management of such condition.

We report a case of missed infective endocarditis of the aortic valve. The patient underwent mechanical aortic valve replacement, with the native valve being sent for histopathological examination. On re-admission 16 months later, he presented with syncope, shortness of breathing and complete heart block. On review of the histopathology of native aortic valve, endocarditis was identified which had not been acted on. The patient underwent redo aortic valve replacement for severe aortic regurgitation.

We highlight the importance of following up histopathological results as well as the need for multidisciplinary treatment of endocarditis with a combination of surgical and antibiotic therapy.

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Tailored lamellar keratoplasty: a new technique

We present a new method for treating complicated corneal gaps among patients who have previously undergone radial keratotomy (RK).

After complete healing of a fungal keratitis in a patient who has undergone RK, we removed the oedematous corneal sector and put anterior tailored segment of a corneal button previously used for Descemet stripping endothelial keratoplasty. The patient's best-corrected visual acuity reached to 20/20 6 months later. This technique will compensate for oedematous parts without putting a significant effect on other parts such as purse string sutures.

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Symbiotic lymph node metastasis from breast cancer and melanoma

Description

A 93-year-old woman with a history of stage 4 breast cancer and stage T4a melanoma presented with pain in her left buttock and groin area. Her breast cancer type was invasive lobular carcinoma, 100% oestrogen receptor positive with normal expression of human epidermal growth factor receptor 2. She had osteosclerotic vertebral metastases at the time of diagnosis and was treated medically with an aromatase inhibitor and an osteoclast inhibitor. Her melanoma had been of polypoid, nodular subtype with Breslow's thickness 6.8 mm, no ulceration and 15 mitoses per mm². The maximal diameter was 2 cm, and it was resected surgically with a 0.5 cm margin.

At current presentation, focal relapse of melanoma of the buttock, with metastases to inguinal lymph nodes, was suspected. Local excision with a 0.5 cm margin and lymph node dissection were performed for diagnostic and palliative purposes (figure 1). Histological examination confirmed melanoma relapse located...

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Converting everolimus to mycophenolate mofetil ameliorated prolonged respiratory syncytial virus infection in a child after heart transplantation

In immunocompromised patients, respiratory syncytial virus (RSV) infections are known to be severe and prolonged, and have significant mortality and morbidity. However, little is known about the clinical courses and treatment strategy of RSV infection in heart transplant recipients. Here, we report a 6-year-old female with heart transplantation who had exhibited prolonged respiratory symptoms and shedding of RSV. She had received everolimus as an immunosuppressant. As immunosuppressants could have been responsible for the prolonged activation of RSV, we converted everolimus to mycophenolate mofetil. After the conversion, RSV promptly disappeared, and her symptoms improved. We speculate that converting the immunosuppressant may be effective for prolonged RSV infection due to the different immunosuppressive mechanisms.

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Limbal cyst following blunt trauma in an operated case of congenital cataract: a rare scenario

A 15-year-old male patient with the history of bilateral operated congenital cataract surgery 5 years back now presents with gradual painless diminution of visual acuity in left eye following a blunt trauma with a fist. Ocular examination showed a well-defined slightly bluish-tinged limbal-based conjunctival cyst without any irregularities in the anterior chamber/pupil. Intraocular pressures were 16 mm Hg in the right eye and 10 mm Hg in the left eye; retinal examination showed findings consistent with hypotonic maculopathy. Anterior segment ultrasound biomicroscopy revealed a well-defined limbal cyst with anterior chamber communication. Under local anaesthesia, the cyst was completely excised with the reconstruction of the sclerocorneal defect. At the end of 6 months, the patient is symptom-free with pretraumatic visual acuity.

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Plasma cells with hairy projections and Auer rod-like inclusions in a patient with multiple myeloma

Description

A 38-year-old woman presented to the outpatient clinic with complaints of easy fatiguability and bone pains for 1 month. On investigating, she was found to have plasma cell (PC) neoplasm of IgA-kappa type with 91% PCs in bone marrow and 5% in peripheral blood (PB). Apart from PCs with classical morphology, there were PCs with distinct circumferential hairy cytoplasmic projections (figure 1) and a few others showing bulbous projections (figure 2). Bone marrow showed Auer rod-like inclusions in some of the PCs (figure 3) and rare histiocytes showing ingested Auer-rod like inclusions (figure 4). She was given two-drug combination chemotherapy (thalidomide and dexamethasone) because of financial limitation. During the second cycle of chemotherapy, she developed generalised weakness and shortness of breath. At this point, the PB smear revealed 27% PCs with cells having hairy projections. Flow cytometry revealed these cells to have...

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Bilateral choroidal neovascular membrane in a young patient with Sorsby fundus dystrophy: the value of prompt treatment

A 45-year-old man had developed a choroidal neovascular membrane (CNVM) in his left eye at the age of 38 years and had received six intravitreal ranibizumab injections with resulting visual acuities of 6/60 in the affected eye and 6/4 in the unaffected right eye (Snellen charts). Family history and genetic testing revealed tissue inhibitor of metalloproteinase-3 (TIMP3) gene positive Sorsby fundus dystrophy (SFD). The patient has been under regular follow-up since. At the age of 45 years, he presented with subretinal fluid accumulation in his right eye suggestive of CNVM and received six intravitreal ranibizumab injections, which maintained visual acuity of 6/7.5 in his right eye. Although SFD is a rare condition, it should be suspected and ruled out in young patients presenting with suspicious fundoscopic findings and subretinal fluid on optical coherence tomography. Early intervention can possibly delay macular fibrosis and loss of vision secondary to SFD associated with CNVM.

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Small cell lung cancer presenting as unilateral rhinorrhoea

The metastatic spread of infraclavicular malignancies to the nasal cavity is rare. We describe the case of a 58-year-old man who presented with a 4-month history of right-sided rhinorrhoea, maxillary hypoesthesia, hyposmia and hypogeusia. Clinical examination revealed an irregular mass within the right nasal cavity. Immunohistochemical analyses of biopsies were consistent with small cell carcinoma of indeterminate origin. A positron emission tomography scan demonstrated extensive mediastinal lymphadenopathy with collapse-consolidation of the right lung's middle lobe and no other sites of metastasis. Following discussion at the lung multidisciplinary team meeting, a diagnosis of metastatic small cell lung cancer (SCLC) was made; the patient was staged with N3, M1b disease and palliative chemo-radiotherapy was started. To the best of our knowledge, this report represents the first documented case of a solitary nasal cavity metastasis arising from a SCLC.

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Invasive lobular breast carcinoma metastasising to the rectum

Gastrointestinal (GI) metastasis from a primary breast carcinoma is uncommon, with the rectum being one of the least reported sites in the literature. We report a case of a 79-year-old woman who underwent treatment for an infiltrative lobular carcinoma of the right breast with nodal involvement, and 10 years later developed recurrence in the form of rectal metastasis. Spread to the GI tract is most commonly seen with lobular breast carcinomas. Any patient with a history of breast cancer presenting typically or atypically with abdominal symptoms or altered bowel habit should raise a high index of suspicion for recurrent or metastatic disease.

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Penetrating head trauma injury with an excellent outcome

Description

A 55-year-old man was found at his workplace with a metal drill penetrating the midline of the posterior region of the sagittal suture. His Glasgow Coma Scale (GCS) was initially 9, but his condition deteriorated and required endotracheal intubation. CT of the brain showed that the drill had taken an intracranial course, from the transition of the middle to the posterior third of the superior sagittal sinus, crossing the right parietal and temporal lobes, and ending in contact with the tentorial surface of the cerebellum (figures 1 and 2). He underwent emergency surgery to remove the drill. The operation revealed complete destruction of the superior sagittal sinus. We performed emergent ligation but were unable to reconstruct the superior sagittal sinus because of profuse bleeding. At the follow-up visit 2 months after the surgery, we performed a CT venography, showing absence of flow in...

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Cervical facet joint septic arthritis: a real pain in the neck

A 64-year-old man with a complex medical history and previous cervical arthritis with discectomy presents with a 2-day history of neck and lower back pain and shortness of breath, associated with left-sided muscle weakness. He has a fever with severe sepsis causing acute renal failure. MRI spine shows evidence of left cervical facet joint septic arthritis at C6-T1. He required 6 weeks of intravenous antibiotics and intensive support with ventilation and haemofiltration. After completion of antibiotics, he made a full recovery and regained neurological function before discharge.

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Rare cause of respiratory failure in a young woman: isolated diffuse alveolar haemorrhage requiring extracorporeal membrane oxygenation

A previously healthy 21-year-old young woman presented with worsening dyspnoea and haemoptysis. Imaging was suggestive of widespread pulmonary haemorrhage. There was no other organ system involvement in particular no evidence of renal involvement. Raised antimyeloperoxidase titres allowed diagnosis of isolated diffuse alveolar haemorrhage (DAH) secondary to microscopic polyangiitis (MPA). The patient rapidly deteriorated with worsening respiratory failure despite invasive mechanical ventilation and required extracorporeal membrane oxygenation (ECMO). This maintained the patient long enough to allow aggressive therapy in the form of immunosuppression and plasma exchange. She made a remarkable recovery and is asymptomatic 2 years on. Isolated DAH in the absence of renal disease is an atypical presentation of MPA and can lead to diagnostic uncertainty. A literature review reveals increasing reports of successful use of ECMO in severe DAH due to pulmonary vasculitis. Despite this, the need for systemic anticoagulation in the presence of pre-existing haemorrhage remains a challenging dilemma.

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Disseminated cryptococcal infection in a patient who had kidney transplant: discrepancy between clinical symptoms and microbiological findings

A 29-year-old man complained of a 2-day history of frontal headache and new-onset fever but no other symptoms. Two months prior to admission, he underwent his third kidney transplantation. Clinical and laboratory examinations were unremarkable. Brain MRI showed a meningeal irritation consistent with viral meningitis. A diagnosis of cryptococcal meningitis and fungaemia was made after detection of a remarkably high and visible load of Cryptococcus neoformans in the cerebrospinal fluid.

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Nephrolithisis in a newborn: a rare case and review of literature

Nephrolithiasis, although a common entity in adults, is less common in children and rare in newborns. The evaluation and management strategies of renal stones in neonates are unclear. We report a rare scenario of renal calculus in a newborn aged 3 days presenting with decreased urine output, fever and crying during micturition. Patient was thoroughly investigated and managed conservatively. Further follow-up showed increase in stone size with recurrent urinary tract infections, hence shock wave lithotripsy was performed to successfully break and clear the stone fragments. Patient recovered well and was doing fine until last follow-up.

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Endotoxaemia during left ventricular assist device insertion: relationship between risk factors and outcome

Br J Anaesth (2004) 92 (1): 131–133.

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Original article: Platelet-rich plasma with microneedling in androgenetic alopecia along with dermoscopic pre- and post-treatment evaluation

Summary

Background

Platelet-rich plasma has shown beneficial effects in the treatment of androgenic alopecia with its growth factor properties in accelerating the dermal papilla. Dermoscopy is a noninvasive method that allows the in vivo evaluation of microstructures not visible to the naked eye.

Objective

To ascertain the role of platelet-rich plasma (PRP) with microneedling, and to compare the pre- and post-treatment dermoscopic features in androgenetic alopecia. (AGA).

Method

Patients with mild to moderate AGA, aged 18-45 years with Hamilton-Norwood score 1-5 were included in both study and control group. Dermoscopy was performed using Dermlite II hybrid m; 3Gen dermoscope at 10× magnification in polarized mode, and photographs were taken. Those not responding or those not having any new hair growth to conventional therapy for at least 1 year were included. The study group were given autologous platelet-rich plasma injections with microneedling over a period of 3 months at 3 weekly interval. Baseline and post-treatment photographs were taken.

Result

Hair growth started after the first session. The patients' satisfaction was more than 75% in 18 patients, on patients' subjective hair growth assessment scale. In post-PRP-treated patients of AGA, increase in the number of vellus and total hairs, increased hair shaft diameter, and reduction in yellow dots were appreciated after 3 sessions. Hair pull test was negative after treatment in 14 patients (70%).

Conclusion

This study reinforces the importance of dermoscopy in not only aiding in the diagnosis, but also in evaluation of pre- and post-treatment response of AGA.

http://ift.tt/2vrNl0d

Lymphocytic hidradenitis: A distinctive histopathological finding of annular erythema of Sjögren syndrome

Abstract

Background/Objectives

Lymphocytic hidradenitis is a non-specific histopathological feature observed in many dermatoses such as lupus erythematosus, morphea or scleroderma. When it occurs it is usually accompanied by the other distinctive histological features of those conditions. Isolated lymphocytic hidradenitis is uncommon and its clinical features and associated underlying medical conditions are still undetermined.

Methods

We performed a retrospective review of patients who clinically presented with annular erythema between 2000 and 2016. Altogether, 30 patients with a histopathological presentation of isolated lymphocytic hidradenitis were identified. Their following characteristics were recorded: clinical features, number and localisation of lesions, serology and other associated medical conditions.

Results

Isolated lymphocytic hidradenitis was found most frequently in middle-aged women. Most patients (n = 28, 93%) presented with many annular erythematous patches and plaques with mild pruritus; 22 (73%) had the SS-A antibody and 17 (57%) met the diagnostic criteria of Sjögren syndrome. Among these patients, 11 had primary and six had secondary Sjögren syndrome associated with systemic lupus erythematosus. Altogether 15 (50%) patients tested positive for a high titre of the antinuclear autoantibody. Other underlying diseases identified during the follow-up period include cryoglobulinaemia, angioimmunoblastic T-cell lymphoma, autoimmune hepatitis, hepatitis C infection and toxic thyroid goitre.

Conclusions

Lymphocytic hidradenitis is a microscopic finding associated with annular erythemas of Sjögren syndrome. Systemic survey for sicca symptoms and work up for autoimmune diseases, including antinuclear antibodies, SS-A, SS-B antibodies, cryoglobulin, lymphoma, viral and autoimmune hepatitis should be performed to facilitate the correct diagnosis.

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Effect of voriconazole on risk of nonmelanoma skin cancer after hematopoietic cell transplantation

Voriconazole has previously been associated with increased risk for cutaneous squamous cell carcinoma (SCC) in solid organ transplant recipients. Less is known about the risk in patients after hematopoietic cell transplantation (HCT).

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The effect of secukinumab on moderate-to-severe scalp psoriasis: Results of a 24-week, randomized, double-blind, placebo-controlled phase 3b study

Moderate-to-severe scalp psoriasis has not been evaluated in prospective trials of patients without moderate-to-severe body psoriasis.

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Risk of cutaneous squamous cell carcinoma after treatment of basal cell carcinoma with vismodegib

Vismodegib is a first-in-class agent targeting the hedgehog signaling pathway for treatment of patients with locally advanced basal cell carcinoma (BCC) and metastatic BCC. There have been concerns about the development of squamous cell carcinoma (SCC) in patients treated with this drug.

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Quality of life outcomes of transoral robotic surgery with or without adjuvant therapy for oropharyngeal cancer

Objectives/Hypothesis

To compare quality of life (QOL) of patients who underwent transoral robotic surgery (TORS) alone, with adjuvant radiation therapy (RT), or adjuvant chemoradiation therapy (CRT) in the treatment of oropharyngeal squamous cell cancer (OPSCCA).

Study Design

Prospective cohort study.

Methods

Medical records were reviewed for 111 patients treated for OPSCCA from 2008 to 2015. Patients were administered the Head and Neck Cancer Inventory (HNCI) to evaluate QOL preoperatively, and at 3 weeks, 3 months, 6 months, and 1 year postsurgery. QOL data were compared between 13 patients treated with TORS alone, 31 with adjuvant RT, and 67 with adjuvant CRT by a linear mixed effects model.

Results

Mean follow-up was 35 months. The HNCI response rates at 3 weeks and 3, 6, and 12 months were 80%, 60%, 55%, and 46%, respectively. TORS alone reported significantly higher eating scores than adjuvant RT or CRT at 3 and 6 months, and higher speech scores compared to adjuvant CRT at 3 months and adjuvant RT at 6 months. TORS alone and adjuvant RT reported less social disruption compared to adjuvant CRT at 3 months. Adjuvant CRT had consistently lower overall QOL scores until 6 months. No TORS-alone patient required percutaneous endoscopic gastrostomy, and no study patient required tracheostomy during treatment.

Conclusions

TORS alone maintained higher QOL than adjuvant RT or CRT in eating, social function, speech, and overall QOL postsurgery. QOL and functional metrics were better for 6 months in TORS-alone patients, and at 12 months, the differences were not significant.

Level of Evidence

4 Laryngoscope, 2017

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