Αρχειοθήκη ιστολογίου

Αλέξανδρος Γ. Σφακιανάκης
ΩτοΡινοΛαρυγγολόγος
Αναπαύσεως 5
Άγιος Νικόλαος Κρήτη 72100
2841026182
6032607174

Πέμπτη 7 Σεπτεμβρίου 2017

SAPHO syndrome associated with hidradenitis suppurativa and pyoderma gangrenosum successfully treated with adalimumab and methotrexate: a case report and review of the literature

Abstract

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare inflammatory condition describing the combination of skin, bone, and joint manifestations that has a heterogeneous presentation. We report a case of severe SAPHO syndrome in association with hidradenitis suppurativa and pyoderma gangrenosum in a 27-year-old male. The patient had an initial migratory arthritis affecting the knees, ankles, metacarpophalangeal joints, proximal interphalangeal joints, wrists, shoulder, and lower back, which progressed to a persistent arthritis and swelling at the sternum, shoulders, wrists, hands, feet, and lower back. Radiographic changes were consistent with the diagnosis of SAPHO syndrome. Serum proinflammatory cytokine levels were significantly elevated and improved substantially after 3 months of therapy. Rationale for therapy in this patient was the observation that tumor necrosis alpha antagonists have been successfully used in SAPHO syndrome, and since arthropathy was so prominent in our patient, we elected to use adalimumab combined with methotrexate.



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Teriflunomide-induced psoriasiform changes of fingernails: a new example of paradoxical side effect?



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Evaluation of the relation between lipid coat, transepidermal water loss, and skin pH

Abstract

Objective

The epidermis is an epidermal barrier which accumulates lipid substances and participates in skin moisturizing. An evaluation of the epidermal barrier efficiency can be made, among others, by the measurement of the following values: the lipid coat, the transepidermal water loss (TEWL) index, and pH.

Materials

The study involved 50 Caucasian, healthy women aged 19–35 years (mean 20.56).

Methods

Measurements were made using Courage & Khazaka Multi Probe Adapter MPA 580: Tewameter TM 300, pH-Meter PH 905, Sebumeter SM 815. The areas of measurements included forehead, nose, left cheek, right cheek, chin, and thigh.

Results

In the T-zone, the lipid coat was in the range between 0 and 270 μg/cm2 (mean 128 μg/cm2), TEWL between 1 and 55 g/m2/h (mean 11.1 g/m2/h), and pH 4.0–5.6 (mean 5.39). Lower values of the lipid coat up to 100 μg/cm2 were accompanied by TEWL greater than 30 g/m2/h and less acidic pH of 5.6–9.0. In the U-zone the range of lipid coat was up to 200 μg/cm2 (mean 65.2 μg/cm2), the skin pH remained 4.0–5.6 (mean 5.47), and TEWL was in the range between 1 and 20 g/m2/h (mean 8.7 g/m2/h). Lower values of the lipid coat up to 100 μg/cm2 were accompanied by TEWL between 1 and 20 g/m2/h and less acidic pH of 5.6–9.0. High values of the lipid coat between 180 and 200 μg/cm2 were connected with TEWL of 1–15 g/m2/h. On the skin of the thigh, we observed a very thin lipid coat – 35 μg/cm2 (mean 5.6 μg/cm2), pH (mean 5.37), and TEWL (mean 8.5 g/m2/h) were considered by us to be within regular limits.

Conclusions

In the T-zone, a thinner lipid coat resulted in relatively high TEWL and pH levels changing toward alkaline. In the U-zone, thinner lipid coat was accompanied by lower TEWL and pH changing toward alkaline. We also observed that lower values of lipid coat up to 100 μg/cm2 were associated with higher pH values ranging toward the basic character pH 5.6–9.0).



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Neonatal skin care: what should we do? A four-week follow-up randomized controlled trial at Zagazig University Hospitals

Abstract

Introduction

Taking care of newborn skin is necessary to avoid skin infections. The normal daily exposure to external factors affects the skin negatively. Skin hygiene and proper skin cleansing as well as protection of the infant skin barrier are essential to maintain barrier function and overall infant health.

Aim

The aim of this work is to assess the neonatal skin care effectiveness in promotion of normal skin development and protection of the optimal skin function by avoiding the different neonatal skin disorders which cause skin infection.

Conclusion

In conclusion, proper care and good hygiene of the normal mature neonatal skin are essential to maintain skin barrier function and overall health. This is achieved by optimizing epidermal barrier integrity that includes:

  • bathing and using emollient;
  • preventing and managing infections and skin injury;
  • minimizing transepidermal water loss (TEWL);
  • minimizing heat loss and percutaneous absorption of toxins.

Baby bath products as well as baby wipes are safe to use and do not appear to affect an infant's skin barrier integrity. It is therefore sensible to use cleansers that have been specially designed for baby's skin, which are pH neutral and very mild to avoid irritant dermatitis and allergic dermatitis.



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A photoconverter gel-assisted blue light therapy for the treatment of rosacea



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Triple cancers concurrently detected in a case of antitranscriptional intermediary factor-1γ antibody-positive dermatomyositis



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EGFR T790M mutation testing of non-small cell lung cancer tissue and blood samples artificially spiked with circulating cell-free tumor DNA: results of a round robin trial

Abstract

The European Commision (EC) recently approved osimertinib for the treatment of adult patients with locally advanced or metastatic non-small-cell lung cancer (NSCLC) harboring EGFR T790M mutations. Besides tissue-based testing, blood samples containing cell-free circulating tumor DNA (ctDNA) can be used to interrogate T790M status. Herein, we describe the conditions and results of a round robin trial (RRT) for T790M mutation testing in NSCLC tissue specimens and peripheral blood samples spiked with cell line DNA mimicking tumor-derived ctDNA. The underlying objectives of this two-staged external quality assessment (EQA) approach were (a) to evaluate the accuracy of T790M mutations testing across multiple centers and (b) to investigate if a liquid biopsy-based testing for T790M mutations in spiked blood samples is feasible in routine diagnostic. Based on a successfully completed internal phase I RRT, an open RRT for EGFR T790M mutation testing in tumor tissue and blood samples was initiated. In total, 48 pathology centers participated in the EQA. Of these, 47 (97.9%) centers submitted their analyses within the pre-defined time frame and 44 (tissue), respectively, 40 (plasma) successfully passed the test. The overall success rates in the RRT phase II were 91.7% (tissue) and 83.3% (blood), respectively. Thirty-eight out of 48 participants (79.2%) successfully passed both parts of the RRT. The RRT for blood-based EGFR testing initiated in Germany is, to the best of our knowledge, the first of his kind in Europe. In summary, our results demonstrate that blood-based genotyping for EGFR resistance mutations can be successfully integrated in routine molecular diagnostics complementing the array of molecular methods already available at pathology centers in Germany.



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Knowledge of food protein–induced enterocolitis syndrome among general pediatricians

Food protein–induced enterocolitis syndrome (FPIES) is a potentially severe, non–IgE-mediated food hypersensitivity. Typically, FPIES presents in infancy, either acutely with profuse emesis, dehydration, and lethargy 1 to 4 hours after intermittent ingestions; or insidiously with frequent, watery diarrhea, intermittent emesis, and failure to thrive with chronic ingestions.1 Common eliciting foods include cow's milk and soy proteins; solids (rice, oat, fish, poultry, fish, and shellfish) are also culprits.

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Lymphoproliferative responses to dendritic cell presentation of sensitizing allergens in atopic children with multiple allergies

Peripheral blood mononuclear cells (PBMCs) proliferate inconsistently, rendering current lymphoproliferation assays unreliable in diagnosis.

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Genetically modified products and food allergy

The effect of genetically modified (GM) products on human health is a timely and important topic. This month's CME Review does an excellent job providing background on this technology, outlining potential health concerns as they relate to food allergy, and summarizing evidence on this topic.1 The article addresses 2 issues that practicing physicians face. First is the increasing disconnect between scientific evidence and popularized fixed health care beliefs and concerns. The second is the many questions we face for which evidence is lacking.

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Information for Readers



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The national cost of asthma among school-aged children in the United States

Recent research has quantified the national health care resource use (HCRU) and health care expenditure (HCE) burden associated with adult asthma; however, estimates specific to school-aged children are more than 2 decades old.

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Instructions for Authors



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Editorial Board



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The allergenicity of genetically modified foods from genetically engineered crops

During the past 2 decades, the diagnosis of food allergy has increased in industrialized countries. Immunoglobulin E (IgE) food allergies affect an estimated 4% to 8% of the American population.1,2 The reason for this increase remains unclear, but food allergy has become an increasingly difficult challenge for patients and their physicians to manage. Repeated exposures lead to significant morbidity, life-threatening anaphylaxis, and substantial health care costs. Because proteins are the primary precipitant of many allergic reactions, some members of the public have become concerned that the proteins produced in genetically modified (GM) crops will lead to an increase in allergic reactions in people who consume those crops.

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An unusual cause of fever in a patient with common variable immunodeficiency

The patient is a 22-year-old man with a history of common variable immunodeficiency (CVID) associated with a heterozygous mutation in TNFRSF13B (TACI), granulomatous lymphocytic interstitial lung disease (GLILD), massive splenomegaly, immune thrombocytopenia, and autoimmune hemolytic anemia. He presented with a 5-day history of fever with temperatures up to 38.9°C, malaise, sinus congestion, intermittent headaches, and loose stools. He had no cough, dyspnea, vomiting, abdominal pain, joint pain, or rash.

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Penicillin skin testing

Although approximately 10% of the population carries a label of penicillin allergy, the vast majority of these individuals turn out to be tolerant of penicillins. The rate of positive penicillin skin test reactions in patients with a history of penicillin allergy seems to have been decreasing in the past 2 decades.1,2 For example, Jost et al1 reported a skin test positive rate of 6% from 1993 through 2003 compared with 27% from 1979 through 1992 in consecutive pediatric patients using the same full set of penicillin skin test reagents.

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Author's response

We would like to thank authors for their comments on our article,1 which are both valid and valuable to consider. We agree that the estimated odds ratios are biased away from the null hypothesis given the sparse data. We also agree that the use of the word independently is incorrect in the abstract. The odds ratios reported in the abstract and in table 4 are univariate odds ratios. The term independently associated should be reserved for results from a multivariable analysis in which we have adjusted for the other variables in the model.

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Unusual presentation of combined immunodeficiency in a child with homozygous DOCK8 mutation

Autosomal recessive hyper-IgE syndrome with combined immunodeficiency was first described in patients and linked to a mutation in the gene encoding the dedicator of cytokinesis 8 (DOCK8) in 2009.1,2 The DOCK8 gene is located on chromosome 9 and encodes for a protein that functions as a guanine nucleotide exchange factor, which interacts with the Rho family of GTPases.3 The activated Rho-GTPases are vital to cytoskeletal development, cell growth, migration, and adhesion. DOCK8 plays a critical role in immunologic synapse formation, linking the T-cell receptor to the actin cytoskeleton, thereby leading to T-cell activation and proliferation and T-, B-, and natural killer cell survival.

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Table of Contents



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Predictors of granulomatous lymphocytic interstitial lung disease in common variable immunodeficiency: Methodological issues

We read with great interest the article by Hartono et al1 that was published in the Annals of Allergy, Asthma & Immunology in May 2017. They intended to determine a set of clinical and/or laboratory parameters associated with granulomatous lymphocytic interstitial lung disease (GLILD). Based on univariable analysis, they stated that splenomegaly was independently associated with GLILD (odds ratio [OR] 17.3, 95% confidence interval [CI] 3.9–74.5). Although the study was useful and very interesting, some methodologic issues need to be considered.

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Single recombinant and purified major allergens and peptides

To review the current knowledge regarding recombinant and purified allergens and allergen-derived peptides.

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Sensitization profiles to peanut allergens across the United States

Measurement of IgE antibody to peanut components can aid in the prediction of allergic responses the food.

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Allergic sensitization and objective measures of sleep in urban school-aged children with asthma

Allergic sensitization is associated with increased child asthma morbidity and decreased pulmonary function. Nocturnal symptoms and/or awakenings typically are measured by self-report from diary data, whereas objective assessments of sleep in child asthma studies are lacking.

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Evaluating proteins for potential allergenicity using bioinformatic approaches

Ever since the introduction of a tougher tomato1 and, more recently, the discovery of a "DNA nanosurgery" system based on CRISPR-CAS,2 we are inexorably headed to a new world where genetically modified (GM) animals will consume GM plants, both of which will be labeled GM foods. These in turn will be (futuristically) consumed by GM humans. It is natural for allergists to wonder whether this could increase the incidence of allergy as we strive to primum non nocere and apply bioinformatics approaches to assess this at the bedside.

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Relationship between swallowing function and breathing/phonation

Clarification of the association between the swallowing function and respiratory and phonatory functions.

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Natural course of tonsillectomy pain: A prospective patient cohort study

The aim of this study was to determine the natural course of pain after tonsillectomy.

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Leveraging blood and tissue CD4+ T cell heterogeneity at the single cell level to identify mechanisms of disease in rheumatoid arthritis

Chamith Y Fonseka | Deepak A Rao | Soumya Raychaudhuri

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Novel mechanisms in immune tolerance to allergens during natural allergen exposure and allergen-specific immunotherapy

Willem van de Veen | Oliver F Wirz | Anna Globinska | Mübeccel Akdis

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Lasers and intense pulsed light (IPL) association with cancerous lesions

Abstract

The development and use of light and lasers for medical and cosmetic procedures has increased exponentially over the past decade. This review article focuses on the incidence of reported cases of skin cancer post laser or IPL treatment. The existing evidence base of over 25 years of laser and IPL use to date has not raised any concerns regarding its long-term safety with only a few anecdotal cases of melanoma post treatment over two decades of use; therefore, there is no evidence to suggest that there is a credible cancer risk. Although laser and IPL technology has not been known to cause skin cancer, this does not mean that laser and IPL therapies are without long-term risks. Light therapies and lasers to treat existing lesions and CO2 laser resurfacing can be a preventative measure against BCC and SCC tumour formation by removing photo-damaged keratinocytes and encouraged re-epithelisation from stem cells located deeper in the epidermis. A review of the relevant literature has been performed to address the issue of long-term IPL safety, focussing on DNA damage, oxidative stress induction and the impact of adverse events.



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Immunotherapies Center Stage at EADV

The designated hot topic of this year's European Academy of Dermatology and Venereology (EADV) Congress will have specialists talking about the latest developments in immunotherapies.
Medscape Medical News

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Endoscopic Endo-Nasal Trans-Sphenoidal Excision of Pituitary Tumors: An Institutional Experience

Abstract

To analyze the clinical presentation, management and outcomes of patients with pituitary adenoma treated by Endoscopic Endo-nasal Trans-sphenoidal (EETS) excision. Study was conducted on the basis of medical records of 14 patients who had undergone EETS excision of pituitary adenomas. The data obtained was assessed for demographic and clinical characteristics, radiographic features and post-operative outcomes. Study included 10 males and 4 females. Mean age of patients was 46.43 years (range 16–70 Years). Most common presentation was diminished vision reported in 79% patients. Features of acromegaly encountered in 21% cases. Location of majority of tumors was sellar with supra-sellar extension (71%) followed by sellar (14%), sellar with para-sellar extension (7%) and sellar with supra-sellar and para-sellar extensions (7%). Total tumor resection was achieved in 64% cases and sub-total resection in 36% cases. One of the lesions on biopsy revealed granulomatous pathology. No post-operative complication was seen in 71% patients. Transient Diabetes Insipidus was observed in 21% cases that resolved within 3–4 days. One patient expired due to meningitis and septicemia. One patient had CSF leak that was effectively managed by placing lumbar drain. There was significant improvement in visual symptoms and hormonal levels. Average hospital stay was 13.54 days and mean follow up period was 13 months. EETS approach is safe, minimally invasive and effective surgical technique for resection of pituitary adenomas with low post-operative morbidity, reduced hospital stay and better remission of symptoms.



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Endoscopic Endo-Nasal Trans-Sphenoidal Excision of Pituitary Tumors: An Institutional Experience

Abstract

To analyze the clinical presentation, management and outcomes of patients with pituitary adenoma treated by Endoscopic Endo-nasal Trans-sphenoidal (EETS) excision. Study was conducted on the basis of medical records of 14 patients who had undergone EETS excision of pituitary adenomas. The data obtained was assessed for demographic and clinical characteristics, radiographic features and post-operative outcomes. Study included 10 males and 4 females. Mean age of patients was 46.43 years (range 16–70 Years). Most common presentation was diminished vision reported in 79% patients. Features of acromegaly encountered in 21% cases. Location of majority of tumors was sellar with supra-sellar extension (71%) followed by sellar (14%), sellar with para-sellar extension (7%) and sellar with supra-sellar and para-sellar extensions (7%). Total tumor resection was achieved in 64% cases and sub-total resection in 36% cases. One of the lesions on biopsy revealed granulomatous pathology. No post-operative complication was seen in 71% patients. Transient Diabetes Insipidus was observed in 21% cases that resolved within 3–4 days. One patient expired due to meningitis and septicemia. One patient had CSF leak that was effectively managed by placing lumbar drain. There was significant improvement in visual symptoms and hormonal levels. Average hospital stay was 13.54 days and mean follow up period was 13 months. EETS approach is safe, minimally invasive and effective surgical technique for resection of pituitary adenomas with low post-operative morbidity, reduced hospital stay and better remission of symptoms.



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To Evaluate and Compare the Result of Ossiculoplasty Using Different Types of Graft Materials and Prosthesis in Cases of Ossicular Discontinuity in Chronic Suppurative Otitis Media Cases

Abstract

Chronic suppurative otitis media, with and without cholesteatoma, frequently results in disruption of the ossicular chain. The present study was designed to compare the outcome using autologous ossicle and titanium prosthesis in two groups of suitable patients presenting with chronic suppurative otitis media for middle ear reconstructive surgeries. A prospective study done from July 2012 to December 2013, at Mahatma Gandhi Medical College and Hospital, Sitapura, Jaipur (Rajasthan). The target group included patients with chronic suppurative otitis media admitted and planned for reconstructive middle ear surgery. The study group patients among the target group requiring certain ossicular reconstruction. The patients included were patients with CSOM with or without cholesteatoma in which there was ossicular discontinuity. The patients were divided in two groups A and B. In group A patients autologous incus was used and in group B titanium prosthesis either TORP or PORP was used for ossicular replacement. Out of 340 patients as target group, 88 patients were included in study group. Further 88 patients of study group, 51 patients were included in group A; rest 37 patients were included in group B. In group B patients partial ossicular replacement platinum prosthesis was used in 31 patients while in rest 6 patients a total ossicular replacement prosthesis was used. Out of 88 patients, 79 were primary cases and 9 were revision cases. Pre and post operative air bone gap were noted. Pre operatively both of the groups were identical in relation to air–bone gap. The postoperative air–bone gap was calculated as the difference between postoperative air conduction and preoperative bone conduction. On comparison of preoperative and post-operative hearing results there was improvement in air conduction threshold and air bone gap in both the groups. Post operatively difference in air bone gap closure between group A and group B was found statistically significant (P value = 0.032) suggesting better hearing outcomes after using titanium ossicular replacement prosthesis. On comparing partial or total ossicular replacement prosthesis no significant difference was found for the audiological outcome (P = 0.434). Various prognostic factors were also noted for air bone gap closure. Titanium ossicular replacement prosthesis have better outcome, and no significant difference found in audiological outcome for TORP and PORP.



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Orbital recurrence of iris melanoma 21 years after enucleation

We present the case of a 56-year-old man who developed a neoplasm of epithelioid histology in his anophthalmic left orbit 21 years after he underwent enucleation for a spindle cell iris melanoma. The recurrent tumour was managed by orbital exenteration. Neither further recurrence nor metastasis was diagnosed over a 5-year follow-up period. This case, along with five other similar cases in the literature,1–3 emphasises the importance of long-term follow-up after treatment of iris melanoma.



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Multicenter prevalence of anaphylaxis in clinic-based oral food challenges

Publication date: Available online 7 September 2017
Source:Annals of Allergy, Asthma & Immunology
Author(s): Kwei Akuete, Danielle Guffey, Ryan B. Israelsen, John M. Broyles, Lori Jo Higgins, Todd D. Green, David R. Naimi, Andrew J. MacGinnitie, Girish Vitalpur, Charles G. Minard, Carla M. Davis
BackgroundAlthough previous single-center studies report the rate of anaphylaxis for oral food challenges (OFCs) as 9% to 11%, little is known regarding the epidemiology of clinical OFCs across multiple centers in the United States.ObjectiveTo examine the epidemiology, symptoms, and treatment of clinical low-risk OFCs in the nonresearch setting.MethodsData were obtained from 2008 to 2013 through a physician survey in 5 food allergy centers geographically distributed across the United States. Allergic reaction rates and the association of reaction rates with year, hospital, and demographics were determined using a linear mixed model. Meta-analysis was used to pool the proportion of reactions and anaphylaxis with inverse-variance weights using a random-effects model with exact confidence intervals (CIs).ResultsA total of 6,377 OFCs were performed, and the pooled estimate of anaphylaxis was 2% (95% CI, 1%-3%). The rate of allergic reactions was 14% (95% CI, 13%-16%) and was consistent during the study period (P = .40). Reaction rates ranged from 13% to 33%. Males reacted 16% more frequently than females (95% CI, 4%-37.5%; P = .04). Foods challenged in 2013 varied geographically, with peanut as the most challenged food in the Northeast, Midwest, and West and egg as the most challenged in the South.ConclusionAs the largest national survey of allergic reactions of clinical open OFCs in a nonresearch setting in the United States, this study found that performing clinical nonresearch open low-risk OFCs results in few allergic reactions, with 86% of challenges resulting in no reactions and 98% without anaphylaxis.



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Versorgungsforschung in onkologischen Schwerpunktpraxen in Deutschland

Zusammenfassung

Hintergrund

Ergebnisse aus randomisierten kontrollierten klinischen Studien (RCT) sind wegen notwendiger Ein- und Ausschlusskriterien der teilnehmenden Patienten häufig nicht auf die Routineversorgung übertragbar. Deswegen werden Daten aus der Routineversorgung nichtselektionierter Patienten benötigt, um entscheiden zu können, wie sich Therapieempfehlungen und Therapieleitlinien im Versorgungsalltag umsetzen lassen.

Ziel der Arbeit

Die Autoren beschreiben die Versorgungsforschung aus onkologischen Schwerpunktpraxen (OSP) und vergleichen die Ergebnisqualität zwischen RCT und der Routineversorgung in OSP.

Material und Methoden

Es erfolgt eine Gegenüberstellung der Daten aus RCT mit Versorgungsforschungsdaten aus OSP, die in Fachzeitschriften mit Gutachtersystem publiziert wurden.

Ergebnisse

Die Versorgungsforschung in OSP kommt zu vergleichbaren Ergebnissen in der Routineversorgung onkologischer Patienten wie RCT, wenn ähnliche Patientengruppen verglichen werden.

Diskussion

Bei vergleichbaren Patientencharakteristika sind die Ergebnisse der RCT auf die Routineversorgung übertragbar. Onkologische Patienten können in einer OSP wohnortnah auf dem Niveau einer RCT behandelt werden.



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Anatomic relationship of the first olfactory neuron and trochlea: cadaveric study with surgical implications

Background

Iatrogenic injury to the trochlea is a potential complication of the Draf III or endoscopic modified Lothrop procedure; however, the location of the trochlea from within the sinus cavity has yet to be clearly characterized. We performed the first cadaveric study assessing the position of the trochlea in relation to the first olfactory neuron, a commonly identified landmark during the Draf III procedure.

Methods

Thirteen external dissections of the trochlea were performed on 7 disarticulated cadaveric heads via an extended Lynch type incision. An endoscopic Draf III procedure was then performed on all cadavers. A burr hole was then created at the trochlear fovea. A straight vector between the trochlear attachment and the ipsilateral first olfactory neuron was then created. The vector was then viewed from within the sinuses using a 30-degree endoscope. The endoscope was maneuvered to create an upright midline view centered on the olfactory neurons. An image was subsequently captured and analyzed using ImageJ software to calculate a vector angle. The linear distance between the 2 structures was also recorded.

Results

Relative to the first olfactory neuron, the trochlea was found to be 19.52 ± 4.68 mm away and at an angle of 39.42 ± 8.54 degrees in the anterolateral, superior direction along the frontal bone.

Conclusion

We report the first anatomical dissection characterizing the position of the trochlea relative to the ipsilateral first olfactory neuron. Intraoperative recognition of this relationship during Draf III procedures can prevent potential injury to the trochlea.



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Pituitary and skull-base lesions and the litigious patient

Background

The objective of this research was to evaluate litigation relating to the diagnosis and management of pituitary and ventral skull base lesions and delineate allegations involved in the decision to pursue medicolegal proceedings.

Methods

Publically available federal and court records were accessed via the Westlaw Next database. Jury verdict and settlement reports relevant to pituitary and anterior skull-base lesions were accessed, and litigation was reviewed for alleged injuries, defendant specialty, patient demographics, and other factors raised in proceedings.

Results

Of 75 cases included, 50.7% were resolved in the defendant's favor. The most frequent physician specialties cited as defendants included primary care (20%), neurosurgery (17%), and radiology (16%), while otolaryngologists were defendants in only 5% of cases. Fifty-two (69%) did not involve surgical intervention; the most common allegations in these proceedings were misdiagnosis, permanent injury (19%), requiring additional procedures as a result of misdiagnosis (17%), permanent endocrine dysfunction (14%), and visual sequelae (12%). Among surgical cases, the most common allegations raised included permanent injury (17%), postoperative complications (14%), intraoperative complications (13%), and death (10%). Among cases resolved with payment, there was no statistical difference in payment between surgical cases ($5.7M) and nonsurgical cases ($4.8M).

Conclusion

Misdiagnosis of endocrinopathy, failure to appropriately workup patients presenting with neurologic complaints, and radiologic misdiagnosis play important roles in the pursuit of litigation in nonsurgical cases. Sustaining permanent sequelae including endocrine and visual injury play an important role in surgical cases. Postoperative management appears to play just as important a role in the decision to pursue litigation as intraoperative considerations.



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Impact of ixekizumab on facial psoriasis and related quality of life measures in moderate-to-severe psoriasis patients: 12-week results from 2 phase III trials

Abstract

Background

Facial psoriasis was reported in 17–68% of patients with psoriasis and shown to have a negative impact on patients' personal and health-related quality of life (HRQoL).

Objectives

Explore the association of facial psoriasis with patients' HRQoL and assess the relationship between ixekizumab (IXE) and improvement in facial psoriasis and changes in HRQoL.

Methods

This work reports the combined results of 2 phase III multicenter, randomized, double-blind, placebo-controlled, active-comparator trials in patients with moderate-to-severe psoriasis. Patients received placebo, etanercept (ETN; 50 mg twice weekly), or IXE (80 mg every 4 weeks [Q4W] or every 2 weeks [Q2W]) for up to 12 weeks following an initial 160-mg dose. HRQoL parameters were analyzed based on facial psoriasis status at baseline using analysis of covariance models. Improvement was assessed as percentage of patients with no facial psoriasis.

Results

The combined database included 1133 patients with facial psoriasis and 1437 without. Patients treated with IXE whose facial psoriasis cleared had improved Dermatology Life Quality Index 0,1 responses (P < 0.01) compared with patients with facial psoriasis at Week 12. At Week 12, clearance of facial psoriasis compared with presence of facial psoriasis was independently associated with significantly better improvement in Psoriasis Skin Appearance Bothersomeness scores in the IXE Q2W treatment group (P < 0.01). At Week 12, facial clearance and overall Psoriasis Area Severity Index (PASI) improvement were observed in significant numbers of patients treated with IXE compared with ETN and placebo. Facial psoriasis clearance at Week 12 in patients treated with IXE or ETN was positively associated with PASI75 and PASI90 achievement.

Conclusion

Facial psoriasis had a larger negative impact on HRQoL than no facial psoriasis. Facial psoriasis clearance was associated with improved HRQoL. Significantly more IXE-treated patients had rapid facial clearance vs. ETN and PBO, which led to better clinical outcomes.

This article is protected by copyright. All rights reserved.



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To Evaluate and Compare the Result of Ossiculoplasty Using Different Types of Graft Materials and Prosthesis in Cases of Ossicular Discontinuity in Chronic Suppurative Otitis Media Cases

Abstract

Chronic suppurative otitis media, with and without cholesteatoma, frequently results in disruption of the ossicular chain. The present study was designed to compare the outcome using autologous ossicle and titanium prosthesis in two groups of suitable patients presenting with chronic suppurative otitis media for middle ear reconstructive surgeries. A prospective study done from July 2012 to December 2013, at Mahatma Gandhi Medical College and Hospital, Sitapura, Jaipur (Rajasthan). The target group included patients with chronic suppurative otitis media admitted and planned for reconstructive middle ear surgery. The study group patients among the target group requiring certain ossicular reconstruction. The patients included were patients with CSOM with or without cholesteatoma in which there was ossicular discontinuity. The patients were divided in two groups A and B. In group A patients autologous incus was used and in group B titanium prosthesis either TORP or PORP was used for ossicular replacement. Out of 340 patients as target group, 88 patients were included in study group. Further 88 patients of study group, 51 patients were included in group A; rest 37 patients were included in group B. In group B patients partial ossicular replacement platinum prosthesis was used in 31 patients while in rest 6 patients a total ossicular replacement prosthesis was used. Out of 88 patients, 79 were primary cases and 9 were revision cases. Pre and post operative air bone gap were noted. Pre operatively both of the groups were identical in relation to air–bone gap. The postoperative air–bone gap was calculated as the difference between postoperative air conduction and preoperative bone conduction. On comparison of preoperative and post-operative hearing results there was improvement in air conduction threshold and air bone gap in both the groups. Post operatively difference in air bone gap closure between group A and group B was found statistically significant (P value = 0.032) suggesting better hearing outcomes after using titanium ossicular replacement prosthesis. On comparing partial or total ossicular replacement prosthesis no significant difference was found for the audiological outcome (P = 0.434). Various prognostic factors were also noted for air bone gap closure. Titanium ossicular replacement prosthesis have better outcome, and no significant difference found in audiological outcome for TORP and PORP.



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Dietary Modifications in the Treatment of Laryngopharyngeal Reflux

Laryngopharyngeal reflux (LPR) can manifest as a number of symptoms that can be challenging for patients to tolerate and for physicians to effectively diagnose and treat. These symptoms have been shown to lead to extensive health care costs, with annual costs in the United States suggested to be as high as over $50 billion, a significant component of which is owing to the use of proton pump inhibitors (PPIs). Not only is this a challenging condition to both define and diagnose, but in the absence of a gold standard diagnostic tool it is also challenging to monitor response to therapy. The Reflux Symptom Index (RSI) is a validated 9-item self-administered questionnaire published in 2002 to assess the severity of LPR symptoms; however, its clinical utility has been questioned and responses are of a subjective nature.

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Mediterranean Diet vs Proton Pump Inhibitors for Treatment of Laryngopharyngeal Reflux

This cohort study examines the efficacy of a dietary approach to treating laryngopharyngeal reflux using alkaline water, a plant-based, Mediterranean-style diet, and standard reflux precautions compared with the traditional treatment approach using proton pump inhibition.

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Perioperative Education Program and Unplanned Readmission After Total Laryngectomy

This pilot study assesses whether a perioperative education program can decrease the rate of 30-day unplanned readmission in patients undergoing total laryngectomy.

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Pancreatic Tuberculosis with Vascular Involvement and Peritoneal Dissemination in a Young Man

Pancreatic tuberculosis (TB) is an extremely rare form of extrapulmonary tuberculosis even in endemic areas that masquerades as a mass or inflammation because of lack of typical clinical manifestations and radiologic features and therefore usually misdiagnosed as a pancreatic malignancy or pancreatitis. Here we present a 23-year-old young man with pancreatic tuberculosis mimicking pancreatic head carcinoma A man who suffered from upper abdominal pain and nausea for half a month was admitted to our hospital. Narrow band imaging (NBI) and gastroscopic imaging, together with endoscopic ultrasonography (EUS), revealed a duodenal bulb mucous prominences lesion. Computed tomography (CT) and magnetic resonance imaging (MRI) both suggested a pancreatic mass which resembled a pancreatic head tumor that had a higher risk of malignancy. The patient therefore accepted an exploratory laparotomy and pancreatoduodenectomy, Whipple operation. Biopsies of pancreas, duodenum, lymph nodes, omentum, and adipose tissues were all performed, revealing tuberculosis infection in pancreas, hepatic portal vein infiltration, and peritoneal dissemination. The patient was treated successfully after operation and recovered with standard anti-TB drugs for 6 months. Timely reporting of this rare case can help physicians improve their ability to identify several specific illnesses and diseases that share confusing signs or symptoms clinically and radiographically.

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Appraisal of the AJCC 8th edition pathologic staging modifications for HPV−positive oropharyngeal cancer, a study of the National Cancer Data Base

Publication date: October 2017
Source:Oral Oncology, Volume 73
Author(s): Kevin Y. Zhan, Antoine Eskander, Stephen Y. Kang, Matthew O. Old, Enver Ozer, Amit A. Agrawal, Ricardo L. Carrau, James W. Rocco, Theodoros N. Teknos
BackgroundThe American Joint Commission on Cancer (AJCC) recently created new staging for human papillomavirus associated oropharyngeal cancer (HPV+ OPSCC) for its 8th edition. These proposals have not yet been validated in a national registry.MethodsReview of National Cancer Database (NCDB) for surgically-treated HPV+ OPSCC for years 2010–2014 to validate the new staging system using the Kaplan Meier method to explore survival outcomes.Results3745 cases were analyzed. Median follow-up was 31.3months. Most patients were Caucasian males with tonsillar cancer. Distribution of stage I disease increased from 3.7% to 80.2% in AJCC 8th. pN1 disease shifted from 17.3% to 75.9%. Treatment and distribution of T-stage varied by pathologic nodal (pN) staging. Extranodal extension (ENE) was positive in 41% cases. Four-year overall survival (OS) for AJCC 8th stages I (92%), II (81%), and stage III (62%) showed excellent hazard discrimination (all pairwise p<0.001). Only 4-year OS by pN staging showed significantly different curves when comparing pN2 (79%) with others (pN0 88%; pN1 91%, p=0.01 and <0.001 respectively). Presence of ENE confers a negative effect on overall survival (92% ENE− vs. 85% ENE+, p<0.001).ConclusionThe NCDB shows improved hazard discrimination and outcome prediction in the AJCC 8th edition staging for HPV+ OPSCC. While overall staging had excellent hazard discrimination, this accounted for poorer discrimination between pN0 and pN1. The majority of patients are reclassified as overall stage I. Presence of extranodal extension demonstrated a statistically significant but modest negative effect on overall survival.Condensed abstract (2 sentences)Using NCDB data for validation, the AJCC 8th ed. pathologic staging system offers much improved hazard discrimination and prognostication in HPV oropharyngeal cancer, with the majority of cases reclassified as pStage I. Of note, only pN2 offered hazard discrimination within nodal staging and presence of pathologic extranodal extension has a modest negative effect on survival.



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Effect of Maternal BMI and Type of Infants Feeding on IGF-1 in Infant

Condition:   IGF1 Deficiency
Intervention:   Diagnostic Test: level of IGF-1 in infants
Sponsor:   Assiut University
Not yet recruiting - verified September 2017

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A Clinical Trial of Endoscopic Surgery Followed by Chemotherapy and Proton Radiation for the Treatment of Tumors in the Sinus and Nasal Passages

Conditions:   Paranasal Sinus Cancer;   Nasal Cavity Tumor;   Nasal Cavity Adenocarcinoma
Interventions:   Drug: Cisplatin;   Radiation: Adjuvant Proton Radiotherapy;   Procedure: Endoscopic Resection
Sponsor:   Memorial Sloan Kettering Cancer Center
Recruiting - verified September 2017

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Comparing Modified with Conventional Parotidectomy for Benign Parotid Tumors

Purpose: We conducted a study to compare the functional outcomes and surgical complications of patients with benign parotid tumors treated with conventional parotidectomy and modified parotidectomy. Methods: This study retrospectively analyzed 99 patients who had benign parotid lesions and underwent parotidectomy using either conventional or modified parotidectomy. The operation time, cosmetic outcome, great auricular nerve anesthesia, incidence of Frey syndrome, and secretory function with the two techniques were compared. Results: The mean operation time was shorter and the total complication rate was obviously lower in the modified surgery group (p p p Conclusion: Modified surgical techniques for benign parotid neoplasms significantly reduced the surgery time and improved the surgery outcomes compared with the conventional approach. This adds to the evidence to support the effectiveness of modified parotidectomy in selected patients with benign parotid tumors.
ORL 2017;79:264-273

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Krebs − eine globale Herausforderung

Zusammenfassung

Hintergrund

Mit einer weltweiten steigender Lebenserwartung sowie der Etablierung des westlichen Lebensstils auch in Schwellen- und Entwicklungsländern ist die Zunahme von Krebsneuerkrankungen eine globale Herausforderung für die Gesundheitspolitik. Die Inzidenzen verschiedener Krebsarten unterscheiden sich nach Kontinent und Index für die menschliche Entwicklung der Länder.

Ziel

Das Ziel dieser Arbeit ist es, eine Übersicht über das Auftreten der weltweit häufigsten Krebserkrankungen zu geben.

Methoden

Grundlage für die hier dargestellten Ergebnisse bilden die Daten zum Krebsgeschehen aus 184 Ländern, die im Rahmen der von der International Agency for Research on Cancer (IARC) initiierten Global Cancer Observatory Plattform zusammengetragen wurden. Die Einteilung für den Index für menschliche Entwicklung (HDI) wurde von dem Entwicklungsprogramm der Vereinten Nationen übernommen.

Ergebnisse

Bei Frauen werden Brust- und Lungenkrebs sowie kolorektale Tumoren weltweit am häufigsten diagnostiziert. Brustkrebs stellt über alle Kontinente hinweg die häufigste Krebserkrankung da. In Regionen mit sehr hohem HDI kommen diese drei Krebsentitäten für 50 % aller Krebsneuerkrankungen auf. In Regionen mit niedrigen HDI stellen Brust‑, Zervix- und Eierstockkrebs 52 % aller Krebsneuerkrankung dar. Bei Männern sind Prostata- und Lungenkrebs sowie kolorektale Tumoren die häufigsten drei Krebsarten. In Regionen mit sehr hohem HDI, stellen Prostata‑, Lungenkrebs und kolorektale Tumoren insgesamt 49 %, in Regionen mit niedrigsten HDI stellen Prostata‑, Lungen- und Speiseröhrenkrebs zusammen 29 % aller dortigen Krebsneuerkrankungen dar.

Diskussion

Der sprunghafte Anstieg der Adipositas-Raten, zunehmender Tabakkonsum wie auch der eingeschränkter Zugang zur Krebsfrüherkennung und -behandlung in Niedrigeinkommensländern ist eine Kernherausforderung für eine globale Krebsbekämpfung. Weltweit, auch in vielen Industrieländern, wird die Zahl der Krebsneuerkrankungen in den kommenden beiden Dekaden ansteigen und bedarf der Entwicklung wirksamer Präventionsstrategien und einem engen Monitoring des Krebsgeschehens.



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Fibular insufficiency fracture: an under-reported complication of advanced tibialis posterior dysfunction

We present a case of fibular insufficiency (stress) fracture in a patient with pre-existing asymptomatic advanced tibialis posterior dysfunction. The patient had severe planovalgus (flat foot) deformity but no pain before the injury; hence had never sought any treatment. She was first treated conservatively for the fracture which failed to control the deformity and had ongoing symptoms. She eventually required tibio-talo-calcaneal fusion. To the best of our knowledge, this is the first case report of this condition in the English literature. In patients with severe tibialis posterior dysfunction, weight-bearing axis passes directly from calcaneum to fibula making it vulnerable to fracture. Patients should be made aware of this risk even if they are asymptomatic and encouraged to use orthotic support for protection against insufficiency fractures.



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Rare case of massive bilateral chylothorax

We present a case of a 48-year-old woman who was referred from an outside hospital. There, she had initially underwent hysterectomy and left salpingo-oophorectomy for tubo-ovarian abscess. She later developed a colovaginal fistula and perforation of sigmoid colon and underwent Hartmann's procedure along with drainage of a left subphrenic abscess. Subsequently, she had to be intubated for acute respiratory failure and was transferred to our hospital. At our hospital, she was found to have massive bilateral pleural effusions. Bilateral small-bore chest tubes were inserted that drained milky fluid. Pleural fluid analysis was consistent with bilateral chylothorax. Thereafter, patient's respiratory status improved and she was extubated. The mechanism of chylothorax was thought be either secondary to the multiple abdominal procedures or alternatively as a complication of the right subclavian catheter that was placed at the outside hospital. Her chest tubes were removed eventually, and she had a slow but definite recovery.



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Laparoscopic adrenalectomy as an effective approach to massive bilateral pheochromocytomas

Pheochromocytomas are catecholamine-secreting neoplasms, arising from adrenomedullary chromaffin cells. In type 2 multiple endocrine neoplasia (MEN2) syndrome, pheochromocytomas are usually benign but with predisposition to be bilateral (50%–80% of cases).

The authors present the case of a young patient diagnosed with uncommonly large bilateral cystic pheochromocytomas and simultaneous detection of medullary thyroid carcinoma. Molecular testing confirmed germline RET codon C634 mutation, consistent with MEN2A syndrome. The patient underwent bilateral laparoscopic adrenalectomy plus total thyroidectomy with central lymph node dissection without associated complications. The histopathological study of the surgical specimens revealed bilateral benign pheochromocytomas (Ki67 of 2%) and a medullary carcinoma of the thyroid T1bN0M0; R0, respectively. One year after surgery, the patient was considered free of disease.

This case demonstrates that bilateral laparoscopic adrenalectomy can be a safe and feasible approach for phechromocytomas in MEN2 syndrome, even in lesions with large diameter. However, due to elevated possibility of recurrence, patients should maintain lifelong follow-up.



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Total knee prosthesis infected with Mycobacterium tuberculosis

Mycobacterium tuberculosis (MTB) infection of a prosthetic joint is rarely reported in developed countries.1 Typically, MTB infection involves the hips or knees, and the infection can occur secondary to crushing and degradation of the granuloma during surgery or, less commonly, from distant foci spreading through the blood. In the present case, MTB infection likely resulted from haematogenous spread since multiple hot spots suggestive of MTB infection were noted in other sites. Early diagnosis allows for antitubercular therapy with retention of the prosthesis, while late diagnosis frequently results in removal and reimplantation of the joint. To avoid major surgery, a high index of suspicion is required to diagnose prosthetic joint tuberculosis.



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Bilateral tarsal tunnel syndrome attributed to bilateral fibrous tarsal coalition and symmetrical hypertrophy of the sustentaculum tali

Tarsal tunnel syndrome (TTS) is one of the frequently encountered entrapment neuropathies of the lower limb. Most often the aetiology is considered to be idiopathic. However, it has to be thoroughly investigated to rule out diverse extrinsic and intrinsic causes. The sustentaculum tali (ST) forms the superior part of the distal tarsal tunnel, and any increase in its size may result in irritation of the tibial nerve or its branches, the medial and lateral plantar nerves or decrease the overall volume of the tarsal tunnel culminating to clinical features of TTS. A hypertrophied ST is an extremely rare cause for TTS, and we report perhaps a first case of bilateral TTS in a young adult due to bilateral symmetrical hypertrophied ST which in turn was a result of bilateral fibrous tarsal coalition. Staged bilateral surgical excision of the hypertrophied part of ST and the underlying fibrous tarsal coalition ensured prompt symptomatic relief.



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Nasogastric tube knotting: a rare and potentially overlooked complication among healthcare professionals

Nasogastric tube placement is a common procedure performed in surgical and medical specialities. The occurrence of knot formation is perhaps one of the least well-recognised complications associated with its usage. We present a case of nasogastric tube knotting to remind colleagues of this rare but important complication. A 75-year-old woman with adhesional bowel obstruction was admitted under the general surgery team. A wide bore nasogastric tube was inserted for drainage and decompression. Although placement of the tube was deemed to be successful, there was no drainage of gastric content evident. On removal of the 'non-functioning' tube a simple knot was seen at its proximal end. A further nasogastric tube was placed and the patient's symptoms resolved with conservative management. Nasogastric tube knotting is a rare and often overlooked complication with the potential to cause significant trauma on tube removal if unrecognised.



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Acute fibrinous and organising pneumonia

Acute fibrinous and organising pneumonia (AFOP) is a rare histological pattern of interstitial lung disease. The authors describe a 60-year-old woman admitted to the hospital for sustained fever, presenting with an alveolar opacity on chest X-ray, with the presumed diagnosis of community-acquired pneumonia and the onset of antibiotics. Since serological results suggested that Legionella pneumophila was the infectious agent, she was discharged on levofloxacin. A week later, she was again admitted with fever. CT scan showed opacities with crescentic morphology and a central ground-glass area suggestive of cryptogenic organising pneumonia. Microbiological, serological and autoimmunity tests were negative. She underwent surgical lung biopsy that revealed inflammatory infiltrate, macrophage desquamation, fibroblasts proliferation and fibrin deposition in the alveolar spaces, consistent with AFOP. She started corticotherapy with good response. Disease relapsed after prednisolone discontinuation, 10 months later. Currently, the patient is on prednisolone 5 mg/day without clinical and radiological recurrence.



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Multicenter prevalence of anaphylaxis in clinic-based oral food challenges

Although previous single-center studies report the rate of anaphylaxis for oral food challenges (OFCs) as 9% to 11%, little is known regarding the epidemiology of clinical OFCs across multiple centers in the United States.

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Connecting the Dots

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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 46-year-old woman presented to…

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Spiroplasma eriocheiris induces mouse 3T6-Swiss albino cell apoptosis that associated with the infection mechanism

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Publication date: November 2017
Source:Molecular Immunology, Volume 91
Author(s): Libo Hou, Wei Gu, Huanxi Zhu, Wei Yao, Wen Wang, Qingguo Meng
Spiroplasma eriocheiris is a novel pathogen similar to the Spiroplasma mirum and also had an ability to infect the newborn mice and caused cataract. Our study was designed to study how S. eriocheiris infects mouse 3T6-Swiss albino cells and to elucidate the cellular molecular pathogenesis of Spiroplasma. FCM analysis and MTT analysis clearly shown that S. eriocheiris could induce 3T6 cell apoptosis and cause cell viability decreased seriously. Immunofluorescence experiments and TEM analysis shown that S. eriocheiris can invade 3T6 cells and form typical inclusion bodies and exhibit vacuolization in vitro. S. eriocheiris-oxytetracycline protection assay show that the infective bacteria already were detected at 1h post infection, and sharply increased at 12h after the bacteria infection. To further study the infection mechanism of S. eriocheiris, global mRNA and microRNA (miRNA) expression profiling were analyzed after the cells infected with the bacteria. A total of 619 non-redundant annotated transcripts (183 up-regulated and 436 down-regulated) and 22 miRNAs (8 up-regulated and 14 down-regulated) were differential expression after 6h S. eriocheiris infection compared to control group. Integrated analysis shown that homologous genes from differential expression miRNA targets and the differential expression genes of the mRNA microarray were major focused on two important pathways focal adhesion and MAPK signaling pathway. To validate the results of microarray, eight focal adhesion (β-Catenin, Parvin, Grb2 and ERK) and MAPK signaling pathway (FGFR, Grb2, ERK, MKK3, p38 and JNK) genes and the housekeeping gene GAPDH were assayed by qPCR and Western blot to confirm the results. Eight miRNAs (miR-143-3p, miR-214-5p, miR-322-3p, miR-328-5p, miR-351-5p, miR-466h-5p, miR-503-5p and miR-30c-1-3p) and the housekeeping gene U6 miRNA were assayed by qPCR to confirm the results of microarray. All the results help us better understand the infection mechanism of S. eriocheiris.



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TNFAIP3 levels in lung dendritic cells instruct Th2 or Th17 cell differentiation in eosinophilic or neutrophilic asthma

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Publication date: Available online 6 September 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Heleen Vroman, Ingird M. Bergen, Jennifer .A.C. van Hulst, Memno van Nimwegen, Denise van Uden, Martin J. Schuijs, Saravanan Y. Pillai, Geert van Loo, Hamida Hammad, Bart N. Lambrecht, Rudi W. Hendriks, Mirjam Kool
BackgroundIt is currently unknown, why allergen exposure or environmental triggers in mild to moderate asthma patients results in Th2-mediated eosinophilic inflammation, whereas severe asthma patients often present with Th17-mediated neutrophilic inflammation. The activation state of dendritic cells (DCs) is crucial for both Th2 and Th17-cell differentiation, and is mediated through NF-κB activation. Ablation of TNFAIP3, one of the crucial negative regulators of NF-κB activation in myeloid cells and DCs was shown to control DC activation.ObjectiveIn this study we investigated the precise role of TNFAIP3 in myeloid cells for the development of Th2 and Th17-cell mediated asthma.MethodsWe exposed mice with conditional deletion of the Tnfaip3 gene in either myeloid cells (using the LysM promotor) or specifically in DCs (using the Cd11c promotor) to acute and chronic house dust mite (HDM)-driven asthma models.ResultsWe demonstrated that reduced Tnfaip3 gene expression in DCs in either Tnfaip3CD11c or Tnfaip3LysM mice dose-dependently controlled development of Th17-mediated neutrophilic severe asthma in both acute and chronic HDM-driven models, whereas wildtype mice developed a purely Th2-mediated eosinophilic inflammation. TNFAIP3-deficient DCs induced HDM-specific Th17-cell differentiation, through increased expression of Th17-instructing cytokines, IL-1ß, IL-6 and IL-23, whereas HDM-specific Th2-cell differentiation was hampered by the increased IL-12 and IL-6 production.ConclusionsThese data show that the extent of TNFAIP3 expression in DCs controls Th2/Th17-cell differentiation. This implies that reducing DC activation could be a new pharmacological intervention to treat severe asthma patients that present with a Th17-mediated neutrophilic inflammation.



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Siglec-8 is an activating receptor mediating β2 integrin-dependent function in human eosinophils

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Publication date: Available online 6 September 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Daniela J. Carroll, Jeremy A. O'Sullivan, David B. Nix, Yun Cao, Michael Tiemeyer, Bruce S. Bochner
BackgroundSiglec-8 is a CD33 subfamily cell surface receptor that is selectively expressed on human eosinophils. Following cytokine-priming, Siglec-8 mAb or glycan ligand binding causes eosinophil apoptosis associated with reactive oxygen species (ROS) production. Most CD33-related Siglecs function as inhibitory receptors, but the ability of Siglec-8 to stimulate eosinophil ROS production and apoptosis suggests that Siglec-8 may instead function as an activating receptor.ObjectiveTo determine the role of IL-5 priming and to identify the signaling molecules involved in Siglec-8 function for human eosinophils.MethodsWe used a mAb and/or a multimeric synthetic sulfated sialoglycan ligand recognizing Siglec-8, in combination with integrin blocking antibodies, pharmacological inhibitors, phosphoproteomics and western blot analysis, to define the necessity of various proteins involved in Siglec-8 function for human eosinophils.ResultsCytokine priming was required to elicit the unanticipated finding that Siglec-8 engagement promotes rapid β2-integrin dependent eosinophil adhesion. Also novel was the finding that this adhesion was necessary for subsequent ROS production and apoptosis. Siglec-8-mediated ROS was generated via NADPH oxidase activation, because pretreatment of eosinophils with catalase (an extracellular superoxide scavenger) or NSC23766 (a Rac GTPase inhibitor) completely inhibited Siglec-8 mediated eosinophil apoptosis. Finally, engagement of Siglec-8 on IL-5 primed eosinophils resulted in increased phosphorylation of Akt, p38 and JNK1 that was also β2-integrin dependent; pharmacologic inhibition of these kinases completely prevented Siglec-8-mediated eosinophil apoptosis.ConclusionsThese data demonstrate that Siglec-8 uniquely functions as an activating receptor on IL-5 primed eosinophils via a novel pathway involving regulation of β2-integrin-dependent adhesion, NADPH oxidase and a subset of protein kinases.

Teaser

Siglec-8 can unexpectedly function as an activating receptor, not an inhibitory receptor, because its engagement on IL-5-primed eosinophils promotes β2-integrin-mediated adhesion and activates NADPH oxidase and protein kinases, all of which are necessary for causing human eosinophil apoptosis.


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Intraoperative imaging during minimally invasive transoral robotic surgery using near-infrared light

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Publication date: Available online 6 September 2017
Source:American Journal of Otolaryngology
Author(s): Nicholas Scott-Wittenborn, Ryan S. Jackson
PurposeThe purpose of this study was to determine if the use of the FIREFLY imaging system could be an asset in transoral robotic surgery (TORS) for oropharyngeal squamous cell carcinoma (OPSCC). The system uses indocyanine green dye (ICG), which fluoresces when illuminated by near-infrared light from the Da Vinci robot. The system may improve visualization of tumor margins, highlight important vascular structures, and help identify the location of tumors and unknown primary head and neck cancers.MethodsSix patients with OPSCC were enrolled in the study. Two of these cases were unknown primaries, one was base of tongue, and three were palatine tonsils. Each patient was given two 3ml doses of ICG, one at the beginning of the surgical case and one during resection of the tumor. The oropharynx was then visualized using the near-infrared light of the Da Vinci robot for a minute after injection.ResultsThe FIREFLY system was unable to detect gross tumors, positive margins, unknown primaries, or vascular structures in any of the six subjects in the study. In addition, there were no adverse events or side effects in any of the subjects.ConclusionThe use of the FIREFLY system with indocyanine green fluorescence did not identify tumor boundaries, unknown primary head and neck cancers, or vascular structures in the oropharynx.



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Editorial Board

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Publication date: September–October 2017
Source:American Journal of Otolaryngology, Volume 38, Issue 5





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Table of Contents

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Publication date: September–October 2017
Source:American Journal of Otolaryngology, Volume 38, Issue 5





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Guidelines for Contributing Authors

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Publication date: September–October 2017
Source:American Journal of Otolaryngology, Volume 38, Issue 5





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Commentary on: Treatment of laryngopharyngeal reflux using a sleep positioning device: A prospective cohort study

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Publication date: Available online 6 September 2017
Source:American Journal of Otolaryngology
Author(s): Zhengcai Lou




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A unique complication of self-expandable metal stent placement in malignant duodenal obstruction

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Abstract
Intestinal obstruction is a common complication in patients with advanced gastrointestinal malignancies. In the last two decades, endoscopic placement of duodenal stents has become a mainstay of palliative treatment in patients with unresectable obstructive duodenal pathology. Self-expandable metal stents have been reported to have excellent success rates, besides dramatically improve the patient's quality of life by reinstating the oral feeding ability. Re-intervention rates remain high, commonly as a consequence of tumour ingrowth resulting stent occlusion. We describe a unique case of duodenal stent obstruction secondary to impacted gallstones. To the best of our knowledge, this is the first case described in the literature and should alert clinicians to this unusual complication.

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Giant pulmonary teratoma with huge splenic lymphangiomatosis: a very rare case

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Abstract
Teratomas are tumors composed of tissues derived from more than one germ cell line. They manifested with a great variety of clinical and radiological features. We report a case of a giant left hemithorax teratoma in a female with huge spleen tumor and review the relevant literature. A 38-year-old female with progressively aggravating dyspnea at rest from a mild trauma. Absent breath sounds on the left side. There was splenomegaly. Computed tomography scan revealed a huge mass (20 × 15 × 18 cm), containing elements of heterogeneous density in the left hemithorax. The spleen tumor was occupying most of the spleen without any other abdominal manifestations. The patient underwent left thoracotomy and laparoscopic splenectomy. Histopathological examination revealed a benign mature teratoma and cystic lymphangiomatosis of the spleen. To the best of our knowledge and after reviewing the available literature this is the first case of huge mature pulmonary teratoma with large cystic spleen lymphangiomatosis.

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The fortuitous repair of a common bile duct injury following placement of a percutaneous transhepatic cholangiogram catheter

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Abstract
Common bile duct injuries are associated with a high rate of morbidity and mortality and are discussed frequently in the literature. These injuries may be difficult to diagnose intraoperatively and are often challenging to repair, necessitating referral to hepatobiliary surgery specialists at academic institutions. This case report highlights the management of a completely disrupted common bile duct identified post-operatively using a percutaneous transhepatic cholangiography (PTC) catheter to bridge the gap between the proximal and distal ductal injury prior to operative repair. In addition, the management of this patient's sickle cell crisis post-operatively using red blood cell exchange transfusion is discussed.

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Regional differences of Turkey in risk factors of newborn hearing loss

Publication date: November 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 102
Author(s): Ozlem Konukseven, Sule Kaya, Aydan Genc, Nuray Bayar Muluk, Figen Suren Basar, Gunay Kirkim, Ulku Tuncer, Erkan Karatas, Cigdem Topcu, Hilal Bolat, Ilknur Dincol
ObjectiveThe aim of this study was to discover Turkish regional differences in the risk factors of newborn hearing loss.MethodA multi-centered retrospective design was used. A total of 443 children, registered to the national newborn hearing screening programme, with bilateral hearing loss, from five different regions of Turkey, were evaluated in terms of the types of hearing loss, the degree of hearing loss, the types of risk factors, parental consanguinity, age at diagnosis and age of auditory intervention, respectively.ResultsThere was no significant difference in the prevalence of hearing loss between regions (χ2 = 3.210, P = 0.523). Symmetric Sensorineural Hearing Loss (SSHL) was the most common type of HL in all regions (91.8%). Profound HL was the most common degree of HL in all regions (46.2%). There were statistically significant differences between regions in terms of types of HL (χ2 = 14.151, P = 0.000). As a total, 323 (72.9%) of subjects did not have any risk factors. There were statistically significant differences between regions in terms of the types of risk factors (pre, peri and post-natal) for SSNHL (χ2 = 16.095, P = 0.000). For all regions, the age of diagnosis was convenient with the JCIH criteria. However the age of hearing aid application was prolonged in some regions. There were statistically significant differences between regions in terms of the age of diagnosis (χ2 = 93.570, P = 0.000) and the age of auditory intervention (χ2 = 47.323, P = 0.000). The confounding effects of gender, age of diagnosis, age of hearing aids applications, HL in the family, types of risk factors for HL on SSNHL were detected.ConclusionTo reach the goal of a high quality newborn hearing screening, there is a need to develop an evidence-based standard for follow up guideline. In addition, risk factors should be re-evaluated according to regional differences and all regions should take their own precautions according to their evidence based data.



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Transcriptomic analysis of the tumor microenvironment to guide prognosis and immunotherapies

Abstract

Tumors are highly heterogeneous tissues where malignant cells are surrounded by and interact with a complex tumor microenvironment (TME), notably composed of a wide variety of immune cells, as well as vessels and fibroblasts. As the dialectical influence between tumor cells and their TME is known to be clinically crucial, we need tools that allow us to study the cellular composition of the microenvironment. In this focused research review, we report MCP-counter, a methodology based on transcriptomic markers that assesses the proportion of several immune and stromal cell populations in the TME from transcriptomic data, and we highlight how it can provide a way to decipher the complex mechanisms at play in tumors. In several malignancies, MCP-counter scores have been used to show various prognostic impacts of the TME, which we also show to be linked with the mutational burden of tumors. We also compared established molecular classifications of colorectal cancer and clear-cell renal cell carcinoma with the output of MCP-counter, and show that molecular subgroups have different TME profiles, and that these profiles are consistent within a given subgroup. Finally, we provide insights as to how knowing the TME composition may shape patient care in the near future.



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Drug Repurposing to treat Asthma and Allergic Disorders: Progress and Prospects

Abstract

Allergy and atopic asthma have continued to become more prevalent in modern society despite the advent of new treatments, representing a major global health problem. Common medications such as antihistamines and steroids may have undesirable long-term side effects and lack efficacy in some resistant patients. Biologic medications are increasingly given to treat resistant patients, but they can represent high costs, complex dosing and management, and are not widely available around the world. The field needs new, cheap and convenient treatment options in order to bring better symptom relief to patients. Beyond continued research and development of new drugs, a focus on drug repurposing could alleviate this problem by repositioning effective and safe small molecule drugs from other fields of medicine and applying them toward the treatment of asthma and allergy. Herein, preclinical models, case reports, and clinical trials of drug repurposing efficacy in allergic disease are reviewed. Novel drugs are also proposed for repositioning based on their mechanism of action to treat asthma and allergy. Overall, drug repurposing could become increasingly important as a way of advancing allergy and atopic asthma treatment, filling a need in treatment for patients today.

This article is protected by copyright. All rights reserved.



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Saddle nose deformity and septal perforation in granulomatosis with polyangiitis

Abstract

Background

Patients who have granulomatosis with polyangiitis (GPA, syn. M. Wegener) often develop an external nose deformity which may have devastating psychological effects. Therefore, reconstruction of nasal deformities by rhinoplasty may become necessary to achieve a normal appearance.

Objective of review

The aim of this systematic review was to investigate the efficacy and safety of surgical reconstruction in external nasal deformities and septal perforation in GPA patients.

Search strategy

A systematic literature search with defined search terms was performed for scientific articles archived in the MEDLINE-Database up to June 10th, 2016 (PubMed Advanced MEDLINE Search), describing management of cases or case series in GPA patients with saddle nose deformity and/or septal perforation.

Results

Eleven of 614 publications met the criteria for this analysis including 41 GPA patients undergoing external nasal reconstruction and/ or septal reconstruction with a median follow-up of 2.6 years. Overall, saddle nose reconstruction in GPA patients is safe even if an increased rate of revision surgery has to be expected compared with individuals without GPA undergoing septorhinoplasty. Most implanted grafts were autografts of calvarial bone or costal cartilage. For septal perforation reconstruction, few studies were available. Therefore, based upon the available data for surgical outcomes, it is impossible to make evidence-based recommendations. All included GPA patients had minimal or no local disease at the time of reconstructive surgery. Therefore, the relationship between disease activity and its impact on surgical outcomes remains unanswered. The potential impact of immune-modulating medications on increased complication rates and the impact of prophylactic antibiotics are unknown.

Conclusions

This study systematically reviews the efficacy and safety of surgical reconstruction of external nasal deformities in GPA patients for the first time. Saddle nose reconstruction in GPA patients with minimal or no local disease is a safe procedure despite an increased rate of revision surgery. Further research is required regarding the impact of antibiotic prophylaxis, immune-modulating therapy, long-term outcomes, and functional outcomes measured with subjective and objective parameters.

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Altered oesophageal mechanosensitivity of secondary peristalsis as a pathophysiological marker in patients with globus sensation

Abstract

Objective

Secondary peristalsis is important for clearance of retained food bolus and refluxate from the oesophagus. We aimed to investigate whether patients with globus sensation have altered physiological characteristics of secondary peristalsis.

Design

Prospective case-controlled study.

Setting

Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Hualien, Taiwan.

Participants

17 globus patients and 18 healthy controls.

Main outcome measures

After a baseline recording of primary peristalsis, secondary peristalsis was stimulated with slow and rapid mid-oesophageal injections of air. Distension thresholds and peristaltic activities of secondary peristalsis were analyzed and compared between the patients and healthy controls.

Results

The threshold volume for generating secondary peristalsis during slow air distension did not differ between the patient and control groups (P = 0.55). The threshold volume for generating secondary peristalsis during rapid air distension was significantly greater in patients with globus than healthy controls (7.0 ± 0.9 vs. 5.0 ± 0.3 mL, P = 0.04). Secondary peristalsis was triggered less frequently in globus patients as compared with healthy control after rapid air distension (40% [30−65%] vs. 60% [60−83%], P = 0.001). There was no difference in any of peristaltic parameters for primary and secondary peristalsis between the groups.

Conclusions

Our work identifies functional defects of oesophageal secondary peristalsis in patients with globus sensation and such defects are characterized with defective triggering of secondary peristalsis during rapid air distension. Whether current findings has therapeutic implication in the management of patients with globus sensation warrants further investigation.

This article is protected by copyright. All rights reserved.



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Granulomatous hepatitis in a patient with Crohn's disease and cholestasis

We describe a case of a 23-year-old woman with a history of Crohn's disease (CD), who initially presented with sepsis-like symptoms, subsequently developed severe cholestasis and following extensive inpatient workup was found to have non-caseating granulomas on her liver biopsy. Infectious aetiologies were excluded and the patient was treated with oral corticosteroids, which ameliorated but did not completely reverse the cholestasis. We review the differential diagnosis of hepatic granulomas and discuss the potential difficulties in establishing their exact aetiology in patients with CD.



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Anomalous left anterior descending artery arising from the pulmonary trunk: a rare cause of angina

A 60-year-old man with a medical history of hypertension and dyslipidaemia presented to our rapid access chest pain clinic with a 2-month history of chest pain on exertion. An exercise stress test was arranged, which showed electrical evidence of inducible ischaemia. Subsequently, a coronary angiogram revealed an anomalous left anterior descending artery arising from the main pulmonary artery that received grade 3 collaterals from a large right coronary artery arising from the aorta. The circumflex arises from the right coronary sinus with a retroaortic course and provided collaterals to the anomalous left anterior descending artery. The patient was managed medically with the recommended pharmacological measures for stable angina and responded well with complete resolution of his symptoms, and he is currently under regular follow-up in the cardiology outpatient department.



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Hickam's dictum: Myasthenia Gravis presenting concurrently with Graves' disease

We present two patients with Graves' disease and concurrent myasthenia gravis. The impact of the dual diagnosis on the clinical course and the potential for a delayed diagnosis of myasthenia gravis is discussed. Patient 1, a 28-year-old man was diagnosed with Graves' disease following his second respiratory arrest. His history was strongly suggestive of a second pathology. Patient 2, a 66-year-old Cantonese woman with established Graves' disease presented with thionamide-related neutropaenia. Examination revealed bilateral ptosis and right lateral rectus palsy. Both patients had thyrotoxicosis secondary to Graves' disease with concurrent myasthenia gravis. Although neuromuscular weakness is common in Graves' disease, coexisting myasthenia gravis (MG) is rare and can cause profound morbidity. Ocular signs in both diseases may cause diagnostic confusion although ptosis suggests coexisting MG. In both cases, the thyrotoxicosis delayed the diagnosis of MG.



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Wasp sting induced STEMI with complete coronary artery occlusion: a case of Kounis syndrome

A 45-year-old previously healthy man with minimal coronary artery disease on imaging presented with an acute MI after sustaining a wasp sting following previous non-eventful exposures throughout his life. This is the first case of Kounis syndrome with optical coherence tomography imaging and proven IgE wasp venom hypersensitivity. The Hymenoptera venom is composed of allergenic proteins and vasoactive amines which are responsible for venom toxicity. This patient also has a history of atopy giving a predisposition for developing IgE-mediated allergic reactions. Hymenoptera stings can be severe in atopic individuals and anaphylaxis may ensue. However, it is a rare cause of myocardial infarction (MI) (Kounis syndrome). Multiple wasp stings in the past may have contributed to sensitisation. Kounis syndrome is a rare clinical manifestation which should remain in the minds of physicians, especially with younger patients with no history of ischaemic heart disease or few risk factors.



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Caecal cancer presenting as leg erythema and a lung opacity

Necrotising infection of the lower limb is a rare presentation for colorectal malignancy. We report a case of a perforated caecal adenocarcinoma presenting with right leg erythema, pain and swelling in the presence of a right lower lobe lung opacity. Following initial debridement and washout, CT imaging demonstrated a thickened terminal ileum, caecum and appendix, in keeping with primary malignancy. This fed the right-sided lower limb sepsis tracking down from the medial aspect of the psoas muscle to give rise to the multiloculated collection seen in the adductor compartment. The lung lesion measured 16 mm and was metastatic. The patient was successfully managed with a subtotal colectomy and an end ileostomy. The biopsy confirmed an adenocarcinoma (T4N1M1). We highlight the importance of perforated colonic carcinoma as a leading differential for lower limb abscesses. Suspicions should be raised further if accompanied by rounded opacifications on plain film radiography of the lungs.



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Livedoid eruption in a patient affected by T-{gamma}{delta} large granular lymphocyte leukaemia

Livedo is an ischaemic dermopathy characterised by a reddish-blue to violaceous mottling of the skin with a net-like reticular appearance. Livedo has been described in association with several medical conditions including lymphoproliferative disorders. Here, we describe the case of a 60-year-old woman who was presented with asymptomatic and persistent livedoid eruption on her trunk, lower and upper extremities as manifestation of an indolent form of T- large granular lymphocyte leukaemia. To the best of our knowledge, this is the first report describing the association between livedo reticularis and T- large granular lymphocyte leukaemia. It is plausible that a pathogenetic role of the neoplastic process is based on a cytotoxic antiendothelial activity.



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Bowel perforation presenting three months after suprapubic catheter insertion

An 82-year-old male with multiple comorbidities, including previous laparotomies, had a suprapubic catheter (SPC) inserted under guidance with ultrasound and a flexible cystoscope. Three months following the procedure, having returned for a flexible cystoscopy for ongoing bladder pain syndrome, he became peritonitic postoperatively.

A CT scan and subsequent laparotomy confirmed the SPC to be passing through a section of terminal ileum. The bowel was resected and he recovered well after a long hospital admission. A literature search found this delayed presentation of bowel perforation following SPC insertion to be rare, with only a few other cases reported. In particular, previous abdominal surgery increases the risk of this complication. This case serves as a reminder of the rare but potentially significant risk of SPC insertion and unusually highlights that this may not present immediately.



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Imatinib dramatically alleviates pulmonary tumour thrombotic microangiopathy induced by gastric cancer

Pulmonary tumour thrombotic microangiopathy (PTTM) is a rare complication of cancer, which can be lethal due to progressive pulmonary hypertension (PH). Several case reports have demonstrated that imatinib, a platelet-derived growth factor receptor–tyrosine kinase inhibitor, can improve severe PH in patients with PTTM.

We describe the case of a 56-year-old woman. Her mean pulmonary arterial pressure (mPAP) was 47 mm Hg, and her dyspnoea worsened rapidly over several days. Although pulmonary embolism was not observed on CT, enlargement of the para-aortic lymph nodes was detected. Gastro-oesophageal endoscopy revealed signet-ring cell carcinoma. We diagnosed her as having PTTM based on her clinical course, and started treatment with imatinib. Five days after its administration, her mPAP decreased dramatically. She was discharged and lived without symptoms of PH until her death due to systemic metastasis of carcinoma. In some cases of PTTM, imatinib may be an effective therapeutic option for PH.



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A case of De Garengeot hernia and literature review

Femoral hernia accounts for only 3% of all the hernias and in only 0.5%–5% of the events, the appendix can travel through the femoral hernia which is called De Garengeot hernia, and the incidence of appendicitis in this type of hernia is as low as 0.08%–0.13%. We present a case of a 69-year-old healthy woman who was referred to the emergency department by her general practitioner for CT-proven appendicitis in the femoral canal. On initial assessment, she was found to have a hard, tender lump in her right groin below the inguinal ligament, and open appendectomy and herniorrhaphy were performed. Surgery is the mainstay of treatment of this type of hernia but due to the rarity of this condition, there is no specific guideline as for the surgical procedure. This article demonstrated a case of De Garengeot hernia which was diagnosed preoperatively and managed surgically.



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Varicella complicated by cellulitis and deep vein thrombosis

We report a 16-month-old girl with varicella complicated by cellulitis, invasive Group A Streptococcus (GAS) infection and deep vein thrombosis. She presented with varicella lesions, fever and a painful firm tumefaction on the right lower leg (RLL). Ultrasound showed a local subcutaneous tissue thickening suggestive of cellulitis and antibiotics were initiated. Further swelling of the RLL motivated a second ultrasound that showed an obstructive thrombus for which she was started on enoxaparin. The blood culture confirmed GAS infection leading to directed antibiotherapy. Additional studies showed positive lupus anticoagulant, decreased protein S and antithrombin. She completed a 2-week course of intravenous antibiotics and anticoagulation therapy with clinical and laboratory markers improvement. However, 3 days later, a recrudescence of symptoms occurred and the ultrasound revealed a local abscess. Further amoxicillin treatment resulted on a complete resolution of symptoms. Doppler ultrasound after 1 month showed markedly increased vein patency.



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Ganglioneuroma of the retropharyngeal space in a patient with glottic cancer

We describe the case of a 71-year-old man with a ganglioneuroma of the retropharyngeal space. The patient presented with a submucosal bulge of the left oropharyngeal wall during follow-up examination of a treated vocal cord carcinoma. CT and MRI revealed a non-specific, well-defined retropharyngeal soft tissue lesion. Positron emission tomography-CT did not show relevant metabolic activity, excluding the hypothesis of metastatic nodal disease. Surgical biopsy of the lesion was compatible with ganglioneuroma. Ganglioneuromas are well-differentiated tumours composed of mature sympathetic ganglion cells that account for approximately 1% of spinal and paraspinal tumours. Peripherally, ganglioneuromas are predominantly found in the posterior mediastinum and retroperitoneal space. Only nine ganglioneuromas of the retropharyngeal space have been reported in the English literature since the early 1980s.



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Tamoxifen maculopathy in a male patient

DescriptionCase description

A 28-year-old male patient presented with the history of blurring of vision in both the eyes for the past 5 months. The patient was a diagnosed case of desmoid fibromatosis of the right axilla 8 years back. The tumour was initially debulked followed by four cycles of systemic chemotherapy, after which patient was symptom-free for 3 years. The patient noticed an increment in the tumour size, for which he consulted another oncologist. A repeat biopsy was done, which proved the same findings. The patient was started on a combination of tamoxifen 80 mg/day and celecoxib 200 mg/day. He had received tamoxifen at a cumulative dose of 116 g over 4 years. Examination in our clinic revealed visual acuity of 20/20 in both eyes with unremarkable anterior chamber findings. Intraocular pressure was 14 and 16 mm Hg in right and left eye, respectively. Fundus showed bilateral crystalline refractile deposits in the macula, rest of the retinal and optic disc...



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Langerhans cell histiocytosis in an adult female presenting with widespread confluent crusted papules and review of adult cases confined to skin



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