Αρχειοθήκη ιστολογίου

Πέμπτη 29 Ιουνίου 2017

Learning organization modelling patterns



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Learning organisation review : a 'good' theory perspective



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Incarcerated congenital transmesenteric hernia in an adult: a case report

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Abstract
Transmesenteric hernia is a rare cause of bowel obstruction and strangulation. It can be due to iatrogenic injury, trauma, inflammatory and less likely congenital. We present a case of true congenital transmesenteric hernia in a 26-year-old male with no previous surgical history or trauma. The patient presented acutely with no prior symptoms. Investigations provided limited information towards the diagnosis of the hernia, but suggestive of mid-gut volvulus. Emergency laparotomy was performed to find >75% of small bowel herniating through a proximal jejunal mesenteric defect leading to incarceration and early strangulation. No resection was required and patient recovered well. This case presents yet another rare pathology and highlights the importance of keeping a low threshold for operative intervention in uncertain cases.

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Traumatic diaphragmatic rupture with underlying lung laceration and tension pneumoperitoneum

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Abstract
Tension pneumoperitoneum is commonly caused by gastrointestinal perforation and pulmonary causes are extremely rare. We present a case of a 47-year-old male post motor vehicle accident with a suspected left-sided haemopneumothorax on initial chest x-ray. CT of the chest post chest tube insertion showed a left-sided diaphragmatic rupture and an extensive diaphragmatic hernia. While en-route to the operating theatre, the intubated patient developed tension pneumoperitoneum with positive pressure ventilation and required immediate surgical intervention and repair. A review of the literature around tension pneumoperitoneum and diaphragmatic hernia in trauma is discussed.

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Hybrid approach to the management of infective endocarditis complicated by coronary artery embolism: a case report

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Abstract
Septic coronary embolization in a patient with endocarditis is a rare and can be a devastating complication. The management of this clinical problem in the current era may be best served with a multi-modality approach. We present an interesting case of a patient with septic coronary embolization managed with the combined use of aspiration thrombectomy followed by surgical management of the infected valve.

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Severe colloid milium presenting as papillomatosis cutis associated with vitiligo



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Acne keloidalis nuchae: risk factors and associated disorders – a retrospective study

Abstract

Background

Acne keloidalis nuchae (AKN) is a chronic scarring folliculitis which usually occurs in young adult males of African descent. Studies have suggested that AKN may be associated with other dermatologic conditions and even general medical disorders. The aim of this study was to identify cutaneous and extracutaneous associated disorders and to examine risk factors in our population for developing AKN.

Methods

The study was a retrospective, descriptive, and analytical study conducted at the Dermatology Outpatient department of the University Hospital of the West Indies. Data were obtained from the medical records of patients diagnosed over a 15-year period (2000–2014).

Results

There were 1031 new patients during the study period. Of these, 43 (4.2%) had AKN. The male to female ratio for AKN was approximately 7:1. Pseudofolliculitis barbae was associated with keloidal plaques on the scalp (OR = 6.22, P = 0.036). Also, when the duration of AKN was divided into two groups (0–5 years and greater than 5 years), there was an association between keloidal plaques and duration of greater than 5 years (OR = 7.5, P = 0.032). However, when the odds ratio was adjusted, only the duration of AKN remained significantly associated with keloidal plaques (OR = 7.08, P = 0.047). Chronic scalp folliculitis (P = 0.001) and the presence of any component disease of the metabolic syndrome (OR = 14, P = 0.008) and specifically hypertension (OR = 6.75, P = 0.036) were significantly associated with the extension of the lesions beyond the nape and occipital scalp.

Conclusions

Pseudofolliculitis barbae, chronic scalp folliculitis, and aspects of the metabolic syndrome may be associated with acne keloidalis nuchae.



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Impact of age and treatment group in childhood high hyperdiploid low risk B-cell acute lymphoblastic leukemia (ALL): results of the CLG-EORTC 58951 study



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Impact of somatic mutations on the outcome of children and adolescents with therapy-related myelodysplastic syndrome



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Endogenous endophthalmitis and osteomyelitis associated with interleukin 17 inhibitor treatment for psoriasis in a patient with diabetes

A 64-year-old man with type 2 diabetes mellitus and plaque psoriasis presented to the emergency room with 3 days of progressive right eye pain and decreased vision. After extensive workup and multidisciplinary team effort, the patient was diagnosed with and treated for unilateral endogenous methicillin-sensitive Staphylococcus aureus endophthalmitis, bacteraemia and osteomyelitis of the foot. The patient had been started on the interleukin 17 (IL-17) inhibitor secukinumab for his treatment-resistant plaque psoriasis 4 weeks prior to presentation. After treatment, his final vision was light perception and the foot infection resolved without sequelae. To our knowledge, this is the first reported case of both endogenous endophthalmitis and osteomyelitis associated with an IL-17 inhibitor.



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An elusive ciliopathy: Joubert syndrome

The police brought a 65-year-old female patient to the EADU after being found 'roaming the streets' in an apparent state of confusion. This was her third admission under the same circumstances during the last 3 years. Neurological examination revealed (1) cognitive impairment, (2) oculomotor apraxia, (3) abnormal cancellation of vestibular ocular reflex, (4) mild ataxia and (5) mild hypotonia. Renal function was abnormal and liver function was normal. No retinal disturbance was found. The head CT on admission was normal for stroke and the lumbar puncture was negative for encephalitis. Her brain MRI showed 'molar tooth sign', suggestive of Joubert syndrome, which was confirmed by genetic testing showing anomalous NPHP1 gene



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Successful management of primary atrophic rhinitis by turbinate reconstruction using autologous costal cartilage

Initial management of primary atrophic rhinitis is conservative, with nasal ointments, saline irrigation, and antibiotics prescribed to relieve symptoms. However, in cases that show no improvement, a surgical approach is considered. Recently, many studies have reported successful surgical outcomes using various nasal implants. However, no study has reported implantation of autologous costal cartilage in PAR patients. We report here the case of a 63-year-old woman diagnosed with PAR that was intractable to medical therapy.

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Letter to the editor

This is in reference to a very well-written article titled 'Surgical timing for facial paralysis after temporal bone trauma' by Xu P et al. [1]. The paper is well-written and the authors have conducted a good study. However, there are certain points of concern regarding the methodology which I would like to highlight through your esteemed journal.

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Successful management of primary atrophic rhinitis by turbinate reconstruction using autologous costal cartilage

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Publication date: Available online 29 June 2017
Source:Auris Nasus Larynx
Author(s): Marn Joon Park, Yong Ju Jang
Initial management of primary atrophic rhinitis is conservative, with nasal ointments, saline irrigation, and antibiotics prescribed to relieve symptoms. However, in cases that show no improvement, a surgical approach is considered. Recently, many studies have reported successful surgical outcomes using various nasal implants. However, no study has reported implantation of autologous costal cartilage in PAR patients. We report here the case of a 63-year-old woman diagnosed with PAR that was intractable to medical therapy. Under general anesthesia, bilateral inferior turbinate reconstruction with autologous costal cartilage was successfully performed without any complications. One month after surgery, her symptoms improved dramatically. At the 2-year follow-up, her Sinonasal Outcome Test 25 (SNOT-25) score was 6, down from an initial score of 108. Her OMU CT showed improved sinonasal mucosal thickness and disappearance of thick mucosal secretion compared with preoperative CT image. Although this is a single case experience, it is suggested that turbinate reconstruction using autologous costal cartilage can serve as promising surgical modality for management of atrophic rhinitis.



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Sleep quality and communication aspects in children

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Publication date: September 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 100
Author(s): Camila de Castro Corrêa, Maria Renata José, Eduardo Carvalho Andrade, Mariza Ribeiro Feniman, Ana Paula Fukushiro, Giédre Berretin-Felix, Luciana Paula Maximino
ObjectivesTo correlate quality of life of children in terms of sleep, with their oral language skills, auditory processing and orofacial myofunctional aspects.MethodsNineteen children (12 males and seven females, in the mean age 9.26) undergoing otorhinolaryngological and speech evaluations participated in this study. The OSA-18 questionnaire was applied, followed by verbal and nonverbal sequential memory tests, dichotic digit test, nonverbal dichotic test and Sustained Auditory Attention Ability Test, related to auditory processing. The Phonological Awareness Profile test, Rapid Automatized Naming and Phonological Working Memory were used for assessment of the phonological processing. Language was assessed by the ABFW Child Language Test, analyzing the phonological and lexical levels. Orofacial myofunctional aspects were evaluated through the MBGR Protocol. Statistical tests used: the Mann-Whitney Test, Fisher's exact test and Spearman Correlation.ResultsRelating the performance of children in all evaluations to the results obtained in the OSA-18, there was a statistically significant correlation in the phonological working memory for backward digits (p = 0.04); as well as in the breathing item (p = 0.03), posture of the mandible (p = 0.03) and mobility of lips (p = 0.04).ConclusionA correlation was seen between the sleep quality of life and the skills related to the phonological processing, specifically in the phonological working memory in backward digits, and related to orofacial myofunctional aspects.



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Otoplasty for prominent ears – demographics and surgical timing in different populations

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Publication date: September 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 100
Author(s): Lauri Kajosaari, Juhani Pennanen, Tuomas Klockars
IntroductionProminent ears are seen in approximately 5% of the population. This benign condition can be treated surgically to reduce or prevent psychological and social problems, most commonly in children before the start of school. Our aim was to examine the demographic characteristics of patients undergoing prominent ear surgery in Finland, and compare findings with international data.MethodsA retrospective study of all the patients treated for prominent ears in our academic tertiary care referral center during 2007-2011 was performed to gather demographic details of operated patients.A systematic review of published series of prominent ear surgery after the year 2000 was performed to gather demographic details for international comparison.ResultsA total of 180 patients were operated in our institution for prominent ears, most of the cases (78.9%) were bilateral. Age at operation ranged between 3 and 36 years, with mean of 9.2 y and median of 7 y. The most common reason for seeking operative treatment was aesthetic complaint, followed by bullying.Review process gathered 20 publications, describing a total of 4433 patients who had been operated for prominent ears. There was wide variation the mean age at operation, ranging 7-38 y while the mean overall was 15.0y. Gender distribution of patient samples was also very variable, with percentage of females ranging from 38 to 71% (overall 52%). There was also considerable variation in the tendency to perform unilateral operations: from 0% up to 21% of the reported population. There were no statistically significant correlations linking these demographic variables, though there was a trend that females are more likely to have this operation performed at an older age.ConclusionsThe treatment culture of prominent ear surgery varies substantially in international comparison. The age at which this operation is performed showed most variation. Omitting some of the basic demographic variables while reporting the results of surgery was common in the reviewed publications.



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Adenotonsillectomy outcomes in children with sleep apnea and narcolepsy

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Publication date: September 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 100
Author(s): Sneh Biyani, Tina D. Cunningham, Cristina M. Baldassari
ObjectiveTo identify improvements in daytime sleepiness following adenotonsillectomy in children with non-severe obstructive sleep apnea and narcolepsy.Study designCase series with chart review over 15 years.SettingTertiary Children's Hospital.Subjects and methodsChildren between 6 and 17 years of age with narcolepsy that underwent adenotonsillectomy for non-severe obstructive sleep apnea (OSA) were included. Narcolepsy was diagnosed based on clinical assessment and the Multiple Sleep Latency Test (MSLT) results. A standardized instrument, the pediatric Epworth Sleepiness Scale (ESS), was used to assess daytime sleepiness before and after adenotonsillectomy.ResultsNine children with a mean age of 12.1 years were included. The majority of the subjects (78%, n = 7) were African American and six children (66.7%) were obese. Four children (44%) were treated with wake promoting agents during the study. The mean preoperative apnea hypopnea index on polysomnography was 4.89 (SD 1.86), while the mean sleep latency on MSLT was 6.32 min (SD 3.14). The mean preoperative ESS was 16.10 and the postoperative ESS was 10.80 (SD 3.96). There was significant improvement (p = 0.02) in the ESS following adenotonsillectomy with seven children (78%) reporting diminished daytime sleepiness.ConclusionsChildren with non-severe OSA and narcolepsy experience significant improvement in daytime sleepiness following adenotonsillectomy. Future studies are needed to determine the incidence and clinical significance of non-severe OSA in children with narcolepsy.



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Heart Failure in Epidermolysis Bullosa

M.T. Villarroel Salcedo
Actas Dermosifiliogr.2017;108:498

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Mycosis Fungoides in Pediatric Patients: A Diagnostic Challenge

Rosa Izu Belloso
Actas Dermosifiliogr.2017;108:499

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Psoriasis and Psychiatric Disorders: The Next Frontier

J.M. Carrascosa, F. Ballesca
Actas Dermosifiliogr.2017;108:502-5

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Hair-follicle Transplant Into Chronic Ulcers: A New Graft Concept

M.L. Martínez Martínez, E. Escario Travesedo, F. Jiménez Acosta
Actas Dermosifiliogr.2017;108:524-31

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Eosinophils as a Warning Sign

E. Rodríguez-Díaz
Actas Dermosifiliogr.2017;108:499-500

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Antimicrobial Photodynamic Therapy: An Unexplored New Field

J. Romaní
Actas Dermosifiliogr.2017;108:500

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Considerations on Subgroup Analyses in Clinical Trials

P. Coto-Segura
Actas Dermosifiliogr.2017;108:501

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Psoriasis and Nonalcoholic Fatty Liver Disease

J.M. Carrascosa, C. Bonanad, E. Dauden, R. Botella, A. Olveira-Martín
Actas Dermosifiliogr.2017;108:506-14

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Angiogenesis in Dermatology – Insights of Molecular Mechanisms and Latest Developments

N.A. Richarz, A. Boada, J.M. Carrascosa
Actas Dermosifiliogr.2017;108:515-23

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Follicular Unit Extraction for Hair Transplantation: An Update

F. Jiménez-Acosta, I. Ponce-Rodríguez
Actas Dermosifiliogr.2017;108:532-7

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Causes and Cures of Skin Diseases in the Work of Hildegard of Bingen

J. Romaní, M. Romaní
Actas Dermosifiliogr.2017;108:538-43

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Cardiomyopathy in Patients With Hereditary Bullous Epidermolysis

A. Batalla, A. Vicente, J. Bartrons, F. Prada, C. Fortuny, M.A. González-Enseñat
Actas Dermosifiliogr.2017;108:544-9

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Comparison of ixekizumab with etanercept or placebo in moderate-to-severe psoriasis: Subgroup analysis of Latin American patients in the phase 3 randomized UNCOVER-3 study

F. Valenzuela, C. de la Cruz Fernandez, R.L. Galimberti, S. Gürbüz, M. McKean-Matthews, L. Goncalves, R. Romiti
Actas Dermosifiliogr.2017;108:550-63

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Mycosis Fungoides: Experience in a Pediatric Hospital

A.B. Cervini, A.N. Torres-Huamani, C. Sanchez-La-Rosa, L. Galluzzo, V. Solernou, J. Digiorge, P. Rubio
Actas Dermosifiliogr.2017;108:564-70

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Pediatric Allergic Contact Dermatitis: Clinical and Epidemiological Study in a Tertiary Hospital

J.M. Ortiz Salvador, A. Esteve Martínez, D. Subiabre Ferrer, A.M. Victoria Martínez, J. de la Cuadra Oyanguren, V. Zaragoza Ninet
Actas Dermosifiliogr.2017;108:571-8

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Eosinophilic Dermatosis of Hematologic Malignancy

S. Lucas-Truyols, B. Rodrigo-Nicolás, C. Lloret-Ruiz, E. Quecedo-Estébanez
Actas Dermosifiliogr.2017;108:e39-44

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Photodynamic Therapy With Methylene Blue for Skin Ulcers Infected With Pseudomonas aeruginosa and Fusarium spp.

C. Aspiroz, M. Sevil, C. Toyas, Y. Gilaberte
Actas Dermosifiliogr.2017;108:e45-8

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Multiple Papules on Both Hands

A. Panés-Rodríguez, A. Jaka-Moreno, I. Arias-Camisón Montero, A. Tuneu-Valls
Actas Dermosifiliogr.2017;108:579-80

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Nodule on the External Ear

S. Heras-González, L. Aspe-Unanue, R. González-Pérez
Actas Dermosifiliogr.2017;108:581-2

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RF-Itraconazole Pulse Therapy for Seborrheic Dermatitis: A Treatment Approach to Consider

V. Fatsini-Blanch, M.I. Martínez-González, S. Heras-González, A. de Quintana-Sancho
Actas Dermosifiliogr.2017;108:583-4

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Rheopheresis in treatment of idiopathic sensorineural sudden hearing loss

Only few therapeutic options exist for patients with refractory sudden idiopathic sensorineural hearing loss (SISHL). Little is known about the efficacy of second-line therapies. Rheopheresis seems to be an ef...

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Intrinsic and Extrinsic Causes of Malignancies in Primary Immunodeficiency Disorders

Publication date: Available online 29 June 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Fabian Hauck, Rebecca Voss, Christian Urban, Markus G. Seidel
Malignancies occur with a higher incidence rate and manifest earlier in life in patients with primary immunodeficiency disorders (PID) than in the general population. However, no universal mechanism of malignancy predisposition in PID can been determined. Despite the strong support for the physiological role of tumor immunosurveillance and increasing success of strategies in immunological tumor therapy, which include checkpoint inhibition, monoclonal antibodies, and engineered T cell antigen receptors, the incidence and pattern of malignancies in PID do not reflect an increased tumor immune escape per se. In contrast, malignancies appear to be restricted to either i) tissue types bearing the same molecular defect that underlies the PID such as syndromes of DNA repair deficiency or immune cell-specific maturation or functional defects that suggest a cell-intrinsic oncogenic basis, or ii) other tissues when they are infected by transforming viruses or chronically inflamed, pointing towards extrinsic causes for transformation that are potentially facilitated by but not predominantly owed to a lack of immunosurveillance. Based on recent studies of preexisting conditions in malignancy patients and on malignancies in large PID cohorts, we conclude that a large part of tumor predisposition in PID is derived from the same molecular defect as the immunodeficiency itself. The presented concept elucidates diverse pathomechanisms and risks of malignancies in PID in the light of current tumor immune therapies.



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Antibiotics Improve Short-Term Outcomes for Simple Abscesses

Despite current recommendations to treat simple abscesses only with incision and drainage, adding antibiotics increased cure rate and reduced recurrence rate for S aureus infections.
Medscape Medical News

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Stay Safe This Fourth of July

The Fourth of July is almost here, and many people are getting ready to revel in the red, white and blue. The holiday is often filled with barbecues, firework displays and patriotic pride, but, for some families, the celebration ends at the hospital. "Fourth of July is a time to celebrate, and it's important to take time to do so, but it is important to keep safety in mind so you can avoid a trip to the Emergency Department," says Susan Peterson, M.D., associate medical director for patient safety and quality in the Department of Emergency Medicine.



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Mechanisms governing inflammasome activation, assembly and pyroptosis induction

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Abstract
Inflammasomes are multimeric protein complexes that regulate inflammatory responses and pyroptotic cell death to exert host defense against microbes. Intracellular pattern-recognition receptors such as nucleotide-binding domain and leucine-rich repeat receptors (NLRs) and absent in melanoma 2 like receptors (ALRs) assemble the inflammasome complexes in response to pathogens and danger or altered-self signals in the cell. Inflammasome sensors, in association with an adaptor protein—apoptosis-associated speck-like protein containing a caspase-activation and -recruitment domain (ASC)—activate inflammatory caspase-1 to enable the release of inflammatory cytokines and induce cell death, conferring host defense against pathogens. Beyond infectious diseases, the importance of inflammasomes is implicated in a variety of clinical conditions such as auto-inflammatory diseases, neuro-degeneration and metabolic disorders and the development of cancers. Understanding inflammasome activation and its molecular regulation can unveil therapeutic targets for controlling inflammasome-mediated disorders. In this review, we describe recent advances in inflammasome biology and discuss its activation, structural insights into inflammasome assembly and mechanisms for the execution of pyroptosis.

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In This Issue

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Integrity of immunoglobulin variable regions is supported by GANP during AID-induced somatic hypermutation in germinal center B cells

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Abstract
Immunoglobulin affinity maturation depends on somatic hypermutation (SHM) in immunoglobulin variable (IgV) regions initiated by activation-induced cytidine deaminase (AID). AID induces transition mutations by C→U deamination on both strands, causing C:G→T:A. Error-prone repairs of U by base excision and mismatch repairs (MMRs) create transversion mutations at C/G and mutations at A/T sites. In Neuberger's model, it remained to be clarified how transition/transversion repair is regulated. We investigate the role of AID-interacting GANP (germinal center-associated nuclear protein) in the IgV SHM profile. GANP enhances transition mutation of the non-transcribed strand G and reduces mutation at A, restricted to GYW of the AID hotspot motif. It reduces DNA polymerase η hotspot mutations associated with MMRs followed by uracil-DNA glycosylase. Mutation comparison between IgV complementary and framework regions (FWRs) by Bayesian statistical estimation demonstrates that GANP supports the preservation of IgV FWR genomic sequences. GANP works to maintain antibody structure by reducing drastic changes in the IgV FWR in affinity maturation.

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Outstanding Merit Award for 2016

The article 'Guanosine and its modified derivatives are endogenous ligands for TLR7' (doi: 10.1093/intimm/dxv062), by Takuma Shibata et al., has been given the Outstanding Merit Award for 2016; that is, it has been selected by the Editor-in-Chief as the best paper published last year in the journal. The full text of the article is available for free online (http://ift.tt/2tpLkkG) but it is summarized here.

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Subscriptions

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Activation of group 2 innate lymphoid cells exacerbates and confers corticosteroid resistance to mouse nasal type 2 inflammation

Abstract
Both Th2 cells and group 2 innate lymphoid cells (ILC2s) contribute to allergic diseases. However, their exact role and relationship in nasal allergic disorders are unclear. In this study, we investigated the cooperation of Th2 cells and ILC2s in a mouse model of nasal allergic disorder. To differentially activate Th2 cells and/or ILC2s in nasal mucosa, mice were intra-nasally administered ovalbumin (OVA) antigen, papain, an ILC2-activator, or both for 2 weeks. Epithelial thickness and number of eosinophils in the nasal mucosa were evaluated at 24 h after the final challenge. Intra-nasal administration of OVA and papain preferentially activated Th2 cells and ILC2s, respectively, in the nose. Both OVA and papain increased the nasal epithelial thickness and number of eosinophils, and their coadministration significantly enhanced the symptoms. Although T-/B-cell-deficient mice showed severely decreased nasal symptoms induced by OVA or OVA-plus-papain, the mice still showed slight papain-induced nasal symptoms. In ILC2-deficient mice, OVA-plus-papain-induced nasal symptoms were suppressed to the same level as OVA-alone. Similarly, IL-33- and ST2-deficient mice showed decreased OVA-plus-papain-induced nasal symptoms. IL-5 induced eosinophilia only, but IL-13 contributed to both nasal epithelial thickening and eosinophilia induced by OVA-plus-papain. Dexamethasone ameliorated OVA-alone-induced nasal epithelial thickening. However, OVA-plus-papain-induced nasal epithelial thickening was only partially controlled by dexamethasone. These results demonstrate that IL-33/ST2-pathway-mediated ILC2 activation exacerbated Th2-cell-induced nasal inflammation by producing IL-13. Although Th2-cell-alone-induced nasal inflammation was controlled by corticosteroid treatment, the activation of ILC2s conferred treatment resistance. Therefore, ILC2s and their activators could be therapeutic targets for treatment-refractory nasal allergic disorders.

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Table of Contents

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Corrigendum

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Dissecting the formation, structure and barrier function of the stratum corneum

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Long-term survival of the mouse ES cell-derived mast cell, MEDMC-BRC6, in mast cell-deficient Kit W-sh/W-sh mice

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Abstract
Mast cells (MCs) play pivotal roles in allergic reactions and the host defense against microbial infection through the IgE-dependent and IgE-independent signaling pathways. MC lines that can be analyzed both in vitro and in vivo would be useful for the study of MC-dependent immune responses. Here, we investigated the functional characteristics of a mouse embryonic stem cell-derived MC-like cell line, MEDMC-BRC6. The cell line expressed FcεRI and c-Kit and showed degranulation and production of inflammatory cytokines and chemokines, including TNF-α, IL-6 and MCP-1, upon cross-linking FcεRI with IgE. These cytokines and chemokines were also produced by the cell line by stimulation of TLR2 and TLR4. MEDMC-BRC6 survived in the peritoneal cavity and the ear skin for at least 6 months after the transfer into genetically compatible MC-deficient KitW-sh/W-sh mice, in which systemic anaphylaxis was successfully induced. Thus, MEDMC-BRC6 cells represent a potent tool for investigating the functions of MCs in vitro and in vivo.

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Brain changes associated with age-related hearing loss.

Purpose of review: To discuss brain changes associated with age-related hearing loss (ARHL), including cognitive abilities and neuroimaging findings. This information will be helpful to hypothesize and ultimately understand how ARHL may be mechanistically related to changes in brain structure and function. It will also be helpful to guide the strength of treatment recommendations. Recent findings: ARHL has recently been associated with cognitive impairment and dementia. This observation is present in both cross-sectional and longitudinal analyses, in diverse patient populations, and after controlling for a variety of potential confounders. Preliminary brain imaging studies show smaller brain volumes as well as white matter tract dysfunction in adults with hearing loss. Summary: ARHL is related to cognitive impairment and dementia. More study is needed to determine if the association is causal, and if treatment efforts could reduce the risk of cognitive impairment and dementia. Given the low risk of treating ARHL and the potential health benefit for the aging brain, it is recommended to proactively discuss ARHL and treatment with patients. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Verification and characterization of an alternative low density lipoprotein receptor-related protein 1 splice variant

by Marlen Kolb, Susanne Kurz, Angelika Schäfer, Klaus Huse, Andreas Dietz, Gunnar Wichmann, Gerd Birkenmeier

Background

Low density lipoprotein (LDL) receptor-related protein 1 (LRP1) is a ubiquitously expressed multi-ligand endocytosis receptor implicated in a wide range of signalling, among others in tumour biology. Tumour-associated genomic mutations of the LRP1 gene are described, but nothing is known about cancer-associated expression of LRP1 splice variants Therefore, the focus of this study was on an annotated truncated LRP1 splice variant (BC072015.1; NCBI GenBank), referred to as smLRP1, which was initially identified in prostate and lung carcinoma.

Methods

Using PCR and quantitative PCR, the expression of LRP1 and smLRP1 in different human tissues and tumour cell lines was screened and compared on tumour biopsies of head and neck squamous cell carcinoma (HNSCC). Using a recently developed anti-smLRP1 antibody, the expression of the putative LRP1 protein isoform in tumour cell lines in Western blot and immunofluorescence staining was further investigated.

Results

The alternative transcript smLRP1 is ubiquitously expressed in 12 human cell lines of different origin and 22 tissues which is similar to LRP1. A shift in expression of smLRP1 relative to LRP1 towards smLRP1 was observed in most tumour cell lines compared to healthy tissue. The expression of LRP1 as well as smLRP1 is decreased in HNSCC cell lines in comparison to healthy mucosa. In vitro results were checked using primary HNSCC. Furthermore, the expression of the protein isoform smLRP1 (32 kDa) was confirmed in human tumour cell lines.

Conclusions

Similar to LRP1, the truncated splice variant smLRP1 is ubiquitously expressed in healthy human tissues, but altered in tumours pointing to a potential role of smLRP1 in cancer. Comparative results suggest a shift in expression in favour of smLRP1 in tumour cells that warrant further evaluation. The protein isoform is suggested to be secreted.



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Prevalence of Hidradenitis Suppurativa Among Patients with Down Syndrome: a population based cross sectional analysis

Summary

Background

Hidradenitis Suppurativa (HS) has been linked to Down syndrome (DS).

Objective

To determine whether Down syndrome patients have a higher prevalence of HS, and whether diagnosis of HS occurs at an earlier age.

Methods

Cross-sectional analysis in a population sample of 11,936 DS patients and 16,813,290 non-DS patients. The primary outcome was diagnosis of HS. SNOMED-CT terms were used to identify patients with DS and HS. We used logistic regression models and significant interaction terms to evaluate the relationship between DS and HS. We also compared proportion of incident HS cases within five-year age groups to determine whether DS patients had earlier diagnosis of HS.

Results

Prevalence of HS among DS patients was 2.1%, compared to 0.3% for patients without DS (p<0.001). HS prevalence was greatest among DS patients who were aged 18-29 years. HS prevalence was not different between female and male DS patients or between white and non-white DS patients, after controlling for age, gender and obesity. Compared to those without DS, patients with the condition had increased odds of HS in unadjusted [OR 7.84, 95% CI 6.93-8.88] and adjusted [OR 5.24, 95% CI 4.62-5.94] analyses. Diagnosis of HS was made by the age of 29 years in 81.8% of patients with DS, compared to 34.0% of patients without the condition (p<0.001).

Conclusion

HS is strongly associated with DS across demographic subgroups, and the disease may present earlier life in these patients. These findings have implications for surveillance and care of patients with DS.

This article is protected by copyright. All rights reserved.



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Adaptive B Cell Responses to Influenza Virus Infection in the Lung

Viral Immunology , Vol. 0, No. 0.


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Bacterial microbiome in the nose of healthy cats and in cats with nasal disease

by Elisabeth S. Dorn, Barbara Tress, Jan S. Suchodolski, Tariq Nisar, Prajesh Ravindran, Karin Weber, Katrin Hartmann, Bianka S. Schulz

Background

Traditionally, changes in the microbial population of the nose have been assessed using conventional culture techniques. Sequencing of bacterial 16S rRNA genes demonstrated that the human nose is inhabited by a rich and diverse bacterial microbiome that cannot be detected using culture-based methods. The goal of this study was to describe the nasal microbiome of healthy cats, cats with nasal neoplasia, and cats with feline upper respiratory tract disease (FURTD).

Methodology/Principal findings

DNA was extracted from nasal swabs of healthy cats (n = 28), cats with nasal neoplasia (n = 16), and cats with FURTD (n = 15), and 16S rRNA genes were sequenced. High species richness was observed in all samples. Rarefaction analysis revealed that healthy cats living indoors had greater species richness (observed species p = 0.042) and Shannon diversity (p = 0.003) compared with healthy cats living outdoors. Higher species richness (observed species p = 0.001) and Shannon diversity (pMoraxella spp. was the most common genus, while it was unclassified Bradyrhizobiaceae in cats with nasal neoplasia. High individual variability was observed.

Conclusion

This study demonstrates that the nose of cats is inhabited by much more variable and diverse microbial communities than previously shown. Future research in this field might help to develop new diagnostic tools to easily identify nasal microbial changes, relate them to certain disease processes, and help clinicians in the decision process of antibiotic selection for individual patients.



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Omalizumab prevents anaphylaxis and improves symptoms in systemic mastocytosis; efficacy and safety observations

Abstract

Background

Patients with systemic mastocytosis (SM) may suffer from mast cell (MC) mediator-related symptoms insufficiently controlled by conventional therapy. Omalizumab is an established treatment in other MC-driven diseases, but experiences in SM are limited.

Objective

To assess the efficacy and safety of omalizumab in SM.

Methods

In our patient cohort we evaluated all SM patients treated with omalizumab. A physician global assessment of type and severity of symptoms was performed at baseline, at 3 and 6 months and at latest follow-up. Quality-of-life was assessed by visual analogue scale. S-tryptase and KIT D816V allele burden were monitored.

Results

A total of 14 adult SM patients (10 ISM, 2 BMM, 1 SSM and 1 ASM-AHN) received omalizumab with a median duration of 17 months (range: 1-73 months). One patient was excluded due to concomitant cytoreductive therapy. In the remaining 13 patients we observed a significant reduction of symptoms, with complete symptom control in five (38.5%), major response in three (23.1%) and a partial response in three (23.1%) patients, whereas two patients (15.4%) withdrew due to subjective side-effects at first dose. The treatment was most effective for recurrent anaphylaxis and skin symptoms, less for gastrointestinal, musculoskeletal and neuropsychiatric symptoms. Patient-reported quality-of-life showed significant improvement. No significant changes in s-tryptase/KIT D816V allele burden were observed. No severe adverse events were recorded.

Conclusions

Omalizumab appears to be a promising treatment option in SM, effectively preventing anaphylaxis and improving chronic MC mediator-related symptoms, insufficiently controlled by conventional therapy. Controlled studies are needed to substantiate findings.

This article is protected by copyright. All rights reserved.



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Treatment of a patient with HIV and metastatic melanoma with consecutive Ipilimumab and Nivolumab

Abstract

Recently new therapeutic agents were developed leading to a paradigm shift in melanoma treatment by the approval of checkpoint inhibitors and targeted therapies. However, in clinical trials HIV-infected melanoma patients have been excluded due to their condition and no randomized double-blind placebo-controlled studies have been published to evaluate the efficacy of these therapies in melanoma patients with concomitant HIV infection.

We report on a HIV-positive melanoma patient treated sequentially with ipilimumab and nivolumab whose CD4 count and virus replication have been monitored closely.

This article is protected by copyright. All rights reserved.



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The association between the clinical diversity of psoriasis and depressive symptoms: The HUNT Study, Norway

Abstract

Background

While a number of observational hospital-based studies have reported an association between psoriasis and depression, less is known about the clinical diversity of psoriasis and depressive symptoms.

Objective

To investigate the associations of inverse psoriasis, psoriasis severity and psoriasis duration with depressive symptoms in a general population.

Methods

We linked data from the population-based third Nord-Trøndelag Health Study (HUNT3) to the Norwegian Prescription Database (NorPD) and Statistics Norway. Depressive symptoms were assessed using the Hospital Anxiety and Depression Scale (HADS). Associations between psoriasis and depressive symptoms (HADS ≥ 8) were estimated using logistic regression.

Results

Among 37 833 participants in HUNT3, we found a weak association between any psoriasis and the prevalence of depressive symptoms (fully adjusted odds ratio (OR) 1.12, 95% confidence interval (CI) 0.97-1.28). The association with depressive symptoms was stronger when psoriasis was characterized by inverse anatomical distribution (OR 1.32, 95% CI 1.02-1.70), requirement of systemic psoriasis medication (OR 1.47, 95% CI 1.00-2.17) or long disease duration (OR 1.33, 95% CI 1.09-1.64). Conversely, when there was no inverse psoriasis distribution, no requirement of systemic medication, or shorter disease duration, psoriasis was not meaningfully associated with depressive symptoms.

Conclusion

Overall, depressive symptoms do not seem to be a major concern among subjects with psoriasis in a general Norwegian population. However, among subjects with inverse anatomical distribution, requirement of systemic psoriasis medication or long disease duration, depressive symptoms may be particularly important to address when evaluating the burden of psoriasis.

This article is protected by copyright. All rights reserved.



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Topical brimonidine gel for extended-duration local anesthesia

Abstract

Clinicians have been increasingly using topical anesthetic agents to diminish the pain associated with dermatologic and cosmetic procedures. Among the developed topical anesthetics, lidocaine-based products are the most widely used due to their ample pain relief and minimal side effects.1 Many local anesthetics and additional techniques have been developed, but the limited duration of local anesthesia is still challenging to the practitioners.

This article is protected by copyright. All rights reserved.



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Genetic predisposition for chronic venous insufficiency in several genes for matrix metalloproteinases (MMP-2, MMP-9, MMP-12) and their inhibitor TIMP-2

Abstract

Objective

The project was scheduled as a case–control study to investigate the correlation between MMP-2 (rs243864), MMP-9 (3918242), MMP-12 (rs7123600) and TIMP-2 (rs8176329) polymorphisms and chronic venous disease (CVD) risk. The genotype and phenotype research envisages the testing of possible associations between MMP and TIMP-2 genotypes and phenotypes of CVD.

Material and Methods

150 CVD patients and 227 controls were enrolled into the study. The MMPs and TIMP-2 genotypes were identified by the PCR method and restriction analysis according to standard protocols.

Results

The G allele of MMP-2 -790 T/G was 1.85 times more frequent in men with CVD than in the control group (p꞊0.008). The T allele of MMP-9 -1562 C/T was observed 2.571times more frequently in CVD patients than in the control individuals (both in men and women) with clinically significant specificity (p= 0.0000009). The G allele of MMP-12 rs7123600 was determined 2.082 times more frequently in CVD female patients than in the control group with clinically significant specificity (p꞊0.02). No significant result in TIMP-2 rs8176329 polymorphism in the case–control study was observed. CVD women with G allele in MMP-2 -790 T/G in the genotype-phenotype study are seen to develop ulceration 2.539 times more frequently (P=0.003). The G allele of MMP12 rs7123600 was detected 3.167 times more frequently in CVD women with ulceration compared with CVD women without ulceration (P=0.007). In CVD men in C6 stage, the incidence of AG genotype in rs7123600v MMP12 polymorphism was found to be 4.675 times higher compared to CVD women with C6 staging (P=0.005). The AG genotype in TIMP 2 rs8176329 polymorphism was found to be associated with higher risk of tumour (P=0.01).

Conclusion

Studying these polymorphisms can contribute to better identification of patients at higher risk of developing CVD, while providing the most appropriate prevention and treatment strategies for limiting the progression and complications of CVD.

This article is protected by copyright. All rights reserved.



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Anaphylaxis to vanilla ice cream: a near fatal cross reactivity phenomenon

Abstract

Although lupine is amongst the 17 foods that have to be declared it still has the features of a hidden allergen because those potentially allergic to lupine are not aware of the fact and, therefore, do not take adequate consequences, i.e. avoidance. Lupine occurs as additive in wheat flour, and is used as wheat and soy substitute. The significance of lupine is: a) its potential cross-reactivity with peanut and b) that the allergic reactions are mostly severe.1

This article is protected by copyright. All rights reserved.



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Advanced film-forming gel formula vs spring thermal water and white petrolatum as primary dressings after full face ablative fractional CO2 laser resurfacing: A comparative split-face pilot study

Abstract

Background

Aesthetically pleasing results and fast, uneventful recovery are highly desirable after rejuvenating ablative laser procedures. Wound dressings following ablative laser procedures should ideally improve and optimize the wound healing environment.

Objective

The purpose of this comparative split face, single blinded, prospective observational study was to assess the efficacy and acceptability of two primary wound dressings immediately after a full face fractional CO2 laser resurfacing procedure.

Methods

The assessments of an innovative film-forming dressing called Stratacel (SC) vs spring thermal water + Vaseline (V+), were conducted after a standardized, single pass, full-face ablative fractional CO2 laser skin resurfacing procedure. Clinical parameters such as haemoglobin – HB; surface temperature – ST; micro-textural modifications – MT; superficial melanin – M; intra-follicular porphyrines – P, were assessed at different phases of the healing process using standardized, non-invasive technologies.

Results

Five female volunteers were enrolled in this in-patient, controlled pilot study. Most of the clinical parameters considered, including 3D surface texture analysis, revealed a better performance of SC vs. V+ during the early, more delicate phases of the healing process.

Conclusions

This preliminary study, even if performed on a small number of volunteers, confirmed a definite advantage of the tested semi-permeable film-forming formula (SC) over a more conventional post-operative skin care regime (V+). Clinical results could be explained by a better uniformity of distribution of SC over the micro-irregularities induced by ablative fractional CO2 laser resurfacing. Its thin, semi-permeable film might, in fact act as an efficient, perfectly bio-compatible, full contact, temporary skin barrier, able to protect extremely delicate healing surfaces from potential environmental irritations.

This article is protected by copyright. All rights reserved.



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Multiple miliary osteoma cutis: An overlooked dermatosis?

Abstract

A 63-year-old, skin type V woman presented with a 3-year history of multiple asymptomatic papules on the face, which were slowly increasing in number and causing cosmetic concerns. These lesions had been interpreted as adult acne, and for the past two years she had been treated with topical and systemic retinoids and submitted to a salicylic acid peel without benefit. She had no prior history of acne and her medical past was unremarkable. Regular medication or other facial treatments were denied.

This article is protected by copyright. All rights reserved.



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Degranulation and shrinkage of dark cells in eccrine glands and elevated serum carcinoembryonic antigen in patients with Acquired Idiopathic Generalized Anhidrosis

Abstract

Background

Acquired idiopathic generalized anhidrosis (AIGA) is characterized by anhidrosis/hypohidrosis without other autonomic and neurological dysfunctions. Pathologically, AIGA is considered to usually present no significant morphological alterations in eccrine glands, the secretory portion of which consists of clear cells, dark cells, and myoepithelial cells. AIGA patients recently have been reported to show high serum concentrations of carcinoembryonic antigen (CEA).

Objective

Our aim is to reveal morphological abnormalities of dark cells and investigate their relationship with serum CEA.

Methods

We performed comparative analysis of eccrine glands between sweat-preserved and non-sweating skin in four AIGA patients. Serum CEA concentrations in 22 cases with AIGA were measured with healthy volunteers. Furthermore, we semiquantitatively investigated dermcidin, FoxA1, and CEA expression in eccrine glands of 12 cases with AIGA and 5 cases with non-AIGA.

Results

Marked degranulation and shrinkage of dark cells consistently occurred in AIGA. Furthermore, high serum CEA concentrations were found in 14 out of 22 AIGA patients (over 60%), but serum CEA levels were not correlated with CEA expression in eccrine glands. Dermcidin expression in dark cells apparently decreased in AIGA patients, severely in those with high serum CEA, and moderately in those with low serum CEA, while well-preserved expression was found in non-AIGA subjects.

Conclusion

Our study suggests morphological damage and molecular dysregulation of dark cells, leading to impairment of their functions in AIGA patients. Severely damaged dark cells correspond to high serum CEA. Accordingly, these pathological changes in eccrine dark cells may be involved in anhidrosis/hypohidrosis of AIGA.

This article is protected by copyright. All rights reserved.



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Differential disease burden and treatment patterns among adults with psoriasis and atopic dermatitis seen in hospital versus private clinics

Abstract

Psoriasis and atopic dermatitis (AD) are chronic inflammatory skin diseases which are prevalent in children and adults. In many countries, treatment of both conditions is managed by general practitioners, as well as dermatologists in private clinics and hospital outpatient settings. All Danish citizens have free and equal access to health care services, including general practitioners (GPs),

This article is protected by copyright. All rights reserved.



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Cost-of-illness of patients with lymphedema

Abstract

Background

Chronic lymphedema is characterized by a continuous need for medical treatment, many comorbidities, and impaired quality of life. In Germany, about 4.5 million patients are affected by lymphedema. Thus, lymphedema causes high direct and indirect costs, even more in case of complications such as erysipelas and ulcers.

Objective

The aim of this study was to determinate the costs of illness of community lymphedema patients living in the metropolitan area of Hamburg, Germany.

Methods

An observational cross-sectional study in patients with lymphedema and combined lipolymphedema of any origin was performed analysing direct and indirect costs for the patients, the statutory health insurance, and society.

Results

In total, 348 patients (90.8% female) were examined and interviewed. The mean age of the patients was 57.3 ± 14.5 years. On average, the total costs per patient and year were € 5784, of which € 4445 (76.0%) were direct costs and € 1338 indirect costs. Within the direct medical costs, € 3796 were accounted for the statutory health insurances and € 649 for the patient. The main cost drivers were costs for manual decongestive therapy and disability costs.

Conclusion

Chronic lymphedema is associated with high direct and indirect costs. This community based study is the first cost analysis of chronic lymph- and combined lipolymphedema giving insights to economic impact of lymphedema treatment. There is a high need for structured disease management programs in order to diagnose and treat lymphedema early and to avoid complications, thus limiting socioeconomic burden.

This article is protected by copyright. All rights reserved.



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Asthma pressurised metered dose inhaler performance: propellant effect studies in delivery systems

Current pressurised metered dose asthma inhaler (pMDI) propellants are not inert pharmacologically as were previous chlorofluorocarbons, have smooth muscle relaxant‚ partial pressure effects in the lungs and i...

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Verification and characterization of an alternative low density lipoprotein receptor-related protein 1 splice variant

by Marlen Kolb, Susanne Kurz, Angelika Schäfer, Klaus Huse, Andreas Dietz, Gunnar Wichmann, Gerd Birkenmeier

Background

Low density lipoprotein (LDL) receptor-related protein 1 (LRP1) is a ubiquitously expressed multi-ligand endocytosis receptor implicated in a wide range of signalling, among others in tumour biology. Tumour-associated genomic mutations of the LRP1 gene are described, but nothing is known about cancer-associated expression of LRP1 splice variants Therefore, the focus of this study was on an annotated truncated LRP1 splice variant (BC072015.1; NCBI GenBank), referred to as smLRP1, which was initially identified in prostate and lung carcinoma.

Methods

Using PCR and quantitative PCR, the expression of LRP1 and smLRP1 in different human tissues and tumour cell lines was screened and compared on tumour biopsies of head and neck squamous cell carcinoma (HNSCC). Using a recently developed anti-smLRP1 antibody, the expression of the putative LRP1 protein isoform in tumour cell lines in Western blot and immunofluorescence staining was further investigated.

Results

The alternative transcript smLRP1 is ubiquitously expressed in 12 human cell lines of different origin and 22 tissues which is similar to LRP1. A shift in expression of smLRP1 relative to LRP1 towards smLRP1 was observed in most tumour cell lines compared to healthy tissue. The expression of LRP1 as well as smLRP1 is decreased in HNSCC cell lines in comparison to healthy mucosa. In vitro results were checked using primary HNSCC. Furthermore, the expression of the protein isoform smLRP1 (32 kDa) was confirmed in human tumour cell lines.

Conclusions

Similar to LRP1, the truncated splice variant smLRP1 is ubiquitously expressed in healthy human tissues, but altered in tumours pointing to a potential role of smLRP1 in cancer. Comparative results suggest a shift in expression in favour of smLRP1 in tumour cells that warrant further evaluation. The protein isoform is suggested to be secreted.



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iFAS: Image Fidelity Assessment

image Fidelity Assessment (iFAS) is a software tool designed to assist image quality researchers providing easy access to a range of state-of-the-art measures which can be applied on a single pair of images and/or in a full database, as well as intuitive visualizations that aid data analysis, e.g., images and histograms of pixel-wise image differences, scatter plots and correlation analysis. The software is freely available for non-commercial use.

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Reactive and non-reactive riser flow: numerical and experimental study



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From ideal gas to relevant three-phase conditions in hydrodenitrogenation reactions and reactors



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Optimal Joint Detection and Estimation That Maximizes ROC-Type Curves

Combined detection-estimation tasks are frequently encountered in medical imaging. Optimal methods for joint detection and estimation are of interest because they provide upper bounds on observer performance, and can potentially be utilized for imaging system optimization, evaluation of observer efficiency, and development of image formation algorithms. We present a unified Bayesian framework for decision rules that maximize receiver operating characteristic (ROC)-type summary curves, including ROC, localization ROC (LROC), estimation ROC (EROC), free-response ROC (FROC), alternative free-response ROC (AFROC), and exponentially-transformed FROC (EFROC) curves, succinctly summarizing previous results. The approach relies on an interpretation of ROC-type summary curves as plots of an expected utility versus an expected disutility (or penalty) for signal-present decisions. We propose a general utility structure that is flexible enough to encompass many ROC variants and yet sufficiently constrained to allow derivation of a linear expected utility equation that is similar to that for simple binary detection. We illustrate our theory with an example comparing decision strategies for joint detection-estimation of a known signal with unknown amplitude. In addition, building on insights from our utility framework, we propose new ROC-type summary curves and associated optimal decision rules for joint detection-estimation tasks with an unknown, potentially-multiple, number of signals in each observation.

http://ift.tt/2slJxNw

New Accelerator Seeks to Attract Connected Health and Fitness Startups from Across the Country to Baltimore

Johns Hopkins Technology Ventures, Plank Industries, the University of Maryland (through UM Ventures), Brown Advisory and the Abell Foundation announced today that they are providing support for M-1 Ventures, a new Baltimore-based startup accelerator focused on connected health and fitness technologies. The 16-week program will be housed in FastForward East, an innovation hub on the Johns Hopkins medical campus, and will challenge startups selected from a national applicant pool to validate their business models, engage with customers and build on traction they have already generated. Additional support for the program comes from the Maryland Department of Commerce and Village Capital.



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Innovative Approach to Triage Oral Precancer

Conditions:   Oral Cancer;   Oral Premalignant Lesion
Intervention:  
Sponsors:   British Columbia Cancer Agency;   BC Cancer Foundation;   University of British Columbia
Recruiting - verified June 2017

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Special Forum: Commodity Frontiers and the Global History of Capitalism: A Discussion about Sven Beckert’s Empire of Cotton



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Special Forum: Commodity Frontiers and the Global History of Capitalism: A Discussion about Sven Beckert’s Empire of Cotton. Editor's Introduction



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Insurance and Community-Level Socioeconomic Status in Pharyngeal Cancer

This study of National Cancer Database records assesses the association of insurance and community-level socioeconomic status with outcomes for patients with squamous cell carcinoma of the pharynx.

http://ift.tt/2ttGbZi

Audiometric Measurement for Vocal-Fold Function Assessment

This study examines whether bedside measurement of infant cry volume using a smartphone application can be a screening tool for vocal-fold movement in flexible nasolaryngoscopy.

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Control of Pain After Tonsillectomy in Children

This review examines the treatment of postoperative pain in children after tonsillectomy.

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Unilateral Nasal Congestion in an Elderly Man

An elderly man had right-sided nasal congestion and intermittent epistaxis; CT imaging of the sinuses revealed opacification of the right nasal cavity with tissue protruding into the nasopharynx and complete opacification of the sinuses. What is your diagnosis?

http://ift.tt/2u2Jm79

Rare splicing defects of FAS underly severe recessive autoimmune lymphoproliferative syndrome

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Publication date: Available online 29 June 2017
Source:Clinical Immunology
Author(s): N. Agrebi, I. Ben-Mustapha, N. Matoussi, N. Dhouib, M. Ben-Ali, N. Mekki, M. Ben-Ahmed, B. Larguèche, S. Ben Becher, M. Béjaoui, M.R. Barbouche
Autoimmune lymphoproliferative syndrome (ALPS) is a prototypic disorder of impaired apoptosis characterized by autoimmune features and lymphoproliferation. Heterozygous germline or somatic FAS mutations associated with preserved protein expression have been described. Very rare cases of homozygous germline FAS mutations causing severe autosomal recessive form of ALPS with a complete defect of Fas expression have been reported.We report two unrelated patients from highly inbred North African population showing a severe ALPS phenotype and an undetectable Fas surface expression. Two novel homozygous mutations have been identified underlying rare splicing defects mechanisms. The first mutation breaks a branch point sequence and the second alters a regulatory exonic splicing site. These splicing defects induce the skipping of exon 6 encoding the transmembrane domain of CD95. Our findings highlight the requirement of tight regulation of FAS exon 6 splicing for balanced alternative splicing and illustrate the importance of such studies in highly consanguineous populations.



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Computational analysis of multimorbidity between asthma, eczema and rhinitis

Background The mechanisms explaining the co-existence of asthma, eczema and rhinitis (allergic multimorbidity) are largely unknown. We investigated the mechanisms underlying multimorbidity between three main allergic diseases at a molecular level by identifying the proteins and cellular processes that are common to them. Methods An in silico study based on computational analysis of the topology of the protein interaction network was performed in order to characterize the molecular mechanisms of multimorbidity of asthma, eczema and rhinitis. As a first step, proteins associated to either disease were identified using data mining approaches, and their overlap was calculated. Secondly, a functional interaction network was built, allowing to identify cellular pathways involved in allergic multimorbidity. Finally, a network-based algorithm generated a ranked list of newly predicted multimorbidity-associated proteins. Results Asthma, eczema and rhinitis shared a larger number of associated proteins than expected by chance, and their associated proteins exhibited a significant degree of interconnectedness in the interaction network. There were 15 pathways involved in the multimorbidity of asthma, eczema and rhinitis, including IL4 signaling and GATA3-related pathways. A number of proteins potentially associated to these multimorbidity processes were also obtained. Conclusions These results strongly support the existence of an allergic multimorbidity cluster between asthma, eczema and rhinitis, and suggest that type 2 signaling pathways represent a relevant multimorbidity mechanism of allergic diseases. Furthermore, we identified new candidates contributing to multimorbidity that may assist in identifying new targets for multimorbid allergic diseases.

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Johns Hopkins Surgeons Report Success With First 20 Patients Undergoing Minimally Invasive Pancreatic Transplant Surgery

Surgeons at Johns Hopkins Medicine report that their first series of a minimally invasive procedure to treat chronic pancreas disease, known as severe pancreatitis, resulted in shorter hospital stays, less need for opioids and fewer complications, compared with standard surgical approaches.



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Epitope mapping and kinetics of CD4 T cell immunity to pneumonia virus of mice in the C57BL/6 strain

Pneumonia virus of mice (PVM) infection has been widely used as a rodent model to study the closely related human respiratory syncytial virus (hRSV). While T cells are indispensable for viral clearance, they also contribute to immunopathology. To gain more insight into mechanistic details, novel tools are needed that allow to study virus-specific T cells in C57BL/6 mice as the majority of transgenic mice are only available on this background. While PVM-specific CD8 T cell epitopes were recently described, so far no PVM-specific CD4 T cell epitopes have been identified within the C57BL/6 strain. Therefore, we set out to map H2-IAb-restricted epitopes along the PVM proteome. By means of in silico prediction and subsequent functional validation, we were able to identify a MHCII-restricted CD4 T cell epitope, corresponding to amino acids 37-47 in the PVM matrix protein (M37-47). Using this newly identified MHCII-restricted M37-47 epitope and a previously described MHCI-restricted N339-347 epitope, we generated peptide-loaded MHCII and MHCI tetramers and characterized the dynamics of virus-specific CD4 and CD8 T cell responses in vivo. The findings of this study can provide a basis for detailed investigation of T cell-mediated immune responses to PVM in a variety of genetically modified C57BL/6 mice.

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Uncovering the Priming Potential of the Green Leaf Volatile Z-3-hexenyl acetate: towards a new disease control tool



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Innovation Center for Chemical Reaction Engineering Science and Technology



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Multiscale analysis and design of industrial and emerging thermal processes involving solids



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Fluctuating neck mass: Letter to the editor and review of the literature on anterior jugular phlebectasia

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Publication date: Available online 28 June 2017
Source:European Annals of Otorhinolaryngology, Head and Neck Diseases
Author(s): M.Y. Abboud, A. de Lafontaine-Ruel, B. Riederer, D. Philippon




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Hamartoma of the tongue

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Publication date: Available online 28 June 2017
Source:European Annals of Otorhinolaryngology, Head and Neck Diseases
Author(s): B. Hemmaoui, M. Sahli, A. Jahidi, F. Benariba




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Assessment and management of cervico-mediastinal goiter

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Publication date: Available online 28 June 2017
Source:European Annals of Otorhinolaryngology, Head and Neck Diseases
Author(s): E. Brenet, X. Dubernard, J.C. Mérol, M.A. Louges, M. Labrousse, M. Makeieff
Cervico-mediastinal goiter is a particular entity from the point of view of thyroid surgery. Its volume, hardness and intrathoracic extension require the surgeon to adapt technique and perform a painstaking preoperative work-up, so as to draw up fully-fledged plan. CT is now indispensable, to anticipate risks and determine whether sternotomy is needed. Surgery seems to induce more postoperative complications than in conventional surgery, although they can be reduced by retrograde dissection of the inferior laryngeal nerve and downward dissection of the posterior side of the lobe to optimize control of adjacent structures. This surgery requires optimal teamwork between all of the specialties involved in patient management: medical, radiological, anesthesiological and surgical.



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Transoral lateral oropharyngectomy

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Publication date: Available online 29 June 2017
Source:European Annals of Otorhinolaryngology, Head and Neck Diseases
Author(s): F. Rubin, O. Laccourreye, G.S. Weinstein, F.C. Holsinger
Based on a review of the indexed scientific medical literature, the authors document the key technical points, the errors to avoid, the limitations, indications and main oncologic and functional results when performing transoral lateral oropharyngectomy in cancer originating from the tonsillar region.



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Anal herpes

Description

A 27-year-old man without significant past medical history presented to the emergency department with a 5-day evolution of abdominal and anal pain and haematochezia. Physical examination revealed perianal erythema and a painful digital anal examination without other findings. CT scans showed signs of proctitis (slight thickening of rectal wall and slight stranding of perirectal fat). Flexible rectosigmoidoscopy didn't reveal any pathological abnormalities. The patient was discharged with antibiotics and analgesics with the suspicion of proctitis. Three days later, the patient returned to the emergency department with worsening of the anal pain that irradiated to the buttocks and the physical examination revealed red bumps with ulceration (figure 1). Anal herpes was suspected and the patient was treated with an antiviral (brivudine) and carbamazepine for symptomatic relief. Seven days after treatment the patient had complete resolution of his symptoms and there were no cutaneous lesions.

Figure...



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Cardiogenic shock from coronary vasculitis in granulomatosis with polyangiitis

Granulomatosis with polyangiitis (GPA) is a systemic vasculitis characterised by necrotising inflammatory changes in small-sized and medium-sized vessels and granuloma formation. It most commonly involves the kidneys and respiratory tract, but it can present with widespread manifestations involving any organ system. Rarely, it causes coronary vasculitis which can precipitate a severe cardiomyopathy. Here, we report a patient who presented in cardiogenic shock requiring vasopressors and was found to have extensive myocardial ischaemia secondary to coronary vasculitis. Further investigation led to a diagnosis of GPA, and he responded to treatment with corticosteroids, cyclophosphamide and plasmapheresis.



http://ift.tt/2sV3KIN

Large prostatic stones with staghorn renal calculus in a 61-year-old man: an unusual presentation of uncommon disease

Prostatic parenchymal calculi are common in ageing men who are evaluated for benign prostatic hyperplasia or prostate cancer. Giant prostatic calculi are very rare, usually associated with local predisposing factors for urinary stasis and infections, and traditionally managed by open surgery. We present the first case of its kind to be associated with a concurrent staghorn nephrolithiasis, and removed successfully by endoscopic approach using an access sheath through the urethra.



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Gangrenous digital infarcts in a severe case of cutaneous polyarteritis nodosa

Cutaneous polyarteritis nodosa (CPAN) is a rare diagnosis which is distinct from polyarteritis nodosa (PAN). PAN is a medium-vessel vasculitis which can affect multiple organs and classically produces microaneurysms in the vasculature. CPAN is limited to the skin mainly affecting small vessels. There is an absence of microaneurysms in CPAN and it does not affect internal organs. However, the histopathological findings on the skin are similar to PAN. CPAN rarely progresses to PAN but relapses more often. We will illustrate a challenging case of a patient with CPAN who developed gangrenous infarcts despite initial immunosuppressive treatment with high-dose steroids and azathioprine. His treatment had to be escalated to intravenous cyclophosphamide which induced disease remission



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TUMOR TISSUE TRANSPORT AFTER INTRAPERITONEAL ANTICANCER DRUG DELIVERY.



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Impact of age and treatment group in childhood high hyperdiploid low risk B-cell acute lymphoblastic leukemia (ALL) : results of the CLG-EORTC 58951 study



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Unique long non-coding RNA expression signature in ETV6/RUNX1-Driven B-cell precursor acute lymphoblastic leukemia



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Coating of Fe2O3 nanoparticles for enhanced redox activity and stability



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Catalytic dehydrogenation of propane on Pt catalysts: the positive effect of hydrogen co-feeding



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First Principles Based Microkinetic Modelling of Methyl Butanoate Pyrolysis



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Exploring the reusability of aminated silicas for aldol condensations



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Insights on bioethanol to hydrocarbons conversion over ZSM-5 zeolite by a transient technique



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A novel multiplex NGS-based digital MLPA assay for copy number detection of 700 target sequences in childhood acute lymphoblastic leukemia



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Chemical Calculus



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CMS RPC tracker muon reconstruction

A new muon reconstruction algorithm is introduced at the CMS experiment. This algorithm reconstructs muons using only the central tracker and the Resistive Plate Chamber (RPC). The aim of this work is to study how a muon reconstructed only with tracker and RPC information would perform compared to the standard muon reconstruction of the CMS detector. The efficiencies to reconstruct and identify a RPC muon with a transverse momentum greater than 20 GeV/c are measured. The probabilities to misidentify hadrons as muons at low transverse momentum are also reported. These probabilities are compared to the standard muon identification used at CMS.

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Web-based monitoring tools for Resistive Plate Chambers in the CMS experiment at CERN

The Resistive Plate Chambers (RPC) are used in the CMS experiment at the trigger level and also in the standard offline muon reconstruction. In order to guarantee the quality of the data collected and to monitor online the detector performance, a set of tools has been developed in CMS which is heavily used in the RPC system. The Web-based monitoring (WBM) is a set of java servlets that allows users to check the performance of the hardware during data taking, providing distributions and history plots of all the parameters. The functionalities of the RPC WBM monitoring tools are presented along with studies of the detector performance as a function of growing luminosity and environmental conditions that are tracked over time.

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CMS RPC muon detector performance with 2010-2012 LHC data

The muon spectrometer of the CMS (Compact Muon Solenoid) experiment at the Large Hadron Collider (LHC) is equipped with a redundant system made of Resistive Plate Chambers (RPCs) and Drift Tube (DT) chambers in the barrel, RPC and Cathode Strip Chambers (CSCs) in the endcap region. In this paper, the operations and the performance of the RPC system during the first three years of LHC activity are presented. The stability of the RPC performance, in terms of efficiency, cluster size and noise, is also reported. Finally, the radiation background levels on the RPC system have been measured as a function of the LHC luminosity. Extrapolations to the High Luminosity LHC conditions are also discussed.

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Status of the Triple-GEM project for the upgrade of the CMS Muon System

The CMS GEM collaboration is performing a feasibility study to install triple-GEM detectors in the forward region of the muon system (1.6 < vertical bar eta vertical bar < 2.4) of the CMS detector at the LHC. Such micro-pattern gas detectors are able to cope with the extreme particle rates that are expected in that region during the High Luminosity phase of the LHC. With their spatial resolution of order 100 micron GEMs would not only provide additional benefits in the CMS muon High Level Trigger, but also in the muon identification and track reconstruction, effectively combining tracking and triggering capabilities in one single device. The present status of the full project will be reviewed, highlighting all importants steps and achievements since the start of the R& D in 2009. Several small and full-size prototypes were constructed with different geometries and techniques. The baseline design of the triple-GEM detector for CMS will be described, along with the results from extensive test measurements of all prototypes both in the lab and in test beams at the CERN SPS. The proposed on-and off-detector electronics for the final system will be presented.

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Intralabyrinthine schwannomas

Abstract

Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss and vertigo. In an own case series of 12 patients, 6 tumors showed an intracochlear, 3 an intravestibular, 1 a transmodiolar including the cerebellopontine angle (CPA), 1a transotic including the CPA, and 1 a multilocular location. The tumors were removed surgically in 9 patients, whereas 3 patients decided for a "wait-and-test-and-scan" strategy. Of the surgical patients, 3 underwent labyrinthectomy and cochlear implant (CI) surgery in a single-stage procedure; 1 patient had extended cochleostomy with CI surgery; 3 underwent partial or subtotal cochleoectomy, with partial cochlear reconstruction and CI surgery (n = 1) or implantation of electrode dummies for possible later CI after repeated MRI follow-up (n = 2); and in 2 patients, the tumors of the internal auditory canal and cerebellopontine angle exhibiting transmodiolar or transmacular growth were removed by combined translabyrinthine–transotic resection. For the intracochlear tumors, vestibular function could mostly be preserved after surgery. In all cases with CI surgery, hearing rehabilitation was successful, although speech discrimination was limited for the case with subtotal cochleoectomy. Surgical removal of intracochlear schwannomas via partial or subtotal cochleoectomy is, in principle, possible with preservation of vestibular function. In the authors' opinion, radiotherapy of ILS is only indicated in isolated cases. Cochlear implantation during or after tumor resection (i. e., as synchronous or staged surgeries) is an option for hearing rehabilitation in cartain cases and represents a therapeutic approach in contrast to a "wait-and-test-and-scan" strategy.



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Rheopheresis in treatment of idiopathic sensorineural sudden hearing loss

Abstract

Backround

Only few therapeutic options exist for patients with refractory sudden idiopathic sensorineural hearing loss (SISHL). Little is known about the efficacy of second-line therapies. Rheopheresis seems to be an effective therapeutic possibility.

Methods

Between 2012 and 2015, 106 patients with SISHL were enrolled in the study, of whom 52 were refractory to initial treatment. As salvage therapy, these patients were offered either 3 sessions of rheopheresis (33 pts) or intratympanic steroid treatment through MicroWick application (19 pts). Pure tone audiometry was performed at diagnosis, at the 1st month and the 1st year during the follow-up.

Results

Patients in the rheopheretic arm had higher hearing loss than in the MicroWick arm (81% vs. 52%, p = 0.04). In spite of this, there was a significant improvement for patients in the rheopheretic arm (27% of hearing loss reduction, p < 0.001) after the 1st month and this remained unchanged during the 1st year, while no improvement was seen in the MicroWick arm (0% of hearing loss reduction, p = 0.424). We found no predictive factor for steroid-failure in first-line therapy. Older age (p = 0.003), presence of vertigo (p = 0.006) and more profound initial hearing loss (p < 0.001) were identified as negative prognostic markers.

Conclusion

Rheopheresis can be used as a potentially effective and safe salvage therapy for patients with cortico-refractory SISHL.



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Case 20-2017: A 48-Year-Old Man with Weight Loss, Confusion, Skin Lesions, and Pancytopenia

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Presentation of Case. Dr. Robert H. Goldstein (Medicine): A 48-year-old man was seen in the emergency department of this hospital because of confusion. The week before this presentation, the patient, who was a lawyer, had traveled alone to Mexico for vacation. His family reported that he seemed to…

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Evaluation of variable selection approaches for pixel-based analysis of GC×GC data



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RPPA-Based Protein Profiling Reveals Enhanced PI3K/AKT/mTOR Signaling in ETV6/RUNX1-Positive Acute Lymphoblastic Leukemia Patients with Low CD200 Expression



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Evaluation of copper-64-ATSM for non invasive imaging of tumour hypoxia by positron emission

Wood, KA; (2009) Evaluation of copper-64-ATSM for non invasive imaging of tumour hypoxia by positron emission. Doctoral thesis, UCL (University College London). Green open access

http://ift.tt/2u2GjvB

Color Afterimages in Autistic Adults

Maule, J; Stanworth, K; Pellicano, E; Franklin, A; (2016) Color Afterimages in Autistic Adults. Journal of Autism and Developmental Disorders 10.1007/s10803-016-2786-5 . (In press). Green open access

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Using Directed Acyclic Graphs in Epidemiological Research in Psychosis: An Analysis of the Role of Bullying in Psychosis

Moffa, G; Catone, G; Kuipers, J; Kuipers, E; Freeman, D; Marwaha, S; Lennox, BR; Moffa, G; Catone, G; Kuipers, J; Kuipers, E; Freeman, D; Marwaha, S; Lennox, BR; Broome, MR; Bebbington, P; - view fewer (2017) Using Directed Acyclic Graphs in Epidemiological Research in Psychosis: An Analysis of the Role of Bullying in Psychosis. Schizophrenia Bulletin 10.1093/schbul/sbx013 . (In press). Green open access

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Exercise and environment: New qualitative work to link popular practice and public health

Hitchings, R; Latham, A; (2017) Exercise and environment: New qualitative work to link popular practice and public health. Health & Place 10.1016/j.healthplace.2017.04.009 . (In press).

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The impact of income inequality and national wealth on child and adolescent mortality in low and middle-income countries

Ward, JL; Viner, RM; (2017) The impact of income inequality and national wealth on child and adolescent mortality in low and middle-income countries. BMC Public Health , 17 , Article 429. 10.1186/s12889-017-4310-z . Green open access

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Team-teaching on a Large Multidisciplinary Engineering Mathematics Class: The Lessons We Have Learnt so far

Nyamapfene, AZ; (2016) Team-teaching on a Large Multidisciplinary Engineering Mathematics Class: The Lessons We Have Learnt so far. In: Kapranos, P, (ed.) ISEE 2016: Sixth International Symposium of Engineering Education. (pp. pp. 285-292). University of Manchester: Manchester, UK. Green open access

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The effect of life course socioeconomic position on crystallised cognitive ability in two large UK cohort studies: a structured modelling approach

Landy, R; Head, J; Richards, M; Hardy, R; (2017) The effect of life course socioeconomic position on crystallised cognitive ability in two large UK cohort studies: a structured modelling approach. BMJ Open , 7 (5) , Article e014461. 10.1136/bmjopen-2016-014461 . Green open access

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Integrating MATLAB Into First Year Engineering Mathematics: A Project Management Approach to Implementing Curriculum Change

Nyamapfene, AZ; (2016) Integrating MATLAB Into First Year Engineering Mathematics: A Project Management Approach to Implementing Curriculum Change. In: Proceedings of the 2016 IEEE International Conference on Teaching and Learning in Education (ICTLE'16). IEEE: Kuala Lumpur, Malaysia. Green open access

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Late Life Employment Histories and Their Association With Work and Family Formation During Adulthood: A Sequence Analysis Based on ELSA

Wahrendorf, M; Zaninotto, P; Hoven, H; Head, J; Carr, E; (2017) Late Life Employment Histories and Their Association With Work and Family Formation During Adulthood: A Sequence Analysis Based on ELSA. Journals of Gerontology: Social Sciences 10.1093/geronb/gbx066 . (In press). Green open access

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Aortic valve calcification: a bone of contention

Bertazzo, S; Gentleman, E; (2017) Aortic valve calcification: a bone of contention. European Heart Journal , 38 (16) pp. 1189-1193. 10.1093/eurheartj/ehw071 . Green open access

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Evaluating an alternative coding manual for the AAI for use with people with Personality Disorders

Pearson, J; (2007) Evaluating an alternative coding manual for the AAI for use with people with Personality Disorders. Doctoral thesis, UCL (University College London). Green open access

http://ift.tt/2u2oFs5

Posthumanism and the Graphic Novel in Latin America

King, Edward and Page, Joanna (Eds). (2017) Posthumanism and the Graphic Novel in Latin America. [Book]. UCL Press: London. Green open access

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Decision problems, complexity, traces, and representations

Lawton, S; Louder, L; McReynolds, DB; (2017) Decision problems, complexity, traces, and representations. Groups, Geometry, and Dynamics , 11 (1) pp. 165-188. 10.4171/GGD/393 . Green open access

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A Conversation about Healthy Eating

Lesica, N; (2017) A Conversation about Healthy Eating. [Book]. UCL Press: London. Green open access

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Diagnosing Melanomas Is Not Easy

Concerns over the accuracy of melanoma diagnoses are raised in a study of US pathologists.
Medscape Medical News

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Consensus Statement: Most Skin Procedures OK With Retinoids

Consensus statement changes recommendation to postpone skin procedures in patients on recent or concurrent systemic isotretinoin, except for manual dermabrasion and fully ablative laser treatments.
Medscape Medical News

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Accuracy assessment of surgical planning and three-dimensional-printed patient-specific guides for orthopaedic osteotomies



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Ook Goed. Een kritische biografie van René Victor, de voedstervader van het Vlaamse rechtsleven



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Estimated GFR poorly reflects the concentration of various uremic toxins in pediatric CKD patients



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Concentrations of representative uremic toxins in a healthy versus uremic pediatric population



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Analysis of a global registration trial of the efficacy and safety of CTL019 in pediatric and young adults with relapsed/refractory acute lymphoblastic leukemia (ALL)



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Scientists Manipulate 'Signaling' Molecules to Control Cell Migration

Johns Hopkins researchers report they have uncovered a mechanism in amoebae that rapidly changes the way cells migrate by resetting their sensitivity to the naturally occurring internal signaling events that drive such movement



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Use of a midline mandibular osteotomy to improve surgical access for transoral robotic resection of the base of tongue in a patient with trismus

Abstract

Background

The utilization of transoral robotic surgery (TORS) in patients with trismus is limited because of poor surgical exposure.

Methods

This report is about a 46-year-old man with a recurrent right base of tongue cancer who had severe postradiation trismus. We performed a midline mandibular osteotomy without a lip split and this resulted in a markedly improved surgical exposure. He underwent a TORS resection of the right base of the tongue with no significant complications.

Results

The midline mandibular osteotomy significantly improved the surgical exposure and facilitated exposure for TORS in a patient who otherwise would not be able to undergo TORS.

Conclusion

Utilization of a midline mandibular osteotomy allowed for increased exposure for TORS in a patient with limited mouth opening from postradiation trismus. Postoperative hemorrhage remains a significant concern and appropriate measures to mitigate the catastrophic consequences of this should be considered.



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Synopsis of transoral endoscopic laryngopharyngeal surgery for superficial pharyngeal cancers

Abstract

Background

Endoscopic laryngopharyngeal surgery (ELPS) was developed for superficial pharyngeal cancers in Japan. In this study, we present our results of ELPS for superficial pharyngeal cancers.

Methods

From November 2009 to December 2015, 258 patients with superficial pharyngeal cancers underwent ELPS. Results, including survival rates, postoperative complications, and vocal function, are reviewed.

Results

The median follow-up period of 258 patients was 31 months. The overall and cause-specific survival rates over 3 years were 85.7% and 100%, respectively. Only 3 patients incurred local recurrences and were successfully salvaged by re-ELPS. Regarding postoperative complications, 4 patients required reoperation because of postoperative bleeding. As for the postoperative quality of life, no patients developed vocal fold paralysis. Every patient was able to consume meals at preoperative levels.

Conclusion

ELPS is a feasible and minimally invasive treatment for superficial pharyngeal cancers with impressive quality of life results.



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Apport de la microscopie confocale par réflectance et de la tomographie par cohérence optique dans le diagnostic de tophus goutteux : premier cas rapporté

Publication date: Available online 28 June 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): C. David, E. Cinotti, B. Labeille, C. Habougit, P. Rubegni, F. Cambazard, J.L. Perrot




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Apport de la microscopie confocale par réflectance dans le diagnostic d’un mélanome du col utérin : premier cas rapporté

Publication date: Available online 28 June 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): J.L. Perrot, B. Labeille, E. Richard Coulet, S. Cochin, A.-C. Biron Schneider, P. Rubegni, F. Cambazard, E. Cinotti




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Dermatomyosite amyopathique avec anticorps anti-MDA-5, associée à une pemphigoïde bulleuse, un syndrome de Sjögren et un lymphome de type MALT

Publication date: Available online 28 June 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): B. Garcia, F. Dabouz, L. Pascal, M. Gillard, P. Modiano
IntroductionLes myosites inflammatoires sont un groupe hétérogène de maladies musculaires. Elles comprennent les polymyosites, les dermatomyosites (DM), les myosites associées aux cancers, les myosites nécrosantes et les myosites à inclusions. Les DM ne comportent parfois que peu ou pas de manifestations musculaires : DM hypo- ou amyopathiques. La dermatomyosite (DM) à anticorps anti-MDA5 est une forme de dermatomyosite rare, souvent amyopathique, dont le pronostic est principalement lié à l'atteinte pulmonaire.ObservationUne femme de 69 ans, suivie pour un lymphome gastrique de type mucosa-associated lymphoid tissue (MALT), était adressée pour une éruption bulleuse. Les investigations réalisées permettaient de retenir le diagnostic de pemphigoïde bulleuse. Parallèlement était découverte une dermatomyosite amyopathique avec pneumopathie interstitielle. Une corticothérapie générale était débutée en association avec le rituximab indiqué pour son lymphome. L'évolution était favorable.DiscussionLa dermatomyosite à anticorps anti-MDA5 doit être évoquée devant toute atteinte pulmonaire associée à des signes cutanés de dermatomyosite, le plus souvent en l'absence d'atteinte musculaire. Elle représenterait jusqu'à 7 % des DM et son pronostic est sévère en raison de l'atteinte pulmonaire.BackgroundThe inflammatory myopathies are a heterogeneous group of muscle diseases and comprise polymyositis, dermatomyositis (DM), myopathies associated with cancers, necrotising myositis and inclusion body myositis. DM occasionally exhibits few or no muscular signs: i.e. hypomyopathic/amyopathic DM. Anti-MDA5 dermatomyositis (DM) is a rare form of dermatomyositis that is frequently amyopathic; the prognosis is linked mainly to pulmonary involvement.Patients and methodsA 69-year-old woman treated for mucosa-associated lymphoid tissue (MALT) gastric lymphoma was referred for a bullous eruption. Based on the investigations performed, a diagnosis was made of bullous pemphigoid. At the same time, amyopathic dermatomyositis was discovered together with interstitial lung disease. Systemic steroids were introduced in combination with rituximab. A favourable outcome was achieved.DiscussionAnti-MDA5 dermatomyositis must be considered systematically in all cases of pulmonary involvement associated with cutaneous signs of dermatomyositis, in which no muscular involvement is generally seen. This condition accounts for up to 7% of DM and carries a severe prognosis due to pulmonary involvement.



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Effectiveness of montelukast in overweight and obese atopic asthmatics

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Publication date: Available online 28 June 2017
Source:Annals of Allergy, Asthma & Immunology
Author(s): Sherry Farzan, Sundas Khan, Claudia Elera, James Tsang, Meredith Akerman, James DeVoti




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Table of Contents

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Publication date: July 2017
Source:Annals of Allergy, Asthma & Immunology, Volume 119, Issue 1





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Instructions for Authors

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Publication date: July 2017
Source:Annals of Allergy, Asthma & Immunology, Volume 119, Issue 1





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Vitamin D and asthma

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Publication date: July 2017
Source:Annals of Allergy, Asthma & Immunology, Volume 119, Issue 1
Author(s): Yong Mao, Yiqiang Zhan, Yixiang Huang




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Editorial Board

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Publication date: July 2017
Source:Annals of Allergy, Asthma & Immunology, Volume 119, Issue 1





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Mammalian raw materials used to produce allergen extracts

Publication date: July 2017
Source:Annals of Allergy, Asthma & Immunology, Volume 119, Issue 1
Author(s): Enrique Fernández-Caldas, Bárbara Cases, David El-Qutob, Jose Fernando Cantillo
ObjectiveTo provide information about the complexity of skin-derived mammalian allergen extracts and recent advances made in their characterization and production.Data SourcesOriginal and review articles (involving nonfood allergy to mammals) published in indexed journals were searched in the PubMed database.Study SelectionsStudies were selected with the following criteria: novelty, species of the study, and date of publication.ResultsThe information provided will help in the understanding and the selection of the appropriate allergen source materials for the preparation of extracts for the diagnosis and treatment of allergic respiratory diseases induced by the inhalation of skin-derived mammalian allergens. The data presented herein suggest the presence of cross-reactive and species-specific allergens in extracts prepared from different mammalian dander. Dander should be strongly considered in the preparation of allergenic extracts not only of cats and dogs but also of other mammalian species.ConclusionNew methods should be developed to estimate the relative quantities of specific allergens in the extracts. The current knowledge illustrates the complexity of these extracts, and more efforts should be undertaken to fully understand the wide spectrum of mammalian allergens.



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Desensitization to protein kinase inhibitors

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Publication date: July 2017
Source:Annals of Allergy, Asthma & Immunology, Volume 119, Issue 1
Author(s): Kelly A. Chillari, Sara R. Britnell, Jamie N. Brown, Julia M. Hammond




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Information for Readers

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Publication date: July 2017
Source:Annals of Allergy, Asthma & Immunology, Volume 119, Issue 1





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Point-of-care blood eosinophil count in a severe asthma clinic setting

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Publication date: July 2017
Source:Annals of Allergy, Asthma & Immunology, Volume 119, Issue 1
Author(s): Enrico Heffler, Giovanni Terranova, Carlo Chessari, Valentina Frazzetto, Claudia Crimi, Silvia Fichera, Giuseppe Picardi, Giuliana Nicolosi, Morena Porto, Rossella Intravaia, Nunzio Crimi
BackgroundOne of the main severe asthma phenotypes is severe eosinophilic or eosinophilic refractory asthma for which novel biologic agents are emerging as therapeutic options. In this context, blood eosinophil counts are one of the most reliable biomarkers.ObjectiveTo evaluate the performance of a point-of-care peripheral blood counter in a patients with severe asthma.MethodsThe blood eosinophil counts of 76 patients with severe asthma were evaluated by point-of-care and standard analyzers.ResultsA significant correlation between blood eosinophils assessed by the 2 devices was found (R2 = 0.854, P < .001); similar correlations were found also for white blood cells, neutrophils, and lymphocytes. The point-of-care device had the ability to predict blood eosinophil cutoffs used to select patients for biologic treatments for severe eosinophilic asthma and the ELEN index, a composite score useful to predict sputum eosinophilia.ConclusionThe results of our study contribute to the validation of a point-of-care device to assess blood eosinophils and open the possibility of using this device for the management of severe asthma management.



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Associations of self-reported allergic diseases and musculoskeletal problems in children

Publication date: Available online 28 June 2017
Source:Annals of Allergy, Asthma & Immunology
Author(s): Benjamin J. Barrick, Somya Jalan, Megha M. Tollefson, Todd A. Milbrandt, A. Noelle Larson, Matthew A. Rank, Christine M. Lohse, Dawn Marie R. Davis
BackgroundPrevious studies have found increased rates of musculoskeletal problems in adults with allergic disease, but whether this association holds true for children is unknown.ObjectiveTo investigate the association of bone, joint, and muscle problems in children with a history of allergic disease.MethodsData were obtained from the 2007 Child and Adolescent Health Measurement Initiative. Univariable and multivariable logistic regression models accounting for the sampling design were used to evaluate associations of bone, joint, and muscle problems with allergic diseases, such as asthma, hay fever, food allergies, and eczema. Associations were summarized with odds ratios (ORs) and 95% confidence intervals (CIs).ResultsThe survey included 91,642 individuals aged 0 to 17 years. Multivariable modeling found statistically significant associations between the number of allergic diseases and bone, joint, and muscle problems (1 allergic disease: adjusted OR, 1.28; 95% CI, 1.04–1.56; P = .02; 2 allergic diseases: adjusted OR, 2.55; 95% CI, 1.92–3.39; P < .001; 3 allergic diseases: adjusted OR, 2.70; 95% CI, 1.88–3.86; P < .001; and 4 allergic diseases: adjusted OR, 4.35; 95% CI, 2.46–7.69; P < .001). Severe eczema (but not mild eczema) was associated with bone, joint, and muscle problems (adjusted OR, 2.81; 95% CI, 1.64–4.81; P < .001) and with bone problems (adjusted OR, 6.08; 95% CI, 1.94–19.12; P = .002).ConclusionSelf-reported allergic diseases in children were associated with bone, joint, and muscle problems, and associations strengthened with allergic disease severity and number of allergic diseases. Severe eczema may be associated with bone problems in children. Bone, joint, and muscle problems must be considered in children with severe allergic disease, and prospective studies are necessary to define this association.



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