Medicine by Sfakianakis Alexandros: 07/17/17

Δευτέρα 17 Ιουλίου 2017

Resectable adenocarcinoma developing in the remnant pancreas 7 years after partial pancreatoduodenectomy for invasive ductal adenocarcinoma of the pancreas: a case report

Pancreatic adenocarcinoma still has an excessively high mortality rate and resection is the only potentially curative treatment. The postoperative 5-year survival rate is approximately 20% and recurrence devel...

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Granulomatous reaction to hyaluronic acid filler material in oral and perioral region: A case report and review of literature

Summary

The use of cosmetic fillers agents in orofacial region has become more often used for esthetic concern. Although adverse effects are rare, some patients may develop foreign body reaction to such fillers. Hyaluronic acid (HA) is a biomaterial in the spotlight, because it is normally present in several tissues of human body. The aim of this study was to report a case of a 54-year-old white woman with granulomatous reaction to the HA located in the lips. In addition, a review of the English-language literature of all previously described cases of this condition in oral and perioral region was performed. The location, clinical features, symptoms, time between injection and reaction, type of treatment and treatment outcome of 17 cases were summarized. The clinical and histopathological examination along with a detailed history about this condition is very important to management of patients with nodular lesions in maxillofacial region.

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New imaging tools for an old disease: Secondary syphilis

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When is a sentinel node biopsy indicated for patients with primary melanoma? An update of the ‘Australian guidelines for the management of cutaneous melanoma’

Abstract

A sentinel lymph node biopsy is a surgical staging procedure performed for patients with primary cutaneous melanoma who are clinically lymph-node negative to determine whether there is low volume nodal metastasis in the draining lymph node field. A systematic review was recently performed to update the Australian clinical practice guidelines for the diagnosis and management of melanoma, addressing the question, 'When is a sentinel lymph node biopsy indicated?' This article discusses the findings of the systematic review and the evidence base for the updated guidelines.

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Plaque psoriasis is associated with subclinical atherosclerosis in a Chinese population

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Intermittent administration of parathyroid hormone ameliorated alveolar bone loss in experimental periodontitis in streptozotocin-induced diabetic rats

Publication date: November 2017
Source:Archives of Oral Biology, Volume 83
Author(s): Helin Chen, Taozi Fu, Yuanyuan Ma, Xiangnan Wu, Xianxian Li, Xinyi Li, Jiefei Shen, Hang Wang
ObjectiveIntermittent administration of parathyroid hormone (PTH) has been demonstrated to have anabolic effects on bone metabolism and is approved for use in the treatment of osteoporosis. This study evaluates the role of intermittent PTH administration on alveolar bone loss in streptozotocin (STZ)-induced diabetic rats.DesignFifty male Sprague Dawley rats were randomly divided into the following five groups: (1) a control group (saline placebo without ligature and STZ injection), (2) a PTH group (PTH administration without ligature and STZ injection), (3) an L group (saline placebo with ligature), (4) an L+STZ group (saline placebo with ligature and STZ injection), and (5) an L+STZ+PTH group (PTH administration with ligature and STZ injection). PTH was administered at 75μg/kg per dose four times a week for 28days. Subsequently, all rats were sacrificed, and their mandibles were extracted for micro-computed tomography (micro-CT) scanning, as well as histological and immunochemical evaluation.ResultsMicro-CT scanning demonstrated the anabolic effect of PTH on alveolar bone metabolism in STZ-induced diabetic rats (P<0.05), and histomorphometry indicated that PTH inhibited inflammation of the periodontium and increased the level of osteoblastic activity (P<0.05). Immunochemical evaluation showed that rats subjected to both ligature placement and STZ injection had the highest receptor activator of nuclear factor kappa B ligand (RANKL)/osteoprotegerin (OPG) ratio and that PTH administration decreased this ratio.ConclusionIntermittent systemic PTH administration effectively reduced alveolar bone loss and ameliorated the manifestation of experimental periodontitis in STZ-induced diabetic rats.

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Endocannabinoid and Mood Responses to Exercise in Adults with Varying Activity Levels

Acute aerobic exercise improves mood and activates the endocannabinoid (eCB) system in physically active individuals; however, both mood and eCB responses to exercise may vary based on habitual levels of physical activity. Purpose: This study aimed to examine eCB and mood responses to prescribed and preferred exercises among individuals with low, moderate, and high levels of physical activity. Methods: Thirty-six healthy adults (21 ± 4 yr) were recruited from low (≤60 min moderate–vigorous physical activity [MVPA] per week), moderate (150–299 min MVPA per week), and high (≥300 MVPA per week) physical activity groups. Participants performed both prescribed (approximately 70%–75% max) and preferred (i.e., self-selected) aerobic exercise on separate days. Mood states and eCB concentrations were assessed before and after exercise conditions. Results: Both preferred and prescribed exercise resulted in significant increases (P

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Variable Accuracy of Wearable Heart Rate Monitors during Aerobic Exercise

Purpose: Athletes and members of the public increasingly rely on wearable HR monitors to guide physical activity and training. The accuracy of newer, optically based monitors is unconfirmed. We sought to assess the accuracy of five optically based HR monitors during various types of aerobic exercise. Methods: Fifty healthy adult volunteers (mean ± SD age = 38 ± 12 yr, 54% female) completed exercise protocols on a treadmill, a stationary bicycle, and an elliptical trainer (±arm movement). Each participant underwent HR monitoring with an electrocardiogaphic chest strap monitor (Polar H7), forearm monitor (Scosche Rhythm+), and two randomly assigned wrist-worn HR monitors (Apple Watch, Fitbit Blaze, Garmin Forerunner 235, and TomTom Spark Cardio), one on each wrist. For each exercise type, HR was recorded at rest, light, moderate, and vigorous intensity. Agreement between HR measurements was assessed using Lin's concordance correlation coefficient (rc). Results: Across all exercise conditions, the chest strap monitor (Polar H7) had the best agreement with ECG (rc = 0.996) followed by the Apple Watch (rc = 0.92), the TomTom Spark (rc = 0.83), and the Garmin Forerunner (rc = 0.81). Scosche Rhythm+ and Fitbit Blaze were less accurate (rc = 0.75 and rc = 0.67, respectively). On treadmill, all devices performed well (rc = 0.88–0.93) except the Fitbit Blaze (rc = 0.76). While bicycling, only the Garmin, Apple Watch, and Scosche Rhythm+ had acceptable agreement (rc > 0.80). On the elliptical trainer without arm levers, only the Apple Watch was accurate (rc = 0.94). None of the devices was accurate during elliptical trainer use with arm levers (all rc

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Effects of Descending Stair Walking on Health and Fitness of Elderly Obese Women

Purpose: Eccentric exercise training produces positive fitness and health outcomes, but whether this is also the case for descending stair walking (DSW) is unknown. This study investigated the hypothesis that DSW would improve insulin sensitivity, lipid profiles and physical fitness better than ascending stair walking (ASW). Methods: Elderly (≥60 yr) obese women were placed to either DSW or ASW group (n = 15 per group). An elevator was used to eliminate ascending stairs for DSW, and descending stairs for ASW. Descending stair walking and ASW were performed twice a week for 12 wk by increasing the repetitions gradually. Overnight fasting blood samples were taken 3 d before the first training session and 4 d after the last training session, and analyzed for insulin sensitivity and lipid profile markers. Resting HR, systolic blood pressure, and diastolic blood pressure, bone mineral density, knee extensor maximal voluntary isometric contraction strength and several functional physical fitness measures were taken before and after the intervention. Results: Average HR during DSW (88.6 ± 7.8 bpm) was lower (P

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Masthead

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Hatchery Vaccination Against Poultry Viral Diseases: Potential Mechanisms and Limitations

Viral Immunology , Vol. 0, No. 0.

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Tandem amplification of SCCmec can drive high level methicillin resistance in MRSA [PublishAheadOfPrint]

Hospital-associated methicillin-resistant Staphylococcus aureus strains typically express high level, homogenous (HoR) β-lactam resistance, whereas community-associated MRSA (CA-MRSA) more commonly express low level heterogeneous (HeR) resistance. Expression of the HoR phenotype typically requires both increased expression of the mecA gene, carried on the Staphylococcus cassette chromosome SCCmec element, and additional mutational event(s) elsewhere on the chromosome. Here the oxacillin concentration in a chemostat culture of the CA-MRSA strain USA300 was increased from 8 μg/ml to 130 μg/ml over 13 days to isolate highly oxacillin resistant derivatives. A stable, small colony variant, designated HoR34, which had become established in the chemostat culture was found to have acquired mutations in gdpP, clpX, guaA and camS. Closer inspection of the genome sequence data further revealed that reads covering SCCmec were ~10 times over-represented compared to other parts of the chromosome. qPCR confirmed >10-fold higher levels of mecA DNA on the HoR34 chromosome, and MinION genome sequencing verified the presence of 10 tandem repeats of the SCCmec element. qPCR further demonstrated that sub-culture of HoR34 in varying concentrations of oxacillin (0--100 μg/ml) was accompanied by accordion-like contraction and amplification of the SCCmec element. Although slower growing than USA300, HoR34 out-competed the parent strain in the presence of sub-inhibitory oxacillin. These data identify tandem amplification of the SCCmec element as a new mechanism of high-level methicillin resistance in MRSA, which may provide a competitive advantage for MRSA under antibiotic selection.

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Modeling the kinetics of the permeation of antibacterial agents into growing bacteria and its interplay with efflux [PublishAheadOfPrint]

A mathematical model of the passive permeation of a novel solute into bacteria is developed that explicitly accounts for intracellular dilution through growth. A bacterial cell envelope permeability coefficient of >10^-8 cm².s^-1 approx., is predicted to ensure passive permeation into rapidly replicating bacterial cells. The relative importance of the permeability coefficients of the cytoplasmic and outer membranes of Gram-negative bacteria in determining the overall envelope permeability coefficient is analyzed quantitatively. A mathematical description of the balance between passive influx and active efflux is also developed that shows that bacterial expansion through growth can usually be neglected for compounds likely to be prepared in antibacterial drug discovery programs, and the balance between passive inward permeation and active outwardly-directed efflux predominates. A new parameter, efflux efficiency = k/P (where k is the rate coefficient for the efflux pump and P is the permeability coefficient for the membrane across which the pump acts), is introduced and the consequences explored for the efficiency of efflux pumping by a single pump, two pumps in parallel across either the cytoplasmic or outer membrane, and two pumps in series, one across the cytoplasmic membrane and one across the outer membrane of Gram-negative bacteria. The results, showing additive efficiency for two pumps acting across a single membrane, and multiplicative efficiency for two pumps acting in series across the cytoplasmic and outer membranes, can be quantitatively related to the ratios between minimum inhibitory concentrations measured against pump-sufficient and -deletion strains, and agree with previous experimental and theoretical studies.

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Flucloxacillin results in suboptimal voriconazole plasma concentrations [PublishAheadOfPrint]

Combining voriconazole and flucloxacillin is indicated in patient cohorts experiencing both invasive aspergillosis and gram-positive infections (e.g. patients with chronic granulomatous disease or post-influenza pulmonary aspergillosis). We report a highly relevant interaction between voriconazole and flucloxacillin resulting in subtherapeutic voriconazole plasma concentrations in more than 50% of patients, posing a severe threat if not managed properly.

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A Human Biofilm-Disrupting Monoclonal Antibody Potentiates Antibiotic Efficacy in Rodent Models of both Staphylococcus aureus and Acinetobacter baumannii Infections [PublishAheadOfPrint]

Many serious bacterial infections are antibiotic-refractory due to biofilm formation. A key structural component of biofilm is extracellular DNA which is stabilized by bacterial proteins, including those from the DNABII family. TRL1068 is a high affinity human monoclonal antibody against a DNABII epitope conserved across both gram-positive and gram-negative bacterial species. In the current study, the efficacy of TRL1068 for disruption of biofilm was demonstrated in vitro in the absence of antibiotics by scanning electron microscopy. In vivo efficacy of this antibody was investigated in a well-characterized catheter-induced aortic valve infective endocarditis model in rats infected with a methicillin-resistant Staphylococcus aureus (MRSA) strain with the ability to form thick biofilm, obtained from the blood of a patient with a persistent clinical infection. Animals were treated with vancomycin alone or in combination with TRL1068. MRSA burdens in cardiac vegetations, and within intracardiac catheters, kidneys, spleen and liver showed a significant reduction in the combination arm vs vancomycin alone (p<0.001). A trend towards mortality reduction was also observed (p=0.09). In parallel, in vivo efficacy of TRL1068 against a multi-drug resistant clinical Acinetobacter baumannii isolate was explored using an established skin and soft tissue catheter-related biofilm infection mouse model. Catheter segments infected with A. baumannii were implanted subcutaneously in mice; animals were treated with imipenem alone or in combination with TRL1068. The combination showed a significant reduction in catheter-adherent bacteria vs. antibiotic alone (p<0.001). TRL1068 shows excellent promise as an adjunct to standard-of-care antibiotics for a broad range of difficult-to-treat bacterial infections.

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IMPACT OF A CARBAPENEM ANTIMICROBIAL STEWARDSHIP PROGRAM ON PATIENT OUTCOMES [PublishAheadOfPrint]

Antimicrobial stewardship programs (ASPs) aim to improve appropriate antimicrobials use. However, concerns of the negative consequences from accepting ASP interventions exist, particularly when de-escalation or discontinuation of broad-spectrum antibiotics are recommended. Hence, we sought to evaluate the impact on clinical outcomes when ASP interventions for inappropriate carbapenem use were accepted or rejected by primary providers.

We retrospectively reviewed all carbapenem prescriptions deemed inappropriate according to institutional guidelines with ASP interventions between July 2011 and December 2014. Intervention acceptance and outcomes including carbapenem utilization, length-of-stay, hospitalization charges, 30-day readmission and mortality rates were reviewed. Data was analyzed in groups where physicians accepted all interventions ("Accepted") versus rejected ("Rejected").

A total of 158 ASP interventions were made. These included: carbapenem discontinuation (35%), change to narrower-spectrum antibiotic (32%), dose optimization (17%), further investigations (including imaging, procalcitonin) (11%), Infectious Diseases referral (3%), antibiotic discontinuation (other than carbapenem) (1%) and source control (1%).

Of 220 unique patients, carbapenem use was inappropriate in 101 (45.9%). Significant reduction in carbapenem utilization was observed in the "Accepted" group versus "Rejected" group (median defined daily doses: 0.224 versus 0.668 per 1000 patient-days, p<0.001). There was a significant reduction in 30-day mortality in "Accepted" (none) versus "Rejected" group (10 deaths, p=0.015) but there was no difference in length-of-stay, hospitalization charge and 30-day readmission rates. Hypotension was independently associated with mortality in multivariate analysis (odds ratio, 5.25, 95% confidence interval, 1.34-20.6).

In our institution, acceptance of carbapenem ASP interventions did not compromise patient safety in terms of clinical outcomes while reducing consumption.

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Activation of STING in hepatocytes suppresses the replication of hepatitis B virus [PublishAheadOfPrint]

Induction of interferon and proinflammatory cytokines is a hallmark of the infection of many different viruses. However, hepatitis B virus (HBV) does not elicit a detectable cytokine response in infected hepatocytes. In order to investigate the molecular mechanism underlying the innate immune evasion, a functional cGAS-STING pathway was reconstituted in a human hepatoma cell line supporting tetracycline-inducible HBV replication. It was demonstrated that induction of HBV replication neither activated nor inhibited this cytosolic DNA sensing pathway. However, human hepatoma cells as well as immortalized mouse hepatocytes express low levels of STING, which upon activation by cGAMP, the natural ligand of STING, led to induction of a proinflammatory cytokine response. Treatment of immortalized mouse hepatocytes supporting HBV replication with either cGAMP or a small molecule pharmacologic STING agonist significantly reduced viral DNA in a STING- and Janus kinase 1 (JAK1)-dependent manner. Moreover, cGAMP treatment was able to induce inflammatory cytokine gene expression and inhibit the transcription of covalently closed circular (ccc) DNA in HBV-infected human hepatoma cells expressing sodium-taurocholate cotransporting polypeptide (NTCP), an essential receptor for HBV infection of hepatocytes. The studies reported here and previously (Guo, F., et al., Antimicrob Agents Chemother, 59:1273--1281) thus support the notion that pharmacological activation of STING in macrophages and hepatocytes induces host innate responses that can efficiently control HBV replication. Hence, despite not playing a significant role in host innate immune response to HBV infection of hepatocytes, STING is potentially a valuable target for immunotherapy of chronic hepatitis B.

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Transmission of resistant Gram-negative bacteria to Healthcare Worker Gowns and Gloves during Care of Nursing Home Residents in VA Community Living Centers [PublishAheadOfPrint]

Objective: To estimate the risk of transmission of antibiotic resistant Gram-negative bacteria (RGNB) to gowns and gloves (G&G) worn by healthcare workers (HCWs) when providing care to nursing home residents and to identify types of care and resident characteristics associated with transmission.

Methods: A multi-center, prospective observational study was conducted enrolling residents and HCWs from VA nursing homes. Perianal swabs to detect RGNB were collected from residents. HCWs wore G&G during usual care activities and at the end of the interaction the G&G were swabbed in a standardized manner. Transmission of RGNB from a colonized resident to G&G by type of care was measured. Odds ratios (OR) associated with type of care or resident characteristics was estimated.

Results: Fifty-seven of 185 (31%) enrolled residents were colonized with at least one RGNB. RGNB transmission to either HCWs gloves or gowns occurred during 9% of the interactions (n=905). Seven percent of time to only gloves and 2% to gowns. Bathing the resident, as well as providing hygiene and toilet assistance were associated with high-risk of transmission. Glucose monitoring and assistance with feeding or medication were associated with low-risk of transmission. In addition, antibiotic use by the resident was strongly associated with greater transmission (OR 2.51, p<0.01).

Conclusions: RGNB were transferred to HCWs during approximately 9% of visits. High-risk types of care were identified for which use of G&G could be prioritized. Antibiotic use was associated with a 2.5 times greater risk of transmission, emphasizing the importance of antibiotic stewardship.

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NEUROTOXIC CONCENTRATION OF PIPERACILLIN DURING CONTINUOUS INFUSION IN CRITICALLY ILL PATIENTS [PublishAheadOfPrint]

This retrospective cohort study included 53 patients admitted in ICU, of average age 69 years, without neurologic disorder before initiation of piperacillin continuous infusion at standard dose and who underwent piperacillin serum concentration monitoring. Among them, 23 developed neurologic disorder for which the piperacillin causality was chronologically and semiologically suggestive. A concentration threshold of 157.2 mg/L independently predicted neurotoxicity with 96.7% specificity and 52.2% sensitivity and may constitute a limitation when targeting less susceptible pathogens.

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Deletion of sigB Causes Increased Sensitivity to para-Aminosalicylic Acid and Sulfamethoxazole in Mycobacterium tuberculosis [PublishAheadOfPrint]

Although the de novo folate biosynthesis pathway has been well studied in bacteria, little is known about its regulation. In this study, the sigB gene in Mycobacterium tuberculosis (M. tb) was deleted. Subsequent drug susceptibility tests revealed that M. tb sigB was more sensitive to para-aminosalicylic acid (PAS) and sulfamethoxazole (SMX). Comparative transcriptional analysis was performed and down-regulation of pabB was observed in the sigB deleted strain, which was further verified by qRT-PCR and Western blot assay. Then production of para-aminobenzoic acid (pABA) were compared between the sigB deletion mutant and wild type strain, and results showed that sigB deletion resulted in decreased production of pABA. Additionally, SigB was found to be able to recognize the promoter of pabB in vitro. Furthermore, we found that deleting pabC also caused increased susceptibility to PAS. Taken together, our data revealed that, in M. tb, sigB affects susceptibility to antifolates through multiple ways, the most of which is regulating the expression of pabB. To our knowledge, this is the first report showing that SigB modulates pABA biosynthesis and thus affecting susceptibility to antifolates, which broadens our understanding of the regulation of bacterial folate metabolism and mechanisms of susceptibility to antifolates.

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Re: “Thyroglobulin as a Functional Biomarker of Iodine Status in a Cohort Study of Pregnant Women in the United Kingdom” by Bath et al. (Thyroid 2017;27:426–433)

Thyroid , Vol. 0, No. 0.

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A case of acquired epidermodysplasia verruciformis in a renal transplant recipient clearing with multimodal treatment including HPV (Gardasil) vaccination

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Erratum to: The German Lipoprotein Apheresis Registry (GLAR) – almost 5 years on

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BCG — old workhorse, new skills

M Gengenbacher | NE Nieuwenhuizen | SHE Kaufmann

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Non-human primates as a model for understanding the mechanism of action of toll-like receptor-based vaccine adjuvants

Elizabeth A .Thompson | Karin Loré

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Aesthetic outcome and complications of simple interrupted versus running subcuticular sutures in facial surgery: A randomized controlled trial

The suturing technique and its associated complications could affect cosmetic outcome after facial surgery. Literature on this topic is limited.

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Temporal Envelope Coding by Inferior Colliculus Neurons with Cochlear Implant Stimulation

Abstract

Modulations in temporal envelopes are a ubiquitous property of natural sounds and are especially important for hearing with cochlear implants (CIs) because these devices typically discard temporal fine structure information. With few exceptions, neural temporal envelope processing has been studied in both normal hearing (NH) and CI animals using only pure sinusoidal amplitude modulation (SAM) which poorly represents the diversity of envelope shapes contained in natural sounds because it confounds repetition rate and the width of each modulation cycle. Here, we used stimuli that allow independent manipulation of the two parameters to characterize envelope processing by inferior colliculus (IC) neurons in barbiturate-anesthetized cats with CIs. Specifically, the stimuli were amplitude modulated, high rate pulse trains, where the envelope waveform interleaved single cycles ("bursts") of a sinusoid with silent intervals. We found that IC neurons vary widely with respect to the envelope parameters that maximize their firing rates. In general, pure SAM was a relatively ineffective stimulus. The majority of neurons (60 %) preferred a combination of short bursts and low repetition rates (long silent intervals). Others preferred low repetition rates with minimal dependence on envelope width (17 %), while the remainder responded most strongly to brief bursts with lesser sensitivity to repetition rate (23 %). A simple phenomenological model suggests that a combination of inhibitory and intrinsic cellular mechanisms suffices to account for the wide variation in optimal envelope shapes. In contrast to the strong dependence of firing rate on envelope shape, neurons tended to phase lock precisely to the envelope regardless of shape. Most neurons tended to fire specifically near the peak of the modulation cycle, with little phase dispersion within or across neurons. Such consistently precise timing degrades envelope coding compared to NH processing of real-world sounds, because it effectively eliminates spike timing as a cue to envelope shape.

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Temporal Envelope Coding by Inferior Colliculus Neurons with Cochlear Implant Stimulation

Abstract

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The “Dangers” of Chronic Proton Pump Inhibitor Use

Publication date: Available online 17 July 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Rena Yadlapati, Peter J. Kahrilas

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Newcomers in paediatric GI pathology: childhood enteropathies including very early onset monogenic IBD

Abstract

Childhood enteropathies are a group of diseases causing severe chronic (>2–3 weeks) diarrhoea often starting in the first week of life with the potential for fatal complications for the affected infant. Early identification and accurate classification of childhood enteropathies are, therefore, crucial for making treatment decisions to prevent life-threatening complications. Childhood enteropathies are classified into four groups based on the underlying pathology: (i) conditions related to defective digestion, absorption and transport of nutrients and electrolytes; (ii) disorders related to enterocyte differentiation and polarization; (iii) defects of enteroendocrine cell differentiation; and (iv) disorders associated with defective modulation of intestinal immune response. While the intestinal mucosa is usually normal in enteropathies related to congenital transport or enzyme deficiencies, the intestinal biopsy in other disorders may reveal a wide range of abnormalities varying from normal villous architecture to villous atrophy and/or inflammation, or features specific to the underlying disorder including epithelial abnormalities, lipid vacuolization in the enterocytes, absence of plasma cells, lymphangiectasia, microorganisms, and mucosal eosinophilic or histiocytic infiltration. This review intends to provide an update on small intestinal biopsy findings in childhood enteropathies, the "newcomers", including very early onset monogenic inflammatory bowel disease (IBD), in particular, for the practicing pathologist.

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DRESS compliqué d’un syndrome d’activation macrophagique chez un nourrisson traité pour une toxoplasmose congénitale

Publication date: Available online 17 July 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): S. Rioualen, J. Dufau, C. Flatres, P. Lavenant, L. Misery, J.-M. Roué
IntroductionLe syndrome d'hypersensibilité médicamenteuse, ou drug reaction with eosinophilia and systemic symptoms (DRESS), est une affection grave qui peut se compliquer d'un syndrome d'activation macrophagique (SAM) ; cette association est toutefois rarement décrite chez l'enfant.ObservationNous rapportons le cas d'un nourrisson âgé de cinq semaines hospitalisé pour un syndrome d'activation macrophagique manifesté par une fièvre prolongée, une splénomégalie, une bicytopénie, une hypofibrinogénémie, une hyperferritinémie et une élévation des récepteurs solubles de l'IL-2. La présence d'un exanthème cutané diffus, associé à une hyperéosinophilie majeure et une atteinte multiviscérale, a permis d'évoquer l'association avec un DRESS. Les premières manifestations cliniques étaient apparues 3 semaines après l'instauration d'un traitement par sulfadiazine, pyriméthamine et folinoral pour une toxoplasmose congénitale.DiscussionL'exposition à la sulfadiazine reste le principal facteur imputable au déclenchement du DRESS dans ce cas. Il s'agit probablement de l'événement princeps qui s'est secondairement compliqué du SAM, même si dans notre observation le diagnostic a été évoqué dans un deuxième temps. Le jeune âge de l'enfant était un facteur de risque supplémentaire.ConclusionLe clinicien doit être alerté de la possibilité d'une association DRESS-SAM d'apparition précoce, en lien avec un traitement instauré en période néonatale, dès la fin du premier mois de vie, pour éviter un retard de prise en charge préjudiciable au pronostic.BackgroundDrug reaction with eosinophilia and systemic symptoms (DRESS) is a severe disease that may be complicated by hemophagocytic lymphohistiocytosis but this is rarely described in children.Patients and methodsWe report the case of a 5-week old infant hospitalized in a pediatric intensive care unit for hemophagocytic lymphohistiocytosis with prolonged fever, splenomegaly, cytopenia, fibrinogen≤1.5g/L, ferritin≥500μg/L, and soluble IL-2 receptor≥2400U/mL. As a result of the presence of a diffuse skin rash, eosinophilia and multiple organ failure that started three weeks after the initiation of a congenital toxoplasmosis treatment, association with DRESS was suggested.DiscussionExposure to sulfadiazine remains the main factor leading to DRESS in this case. This is probably the trigger event, secondarily complicated by hemophagocytic lymphohistiocytosis, although in our case the diagnosis was made subsequently. The unfortunately poor outcome of this association is probably exacerbated in fragile patients such as young infants.ConclusionClinicians should be aware of the possibility of DRESS of every early onset associated with hemophagocytic lymphohistiocytosis linked to a treatment started during the neonatal period to avoid any delay in care that might adversely affect the prognosis.

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Vitiligo dévéloppé sur une mélanocytose dermique (nævus de Ota) chez une patiente à peau foncée

Publication date: Available online 17 July 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): K.-C. Ahogo, I.-P. Gbery, B. Vagamon, O.-A. Ouattara, K.-A. Kouassi, H.-S. Kourouma, Y.-I. Kouassi
IntroductionLe nævus de Ota est une pathologie rare, observée plus fréquemment chez des personnes d'ascendance asiatique. Il se présente cliniquement comme une hyperpigmentation maculeuse gris-bleu unilatérale du visage. La transformation en mélanome et le glaucome sont les principaux risques. L'apparition de lésions de vitiligo avec poliose au sein d'un nævus de Ota est exceptionnelle.ObservationUne femme de 22 ans, aux antécédents de nævus de Ota, consultait pour une dépigmentation des cils. L'examen physique trouvait une hyperpigmentation en nappe orbitofrontale droite, des macules achromiques ainsi qu'une poliose des cils. Le diagnostic de vililigo developpé sur nævus de Ota était retenu. L'examen ophtalmologique trouvait une hyperpigmentation de la sclérotique. Un suivi régulier en dermatologie et en ophtalmologie était institué.DiscussionLe vitiligo est une affection caractérisée par le développement de lésions dépigmentées secondaires à une disparition mélanocytaire chronique dans l'épiderme et le follicule pileux. Sa survenue sur des nævus congénitaux et sur des mélanomes a déjà été rapportée, mais son apparition au sein d'une mélanocytose dermique est très rare. Depuis la première observation de cette association en 1979, seuls 4 autres cas ont été rapportés. Les mécanismes pathogéniques de cette association sont encore mal élucidés. L'examen histopathologique montre en général une perte des mélanocytes épidermiques, alors que les mélanocytes dermiques ne sont pas affectés. Le vitiligo développé sur mélanocytose dermique résulterait ainsi de différences entre les mélanocytes normaux (épidermiques) et ectopiques (dermiques).ConclusionL'association vitiligo/nævus de Ota est exceptionnelle. Nous en rapportons un nouveau cas sur peau foncée.BackgroundNevus of Ota is a rare disease most frequently found in Asians. It presents clinically as a bluish gray hyperpigmentation of one side of the face. Transformation into melanoma and glaucoma are the main risks. The appearance of vitiligo lesions with poliosis within a nevus of Ota is exceptional.Patients and methodsA 22-year-old female patient with a nevus of Ota consulted for depigmentation of the eyelashes. Physical examination revealed hyperpigmentation in the right orbitofrontal part of her face, achromic macules and eyelash poliosis. A diagnosis was made of vitiligo developing on a nevus of Ota. Ophthalmologic examination showed hyperpigmentation of the sclera. Regular dermatologic and ophthalmologic follow-up was instituted.DiscussionVitiligo is a condition characterized by the development of depigmented lesions secondary to chronic degradation of the melanocytes of the epidermis and the follicles. Its occurrence on congenital nevus and melanoma has already been reported. However, its appearance in dermal melanocytosis is very rare. Since the first observation of this association in 1979, only 4 other cases have been reported. The pathogenic mechanisms of this association are still poorly understood. Histopathological examination generally shows a loss of epidermal melanocytes, especially in the basal layer, while dermal melanocytes remain unaffected. In this context, vitiligo developed on dermal melanosis appears to result from the difference between the properties of normal (epidermal) melanocytes and ectopic (dermal) melanocytes.ConclusionAssociation of vitiligo with nevus of Ota is rare. Herein, we report a new case in a dark-skinned subject.

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Tumeurs à cellules granuleuses multiples chez un enfant atteint d’un syndrome de Noonan compliqué de leucémie myélomonocytaire juvénile

Publication date: Available online 17 July 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): J. Castagna, J. Clerc, A.-S. Dupond, C. Laresche
IntroductionLa tumeur à cellules granuleuses (TCG) est une tumeur rare constituée de cellules de Schwann. Nous rapportons le cas d'un enfant atteint d'un syndrome de Noonan compliqué de leucémie myélomonocytaire juvénile (LMMJ) et qui a développé aussi une forme multiple de TCG. Les mécanismes moléculaires qui pourraient expliquer une telle association sont discutés.ObservationUn garçon de 6 ans atteint de syndrome de Noonan compliqué de LMMJ présentait trois nodules sous-cutanés asymptomatiques du dos, de l'avant-bras et du cou. L'analyse histologique montrait des TCG. Une revue de la littérature montre que sept cas de syndrome de Noonan présentant des TCG ont été décrits, aucun en association à une LMMJ. La mutation du gène PTPN11, via l'hyperactivation du signal Ras intracellulaire, pourrait être responsable du développement des TCG et de la LMMJ chez les enfants souffrant d'un syndrome de Noonan.DiscussionUn examen clinique détaillé est recommandé chez un enfant présentant une TCG, pour rechercher une forme multiple et des signes malformatifs orientant vers un syndrome génétique. Notre observation correspond au premier cas décrit de syndrome de Noonan compliqué de LMMJ associé à des TCG multiples. Cette association soulève une fois de plus l'importance de la voie de signalisation Ras-MAPK dans le développement des tumeurs bénignes ou malignes, solides ou sanguines, associées aux syndromes génétiques.BackgroundGranular cell tumour (GCT) is a rare form of tumour comprising Schwann cells. Herein, we report a case of a child presenting Noonan syndrome complicated by juvenile myelomonocytic leukaemia (JMML) and who also developed a multiple form of GCT. We discussed the molecular mechanisms that might account for this association.Patients and methodsA six-year-old boy with Noonan syndrome complicated by JMML presented three asymptomatic subcutaneous nodules on his back, forearm and neck. Histological analysis revealed GCT. A literature review revealed seven cases of Noonan syndrome presenting GCT, none of which were associated with JMML. Mutation of gene PTPN11, via hyperactivation of intracellular Ras signalling may cause the development of GCT and JMML in children presenting Noonan syndrome.DiscussionDetailed clinical examination is recommended in children presenting GCT to screen for multiple forms and for signs of malformation suggestive of a genetic syndrome. Ours is the first case to be described of Noonan syndrome complicated by JMML associated with multiple GCT. This association once again raises the important question of the role of the Ras-MAPK signalling pathway in the development of benign and malignant tumours of solid organs or blood, associated with genetic syndromes.

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X-Linked Agammaglobulinaemia: Outcomes in the modern era

Publication date: Available online 17 July 2017
Source:Clinical Immunology
Author(s): Ben Shillitoe, Andrew Gennery
Colonel Ogden Bruton reported X-Linked Agammaglobulinaemia in 1952 and treated the child with replacement immunoglobulin therapy. Over 60years later, the treatment for XLA has largely remained unchanged. Replacement immunoglobulin lacks the isotypes IgA and IgM, leading to concerns that patients continue to experience recurrent sinopulmonary tract infections and be at increased risk of bronchiectasis. There is potential hope of earlier diagnosis with newborn screening, and a potential cure for these patients, in the form of gene therapy. However, it is first necessary to evaluate current management and outcomes to aid decisions regarding further research and clinical trials. This article reviews current management and outcomes of XLA, whilst identifying gaps in our knowledge base that may need answering before we proceed with novel diagnostic methods and treatment for XLA.

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Erratum zu: SOP – Darmpassagestörung in der Palliativmedizin

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C-Section: Who Decides and When?

The goal of a delivery is always the same: healthy mom, healthy baby. But who makes that call — and what do they consider? And what are the implications of that decision? Abby and Dr. Eric Strand answer all your questions on this weeks Belly to Baby episode.

The post C-Section: Who Decides and When? appeared first on ChildrensMD.

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A Study of MGD013 in Patients With Unresectable or Metastatic Neoplasms

Conditions: Advanced Solid Tumors; Non-small Cell Lung Cancer; Squamous Cell Carcinoma of the Head and Neck; Small Cell Lung Cancer; Diffuse Large B Cell Lymphoma; Epithelial Ovarian Cancer
Intervention: Biological: MGD013
Sponsor: MacroGenics
Not yet recruiting - verified July 2017

http://ift.tt/2vtYP01

Quantifying Effectiveness of Treatment for Irregular Heartbeat

In a small proof-of-concept study, researchers at Johns Hopkins report a complex mathematical method to measure electrical communications within the heart can successfully predict the effectiveness of catheter ablation, the standard of care treatment for atrial fibrillation, the most common irregular heartbeat disorder. This has the potential to let physicians and patients know immediately following treatment whether it was effective, or whether they'll need to anticipate another procedure in the future.

http://ift.tt/2u1ipkv

Design and implementation of low-power CMOS analogue convolutional decoders using the modified feedback decoding algorithm

Tomatsopoulos, B; (2007) Design and implementation of low-power CMOS analogue convolutional decoders using the modified feedback decoding algorithm. Doctoral thesis, UCL (University College London). Green open access

http://ift.tt/2tjaPVl

Cost-effectiveness of population based BRCA testing with varying Ashkenazi Jewish ancestry

Manchanda, R; Patel, S; Antoniou, AC; Levy-Lahad, E; Turnbull, C; Evans, G; Hopper, J; Manchanda, R; Patel, S; Antoniou, AC; Levy-Lahad, E; Turnbull, C; Evans, G; Hopper, J; Macinnis, RJ; Menon, U; Jacobs, I; Legood, R; - view fewer (2017) Cost-effectiveness of population based BRCA testing with varying Ashkenazi Jewish ancestry. American Journal of Obstetrics and Gynecology 10.1016/j.ajog.2017.06.038 . (In press).

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Design and development of spherical array antennas

De Witte, E; (2007) Design and development of spherical array antennas. Doctoral thesis, UCL (University College London). Green open access

http://ift.tt/2tj6eTa

Pathways to Adulthood Annual Report 2008/09

Schoon, I; (2009) Pathways to Adulthood Annual Report 2008/09. (Pathways to Adulthood Annual Reports ). UCL Institute of Education: London, UK. Green open access

http://ift.tt/2u1z2fZ

Pathways to Adulthood Annual Report 2009/10

Schoon, I; (2010) Pathways to Adulthood Annual Report 2009/10. (Pathways to Adulthood Annual Reports ). UCL Institute of Education: London, UK. Green open access

http://ift.tt/2tixbGy

Therapies targeting DNA and RNA in Huntington's disease

Wild, EJ; Tabrizi, SJ; (2017) Therapies targeting DNA and RNA in Huntington's disease. The Lancet Neurology (In press).

http://ift.tt/2u1yDdw

Pathways to Adulthood Annual Report 2010/11

Schoon, I; (2011) Pathways to Adulthood Annual Report 2010/11. (Pathways to Adulthood Annual Reports ). UCL Institute of Education: London, UK. Green open access

http://ift.tt/2tiBuBU

Pathways to Adulthood Annual Report 2011/12

Schoon, I; (2012) Pathways to Adulthood Annual Report 2011/12. (Pathways to Adulthood Annual Reports ). UCL Institute of Education: London, UK. Green open access

http://ift.tt/2u1HVGr

Pathways to Adulthood Annual Report 2012/13

Schoon, I; (2013) Pathways to Adulthood Annual Report 2012/13. (Pathways to Adulthood Annual Reports ). UCL Institute of Education: London, UK. Green open access

http://ift.tt/2tiJKlb

Cortico-cortical interactions in visual awareness

Silvanto, JT; (2007) Cortico-cortical interactions in visual awareness. Doctoral thesis, UCL (University College London). Green open access

http://ift.tt/2u1mIwa

Pathways to Adulthood Annual Report 2013/14

Schoon, I; (2014) Pathways to Adulthood Annual Report 2013/14. (Pathways to Adulthood Annual Reports ). UCL Institute of Education: London, UK. Green open access

http://ift.tt/2tjdUVt

Pathways to Adulthood Annual Report 2014/15

Schoon, I; (2015) Pathways to Adulthood Annual Report 2014/15. (Pathways to Adulthood Annual Reports ). UCL Institute of Education: London. Green open access

http://ift.tt/2u12enw

Contact, interaction, control: The archaeology of inter-regional relations in late Bronze Age Anatolia

Glatz, C; (2007) Contact, interaction, control: The archaeology of inter-regional relations in late Bronze Age Anatolia. Doctoral thesis, UCL (University College London). Green open access

http://ift.tt/2tiJIK5

Pathways to Adulthood Annual Report 2015/16

Schoon, I; (2016) Pathways to Adulthood Annual Report 2015/16. (Pathways to Adulthood Annual Reports ). UCL Institute of Education: London, UK. Green open access

http://ift.tt/2u1Gmsc

A neutron study of sealed pottery from the grave-goods of Kha and Merit

Andreani, C; Aliotta, F; Arcidiacono, L; Borla, M; Di Martino, D; Facchetti, F; Ferraris, E; Andreani, C; Aliotta, F; Arcidiacono, L; Borla, M; Di Martino, D; Facchetti, F; Ferraris, E; Festa, G; Gorini, G; Kockelmann, W; Keller, J; Malfitana, D; Micieli, D; Minniti, T; Perelli Cippo, E; Ponterio, R; Salvato, G; Senesi, R; Turina, V; Vasi, CS; Greco, C; - view fewer (2017) A neutron study of sealed pottery from the grave-goods of Kha and Merit. Journal of Analytical Atomic Spectrometry , 32 (7) pp. 1342-1347. 10.1039/C7JA00099E .

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Accurate age estimation in small-scale societies

Diekmann, Y; Smith, D; Gerbault, P; Dyble, M; Page, AE; Chaudhary, N; Migliano, AB; Diekmann, Y; Smith, D; Gerbault, P; Dyble, M; Page, AE; Chaudhary, N; Migliano, AB; Thomas, MG; - view fewer (2017) Accurate age estimation in small-scale societies. PNAS - Proceedings of the National Academy of Sciences 10.1073/pnas.1619583114 . (In press). Green open access

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Otolaryngology externships and the match: Productive or futile?

Objectives/Hypothesis

The objectives of this study were to summarize externship experiences among recent graduates and current residents in otolaryngology residency programs and determine whether externships affect the match process.

Study Design

Cross-sectional survey.

Methods

A survey was distributed to otolaryngology residents in allopathic US residency programs and otolaryngology graduates from the past 5 years (2011–2015). There were 2,141 surveys distributed.

Results

There were 654 subjects who responded, for a 30.5% response rate. Most respondents were residents (n = 438, 67%). Of the residents, 85.6% had completed at least one externship compared to 75.9% of graduates (P = .002). The most common reasons for selecting a particular externship were geographic location (74.2%) and program reputation (71.1%), whereas the most common reason for not completing an externship was being advised not to (59.1%). Furthermore, 82.6% of respondents received at least one interview from their externships, 90% went to those interviews, and 89.1% reported that externship experiences affected their rank list. Respondents had a 32.7% match rate to the externship residency program if it was ranked versus a 48.8% match rate if the program was ranked first. Respondents who matched at the externship residency program matched higher on their rank list (P < .001). Of the respondents, 90.7% found externships to be valuable, and 74.5% recommended completing one.

Conclusions

Externships are beneficial because they influence the rank list of applicants and are viewed as valuable experiences. Completing an externship is advisable for the experience, but applicants should weigh the benefits versus the possible risk of being judged more harshly during a month-long rotation.

Level of Evidence

NA Laryngoscope, 2017

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Issue Information

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Announcement

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Immune System Found to Control Eye Tissue Renewal in Zebrafish

Researchers at Johns Hopkins Medicine report evidence that zebrafishes' natural ability to regenerate their eyes' retinal tissue can be accelerated by controlling the fishes' immune systems. Because evolution likely conserved this mechanism of regenerative potential in other animals, the new findings may one day advance efforts to combat degenerative eye disease damage in humans.

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Comparison of two stroke volume variation-based goal-directed fluid therapies for supratentorial brain tumour resection: a randomized controlled trial

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Abstract

Background: The optimal volume status for neurosurgery has yet to be determined. We compared two fluid protocols based on different stroke volume variation (SVV) cut-offs for goal-directed fluid therapy (GDFT) during supratentorial brain tumour resection.Methods: A randomized, single-blind, open-label trial was conducted. Eighty adult patients undergoing elective supratentorial brain tumour resection were randomly divided into a low SVV and a high SVV group. The SVV cut-offs were used to determine when to initiate colloid infusion. Clinical outcomes and perioperative changes in serum neuronal biomarkers, including S100β, neurone-specific enolase (NSE) and glial fibrillary acidic protein (GFAP), were compared.Results: Patients in the low SVV group received a higher volume of colloid [869 (SD 404) vs 569 (453) ml; P=0.0025], had a higher urine output [3.4 (2.4) vs 2.5 (1.7) ml kg^–1 h^–1; P=0.0416] and a higher average cardiac index [3.2 (0.7) vs 2.8 (0.6) litres min^–1 m^–2; P=0.0204]. Patients in the low SVV group also had a shorter intensive care unit stay [1.4 (0.7) vs 2.6 (3.3) days, P=0.0326], fewer postoperative neurological events (17.5 vs 40%, P=0.0469), attenuated changes in the NSE and GFAP levels, lower intraoperative serum lactate and a higher Barthel index at discharge (all P<0.05).Conclusions: During GDFT for supratentorial brain tumour resection, fluid boluses targeting a lower SVV are more beneficial than a restrictive protocol.Clinical trial registration. NCT02113358.

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What do recent human studies tell us about the association between anaesthesia in young children and neurodevelopmental outcomes?

Abstract

Abstract

Background: Postgraduate specialty training has traditionally been based on a time- and rotation-based model, but competency-based models are emerging. Because anaesthesia training evolves differently across Europe, variations in assessment and certification processes are expected, but the extent of similarities and differences is unknown. The aim of this study was to compare anaesthesia training programmes in Europe, focusing on assessment and certification processes.Methods: We performed an online survey among national representatives of the Union of European Medical Specialists/European Board of Anaesthesiology. Results: All 36 countries participated. Duration of training had a median of 5 yr (range 2.75–7). Mean number of different assessment tools was 7.45 (range 4–13), with more tools being used in competency-based programmes [mean 9.1 (sd 2.97) vs 7.0 (sd 1.97); P=0.03]. Most countries had a nationally uniform certification process. Based on a qualitative analysis of the survey findings, a categorization of countries emerged, reflecting the approach to assessment and certification. We observed two main streams of countries with an underlying knowledge or procedural focus within a time- and rotation-based apprenticeship model. These main streams are evolving, to different extents, towards a third orientation, competency-based training.Conclusions: Assessment and certification processes in European anaesthesia training are diverse. In many countries, a time-based apprenticeship model is evolving towards a competency-based certification process. This diversity precludes comparison of competence of graduating anaesthetists across Europe.

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Acute rehabilitation after resuscitative endovascular balloon occlusion of the aorta (REBOA) in major trauma

We report a 23-year-old woman admitted post cyclist versus heavy goods vehicle accident in December 2014. This was the second case the life-saving procedure, that is, resuscitative endovascular balloon occlusion of the aorta (REBOA) was performed on at the roadside. This advanced procedure was performed due to the extensive haemorrhage from this patient's complex pelvic fracture . As a result of REBOA, the patient consequently lost her left lower limb and underwent a variety of complex pelvic and lower limb surgeries.

The patient was admitted to the acute critical care unit and underwent repeated operations and was not ready to start active rehabilitation until 12 days into her admission. Prior to this she was on movement restrictions and received physiotherapy for limb care and dietetics in order to meet her nutritional requirements. The patient was stepped down to a ward setting and started on an extensive physiotherapy programme and was then transferred to the rehabilitation unit for amputees at Roehampton.

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Uncommon cause of cervicobrachial neuralgia: epidural abscess complicating tuberculous arthritis

Description

A 59-year-old patient was admitted to the rheumatology department with cervicobrachial neuralgia in the upper extremities and paresthesia. The symptoms appeared 3 months ago.

Physical examination showed neck stiffness with decreased sensitivity in the territory of the C4 root in both arms. There was no motor weakness or fever. The inflammatory blood markers were increased.

Spinal MRI was performed showing posterior epidural collection hyperintense in T2-weighted images (figure 1) with intense peripheral enhancement extending from C3-C4 to C4-C5 (figure 2). A facet joint arthritis at the level of C3-C4 was also noticed with a T2 hyperintense lesion of the spinal cord (figure 1).

Figure 1

T2-weighted sagittal and axis images showing posterior epidural mass with mass effect on the spinal cord at C3-C4 and medullar oedema.

Figure 2

T1FAT SAT post-gadolinium axial images showing intense...

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Customised crescentic corneal transplant with conjunctival resection in an atypical case of malignant Moorens ulcer

Description

Mooren's ulcer is a painful peripheral ulcerative keratitis of unknown aetiology. It is rapidly progressive inflammatory destruction of the juxtalimbal corneal stroma with no associated scleritis. Mooren's ulcer is a diagnosis of exclusion, after ruling out all the relevant systemic and local causes that lead to peripheral ulcerative keratitis. It usually present as bilateral disease with male to female distribution of 1:0.74 being more common in older age group.1 Mooren's ulcer has been seen to be associated with human leucocyte antigen (HLA)-DR17(3) and DQ2.2

A 20-year-old male patient presented with complaints of sudden onset pain, redness and watering in the left eye. History revealed recurrent episodes of similar complaints for the last 2 years. On examination, the visual acuity in the left eye was counting finger at 2 m and 20/20 in the right eye. The slit-lamp biomicroscopic examination showed juxtalimbal severe congestion associated with 2x2 mm of...

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Social considerations of inflammatory bowel disease in Southern Israel

Chronic diseases pose unique social challenges beyond traditional health considerations that require specific attention. In this report, we examine the case of a middle-aged woman with ulcerative colitis, living in Southern Israel. Trust between the patient and physician is shown to positively influence a variety of therapeutic outcomes and should be considered a fundamental component of successful care. In context of the military conflict between Israel and Gaza, the needs of patients with chronic diseases cannot be forgotten. The work environment is also identified as an area of particular concern, as a supportive work environment is essential in order to maintain satisfaction in the workplace and sustain a high quality of life. Out-of-pocket costs for medications are confirmed to be a significant barrier to adherence. Better understanding of patientsâ™ financial capabilities, along with affordable therapeutic interventions, will alleviate healthcare-related financial burdens and improve health outcomes.

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Increased risk for thionamide-induced agranulocytosis in elderly patients: a case presentation and literature review

Thionamides, such as methimazole and propylthiouracil, are used for the management of hyperthyroidism. Agranulocytosis is a rare adverse effect of thionamides and elderly patients are especially vulnerable. Here we discuss a case of an 80-year-old woman who developed agranulocytosis and pneumonia approximately 4 weeks after starting low dose methimazole therapy. Despite aggressive treatment with broad-spectrum antibiotics and granulocyte colony stimulating factor, she developed multiorgan failure and died. Our goals are to identify risk factors common to elderly patients and hopefully improve outcomes in this population when prescribed thionamides.

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Thrombotic microangiopathy associated with intravenous injection of extended-release oxycodone

We describe the case of a 35-year-old man presenting with thrombotic microangiopathy (TMA) and renal impairment following, as he later disclosed, intravenous injection of oral formulation tamper-resistant extended-release oxycodone hydrochloride (Oxycontin). Recurrent misuse of this agent was associated with relapsing TMA despite treatment with terminal complement inhibitor eculizumab. Cases of TMA have been reported in the USA in association with intravenous misuse of extended-release oxymorphone (Opana ER) after the introduction of a new non-crushable formulation in 2012. There are two reported accounts of TMA associated with tamper-resistant Oxycontin, which became available in Australia in 2014. This is the first documented case in which eculizumab was used. This case illustrates the practical diagnostic challenges in identifying TMA disorders, and the importance of a detailed drug history. It also highlights the need to clarify what role, if any, eculizumab therapy has in cases of drug-associated TMA.

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Chewing-induced facial dystonia: a rare presentation of task-specific dystonia

This case is an addition to scarce literature available for a rare condition, chewing-induced task-specific dystonia. The patient was a 63-year-old woman who presented with a 4-year history of progressive difficulty in eating food only during chewing associated with abnormal facial grimaces without any difficulty in drinking, swallowing, speaking or singing. Examination revealed dystonia of facial muscles every time she chewed but absent during drinking and speaking. As movements were consistent and reproducible with the specific task, other differential diagnosis like motor tics, psychogenic disorder, tardive dystonia and parkinsonism syndrome were excluded leading to a diagnosis of task-specific facial dystonia triggered by chewing. Treatment was started with trihexyphenidyl and later on tetrabenazine was also added but she got only mild relief of symptoms. As she did not agreed for botulinum toxin therapy, so we continued with the same treatment without much improvement.

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Localised pneumoperitoneum following liver mass biopsy in the setting of post-ERCP pneumobilia

Description

A 72-year-old woman presented with a 1-week history of fatigue, weight loss and painless jaundice. Liver chemistries confirmed cholestasis with hyperbilirubinaemia of 25.6 mg/dL. An abdominal CT scan revealed multifocal liver lesions and dilation of intrahepatic and extrahepatic bile ducts. On endoscopic retrograde cholangiopancreatography (ERCP), a common bile duct stricture was found requiring dilation and insertion of a biliary stent. Cholangiogram demonstrated free bile drainage without contrast medium leakage. The patient then underwent CT-guided biopsy of a left lobe liver mass. On the next day, she complained of constant abdominal pain. She was afebrile and there were no peritoneal signs. Abdominal CT scan demonstrated localised free air in the peritoneal cavity (figure 1). The stent was at the proper position and pneumobilia of the common bile duct and intrahepatic bile ducts was noted. The occurrence of free air in the peritoneal cavity post-ERCP raised significant concerns...

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A rare consequence of very common osteoporotic pubic rami fracture in a patient with myeloproliferative disease

Description

An 80-year-old female with janus kinase 2 positive myeloproliferative disease with stable blood parameters on aspirin and anagrelide admitted to the emergency department after a fall over the kerb complaining of pain limiting mobility. Initial assessment was stable without any external injuries noted. Pelvic X-ray demonstrated a stable pubic rami fracture (figure 1). Later on, her haemoglobin dropped 106 g/dL to 67 g/dL for no apparent cause. She was also noted to have an abdominal fullness and examination revealed a suprapubic mass. CT scan showed a well-defined hypointense collection measuring 85x60x105 mm extending superiorly from the symphysis pubis compressing bladder (figure 2-4). It was compatible with a late development of a haematoma. There was no contrast extravasation suggesting active bleeding. Her platelets and coagulation were normal. She was managed conservatively with blood transfusions but there was a place for angiography if she was...

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Compliance with command hallucinations: The role of the power of the voice, social rank and moral disengagement

Reynolds, N; (2008) Compliance with command hallucinations: The role of the power of the voice, social rank and moral disengagement. Doctoral thesis, UCL (University College London). Green open access

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Clinical applications of pharmacogenetics

Tate, SK; (2007) Clinical applications of pharmacogenetics. Doctoral thesis, UCL (University College London). Green open access

http://ift.tt/2v9iKBY

Engineering commodifiable workers: language, migration and the governmentality of the self

Del Percio, A; (2017) Engineering commodifiable workers: language, migration and the governmentality of the self. Language Policy 10.1007/s10993-017-9436-4 . (In press). Green open access

http://ift.tt/2uAeyhK

Are social inequalities in early childhood smoking initiation explained by exposure to adult smoking? Findings from the UK Millennium Cohort Study

Taylor-Robinson, DC; Wickham, S; Campbell, M; Robinson, J; Pearce, A; Barr, B; (2017) Are social inequalities in early childhood smoking initiation explained by exposure to adult smoking? Findings from the UK Millennium Cohort Study. PLoS ONE , 12 (6) , Article e0178633. 10.1371/journal.pone.0178633 . Green open access

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Clinical and molecular genetics of Fraser syndrome

van Haelst, MM; (2006) Clinical and molecular genetics of Fraser syndrome. Doctoral thesis, UCL (University College London). Green open access

http://ift.tt/2uAcN4b

Loops D, E and G in the Drosophila Dα1 subunit contribute to high neonicotinoid sensitivity of Dα1-chicken β2 nicotinic acetylcholine receptor

Ihara, M; Hikida, M; Matsushita, H; Yamanaka, K; Kishimoto, Y; Kubo, K; Watanabe, S; Ihara, M; Hikida, M; Matsushita, H; Yamanaka, K; Kishimoto, Y; Kubo, K; Watanabe, S; Sakamoto, M; Matsui, K; Yamaguchi, A; Okuhara, D; Furutani, S; Sattelle, DB; Matsuda, K; - view fewer (2017) Loops D, E and G in the Drosophila Dα1 subunit contribute to high neonicotinoid sensitivity of Dα1-chicken β2 nicotinic acetylcholine receptor. British Journal of Pharmacology 10.1111/bph.13914 . (In press).

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A scoring system predicting the clinical course of CLPB defect based on the foetal and neonatal presentation of 31 patients

Pronicka, E; Ropacka-Lesiak, M; Trubicka, J; Pajdowska, M; Linke, M; Ostergaard, E; Saunders, C; Pronicka, E; Ropacka-Lesiak, M; Trubicka, J; Pajdowska, M; Linke, M; Ostergaard, E; Saunders, C; Horsch, S; van Karnebeek, C; Yaplito-Lee, J; Distelmaier, F; Õunap, K; Rahman, S; Castelle, M; Kelleher, J; Baris, S; Iwanicka-Pronicka, K; Steward, CG; Ciara, E; Wortmann, SB; Additional individual contributors, .; - view fewer (2017) A scoring system predicting the clinical course of CLPB defect based on the foetal and neonatal presentation of 31 patients. Journal of Inherited Metabolic Disease 10.1007/s10545-017-0057-z . (In press). Green open access

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Characterisation of two new mouse models of neurodegenerative diseases

Achilli, F; (2007) Characterisation of two new mouse models of neurodegenerative diseases. Doctoral thesis, UCL (University College London). Green open access

http://ift.tt/2v9JmTh

Contribution of a deformable striking-ship structure to the structural crashworthiness of ship-ship collisions

Paik, J; (2017) Contribution of a deformable striking-ship structure to the structural crashworthiness of ship-ship collisions. In: (Proceedings) International Conference on Ships and Offshore Structures. (In press).

http://ift.tt/2uAnNhH

Characterisation of a block to HIV-1 infection in rabbit cells as a model to study HIV-1 trafficking

Moguel, MTC; (2007) Characterisation of a block to HIV-1 infection in rabbit cells as a model to study HIV-1 trafficking. Doctoral thesis, UCL (University College London). Green open access

http://ift.tt/2v9dFJP

Cancer vaccines: using lentiviral vectors to deliver antigens to dendritic cells in vivo

Rowe, H; (2006) Cancer vaccines: using lentiviral vectors to deliver antigens to dendritic cells in vivo. Doctoral thesis, UCL (University College London). Green open access

http://ift.tt/2uA6bCv

Behavioural and neurochemical abnormalities in NK1 receptor knockout (NK1R-/-) mice: a novel rodent model of attention deficit/hyperactivity disorder

Yan, T; (2008) Behavioural and neurochemical abnormalities in NK1 receptor knockout (NK1R-/-) mice: a novel rodent model of attention deficit/hyperactivity disorder. Doctoral thesis, UCL (University College London). Green open access

http://ift.tt/2v9Jke7

Multiple Osteolytic Lesions Causing Hypercalcemia: A Rare Presentation of Acute Lymphoblastic Leukemia

Acute lymphoblastic leukemia is characterized by unchecked proliferation of malignant lymphoblasts which replaces the normal bone marrow culminating in anemia due to red blood cells inadequacy as well as in easy bruising/bleeding secondary to insufficient platelets production. Even the white blood cells which are produced excessively are immature and abnormal. ALL is the most common hematological malignancy in children. Most commonly, patients present with lymphadenopathy, recurrent infections, bleeding, fatigue, and bone pains. Bone pains, often particularly involving long bones, occur in about 21–38% of cases and are due to overcrowding of bone marrow with malignant cells. Vast majority of children with ALL have thrombocytopenia and/or anemia with a normal or mildly elevated white blood cells count with the presence of lymphoblasts on peripheral smear. About 50% of children present with bleeding while about 75% of patients have platelet count 100,000/microL. Visceromegaly is not uncommon but osteolytic lesions and hypercalcemia are rather uncommon. We present a 22-year-old gentleman with generalized fatigue and bone pains without visceromegaly. There was severe hypercalcemia with normal parathyroid levels but multiple osteolytic lesions. Peripheral smear showed anemia without blasts, whereas a bone marrow biopsy revealed > 30% blasts with interspersed CD 10 positive cells.

http://ift.tt/2uujROT

Simplified models: a different perspective on models as mediators

Abstract

We introduce a novel point of view on the "models as mediators" framework in order to emphasize certain important epistemological questions about models in science which have so far been little investigated. To illustrate how this perspective can help answer these kinds of questions, we explore the use of simplified models in high energy physics research beyond the Standard Model. We show in detail how the construction of simplified models is grounded in the need to mitigate pressing epistemic problems concerning the uncertainty inherent in the present theoretical and experimental contexts.

http://ift.tt/2u0GpEF

SASDS for salivary duct stenosis often can improve obstructive salivary symptoms; however, many patients report persistent symptoms after surgery. Partial duct stenoses or distal duct stenoses are associated with the greatest improvements in COSS scores after SASDS.

Level of Evidence

4. Laryngoscope, 2017

http://ift.tt/2twVZFT

Histomorphological Description of the Digestive System of Pebbly Fish, Alestes baremoze (Joannis, 1835)

Histomorphological studies of the digestive system of Alestes baremoze captured from Lake Albert, Uganda, were done using standard procedures. These revealed that A. baremoze has a fleshy-lipped terminal small mouth, large molar, short oesophagus, a three-lobed liver, pouch-like stomach, a nine-fingered caeca, and a long tubular intestine. A stratified squamous epithelium with numerous mucus-secreting cells lined the lips with no taste buds. Stratified squamous epithelia lined the oesophagus in the anterior portion which turned into a columnar epithelium towards the stomach. The lamina propria had numerous tubular glands throughout the entire oesophageal length. The stomach consisted of three distinct regions (cardiac, fundic, and pyloric) with distinguished lamina propria glands. The intestinal mucosa was thrown into villi of varying heights, with the tallest in the anterior part, lined with a simple columnar epithelium with numerous lymphocytes-like infiltrations. Numerous goblet cells appeared in the intestinal lamina epithelialis; these increased uniformly towards the anal opening. The liver was divided into lobules, with a central vein. Hepatocytes were visibly arranged closely, forming irregular cords, and the scattered tubular acinar glands formed the exocrine pancreas (hepatopancreas). Stomach content analysis indicated that the fish eats plankton, mollusks, crustaceans, and insects as the main proportion of its diet.

http://ift.tt/2u0hFw2

Establishing and Prioritising Research Questions for the Prevention, Diagnosis and Treatment of Hair Loss (excluding Alopecia Areata): The Hair Loss Priority Setting Partnership

Summary

Background

Hair and scalp problems are common. Unfortunately, many uncertainties exist around the most effective management and treatments strategies for these disorders.

Objectives

To identify uncertainties in hair loss management, prevention, diagnosis and treatment that are important to both people with hair loss and healthcare professionals.

Methods

A hair loss priority setting partnership was established between patients, their carers and relatives, and healthcare professionals to identify the most important uncertainties in hair loss. The methodology of the James Lind Alliance was followed to ensure a balanced, inclusive and transparent process.

Results

In total 2747 treatment uncertainties were submitted by 912 participants; following exclusions 884 uncertainties relating to hair loss (excluding alopecia areata) were analyzed. Questions were combined into "indicative uncertainties" following a structured format. A series of ranking exercises further reduced this list to a top 25 that was taken to a final prioritization workshop where the top 10 priorities were agreed.

Conclusions

We present the top 10 research priorities for hair loss (excluding alopecia areata) to guide researchers and funding bodies to support studies important to both patients and clinicians.

http://ift.tt/2uzGPow

The first sporadic case of DFNA11 identified by next-generation sequencing

Publication date: September 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 100
Author(s): Yuka Kaneko, Atsuko Nakano, Yukiko Arimoto, Kiyomitsu Nara, Hideki Mutai, Tatsuo Matsunaga
We report the first sporadic case of nonsyndromic autosomal dominant hearing loss (DFNA11). The patient was a 5-year-old boy with moderate bilateral hearing loss. Targeted next-generation sequencing analysis of patient DNA identified a known heterozygous DFNA11 mutation, c.689C > T, in MYO7A, encoding p.Ala230Val. The mutation was not detected in the parents of the patient and is considered to be de novo. This mutation is identical to the one reported previously in an Italian family. Accumulation of mutation data increases the feasibility of identifying autosomal dominant mutations in sporadic sensorineural hearing loss.

http://ift.tt/2u07qrM

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Δευτέρα 17 Ιουλίου 2017

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