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https://www.readbyqxmd.com/read/29340169/autoimmune-retinopathy-a-review
#1
REVIEW
Aristófanes Mendonça Canamary, Walter Yukihiko Takahashi, Juliana Maria Ferraz Sallum
Autoimmune retinopathy (AIR) is a rare and still poorly understood immune-mediated disease that may cause inflammation from circulating autoantibodies against the retina. It may be related to history of autoimmune disease in the patient or in a family member or the presence of neoplastic disease in the individual. The disease may be subdivided into paraneoplastic and non-paraneoplastic AIR. When related to melanoma, it is referred to as MAR, and when related to other cancers, it is called CAR. The exact prevalence of AIR is unknown...
2018: International Journal of Retina and Vitreous
https://www.readbyqxmd.com/read/29337168/aggressive-resection-of-congenital-lumbosacral-lipomas-in-adults-indications-techniques-and-outcomes-in-122-patients
#2
S C Bai, B Z Tao, L K Wang, X G Yu, B N Xu, A J Shang
OBJECTIVE: The authors reviewed the treatment of adult patients with congenital intraspinal lipomas with total/near-total resection and discussed their preoperative characteristics, prognostic factors and surgical outcomes. METHODS: Medical records of 122 adult patients with congenital lumbosacral lipomas undergoing total/near-total resection were systematically analyzed. The cohort was subdivided into three groups depending on symptom onset age: Group 1 (≤5 years, n = 40), Group 2 (>5 years but <18 years, n = 33), and Group 3 (>18 years, n = 49)...
January 11, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29334750/-remission-of-steroid-resistant-stills-disease-treated-with-anakinra-evidenced-by-fdg-pet-ct-examination-case-report
#3
Zdeněk Adam, Jana Skřičková, Milan Krtička, Zdeněk Řehák, Renata Koukalová, Andrea Šprláková, Marta Krejčí, Luděk Pour, Zdenka Adamová, Eva Pourová, Zdeněk Král
After elimination of infectious causes, neoplastic causes and the systemic autoimmune disease of connective tissue, a patient with high fevers over 39 °C was diagnosed with Stills disease. High doses of prednisone led to resolution of symptoms, however after reducing the doses of prednisone to 15 mg, high fevers over 39 °C returned, as well as joint pains. The high doses of prednisone led to decompensation of diabetes mellitus even with 4 daily insulin dosages. Therefore it was proceeded to regular subcutaneous administration of anakinra once a day...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29334520/invited-commentary-evaluation-of-horner-syndrome-in-the-mri-era
#4
Aki Kawasaki
This Invited Commentary discusses the following article: BACKGROUND:: To identify the etiologies of adult Horner syndrome (HS) in the MRI era using a targeted evaluation approach and to assess the value and yield of targeted imaging. METHODS: A retrospective chart review was performed of 200 adult outpatients with HS, confirmed with cocaine eyedrop testing. Patients were divided into subgroups based on the presence or absence of symptoms and those who did or did not receive additional testing with hydroxyamphetamine drops...
January 11, 2018: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/29322632/approaches-to-sample-size-calculation-for-clinical-trials-in-rare-diseases
#5
Frank Miller, Sarah Zohar, Nigel Stallard, Jason Madan, Martin Posch, Siew Wan Hee, Michael Pearce, Mårten Vågerö, Simon Day
We discuss 3 alternative approaches to sample size calculation: traditional sample size calculation based on power to show a statistically significant effect, sample size calculation based on assurance, and sample size based on a decision-theoretic approach. These approaches are compared head-to-head for clinical trial situations in rare diseases. Specifically, we consider 3 case studies of rare diseases (Lyell disease, adult-onset Still disease, and cystic fibrosis) with the aim to plan the sample size for an upcoming clinical trial...
January 10, 2018: Pharmaceutical Statistics
https://www.readbyqxmd.com/read/29313056/generalized-purpura-as-an-atypical-skin-manifestation-of-adult-onset-still-s-disease-in-a-patient-with-beh%C3%A3-et-s-disease
#6
Chika Omigawa, Takashi Hashimoto, Takaaki Hanafusa, Takeshi Namiki, Ken Igawa, Hiroo Yokozeki
No abstract text is available yet for this article.
January 9, 2018: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/29285947/eslicarbazepine-acetate-in-epilepsies-with-focal-and-secondary-generalised-seizures-systematic-review-of-current-evidence
#7
Laurent M Willems, Johann Philipp Zöllner, Esther Paule, Susanne Schubert-Bast, Felix Rosenow, Adam Strzelczyk
Eslicarbazepine acetate (ESL) is a third-generation antiepileptic drug (AED) approved for adjunctive treatment in adults, children, and adolescents with focal-onset seizures. Recently ESL was approved for initial monotherapy in adults. The intention of this article is to review current evidence for ESL and to summarise its pharmacological profile in comparison to other AEDs of the dibenzazepine group. Areas covered: We performed a systematic literature search in electronic databases (MEDLINE database, Cochrane Central Register of Controlled Trials, Excerpta Medica dataBASE) using a combined search strategy including the following keywords: eslicarbazepine, epilepsy and seizure...
December 29, 2017: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/29282446/refractory-adult-onset-still-s-disease
#8
Wahinuddin Sulaiman, Aris Chandran Abdullah, Jerome Tan Tsen Chuen, Shaffie Baba, Norain Karim
It is often a challenge and a dilemma for clinicians encountering patients with pyrexia of unknown origin. Numerous tests performed to determine the underlying cause often give inconclusive results. We present a 52-year-old man with undulating fever for more than 10 months with persistent hyperferritinaemia, and negative immunological and serological markers. Despite corticosteroids, disease modifying anti-rheumatic agents and immune-modulator therapy, he succumbed to the illness. A diagnosis of refractory Adult onset Still's disease complicated by haemophagocytic lymphohistiocytosis was made...
October 25, 2017: Curēus
https://www.readbyqxmd.com/read/29278009/beneficial-use-of-serum-ferritin-and-heme-oxygenase-1-as-biomarkers-in-adult-onset-still-s-disease-a-multicenter-retrospective-study
#9
Yohei Kirino, Yasushi Kawaguchi, Yoshifumi Tada, Hiroshi Tsukamoto, Toshiyuki Ota, Masahiro Iwamoto, Hiroki Takahashi, Kohei Nagasawa, Shuji Takei, Takahiko Horiuchi, Hisae Ichida, Seiji Minota, Atsuhisa Ueda, Akihide Ohta, Yoshiaki Ishigatsubo
Background Heme oxygenase (HO)-1 is a heme degrading enzyme highly expressed in monocyte/macrophage serum levels, which may be promising biomarker for adult-onset Still's disease (AOSD). We here report data on the use of serum ferritin and HO-1 levels in AOSD. Methods Under the Hypercytokinemia Study Group collaboration, we collected sera from a total of 145 AOSD patients. Three independent experts judged whether the patients were definite AOSD depending on the clinical information. These 91 "definite AOSD" patients were further divided into active, remission, and relapse groups...
December 26, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/29275557/-forensic-analysis-of-63-cases-of-non-violent-death-occurred-in-custody
#10
S M Yang, Y B Cheng
OBJECTIVES: To collect cases of non-violent death occurred in custody for analyzing the forensic characteristics and related influencing factors. METHODS: Sixty-three cases of non-violent death among detainees in custody that handled by a forensic science center from 2000 to 2015 were collected. The type, onset season, medical history, clinical manifestation, treatment and duration of related fatal diseases were analyzed retrospectively. RESULTS: The deaths due to diseases in custody were common in male, and with a high incidence in middle aged adults...
October 2017: Fa Yi Xue za Zhi
https://www.readbyqxmd.com/read/29259808/how-is-rating-of-perceived-capacity-related-to-vo2max-and-what-is-vo2max-at-onset-of-training
#11
Christina Gjestvang, Trine Stensrud, Lene A H Haakstad
Objective: To evaluate how rating of perceived capacity (RPC) is related to maximal oxygen uptake (VO2max) and examine VO2max at onset of training in healthy adults. Methods: In total, 125 newly registered fitness centre members, equally men and women, answered the RPC scale and performed a treadmill test for measurement of VO2max. Eligible criteria were <4 weeks of fitness centre membership, physically inactive, ≥18 years and not pregnant. The RPC is a one-page scale (1-20) based on metabolic equivalent tasks, where the individual chooses the most strenuous activity that can be sustained for at least 30 min...
2017: BMJ Open Sport & Exercise Medicine
https://www.readbyqxmd.com/read/29251027/efficacy-and-safety-of-tocilizumab-with-inhibition-of-interleukin-6-in-adult-onset-still-s-disease-a-meta-analysis
#12
Yubo Ma, Meng Wu, Xu Zhang, Qing Xia, Jiajia Yang, Shengqian Xu, Faming Pan
OBJECTIVE: Tocilizumab (TCZ) is the only available biologic inhibiting interliukine-6 presently, and emerging evidences have figured that elevated serum level of interleukine-6 is a crucial link of the pathogenesis of adult-onset Still's disease (AOSD). However, evidence about the efficacy and safety of TCZ in AOSD with strong power is still scarce. Thus, this meta-analysis was conducted to synthetically assess the efficacy and safety of TCZ on AOSD. METHODS: PubMed, Web of Science, Cochrane Library, Chinese National Knowledge Infrastructure and Chinese Biological Medical Literature database were comprehensively searched up to 31 July, 2017 for the pertinent studies...
December 18, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/29249782/-risk-factors-for-post-operative-respiratory-failure-and-respiratory-tract-infections-following-cardiovascular-surgery
#13
Yui Tabuchi, Yosuke Morimoto, Yudai Yano, Yuichi Tawara, Shuntaro Sato, Kazuyoshi Tanigawa, Kiyoyuki Eishi, Ryo Kozu
INTRODUCTION: Respiratory failure and respiratory tract infections are frequently observed as post-operative pulmonary complications, and significantly contribute to morbidity and mortality. However, the risk factors of post-operative respiratory failure and respiratory tract infections are controversial, and how these factors affect on incidence of complications is still unclear. OBJECTIVE: To identify risk factors of post-operative respiratory failure and respiratory tract infections, and evaluate its impact on incidences after cardiovascular surgery...
December 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/29249373/idiopathic-cerebellar-ataxia-idca-diagnostic-criteria-and-clinical-analyses-of-63-japanese-patients
#14
Kunihiro Yoshida, Satoshi Kuwabara, Katsuya Nakamura, Ryuta Abe, Akira Matsushima, Minako Beppu, Yoshitaka Yamanaka, Yuji Takahashi, Hidenao Sasaki, Hidehiro Mizusawa
Cortical cerebellar atrophy (CCA) and multiple system atrophy with predominant cerebellar ataxia (MSA-C) are the two major forms of adult-onset sporadic ataxia. Contrary to MSA-C, there are neither diagnostic criteria nor neuroimaging features pathognomonic for CCA. Therefore, it is assumed that the category of CCA in the Japanese national registry include heterogeneous cerebellar ataxic disorders. To refine this category in more detail, we here used a clinical-based term, "idiopathic cerebellar ataxia (IDCA)", and proposed its diagnostic criteria...
January 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29247988/interleukin-18-biological-properties-and-role-in-disease-pathogenesis
#15
REVIEW
Gilles Kaplanski
Initially described as an interferon (IFN)γ-inducing factor, interleukin (IL)-18 is indeed involved in Th1 and NK cell activation, but also in Th2, IL-17-producing γδ T cells and macrophage activation. IL-18, a member of the IL-1 family, is similar to IL-1β for being processed by caspase 1 to an 18 kDa-biologically active mature form. IL-18 binds to its specific receptor (IL-18Rα, also known as IL-1R7) forming a low affinity ligand chain. This is followed by recruitment of the IL-18Rβ chain. IL-18 then uses the same signaling pathway as IL-1 to activate NF-kB and induce inflammatory mediators such as adhesion molecules, chemokines and Fas ligand...
January 2018: Immunological Reviews
https://www.readbyqxmd.com/read/29246865/chronic-adolescent-morphine-exposure-alters-the-responses-of-lateral-paragigantocellular-neurons-to-acute-morphine-administration-in-adulthood
#16
Hamed Salmanzadeh, Hossein Azizi, S Mohammad Ahmadi Soleimani, Narges Pachenari, Saeed Semnanian
Accumulating evidence support the growing non-medical use of morphine during adolescence. Despite this concern which has recently been addressed in some studies, cellular mechanisms underlying the long-term neurobiological and behavioral effects of opiate exposure during this critical period have still remained largely unexplored. Several reports have proposed that subtle long-lasting neurobiological alterations might be triggered by exposure to opiate derivatives or drugs of abuse particularly when this occurs during a critical phase of brain maturation such as adolescence...
December 12, 2017: Brain Research Bulletin
https://www.readbyqxmd.com/read/29246252/fever-with-lymphadenopathy-kikuchi-fujimoto-disease-a-great-masquerader-a-case-report
#17
Chamara Dalugama, Indika Bandara Gawarammana
BACKGROUND: Kikuchi Fujimoto disease is an uncommon benign condition of necrotizing histiocytic lymphadenitis commonly seen in East Asian and Japanese populations. It commonly presents with fever, cervical lymphadenopathy, and elevated inflammatory markers. Diagnosis of Kikuchi Fujimoto disease is based on histopathological studies of the involved lymph nodes. The presentation of Kikuchi Fujimoto disease can mimic many sinister conditions including lymphoma. Treatment is mainly supportive provided that accurate diagnosis is made and sinister conditions like lymphoma ruled out...
December 16, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29241877/adult-onset-still-s-disease
#18
REVIEW
Javier Narváez
Adult onset Still's disease is a rare systemic condition at the crossroads between auto-inflammatory syndromes and autoimmune diseases, with considerable heterogeneity in terms of clinical presentation, evolution and severity. This article reviews the main advances and lesser known aspects of this entity related to its clinical spectrum (atypical cutaneous lesions, unusual manifestations, macrophage activation syndrome, disease phenotypes), the emerging controversy around its association with delayed malignancy, the search for new biomarkers for its diagnosis, evaluation of prognosis (clinical factors, prognostic indexes and biomarkers to identify patients at risk of severe organ failure or life-threatening complications), and the determinants in the choice of biological treatment...
December 11, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/29239928/aspergillus-pneumonia-in-a-patient-with-adult-onset-still-disease-successfully-treated-with-anakinra
#19
Emre Bilgin, Abdulsamet Erden, Levent Kilic, Alper Sari, Berkan Armagan, Umut Kalyoncu, Ömer Karadag
No abstract text is available yet for this article.
December 12, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29239927/an-adult-patient-with-a-novel-mutation-in-nlrp3-gene-associated-with-cryopyrin-associated-periodic-syndrome-mimicking-adult-onset-still-disease
#20
Vanessa Ocampo, Maria-Clara Ortiz-Sierra, Andrés F Echeverri, Iván Posso-Osorio, Juan-Pablo Suso, Gabriel J Tobón
No abstract text is available yet for this article.
December 12, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
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