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https://www.readbyqxmd.com/read/29411231/analysis-of-chronic-granulomatous-disease-in-the-kavkazi-population-in-israel-reveals-phenotypic-heterogeneity-in-patients-with-the-same-ncf1-mutation-c-579g-a
#1
Baruch Wolach, Ronit Gavrieli, Martin de Boer, Karin van Leeuwen, Ofir Wolach, Galia Grisaru-Soen, Arnon Broides, Amos Etzioni, Raz Somech, Dirk Roos
PURPOSE: Chronic granulomatous disease (CGD) is an innate immune deficiency disorder of phagocytes, resulting from mutations in the components of the NADPH oxidase complex that impair the synthesis of oxygen radicals, thus rendering patients susceptible to recurrent infections and excessive hyperinflammatory responses. The most common autosomal recessive form of CGD is p47phox deficiency, which is often clinically milder than the more common X-linked recessive form. Here, we report data on genetics, clinical and biochemical findings in 17 CGD patients of Kavkazi origin with the nonsense mutation c...
February 6, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29405145/aetiological-study-of-viruses-causing-acute-encephalitis-syndrome-in-north-west-india
#2
Jitendra Kumar Tiwari, Bharti Malhotra, Aradhana Chauhan, Hemant Malhotra, Pratibha Sharma, Farah Deeba, Khushbu Trivedi, Anjenya M Swamy
CONTEXT: Acute encephalitis syndrome (AES) is a serious public health problem, caused mainly by viruses. However, the profile of viruses causing AES in Rajasthan is not well characterised. AIMS: The present study was undertaken to identify the viruses causing AES and develop diagnostic algorithm so as to help in improved diagnosis, treatment, prevention and control. SETTINGS AND DESIGN: The present study is a hospital-based descriptive, observational study...
October 2017: Indian Journal of Medical Microbiology
https://www.readbyqxmd.com/read/29384109/local-anesthetics-allergy-who-should-be-tested
#3
I YIlmaz, S K Özdemir, Ö Aydin, G E Çelik
Objective. To document the test results of patients referred to our clinic for testing with local anesthetics (LAs) in real life conditions and provide data related to the necessity of these tests. Methods. All consecutive subjects who were referred to be evaluated for LA allergy during a two-year follow up were included in the analysis. All subjects underwent skin prick / intradermal tests followed by a subcutaneous provocation test with the LAs tested. Results. A total of 228 subjects were included. The main referral reason was the presence of a history of drug hypersensitivity reaction (DHR) to drugs other than LAs (n = 128; 56%), whereas a history of LA allergy constituted the second most common referral reason (n = 64, 28...
November 22, 2017: European Annals of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29167593/establishing-brdf-calibration-capabilities-through-shortwave-infrared
#4
Georgi T Georgiev, James J Butler, Kurt Thome, Catherine Cooksey, Leibo Ding
Satellite instruments operating in the reflective solar wavelength region require accurate and precise determination of the Bidirectional Reflectance Distribution Functions (BRDFs) of the laboratory and flight diffusers used in their pre-flight and on-orbit calibrations. This paper advances that initial work and presents a comparison of spectral Bidirectional Reflectance Distribution Function (BRDF) and Directional Hemispherical Reflectance (DHR) of Spectralon, a common material for laboratory and on-orbit flight diffusers...
2017: Proceedings of SPIE
https://www.readbyqxmd.com/read/29101222/structural-basis-for-the-interaction-of-diapause-hormone-with-its-receptor-in-the-silkworm-bombyx-mori
#5
Zhangfei Shen, Xue Jiang, Lili Yan, Yu Chen, Weiwei Wang, Ying Shi, Liangen Shi, Dongxiang Liu, Naiming Zhou
Diapause hormone (DH) is a 24-aa amidated neuropeptide that elicits the embryonic diapause of the silkworm, Bombyx mori (Bommo), via sensitive and selective interaction with its receptor, Bommo DH receptor (Bommo-DHR). Previous studies of the structure-activity relationship of Bommo-DH were all based on an in vivo diapause-induction bioassay, which has provided little information on the structure of Bommo-DHR or its iteration with DH. Here, to unveil the interaction of Bommo-DH with its receptor, N-terminally truncated analogs and alanine-scanning mutants of Bommo-DH were chemically synthesized and functionally evaluated by using a Cy5...
November 3, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29063637/phenotypic-prenatal-diagnosis-of-chronic-granulomatous-disease-a-useful-tool-in-the-absence-of-molecular-diagnosis
#6
M Kulkarni, M Gupta, M Madkaikar
Chronic granulomatous disease (CGD) is an inherited immunodeficiency disorder affecting the microbicidal function of the phagocytes. It is characterized by susceptibility to recurrent infections leading to significant morbidity and mortality. Antibacterial and antifungal prophylaxis, though, has significantly reduced the rate and severity of the infections; the breakthrough infections still remain a challenge. Currently, allogenic haematopoietic stem cell transplantation is the only curative option which is very expensive and unavailable for many due to lack of suitable donor...
December 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29058101/dock8-deficiency-presenting-as-an-ipex-like-disorder
#7
Fayhan J Alroqi, Louis-Marie Charbonnier, Sevgi Keles, Fatima Ghandour, Pierre Mouawad, Rami Sabouneh, Reem Mohammed, Abduarahman Almutairi, Janet Chou, Michel J Massaad, Raif S Geha, Zeina Baz, Talal A Chatila
PURPOSE: The dedicator of cytokinesis 8 (DOCK8) deficiency is an autosomal recessive-combined immunodeficiency whose clinical spectra include recurrent infections, autoimmunity, malignancies, elevated serum IgE, eczema, and food allergies. Here, we report on patients with loss of function DOCK8 mutations with profound immune dysregulation suggestive of an immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX)-like disorder. METHODS: Immunophenotyping of lymphocyte subpopulations and analysis of DOCK8 protein expression were evaluated by flow cytometry...
November 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29056660/in-vitro-research-tools-in-the-field-of-human-immediate-drug-hypersensitivity-and-their-present-use-in-small-animal-veterinary-medicine
#8
REVIEW
Lavergne S Lavergne
Drug hypersensitivity reactions (DHR) are immune-mediated idiosyncratic adverse drug events. Type I DHR are often referred to as "immediate" and involve B lymphocyte-secreted IgE that bind to the membrane of basophils and mast cells, inducing their degranulation. This review presents various in vitro tests that were developed in the field of human type I HS and implemented as clinical diagnostic tools in human cases of immediate DHR. The respective strengths and weaknesses of each test will be discussed in parallel of validation data such as specificity and sensitivity whenever available...
December 22, 2016: Veterinary Sciences
https://www.readbyqxmd.com/read/28980485/sympathetic-vascular-response-to-facial-cooling-is-increased-in-flail-phenotypes-of-amyotrophic-lateral-sclerosis
#9
Marcin Tutaj, MaŁgorzata Miller, Barbara Tomik, Aleksandra Golenia, Agnieszka Stanuszek, Katarzyna BŁońska, Agnieszka SŁowik
OBJECTIVE: To assess cardiovascular responses to cold face test (CFT) in patients with classic-onset ALS (bulbar or limb onset, ALS-C) and in patients with flail arm and flail leg phenotypes (FA/FL). METHODS: In 18 ALS-C, eight FA/FL patients and 10 age-matched controls we continuously monitored heart rate (HR), systolic (SBP), diastolic (DBP) and mean blood pressure (MBP) during two-minute baseline and one-minute cold stimulus application. HR and BP responses to CFT were calculated as differences between the peak responses and baseline values (dHR, dSBP, dDBP, dMBP), as percent changes from baseline (dHR%, dSBP%, dDBP%, dMBP%), and also latencies and durations of HR and BP responses were assessed (LatHR, tHR, LatBP, tBP)...
October 5, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28977756/hypersensitivity-reactions-to-lmwhs-different-patterns-of-cross-reactivity-in-three-patients-case-series-and-a-literature-review
#10
Danica Juricic Nahal, Ivana Cegec, Viktorija Erdeljic Turk, Ksenija Makar Ausperger, Iva Kraljickovic, Iveta Simic
Low molecular weight heparins (LMWH) are used for a variety of indications. The most common type of hypersensitivity reactions to LMWHs are delayed-type hypersensitivity reactions (DHR). Immediate-type hypersensitivity reactions (IHR) occur only sporadically. Cross-reactivity of different LMWHs is a common and unpredictable problem. We present two cases of patients who developed DHR to nadroparin and enoxaparin, respectively. The third case presents a patient who developed IHR to nadroparin. Skin tests confirmed the hypersensitivity in all cases...
October 4, 2017: Canadian Journal of Physiology and Pharmacology
https://www.readbyqxmd.com/read/28893093/practical-approach-to-the-treatment-of-nsaid-hypersensitivity
#11
Natalia Blanca-Lopez, Diana Perez-Alzate, Gabriela Canto, Miguel Blanca
Non-steroidal anti-inflammatory drugs (NSAIDs) are the most frequently involved in drug hypersensitivity reactions (DHR). NSAIDs are prescribed for different processes and some NSAIDs can be obtained over the counter. Areas covered: We analyse the practical approaches for managing and treating NSAID-DHR considering the five major groups of entities recognised, divided into two categories: those responding to strong COX-1 inhibitors and possibly weak COX-1 or selective COX-2 inhibitors named cross-intolerant (CI), and those induced by a single drug or drug group with good tolerance to strong COX-1 inhibitors, known as allergic reactions (SR)...
November 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28874245/prosthetic-joint-infection-following-total-hip-arthroplasty-incidence-mortality-and-validation-of-the-diagnosis-in-the-danish-hip-arthroplasty-register
#12
Per Hviid Gundtoft
Prosthetic joint infection (PJI) is a rare, but devastating complication following primary total hip arthroplasty (THA). As PJI is a rare event, large cohorts of patients are required in order to study this complication. National arthroplasty registers offer such large and unselected cohorts, but studies have shown that these registers - used alone - underestimate the incidence of PJI. The aim of this thesis was to estimate the incidence of PJI and the mortality risk following a PJI by combining data from the Danish Hip Arthroplasty Register (DHR), the National Register of Patients (NRP), the Microbiology Databases, the Civil Registration System, the medical records, the Danish National Prescription Registry and the Clinical Biochemistry Databases...
September 2017: Danish Medical Journal
https://www.readbyqxmd.com/read/28814352/characteristics-of-drug-induced-anaphylaxis-in-children-and-adolescents
#13
Ozlem Cavkaytar, Betül Karaatmaca, Pınar Gur Cetinkaya, Saliha Esenboga, Ebru Arik Yilmaz, Umit M Sahiner, Bulent E Sekerel, Ozge Soyer
BACKGROUND: Although data on anaphylaxis in the general population exist for different allergens, there is still lack of detailed etiologic data on drug-induced anaphylaxis (DIA), particularly in children. OBJECTIVE: To define the etiology of DIA, to determine the accuracy of drug-related anaphylaxis histories, along with the severity and culprit drug associations among individuals <18 years old. METHODS: Patients with a history of drug hypersensitivity reaction (DHR) referred to our center between January 2012 and February 2016 were included...
September 1, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28799301/rickettsial-infections-in-goa-not-just-scrub-typhus
#14
Kedareshwar Narvencar, Gurleen Kaur, Savio Rodrigues
BACKGROUND AND OBJECTIVE: Rickettsial infections are an important cause of undifferentiated febrile illness in tropics. While scrub typhus was reported from Goa, other rickettsial infections have not been reported earlier. The present study was planned to identify pattern of rickettsial infections in Goa. MATERIALS AND METHODS: All patients presenting with undiagnosed acute febrile illness were recruited over a two-year period. Other causes of febrile illness were ruled out by appropriate tests...
August 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28743986/an-extended-prospect-advance-in-the-leaf-optical-properties-model-separating-total-chlorophylls-into-chlorophyll-a-and-b
#15
Yao Zhang, Jingfeng Huang, Fumin Wang, George Alan Blackburn, Hankui K Zhang, Xiuzhen Wang, Chuanwen Wei, Kangyu Zhang, Chen Wei
The PROSPECT leaf optical model has, to date, well-separated the effects of total chlorophyll and carotenoids on leaf reflectance and transmittance in the 400-800 nm. Considering variations in chlorophyll a:b ratio with leaf age and physiological stress, a further separation of total plant-based chlorophylls into chlorophyll a and chlorophyll b is necessary for advanced monitoring of plant growth. In this study, we present an extended version of PROSPECT model (hereafter referred to as PROSPECT-MP) that can combine the effects of chlorophyll a, chlorophyll b and carotenoids on leaf directional hemispherical reflectance and transmittance (DHR and DHT) in the 400-800 nm...
July 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28723995/the-alma-ata-decade-the-crisis-of-development-and-international-health
#16
Fernando Antônio Pires-Alves, Marcos Cueto
The paper analyzes formulations in the area of international health at the time of the International Conference on Primary Health Care, held in Alma Ata, Kazakhstan, in 1978. It is set within the context of the broader debate on development and international cooperation throughout the 1970s. Three sets of concepts and prescriptive formulations were examined namely: the New International Economic Order (NIEO), the Dag Hammarskjöld Report (DHR) and the Basic Human Needs Approach (BHN). They were compared with the proposals in documents from the World Health Organization and in the statements from Halfdan Mahler, its director...
July 2017: Ciência & Saúde Coletiva
https://www.readbyqxmd.com/read/28658503/delayed-type-hypersensitivity-reactions-induced-by-proton-pump-inhibitors-a-clinical-and-in-vitro-t-cell-reactivity-study
#17
C-Y Lin, C-W Wang, C-Y R Hui, Y-C Chang, C-H Yang, C-Y Cheng, W-W Chen, W-M Ke, W-H Chung
BACKGROUND: Proton pump inhibitors (PPIs) have been known to induce type I hypersensitivity reactions. However, severe delayed-type hypersensitivity reactions (DHR) induced by PPI, such as Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), or drug rash with eosinophilia and systemic symptoms (DRESS), are rarely reported. We conducted a study of a large series of PPI-related DHR, followed up their tolerability to alternative anti-ulcer agents, and investigated the T-cell reactivity to PPI in PPI-related DHR patients...
June 28, 2017: Allergy
https://www.readbyqxmd.com/read/28623810/platelet-granule-release-is-associated-with-reactive-oxygen-species-generation-during-platelet-storage-a-direct-link-between-platelet-pro-inflammatory-and-oxidation-states
#18
Mehran Ghasemzadeh, Ehteramolsadat Hosseini
BACKGROUND: Upon platelet stimulation with agonists, reactive oxygen species (ROS) generation enhances platelet activation and granule release. Whether ROS generation during platelet storage could be directly correlated with the expression of proinflammatory molecules and granule release has been investigated in this study. MATERIAL AND METHOD: PRP-platelet concentrates were subjected to flowcytometry analysis to assess the expression of platelet activation marker, P-selectin and CD40L during storage...
June 10, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28577521/seven-chronic-granulomatous-disease-cases-in-a-single-center-experience-and-a-review-of-the-literature
#19
Şeyhan Kutluğ, Gülnar Şensoy, Asuman Birinci, Berkay Saraymen, Mustafa Yavuz Köker, Alişan YΙldΙran
BACKGROUND: Chronic granulomatous disease (CGD) is a rare primary immunodeficiency caused by defects in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase enzyme system. This disease causes the disordered functioning of phagocytic cells. It is characterized by life-threatening and/or recurrent infections by bacteria and fungi. CGD has one X-linked recessive subtype (X-CGD) and four autosomal recessive subtypes (AR-CGD), and the differential diagnosis is important in such chronic inflammatory disorders...
June 1, 2017: Asian Pacific Journal of Allergy and Immunology
https://www.readbyqxmd.com/read/28535128/low-and-high-let-ionizing-radiation-induces-delayed-homologous-recombination-that-persists-for-two-weeks-before-resolving
#20
Christopher P Allen, Hirokazu Hirakawa, Nakako Izumi Nakajima, Sophia Moore, Jingyi Nie, Neelam Sharma, Mayumi Sugiura, Yuko Hoki, Ryoko Araki, Masumi Abe, Ryuichi Okayasu, Akira Fujimori, Jac A Nickoloff
Genome instability is a hallmark of cancer cells and dysregulation or defects in DNA repair pathways cause genome instability and are linked to inherited cancer predisposition syndromes. Ionizing radiation can cause immediate effects such as mutation or cell death, observed within hours or a few days after irradiation. Ionizing radiation also induces delayed effects many cell generations after irradiation. Delayed effects include hypermutation, hyper-homologous recombination, chromosome instability and reduced clonogenic survival (delayed death)...
July 2017: Radiation Research
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