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https://www.readbyqxmd.com/read/28915908/advances-in-paediatrics-in-2016-current-practices-and-challenges-in-allergy-autoimmune-diseases-cardiology-endocrinology-gastroenterology-infectious-diseases-neonatology-nephrology-neurology-nutrition-pulmonology
#1
REVIEW
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Silvia Montella, Sergio Bernasconi
This review reports main progresses in various pediatric issues published in Italian Journal of Pediatrics and in international journals in 2016. New insights in clinical features or complications of several disorders may be useful for our better understanding. They comprise severe asthma, changing features of lupus erythematosus from birth to adolescence, celiac disease, functional gastrointestinal disorders, Moebius syndrome, recurrent pneumonia. Risk factors for congenital heart defects, Kawasaki disease have been widely investigated...
September 16, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28913776/interactions-of-antisera-to-different-chlamydia-and-chlamydophila-species-with-the-ribosomal-protein-rps27a-correlate-with-impaired-protein-synthesis-in-a-human-choroid-plexus-papilloma-cell-line
#2
Abdullah Almamy, Christian Schwerk, Horst Schroten, Hiroshi Ishikawa, Abdul Rahman Asif, Bernhard Reuss
Chlamydia trachomatis (CT) and the Chlamydophila species (CS) Chlamydophila pneumoniae (CPn), and Chlamydophila psittaci (CPs) are suggested to induce autoantibodies causative of several human autoimmune disorders like rheumatoid arthritis and systemic lupus erythematosus (SLE). The aim of the present study was therefore to identify cellular protein interaction partners with antisera to CT (α-CT) or CS (α-CS) and to identify functional consequences of such interaction in vitro. As detected with a commercial first trimester human prenatal brain multiprotein array (hEXselect, Engine, Germany), the most frequent interaction partner with both α-CT and α-CS was the ribosomal small subunit protein RPS27a...
September 14, 2017: Immunologic Research
https://www.readbyqxmd.com/read/28913566/adar-rna-editing-in-human-disease-more-to-it-than-meets-the-i
#3
REVIEW
Angela Gallo, Dragana Vukic, David Michalík, Mary A O'Connell, Liam P Keegan
We review the structures and functions of ADARs and their involvements in human diseases. ADAR1 is widely expressed, particularly in the myeloid component of the blood system, and plays a prominent role in promiscuous editing of long dsRNA. Missense mutations that change ADAR1 residues and reduce RNA editing activity cause Aicardi-Goutières Syndrome, a childhood encephalitis and interferonopathy that mimics viral infection and resembles an extreme form of Systemic Lupus Erythmatosus (SLE). In Adar1 mouse mutant models aberrant interferon expression is prevented by eliminating interferon activation signaling from cytoplasmic dsRNA sensors, indicating that unedited cytoplasmic dsRNA drives the immune induction...
September 14, 2017: Human Genetics
https://www.readbyqxmd.com/read/28912980/sporotrichoid-like-spread-of-cutaneous-mycobacterium-chelonae-in-an-immunocompromised-patient
#4
Daria Marley Kemp, Anusha G Govind, Jun Kang, Caroline C Brugger, Young C Kauh
Mycobacterium chelonae is a rapidly growing mycobacterium found in water and soil that can cause local cutaneous infections in immunocompetent hosts but more frequently affects immunocompromised patients. Typically, patients will present with painful subcutaneous nodules of the joints or soft tissues from traumatic inoculation. However, exhibiting a sporotrichoid-like pattern of these nodules is uncommon. Herein, we report a case of sporotrichoid-like distribution of cutaneous Mycobacterium chelonae in a patient with systemic lupus erythematosus on significant immunosuppressive medications...
2017: Case Reports in Dermatological Medicine
https://www.readbyqxmd.com/read/28901731/response-to-combination-of-mycophenolate-mofetil-cyclosporin-a-and-corticosteroid-treatment-in-lupus-nephritis-patients-with-persistent-proteinuria
#5
Nuntana Kasitanon, Pornkamon Boripatkosol, Worawit Louthrenoo
OBJECTIVE: To study the response of lupus nephritis (LN) patients with persistent proteinuria (≥ 1 g/day after ≥ 6 months corticosteroid and single immunosuppressant treatment, or ≥ 3 g/day after ≥ 3 months of corticosteroid and single immunosuppressant treatment) to corticosteroid combined with two immunosuppressants, and to evaluate associated factors of response within 1 year. METHOD: A retrospective study of proteinuria and renal function observed in LN patients with persistent proteinuria after adding a second immunosuppressant at 3, 6, 9 and 12 months...
September 13, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28899799/neutrophil-extracellular-traps-nets-in-autoimmune-diseases-a-comprehensive-review
#6
REVIEW
Keum Hwa Lee, Andreas Kronbichler, David Duck-Young Park, YoungMin Park, Hanwool Moon, Hyungdo Kim, Jun Hyug Choi, YoungSeo Choi, Songjoo Shim, Il Suk Lyu, Byung Hwan Yun, Yeonseung Han, Donghee Lee, Sang Yoon Lee, Byung Hun Yoo, Kyung Hwan Lee, Tai Lim Kim, Heonki Kim, Joo Sung Shim, Wonseok Nam, Heesung So, SooYeon Choi, Sangmok Lee, Jae Il Shin
The structures named neutrophil extracellular traps (NETs) are fibrous networks which protrude from the membrane of activated neutrophils. NETs are found in a variety of conditions, such as infection, malignancy, atherosclerosis, and autoimmune diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), psoriasis, and gout. The impact of NETs on the development mechanisms of autoimmune diseases are proposed to arise from an imbalance between "NETosis" which is a process of NET formation and NET degradation...
September 9, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28887790/polyspecificity-of-anti-lipid-a-antibodies-and-its-relevance-to-the-development-of-autoimmunity
#7
Omid Haji-Ghassemi, Susannah M L Gagnon, Sven Müller-Loennies, Stephen V Evans
The process of natural selection favours germ-line gene segments that encode CDRs that have the ability to recognize a range of structurally related antigens. This presents an immunological advantage to the host, as it can confer protection against a common pathogen and still cope with new or changing antigens. Cross-reactive and polyspecific antibodies also play a central role in autoimmune responses, and a link has been shown to exist between auto-reactive B cells and certain bacterial infections. Bacterial DNA, lipids, and carbohydrates have been implicated in the progression of autoimmune diseases such as systemic lupus erythematosus...
September 9, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28882940/varicella-complicated-by-cellulitis-and-deep-vein-thrombosis
#8
Gracinda Nogueira Oliveira, Susana Basso, Teresa Sevivas, Nelson Neves
We report a 16-month-old girl with varicella complicated by cellulitis, invasive Group A Streptococcus (GAS) infection and deep vein thrombosis. She presented with varicella lesions, fever and a painful firm tumefaction on the right lower leg (RLL). Ultrasound showed a local subcutaneous tissue thickening suggestive of cellulitis and antibiotics were initiated. Further swelling of the RLL motivated a second ultrasound that showed an obstructive thrombus for which she was started on enoxaparin. The blood culture confirmed GAS infection leading to directed antibiotherapy...
September 7, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28874654/agranulocytosis-induced-by-sinomenine-hydrochloride
#9
Juerong Chen, Bing Zhong, Yong Wang
BACKGROUND Sinomenine hydrochloride is an alkaloid that is extracted from the Chinese herbal plant Sinomenium acutum, and is used as a herbal medicine in the treatment or rheumatic disease. This report is the first to describe a case of sinomenine hydrochloride-induced agranulocytosis. CASE REPORT A 44-year-old woman with systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) was treated with sinomenine hydrochloride and developed agranulocytosis with a neutrophil count of 0.01×10^9/L. She subsequently developed an opportunistic bacterial infection...
September 6, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28872084/mortality-trend-of-inpatients-with-connective-tissue-diseases-2005-2014
#10
Liya Li, Xiaoxia Zuo, Hui Luo, Yisha Li, Yunhui You, Liping Duan, Weiru Zhang, Hongjun Zhao, Tong Li, Wangbin Ning, Yanli Xie, Sijia Liu, Xiaoyun Xie, Ying Jiang, Shiyao Wu, Honglin Zhu, Ya'ou Zhou
To analyze the trend relevant factors leading to death and their patterns over a 10-year period in inpatients with connective tissue diseases (CTDs).
 Methods: All clinical data about death in inpatients with CTDs were retrospectively reviewed between 2005 and 2014 at the Department of Rheumatology and Immunology in Xiangya Hospital of Central South University.
 Results: In the 10-year time period, the overall hospital mortality was 15.68‰. The disease itself accounted for 44.71% of the total causes of death, infection accounted for 42...
August 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28868299/tubuloreticular-inclusions-in-the-absence-of-systemic-lupus-erythematosus-and-hiv-infection-a-report-of-three-pediatric-cases
#11
Ayah Elmaghrabi, Elizabeth Brown, Ei Khin, Jared Hassler, Allen R Hendricks
Tubuloreticular inclusions (TRIs) are subcellular structures located within the cisternae of endoplasmic reticulum. Formation of TRIs has been linked to the exposure of excess interferon (IFN), either from endogenous or exogenous sources. In renal disease, TRIs have been most commonly associated with systemic lupus erythematosus (SLE), and human immunodeficiency virus-associated nephropathy (HIVAN). Case reports of patients with renal biopsies showing TRIs without underlying SLE or HIV are infrequent in adults, and to our knowledge none have been reported in children...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28862513/relationship-between-glucocorticoid-dose-and-adverse-events-in-systemic-lupus-erythematosus-data-from-a-randomized-clinical-trial
#12
S Emamikia, C Gentline, K Chatzidionysiou, L Arnaud, R van Vollenhoven
OBJECTIVE: The aim was to obtain a better understanding of the relationship between exposure to glucocorticoids (GCs) and adverse events (AEs) reported in a randomized controlled trial (RCT) in systemic lupus erythematosus (SLE) patients. METHOD: We used data from the BLISS-76 trial on belimumab. The data were accessed through an agreement under the SHARE mechanism. AEs were grouped according to medical relevance, i.e. based on similarity of symptoms and pathophysiology...
September 1, 2017: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/28854632/rapid-evolution-of-primate-type-2-immune-response-factors-linked-to-asthma-susceptibility
#13
Matthew F Barber, Elliott M Lee, Hayden Griffin, Nels C Elde
Host immunity pathways evolve rapidly in response to antagonism by pathogens. Microbial infections can also trigger excessive inflammation that contributes to diverse autoimmune disorders including asthma, lupus, diabetes, and arthritis. Definitive links between immune system evolution and human autoimmune disease remain unclear. Here we provide evidence that several components of the type 2 immune response pathway have been subject to recurrent positive selection in the primate lineage. Notably, substitutions in the central immune regulator IL13 correspond to a polymorphism linked to asthma susceptibility in humans...
June 1, 2017: Genome Biology and Evolution
https://www.readbyqxmd.com/read/28846194/spectrum-of-biopsy-proven-renal-disease-in-south-asian-children-two-decades-at-a-tropical-tertiary-care-centre
#14
Anjali Mohapatra, Shailesh Kakde, Vellaichamy M Annapandian, Anna T Valson, Neelaveni Duhli, Anila Korula, Smita Mary Matthai, Anna B Pulimood, Vinoi G David, Suceena Alexander, Shibu Jacob, Santosh Varughese, Basu Gopal, Tamilarasi Veerasamy, George T John
AIM: We report findings from a large single centre paediatric renal biopsy cohort in South Asia. METHODS: We analyzed all renal biopsies performed on children aged ≤18 years between 1996 and 2015 at our centre. The clinical characteristics and histological diagnosis pertaining to each case, distribution of renal diseases in children with various clinical presentations, and changes in the pattern of kidney disease during the study period were analyzed. RESULTS: A total of 1740 paediatric kidney biopsies were performed during the study period...
August 28, 2017: Nephrology
https://www.readbyqxmd.com/read/28840341/usefulness-of-neutrophil-to-lymphocyte-ratio-as-a-biomarker-for-diagnosing-infections-in-patients-with-systemic-lupus-erythematosus
#15
Hyoun-Ah Kim, Ju-Yang Jung, Chang-Hee Suh
Neutrophil-to-lymphocyte ratio (NLR), monocyte-to-lymphocyte ratio (MLR), and platelet-to-lymphocyte ratio (PLR) have been investigated as disease activity markers for systemic lupus erythematosus (SLE). Hence, we investigated the clinical significance of these parameters in diagnosing infection in patients with SLE. In total, 120 patients with SLE, who were admitted to hospital due to disease flares or infection, were recruited for the study. Of the 120 patients, 60 had a concurrent infection (SLE with infection), while the remaining 60 patients were admitted with a flare without any evidence of infection (SLE with flare)...
August 24, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28840094/the-risk-benefit-ratio-of-glucocorticoids-in-sle-have-things-changed-over-the-past-40-years
#16
G Stojan, M Petri
PURPOSE OF REVIEW: Glucocorticoids have been the mainstay of treatment in systemic lupus erythematosus for more than half a century. Despite advancements in knowledge concerning the pathophysiology of systemic lupus, the genomic/non-genomic actions of glucocorticoids, and the use of novel therapeutic agents in SLE, the burden of toxicity from glucocorticoid use remains unchanged. RECENT FINDINGS: SLE patients receiving long-term prednisone therapy are at significant risk of morbidity due to permanent organ damage and prednisone daily dosages above 6 mg have been shown to increase the risk of future organ damage by 50%...
September 2017: Current Treatment Options in Rheumatology
https://www.readbyqxmd.com/read/28839394/eyelash-trichomegaly-a-rare-presenting-feature-of-systemic-lupus-erythematosus
#17
Ashish Dalal, Sudhanshu Sharma, Ajay Kumar, Nidhi Sharma
An increase in length, curling, pigmentation, or thickness of eyelashes is termed eyelash trichomegaly. It may be inherited as an isolated trait or as a feature of a congenital syndrome such as Oliver-McFarlane syndrome or oculocutaneous albinism. Acquired conditions associated with eyelash trichomegaly include HIV infection, connective tissue disorders, and the administration of drugs such as cyclosporine and topical latanoprost. We hereby report a rare case of acquired eyelash trichomegaly with diffuse hair loss and "lupus hairs" on the scalp in a 16-year-old female diagnosed with systemic lupus erythematosus...
April 2017: International Journal of Trichology
https://www.readbyqxmd.com/read/28837372/update-upon-efficacy-and-safety-of-etanercept-for-the-treatment-of-spondyloarthritis-and-juvenile-idiopathic-arthritis
#18
Giuseppe Murdaca, Simone Negrini, Ottavia Magnani, Elena Penza, Marco Pellecchio, Rossella Gulli, Paola Mandich, Francesco Puppo
TNF-α inhibitors have demonstrated efficacy both as monotherapy and in combination with disease-modifying anti-rheumatic drugs (DMARDs) in the treatment of chronic inflammatory immune-mediated diseases such as rheumatoid arthritis, Crohn's disease, ulcerative colitis, ankylosing spondylitis (AS), psoriasis (Ps) and/or psoriatic arthritis (PsA) and may be administered off-label to treat disseminated granuloma annulare, systemic lupus erythematosus and systemic sclerosis. There are several TNF-α inhibitors available for clinical use including infliximab, adalimumab, golimumab, certolizumab pegol and etanercept...
August 24, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28829212/targeting-methionine-cycle-as-a-potential-therapeutic-strategy-for-immune-disorders
#19
Heng Li, Huimin Lu, Wei Tang, Jianping Zuo
Methionine cycle plays an essential role in regulating many cellular events, especially transmethylation reactions, incorporating the methyl donor S-adenosylmethionine (SAM). The transmethylations and substances involved in the cycle have shown complicated effects and mechanisms on immunocytes developments and activations, and exert crucial impacts on the pathological processes in immune disorders. Areas covered: Methionine cycle has been considered as an effective means of drug developments. This review discussed the role of methionine cycle in immune responses and summarized the potential therapeutic strategies based on the cycle, including SAM analogs, methyltransferase inhibitors, S-adenosylhomocysteine hydrolase (SAHH) inhibitors, adenosine receptors specific agonists or antagonists and homocysteine (Hcy)-lowering reagents, in treating human immunodeficiency virus (HIV) infections, systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), multiple sclerosis (MS), systemic sclerosis (SSc) and other immune disorders...
August 22, 2017: Expert Opinion on Therapeutic Targets
https://www.readbyqxmd.com/read/28826529/complement-system-in-neural-synapse-elimination-in-development-and-disease
#20
Jessy Presumey, Allison R Bialas, Michael C Carroll
Recent discoveries implicate the classical complement cascade in normal brain development and in disease. Complement proteins C1q, C3, and C4 participate in synapse elimination, tagging inappropriate synaptic connections between neurons for removal by phagocytic microglia that exist in a special, highly phagocytic state during the synaptic pruning period. Several neurodevelopmental disorders, such as schizophrenia and autism, are thought to be caused by an imbalance in synaptic pruning, and recent studies suggest that dysregulation of complement could promote this synaptic pruning imbalance...
2017: Advances in Immunology
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