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pediatrics lupus

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https://www.readbyqxmd.com/read/29739689/exome-sequencing-revealed-c1q-homozygous-mutation-in-pediatric-systemic-lupus-erythematosus
#1
S Zoghi, V Ziaee, T Hirschmugl, R Jimenez-Heredia, A Krolo, K Boztug, N Rezaei
INTRODUCTION AND OBJECTIVES: Pediatric Systemic Lupus Erythematosus (pSLE) is an autoimmune disorder of children. Early disease onset raises the probability of genetic etiology and it is more severe than adult SLE. PATIENTS AND METHODS: Herein an eight-year-old girl with pSLE from consanguineous parents is reported. RESULTS: Although she was diagnosed as pSLE since the age of two years, Whole Exome Sequencing (WES) revealed a rare stop-gained C>T mutation in C1QA gene...
May 5, 2018: Allergologia et Immunopathologia
https://www.readbyqxmd.com/read/29692593/complement-deficiency-in-pediatric-onset-systemic-lupus-erythematosus
#2
Parisa Afzali, Anna Isaeian, Peyman Sadeghi, Bobak Moazzami, Nima Parvaneh, Masoumeh Robatjazi, Vahid Ziaee
BACKGROUND: Pediatric-onset systemic lupus erythematosus (pSLE) accounts for about 10%-20% of all patients with SLE. Deficiencies in early complement components of the classical pathway are the strong genetic risk factor for the development of SLE. In this study, clinical and laboratory manifestations of both complement-deficient and normal complement pSLE patients were compared. MATERIALS AND METHODS: To investigate clinical and immunological manifestations of pSLE in Iran, 36 consecutive pSLE patients (onset before 18 years) who were followed up over a period of 2 years, were studied...
April 2018: Journal of Laboratory Physicians
https://www.readbyqxmd.com/read/29625558/predominant-but-silent-c1q-deposits-in-mesangium-on-transplanted-kidneys-long-term-observational-study
#3
Takahiro Kanai, Yuko Akioka, Kenichiro Miura, Masataka Hisano, Junki Koike, Yutaka Yamaguchi, Motoshi Hattori
BACKGROUND: C1q nephropathy (C1qN) was first described as glomerular disease characterized by predominant meangial C1q deposits in patients with proteinuria and no evidence of systemic lupus erythematosus. Several studies, however, revealed the clinical heterogeneity of C1qN, showing some cases with normal urinalysis. To confirm the existence of cases with predominant mesangial C1q deposits and negative or mild proteinuria and/or hematuria, we investigated renal graft biopsy specimens showing negative to mild proteinuria (less than or equal to 1+ by dip stick test) and/or hematuria...
April 6, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29619486/assessment-of-the-etiologies-and-renal-outcomes-of-rapidly-progressive-glomerulonephritis-in-pediatric-patients-at-king-abdulaziz-university-hospital-jeddah-saudi-arabia
#4
Faisal G Mosaad, Omar Mohammed Saggaf, Khaled T Aletwady, Khaled Y Mohammed Jan, Khalid Al-Qarni, Rakan S Al-Harbi, Osama Y Safdar
OBJECTIVES: To investigate the etiologies and outcomes of rapidly progressive glomerulonephritis (RPGN) in pediatric patients at King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia. METHODS: A retrospective study was conducted in 19 pediatric patients who were diagnosed with RPGN between 2006 and 2016 at the Department of Pediatric Medicine at KAUH. Associations between variables were evaluated using independent t-test, one-way analysis of variance (ANOVA) and Chi-squared tests...
April 2018: Saudi Medical Journal
https://www.readbyqxmd.com/read/29615791/autoantibody-profiling-in-lupus-patients-using-synthetic-nucleic-acids
#5
Martin Klecka, Christina Thybo, Claudia Macaubas, Ilia Solov'yov, Julia Simard, Imelda Maria Balboni, Emily Fox, Anne Voss, Elizabeth D Mellins, Kira Astakhova
Autoantibodies to nuclear components of cells (antinuclear antibodies, ANA), including DNA (a-DNA), are widely used in the diagnosis and subtyping of certain autoimmune diseases, including systemic lupus erythematosus (SLE). Despite clinical use over decades, precise, reproducible measurement of a-DNA titers remains difficult, likely due to the substantial sequence and length heterogeneity of DNA purified from natural sources. We designed and tested a panel of synthetic nucleic acid molecules composed of native deoxyribonucleotide units to measure a-DNA...
April 3, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29595356/chorea-revealing-systemic-lupus-erythematosus-in-a-13-year-old-boy-a-case-report-and-short-review-of-the-literature
#6
E Athanasopoulos, I Kalaitzidou, G Vlachaki, S Stefanaki, A Tzagkaraki, G Niotakis, I Tritou, F Ladomenou
Among the neurological manifestations of systemic lupus erythematosus (SLE), chorea is rare, presenting in less than 7% of the pediatric SLE patients. It can appear early in the onset of SLE, be the first or even the sole clinical feature of the illness and has strongly been associated with the presence of antiphospholipid antibodies. We report on the case of a 13-year old boy, admitted with acute onset chorea and finally diagnosed with SLE. Subsequently, we present a short review of the literature on the epidemiology, suggested pathogenesis, clinical presentation and treatment of this rare presentation of SLE...
March 29, 2018: International Reviews of Immunology
https://www.readbyqxmd.com/read/29552446/the-ratio-of-urinary-%C3%AE-1-microglobulin-to-microalbumin-can-be-used-as-a-diagnostic-criterion-for-tubuloproteinuria
#7
Hongwen Zhang, Fang Wang, Huijie Xiao, Yong Yao
Low-molecular-weight proteinuria is one of the characteristic clinical manifestations of renal tubular and interstitial diseases. Low-molecular-weight proteinuria is defined as excessive urinary loss of α1-microglobulin, β2-microglobulin, or other low-molecular-weight plasma proteins. The current study examined the ratio of urinary α1-microglobulin to microalbumin in 24 Chinese pediatric patients with renal tubular and interstitial diseases, including 10 patients with Dent disease, 2 patients with Lowe syndrome, 6 patients with acute tubulointerstitial nephritis (ATIN), 4 patients with acute tubulointerstitial nephritis with uveitis syndrome (TINU), and 2 patients with nephronophthisis (NPHP)...
February 2018: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/29518638/thrombophilia-testing-in-children-what-and-when-should-be-tested
#8
REVIEW
Ulrike Nowak-Göttl, Heleen van Ommen, Gili Kenet
Venous thrombosis (VTE) in children is gaining increased awareness and apart from underlying medical conditions, recently reported systematic reviews on pediatric VTE (70% provoked) have shown significant associations between thrombosis and presence of inherited thrombophilic risk factors (IT), such as protein C-, protein S- and antithrombin deficiency, mutations of factor 5 (F5: rs6025) and factor 2 (F2: rs1799963), even more pronounced when combined IT were involved. Although we have learned more about the pathophysiology of VTE with the increased discovery of IT evidence is still lacking as to whether IT influence the clinical outcome in pediatric VTE...
April 2018: Thrombosis Research
https://www.readbyqxmd.com/read/29515298/a-study-of-clinical-presentation-and-correlative-histopathological-patterns-in-renal-parenchymal-disease
#9
K Ganesh, R R Nair, N V Seethalekshmy, G Kurian, A Mathew, S Sreedharan, Z Paul
Suspicion and subsequent detection of renal disease is by an assessment of the urinalysis and renal function in the clinical context. Our attempt in this study is to correlate initial presenting features of urinalysis and renal function to the final histopathological diagnosis. A retrospective analysis of 1059 native kidney biopsies performed from January 2002 to June 2015 at Amrita Institute of Medical Sciences was conducted. Correlative patterns between urinalysis, renal function, and final histopathological diagnosis were studied...
January 2018: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/29511890/urinary-clusterin-a-novel-urinary-biomarker-associated-with-pediatric-lupus-renal-histopathologic-features-and-renal-survival
#10
Chao-Yi Wu, Huang-Yu Yang, Hui-Ping Chien, Min-Hua Tseng, Jing-Long Huang
BACKGROUND: Lupus nephritis (LN) is a major risk factor for systemic lupus erythematous (SLE)-related morbidity and mortality. With the aim of bypassing renal biopsy, we analyzed urinary biomarkers for their ability to predict renal histopathologic features and end-stage kidney disease (ESKD). METHODS: Urinary albumin, ß2-microglobulin (B2M), cystatin C, kidney injury molecule-1 (KIM-1), monocyte chemoattractant protein 1 (MCP-1), clusterin, calbindin, interleukin-18 (IL-18), neutrophil gelatinase-associated lipocalin (NGAL), trefoil factor 3 (TFF3), osteopontin, and glutathione S-transferase π (GST-π) levels were measured at time of renal biopsy...
March 6, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29504978/lupus-erythematosus-panniculitis-in-a-10-year-old-female-child-with-severe-systemic-lupus-erythematosus-a-case-report
#11
Ruolin Zhang, Xiqiang Dang, Lanjun Shuai, Qingnan He, Xiaojie He, Zhuwen Yi
RATIONALE: Lupus erythematosus panniculitis (LEP) is a rare subset of lupus erythematosus. The incidence of LEP in systemic lupus erythematosus (SLE) ranges from 2% to 5%. In the previous literature, most LEP patients were women aged from 20 to 60 years, while pediatric cases were rare, all of whom appeared on their own without SLE.A rare LEP in a 10-year-old female child with severe SLE is presented. PATIENT CONCERNS: A 10-year-old girl was admitted to our hospital for marasmus and fatigue without other typical manifestations of SLE well before the appearance of skin lesions...
January 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29494658/anti-epstein-barr-virus-antibodies-in-beijing-during-2013-2017-what-we-have-found-in-the-different-patients
#12
Jingtao Cui, Wenjuan Yan, Shaoxia Xu, Qiaofeng Wang, Weihong Zhang, Wenjing Liu, Anping Ni
BACKGROUND: Epstein-Barr virus (EBV) is associated with nasopharyngeal carcinoma (NPC) which is prevalent in South China, and its association with systemic lupus erythematosus (SLE) or other autoimmune diseases has not been studied in the mainland of China. The EBV serological tests have been performed on patients with various diseases or manifestations for years at our institution and their values need to be evaluated. METHODS: For routine medical purposes, anti-EB viral capsid antigen (VCA) IgG, IgA and IgM antibodies, anti-EBV diffuse early antigen (EA-D) IgA antibodies, and anti-EBV nuclear antigen-1(EBNA-1) IgG antibodies were tested with commercial enzyme-linked immunosorbent assay (ELISA) in patients visiting Peking Union Medical College Hospital between 2013 and 2017...
2018: PloS One
https://www.readbyqxmd.com/read/29492211/serum-complement-factor-i-is-associated-with-disease-activity-of-systemic-lupus-erythematosus
#13
Min-Hua Tseng, Shih-Hua Lin, Chao-Yi Wu, Hui-Ping Chien, Huang-Yu Yang, Yung-Chang Chen, Yu-Ching Chou, Jing-Long Huang
Although aberrant complement activation is involved in the pathogenesis of systemic lupus erythematosus (SLE), the role of complement regulatory proteins in disease activity of SLE remains limited. We enrolled the pediatric-onset SLE patients from our cohort study over 10 years. The clinical and laboratory data including SLEDAI disease activity score, and serum complement factor H (CFH), CFI, CD46, C5a, and C5b-9 in the active and remission phases were determined. Glomerular C5b-9 deposition as a complement activity marker was also examined...
February 2, 2018: Oncotarget
https://www.readbyqxmd.com/read/29469756/essential-telangiectasia-in-an-infant-a-diagnosis-to-be-considered
#14
Pablo Fernández-Crehuet, Ricardo Ruiz-Villaverde
Essential generalized telangiectasia as the result of postcapillary venule dilatation, is characterized by the sudden development of generalized telangiectasias, sometimes involving the conjunctiva. A few pediatric cases have been reported. The main condition in the differential diagnosis includes syndromes with telangiectasia such as hereditary hemorrhagic telangiectasia of Rendu-Osler, unilateral nevoid telangiectasia, and neonatal lupus erythematous. We present an 11-month-old boy that presented because of telangiectasia located on the face and neck...
August 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/29457995/translation-and-validation-of-the-transition-readiness-assessment-questionnaire-traq
#15
Catherine G Anelli, Claudio A Len, Maria Teresa R A Terreri, Gleice C S Russo, Andreas O Reiff
OBJECTIVE: To translate and validate the Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire in a population of adolescents and young adults with chronic rheumatologic disorders. This questionnaire evaluates the patient's readiness for making the transition from the pediatric health service to adult care. METHODS: The four-phase methodology for the translation and validation of generic questionnaires was followed, including translation, back-translation, pilot testing and clinical validation of the final tool...
February 16, 2018: Jornal de Pediatria
https://www.readbyqxmd.com/read/29457372/clinical-characteristics-and-factors-associated-with-disability-and-impaired-quality-of-life-in-children-with-juvenile-systemic-sclerosis
#16
Brandi E Stevens, Kathryn S Torok, Suzanne C Li, Nicole Hershey, Megan Curran, Gloria C Higgins, Katharine F Moore, C Egla Rabinovich, Samuel Dodson, Anne M Stevens
OBJECTIVE: To investigate clinical manifestations of juvenile systemic sclerosis (jSSc), including disease characteristics and patient quality of life, through the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry. METHODS: Subjects with jSSc were prospectively enrolled between 2010 and 2013. Diagnosis of jSSc was determined by the enrolling pediatric rheumatologist, with disease onset required prior to age 18. Collected data included demographics, disease characteristics, medication exposure, and quality of life metrics...
February 18, 2018: Arthritis Care & Research
https://www.readbyqxmd.com/read/29429194/-evidence-based-guideline-on-diagnosis-and-treatment-of-lupus-nephritis-2016
#17
(no author information available yet)
No abstract text is available yet for this article.
February 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29371779/hyperferritinemia-in-hemophagocytic-lymphohistiocytosis-a-single-institution-experience-in-pediatric-patients
#18
Surupa Basu, Biplab Maji, Santanu Barman, Apurba Ghosh
Hemophagocytic lymphohistiocytosis (HLH) is an inflammatory condition that may run a rapid fatal course and calls for prompt diagnosis. Early intervention with steroids and other immunosuppressive drugs can contain the disease process and favours positive outcome. Ferritin ≥500 ng/ml is a HLH diagnostic criterion. We evaluated the diagnostic potential of admission ferritin, in children with HLH. Pediatric patients of a referral teaching hospital from Feb 2010-Oct 2013 having been investigated for ferritin on admission were included...
January 2018: Indian Journal of Clinical Biochemistry: IJCB
https://www.readbyqxmd.com/read/29333701/the-childhood-arthritis-and-rheumatology-research-alliance-consensus-treatment-plans-toward-comparative-effectiveness-in-the-pediatric-rheumatic-diseases
#19
Sarah Ringold, Peter A Nigrovic, Brian M Feldman, George A Tomlinson, Emily von Scheven, Carol A Wallace, Adam M Huber, Laura E Schanberg, Suzanne C Li, Pamela F Weiss, Robert C Fuhlbrigge, Esi M Morgan, Yukiko Kimura
The pediatric rheumatic diseases are a heterogeneous group of rare diseases, posing a number of challenges for the use of traditional clinical and translational research methods. Innovative comparative effectiveness approaches are needed to efficiently study treatment strategies and disease outcomes. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed the consensus treatment plan (CTP) approach as a comparative effectiveness tool for research in pediatric rheumatology. CTPs are treatment strategies, developed by consensus methods among CARRA members, intended to reduce variation in treatment approaches, standardize outcome measurements, and allow for comparison of the effectiveness of different approaches with the goal of improving disease outcomes...
January 15, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29324237/dna-induction-of-mdm2-promotes-proliferation-of-human-renal-mesangial-cells-and-alters-peripheral-b-cells-subsets-in-pediatric-systemic-lupus-erythematosus
#20
Chen-Xing Zhang, Ji Chen, Li Cai, Jing Wu, Jia-Yuan Wang, Lan-Fang Cao, Wei Zhou, Tong-Xin Chen
The study is aimed to investigate the role of MDM2 in the pathogenesis of lupus nephritis (LN) in pediatric SLE (pSLE). We confirmed that MDM2 expression was increased in peripheral blood mononuclear cells (PBMCs) as well as renal specimen of SLE compared with that of controls by western blot and immunofluorescence staining. Percentage of apoptotic and necrotic CD4+ T, CD8+ T and B cells were detected by flow cytometry respectively and levels of plasma cell free DNA (cfDNA) were quantified in SLE and healthy controls (HC)...
February 2018: Molecular Immunology
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