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pediatric lupus nephritis

Chao-Yi Wu, Huang-Yu Yang, Tsung-Chieh Yao, Su-Hsun Liu, Jing-Long Huang
An urge of biomarker identification is needed to better monitor lupus nephritis (LN) disease activity, guide clinical treatment, and predict patient's long-term outcome. With the proinflammatory effect and its association with inflammasomes, the significance of interleukin-18 (IL-18) among pediatric-onset systemic lupus erythematous (pSLE) patient, especially, its importance in predicting long-term renal outcome was investigated.In a pSLE cohort of 96 patients with an average follow-up period of 10.39 ± 3...
October 2016: Medicine (Baltimore)
Laura Penna Rocha, Samuel Cavalcante Xavier, Fernanda Rodrigues Helmo, Juliana Reis Machado, Fernando Silva Ramalho, Marlene Antônia Dos Reis, Rosana Rosa Miranda Corrêa
INTRODUCTION: Epithelial-mesenchymal transition (EMT) is a process in which epithelial cells may express mesenchymal cell markers with subsequent change in their functions, and it may be part of the etiopathogenesis of kidney disease. OBJECTIVE: The aim of this study was to evaluate the immunexpression of some EMT inducers and markers in frequent nephropathies in pediatric patients. METHODS: 59 patients aged 2-18 years old were selected and divided into 6 groups of frequent nephropathies in children and adolescents, as well as one control group...
September 22, 2016: Pathology, Research and Practice
A Boneparth, S E Wenderfer, L Nandini Moorthy, S M Radhakrishna, A C P Sagcal-Gironella, E von Scheven
OBJECTIVE: The objective of this article is to describe and compare clinical features, treatment, and renal outcomes of children with membranous lupus nephritis (MLN), through analysis of a national multicenter registry. METHODS: Patients with pediatric systemic lupus erythematosus (SLE) and MLN from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry were included. Demographic, disease and medication-related data were collected between 2010 and 2014 from 59 CARRA Legacy Registry sites...
August 9, 2016: Lupus
L B Lewandowski, L E Schanberg, N Thielman, A Phuti, A A Kalla, I Okpechi, P Nourse, P Gajjar, G Faller, P Ambaram, H Reuter, G Spittal, C Scott
BACKGROUND: Systemic lupus erythematosus (SLE) is a life-threatening multisystem autoimmune disease that is more severe in patients of African ancestry and children, yet pediatric SLE on the African continent has been understudied. This study describes a cohort of pediatric SLE (PULSE) patients in South Africa. METHODS: Patients with a diagnosis of SLE (1997 American College of Rheumatology criteria) diagnosed prior to age 19 years in Cape Town, South Africa, were enrolled in this cross-sectional study from September 2013 to December 2014...
August 3, 2016: Lupus
S Imtiaz, K Nasir, M F Drohlia, B Salman, A Ahmad
Kidney biopsy occupies a fundamental position in the management of kidney diseases. There are very few renal pathology studies available in the literature from developing world. This study scrutinized the frequency and clinicopathological relationship of kidney biopsies done at the kidney center from 1997 to 2013 amongst pediatric patients. Kidney allograft biopsy were excluded. The specimen was examined under light microscopy and immunofluorescence while electron microscopy was not done. The study includes 423 patients, mean age was 10...
May 2016: Indian Journal of Nephrology
Martine T P Besouw, Johan G Vande Walle, Mohamad I Ilias, Ann M Raes, Agnieszka A Prytula, Lieve Claeys, Jo L Dehoorne
A 12-year-old Congolese girl presented with acute renal failure, edema, hypertension, hemoptysis, hematuria, and proteinuria after a history of throat infection. Renal ultrasound showed kidneys of normal size, with increased echogenicity of the cortical parenchyma and decreased corticomedullary differentiation. Other additional investigations showed pancytopenia with decreased complement (low C3 and C4). Antinuclear antibodies were strongly positive, including anti-double stranded DNA. Renal biopsy confirmed severe grade IV lupus nephritis...
May 12, 2016: Acta Clinica Belgica
Ting-Chun Lin, Jhong-Yong Wu, Ming-Ling Kuo, Liang-Shiou Ou, Kuo-Wei Yeh, Jing-Long Huang
BACKGROUND: Vitamin D deficiency has been associated with systemic lupus erythematosus (SLE), but there is no consensus on the role of serum vitamin D in evaluating or predicting disease activity. This study aimed to demonstrate the direct correlation between vitamin D level and pediatric-onset SLE disease activity by a retrospective cohort study design. PATIENTS AND METHODS: Thirty-five patients with pediatric-onset SLE and paired sera at the active and inactive disease states were enrolled...
January 12, 2016: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
J Reis, F Aguiar, I Brito
OBJECTIVES: We aim to report the efficacy and safety of rituximab (RTX) in patients diagnosed with juvenile systemic lupus erythematosus (JSLE) or juvenile idiopathic arthritis (JIA) refractory to conventional treatment. METHODS: A retrospective review was made of all medical records of patients with JSLE or JIA treated with RTX between January 2009 and January 2015 in the Pediatric Rheumatology Unit of a central hospital. RESULTS: Five patients, 4 with JSLE and 1 with extended oligoarticular JIA, received 10 cycles of RTX (23 infusions)...
January 2016: Acta Reumatológica Portuguesa
Neha Thakur, Narendra Rai, Prerna Batra
Systemic lupus erythematous (SLE) is a multisystem autoimmune disorder characterized by immune dysregulation and formation of autoantibodies. A high index of suspicion is necessary to diagnose SLE. Children have more systemic involvement than adults. Kidney involvement is seen in a significant proportion of children. With advancement of therapy the survival rate of patients with SLE has significantly improved. Even then lupus nephritis is still the most important predictor of morbidity and mortality. Treatment of lupus nephritis is mostly derived from studies in adults as data on children is still lacking...
April 4, 2016: Current Rheumatology Reviews
Romain Banchereau, Seunghee Hong, Brandi Cantarel, Nicole Baldwin, Jeanine Baisch, Michelle Edens, Alma-Martina Cepika, Peter Acs, Jacob Turner, Esperanza Anguiano, Parvathi Vinod, Shaheen Kahn, Gerlinde Obermoser, Derek Blankenship, Edward Wakeland, Lorien Nassi, Alisa Gotte, Marilynn Punaro, Yong-Jun Liu, Jacques Banchereau, Jose Rossello-Urgell, Tracey Wright, Virginia Pascual
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by loss of tolerance to nucleic acids and highly diverse clinical manifestations. To assess its molecular heterogeneity, we longitudinally profiled the blood transcriptome of 158 pediatric patients. Using mixed models accounting for repeated measurements, demographics, treatment, disease activity (DA), and nephritis class, we confirmed a prevalent IFN signature and identified a plasmablast signature as the most robust biomarker of DA...
April 21, 2016: Cell
Roberta C Gomes, Marco F Silva, Katia Kozu, Eloisa Bonfá, Rosa M Pereira, Maria T Terreri, Claudia S Magalhães, Silvana B Sacchetti, Roberto Marini, Melissa Fraga, Luciana M Carvalho, Cássia M Barbosa, Magda Carneiro-Sampaio, Clovis A Silva
OBJECTIVE: To evaluate demographic data, clinical and laboratory features at disease diagnosis in three different age groups of childhood systemic lupus erythematosus (cSLE): group A early-onset(<6 years), group B school age(≥6 and <12 years) and group C adolescent(≥12 and <18 years). METHODS: Brazilian multicenter cohort retrospective study in 10 Pediatric Rheumatology centers, including 847 cSLE patients. RESULTS: Patients were divided in three groups: A with 39(4%), B 395(47%) and C 413(49%)...
March 25, 2016: Arthritis Care & Research
Shankar Prasad Yadav, Gauri Shankar Shah, Om Prakash Mishra, Nirmal Baral
Spectrum of renal disease varies in different ethnic population, geographical location, and by environmental factors. The purpose of this study was to find out the clinical spectrum and occurrence of different pediatric renal diseases at a teaching hospital in the Eastern part of Nepal. All cases of renal diseases from one month to 15 years of age, attending the pediatric renal outpatient department and/or were admitted to the wards during the period of February 2012 to January 2013, were included in the study...
March 2016: Saudi Journal of Kidney Diseases and Transplantation
Joyce S Hui-Yuen, Tran Tran, Jennifer Taylor, Kristi Truong, Xiaoqing Li, Liza M Bermudez, Amy J Starr, Andrew H Eichenfield, Lisa F Imundo, Anca D Askanase
BACKGROUND/OBJECTIVES: Mycophenolate mofetil (MMF) is used to treat pediatric-onset lupus nephritis (pLN). Data are equivocal on the use of plasma mycophenolic acid (MPA) levels as a measure of efficacy and predictor of therapeutic outcomes in pLN. Glucuronidated MPA (MPA-G) is an inactive metabolite that is a marker of adequate absorption and normal metabolism of MMF. We evaluated the use of MPA and MPA-G levels in routine care of pLN. METHODS: This was a retrospective study of pLN patients treated with MMF dosed at 600 mg/m...
March 2016: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
K M Abulaban, H Song, X Zhang, P L Kimmel, J W Kusek, R G Nelson, H I Feldman, R S Vasan, J Ying, M Mauer, G L Nelsestuen, M Bennett, H I Brunner, B H Rovin
OBJECTIVE: To evaluate candidate biomarkers to predict future renal function decline (RFD) in children and adults with lupus nephritis (LN). METHODS: At the time of enrollment into prospective observational LN cohort studies liver-type fatty acid binding protein (LFABP), albumin, monocyte chemoattractant protein-1 (MCP-1), uromodulin, transferrin, and hepcidin were measured in urine samples of two cohorts of patients with LN, one followed at a pediatric (cohort-1; n = 28) and one at an adult institution (cohort-2; n = 69)...
August 2016: Lupus
J C O A Ferreira, H H Marques, M P L Ferriani, N W S Gormezano, M T Terreri, R M Pereira, C S Magalhães, L M Campos, V Bugni, E M Okuda, R Marini, G S Pileggi, C M Barbosa, E Bonfá, C A Silva
OBJECTIVE: The aim of this multicenter study in a large childhood-onset systemic lupus erythematosus (cSLE) population was to assess the herpes zoster infection (HZI) prevalence, demographic data, clinical manifestations, laboratory findings, treatment, and outcome. METHODS: A retrospective multicenter cohort study (Brazilian cSLE group) was performed in ten Pediatric Rheumatology services in São Paulo State, Brazil, and included 852 cSLE patients. HZI was defined according to the presence of acute vesicular-bullous lesions on erythematous/edematous base, in a dermatomal distribution...
June 2016: Lupus
Mengjie Jiang, Zizheng Xiao, Liping Rong, Yuanyuan Xu, Lizhi Chen, Ying Mo, Liangzhong Sun, Wei Sun, Xiaoyun Jiang
AIMS: This study aimed to investigate the clinicopathologic characteristics of biopsy-proven childhood renal diseases and to compare the trends and changes during 2 different time intervals between 1984 and 2011 at the 1st affiliated hospital of Sun Yat-sen University in China. METHODS: We retrospectively analyzed kidney biopsy data from children with renal diseases and compared the data during 2 time intervals, namely 1984-1997 and 1998-2011. RESULTS: This study enrolled 1,313 children...
December 23, 2015: Nephrology
Kamal V Kanodia, Aruna V Vanikar, Lovelesh K Nigam, Rashmi D Patel, Kamlesh S Suthar, Dinesh N Gera, Hargovind L Trivedi
BACKGROUND: Renal biopsy is a well-established diagnostic modality for the assessment of kidney diseases in children. It can provide diagnostic precision and prognostic value and guide in therapeutic options for many renal diseases. OBJECTIVES: This report describes the indication, histopathological patterns, and epidemiology of renal diseases in children in India. PATIENTS AND METHODS: This is a single-center study on renal biopsies performed between January 2008 and December 2013 in 346 children (age ≤ 14 years)...
July 2015: Nephro-urology Monthly
Victor L S Marques, Natali W S Gormezano, Eloisa Bonfá, Nadia E Aikawa, Maria T Terreri, Rosa M Pereira, Claudia S Magalhães, Andressa Guariento, Simone Appenzeller, Virgínia P Ferriani, Cássia M Barbosa, Valéria C Ramos, Simone Lotufo, Clovis A Silva
OBJECTIVE: Pancreatitis is a rare and a life-threatening systemic lupus erythematosus (SLE) manifestation in childhood-onset SLE (cSLE). The objective of this study was to systematically classify pancreatitis in cSLE according to the International Study Group of Pediatric Pancreatitis and determine the overall prevalence, clinical features, laboratory, and first episode outcomes. METHODS: A multicenter cohort study in 10 pediatric rheumatology centers, including 852 patients with cSLE...
February 2016: Journal of Pediatric Gastroenterology and Nutrition
Joel Reis, Francisca Aguiar, Iva Brito
OBJECTIVES: We aim to report the efficacy and safety of rituximab (RTX) in patients diagnosed with juvenile systemic lupus erythematosus (JSLE) or juvenile idiopathic arthritis (JIA) refractory to conventional treatment. METHODS: A retrospective review was made of all medical records of patients with JSLE or JIA treated with RTX between January 2009 and January 2015 in the Pediatric Rheumatology Unit of a central hospital. RESULTS: Five patients, 4 with JSLE and 1 with extended oligoarticular JIA, received 10 cycles of RTX (23 infusions)...
August 3, 2015: Acta Reumatológica Portuguesa
Rina Mina, Julia G Harris, Marisa S Klein-Gitelman, Simone Appenzeller, Maraisa Centeville, Diane Eskra, Jennifer L Huggins, Anne L Johnson, Raju Khubchandani, Prachi Khandekar, Jiha Lee, Hai Mei Liu, Joshua D Pendl, Clovis A Silva, Marco F Silva, Ahmad I Zaal, Esi Morgan DeWitt, Stacy P Ardoin, Hermine I Brunner
OBJECTIVE: To assess the quality of medical care in childhood-onset systemic lupus erythematosus (SLE) at tertiary pediatric rheumatology centers as measured by observance of SLE quality indicators (SLE-QIs). METHODS: International consensus has been achieved for childhood-onset SLE-QIs capturing medical care provision in 9 domains: diagnostic testing, education of cardiovascular (CV) risk and lifestyles, lupus nephritis (LN), medication management, bone health, ophthalmologic surveillance, transition, pregnancy, and vaccination...
February 2016: Arthritis Care & Research
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