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Pediatric lupus

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https://www.readbyqxmd.com/read/27921164/m-type-phospholipase-a2-receptor-pla2r-glomerular-staining-in-pediatric-idiopathic-membranous-nephropathy
#1
Shoichiro Kanda, Shigeru Horita, Takeshi Yanagihara, Akira Shimizu, Motoshi Hattori
BACKGROUND: Identifying M-type phospholipase A2 receptor (PLA2R) is a landmark breakthrough for understanding adult idiopathic membranous nephropathy (iMN). However, potential roles for PLA2R in pediatric iMN have not been well investigated. METHODS: A total of 34 pediatric iMN patients who underwent kidney biopsy between 1972 and 2015 were enrolled in this study. The study cohort consisted of 15 children aged from 3 to 9 years and 19 aged from 10 to 15 years...
December 5, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27921149/a-case-of-urticarial-vasculitis-in-a-female-patient-with-lupus-mycoplasma-pneumoniae-infection-or-lupus-reactivation
#2
Mario Diplomatico, Maria Francesca Gicchino, Orsola Ametrano, Pierluigi Marzuillo, Alma Nunzia Olivieri
A 17-year-old female patient affected by systemic lupus erythematosus (SLE) (who had been taking 300 mg/die of hydroxychloroquine for 3 years), Graves' disease (treated with 10 mg/die of tapazole), and celiac disease came to our attention for urticarial vasculitis. She had been taking prednisone (25 mg/die) for 3 days, and her blood tests showed high levels of Mycoplasma pneumoniae IgM and IgG antibodies. The association between urticaria and M. pneumoniae infections can be present in up to 7% of the cases and, to the best of our knowledge, only two reports of urticarial vasculitis and M...
December 5, 2016: Rheumatology International
https://www.readbyqxmd.com/read/27900972/failure-to-thrive-and-nephrocalcinosis-due-to-distal-renal-tubular-acidosis-a-rare-presentation-of-pediatric-lupus-nephritis
#3
Madhumita Nandi, Mrinal Kanti Das, Sukanta Nandi
A 9-year-old female child was initially diagnosed of having nephrocalcinosis with distal renal tubular acidosis (dRTA) while investigating for short stature. She later on developed features of nephrotic syndrome (NS) while on treatment for RTA. Investigation for the cause of NS revealed very strong serological evidence in favor of systemic lupus erythematosus (SLE). Histopathological confirmation could not be done due to bilateral severely contracted kidneys. There are a few case reports of dRTA as the presentation of SLE, but nephrocalcinosis with dRTA with subsequent manifestation of SLE has hitherto not been reported in literature...
November 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27900491/treatable-renal-disease-in-children-with-silent-lupus-nephritis-detected-by-baseline-biopsy-association-with-serum-c3-levels
#4
Hiroyuki Wakiguchi, Syuji Takei, Tomohiro Kubota, Akinori Miyazono, Yoshifumi Kawano
Lupus nephritis is identified in up to 75% of patients with juvenile systemic lupus erythematosus and may present with abnormal urinary findings (overt lupus nephritis) or be apparent only upon renal biopsy (silent lupus nephritis). We investigated whether serum complement levels correlate with renal pathology in pediatric patients with silent lupus nephritis. We performed baseline renal biopsy in 45 children diagnosed with juvenile systemic lupus erythematosus who were admitted to Kagoshima University Hospital between January 2000 and June 2015...
November 30, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27855151/advances-in-the-care-of-children-with-lupus-nephritis
#5
Scott E Wenderfer, Natasha M Ruth, Hermine I Brunner
The care of children with lupus nephritis (LN) has changed dramatically over the past 50 years. The majority of patients with childhood-onset systemic lupus erythematosus (cSLE) develop LN. In the 1960's, prognosis in children was worse than in adults; therapies were limited and toxic. Nearly half of cases resulted in death within 2-years. Since this time, several diagnostic recommendations and disease-specific indices have been developed to assist physicians caring for patients with LN. Pediatric researchers are validating and adapting these indices and guidelines for the treatment of LN in cSLE...
November 17, 2016: Pediatric Research
https://www.readbyqxmd.com/read/27837194/lenalidomide-for-refractory-cutaneous-manifestations-of-pediatric-systemic-lupus-erythematosus
#6
E Y Wu, L E Schanberg, E C Wershba, C E Rabinovich
OBJECTIVE: Cutaneous manifestations of pediatric systemic lupus erythematosus cause significant morbidity. Lenalidomide, a thalidomide analogue, has shown promise treating cutaneous lupus erythematosus in adults. Our objective was to evaluate lenalidomide's efficacy and safety in treating refractory cutaneous manifestations of pediatric systemic lupus erythematosus. METHODS: We performed a retrospective chart review of 10 adolescents who received lenalidomide for recalcitrant cutaneous lupus erythematosus...
November 10, 2016: Lupus
https://www.readbyqxmd.com/read/27826712/subclinical-pulmonary-hypertension-in-childhood-systemic-lupus-erythematosus-associated-with-minor-disease-manifestations
#7
Pedro Anuardo, Monica Verdier, Natali W S Gormezano, Gabriela R V Ferreira, Gabriela N Leal, Alessandro Lianza, Juliana C O A Ferreira, Rosa M R Pereira, Nadia E Aikawa, Maria Teresa Terreri, Claudia S Magalhães, Simone Appenzeller, Maria Carolina Dos Santos, Silvana B Sachetti, Claudio A Len, Gecilmara S Pilleggi, Simone Lotufo, Eloisa Bonfá, Clovis A Silva
The aim of this study was to evaluate pulmonary hypertension (PH) in 852 childhood-onset systemic lupus erythematosus (cSLE) patients. This was a large multicenter study conducted in 10 Pediatric Rheumatology Services of São Paulo state, Brazil. PH was defined as systolic pulmonary artery pressure >35 mmHg and/or measurement of the mean pulmonary artery pressure >25 mmHg and/or diastolic pressure >15 mmHg by transthoracic echocardiogram. Demographic data, clinical manifestations, disease activity score (SLEDAI-2K), disease damage score (SLICC/ACR-DI) and treatments were also evaluated...
November 8, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27821514/autoimmune-hemolytic-anemia-in-systemic-lupus-erythematosus-at-diagnosis-differences-between-pediatric-and-adult-patients
#8
N W S Gormezano, D Kern, O L Pereira, G C X Esteves, A M E Sallum, N E Aikawa, R M R Pereira, C A Silva, E Bonfá
OBJECTIVE: To determine the overall prevalence of autoimmune hemolytic anemia (AIHA), and to compare clinical and laboratory features in a large population of children and adult lupus patients at diagnosis. METHODS: This retrospective study evaluated the medical charts of 336 childhood-onset systemic lupus erythematosus (cSLE) and 1830 adult SLE (aSLE) patients followed in the same tertiary hospital. Demographic data, clinical features and disease activity were recorded...
November 7, 2016: Lupus
https://www.readbyqxmd.com/read/27778386/case-of-pediatric-bullous-systemic-lupus-erythematosus-treated-with-intravenous-immunoglobulin
#9
Margit Juhász, Megan Rogge, Mary Chen, Annette Czernik, Soo Jung Kim, Lauren Geller
This is a case report of a 16-year-old girl recently diagnosed with systemic lupus erythematosus (SLE) who presented with multiple blisters on the face, hands, arms, legs, trunk, and vaginal and oral mucosa. Skin biopsy was consistent with bullous SLE (BSLE). Dapsone is often the first-line treatment option for BSLE, but the patient's history of anemia and leukopenia and long-term immunosuppression requirement for her systemic symptoms raised concerns about dapsone and bone marrow toxicity, especially hemolytic anemia and agranulocytosis...
October 25, 2016: Pediatric Dermatology
https://www.readbyqxmd.com/read/27775006/synthetic-oligonucleotide-antigens-modified-with-locked-nucleic-acids-detect-disease-specific-antibodies
#10
Simone V Samuelsen, Ilia A Solov'yov, Imelda M Balboni, Elizabeth Mellins, Christoffer Tandrup Nielsen, Niels H H Heegaard, Kira Astakhova
New techniques to detect and quantify antibodies to nucleic acids would provide a significant advance over current methods, which often lack specificity. We investigate the potential of novel antigens containing locked nucleic acids (LNAs) as targets for antibodies. Particularly, employing molecular dynamics we predict optimal nucleotide composition for targeting DNA-binding antibodies. As a proof of concept, we address a problem of detecting anti-DNA antibodies that are characteristic of systemic lupus erythematosus, a chronic autoimmune disease with multiple manifestations...
October 24, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27749566/serum-il-18-as-biomarker-in-predicting-long-term-renal-outcome-among-pediatric-onset-systemic-lupus-erythematosus-patients
#11
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)
https://www.readbyqxmd.com/read/27747587/an-update-on-treatment-and-management-of-pediatric-systemic-lupus-erythematosus
#12
REVIEW
Amit Thakral, Marisa S Klein-Gitelman
Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disorder in which 20 % of patients are diagnosed in childhood. Childhood-onset SLE is associated with higher morbidity and mortality than adult-onset SLE. The aims of disease management with early immunosuppression are to decrease disease activity and improve quality of life. A multidisciplinary approach is necessary due to the complexity of lupus in pediatric patients. It is important to provide patients with high quality of care and to instill ownership of their disease process from a young age to prepare them to manage this life-long illness...
December 2016: Rheumatology and Therapy
https://www.readbyqxmd.com/read/27746022/association-between-demyelinating-disease-and-autoimmune-rheumatic-disease-in-a-pediatric-population
#13
Ana Luiza M Amorim, Nadia C Cabral, Fabiane M Osaku, Claudio A Len, Enedina M L Oliveira, Maria Teresa Terreri
INTRODUCTION: Multiple sclerosis (MS) and neuromyelitis optica (NMO) are demyelinating diseases of the central nervous system. Autoimmunity in patients with demyelinating disease and in their families has been broadly investigated and discussed. Recent studies show a higher incidence of rheumatic autoimmune diseases among adult patients with MS or NMO and their families, but there are no studies in the pediatric population. OBJECTIVE: To evaluate an association of MS and NMO with autoimmune rheumatic diseases in pediatric patients...
September 28, 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27707584/epithelial-mesenchymal-transition-in-pediatric-nephropathies
#14
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...
December 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27692389/the-representation-of-getting-ill-in-adolescents-with-systemic-lupus-erythematosus
#15
Ondina Lúcia Ceppas Resende, Maria Tereza Serrano Barbosa, Bruno Francisco Teixeira Simões, Luciane de Souza Velasque
INTRODUCTION: This study, developed in a federal hospital in the city of Rio de Janeiro, has aimed to analyze the social representation of chronic disease and its treatment, in the perspective of adolescents and their caregivers. METHODS: The sample consisted of 31 adolescents (11-21 years) with systemic lupus erythematosus and 19 caregivers (32-66 years), followed in the pediatrics and in the internal medicine outpatient clinics for a period of six months. Data was collected from the free association of words test, using chronic disease and treatment of chronic disease impulses, and later submitted to the Multiple Correspondence Analysis using the R software...
September 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27629113/characterization-of-chronic-arthritis-in-a-multicenter-study-of-852-childhood-onset-systemic-lupus-erythematosus-patients
#16
Ana Paula Sakamoto, Clovis Artur Silva, Mariana Paes Leme Ferriani, Rosa Maria Rodrigues Pereira, Eloisa Bonfá, Claudia Saad-Magalhães, Eunice Okuda, Simone Appenzeller, Francisco Hugo Gomes, Ana Luiza Garcia Cunha, Mirna Henriques Tomich Salume, Daniela Petry Piotto, Maria Teresa Terreri
Chronic arthritis (CA) is an unusual condition in childhood-onset systemic lupus erythematosus (cSLE) and data in children is very limited. The aim of the study is to assess CA in a large population of cSLE patients, in a multicenter cross-sectional study including 852 cSLE patients followed in ten Pediatric Rheumatology referral services in state of São Paulo, Brazil. CA was observed in 32/852 (3.7 %) cSLE patients mostly in hands and ankles. Chronic monoarthritis was diagnosed in four cSLE patients, oligoarthritis in nine and polyarthritis in 19...
September 14, 2016: Rheumatology International
https://www.readbyqxmd.com/read/27615277/what-is-known-about-pediatric-antiphospholipid-syndrome
#17
Pier Luigi Meroni, Lorenza Maria Argolini, Irene Pontikaki
INTRODUCTION: Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by vascular thrombosis and/or pregnancy morbidity associated with the persistent presence of antiphospholipid antibodies (aPL) including lupus anticoagulant (LA), anticardiolipin antibodies (aCL), and anti-β2 glycoprotein I antibodies (aβ2GPI). AREAS COVERED: APS is considered as the most common acquired hypercoagulation state of autoimmune origin in children. Unfortunately, data about incidence, prevalence, thrombosis risk and effective treatment in paediatric APS are limited and unmethodical...
October 2016: Expert Review of Hematology
https://www.readbyqxmd.com/read/27521770/fertility-counseling-and-preservation-practices-in-youth-with-lupus-and-vasculitis-undergoing-gonadotoxic-therapy
#18
Leena Nahata, Vidya Sivaraman, Gwendolyn P Quinn
OBJECTIVE: To assess fertility counseling and preservation practices among children, adolescents, and young adults with rheumatic diseases undergoing cyclophosphamide (CTX) treatment. DESIGN: Retrospective chart review (2006-2016). SETTING: Academic pediatric center. PATIENT(S): Male and female patients with systemic lupus erythematosus, Wegener's granulomatosis/granulomatosis with polyangiitis, or other vaculitides, receiving CTX treatment...
August 10, 2016: Fertility and Sterility
https://www.readbyqxmd.com/read/27510603/clinical-characteristics-of-children-with-membranous-lupus-nephritis-the-childhood-arthritis-and-rheumatology-research-alliance-legacy-registry
#19
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
https://www.readbyqxmd.com/read/27510530/macrophage-activation-syndrome-in-children-with-systemic-juvenile-idiopathic-arthritis-and-systemic-lupus-erythematosus
#20
Selin Aytaç, Ezgi Deniz Batu, Şule Ünal, Yelda Bilginer, Mualla Çetin, Murat Tuncer, Fatma Gümrük, Seza Özen
Macrophage activation syndrome (MAS) is a hyper-inflammatory disorder secondary to a rheumatic disease such as systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus (SLE). We aimed to present the characteristics of our pediatric MAS patients. Clinical features, laboratory parameters, treatment, and outcome of 34 patients (28 SJIA; six SLE; 37 MAS episodes) followed at a tertiary health center between 2009 and 2015 were retrospectively reviewed. The median age at MAS onset was 11 years...
October 2016: Rheumatology International
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