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Pediatric lupus

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)
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...
September 30, 2016: Rheumatol Ther
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
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
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
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
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
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
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
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
J S Hui-Yuen, S C Nguyen, A D Askanase
Belimumab (Benlysta) is a fully-humanized monoclonal antibody that inhibits B-lymphocyte stimulator (also known as B cell activating factor) and was approved by the U.S. Federal Drug Administration and European Medicines Evaluation Agency for treatment in adults with autoantibody-positive systemic lupus erythematosus (SLE). Rituximab (Rituxan) is a chimeric anti-CD20 monoclonal antibody targeting B lymphocytes. This review discusses the key findings of the phase III trials in adults with SLE and of real-world use of belimumab and rituximab in the care of both adult and pediatric SLE patients...
September 2016: Lupus
Olcay Ünver, Gazanfer Ekinci, Büşra Işın Kutlubay, Thomas Gülten, Sağer Güneş, Nilüfer Eldeş Hacıfazlıoğlu, Dilşad Türkdoğan
AIM: We aimed to evaluate the patients who were followed up in our clinic with a diagnosis of cerebral sinovenous thrombosis in terms of age, sex, clinical findings, etiology, thrombophilic factors, imaging findings, treatment and prognosis. MATERIAL AND METHODS: The files of 11 patients who were followed up in our pediatric neurology clinic with a diagnosis of cerebral thrombosis between 1 December 2010 and 31 December 2014 were retrospectively analyzed. RESULTS: Seven of 11 patients were male (63...
June 2016: Türk Pediatri Arşivi
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
Yoomie J Lee, Jennifer Toh, Elina Jerschow, Jenny Shliozberg
No abstract text is available yet for this article.
September 2016: Annals of Allergy, Asthma & Immunology
Jing Jin, James L Zehnder
OBJECTIVES: The main clinical sequela of a lupus anticoagulant is increased thrombosis risk. However, bleeding due to lupus anticoagulant-hypoprothrombinemia syndrome is a rare but well-described manifestation of antiphospholipid syndrome. The association of acute acquired hypoprothrombinemia is caused by a lupus anticoagulant's specificity to prothrombin, which results in clearance of prothrombin and bleeding due to hypoprothrombinemia (usually <10% of normal). Severe life-threatening bleeding is most frequently reported in children with systemic lupus erythematosus or in healthy children after viral infection...
August 2016: American Journal of Clinical Pathology
Rona Dagan, Roxana Cleper, Miriam Davidovits, Levana Sinai-Trieman, Irit Krause
BACKGROUND: The incidence of post-infectious glomerulonephritis (PIGN) has decreased over the last decades. As a result, recent epidemiological data from industrialized countries are scarce. OBJECTIVES: To evaluate patterns of PIGN in children and detect possible predictors of disease severity. METHODS: We collected clinical and laboratory data of patients with PIGN admitted to Schneider Children's Medical Center during 1994-2011. Diagnostic criteria included presence of hematuria with/without other features of nephritic syndrome along with hypocomplementemia and/or microbiological/serological evidence of streptococcal infection...
June 2016: Israel Medical Association Journal: IMAJ
Kyung-Ran Kim, Jong Min Kim, Ji-Man Kang, Yae-Jean Kim
PURPOSE: Pneumocystis jirovecii pneumonia occurs in various immunocompromised patients. Despite the prophylaxis strategies in clinical practice, certain patients develop P. jirovecii pneumonia. This study was performed to investigate pediatric cases with P. jirovecii pneumonia in a single center. METHODS: We identified pediatric patients younger than 19 years with microbiologically confirmed P. jirovecii pneumonia from January 2000 to February 2014. A retrospective chart review was performed...
June 2016: Korean Journal of Pediatrics
Saimun Singla, Debra L Canter, Timothy J Vece, Eyal Muscal, Marietta DeGuzman
BACKGROUND: Diffuse alveolar hemorrhage (DAH) is a devastating clinical syndrome characterized by a falling hematocrit, respiratory insufficiency, and radiographic evidence of pulmonary infiltrates. Literature regarding management of DAH in childhood-onset SLE (cSLE) is limited. METHODS: We reviewed the presentation, management, and outcome of DAH in a pediatric tertiary medical center with one of the largest cSLE cohorts in North America. During a 10 year period 7 of 410 children with cSLE had DAH...
August 2016: Hospital Pediatrics
Eva DE Caluwé, Alexander VAN DE Bruaene, Rik Willems, Els Troost, Marc Gewillig, Filip Rega, Werner Budts
BACKGROUND: Children from mothers with systemic lupus erythematosus are frequently born with congenital heart block. This study aimed at evaluating long-term outcome because long-term data are scarce. METHODS: In the database of pediatric and congenital heart disease (University Hospitals Leuven), 19 children from systemic lupus erythematosus mothers and who were born with or developed atrioventricular block were identified. All records were reviewed for disease course and outcome...
September 2016: Pacing and Clinical Electrophysiology: PACE
J Sipurzynski, B Fahrner, R Kerbl, R Crazzolara, N Jones, G Ebetsberger, B Jauk, V Strenger, B Wohlmuther, W Schwinger, H Lackner, C Urban, W Holter, M Minkov, L Kager, M Benesch, M G Seidel
Chronic immune thrombocytopenia (cITP) is often associated with an underlying predisposition towards autoimmunity, recognition of which is relevant to guide treatment. International recommendations on diagnostic steps and therapeutic measures of cITP in childhood exist. However, due to the low prevalence (1-2/100,000) and a variation of availability of immunological and hematological tests and treatments across pediatric units, we postulated that these guidelines are not uniformly adhered to and that immune dysregulation syndromes remained undiscovered...
April 2016: Seminars in Hematology
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