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https://www.readbyqxmd.com/read/28088248/the-spectrum-of-rheumatic-in-patient-diagnoses-at-a-pediatric-hospital-in-kenya
#1
Angela Migowa, Inés Colmegna, Carol Hitchon, Eugene Were, Evelyn Ng'ang'a, Thomas Ngwiri, John Wachira, Sasha Bernatsky, Rosie Scuccimarri
BACKGROUND: Pediatric rheumatic diseases are chronic illnesses that can cause considerable disease burden to children and their families. There is limited epidemiologic data on these diseases in East Africa. The aim of this study was to assess the spectrum of pediatric rheumatic diagnoses in an in-patient setting and determine the accuracy of ICD-10 codes in identifying these conditions. METHODS: Medical records from Gertrude's Children's Hospital in Kenya were reviewed for patients diagnosed with "diseases of the musculoskeletal system and connective tissue" as per ICD-10 diagnostic codes assigned at discharge between January and December 2011...
January 14, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28087478/renal-involvement-in-lysinuric-protein-intolerance-contribution-of-pathology-to-assessment-of-heterogeneity-of-renal-lesions
#2
Emmanuel Estève, Pauline Krug, Aurélie Hummel, Jean-Baptiste Arnoux, Olivia Boyer, Anais Brassier, Pascale de Lonlay, Vincent Vuiblet, Stéphanie Gobin, Rémi Salomon, Christine Piètrement, Jean-Paul Bonnefont, Aude Servais, Louise Galmiche
Lysinuric protein intolerance (LPI) is a rare autosomal recessive disease caused by mutations in the SLC7A7 gene encoding the light subunit of a cationic amino acid transporter. Symptoms mimic primary urea cycle defects but dysimmune symptoms are also described. Renal involvement in LPI was first described in the 1980's. In 2007, it appeared that it could concern as much as 75% of LPI patients and could lead to end stage renal disease. The most common feature is proximal tubular dysfunction and nephrocalcinosis but glomerular lesions are also reported...
January 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/28079914/intravenous-immunoglobulin-in-pediatric-rheumatology-when-to-use-it-and-what-is-the-evidence
#3
Martha M Rodriguez, Linda Wagner-Weiner
Intravenous immunoglobulin (IVIG) is given to children with a variety of rheumatologic illnesses. The mechanism of action by which it exerts therapeutic effects is not well understood and likely differs in the medical conditions for which it is given. IVIG is approved by the US Food and Drug Administration and is the standard of care for Kawasaki disease, but most IVIG use in pediatric rheumatology is "off-label. " The literature supports the use of IVIG for juvenile dermatomyositis, although it is unclear whether its use should be limited to those children with more severe or refractory disease...
January 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28072955/-clinical-analysis-of-12-patients-with-pediatric-antiphospholipid-syndrome-with-pulmonary-embolism
#4
J R Ma, H M Song, J Xiao, X Y Tang, Y Y He, M Wei
Objective: To identify the clinical and immunological characteristics of pediatric antiphospholipid syndrome (APS) patients with pulmonary embolism. Method: Among 47 pediatric APS patients from Peking Union Medical College Hospital during the year of 2000 to 2015, 12 patients were diagnosed of pulmonary embolism, who were investigated and compared with APS patients without pulmonary embolism. Result: Twelve patients (among whom 6 cases were primary and the other 6 were secondary APS)had pulmonary embolism and all of them were non-shock type, which was the first presenting manifestation in 6 of them...
January 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28070763/uveitis-in-childhood-onset-systemic-lupus-erythematosus-patients-a-multicenter-survey
#5
Paola Pinheiro Kahwage, Mariana Paes Leme Ferriani, João M Furtado, Luciana Martins de Carvalho, Gecilmara Salviato Pileggi, Francisco Hugo Rodrigues Gomes, Maria Teresa Terreri, Claudia Saad Magalhães, Rosa Maria Rodrigues Pereira, Silvana Brasilia Sacchetti, Roberto Marini, Eloisa Bonfá, Clovis Artur Silva, Virgínia Paes Leme Ferriani
The aim of this study is to assess uveitis prevalence in a large cohort of childhood-onset systemic lupus erythematosus (cSLE) patients. A retrospective multicenter cohort study including 852 cSLE patients was performed in ten pediatric rheumatology centers (Brazilian cSLE group). An investigator meeting was held and all participants received database training. Uveitis was diagnosed through clinical assessment by the uveitis expert ophthalmologist of each center. Patients with and without uveitis were assessed for lupus clinical/laboratory features and treatments...
January 9, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28013435/evaluation-of-quality-indicators-and-disease-damage-in-childhood-onset-systemic-lupus-erythematosus-patients
#6
Julia G Harris, Kristyn I Maletta, Evelyn M Kuhn, Judyann C Olson
The aim of this study was to describe compliance with select quality indicators and assess organ-specific dysfunction in a childhood-onset systemic lupus erythematosus population by using a validated damage index and to evaluate associations between compliance with quality indicators and disease damage. A retrospective chart review was performed on patients diagnosed with systemic lupus erythematosus prior to age 18 followed at a single center in the USA from 1999 to 2012 (n = 75). Data regarding quality indicators and outcome variables, including the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index, were collected...
December 24, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27994909/assessment-of-ten-year-long-results-of-kidney-biopsies-performed-on-children-in-the-thrace-region-of-turkey
#7
Neşe Özkayın, Gökçe Çıplak, Ufuk Usta, Hakan Gençhellaç, Osman Temizöz
BACKGROUND: Many children with kidney diseases can be diagnosed and treated without a biopsy. However, biopsy is a valuable method for the diagnostic and prognostic evaluation of children with kidney diseases. AIMS: To evaluate the clinical and pathological profiles of the kidney biopsies in our department to provide epidemiological data for clinical practice. STUDY DESIGN: Retrospective cross-sectional study. METHODS: Kidney biopsies and patient's charts in pediatric patients performed between May 2005 and February 2015 at the Pediatric Nephrology Department, Trakya University School of Medicine were assessed retrospectively...
November 2016: Balkan Medical Journal
https://www.readbyqxmd.com/read/27988434/anti-ro-ssa-and-anti-la-ssb-antibodies-association-with-mild-lupus-manifestations-in-645-childhood-onset-systemic-lupus-erythematosus
#8
REVIEW
Glaucia V Novak, Mariana Marques, Verena Balbi, Natali W S Gormezano, Kátia Kozu, Ana P Sakamoto, Rosa M R Pereira, Maria T Terreri, Claudia S Magalhães, Andressa Guariento, Adriana M E Sallum, Roberto Marini, Virginia Paes Leme Ferriani, Cássia Maria Barbosa, Tânia Caroline Monteiro de Castro, Valéria C Ramos, Eloisa Bonfá, Clovis A Silva
BACKGROUND: To our knowledge there are no studies assessing anti-Ro/SSA and anti-La/SSB autoantibodies in a large population of childhood-systemic lupus erythematosus(cSLE) patients. METHODS: This was a retrospective multicenter cohort study performed in 10 Pediatric Rheumatology services, São Paulo state, Brazil. Anti-Ro/SSA and anti-La/SSB antibodies were measured by enzyme linked immunosorbent assay(ELISA) in 645 cSLE patients. RESULTS: Anti-Ro/SSA and anti-La/SSB antibodies were evidenced in 209/645(32%) and 102/645(16%) of cSLE patients, respectively...
December 14, 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27977505/unusual-presentations-of-childhood-systemic-lupus-erythematosus-to-emergency-department
#9
Ayşe Gültekingil Keser, İlker Ertuğrul, Özlem Tekşam, Bahadir Konuşkan, Süheyla Özkutlu, Seza Özen
INTRODUCTION: Systemic lupus erythematosus (SLE) is a multisystemic autoinflammatory disease that can involve any organ system; therefore, diagnosis can be challenging. Hereby, we present 4 cases that presented to pediatric emergency department with unusual clinical pictures of SLE. CASES: Case 1 presented with inability to walk or talk for the last 1 week as well as intermittent pain and swelling in her joints. Case 2 presented with generalized edema and severe dyspnea...
December 13, 2016: Pediatric Emergency Care
https://www.readbyqxmd.com/read/27960128/effect-of-donor-stat4-polymorphism-rs7574865-on-clinical-outcomes-of-pediatric-acute-leukemia-patients-after-hematopoietic-stem-cell-transplant
#10
Cheng Mun Wun, Zhe Piao, Kyung Taek Hong, Jung Yoon Choi, Che Ry Hong, June Dong Park, Kyung Duk Park, Hee Young Shin, Hyoung Jin Kang
STAT4 polymorphism, rs7574865 is linked to various autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis. Its T minor allele is associated with higher STAT4 mRNA and protein expression, indicating a stronger skewed immune response than the norm. Although widely studied in autoimmune disease patients and the general population, its effect on immunocompromised subjects is still unknown. Especially in situations, i.e. post-hematopoietic stem cell transplantation (post-HSCT), where control of the immune response is crucial...
February 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/27943079/assessment-of-type-i-interferon-signaling-in-pediatric-inflammatory-disease
#11
Gillian I Rice, Isabelle Melki, Marie-Louise Frémond, Tracy A Briggs, Mathieu P Rodero, Naoki Kitabayashi, Anthony Oojageer, Brigitte Bader-Meunier, Alexandre Belot, Christine Bodemer, Pierre Quartier, Yanick J Crow
PURPOSE: Increased type I interferon is considered relevant to the pathology of a number of monogenic and complex disorders spanning pediatric rheumatology, neurology, and dermatology. However, no test exists in routine clinical practice to identify enhanced interferon signaling, thus limiting the ability to diagnose and monitor treatment of these diseases. Here, we set out to investigate the use of an assay measuring the expression of a panel of interferon-stimulated genes (ISGs) in children affected by a range of inflammatory diseases...
December 9, 2016: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/27936517/-novel-biomarkers-for-the-assessment-of-pediatric-systemic-lupus-erythematosus-nephritis
#12
Artemis Koutsonikoli, Maria Trachana, Evangelia Farmaki, Vasiliki Tzimouli, Polyxeni Pratsidou-Gertsi, Nikoleta Printza, Alexandros Garyphallos, Vasiliki Galanopoulou, Florence Kanakoudi-Tsakalidou, Fotios Papachristou
OBJECTIVES: The discovery of serum biomarkers specific for pediatric Lupus Nephritis (pLN) will facilitate the non-invasive diagnosis, follow-up and more appropriate use of treatment. The aim of this study was to explore the role of serum High-Mobility-Group-Box-1 (HMGB1) protein, antibodies against nucleosomes (anti-NCS), complement factor C1q (anti-C1q) and glomerular basement membrane (anti-GBM) in pLN. METHODS: Serum samples of 42 patients with pediatric Systemic Lupus Erythematosus (pSLE) (22 with pLN and 20 without renal involvement), fifteen patients with other autoimmune nephritis (AN) and 26 healthy controls (HCs) were examined using ELISA...
December 9, 2016: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27921164/m-type-phospholipase-a2-receptor-pla2r-glomerular-staining-in-pediatric-idiopathic-membranous-nephropathy
#13
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
#14
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
#15
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
#16
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
#17
REVIEW
Scott E Wenderfer, Natasha M Ruth, Hermine I Brunner
The care of children with lupus nephritis (LN) has changed dramatically over the past 50 y. 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 y. 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...
January 4, 2017: Pediatric Research
https://www.readbyqxmd.com/read/27837194/lenalidomide-for-refractory-cutaneous-manifestations-of-pediatric-systemic-lupus-erythematosus
#18
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
#19
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
#20
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
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