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Henoch schonlein purpura in children

Dong-Feng Zhang, Guo-Xiang Hao, Chun-Zhen Li, Yan-Jun Yang, Fu-Juan Liu, Ling Liu, Xiao-Ying Yuan, Rui-Hong Li, Lei Dong, Qian Dong, Evelyne Jacqz-Aigrain, Wei Zhao
BACKGROUND: Tacrolimus is used off-label in the treatment of Henoch-Schönlein purpura nephritis (HSPN) in children, with limited evidence-based data. Based on clinical empirical experience and mechanism of action, tacrolimus might be promoted as treatment for childhood HSPN. The objectives of this pilot study were to assess its effectiveness and safety, and to explore the potential impact of CYP3A5 genotype. METHODS: Children with HSPN receiving tacrolimus as empirical treatment were included in this prospective, observational study...
March 13, 2018: Archives of Disease in Childhood
Xingcui Wang, Lei Zhang, Ying Wang, Xuemei Liu, Hongxia Zhang, Yi Liu, Nan Shen, Junjie Yang, Zhongtao Gai
BACKGROUND: Alterations in the intestinal microbiota have been associated with the development of allergic diseases, such as asthma and food allergies. However, there is no report detailing the role of microbiota alterations in Henoch-Schönlein Purpura (HSP) development. METHOD: A total of 85 children with HSP and 70 healthy children were recruited for this study. Intestinal microbiota composition was analyzed by 16S rRNA gene-based pyrosequencing. Fecal microbial diversity and composition were compared...
March 8, 2018: International Immunopharmacology
M Mossberg, M Segelmark, R Kahn, M Englund, A J Mohammad
OBJECTIVES: To estimate the annual incidence rate of paediatric primary systemic vasculitis (PSV) in a defined geographical area in southern Sweden. METHODS: Potential cases of PSV [IgA vasculitis (IgAV, Henoch-Schönlein purpura), Kawasaki disease (KD), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and Takayasu's arteritis (TAK)] were identified in a comprehensive regional healthcare register...
February 7, 2018: Scandinavian Journal of Rheumatology
Lin Sun, Biao Xie, Qiuju Zhang, Yupeng Wang, Xinyu Wang, Bing Gao, Meina Liu, Maoqing Wang
Background: In children with Henoch-Schonlein purpura (HSP), the severity of Henoch-Schonlein purpura nephritis (HSPN) is considered responsible for the prognosis of HSP. The pathological process from HSP to HSPN is not clear yet and current diagnostic tools have shortcomings in accurate diagnosis of HSPN. This study aims to assess clinical characteristics of HSP and HSPN, to identify metabolic perturbations involved in HSP progress, and to combine metabolic biomarkers and clinical features into a better prediction for HSPN...
December 26, 2017: Oncotarget
Scott E Wenderfer, Joseph P Gaut
Unique challenges exist in the diagnosis and treatment of glomerular diseases with their onset during childhood. Mounting evidence supports the notion that earlier onset cases occur due to larger numbers of genetic risk alleles. Nearly all causes of adult-onset glomerulonephritis, nephrotic syndrome, and thrombotic microangiopathy have also been described in children, although the prevalence of specific causes differs. Postinfectious glomerulonephritis, Henoch-Schönlein purpura nephritis, and minimal change disease remain the most common causes of glomerular disease in younger children in the United States and can be diagnosed clinically without need for biopsy...
November 2017: Advances in Chronic Kidney Disease
Nikita Lakdawala, Flavia Fedeles
Cutaneous vasculitis, inflammatory destruction of blood vessels, can present with a wide range of clinical and pathologic findings across a number of heterogeneous conditions. Although some vasculitides are present in both children and adults, some important differences exist in clinical presentation, etiology, management, and prognosis in childhood vasculitis versus adult vasculitis. Cutaneous vasculitis is rare in children, and most childhood vasculitides, of which Henoch-Schönlein purpura is the most common, histologically are small vessel leukocytoclastic vasculitis...
November 2017: Clinics in Dermatology
Evangeline Pillebout, Agnès Jamin, Hamza Ayari, Pierre Housset, Melissa Pierre, Virginia Sauvaget, Denis Viglietti, Georges Deschenes, Renato C Monteiro, Laureline Berthelot
Henoch-Schönlein purpura is a systemic vasculitis characterized by IgA deposits, which target the skin, joints, and kidneys, among other organs. In children, prognosis is often good but little is known about biomarkers of pediatric nephritis. We hypothesized that biological markers, including cytokines, immunoglobulins, IgA-immune complexes, IgA glycosylation and neutrophil gelatinase-associated lipocalin (NGAL), may discriminate IgA vasculitis (IgAV) pediatric patients with renal involvement from those without renal involvement...
2017: PloS One
Ke Xu, Lili Zhang, Jie Ding, Suxia Wang, Baige Su, Huijie Xiao, Fang Wang, Xuhui Zhong, Yanming Li
BACKGROUND: The widely used International Study of Kidney Disease in Children (ISKDC) classification for Henoch-Schönlein purpura nephritis (HSPN) does not completely correlate with the clinical presentation and long-term prognosis of this disease. Primary IgA nephropathy (IgAN) and HSPN share common features; thus, the Oxford classification of IgAN might be useful in predicting the long-term outcomes of HSPN. However, its value has not been confirmed in children with HSPN. METHODS: We selected children with HSPN diagnosed between 2003 and 2015, and reclassified their renal biopsies according to the Oxford classification scoring system...
April 2018: Journal of Nephrology
Agnes Hackl, Jan U Becker, Lisa M Körner, Rasmus Ehren, Sandra Habbig, Eva Nüsken, Kai-Dietrich Nüsken, Kathrin Ebner, Max C Liebau, Carsten Müller, Martin Pohl, Lutz T Weber
BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood and traditionally considered as a self-limiting disease. However, renal involvement can unfavorably determine long-term prognosis. The reported regimens to treat HSP nephritis (HSPN) are diverse, indicating that the most effective treatment remains controversial. METHODS: This retrospective, single-center study involved 18 patients presenting with HSPN and nephrotic-range proteinuria...
November 25, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Emel Isiyel, Kibriya Fidan, Bahar Buyukkaragoz, Meltem Akcaboy, Yasar Kandur, Ipek Isik Gonul, Necla Buyan, Sevcan Bakkaloglu, Oguz Soylemezoglu
Renal biopsy is an important diagnostic procedure in pediatric nephrology. This study retrospectively investigates the indications, results and complications in a single tertiary children's hospital in Turkey. We evaluated the native and transplant kidney biopsies in Gazi University Pediatric Nephrology Department between 2001 and 2015. A total of 196 biopsies (144 natives and 52 transplants) were included into the study. The mean age of the patients was respectively 10.8 ± 3.5, 13.9 ± 1.5 years...
November 2017: Renal Failure
Narangerel Purevdorj, Yun Mu, Yajun Gu, Fang Zheng, Ran Wang, Jinwei Yu, Xuguo Sun
OBJECTIVE: To explore a panel of serum biomarkers for laboratory diagnosis of pediatric Henoch-Schönlein purpura (HSP). METHODS: The blood white blood cells (WBC) and serum levels of serum amyloid A (SAA), interleukin 6 (IL-6), immunoglobulin A (IgA), immunoglobulin G (IgG), immunoglobulin M (IgM), immunoglobulin E (IgE), C-reactive protein (CRP), complement component 3 (C3), complement component 4 (C4), and ASO (anti-streptolysin O) were detected in 127 patients with Henoch-Schonlein purpura (HSP), 110 cases of septicemia patients, and 121 healthy volunteers...
February 2018: Clinical Biochemistry
Vikas Gupta, Amita Aggarwal, Ranjan Gupta, Abhra Chandra Chowdhury, Vikas Agarwal, Able Lawrence, Ramnath Misra
AIM: Henoch-Schönlein purpura (HSP), a primary vasculitis, characterized by purpura, abdominal pain, arthritis and renal involvement, is predominantly a disease of childhood. However, rarely it can occur in adults in whom it is believed to be a more severe form with poor renal outcomes. We aimed to answer if the age of onset affected the clinical spectrum and renal outcomes of the disease in a north Indian population. Hence, we studied the differences in clinical spectrum and renal outcomes between adult-onset HSP and childhood-onset HSP...
January 2018: International Journal of Rheumatic Diseases
Jana Volejnikova, Jaroslav Horacek, Frantisek Kopriva
BACKGROUND: Henoch-Schönlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries and by the deposition of IgA immune complexes. An association between HSP and atypical bacteria is uncommon in children. METHODS AND RESULTS: Here we report three cases of children, aged 5, 4 and 16 years, who were diagnosed with HSP associated with Mycoplasma pneumoniae or Chlamydia pneumoniae infection. In all presented cases, persistent cutaneous manifestations and abdominal pain were resistant to antibiotics and corticosteroids, but resolved during 48 h after the introduction of dapsone...
November 2, 2017: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
Emmanuel Ofori, Daryl Ramai, Mel A Ona, Charilaos Papafragkakis, Madhavi Reddy
Henoch-Schonlein purpura (HSP) is an immune-mediated vasculitis. HSP presents with purple spots on the skin (purpura), arthralgia, digestive problems, and kidney injury. HSP is most commonly seen in children, and rarely presents in adults. The pathogenesis involves the deposition of immune complexes in small to medium size blood vessels which leads to necrosis and inflammation. Most patients recover after symptomatic treatment, while more severe cases are treated with steroids. We report a 28-year-old female patient who presented with two episodes of hematemesis, worsening abdominal pain, and bloody diarrhea...
November 2017: Journal of Clinical Medicine Research
Xu Teng, Cuiyun Gao, Mei Sun, Jie Wu
The objective of this study is to explore the value of fecal calprotectin (FC) for early screening of the abdominal type of Henoch-Schonlein purpura (AHSP) in children. The study cohort included 40 children with AHSP treated at Shengjing Hospital of China Medical University from November 2014 to November 2015, and 40 children hospitalized in the Division of Pediatric Orthopedics in the corresponding period were selected as a control group. Fresh fecal samples were collected in the acute phase of the first visit (FC1), 3 days after treatment (FC2), and 7 days after treatment (FC3) from the AHSP group and the control group...
October 10, 2017: Clinical Rheumatology
X Zhu, M Zhang, F Lan, H Wei, Q He, S Li, X Qin
INTRODUCTION: Red blood cell distribution width (RDW) is elevated in various inflammatory diseases, but its clinical significance in Henoch-Schönlein purpura nephritis (HSPN) in unknown. The aim of this study was to determine the value of RDW as a risk factor or marker for HSPN in children. METHODS: This was a case-control study of 105 Henoch-Schönlein purpura (HSP) patients, 120 HSPN patients and 192 healthy controls. The relationship between RDW-coefficient of variation (RDW-CV) and the clinical characteristics of HSPN patients was determined by a multiple logistic regression analysis (MVLRA)...
October 9, 2017: British Journal of Biomedical Science
Imke Hennies, Charlotte Gimpel, Jutta Gellermann, Kristina Möller, Brigitte Mayer, Katalin Dittrich, Anja K Büscher, Matthias Hansen, Wiebke Aulbert, Elke Wühl, Richard Nissel, Gessa Schalk, Lutz T Weber, Michael Pohl, Simone Wygoda, Rolf Beetz, Günter Klaus, Henry Fehrenbach, Sabine König, Hagen Staude, Ortraud Beringer, Martin Bald, Ulrike Walden, Christian von Schnakenburg, Gunhard Bertram, Michael Wallot, Karsten Häffner, Thorsten Wiech, Peter F Hoyer, Martin Pohl
BACKGROUND: This study correlates the clinical presentation of Henoch-Schönlein purpura nephritis (HSPN) with findings on initial renal biopsy. METHODS: Data from 202 pediatric patients enrolled in the HSPN registry of the German Society of Pediatric Nephrology reported by 26 centers between 2008 and 2014 were analyzed. All biopsy reports were re-evaluated for the presence of cellular crescents or chronic pathological lesions (fibrous crescents, glomerular sclerosis, tubular atrophy >5%, and interstitial fibrosis >5%)...
February 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
D-Q Ma, Y Li, Z-G Han, M Zheng, N Xu, X-J Fan
OBJECTIVE: To investigate risk factors related to kidney injury in children with Henoch-Schonlein purpura (HSP) and to study the therapeutic effects of hemoperfusion (HP) on kidney injury in HSP children, providing clinical evidence for early prevention and treatment of HSP. PATIENTS AND METHODS: Children who suffered from HSP for the first time were selected as study objects and they were followed up for 12 months. Single factor analysis and multi-factor Logistic regression analysis were performed for children's demographic characteristics (age, gender), clinical manifestations (rash duration time, rash recurrence times, digestive tract hemorrhage, abdominal pain, arthralgia, HSP recurrence) and laboratory indexes (peripheral blood WBC, PLT, ESR, CRP, serum IgG, serum IgA, IgM, serum C3, serum C4, TC, TG, HDL, LDL)...
October 2017: European Review for Medical and Pharmacological Sciences
Hui Xu, Guizhen Jiang, Hongqiang Shen, Yanxiang Pan, Junfeng Zhang, Wei Li, Jianhua Mao
Interleukin-10 (IL-10), one of the anti-inflammatory cytokines, plays a major role in the pathogenesis of Henoch-Schönlein purpura (HSP). In present study, we investigated the association between genetic variation in IL-10 gene and susceptibility to HSP in a Chinese childhood population. Considering the overlapping clinical manifestations during the course of disease, the relation between IL-10 gene polymorphisms and HSP clinical heterogeneity was also assessed. We analyzed three IL-10 tag single nucleotide polymorphisms (SNPs; rs3021094, rs3790622, and rs1800872) using the Sequenom MassARRAY system by means of matrix-assisted laser desorption ionization-time of flight mass spectrometry method in 182 patients with HSP and 202 healthy controls...
December 2017: Clinical Rheumatology
F Almassinokiani, A Mehdizadeh Kashi, A Musavi, S Khodaverdi, K Tahermanesh, S Ariana
Henoch-Schönlein purpura (HSP) is caused by deposition of IgA-containing immune complexes within the blood vessels. HSP mostly occurs in children and is less common in adults. In addition to palpable purpura, arthritis or arthralgia, renal disease and abdominal pain, gastrointestinal symptoms occur in 50% of children. These can be caused by gastrointestinal hemorrhage, bowel ischemia and necrosis, intussusception, and bowel perforation. In adults, intussusception is less frequent than in children. We report a 42-year-old woman referred to our hospital with acute abdominal pain...
September 21, 2017: Reumatismo
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