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Schonlein Henoch Purpura

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
Yue Yang, Zheng Zhang, Li Zhuo, Da-Peng Chen, Wen-Ge Li
Background: Chronic kidney disease has become a leading public health concern in China, as it is associated with increased morbidity, mortality, and costs. However, the overall situation regarding common glomerular diseases in China remains unclear. Hence, the aim of this study was to assess the national profile of the common types of glomerulonephritis in China. Methods: We searched Medline, Embase, Cochrane Library, CNKI, SinoMed, VIP, and Wanfang databases for English and Chinese language articles from inception to September 2017...
March 20, 2018: Chinese Medical Journal
Annamaria Milillo, Clelia Molinario, Stefano Costanzi, Gisella Vischini, Francesca La Carpia, Francesco La Greca, Donato Rigante, Giovanni Gambaro, Fiorella Gurrieri, Eugenio Sangiorgi
Studies on IgA nephropathy (IgAN) have identified, through GWAS, linkage analysis, and pathway scanning, molecular defects in familial and sporadic IgAN patients. In our previous study, we identified a novel variant in the SPRY2 gene that segregates with the disease in one large family. The functional characterization of this variant led us to discover that the MAPK/ERK pathway was defective not only in this family, but also in two sporadic IgAN patients wild type for SPRY2. In the present study, we have deepened the molecular analysis of the MAPK/ERK pathway and extended our evaluation to a larger cohort of sporadic patients and to one additional family...
March 1, 2018: Journal of Nephrology
Imen Gargouri, Ameni Kacem, Halima Ben Salem, Imen El Mjendel, Sana Aissa, Abdelhamid Garrouche, Abdelaziz Hayouni, Ahmed Abdelghani, Mohamed Benzarti
No abstract text is available yet for this article.
April 2017: La Tunisie Médicale
Yingrou Tan, Jackson Liang Li, Bernett Teck Kwong Lee, Josephine Lum, Zacharias Aloysius Dwi Pramono, Maximilien Evrard, Michael Poidinger, Hong Liang Tey, Lai Guan Ng
No abstract text is available yet for this article.
February 28, 2018: Acta Dermato-venereologica
Ivka Djakovic, Drazan Butorac, Zeljko Vucicevic, Vesna Kosec, Andrea Tesija Kuna, Liborija Lugović-Mihić
Henoch-Schönlein purpura (HSP) is an IgA-mediated small vessels' vasculitis that affects the skin, intestines and kidneys. Pregnancy has been reported as an exacerbating factor. We present the case of a 24-year-old primigravida with HSP that occurred in the third trimester and lasted up to the end of the successful delivery. She had pruritic maculopapular exanthema on her legs. Biopsy of a cutaneous lesion was performed for histopathologic features and direct immunofluorescence (DIF) for the presence of perivascular IgA deposition...
February 15, 2018: Biochemia Medica: časopis Hrvatskoga Društva Medicinskih Biokemičara
Vitorino Modesto Dos Santos, Sergio Luiz da Costa
No abstract text is available yet for this article.
October 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
Jakub Zieg, Sarka Stolbova, Veronika Kroulikova, Jaromir Hacek
No abstract text is available yet for this article.
April 2018: Minerva Pediatrica
Christine Y Louie, Adam J Gomez, Richard K Sibley, Dorsey Bass, Teri A Longacre
Immunoglobulin A (IgA) vasculitis or Henoch-Schönlein purpura (HSP) typically occurs in the pediatric population, although rare cases also occur in adults. Gastrointestinal (GI) involvement is common. The "classic" histologic finding in IgA vasculitis (HSP) is leukocytoclastic vasculitis (LCV); other histologic features in biopsies of IgA vasculitis (HSP) have only been rarely described. The pathology archival files at our institution were searched for GI biopsies from patients with IgA vasculitis (HSP)...
February 12, 2018: American Journal of Surgical Pathology
Caroline Chanussot-Deprez, María Elisa Vega-Memije, Luis Flores-Suárez, Celia Ríos-Romero, Javier Cabiedes-Contreras, Edgardo Reyes, Lucia Rangel-Gamboa
Objetivo: Las vasculitis cutáneas (VC), primarias o como manifestación de enfermedades sistémicas, constituyen un reto diagnóstico. Determinar las causas de VC. Métodos: Se incluyeron pacientes con diagnóstico de CV, a los cuales se les realizó valoración clínica, biopsia cutánea y exámenes de laboratorio. En la mayoría de los casos se realizó inmunofluorescencia directa. Los casos se clasificaron con los criterios del American College of Rheumatology (ACR) y la Chapel Hill Consensus Conference (CHCC)...
2018: Gaceta Médica de México
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
Izabel M Buscatti, Beatriz B Casella, Nadia E Aikawa, Andrea Watanabe, Sylvia C L Farhat, Lucia M A Campos, Clovis Artur Silva
One of the author's name on this article was incorrectly spelled as "Sylvia C. L. Fahrat" . The correct spelling is "Sylvia C. L. Farhat" and is now presented correctly in this article. The original article has been corrected.
January 29, 2018: Clinical 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
Cristina Guerriero, Gaia Moretta, Giulia Bersani, Piero Valentini, Antonio Gatto, Donato Rigante
Background: Necrotizing vasculitides are basically characterized by vessel wall neutrophil infiltration and necrosis and they can occur as a primary process or secondary to an underlying disease. Although Henoch-Schönlein purpura (HSp) is the more frequent primary vasculitis in childhood, sometimes it has to be distinguished from other secondary vasculitides induced by infections, drugs, vaccines, or immune-mediated disorders. Main observations: We report a case of a 14-year-old girl with cutaneous necrotizing vasculitis, appearing in the course of acute Epstein-Barr virus infection...
December 1, 2017: Journal of Dermatological Case Reports
Raquel López-Mejías, Santos Castañeda, Fernanda Genre, Sara Remuzgo-Martínez, F David Carmona, Javier Llorca, Ricardo Blanco, Javier Martín, Miguel A González-Gay
Immunoglobulin-A vasculitis (IgAV) is classically a childhood small-sized blood vessel vasculitis with predominant involvement of the skin. Gastrointestinal and joint manifestations are common in patients diagnosed with this condition. Nephritis, which is more severe in adults, constitutes the most feared complication of this vasculitis. The molecular bases underlying the origin of IgAV have not been completely elucidated. Nevertheless, several pieces of evidence support the claim that genes play a crucial role in the pathogenesis of this disease...
January 15, 2018: Autoimmunity Reviews
Izabel M Buscatti, Beatriz B Casella, Nadia E Aikawa, Andrea Watanabe, Sylvia C L Farhat, Lucia M A Campos, Clovis Artur Silva
The objective of this study was to evaluate prevalence, initial risk factors, and outcomes in Henoch-Schönlein purpura nephritis (HSPN) patients in Latin America. Two hundred ninety-six patients (validated EULAR/PRINTO/PRES HSP criteria) were assessed by demographic data, clinical/laboratorial involvements, and treatments in the first 3 months after diagnosis. They were followed-up in a Latin American tertiary center and were divided in two groups: with and without nephritis. Persistent non-nephrotic proteinuria, nephrotic proteinuria, and acute/chronic kidney injury were also systematically evaluated at 1, 5, 10, and 15 years after diagnosis...
January 13, 2018: Clinical Rheumatology
Jessica St John, Priyanka Vedak, Anna Cristina Garza-Mayers, Mai P Hoang, Sagar U Nigwekar, Daniela Kroshinsky
BACKGROUND: Henoch-Schönlein purpura (HSP) is a small vessel IgA-predominant vasculitis. OBJECTIVE: To describe adult patients with HSP and determine if the distribution of skin lesions (ie, purpura above the waist or purpura below the waist only), is a predictor of significant renal involvement at the time of the skin biopsy and the months following. METHODS: A retrospective study on renal function from 72 adult patients with skin-biopsy proven HSP...
January 2018: Journal of the American Academy of Dermatology
Yoshiko Mori, Tomohiro Masuda, Tomoki Kosugi, Tomoki Yoshioka, Mayuko Hori, Hiroshi Nagaya, Kayaho Maeda, Yuka Sato, Hiroshi Kojima, Noritoshi Kato, Takuji Ishimoto, Takayuki Katsuno, Yukio Yuzawa, Kenji Kadomatsu, Shoichi Maruyama
BACKGROUND: Precise understanding of kidney disease activity is needed to design therapeutic strategies. CD147/basigin is involved in the pathogenesis of acute kidney injury and renal fibrosis through inflammatory cell infiltration. The present study examined the clinical relevance of CD147 in biopsy-proven kidney diseases that lead to the progression of chronic kidney disease. METHODS: Kidney biopsy specimens and plasma and urine samples were obtained from patients with kidney diseases, including IgA nephropathy (IgAN), Henoch-Schönlein purpura nephritis (HSPN), diabetic kidney disease (DKD), focal segmental glomerulosclerosis (FSGS), and membranous nephropathy (MN), who underwent renal biopsy between 2011 and 2014...
December 12, 2017: Clinical and Experimental Nephrology
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
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