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Pediatric vasculitis

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https://www.readbyqxmd.com/read/28516235/clinical-imaging-and-genotypical-features-of-three-deceased-and-five-surviving-cases-with-ada2-deficiency
#1
Sezgin Sahin, Amra Adrovic, Kenan Barut, Serdal Ugurlu, Eda Tahir Turanli, Huri Ozdogan, Ozgur Kasapcopur
Deficiency of adenosine deaminase type 2 (DADA2) is a rare form of autoinflammatory disorder with limited reported cases. In this paper, we have presented the clinico-immunological, radiological and genetic characteristics of five surviving and three deceased childhood-onset DADA2 patients. We aimed to compare surviving and deceased patients in terms of clinical features and treatment modalities. Moreover, we have evaluated the causes of death in our DADA2 subjects together with the previously reported cases...
May 17, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28503078/variations-in-the-clinical-practice-of-physicians-managing-takayasu-arteritis-a-nationwide-survey
#2
Lillian Barra, Patrick Liang, Susanne M Benseler, David A Cabral, Aurore Fifi-Mah, Yueyang Li, Nataliya Milman, Marinka Twilt, Elaine Yacyshyn, Christian Pagnoux
OBJECTIVE: Takayasu arteritis (TAK) is a large vessel vasculitis that predominately affects young women and can cause severe ischemic complications. Given the rarity of TAK, the management of this condition is challenging. We aim to describe current rheumatologist practices for the management of TAK and identify discrepancies and gaps in knowledge. METHODS: An online survey (developed by the Canadian Vasculitis Network and approved by the Canadian Rheumatology Association) containing 48 questions with regard to the diagnosis, monitoring and treatment of TAK was distributed to 495 Canadian adult and pediatric rheu-matologists by email...
2017: Open Access Rheumatology: Research and Reviews
https://www.readbyqxmd.com/read/28499760/henoch-sch%C3%A3-nlein-purpura-with-testicular-necrosis-sonographic-findings-at-the-onset-during-treatment-and-at-follow-up
#3
Lixia Zhao, Shuguang Zheng, Xiaojun Ma, Weihua Yan
Henoch-Schönlein purpura with involvement of the testes is extremely rare. Here, we present a pediatric case of Henoch-Schönlein purpura involving testicular ischemic necrosis in an 8-year-old child. Ultrasonography plays an important role not only in the differential diagnosis at onset, but also in the assessment of treatment response and prognosis during treatment and at follow-up. In this case report, we present the sonographic images for the entire course of testicular involvement in HSP, and revealed histopathologically the pathogeny as testicular autoimmune vasculitis caused by the deposition of immunoglobulin A-containing immune complexes in the testicular vessels...
May 9, 2017: Urology
https://www.readbyqxmd.com/read/28470578/acute-hemorrhagic-edema-of-infancy-the-experience-of-a-large-tertiary-pediatric-center-in-israel
#4
Limor Parker, Keren Shahar-Nissan, Liat Ashkenazi-Hoffnung, Liora Harel, Jacob Amir, Omer Trivizki, Efraim Bilavsky
BACKGROUND: Acute hemorrhagic edema of infancy (AHEI) is a rare leukocytoclastic vasculitis of the small vessels occurring at a young age and considered as a benign self-limited disease. Due to its low prevalence, there are limited data on the presentation and complications of this disease. METHODS: All computerized files of children who were hospitalized at a tertiary pediatric center due to AHEI over a 10 year period were reviewed. Clinical, laboratory and histopathological data were collected...
April 29, 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28461649/canadian-rheumatology-association-meeting-the-westin-ottawa-ottawa-ontario-canada-february-8-11-2017
#5
Earl D Silverman
The 72nd Annual Meeting of The Canadian Rheumatology Association (CRA) was held at The Westin Ottawa, Ottawa, Ontario, Canada, February 8-11, 2017. The program consisted of presentations covering original research, symposia, awards, and lectures. Highlights of the meeting include the following 2017 award winners: Dr. Vinod Chandran, Young Investigator; Dr. Jacques P. Brown, Distinguished Investigator; Dr. David Robinson, Teacher-Educator; Dr. Michel Zummer, Distinguished Rheumatologist; Ms. Rebecca Gole, Best Abstract on SLE Research by a Trainee - Ian Watson Award; Ms...
May 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28438265/autoinflammatory-diseases-in-pediatric-dermatology-part-2-histiocytic-macrophage-activation-and-vasculitis-syndromes
#6
S Hernández-Ostiz, G Xirotagaros, L Prieto-Torres, L Noguera-Morel, A Torrelo
The discovery of new autoinflammatory syndromes and novel mutations has advanced at breakneck speed in recent years. Part 2 of this review focuses on vasculitis syndromes and the group of histiocytic and macrophage activation syndromes. We also include a table showing the mutations associated with these autoinflammatory syndromes and treatment alternatives.
April 21, 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/28437291/behcet-disease-initially-presenting-as-deep-venous-thrombosis-a-case-report
#7
Arun Gurunathan, David Teachey, Kudakwashe R Chikwava, Char Witmer, Ami V Desai
Behcet disease is a potentially life-threatening multisystemic vasculitis with thrombotic tendency. Mucocutaneous ulcers, arthritis, and uveitis are the most recognizable features, but may be absent at the time of medical evaluation. We report a case in which a 8-year old patient presented with spontaneous bilateral lower extremity deep venous thromboses, and screening for rheumatologic symptoms led to diagnosing Behcet. This case demonstrates that deep venous thromboses can be the initial event bringing a patient with Behcet to medical attention, highlighting the importance of screening for underlying rheumatologic diseases in pediatric patients who present with unprovoked thrombosis...
April 21, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28427414/observational-study-of-interleukin-21-il-21-does-not-distinguish-kawasaki-disease-from-other-causes-of-fever-in-children
#8
Rachel Engelberg, Meghan Martin, Brian H Wrotniak, Mark Daniel Hicar
BACKGROUND: Kawasaki disease (KD) is a febrile childhood vasculitis of unknown etiology. The diagnosis is highly concerning as over a quarter of children who fail to receive timely treatment with intravenous immunoglobulin (IVIG) will develop coronary aneurysms. Diagnosis relies on proper symptomatology and is supported by non-specific markers of inflammation. Previous studies have identified elevated plasma levels of interleukin-21 (IL-21) as a sensitive and specific biomarker in KD...
April 20, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28420068/pentraxin-3-levels-are-associated-with-vasculitis-and-disease-activity-in-childhood-onset-systemic-lupus-erythematosus
#9
S Sahin, A Adrovic, K Barut, S Durmus, R Gelisgen, H Uzun, O Kasapcopur
Objectives Childhood-onset systemic lupus erythematosus (cSLE) is a multisystemic autoimmune disease characterized by inflammatory organ damage by means of vasculitis. Pentraxin-3 (PTX3) is expressed locally at the sites of inflammatory processes, predominantly from endothelial cells. In adult studies, PTX3 has shown to be an indicator of active vasculitis both in large-vessel and small-vessel vasculitides, as well as in SLE. Moreover, in SLE it has found to be correlated with disease activity, and with some of the clinical manifestations and laboratory parameters...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28416004/clinical-study-of-children-with-takayasu-arteritis-a-retrospective-study-from-a-single-center-in-china
#10
Ye Feng, Xuemei Tang, Mingyue Liu, Juan Zhou, Xiaodong Zhao, Qiu Li
BACKGROUND: Delayed diagnosis of childhood Takayasu arteritis (TA) is common due to its atypical symptoms. The objective of the present study was to summarize the clinical features of childhood TA to raise awareness and improve management. METHODS: Eleven children diagnosed with TA at our hospital were enrolled. Clinical information, diagnosis, treatment, and outcome were then examined retrospectively. The Pediatric Vasculitis Activity Score (PVAS) and the Indian Takayasu Clinical Activity Score (ITAS2010) were used to assess disease activity...
April 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28410589/lung-involvement-in-childhood-onset-granulomatosis-with-polyangiitis
#11
REVIEW
Giovanni Filocamo, Sofia Torreggiani, Carlo Agostoni, Susanna Esposito
Granulomatosis with polyangiitis is an ANCA-associated systemic vasculitis with a low incidence in the pediatric population. Lung involvement is a common manifestation in children affected by granulomatosis with polyangiitis, both at disease's onset and during flares. Its severity is variable, ranging from asymptomatic pulmonary lesions to dramatic life-threatening clinical presentations such as diffuse alveolar haemorrhage. Several radiologic findings have been described, but the most frequent abnormalities detected are nodular lesions and fixed infiltrates...
April 14, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28371513/early-outcomes-in-children-with-antineutrophil-cytoplasmic-antibody-anca-associated-vasculitis-aav
#12
Kimberly A Morishita, Lakshmi N Moorthy, Joanna M Lubieniecka, Marinka Twilt, Rae S M Yeung, Mary B Toth, Susan Shenoi, Goran Ristic, Susan M Nielson, Raashid A Luqmani, Suzanne C Li, Tzielan Lee, Erica F Lawson, Mikhail M Kostik, Marisa Klein-Gitelman, Adam M Huber, Aimee O Hersh, Dirk Foell, Melissa E Elder, Barbara A Eberhard, Paul Dancey, Sirirat Charuvanij, Susanne M Benseler, David A Cabral
Objective To characterize early disease course in childhood onset antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) and 12-month outcomes. Methods Eligible subjects were children diagnosed with GPA, MPA, EGPA, and ANCA-positive pauci-immune glomerulonephritis before their eighteenth birthday and entered into The Pediatric Vasculitis Initiative (PedVas) study. The primary outcome was remission (Pediatric Vasculitis Activity Score (PVAS) = 0 with corticosteroid dose (CS) <0.2mg/kg/day) at 12-months...
March 28, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28365793/a-child-with-x-linked-agammaglobulinemia-and-kawasaki-disease-an-unusual-association
#13
REVIEW
Dhrubajyoti Sharma, Sandesh Guleria, Deepti Suri, Amit Rawat, Ravinder Garg, Surjit Singh
An association of X-linked agammaglobulinemia (XLA) with Kawasaki disease (KD) is very uncommon. Only two case reports are available so far in pediatric literature. Patients with XLA have recurrent infections and physical examination have absent lymph nodes and tonsils. Laboratory investigations reveal hypogammaglobulinemia and reduced or absent B cells on flow cytometry. KD is a medium vessel vasculitis. Here, we report a 12 year old boy with X-linked agammaglobulinemia on regular replacement intravenous immunoglobulin who developed KD on follow-up...
April 1, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28325522/giant-aneurysms-a-gender-specific-complication-of-kawasaki-disease
#14
Sanne M Dietz, Irene M Kuipers, Carline E A Tacke, Jeffrey C D Koole, Barbara A Hutten, Taco W Kuijpers
BACKGROUND: Kawasaki disease (KD) is a pediatric vasculitis of unknown origin. Its main complication is the development of coronary artery aneurysms (CAA) with giant CAA at the end of the spectrum. METHODS: In this cohort study, we evaluated the association between patient characteristics and the development of giant CAA based on z-scores. Multivariable, multinomial logistic regression analysis was used to identify variables associated with giant CAA. RESULTS: A total of 301 KD patients, comprising 216 patients without enlargement, 45 with small-sized, 19 with medium-sized, and 21 with giant CAA with all echocardiographies at our center were retrospectively included...
March 18, 2017: Journal of Cardiology
https://www.readbyqxmd.com/read/28318198/-subglottic-stenosis-as-the-initial-manifestation-of-wegener-s-granulomatosis-in-a-teenager-case-report
#15
Giselle Cuestas, Verónica Rodríguez, Flavia Doormann, Alejandra Pringe, Patricio Bellia Munzón, Gastón Bellia Munzón, Carlos Ortega, Rubén Álvarez
Wegener's granulomatosis is a necrotizing granulomatous vasculitis of autoimmune origin that primarily affects the upper and lower airways and kidneys. It is very rare in children and adolescents. When started at a young age it is often associated with subglottic stenosis. Subglottic stenosis is a potentially fatal manifestation of Wegener's granulomatosis. Its diagnosis requires a high index of suspicion since it might develop in the absence of other signs of activity. Occasionally, subglottic stenosis may present as the initial manifestation of the disease...
April 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28316855/central-nervous-system-involvement-in-henoch-schonlein-purpura-in-children-and-adolescents
#16
Iliyana H Pacheva, Ivan S Ivanov, Krastina Stefanova, Elena Chepisheva, Lyubov Chochkova, Dafina Grozeva, Angelina Stoyanova, Stojan Milenkov, Penka Stefanova, Anna Petrova
Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009-2013). Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28285838/incidental-findings-on-pediatric-abdominal-magnetic-resonance-angiography
#17
Nattinee Leelakanok, Matthew A Zapala, Emily A Edwards, Andrew S Phelps, John D Mackenzie, Jesse Courtier
RATIONALE AND OBJECTIVES: Abdominal magnetic resonance angiography (MRA) has gained favor in pediatric patients owing to its lack of ionizing radiation and noninvasive nature. Reports exist regarding incidental findings on body MRA in adult patients. However, the incidental findings in pediatric abdominal MRA have not been previously reported. Our study aims to determine the frequencies, characteristics, and categories of incidental findings in pediatric patients undergoing abdominal MRA...
March 9, 2017: Academic Radiology
https://www.readbyqxmd.com/read/28261032/eosinophilic-colitis-in-children
#18
Urszula Grzybowska-Chlebowczyk, Sabina Horowska-Ziaja, Maciej Kajor, Sabina Więcek, Wojciech Chlebowczyk, Halina Woś
INTRODUCTION: Eosinophilic colitis, which is a rare form of eosinophilic gastrointestinal diseases, occurs as primary and secondary allergic eosinophilic colitis of the gastrointestinal tract infection, inflammatory bowel disease, celiac disease, and vasculitis. The diagnosis is based on a significant amount of eosinophils in the inflammatory infiltrate of the colon wall. AIM: To analyze the clinical picture taking into account comorbidities and endoscopic picture in children with eosinophilic colitis...
February 2017: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/28251502/avoiding-rash-conclusions-challenge-of-iga-vasculitis-in-adults
#19
S Hu, A Birg, M Hovaida, M W Gavin, D McCarthy
IgA vasculitis is primarily a pediatric disease that is rarely encountered in adults. With adults, gastrointestinal manifestations are quite common, yet are nonspecific and may overlap with other diseases, particularly Crohn's disease, which can make the diagnosis a challenging task. Treatment is controversial given the disease course is usually self-limited with few serious complications. We present a case of IgA vasculitis in an adult patient with limited extraintestinal findings illustrating the complexity of arriving at the correct diagnosis...
March 1, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28238636/-kawasaki-disease-is-more-prevalent-in-rural-areas-of-catalonia-spain
#20
Judith Sánchez-Manubens, Jordi Antón, Rosa Bou, Estibaliz Iglesias, Joan Calzada-Hernandez, Xavier Rodó, Josep-Antón Morguí
INTRODUCTION: Kawasaki disease (KD) is an acute self-limited systemic vasculitis relatively common in childhood. The etiology of KD is still unknown, although clinical, laboratory and epidemiological features suggest an infectious origin or trigger. Differences on incidence between countries have been related to specific genetic factors, ethnicity, country of birth and some other sociocultural and environmental factors. We present a population-based study on incidence of KD in Catalonia (Spain), focusing on differences between patients in rural and non-rural areas of the region...
February 23, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
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