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Journals Pediatric Rheumatology Online ...

Pediatric Rheumatology Online Journal

https://read.qxmd.com/read/38238744/psoriatic-arthritis-and-covid-19-a-new-challenge-for-rheumatologists-and-dermatologists
#21
LETTER
Zohreh Jadali
COVID-19 has changed the global health system and has great impact on different types of medical specialties including, dermatology and rheumatology. This point is important because although these two specialties are distinct subfields of medicine, there is some overlap between them. The overlap can be described by a number of rheumatic diseases that have cutaneous manifestations and vice versa. A good example of this is psoriatic arthritis because, in up to 42% of people, cutaneous lesions and arthritis coexist...
January 18, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38238724/monogenic-systemic-lupus-erythematosus-onset-in-a-13-year-old-boy-with-noonan-like-syndrome-a-case-report-and-literature-review
#22
JOURNAL ARTICLE
Patricia Morán-Álvarez, Alessandra Gianviti, Francesca Diomedi-Camassei, Monia Ginevrino, Fabrizio de Benedetti, Claudia Bracaglia
BACKGROUND: Childhood systemic lupus erythematosus (cSLE) has been considered as a polygenic autoimmune disease; however, a monogenic lupus-like phenotype is emerging with the recent recognition of several related novel high-penetrance genetic variants. RASopathies, a group of disorders caused by mutations in the RAS/MAPK pathway, have been recently described as a cause of monogenic lupus. CASE PRESENTATION: We present a 13-year-old boy with Noonan-like syndrome with loose anagen hair who developed a monogenic lupus...
January 18, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38229098/shaping-the-future-of-pediatric-rheumatology
#23
EDITORIAL
Tadej Avčin, Angelo Ravelli
No abstract text is available yet for this article.
January 16, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38225660/correction-transition-readiness-among-finnish-adolescents-with-juvenile-idiopathic-arthritis
#24
Katriina Mikola, Katariina Rebane, Hannu Kautiainen, Kristiina Aalto
No abstract text is available yet for this article.
January 15, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38212775/longitudinal-follow-up-of-mixed-connective-tissue-disease-and-overlapping-autoimmune-diseases-of-childhood-onset-in-the-afro-descendant-population-of-the-french-west-indies
#25
JOURNAL ARTICLE
Arthur Felix, Lindsay Osei, Frederique Delion, Benoit Suzon, Aurore Abel, Moustapha Drame, Yves Hatchuel, Christophe Deligny, Fabienne Louis-Sidney
INTRODUCTION: Overlap autoimmune syndromes (OAS) and mixed connective tissue disease (MCTD) are rare in children. We performed a retrospective, longitudinal and descriptive study of Afro-Caribbean patients from the French West Indies followed for MCTD and OAS to describe their characteristics and outcomes during childhood. METHODS: Retrospective study from January 2000 to 2023. Listings of patients were obtained from multiple sources: computerized hospital archives and national hospital-based surveillance system, registry of pediatricians and adult specialists in internal medicine and the national registry for rare diseases...
January 11, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38183114/the-4th-nextgen-therapies-for-sjia-and-mas-part-1-the-elephant-in-the-room-diagnostic-classification-criteria-for-systemic-juvenile-idiopathic-arthritis-and-adult-onset-still-s-disease
#26
JOURNAL ARTICLE
Peter A Nigrovic, Fabrizio de Benedetti, Yukiko Kimura, Daniel J Lovell, Sebastiaan J Vastert
Currently, the criteria used to classify patients with SJIA are different from those used for AOSD. However, it has been recognized that the existing terms are too narrow, subdividing the Still's population unnecessarily between pediatric-onset and adult-onset disease and excluding an appreciable group of children in whom overt arthritis is delayed or absent. Government regulators and insurers rely upon the guidance of subject experts to provide disease definitions, and when these definitions are flawed, to provide new and better ones...
January 5, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38183098/the-4th-nextgen-therapies-for-sjia-and-mas-part-2-phenotypes-of-refractory-sjia-and-the-landscape-for-clinical-trials-in-refractory-sjia
#27
JOURNAL ARTICLE
Grant Schulert, Sebastiaan J Vastert, Alexei A Grom
Although the introduction of the IL-1 and IL-6 inhibiting biologics in 2012 has revolutionized the treatment and markedly improved outcomes for many patients with SJIA, about 20% of these patients continue to have active disease, have markedly decreased quality of life and high disease activity as well as treatment-related morbidity and mortality. There is a clear need to define these disease states, and then use these definitions as the basis for further studies into the prevalence, clinical features, and pathophysiologic mechanisms...
January 5, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38183096/part-5-allogeneic-hsct-in-refractory-sjia-with-lung-disease-recent-cases-from-centers-in-north-america-europe
#28
JOURNAL ARTICLE
Alexei A Grom, Scott W Canna, Rolla F Abu-Arja, Rashmi Sinha, Luciana Peixoto, Elvira Cannizzaro, Shanmuganathan Chandrakasan, Kyla Driest, Rebecca Marsh, Bénédicte Neven, Karen Onel, Sampath Prahalad, Susan Prockop, Pierre Quartier, Johannes Roth, Grant Schulert, Juliana M F Silva, Donna Wall, Ulrike Zeilhofer
It has been increasingly recognized that there is a subset of patients with refractory systemic JIA, who have failed all available medications and may benefit from HSCT. The increasing experience with HSCT in SJIA, suggests that despite the complicated post-HSCT course, short-term, the transplanted patients either achieved SJIA remission or reduced burden of disease. Longer follow-up, however, is needed to better define the long-term outcomes. The discussion at the NextGen 2022 conference was focused on the optimal timing for the procedure, the need for a good control of inflammatory SJIA activity prior to HSCT, and the role of the reduced intensity conditioning regimens as there was a remote concern that such regimens might increase the risk of SJIA relapse after the transplantation...
January 5, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38183093/proceedings-from-the-4-th-nextgen-therapies-for-sjia-and-mas-virtual-symposium-held-february-13-14-2022
#29
JOURNAL ARTICLE
Rashmi Sinha, Fabrizio De Benedetti, Alexei A Grom
No abstract text is available yet for this article.
January 5, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38183056/the-4-th-nextgen-therapies-of-sjia-and-mas-part-4-it-is-time-for-il-18-based-trials-in-systemic-juvenile-idiopathic-arthritis
#30
JOURNAL ARTICLE
Scott W Canna, Fabrizio De Benedetti
Since IL-18 has recently emerged as a biomarker associated with refractory disease course in SJIA, the focus of the discussion was the feasibility of the biomarker-driven drug development to SJIA. Overall, there was broad agreement on the conclusion that IL-18 is a uniquely specific biomarker for many of the subsets of SJIA most in need of new therapies, and it may define a class of diseases mediated by IL-18 excess. The consensus was that leveraging IL-18 remains our most promising "lead" for use in refractory SJIA as it may mechanistically explain the disease pathophysiology and lead to more targeted therapies...
January 5, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38183052/a20-haploinsufficiency-in-a-neonate-caused-by-a-large-deletion-on-chromosome-6q
#31
REVIEW
Fan Zhang, Liang Zhang
Haploinsufficiency of A20 (HA20) is a rare monogenic disease caused by heterozygous loss-of-function mutations in the tumor necrosis factor alpha-induced protein 3 (TNFAIP3) gene located on chromosome 6q23.3. The majority of disease-causing mutations in most cases of HA20 comprise single nucleotide variations, small insertions, or deletions in TNFAIP3, which result in a premature termination codon and subsequent disruption of its anti-inflammatory role. Large deletions have been reported sporadically. HA20 patients may present with a variety of autoinflammatory and autoimmune features during early childhood; however, cases with neonatal onset are rare...
January 5, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38183044/mental-comorbidities-in-adolescents-and-young-adults-with-juvenile-idiopathic-arthritis-an-analysis-of-german-nationwide-health-insurance-data
#32
JOURNAL ARTICLE
Florian Milatz, Katinka Albrecht, Kirsten Minden, Ursula Marschall, Jens Klotsche, Johanna Callhoff
BACKGROUND: Studies on prevalence rates of mental comorbidities in patients with juvenile idiopathic arthritis (JIA) have reported varying results and provided limited information on related drugs. The purpose of this study was to determine the prevalence of selected mental health diagnoses and the range of associated drug prescriptions among adolescents and young adults (AYA) with JIA compared with general population controls. FINDINGS: Nationwide statutory health insurance data of the years 2020 and 2021 were used...
January 5, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38183017/tocilizumab-for-treating-mevalonate-kinase-deficiency-and-tnf-receptor-associated-periodic-syndrome-a-case-series-and-literature-review
#33
REVIEW
Yandie Li, Meiping Lu
BACKGROUND: Mevalonate kinase deficiency (MKD) and TNF receptor-associated periodic syndrome (TRAPS) are categorized as systemic autoinflammatory diseases (SAIDs), which are rare diseases characterized by early onset, severe conditions, and challenging diagnosis and treatment. Although different SAIDs have varying standard treatments, some SAIDs are poorly controlled after routine treatment, seriously affecting the growth and development of children and their quality of life. This study aims to provide more treatment strategies for SAIDs...
January 5, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38178123/juvenile-idiopathic-recurrent-parotitis-jirp-treated-with-short-course-steroids-a-case-series-study-and-one-decade-follow-up-for-potential-autoimmune-disorder
#34
LETTER
Farhad Salehzadeh, Rasol Molatefi, Ali Mardi, Negin Nahanmoghaddam
BACKGROUND: Juvenile idiopathic recurrent parotitis (JIRP) in children is a condition characterized with recurrent episodes of idiopathic parotid gland inflammation. Since there are no definitive guidelines for diagnosis and management of this condition, we present a consecutive case series of patients with more than one decade follow up and their dramatic response to short course treatment by prednisolone. METHODS: We conducted this study by retrospectively reviewed medical charts of children who were diagnosed with JIRP, from 1 January 2002 to 29 February 2023...
January 4, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38178067/a-rare-manifestation-of-sting-associated-vasculopathy-with-onset-in-infancy-a-case-report
#35
JOURNAL ARTICLE
Sophia Weidler, Sarah Koss, Christine Wolf, Nadja Lucas, Jürgen Brunner, Min Ae Lee-Kirsch
BACKGROUND: STING-associated vasculopathy with onset in infancy (SAVI) is a rare type I interferonopathy caused by heterozygous variants in the STING gene. In SAVI, STING variants confer a gain-of-function which causes overactivation of type I interferon (IFN) signaling leading to autoinflammation and various degrees of immunodeficiency and autoimmunity. CASE PRESENTATION: We report the case of a 5 year old child and his mother, both of whom presented with systemic inflammatory symptoms yet widely varying organ involvement, disease course and therapeutic response...
January 4, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38172909/the-4-th-nextgen-therapies-for-sjia-and-mas-part-3-clinical-trials-in-refractory-sjia-historic-controls-as-an-alternative-to-a-withdrawal-design-study
#36
JOURNAL ARTICLE
Fabrizio de Benedetti, Alexei A Grom, Hermine Brunner
The substantial morbidity and mortality associated with refractory systemic JIA underlies the need for new treatment approaches. However, progress in this area has been limited by the difficulty of enrolling these patients in clinical trials with traditional designs, particularly in patients presenting with the life-threatening macrophage activation syndrome. At the NextGen 2022 conference, there was group consensus that using historical cohorts as a control group to avoid the need for a placebo-arm or drug withdrawal was highly desirable and might be acceptable for clinical trials in MAS to support medication efficacy and safety...
January 3, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38167502/pediatrics-severe-low-back-pain-by-disc-herniation-an-uncommon-entity
#37
JOURNAL ARTICLE
Wendlassida Joelle Stéphanie Tiendrebeogo/Zabsonre, Denlewende Sylvain Zabsonre, Fulgence Kabore, Abdoulaye Sanou, Yakouba Haro, Inoussa Zoungrana, Dieu-Donné Ouedraogo
BACKGROUND: Common severe back pain due to disc herniation is rare in the paediatric population which involves children under eighteen years of age. Paediatric lumbar disc herniation (LDH) cannot be considered the same disease as in adults, as it has potentially different natural and clinical backgrounds. The treatment of pediatric LDH is the other particularity of this condition. Indeed, in children, delaying surgery for a conservative treatment is justified. We report 3 cases treated in 8 years...
January 2, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38167070/towards-a-standardized-program-of-transitional-care-for-adolescents-with-juvenile-idiopathic-arthritis-for-turkey-a-national-survey-study
#38
JOURNAL ARTICLE
Betül Sözeri, Nihal Şahin, Ceyhun Açarı, Pinar Ozge Avar Aydın, Ozge Baba, Esra Bağlan, Sevcan Bakkaloğlu, Sibel Bakırcı, Yelda Bilginer, Burcu Yücel Bozkaya, Şengül Çağlayan, Mustafa Çakan, Figen Çakmak, Taner Coşkuner, Ferhat Demir, Fatma Gül Demirkan, Şeyda Doğantan, Hatice Adıgüzel Dündar, Emine Duygu Ersözlü, Sercan Gücenmez, Oğuz Gürler, Rana İşgüder, Adem Küçük, Mukaddes Kalyoncu, Levent Kılıç, Sara Şebnem Kılıç, Hakan Kısaoğlu, Ayşenur Paç Kısaarslan, Zehra Kızıldağ, Duygu Kurtuluş, Semanur Özdel, Kübra Öztürk, Pelin Şenol, Ayşe Tanatar, Sema Nur Taşkın, Fatma Tuncer Kuru, Serkan Türkuçar, Kadir Ulu, Erbil Ünsal, Ayten Yazıcı, Deniz Gezgin Yıldırım, Selçuk Yüksel, Özgür Kasapçopur, Seza Özen, Nuray Aktay Ayaz, Hafize Emine Sönmez
BACKGROUND: Juvenile idiopathic arthritis (JIA) is a prevalent childhood chronic arthritis, often persisting into adulthood. Effective transitional care becomes crucial as these patients transition from pediatric to adult healthcare systems. Despite the concept of transitional care being recognized, its real-world implementation remains inadequately explored. This study aims to evaluate the thoughts and practices of healthcare providers regarding transitional care for JIA patients. METHODS: A cross-sectional survey was conducted among pediatric and adult rheumatologists in Turkey...
January 2, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38167019/prospective-study-to-characterize-adalimumab-exposure-in-pediatric-patients-with-rheumatic-diseases
#39
JOURNAL ARTICLE
Tatjana Welzel, Klervi Golhen, Andrew Atkinson, Verena Gotta, David Ternant, Jasmin B Kuemmerle-Deschner, Christine Michler, Gilbert Koch, Johannes N van den Anker, Marc Pfister, Andreas Woerner
BACKGROUND: In pediatric rheumatic diseases (PRD), adalimumab is dosed using fixed weight-based bands irrespective of methotrexate co-treatment, disease activity (DA) or other factors that might influence adalimumab pharmacokinetics (PK). In rheumatoid arthritis (RA) adalimumab exposure between 2-8 mg/L is associated with clinical response. PRD data on adalimumab is scarce. Therefore, this study aimed to analyze adalimumab PK and its variability in PRD treated with/without methotrexate...
January 2, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38166957/a-rare-case-of-coronary-artery-complication-in-a-child-with-systemic-juvenile-idiopathic-arthritis-and-macrophage-activation-syndrome-case-report-and-literature-review
#40
REVIEW
Lian Zhang, Yanwen Wei, Ningjing Zeng, Lianyu Wang, Xinying Chen, Jinghua Yang, Xiaolan Xiao
A rare case of coronary artery involvement in a child with Systemic Juvenile Idiopathic Arthritis (sJIA) complicated by Macrophage Activation Syndrome (MAS) is reported. The patient initially received an inaccurate diagnosis of Kawasaki Disease, sepsis, and mycoplasma infection and showed no improvement after Intravenous Immune Globulin (IVIG) treatment. Upon admission, symptoms included diffuse red rash, swelling of the limbs, lymph node enlargement, and hepatosplenomegaly. Post investigations, a diagnosis of sJIA and MAS was confirmed, and treatment involved a combination of hormones (methylprednisolone) and immunosuppressive drugs (methotrexate)...
January 2, 2024: Pediatric Rheumatology Online Journal
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