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  1. Mendelian susceptibility to mycobacterial disease (MSMD) is an inborn error of immunity, resulting in susceptibility to weakly virulent mycobacteria and other intramacrophagic pathogens. Rheumatologic manifest...

    Authors: Niusha Sharifinejad, Seyed Alireza Mahdaviani, Mahnaz Jamee, Zahra Daneshmandi, Afshin Moniri, Majid Marjani, Payam Tabarsi, Parisa Farnia, Mahsa Rekabi, Mazdak Fallahi, Seyedeh Atefeh Hashemimoghaddam, Masoumeh Mohkam, Jacinta Bustamante, Jean-Laurent Casanova, Davood Mansouri and Ali Akbar Velayati
    Citation: Pediatric Rheumatology 2021 19:121
  2. High-dose glucocorticoids (GC) remain the primary therapy to induce remission in Juvenile Dermatomyositis (JDM). Studies of the natural history of GC associated weight gain in children are very limited, especi...

    Authors: Amer Khojah, Victoria Liu, Gabrielle Morgan, Richard M. Shore and Lauren M. Pachman
    Citation: Pediatric Rheumatology 2021 19:118
  3. To describe a case of pediatric sarcoidosis which initially presented as papillary conjunctivitis before manifesting as bilateral lacrimal gland swelling without other known systemic involvement.

    Authors: Margaret S. Powell, Ashley W. Cross, Jared Tallo, Edward W. Cheeseman and Mileka R. Gilbert
    Citation: Pediatric Rheumatology 2021 19:117
  4. Despite being at high risk for depression, patients with childhood-onset systemic lupus erythematosus (c-SLE) are infrequently and inconsistently screened for depression by their pediatric rheumatologists. We ...

    Authors: Evan Mulvihill, Rebecca Furru, Alana Goldstein-Leever, Kyla Driest, Stephanie Lemle, Darby MacDonald, Emily Frost and Vidya Sivaraman
    Citation: Pediatric Rheumatology 2021 19:116

    The Correction to this article has been published in Pediatric Rheumatology 2021 19:133

  5. Authors: Francesco Baldo, Michela Brena, Simone Carbogno, Francesca Minoia, Stefano Lanni, Sophie Guez, Antonella Petaccia, Carlo Agostoni, Rolando Cimaz and Giovanni Filocamo
    Citation: Pediatric Rheumatology 2021 19:115

    The original article was published in Pediatric Rheumatology 2021 19:80

  6. Respiratory conditions are the leading cause of hospitalization and death in children with Trisomy 21 (T21). Diffuse alveolar hemorrhage (DAH) occurs at higher frequency in children with T21; yet, it is not wi...

    Authors: Jessica L. Bloom, Benjamin Frank, Jason P. Weinman, Csaba Galambos, Sean T. O’Leary, Deborah R. Liptzin and Robert C. Fuhlbrigge
    Citation: Pediatric Rheumatology 2021 19:114
  7. Despite the commonality of adolescent knee pain, there are no tools to support medical doctors to correctly diagnose knee pain. This study aimed to develop and evaluate a support tool for diagnosing the most c...

    Authors: Clara Guldhammer, Sinead Holden, Marina Elmelund Sørensen, Jens Lykkegaard Olesen, Martin Bach Jensen and Michael Skovdal Rathleff
    Citation: Pediatric Rheumatology 2021 19:110
  8. Glucocorticoid exposure is a significant driver of morbidity in children with systemic juvenile idiopathic arthritis (sJIA). We determined the effect of early initiation of biologic therapy (IL-1 or IL-6 inhib...

    Authors: Rosemary G. Peterson, Rui Xiao, Hannah Katcoff, Brian T. Fisher and Pamela F. Weiss
    Citation: Pediatric Rheumatology 2021 19:109
  9. Immunosuppressive drugs, incomplete vaccine coverage, immune system dysregulation might be factors of a low level of anti-vaccine antibodies in JIA patients. The study aimed to evaluate vaccine coverage, post-...

    Authors: Mikhail M. Kostik, Natalia A. Lubimova, Irina V. Fridman, Olga V. Goleva and Susanna M. Kharit
    Citation: Pediatric Rheumatology 2021 19:108
  10. High-dose intravenous immunoglobulin (IVIG) is the mainstay of treatment for Kawasaki disease (KD). Usually, 2 g/kg of IVIG is administered over 10–24 h, depending on the institution or physician, but the asso...

    Authors: Saori Fukui, Mitsuru Seki, Takaomi Minami, Kazuhiko Kotani, Kensuke Oka, Akiko Yokomizo, Daisuke Matsubara, Tomoyuki Sato, Yasuyuki Nozaki, Mari Saito, Yutaka Kikuchi, Kenji Miyamoto, Yukifumi Monden and Takanori Yamagata
    Citation: Pediatric Rheumatology 2021 19:107
  11. Recognition of temporomandibular joint (TMJ) involvement in children with juvenile idiopathic arthritis (JIA) has gained increasing attention in the past decade. The clinical assessment of mandibular range of ...

    Authors: Willemijn F. C. de Sonnaville, Caroline M. Speksnijder, Nicolaas P. A. Zuithoff, Daan R. C. Verkouteren, Nico W. Wulffraat, Michel H. Steenks and Antoine J. W. P. Rosenberg
    Citation: Pediatric Rheumatology 2021 19:106
  12. Chronic illness, such as juvenile idiopathic arthritis (JIA), appears to have an impact on the mental health of children and adolescents. The aim of this study was to explore the incidence of mental and behavi...

    Authors: Minna S. Kyllönen, Hanna Ebeling, Hannu Kautiainen, Kari Puolakka and Paula Vähäsalo
    Citation: Pediatric Rheumatology 2021 19:105
  13. H syndrome (HS) is a rare autoinflammatory disease caused by a mutation in the solute carrier family 29, member 3 (SCL29A3) gene. It has a variable clinical presentation and little phenotype-genotype correlati...

    Authors: Laura Ventura-Espejo, Inés Gracia-Darder, Silvia Escribá-Bori, Eva Regina Amador-González, Ana Martín-Santiago and Jan Ramakers
    Citation: Pediatric Rheumatology 2021 19:104
  14. Dermatomyositis with positive anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody has a distinct phenotype associated with small hand joint arthritis, mucocutaneous ulceration, palmar papules ...

    Authors: Tsz-Wing Yeung, Kai-Ning Cheong, Yu-Lung Lau and Kei-Chiu Niko Tse
    Citation: Pediatric Rheumatology 2021 19:103
  15. The inflammatory process in juvenile idiopathic arthritis (JIA) involves both the innate and the adaptive immune system. The turnover and activity of neutrophil granulocytes may be reflected by proteins secret...

    Authors: Malin Backlund, Per Venge and Lillemor Berntson
    Citation: Pediatric Rheumatology 2021 19:102
  16. Most childhood-onset rheumatic diseases are chronic health conditions, which need long-term care throughout adulthood. A well-organized transition care is challenging and patient assessment of transition skill...

    Authors: Sirinthip Kittivisuit, Butsabong Lerkvaleekul, Sirisucha Soponkanaporn, Pintip Ngamjanyaporn and Soamarat Vilaiyuk
    Citation: Pediatric Rheumatology 2021 19:101
  17. Dermatomyositis is an inflammatory muscle disease caused by immune-mediated muscle injury, and central core disease (CCD) is a congenital myopathy associated with disturbed intracellular calcium homeostasis an...

    Authors: Min Jung Kim, Mi Hyeon Kim, Sung-Hye Park and Yeong Wook Song
    Citation: Pediatric Rheumatology 2021 19:100
  18. Primary cutaneous mucinosis are a heterogeneous group of diseases characterized by the deposition of glycosaminoglycans in the dermis and the follicles. These diseases are rare in children therefore their diag...

    Authors: Cristina Morreale, Dario Bleidl, Angela Rita Sementa and Clara Malattia
    Citation: Pediatric Rheumatology 2021 19:99
  19. Subcutaneous anakinra is an interleukin-1 inhibitor used to treat juvenile idiopathic arthritis. Recent reports suggest anakinra can be a valuable addition to the treatment of COVID-19 associated cytokine stor...

    Authors: Omkar Phadke, Kelly Rouster-Stevens, Helen Giannopoulos, Shanmuganathan Chandrakasan and Sampath Prahalad
    Citation: Pediatric Rheumatology 2021 19:98
  20. Physical activity (PA) patterns in children with juvenile idiopathic arthritis (JIA) over time are not well described. The aim of this study was to describe associations of physical activity (PA) with disease ...

    Authors: Liane D. Heale, Kristin M. Houghton, Elham Rezaei, Adam D. G. Baxter-Jones, Susan M. Tupper, Nazeem Muhajarine, Susanne M. Benseler, Gilles Boire, David A. Cabral, Sarah Campillo, Gaëlle Chédeville, Anne-Laure Chetaille, Paul Dancey, Ciaran Duffy, Karen Watanabe Duffy, Janet Ellsworth…
    Citation: Pediatric Rheumatology 2021 19:97
  21. Coronary artery lesions (CALs) are the most severe complication of Kawasaki disease (KD). Approximately 9–20% of the patients with KD develop CAL despite receiving regular treatment (intravenous immunoglobulin...

    Authors: Xuting Zhang, Yuee He, Yiping Shao, Biyao Hang, Zhipeng Xu and Maoping Chu
    Citation: Pediatric Rheumatology 2021 19:96
  22. Here, we investigated the predictive efficiency of a newly developed model based on single nucleotide polymorphisms (SNPs) and laboratory data for intravenous immunoglobulin (IVIG) resistance in Kawasaki disea...

    Authors: Li Meng, Zhen Zhen, Qian Jiang, Xiao-hui Li, Yue Yuan, Wei Yao, Ming-ming Zhang, Ai-jie Li and Lin Shi
    Citation: Pediatric Rheumatology 2021 19:95
  23. There are few reports of COVID-19 in pediatric patients with rheumatic diseases. This study describes the clinical presentation and outcomes of COVID-19 in this population.

    Authors: D. Sofia Villacis-Nunez, Christina A. Rostad, Kelly Rouster-Stevens, Arezou Khosroshahi, Shanmuganathan Chandrakasan and Sampath Prahalad
    Citation: Pediatric Rheumatology 2021 19:94
  24. Juvenile idiopathic inflammatory myopathies (JIIMs) is a group of autoimmune disorders, including juvenile dermatomyositis (JDM), juvenile polymyositis (JPM) and overlap myositis, that are characterized by pro...

    Authors: Megan Mariko Perron, Natalia Vasquez-Canizares, Gabriel Tarshish and Dawn M. Wahezi
    Citation: Pediatric Rheumatology 2021 19:92
  25. Fabry disease (FD) is a rare, X-linked, multisystemic lysosomal storage disorder (LSD) that results from a deficiency in the hydrolase alpha-galactosidase A (⍺-GalA). During childhood, classic FD symptomatolog...

    Authors: Luciana Paim-Marques, Amanda Virginia Cavalcante, Islane Verçosa, Paula Carneiro, Marcia Souto-Maior, Erlane Marques and Simone Appenzeller
    Citation: Pediatric Rheumatology 2021 19:91
  26. Juvenile idiopathic arthritis (JIA) is a heterogeneous group of disorders, including all forms of arthritis, which develops in children who are less than 16 years old. This study aimed to evaluate the clinical...

    Authors: Raed M. Alzyoud, Motasem O. Alsuweiti, Heba Q. Almaaitah, Bushra N. Aladaileh, Mohammad K. Alnoubani and Adel M. Alwahadneh
    Citation: Pediatric Rheumatology 2021 19:90
  27. Although, preliminary reports of Severe Acute Respiratory Syndrome (SARS)-CoV-2 infection suggest that the infection causes a less severe illness in children, there is now growing evidence of other rare or eve...

    Authors: Reza Sinaei, Sara Pezeshki, Saeedeh Parvaresh, Roya Sinaei, Reza Shiari, Mehrnoush Hassas Yeganeh, Nasrin Bazargn and Nava Gharaei
    Citation: Pediatric Rheumatology 2021 19:89
  28. With the advent of innovative therapies including biologics and Janus kinase inhibitors, children with rheumatic diseases are more likely to have improved outcomes. Despite these advances, some children do not...

    Authors: Stephen C. Wong, Leah C. Medrano, Alice D. Hoftman, Olcay Y. Jones and Deborah K. McCurdy
    Citation: Pediatric Rheumatology 2021 19:87
  29. Children with rheumatic diseases (cRD) receiving immunosuppressive medications (IM) are at a higher risk for acquiring potentially lethal pathogens, including Histoplasma capsulatum (histoplasmosis), a fungal inf...

    Authors: Rachel A. Brown, Fatima Barbar-Smiley, Cagri Yildirim-Toruner, Monica I. Ardura, Stacy P. Ardoin and Shoghik Akoghlanian
    Citation: Pediatric Rheumatology 2021 19:84
  30. Current evidence suggests that many adolescents with juvenile idiopathic arthritis (JIA) do not successfully transfer to adult care, which can result in adverse health outcomes. Although a growing number of cl...

    Authors: Lynn Spiegel, Lori Tucker, Karen Watanabe Duffy, Chitra Lalloo, Amos Hundert, Josiane Bourre-Tessier, Elizabeth Hazel, Nadia Luca, Dianne Mosher, Cynthia Nguyen, Elizabeth Stringer, Charles Victor and Jennifer Stinson
    Citation: Pediatric Rheumatology 2021 19:83
  31. Tenascin-C (TN-C) is an extracellular matrix glycoprotein related to tissue inflammation. Our previous retrospective study conducted in 2016 revealed that the serum tenascin-C level was higher in patients with...

    Authors: Yukako Yoshikane, Yoshiaki Okuma, Tatsuki Miyamoto, Junichi Hashimoto, Ryuji Fukazawa, Taichi Kato, Atsuhito Takeda, Kenji Suda, Takeji Matsushita, Michiaki Hiroe and Kyoko Imanaka-Yoshida
    Citation: Pediatric Rheumatology 2021 19:82
  32. Granulomatosis with polyangiitis (GPA) is an anti-neutrophilic cytoplasmic antibody-associated vasculitis affecting small to medium-sized vessels and involves most commonly the kidneys and the respiratory trac...

    Authors: Rotem Semo Oz, Oluwakemi Onajin, Liora Harel, Rotem Tal, Tomas Dallos, Adena Rosenblatt, Lukas Plank and Linda Wagner-Weiner
    Citation: Pediatric Rheumatology 2021 19:81
  33. Harlequin ichthyosis (HI) is the most severe phenotype of autosomal recessive congenital ichthyosis. Juvenile Idiopathic Arthritis (JIA) represents a heterogenous group of disorders all sharing the clinical ma...

    Authors: Francesco Baldo, Michela Brena, Simone Carbogno, Francesca Minoia, Stefano Lanni, Sophie Guez, Antonella Petaccia, Carlo Agostoni, Rolando Cimaz and Giovanni Filocamo
    Citation: Pediatric Rheumatology 2021 19:80

    The Correction to this article has been published in Pediatric Rheumatology 2021 19:115

  34. The present study focuses on the associations of streptococcal infection with the clinical phenotypes, relapse/recurrence and renal involvement in Henoch-Schönlein purpura (HSP) children.

    Authors: Guo Zhen Fan, Rui Xue Li, Qi Jiang, Man Man Niu, Zhen Qiu, Wei Xia Chen, Hui Hui Liu, Jin Wei Ruan and Peng Hu
    Citation: Pediatric Rheumatology 2021 19:79
  35. This study aimed to assess the occurrence of coronary artery lesions (CAL) in patients with Kawasaki disease (KD) according to serum C-reactive protein (CRP) levels.

    Authors: Hyo Soon An, Gi Beom Kim, Mi Kyoung Song, Sang Yun Lee, Hye Won Kwon, Joo Won Lee and Eun Jung Bae
    Citation: Pediatric Rheumatology 2021 19:78
  36. Cryopyrin-associated periodic syndrome (CAPS) is a life-long, autoinflammatory disease associated with a gain-of-function mutation in the nucleotide-binding domain, leucine-rich repeat family, pyrin domain con...

    Authors: Kyoko Yokoi, Sachiko Minamiguchi, Yoshitaka Honda, Mizuho Kobayashi, Satoru Kobayashi and Ryuta Nishikomori
    Citation: Pediatric Rheumatology 2021 19:77
  37. Kawasaki disease (KD) is an acute, self-limited febrile illness of unknown cause. Intravenous immunoglobulin (IVIG)-resistance are related to greater risk for permanent cardiac complications. We aimed to deter...

    Authors: Yi Seul Kim, Hyun Jin Yang, Seung-Jung Kee, Insu Choi, Kisoo Ha, Katrina K Ki, In Seok Jeong and Hwa Jin Cho
    Citation: Pediatric Rheumatology 2021 19:76
  38. Juvenile primary fibromyalgia syndrome (JPFS) is a chronic musculoskeletal pain syndrome affecting children and adolescents. In part one of this review, we discussed the epidemiology, etiology, pathogenesis, c...

    Authors: Maya Levy Coles and Yosef Uziel
    Citation: Pediatric Rheumatology 2021 19:74

    The Review to this article has been published in Pediatric Rheumatology 2021 19:22

  39. Childhood-onset systemic lupus erythematosus (cSLE) is a complex autoimmune disorder with multi-organ manifestations and can be associated with other rheumatic diseases including Sjögren’s syndrome (SS). Saliv...

    Authors: Joseph McDonald, Patricia Vega-Fernandez and Tracy Ting
    Citation: Pediatric Rheumatology 2021 19:73
  40. Among school-age children, the decrease of cartilage thickness (Cth) with increasing age is well known. However, the influence of body mass index (BMI), height or weight on Cth has not been revealed. Here in, ...

    Authors: Chun-Chun Gau, Tsung-Chieh Yao, Shu-Ting Gan, Syh-Jae Lin, Kuo-Wei Yeh, Li-Chen Chen, Liang- Shiou Ou, Wen-I Lee, Chao-Yi Wu and Jing-Long Huang
    Citation: Pediatric Rheumatology 2021 19:71

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