{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T06:03:26Z","timestamp":1785305006777,"version":"3.55.0"},"reference-count":75,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2024,6,28]],"date-time":"2024-06-28T00:00:00Z","timestamp":1719532800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"NIH","award":["R-21 AR077565"],"award-info":[{"award-number":["R-21 AR077565"]}]},{"name":"NIH","award":["UL1TR001422"],"award-info":[{"award-number":["UL1TR001422"]}]},{"name":"CureJM Foundation","award":["R-21 AR077565"],"award-info":[{"award-number":["R-21 AR077565"]}]},{"name":"CureJM Foundation","award":["UL1TR001422"],"award-info":[{"award-number":["UL1TR001422"]}]},{"name":"DenUyl Family Research Fund","award":["R-21 AR077565"],"award-info":[{"award-number":["R-21 AR077565"]}]},{"name":"DenUyl Family Research Fund","award":["UL1TR001422"],"award-info":[{"award-number":["UL1TR001422"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Juvenile Dermatomyositis (JDM) is the most common inflammatory myopathy in pediatrics. This study evaluates the role of Natural Killer (NK) cells in Juvenile Dermatomyositis (JDM) pathophysiology. The study included 133 untreated JDM children with an NK cell count evaluation before treatment. NK cell subsets (CD56low\/dim vs. CD 56bright) were examined in 9 untreated children. CD56 and perforin were evaluated in situ in six untreated JDM and three orthopedic, pediatric controls. 56% of treatment-naive JDM had reduced circulating NK cell counts, designated \u201clow NK cell\u201d. This low NK group had more active muscle disease compared to the normal NK cell group. The percentage of circulating CD56low\/dim NK cells was significantly lower in the NK low group than in controls (0.55% vs. 4.6% p &lt; 0.001). Examination of the untreated JDM diagnostic muscle biopsy documented an increased infiltration of CD56 and perforin-positive cells (p = 0.023, p = 0.038, respectively). Treatment-naive JDM with reduced circulating NK cell counts exhibited more muscle weakness and higher levels of serum muscle enzymes. Muscle biopsies from treatment-naive JDM displayed increased NK cell infiltration, with increased CD56 and perforin-positive cells.<\/jats:p>","DOI":"10.3390\/ijms25137126","type":"journal-article","created":{"date-parts":[[2024,7,2]],"date-time":"2024-07-02T11:33:53Z","timestamp":1719920033000},"page":"7126","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Decreased Peripheral Blood Natural Killer Cell Count in Untreated Juvenile Dermatomyositis Is Associated with Muscle Weakness"],"prefix":"10.3390","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3357-4863","authenticated-orcid":false,"given":"Amer","family":"Khojah","sequence":"first","affiliation":[{"name":"Department of Pediatrics, College of Medicine, Umm Al-Qura University, Makkah 21955, Saudi Arabia"},{"name":"Division of Pediatric Rheumatology, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, 225 East Chicago Avenue, Box 50, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8117-574X","authenticated-orcid":false,"given":"Lauren M.","family":"Pachman","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, 225 East Chicago Avenue, Box 50, Chicago, IL 60611, USA"},{"name":"Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ameera","family":"Bukhari","sequence":"additional","affiliation":[{"name":"College of Science, Taif University, Taif 21944, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-1787-899X","authenticated-orcid":false,"given":"Chi","family":"Trinh","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, 225 East Chicago Avenue, Box 50, Chicago, IL 60611, USA"},{"name":"Wellesley College, 106 Central St, Wellesley, MA 02481, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4058-7112","authenticated-orcid":false,"given":"Gabrielle","family":"Morgan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, 225 East Chicago Avenue, Box 50, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Surya","family":"Pandey","sequence":"additional","affiliation":[{"name":"Robert H. Lurie Comprehensive Cancer Center, Skin Biology and Diseases Resource-Based Center, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6853-4007","authenticated-orcid":false,"given":"I. Caroline","family":"Le Poole","sequence":"additional","affiliation":[{"name":"Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA"},{"name":"Robert H. Lurie Comprehensive Cancer Center, Skin Biology and Diseases Resource-Based Center, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marisa S.","family":"Klein-Gitelman","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, 225 East Chicago Avenue, Box 50, Chicago, IL 60611, USA"},{"name":"Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,6,28]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1016\/j.jpeds.2017.12.053","article-title":"Advances in Juvenile Dermatomyositis: Myositis Specific Antibodies Aid in Understanding Disease Heterogeneity","volume":"195","author":"Pachman","year":"2018","journal-title":"J. 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