Introduction The term anti-nuclear antibody (ANA) is used to define a large group of autoantibodies which specifically bind to nuclear elements. Although healthy individuals may also have ANA positivity, the measurement of ANA is generally used in the diagnosis of autoimmune disorders. However, various studies have shown that ANA testing may be overused, especially in pediatrics clinics. Our aim was to investigate the reasons for antinuclear antibody (ANA) testing in the general pediatrics and pediatric rheumatology clinics of our hospital and to determine whether ANA testing was ordered appropriately by evaluating chief complaints and the ultimate diagnoses of these cases. Methods The medical records of pediatric patients in whom ANA testing was performed between January 2014 and June 2016 were retrospectively evaluated. Subjects were grouped according to the indication for ANA testing and ANA titers. Results ANA tests were ordered in a total of 409 patients during the study period, with 113 positive ANA results. The ANA test was ordered mostly due to joint pain (50% of the study population). There was an increased likelihood of autoimmune rheumatic diseases (ARDs) with higher ANA titer. The positive predictive value of an ANA test was 16% for any connective tissue disease and 13% for lupus in the pediatric setting. Conclusion in the current study, more than one-fourth of the subjects were found to have ANA positivity, while only 15% were ultimately diagnosed with ARDs. Our findings underline the importance of an increased awareness of correct indications for ANA testing.
Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disease of childhood and adulthood. Development of systemic amyloidosis and frequent attack influence quality of life and survival. There is sporadic evidence indicating subclinical inflammation in patients with FMF. We aimed to assess subclinical inflammation using neutrophil to lymphocyte ratio (NLR), platelet to lymphocyte ratio (PLR), and C-reactive protein (CRP) in pediatric patients with FMF in the attack-free period. In this retrospective study, we reviewed the files of all FMF patients in our pediatric rheumatology outpatient clinic in a tertiary center and enrolled those with sufficient clinical and laboratory data. We also enrolled 73 controls. We grouped the patients according to being in attack period or attack-free period. We compared CRP, NLR, PLR, and WBC (white blood cell) levels between different mutations and polymorphisms. We also compared patients in the attack period with those in attack-free period. We enrolled 61 patients in attack period, 509 patients in attack-free period, and 73 controls. There was no difference between patients with different mutations with respect to NLR or PLR levels in the attack-free period. However, CRP levels were higher in patients with homozygous exon 10 mutations, especially those with homozygous M694V mutations compared with other mutations. However, CRP levels were mostly normal in these patients. Our data are against the reported fact that patients with FMF have higher NLR or PLR levels in attack-free periods. However, CRP levels were higher in the presence of homozygous exon 10 mutations (in particular homozygous M694V mutations).
Takayasu arteriti genellikle yaşamın ikinci ve üçüncü on yılında genç kadınları etkileyen bir büyük damar vaskülitidir. Daha az sıklıkta çocukluk çağında da görülebilir. Bu yazıda tek taraflı çomak parmağı olan Takayasu arteritli 17 yaşındaki bir erkek olgu sunuldu. Hastanın sol üst ve alt ekstremitelerinde bir aydan beri devam eden kas zayıflığı ve miyaljisi vardı. Hipertansiyon ve abdominal üfürüm yoktu. Dijital subtraksiyon anjiyografide sol subklaviyan arterde total tıkanma ve sağ renal arterde ciddi daralma olduğu görüldü. Çomak parmak ve Takayasu arteriti birlikteliği yaygın değildir. Bilgilerimize göre bugüne kadar yalnız iki olgunun sunumu yapılmıştır.Anahtar sözcükler: Çomak parmak; dijital subtraksiyon anjiyografi; hipertansiyon; Takayasu arteriti.Takayasu arteritis is a large vessel vasculitis that usually affects young female patients during the second and third decades of life. Less commonly, it can also be seen childhood. In this article, we describe a case of Takayasu arteritis in a 17 year-old-boy who presented with unilateral digital clubbing. The patient had also a one-month history of muscle weakness and myalgia in the left upper and lower extremity. There was no hypertension or abdominal bruit. Digital subtraction angiography revealed a total occlusion of the left subclavian artery and a severe narrowing of the right renal artery. The association of digital clubbing and Takayasuʼs arteritis is not common. To our knowledge, only two cases have been reported to date.
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