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Author(s): Belde Kasap Demir [sup.1] [sup.2] , Ali Kanik [sup.2] [sup.3] , Melis Köse [sup.2] [sup.4] , Burcu Öztürk Hismi [sup.5] , Masallah Baran [sup.2] [sup.6] Author Affiliations: (1) grid.411795.f, [...]

Objectives The aim of this study is to investigate the effect of anti-interleukin (IL)-1/-6 biologics on systemic juvenile idiopathic arthritis (sJIA)-associated macrophage activation syndrome (MAS). Methods Demographic, clinical and laboratory data of patients followed up with a diagnosis of sJIA-associated MAS assessed from sixteen paediatric rheumatology centres across the country. The clinical and laboratory features of MAS developing while on biological drugs were compared with those without this treatment. Results One hundred and sixty-two patients were included in the study. Forty-five of the MAS events were detected under the effect of anti-IL-1/-6 biologics, while the patients experiencing the remaining 155 events have not received biological treatment in the last three months. Platelet count [128 (72–232) vs 199 (130–371) 109/l], ferritin level on admission [1107 (676–2050) vs 2863 (1193–9562) ng/ml], C-reactive protein level [15.4 (2.9–56) vs 90 (32–160) mg/l], erythrocyte sedimentation rate [13 (3–36) vs 43.5 (13–77) mm/h] and fever duration [5 (4–7.5) vs 10 (7–14.3) days] were found lower in the group under the impact of anti-IL-1/-6 biologics. Among patients treated with biologics, 26.6% did not meet the published 2016 MAS classification criteria at presentation. The rates of hepatomegaly and splenomegaly were relatively lower in the canakinumab-treated group when compared with those receiving other biologicals or to patients, not on biologicals. Conclusion Anti-IL-1/-6 therapies can mask the clinical and laboratory features of MAS, and proposed guidelines for MAS classification criteria may not be met. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Behcet Uz Children's Hospital is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Author(s): Demet Alaygut [sup.1] , Gökçen Erfidan [sup.1] , Eren Soyaltin [sup.1] , Zuhal Önder Sivis [sup.2] , Seçil Arslansoyu Çamlar [sup.1] , Fatma Mutlubas [sup.1] , Belde Kasap Demir [...]

Author(s): Eren Soyaltin [sup.1] , Seçil Arslansoyu Çamlar [sup.1] , Demet Alaygut [sup.1] , Fatma Mutlubas [sup.1] , Önder Yavascan [sup.1] , Seyhun Solakoglu [sup.2] , Belde Kasap Demir [sup.1] [...]

Copyright of Ege Journal of Medicine is the property of Ege University, Faculty of Medicine and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background. Fatigue is a common problem in pediatric rheumatic diseases and is associated with poor quality of life. However, no validated methods are available to measure fatigue in adolescents with familial Mediterranean fever (FMF). The aim of the study was to establish validity and reliability for the child self-report PedsQL Multidimensional Fatigue Scale (PedsQL-MFS) and to investigate the effects of physical characteristics, diseaserelated characteristics, sleep quality/duration, and the amount of physical activity on fatigue in adolescents with FMF. Methods. Seventy-one adolescents with FMF (13-18 years) were included. Children were examined regarding physical- and disease-related characteristics and completed patient-reported outcome measures (PROMs) regarding sleep quality/duration, physical activity levels, and fatigue. PedsQL-MFS was re-completed within the following 7-14 days. Results. PedsQL-MFS demonstrated excellent test-retest reliability (ICC in 95% CI: 0.877-0.958) and internal consistency (Cronbach's α: 0.928). All items contributed to the total score (item-total correlation >0.3). PedsQLMFS scores were significantly correlated to fatigue (r: -0.666, p<0.001), physical activity (r: 0.373, p<0.001), sleep quality (rs: 0.678, p<0.001), and sleep duration (r: 0.473, p<0.001). Being female, having attacks in the last six months, a sleep duration of less than seven hours, and engaging in less physical activity resulted in higher fatigue. Conclusions. PedsQL-MFS seems to be feasible for assessing fatigue in adolescents with FMF. Sex, recent attacks, sleep, and physical activity should be taken into consideration in the fatigue management of patients with FMF. [ABSTRACT FROM AUTHOR]

Background. Alport syndrome (AS) is characterized by progressive kidney disease. There is increasing evidence that renin-angiotensin-aldosterone system (RAAS) inhibition delays chronic kidney disease (CKD) while the effectiveness of immunosuppressive (IS) therapy in AS is still uncertain. In this study, we aimed to analyze the outcomes of pediatric patients with X-linked AS (XLAS) who received RAAS inhibitors and IS therapy. Methods. Seventy-four children with XLAS were included in this multicenter study. Demographic features, clinical and laboratory data, treatments, histopathological examinations, and genetic analyses were analyzed retrospectively. Results. Among 74 children, 52 (70.2%) received RAAS inhibitors, 11 (14.9%) received RAAS inhibitors and IS, and 11 (14.9%) were followed up without treatment. During follow-up, glomerular filtration rate (GFR) decreased <60 ml/min/1.73 m2 in 7 (9.5%) of 74 patients (M/F=6/1). In male patients with XLAS, kidney survival was not different between RAAS and RAAS+IS groups (p=0.42). The rate of progression to CKD was significantly higher in patients with nephrotic range proteinuria and nephrotic syndrome (NS), respectively (p=0.006, p=0.05). The median age at the onset of RAAS inhibitors was significantly higher in male patients who progressed to CKD (13.9 vs 8.1 years, p=0.003). Conclusions. RAAS inhibitors have beneficial effects on proteinuria and early initiation of therapy may delay the progression to CKD in children with XLAS. There was no significant difference between the RAAS and RAAS+IS groups in kidney survival. AS patients presenting with NS or nephrotic range proteinuria should be followed up more carefully considering the risk of early progression to CKD. [ABSTRACT FROM AUTHOR]

Aim Urinary tract infections (UTIs) represent a common febrile illness in infancy. The study compared two UTI guidelines in terms of number of imaging studies, presence of parenchymal damage and radiation exposure in patients with the first febrile UTI between 2 and 24 months of age. Method The results of Tepecik UTI Guideline-1 used until 2012 (Group 1, n  = 105) were retrospectively compared with Tepecik UTI Guideline-2 (Group 2) used after 2013. In Group 1, urinary tract ultrasonography (US), dimercaptosuccinic acid (DMSA) and voiding cystourethrography (VCUG) were made in all patients. In Group 2, if the US result was abnormal, patients were evaluated with VCUG and DMSA. If the US was normal, only DMSA was performed. If the DMSA was abnormal, the VCUG was undergone (n : 43, 40.9%). Results The abnormal VCUG detection rate was 69.2% in Group 1 and 30.8% in Group 2 (p  = 0.09). Sensitivity and specificity of US in the diagnosis of vesicoureteral reflux (VUR) was 15.9% and 96.7% in Group 1 and 61.5% and 70.5% in Group 2, respectively. Abnormal DMSA findings were observed among 33.3% (Groups 1) and 66.7% (Groups 2) subjects, respectively (p  > 0.05). The median radiation exposure (500 mrem) of patients in Group 1 was statistically significantly higher than those in Group 2 (200 mrem) (p  < 0.001). Conclusion The VCUG should not be the first examination to be considered in such patients. We think that Tepecik UTI Guideline-2 reduces unnecessary invasive procedure and radiation exposure and not missed VUR in the management of children with UTI at 2–24 months. Needs prospective follow-up studies before considering this recommendation. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Tepecik Education & Research Hospital / İzmir Tepecik Eğitim ve Araştırma Hastanesi Dergisi is the property of Logos Medical Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: Primary hyperoxaluria type 1 is an autosomal recessive rare disorder, caused by mutations in the alanine: glyoxylate aminotransferase (AGXT) gene. We aimed to detect the AGXT gene mutations causing primary hyperoxaluria type I in combined liver-kidney and isolated liver transplanted children with phenotypic characteristics of primary hyperoxaluria type 1. Methods: This study was carried out by including 6 Turkish children and their families followed by Dokuz Eylül. University Faculty of Medicine, Department of Pediatric Nephrology and diagnosed as primary hyperoxaluria with their phenotypic features. Clinical features, transplantation characteristics, and AGT catalytic activities of the cases were noted. The entire coding region including exon-intron boundaries of the AGXT gene was sequenced in patients and their family. Results: We detected 6 mutations primary hyperoxaluria type 1 causing and 2 minor allele polymorphism in 6 patients (5 families) The entire patients had at least one primary hyperoxaluria type 1-related mutation. Patient 1 had homozygous minor allele polymorphisms Pro11Leu in exon 1 and Ile340Met in exon 10, and mutation Met195Arg in exon 5. Patient 2 had homozygous mutation c. 33_34insC in exon 1. Patient 3 was compound heterozygous for mutations Gly170Arg in exon 4 and c.846+1G>A in intron 8 and heterozygous minor allele polymorphisms Ile340Met in exon 10. Patient 4 had homozygous mutation c.823-824dupAG in exon 8. Patient 5 and 6 had homozygous mutation c.976delG in exon 10. Conclusions: Our studies emphasize the mutation analysis of the entire coding region instead of targeted (exons 1, 4, and 7) mutation analysis of AGXT. [ABSTRACT FROM AUTHOR]

Acute focal bacterial nephritis is a localized, non liquefied bacterial inflammatory kidney mass affecting one or more lobes of the kidney. It is a midpoint between pyelonephritis and the formation of a kidney abscess. Mild encephalopathy with a reversible splenial lesion is identified by a temporary diffusion restriction in the selenium of the corpus callosum in magnetic resonance imaging. Neurological symptoms sometimes seen in mild encephalopathy with a reversible splenial lesion include changes in consciousness, behavioral changes, and seizures. Many infectious agents, particularly viruses, may be associated with the diagnosis. In recent years, the association between mild encephalopathy and a reversible splenial lesion has been described in some cases with acute focal bacterial nephritis. We present a case of a 9-year-old pediatric patient admitted to the pediatric emergency department with fever and seizures. Even though his urine sample revealed normal findings, he had higher levels of acute-phase reactants. With the aid of the computerized tomography and the magnetic resonance imaging scan, he was diagnosed with mild encephalopathy with reversible corpus callosum splenium lesion associated with acute focal bacterial nephritis. After appropriate treatment, he was discharged home successfully. As a conclusion, mild encephalopathy with reversible corpus callosum splenium lesion should be kept in mind for patients with neurological signs or symptoms accompanied by acute focal bacterial nephritis. [ABSTRACT FROM AUTHOR]