Aim: Juvenile idiopathic arthritis (JIA) is the most prevalent chronic rheumatologic condition in children. Determining radiological progression in relation to clinical and laboratory parameters is crucial for initiating early intervention. This study aimed to investigate the association between diagnostic delay, human leukocyte antigen B27 (HLA-B27) positivity, and the development of late radiological changes in children with JIA.Methods: This retrospective single-center study included 96 patients diagnosed with JIA between January 2006 and April 2015. Demographic, clinical, laboratory, and radiological data were reviewed. Radiological changes emerging after one year of disease onset were defined as late radiological findings. Clinical features, laboratory markers, and treatment modalities were compared between patients with early and late radiological changes.Results: The mean age at diagnosis was 8.8 ± 4.0 years; 67.7% were female. Oligoarticular JIA was the most common subtype, occurring in 67 patients (69.8%), followed by systemic JIA in 20 patients (20.8%) and polyarticular JIA in 9 patients (9.4%). Late radiological changes were observed in 35 patients (36.5%). HLA-B27 positivity (20.0% vs. 6.6%, p=0.049), longer diagnostic delay (3.3 ± 2.0 vs 2.0 ± 1.8 years, p=0.002), higher joint involvement (5.6 ± 3.6 vs. 4.2 ± 2.7, p=0.033), and extra-articular findings (68.6% vs. 27.9%, p=0.001) were associated with late radiological changes. Antinuclear antibody (ANA) test positivity was inversely associated (40.0% vs. 62.3%, p=0.034). Conclusions: HLA-B27 positivity and diagnostic delay were associated with late radiological changes in children with JIA. Early diagnosis and close radiological monitoring may help prevent long-term joint damage.

Aim: Familial Mediterranean Fever (FMF) is an autoinflammatory disease with abdominal pain, recurrent fever, pleuritis, arthritis, and skin lesions. Tel-Hashomer, Livneh and Yalçınkaya-Özen Criteria amongst various diagnostic criteria are introduced.Material and Methods: This study is conducted to evaluate the Tel-Hashomer, Livneh, and Yalçınkaya-Özen Criteria among pediatric FMF patients for practical application. A total of 113 patients’ records were reassessed according to three criteria, and the likelihood of an FMF diagnosis was calculated based on the logistic model developed by Yalçınkaya et al. Patients’ likelihood of having FMF according to the logistic model, the rates of FMF diagnosis at presentation and after six months of colchicine treatment were calculated, and the rates of diagnosis after six months of colchicine treatment according to the Tel-Hashomer criteria were compared with the rates of diagnosis at presentation according to the Livneh and Yalçınkaya-Özen criteria.Results: A statistically significant difference was observed between the criteria for making a positive diagnosis at presentation. Although diagnostic rates increased by the end of 6-months, statistical analysis still showed significant difference. Using the logistic model 85.84% of our patients (n=97) had a likelihood of FMF diagnosis above 80%.Conclusion: Tel-Hashomer criteria should be preferred for their simplicity in diagnosing FMF but they appear less sensitive compared to the Livneh and Yalçınkaya-Özen Criteria. Therefore, for patients who do not receive a diagnosis with the Tel-Hashomer criteria at presentation or after six months of treatment, the more sensitive Livneh and YalçınkayaÖzen Criteria can be applied in practice.

Gastrointestinal (GI) involvement of IgA vasculitis (IgAV) has various clinical presentations from isolated abdominal pain to severe gastrointestinal bleeding. The aim of this study is to determine the incidence of GI involvement in children with IgAV and identify the predictors of severe GI involvement. Medical records of IgAV patients with GI involvement from 30 centers in Türkiye between 2014 and 2024 were retrospectively reviewed. Patients were classified into two groups according to the severity of GI presentations: Group 1 consisted of mild GI involvement, while Group 2 had severe GI involvement. Severe GI involvement was observed in 476 (42

OBJECTIVE:Juvenile idiopathic arthritis (JIA) is associated with impaired overall health-related quality of life (HRQoL). We evaluated the impact of tofacitinib on patient-reported outcomes (PROs) in patients with JIA. METHODS:This was a post hoc analysis of a phase 3, randomized, double-blind, placebo-controlled withdrawal trial (NCT02592434) in patients with JIA. In the open-label phase (part 1; weeks 0-18), patients received body weight-based doses of tofacitinib. During the double-blind phase (part 2; weeks 18-44), responders (per JIA-American College of Rheumatology 30 response criteria) were randomized 1:1 to continue tofacitinib or switch to placebo for up to 26 weeks. Assessed PROs included the validated parent and/or legal guardian versions of the Childhood Health Assessment Questionnaire for evaluation of disability, arthritis pain, overall well-being, and the Child Health Questionnaire (CHQ). RESULTS:Overall, 225 patients were enrolled and received open-label tofacitinib in part 1, and 173 patients were randomized in part 2. During part 1, least-squares (LS) mean (SE) disability, arthritis pain, and overall well-being scores numerically improved from mean 1.04 (SE 0.05), mean 5.53 (SE 0.20), and mean 5.07 (SE 0.20) at baseline to mean 0.57 (SE 0.05), mean 2.46 (SE 0.18), and mean 2.47 (SE 0.18) at week 18, respectively. LS mean (SE) CHQ physical summary and psychological summary scores numerically improved from mean 29.51 (SE 1.15) and mean 47.24 (SE 0.85) at baseline to mean 42.70 (SE 0.98) and mean 51.53 (SE 0.80) at week 18, respectively. Improvements were generally maintained to week 44 in part 2. CONCLUSION:Tofacitinib improved a range of PROs in patients with JIA, suggesting potential HRQoL benefits.

Background and Objectives: Familial Mediterranean fever (FMF) is a lifelong autoinflammatory disease characterized by episodes of fever and aseptic polyserositis. Commonly associated with vasculitis, FMF’s impact on microcirculation was investigated by examining nailfold capillaries using capillaroscopy. Materials and Methods: This study included 32 female and 28 male FMF patients diagnosed according to the Tel Hashomer and Yalçınkaya criteria and a control group of 20 female and 10 male age-matched cases. Demographic characteristics, medical history (abdominal pain, fever, chest pain, and joint pain), and physical examination findings of the cases were assessed. FMF gene mutations, acute-phase reactants, urine analysis, and spot urine protein/creatinine ratios were evaluated. Nailfold capillaries were examined via capillaroscopy by the same dermatology specialist. Results: There was no significant age or gender difference between groups. The most common symptoms in the case group were abdominal pain (81.7%) and joint pain (65%). Pathological findings in capillaroscopy, such as microhemorrhages and avascular areas, were significantly more frequent in the FMF case group (p < 0.001; p < 0.001). Physiological findings, including hairpin-shaped capillaries and shortened loops, were significantly more common in the control group (p = 0.001; p = 0.034). No significant relationships were found between kidney involvement, subclinical inflammation, presence of microhemorrhages and avascular areas in capillaroscopy, and disease duration. Additionally, no significant differences were observed in capillaroscopic findings between those with exon-10 mutations in the MEFV gene and those with non-exon-10 mutations. Conclusions: In conclusion, our study demonstrated secondary microvascular findings due to inflammation in FMF patients using capillaroscopy, a cost-effective and safe tool.

Aim: Immunoglobulin A vasculitis (IgAV) is the most common vasculitis of childhood, but its pathogenesis is largely unknown, despite evidence pointing to various environmental and genetic factors. We investigated the frequency of MEFV gene mutations that are considered in the pathogenesis and their effect on the clinical features of patients with IgAV. Methods: The study included 244 children diagnosed with IgAV, who underwent MEFV gene analyses. We recorded the demographic and clinical characteristics of the patients, along with their laboratory results. We grouped the patients based on the presence of MEFV gene mutations and MEFV variants. Results: At least one MEFV mutation was detected in 89 (36.5%) patients, with E148Q being the most common (n=31, 34.8%). Age at diagnosis and the frequency of hematuria and recurrence were significantly greater among patients with MEFV mutations (p=0.043, p=0.008, and p=0.009, respectively). Serum IgA levels were significantly higher in patients with the M694V mutation (p=0.040). Conclusion: The presence of MEFV mutations, particularly E148Q and M694V, could be associated with the development and clinical course of IgA vasculitis.

Objective: Familial Mediterranean Fever (FMF) is an autosomal recessive disease characterized by recurrent fever and polyserositis. Studies conducted in recent years emphasize the importance of platelet parameters in chronic diseases. This study examined changes in attack and attack-free periods in children with FMF, focusing on Mean Platelet Volume (MPV), an indicator of disease severity. Method: 150 FMF patients (90 girls, 60 boys) and 50 healthy individuals (29 men, 21 women) were included in the study. Data were analyzed according to colchicine treatment, attack, and attack-free periods. The severity of the disease was classified as mild, moderate, and severe. Results: MPV levels of patients with FMF were higher than the healthy group. In particular, MPV levels decreased significantly during attacks, with a more pronounced decrease in severe cases. Conclusion: As a result, MPV measurement is a cost-effective and rapid method that can support the evaluation of disease severity and attack periods in FMF patients.

Juvenile idiopathic arthritis (JIA) is a common, chronic and inflammatory rheumatological disease of childhood. The disease can affect all synovial joints in the body. Temporomandibular joints (TMJs) are important areas of involvement in JIA, which are frequently involved but often not noticed because the involvement is usually asymptomatic. The aim of this study is to determine the frequency and risk factors of TMJ joint involvement in juvenile idiopathic arthritis patients admitted to our clinic, and to guide for early diagnosis and treatment. Patients who applied to this study with the diagnosis of JIA between January 2014 and May 2017 at Pediatric Rheumatology Clinic, were followed up regularly in our clinic, had a accessible medical history, and a rheumatology polyclinic record. Patients with contrast-enhanced TMJ Magnetic Resonance Imaging (MRI) taken and reported by the radiologist were included. TMJ involvement was detected in 51.2

Objective: In patients with juvenile idiopathic arthritis (JIA), bone metabolism may be negatively affected due to both the activity of the disease and the medications used. Our study aimed to investigate the necessity of evaluating 25 hydroxyvitamin D (25 (OH) Vit D), calcium (Ca), phosphorus (P), alkaline phosphatase (ALP) levels and possible related factors in the follow-up of patients diagnosed with JIA. Materials and Methods: The records of 68 patients with JIA were retrospectively evaluated. Disease subtypes, medications used, and whether they were in remission or active disease were reviewed. Results: 25 OH Vit D levels were low in 14.7% of patients with JIA compared to the control group. 66.6% of the patients with systemic arthritis had high ALP levels. 25 OH Vit D level was low in 16.6% of steroid users, and Vitamin D level was low in 55.5% of the patients in the active disease group. It was determined that patients in the active disease group had the highest ALP and lowest vitamin D levels compared to patients in remission with and without medication. Conclusions: Bone metabolism in patients with JIA is negatively affected. Since vitamin D plays a crucial role in bone metabolism, it was emphasized that vitamin D levels should be evaluated especially during active disease and supplements should be provided for patients with low vitamin D levels.

Abstract Study question Does hyaluronidase (HAse) have possible negative or positive effects on cumulus cells (CCs) during oocyte denudation before ICSI by altering midkine (MK) levels? Summary answer Hyaluronidase inhibits the proliferation of CCs via the inhibition of MK activity which may result in poor embryo quality and low ICSI success. What is known already MK is abundantly expressed in ovarian follicles. MK secreted from the cumulus-granulosa cells that surround oocytes was shown to promote the cytoplasmic maturation of oocytes. This effect of MK could be mediated via its' anti-apoptotic effect and some other mechanisms. The removal of CCs during oocyte denudation (OD) is done in order to select and grade the oocytes before ICSI. HAse is applied for ICSI to break down hyaluronic acid, which is present at high levels in the cumulus-oocyte complex during OD. To date, there have only been a few reported cases of the toxic effects of hyaluronidase on oocytes. Study design, size, duration This study is a prospective, randomized study done with 90 healthy women between September 2017 and September 2018. Participants/materials, setting, methods This study was done with women diagnosed as a male factor between the ages of 21 and 40 who underwent ICSI. HAse was applied to the cultured CCs at concentrations of 0.1 IU/ml, 1 IU/ml, and 10 IU/ml. The proliferation and apoptosis indices (Flow cytometry), structures [Transmissive electron microscopy (TEM)], and MK levels (ELISA) of CCs were evaluated every 24 hours for 48 hours. One way-Anova was used and p < 0.05 was considered statistically significant. Main results and the role of chance The application of HAse at all concentrations decreased cumulus cell numbers for 48 hours (p < 0.05). The highest decrease in cell number and cell viability with the highest number of apoptotic cells were detected at the 48th hour at the application of the highest concentration of HAse (p < 0.05). The highest concentration of HAse application caused the highest decrease in MK levels at the end of 48 hours (p < 0.05). Limitations, reasons for caution The effect of HAse on the CCs of women aged below 21 and above 40 could not be evaluated. Therefore, the overall resistance and fragility of CCs to these HAse concentrations could not be evaluated. In addition, the HAse effects should be evaluated in female infertility cases such as PCOS. Wider implications of the findings This is the first report to examine the effect of HAse on MK activity. 2-3 lines of CCs are commonly left during oocyte denudation in order to protect the oocyte from stress-induced reactions. The low number of CCs means low MK levels resulting in a loss in oocyte competence. Trial registration number Tthe Ethics Committee Directive on Non-Interventional Studies of Biruni University with the permission numbers 2017/5-1.

Background/aim: Immunoglobulin A vasculitis (IgAV) is one of the most common types of vasculitis in children. The aims of this study were to investigate the clinical characteristics of the disease, and the effects of age and sex on the clinical course in children with IgAV. Materials and methods: This was a retrospective study including pediatric patients diagnosed with IgAV who attended follow-ups at the pediatric rheumatology department of a tertiary healthcare institution between January 1997 and December 2020. The patients were grouped and compared according to sex and age at diagnosis (<7 years vs. ?7 years). Results: The study included 709 children with IgAV, 392 (55.3%) of whom were male. The mean age at diagnosis was 7.9 ± 3.2 years. The most common disease onset season was autumn (31.2%). Upper respiratory infections (27.8%) were the most common predisposing factors. Gastrointestinal system (GIS), joint, and renal involvement were observed in 52.8%, 47.5%, and 17.5% of patients, respectively. Renal involvement, GIS involvement, and disease relapse were significantly more common among those diagnosed after 7 years of age compared to those diagnosed before the age of 7 (p < 0.001, p = 0.033, and p < 0.001, respectively). Scrotal involvement and subcutaneous edema were more common among those diagnosed at younger than 7 years compared to those aged ?7 years at diagnosis (p < 0.001 and p = 0.016, respectively). GIS involvement was more frequently seen in males compared to females (p = 0.046). Conclusion: It was demonstrated that being ?7 years of age at diagnosis or being a male were associated with higher likelihood of renal and GIS involvement in children with IgAV.

Abstract Background Familial Mediterranean Fever (FMF) is an ethnic and autosomal recessive disorder characterized by recurrent attacks of fever and polyserositis. Recently, several studies have demonstrated the importance of platelet count and volume changes associated with chronic diseases. Mean platelet volume(MPV), platelet distribution width (PDW), and neutrophil/lymphocyte ratio are shown to be essential indicators in many diseases' activation periods. In this study, we aimed to investigate the changes in children with FMF in attacks and attack-free. Materials and Methods In this study, 150 patients had been diagnosed with FMF (90 girls, 60 boys), and 50 healthy subjects (29 male, 21 female) were included. Patients’ data were recorded separately according to under treatment with colchicine, in the attacks and attack-free periods retrospectively. Patients were scored as mild, moderate, and severe. Results MPV levels in patients with FMF were higher than in the healthy group, and MPV levels were significantly lower in attacks than in attack-free periods. This decline was more pronounced with increasing disease severity scores. Conclusion In conclusion, as an easy, inexpensive, and rapid method, MPV measurement is a supportive parameter in determining the severity of the disease and attacks in patients with FMF.

Aim: In this case-control study, we investigated parental history and habits in children with acute lymphoblastic leukemia (ALL) and aimed to find possible factors that could affect the risk of leukemia. Methods: Parents of 105 children treated for ALL over a period of ten years in our Clinic of Pediatric Hematology-Oncology were questioned in terms of age, family history, nutritional habits, maternal supplement intake, smoking, alcohol consumption, X-ray exposure, and accompanying diseases. Results were compared with a control group which consisted of 102 healthy children of similar age and gender. Results: There was no significant difference between the groups regarding maternal and paternal age. Even though the rates of family history of leukemia and other cancer types were higher in the leukemia group, the difference was not statistically significant. The rate of vitamin and iron supplement intake during pregnancy was significantly lower in the leukemia group (p=0.017; p=0.002). The rate of folic acid and fish oil supplement use was higher in the control group; however, there was no significant difference. There was no difference between the groups in terms of maternal X-ray exposure, infections, and other diseases during pregnancy. Paternal smoking history ≥1 pack/day and ≥20 packs/year; and paternal routine alcohol consumption was significantly higher in the leukemia group (p=0.041; p=0.048; p=0.029). Conclusion: Leukemia is a disease with a multifactorial etiology that arises from interactions between genes and the environment. Paternal cigarette smoking, alcohol exposure, and maternal iron and vitamin deficiency may be among the factors that increase the risk of childhood ALL.

The aim of the study is to investigate how renal involvement is correlated with frequency of amyloidosis, risk factors, and demographic and clinical characteristics in pediatric patients with Familial Mediterranean fever (FMF). Demographic and clinical characteristics and laboratory data of the pediatric patients diagnosed with FMF between 1990 and 2018 were recorded from their files. The diagnosis of patients with amyloidosis (AA) was proven by renal biopsy, and as for patients with non-amyloidosis renal involvement (RI wo AA), amyloidosis could not be detected but they were followed up with the diagnosis of proteinuria and/or hematuria. A total of 1929 FMF pediatric patients were included in the study. About 962 (49.9

Aim: In juvenile idiopathic arthritis (JIA), systemic corticosteroids are reserved for cases with serious organ involvement, those with macrophage activation syndrome, and in the presence of high disease activity in oligoarticular and polyarticular JIA. However, systemic steroids may lead to serious side effects linked to adrenal insufficiency (AI). This study aimed to investigate factors related to AI in children with JIA who received systemic steroids.Materials and Methods: Twenty-five children with AI (serum cortisol <18 mu g/dL 30 minutes after adrenocorticotropic hormon stimulation) and 25 children without AI were included in this study. The subjects' characteristics, type of JIA, arthritis location, laboratory measurements, and number of joints involved were recorded. The type of glucocorticoid administered, the treatment protocol, and the cumulative steroid dose were recorded. The primary endpoint was the difference in clinical characteristics, laboratory measurements and systemic corticosteroid dose in those children with or without AI.Results: The median cumulative steroid dose was significantly higher in those patients with AI compared to those without [2,500 (1,370-4,400) mg vs. 963 (650-2,500) mg, p=0.010]. Patients with oligoarticular JIA had a 6.7-fold lower risk of AI compared to those with other JIA types [odds ratio (OR): 0.149, 95% confidence interval (CI): 0.035-0.643, p=0.011]. Those patients with higher cumulative steroid doses (>1,000 mg) had a 7.5-fold higher risk of AI than those with lower doses (OR: 7,500, 95% CI: 1,634-34,416, p=0.010). Conclusion: Our findings show that non-oligoarticular JIA and high cumulative steroid doses are predictive for AI development in this patient subset; thus, systemic corticosteroids should be reserved for more aggressive JIA types and the cumulative dose should be limited to 1,000 mg.

Objective: Familial Mediterranean Fever (FMF) is a hereditary autosomal recessive chronic periodic inflammatory disease, characterized by recurrent fever polyserositis and skin manifestations. The aim of this study was to investigate the bone mineral density (BMD) in prepubertal children with FMF and to determine the relationship between bone density and demographic, laboratory characteristics of the disease. Material and Method: Thirty two previously diagnosed, attack-free, prepubertal FMF patients who were receiving regular colchicine treatment were included in the study. Demographic and clinical data of patients, acute phase reactants, biochemical parameters of bone turnover, bone age, and L1-L4 vertebral mineral density were evaluated. The results of the patients were compared with values determined for healthy Turkish children. Results: All of the patients had z-scores which were greater than -2. Acute phase values and bone metabolism markers were within normal limits. No correlation was found between the aBMD Z-score and demographic data, acute phase reactants and bone metabolism markers. There was a negative correlation between delay in diagnosis and aBMD Z- score. Conclusion: Although aBMD Z-scores were normal in children with FMF the aBMD Z score was found to be lower in children who had had a delay in treatment. Early initiated colchicine therapy is important for optimal bone health in these patients.

Objective: Henoch Schönlein Purpura also known as IgA vasculitis is the most common form of pediatric vasculitis and renal involvement is responsible for the mortality and long-term morbidity. We aimed to describe the epidemiological, clinical, and laboratory characteristics of patients with IgAV and analyze the predicting factors associated with renal involvement. Materials and Methods: This study included188 children diagnosed with IgA vasculitis. Demographical, and clinical data were retrospectively reviewed from the patient files. Results: Of the 188 IgA vasculitis patients, 51.6% were female. The mean±SD age at diagnosis was 8.49±3.35 years, and 66% of them were diagnosed before 10 years of age. All the patients had palpable purpura, 35.6% had arthritis, 34.6% had gastrointestinal system involvement, 12.2% had renal disorders, at the time of diagnosis. Besides 23(12.2%) patients presented with renal involvement, 42(22%) patients developed renal involvement at follow-up. Patients under 10 years of age had significantly more arthritis, patients over 10 years of age had significantly more renal involvement. Among laboratory work-up, erythrocyte sedimentation rate levels were found significantly higher in patients with renal involvement. In multivariate analysis, the occurrence of renal involvement was not associated with any of the defined demographic and clinical characteristics of the disease. Although erythrocyte sedimentation rate levels showed a higher risk ratio, it has only borderline significance. Conclusion: Although IgA vasculitis is a self-limiting disease, renal involvement can cause serious complications. In the presented study, being older than 10 years of age and having high levels of erythrocyte sedimentation rate at the time of diagnosis could serve as a possible predictor of renal involvement.

PURPOSE:Familial Mediterranean fever (FMF) is an idiopathic disease with chronic inflammation. We aimed to determine the changes caused by the chronic inflammatory nature of FMF on the ocular surface, meibomian glands (MG), and conjunctiva via conjunctival impression cytology (CIC).MATERIAL-METHOD:Forty-two FMF patients with a mean age of 11.93±3.92 years and 36 control patients with a mean age of 11.83±3.38 years were included in the study. Ocular surface anomalies of the patients were evaluated using Schirmer II, TBUT and OSDI. MG function (meibum quality), morphology (meiboography), and CIC were evaluated.RESULTS:Although there was a significant difference between the groups in terms of Schirmer II and TBUT, OSDI scores did not significantly differ (P=0.022, 0.010, and 0.099 respectively), and no significant dry eye sign was observed in either group. There was significant difference between the groups in terms of the percent area of MG dropout, MG density, meiboscore (P=0.020, 0.023, and 0.031 respectively), but no significant difference was observed in relation to MG quality (P=0.650). Although conjunctival impression cytology was of a higher grade in the patients with FMF according to Nelson's classification, no significant difference was observed between the groups (P=0.109).CONCLUSION:Although there was a decrease in the number of MGs in FMF patients, no significant deterioration was observed in conjunctival cytology. In these patients, tear film stability may deteriorate in particular. Clinicians should be aware of the possibility of ocular surface disease secondary to MG dropout in patients with FMF.