Severe haematological involvement in children with systemic lupus erythematosus and clinical associations.

OBJECTIVES: To investigate the severe haematological involvement in children with SLE and assess its clinical associations, treatments, outcome and damage accrual. METHODS: The medical charts of children with SLE in whom haematological involvement was observed were reviewed. Severe haematological indices were defined as autoimmune haemolytic anaemia with a haemoglobin concentration < 8 g/dL, thrombocyte count < 30 000/µL, and neutrophil count < 500/µL. RESULTS: Among the 224 patients included, 102 (45.5%) displayed severe indices, predominantly at the initial involvement, and most frequently as severe anaemia in 54 (24.1%) and severe thrombocytopenia in 45 (20.1%). Disease activity did not differ according to the presence of severe disease indices. In addition, the presence of severe indices at initial involvement did not affect the damage accrual. However, a higher rate of damage (51.1% vs. 29.9%, p = 0.002) and steroid-induced damage (28.9% vs. 8.2%, p < 0.001) was evident in patients with flares of the haematological system. Regression analysis revealed that rituximab treatment during the initial episode (OR:4.5, p = 0.006) and the presence of anticardiolipin antibodies (OR:2.3, p = 0.014) significantly increases the odds for haematological system flare. However, severe indices at initial involvement did not increase the odds of a haematological flare. CONCLUSION: Severe haematological indices at onset are common but not related with disease outcomes. Prevention of flares is important to improve outcomes, and a more rigorous maintenance strategy would benefit most to children who display haematological indices refractory to conventional immunosuppressants and those with anti-cardiolipin antibodies.

Anatomical variations of the lateral pterygoid plate and their implications for temporomandibular disorders: A CBCT study.

OBJECTIVE: TMD is a multifaceted condition with various contributing factors, but the actual impact of the lateral pterygoid plate on the development of TMD remains uncertain. This research aims to investigate the relationship between anatomical variations of the lateral pterygoid plate (LPP), including its angle and proximity to the condyle, and the prevalence of temporomandibular disorders (TMD), to improve diagnostic accuracy and therapeutic approaches for TMD. MATERIALS & METHODS: A retrospective analysis was conducted on 189 Cone Beam Computed Tomography (CBCT) images of individuals aged 18 to 45. Inclusion criteria were based on definitive diagnoses of degenerative joint disease, excluding individuals with orthodontic treatments, injuries, or craniofacial disorders.Participants were divided into TMD and control groups using standardized DC/TMD protocols for assessment. A radiologist, blinded to the patient's clinical status, then analyzed the CBCT images. LPP dimensions, angles, and condyle distances were measured using OnDemand 3D Imaging Software. RESULTS: The study found a statistically significant higher average LPP length in females compared to males (right LPP p < 0,001, left LPP p = 0,004), with no significant differences in LPP-condyle distances and angles between genders. Comparing the TMD and control groups revealed a positive correlation between lateral plate angles and TMJ disorders (p = 0,044), suggesting a potential biomechanical linkage. CONCLUSION: Conclusively, the study challenges the assumption that LPP anatomical variations significantly impact TMD while underscoring a potential link between LPP angle and TMD. The novel insight into the potential role of the LPP angle in TMD provides a new direction for research and clinical practice, emphasizing the importance of considering subtle anatomical differences in the management of TMD.

Clinical characteristics of pediatric noninfectious uveitis and risk factors for severe disease: a single-center study.

OBJECTIVES: We aimed to present the demographic, clinical, laboratory, and treatment data of children with non-infectious uveitis and to evaluate the risk factors for the development of complications and the need for biological treatment. METHOD: Patients diagnosed with non-infectious uveitis in childhood and followed up for at least 1 year were included in the study. Demographic data, including age, gender, age at diagnosis, uveitis in first-degree relatives, and rheumatologic diseases, were obtained retrospectively from medical records. The presence of complications or the need for biologic therapy was considered a composite outcome suggesting severe disease. RESULTS: The study included 123 patients (female: n = 59, 48%). The mean age at diagnosis was 14.89 ± 4.86 years. Uveitis was symptomatic in 104 patients (84.6%). Approximately one-quarter of the patients had at least one rheumatic disease (n = 35, 28.5%), the most common being juvenile idiopathic arthritis. Thirty-three patients (26.8%) had anti-nuclear antibody positivity. Biologic agents were needed in 60 patients (48.8%). Complications developed in 14 patients (11.4%). Early age at disease onset (aOR, 0.875; 95% C.I. 0.795-0.965, p = 0.007) and female gender (aOR, 2.99; 95% C.I. 1.439-6.248, p = 0.003) were significantly associated with the need for biologic treatment, while Behçet's disease (BD) was strongly associated with uveitis-related complications (aOR, 14.133; 95% C.I. 2.765-72.231, p = 0.001). CONCLUSION: We suggest that among pediatric patients with non-infectious uveitis, females, those with an early age of disease onset, and those with BD need to be closely monitored due to a significantly increased risk of severe disease. Key Points • Limited data exist on the clinical course of non-infectious uveitis in children and the associated risk factors for severe disease. • Our study reveals that nearly a quarter of pediatric patients with non-infectious uveitis also have a rheumatic disease. • Among pediatric patients diagnosed with non-infectious uveitis, we observed an increased risk of severe disease in those with an earlier onset age, in female patients, and in those diagnosed with Behçet's disease.

Exploring factors for predicting colchicine responsiveness in children with PFAPA.

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) are the most common autoinflammatory syndromes in children. This study aimed to evaluate the clinical and laboratory parameters that may predict colchicine responsiveness.This retrospective, multicenter, cross-sectional study involved nine pediatric rheumatology centers from our country., The patients diagnosed with PFAPA were compared on the basis of their responses to colchicine. In the 806 (42.3% female 57.7% male) patients, the most common clinical findings were fever (100%), exudative tonsillitis (86.5%), pharyngitis (80.9%), and aphthous stomatitis (50.5%). The mean attack frequency was 13.5 ± 6.8 attacks per year lasting for a mean of 3.9 ± 1.1 days. Colchicine treatment was attempted in 519 (64.4%) patients, with 419 (80.7%) showing a favorable response. In patients who underwent MEFV gene analysis (70.8%), the most common variant was M694V heterozygous (16.8%). The presence of pharyngitis (p = 0.03, 95% CI 0.885 to 0.994), the presence of arthralgia (p = 0.04, 95% CI 0.169 to 0.958), and having more frequent attacks (p = 0.001, 95% CI 0.028 to 0.748) were found to be associated with colchicine unresponsiveness, whereas the carriage of the M694V variant (p = 0.001, 95% CI 0.065 to 0.242) was associated with colchicine responsiveness. CONCLUSION: This study identified the presence of pharyngitis, arthralgia, and increased attack frequency in patients with PFAPA as factors predicting colchicine unresponsiveness, whereas the carriage of the M694V variant emerged as a predictor of colchicine responsiveness. Predicting colchicine response at disease onset may facilitate a more effective management of PFAPA. WHAT IS KNOWN: • Colchicine treatment can be used in the prophylaxis of PFAPA disease. • Having the MEFV variant is the most commonly known factor in predicting response to colchicine. WHAT IS NEW: • The presence of pharyngitis or arthralgia, and more frequent attacks in PFAPA disease were found to be independently associated with colchicine unresponsiveness. • Carrying the M694V variant was identified as the sole factor predicting colchicine responsiveness.

Evaluation of pediatric rheumatologists' approach to rituximab use: a questionnaire study.

Rituximab (RTX) is a chimeric monoclonal antibody that targets the CD20 antigen on B cells and is used in various autoimmune disorders. In this study, we aimed to measure the awareness of pediatric rheumatologists about the use of RTX through a survey. Between February and March 2023, a 42-question survey was sent via email to pediatric rheumatology specialists in Turkey. The participants were questioned for which diagnoses and system involvement they preferred to use RTX, which routine tests they performed, vaccination policy, and adverse events that occurred during or after infusion. Forty-one pediatric rheumatologists answered the survey. They prescribed RTX most frequently for systemic lupus erythematosus (87.8%) and ANCA-associated vasculitis (9.8%). Prior to the administration of RTX, 95% of clinicians checked renal and liver function tests, as well as immunoglobulin levels. The most frequently tested hepatitis markers before treatment were HBsAg and anti-HBs antibody (97.6%), while 85.4% of rheumatologists checked for anti-HCV. Clinicians (31.4%) reported that they postpone RTX infusion 2 weeks following an inactivated vaccine. Sixty-one percent of rheumatologists reported starting RTX treatment 1 month after live vaccines, while 26.8% waited 6 months. The most frequent adverse events were an allergic reaction during RTX infusion (65.9%), hypogammaglobulinemia (46.3%), and rash (36.6%). In the event of hypogammaglobulinemia after RTX treatment, physicians reported that they frequently (58.5%) continued RTX after intravenous immunoglobulin administration. CONCLUSIONS: RTX has become a common treatment option in pediatric rheumatology in recent years. Treatment management may vary between clinician such as vaccination and routine tests. WHAT IS KNOWN: • During the course of rituximab therapy, clinicians should be attentive to specific considerations in pre-treatment, during administration, and in post-treatment patient monitoring. WHAT IS NEW: • There are differences in practice among clinicians in the management of RTX therapy. These practice disparities have the potential to impact the optimal course of treatment. • This study highlights that standardized guidelines are needed for RTX treatment in pediatric rheumatology, particularly for vaccination policies and routine tests.

Effective diagnosis of sacroiliitis in children: evaluating magnetic resonance imaging with the Canadian scoring system.

OBJECTIVES: To reveal the differences by comparing the magnetic resonance imaging (MRI) findings of patients with clinically symptomatic sacroiliitis with those undergoing sacroiliac imaging for other reasons using the Canadian Spondyloarthritis Research Consortium-Sacroiliac Inflammatory Scoring System (SPARCC-SIS). METHODS: In this retrospective single-center study, sacroiliac MRIs performed between 2017 and 2023 were evaluated from the database. The SPARCC-SIS scoring system is used to evaluate and grade the inflammation of the sacroiliac joints. Mild inflammation is indicated by a score below 24, moderate by a score of 24-48, and severe by a score above 49. Additionally, structural defects of the sacroiliac joint, such as erosion, sclerosis, and ankylosis, were observed. After MRI evaluation, clinically symptomatic (group 1) and non-symptomatic (group 2) patients were divided into two groups. The clinical and laboratory findings of the patients and MRI findings were compared. The patient's age, gender, clinical information from hospital records, acute phase reactants (APRs), and the presence of the Human Leukocyte Antigen (HLA-B27) gene (if applicable) were thoroughly recorded. RESULTS: One hundred thirty-six children who performed sacroiliac MRI for any indication were included in the study. The APRs positivity, presence of HLA-B27, and SPARCC scoring system were significantly higher in 24 patients with clinical sacroiliitis (group 1) than in 112 patients without sacroiliitis (group 2). In our study, the most common MRI findings in children were bone marrow edema, capsulitis, synovitis, and erosion, while chronic structural changes such as sclerosis and ankylosing were rare. CONCLUSION: In this study, the SPARCC scoring method, which shows the severity of sacroiliac joint inflammation, correlates with the clinical diagnosis of sacroiliitis. In cases with suspected sacroiliitis, except for extraordinary reasons, it can be evaluated with MRI without contrast material and can be graded to guide the clinician in treatment and approach.

Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis on anti-interleukin-1 or -6 therapy.

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 pediatric rheumatology centers 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. 45 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.

Evaluation of childhood malignancies presenting with musculoskeletal manifestations from two different divisions: a multicenter study.

BACKGROUND: The aim of the study was to evaluate the approaches of pediatric rheumatologists and pediatric hematologists to patients with similar musculoskeletal (MSK) complaints and to highlight the differences that general pediatricians should consider when referring patients to these specialties. METHODS: This is a cross-sectional study involving the patients who applied to pediatric rheumatology centers with MSK complaints and were diagnosed with malignancy, as well as patients who were followed up in pediatric hematology centers with a malignancy diagnosis, and had MSK complaints at the time of admission. RESULTS: A total of 142 patients were enrolled in the study. Of these patients, 83 (58.4%) applied to pediatric rheumatology centers, and 59 (41.6%) applied to pediatric hematology centers. Acute lymphoblastic leukemia (ALL) was the most common diagnosis among the patients who applied to both centers, with 80 cases (56.3%). The median age of diagnosis was 87 (interquartile range, IQR: 48-140) months. The most common preliminary diagnosis in pediatric rheumatology centers was juvenile idiopathic arthritis (JIA), with 37 cases (44.5%). MSK involvement was mainly seen as arthralgia, and bone pain. While arthralgia (92.7%) was the most common complaint in rheumatology centers, bone pain (88.1%) was more common in hematology centers. The most frequently involved joints were the knee (62.9%), ankle (25.9%), hip (25%), and wrist (14%). The most common laboratory abnormalities were high lactate dehydrogenase (LDH), high C-reactive protein (CRP), anemia, and high erythrocyte sedimentation rate (ESR). Thrombocytopenia, neutropenia, and high LDH were statistically significantly more frequent in patients admitted to hematology centers than in patients admitted to rheumatology centers (p < 0.001, p=0.014, p=0.028, respectively). Patients who applied to rheumatology clinics were found to have statistically significantly higher CRP levels (p=0.032). CONCLUSIONS: Malignancies may present with only MSK system complaints in childhood. Therefore, malignancies should be included in the differential diagnosis of patients presenting with MSK complaints.

Towards a standardized program of transitional care for adolescents with juvenile idiopathic arthritis for Turkey: a national survey study.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is a prevalent childhood chronic arthritis, often persisting into adulthood. Effective transitional care becomes crucial as these patients transition from pediatric to adult healthcare systems. Despite the concept of transitional care being recognized, its real-world implementation remains inadequately explored. This study aims to evaluate the thoughts and practices of healthcare providers regarding transitional care for JIA patients. METHODS: A cross-sectional survey was conducted among pediatric and adult rheumatologists in Turkey. Based on the American Academy of Pediatrics' six core elements of transitional care, the survey included 86 questions. The respondents' demographic data, attitudes towards transitional care, and practical implementation were assessed. RESULTS: The survey included 48 rheumatologists, with 43.7% having a transition clinic. The main barriers to establishing transition programs were the absence of adult rheumatologists, lack of time, and financial constraints. Only 23.8% had a multidisciplinary team for transition care. Participants agreed on the importance of coordination and cooperation between pediatric and adult healthcare services. The timing of the transition process varied, with no consensus on when to initiate or complete it. Participants advocated for validated questionnaires adapted to local conditions to assess transition readiness. CONCLUSIONS: The study sheds light on the challenges and perspectives surrounding transitional care for JIA patients in Turkey. Despite recognized needs and intentions, practical implementation remains limited due to various barriers. Cultural factors and resource constraints affect the transition process. While acknowledging the existing shortcomings, the research serves as a ground for further efforts to improve transitional care and ensure better outcomes for JIA patients transitioning into adulthood.

Inflammatory comorbidities in the largest pediatric Familial Mediterranean fever cohort: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG).

AIM: The aim of this study was to investigate the frequency and type of FMF-associated inflammatory diseases in a large FMF pediatric patients and to compare them to those FMF patients without concomitant inflammatory diseases. MATERIALS AND METHODS: Familial Mediterranean fever patients enrolled in the Pediatric Rheumatology Academy (PeRA)-Research Group (RG) were included. The patients were divided into two groups according to concomitant inflammatory disease as FMF patients who had a concomitant inflammatory disease (group 1) and FMF patients who did not have a concomitant inflammatory disease (group 1). The clinical findings and treatments were compared between the two groups. RESULTS: The study group comprised 3475 patients with FMF. There were 294 patients (8.5%) in group 1 and 3181 patients (91.5%) in group 2. Juvenile idiopathic arthritis (n = 136) was the most common accompanying inflammatory disease. Arthritis, M694V homozygosity, and the need for biological therapy were more frequently observed in Group 1 (p < 0.05). Fever and abdominal pain were more frequently detected in Group 2 (p < 0.05). FMF patients with concomitant inflammatory diseas more frequently demonstrated colchicine resistance. There were no significant differences in the median attack frequency, chest pain, amyloidosis, erysipelas-like erythema, or family history of FMF between the two patient groups. CONCLUSION: To the best of our knowledge, this is the largest pediatric cohort reviewed to date. FMF patients may have different clinical profiles and colchicine responses if they have with concomitant inflammatory diseases. Key points • FMF is associated with some inflammatory comorbidities diseases. • To the best of our knowledge, this is the largest cohort evlauated pediatric FMF associated inflammatory comorbidities diseases reviewed to date.

The effect of temporomandibular joint dysfunction on the craniocervical mandibular system: A retrospective study.

BACKGROUND: Temporomandibular disorders are the most common condition affecting the orofacial region, resulting in pain and dysfunction. OBJECTIVE: This study aimed to elucidate the ambiguous association between cervical features and temporomandibular disorders by measuring the rotations between the skull-atlas, atlas-axis and mandible-atlas and examining the relationship between these rotations and temporomandibular disorders. METHODS: Cone-beam computed tomography (CBCT) images from 176 patients, 97 females and 79 males with an average age of 25.7 years were used in this study. The patients were divided into two groups: those with joint dysfunction (n = 88) and those without (n = 88). The study employed various methods to determine rotations in the skull-atlas, atlas-axis and mandible atlas based on anatomical landmarks and measurements. These methods include the use of specific planes, angles and distances to identify and measure rotation. Data analysis was performed using the TURCOSA statistical software (Turcosa Analytics Ltd Co, Turkey, www.turcosa.com.tr). RESULTS: The results showed that the degree of rotation between the skull and the atlas was higher in the TMD group than in the control group (p < .001). Similarly, Atlas-axis rotation was significantly higher in the TMD group (p < .001). However, no significant difference was found between mandible atlas rotations in the two groups (p = .546). The study also found a significant difference between the direction of rotation between the atlas and axis and the direction of mandible atlas rotation (p < .001) as well as between skull and atlas rotations and mandible-atlas rotations (p < .001). CONCLUSION: Overall, the study suggests that there is a relationship between the skeletal structures of the cranio-cervico-mandibular system and TMD. Skull-atlas and atlas-axis rotations may play an important role in the aetiology of TMD in individuals with TMD. Therefore, it is important to evaluate rotations in the skull-atlas-axis region for the treatment of TMD.

A clinical overview of paediatric sarcoidosis: Multicentre experience from Turkey.

OBJECTIVES: We aimed to outline the demographic data, clinical spectrum, and treatment approach of sarcoidosis in a large group of patients and sought to figure out the variations of early-onset (EOS) and late-onset paediatric sarcoidosis (LOS). METHODS: The study followed a retrospective-descriptive design, with the analysis of medical records of cases diagnosed as paediatric sarcoidosis. RESULTS: Fifty-two patients were included in the study. The median age at disease onset and follow-up duration were 83 (28.2-119) and 24 (6-48) months, respectively. Ten (19.2%) cases had EOS (before 5th birthday) and 42 (80.7%) cases had LOS. The most common clinical findings at the time of the disease onset were ocular symptoms (40.4%) followed by joint manifestation (25%), dermatological symptoms (13.5%), and features related to multi-organ involvement (11.5%). Anterior uveitis was the most common (55%) one among ocular manifestations. Patients with EOS displayed joint, eye, and dermatological findings more commonly than patients with LOS. The recurrence rate of disease in patients with EOS (5.7%) and LOS (21.1%) were not statistically different (P = .7). CONCLUSIONS: Patients with EOS and LOS may present with variable clinical features and studies addressing paediatric sarcoidosis cases in collaboration between disciplines will enhance the awareness of this rare disease among physicians and assist early diagnosis with lesser complications.

Alleviation of Aluminum-Induced Oxidative Stress, Trace Element, and Mineral Levels in Rat Tissues Protective Role of Pomegranate Juice (Punica Granatum L.).

The present investigation examined the impact of pomegranate (Punica granatum L.) juice on trace elements, minerals, and oxidative stress in relation to the potential harm inflicted by aluminum chloride (AlCl3) in rats. Rats were split into four groups at random for this purpose: control (C), pomegranate juice (PJ), aluminum chloride (A), and PJ + A. For 30 days, PJ was orally administered by gavage at a rate of 4 mL/kg every other day, whereas AlCl3 was administered intraperitoneally at 8.3 mg/kg. Spectrophotometric analysis was used to measure the levels of malondialdehyde (MDA), glutathione (GSH), superoxide dismutase (SOD), and catalase (CAT) enzyme activity in various tissues. In addition, high-resolution continuum source flame atomic absorption spectrometry (HR-CS FAAS) was used to determine the amounts of the elements Al, Cu, Fe, Mn, Zn, Ca, and Mg in the tissues. It was discovered that when PJ therapy was applied to all tissues, the antioxidant enzymes SOD and CAT activity increased, the GSH level rose, and the MDA level, a sign of lipid peroxidation, decreased. Al and Ca levels increased in the A group relative to the C group in all tissues, whereas they decreased in the A + PJ group relative to the A group. Group A exhibited a proportionate increase in Fe levels in the liver and renal tissues compared with group C. Furthermore, the A group's brain tissue had a higher Fe level than the C group's. The A + PJ group's brain tissue had a lower Fe level than the A group's. Our findings demonstrate that PJ therapy greatly decreased Al buildup and oxidative stress in tissues while controlling variations in trace element levels. In addition, it is concluded that PJ might have value as a strong chelating agent to prevent Al poisoning.

Effect of systemic atorvastatin on bone regeneration in critical-sized defects in hyperlipidemia: an experimental study.

PURPOSE: Hypocholesterolemic medications similar to atorvastatin are efficient in lowering blood lipid levels; however, compared to other medications in the statin family, their impact on bone metabolism is claimed to be insufficient. The impact of atorvastatin on bone regeneration in dental implantology in individuals with hyperlipidemia who received atorvastatin in the clinic is doubtful. METHODS: In the study, 16 male New Zealand rabbits of 6 months were used. All rabbits were fed a high-cholesterol diet for 8 weeks, and hyperlipidemia was created. It was confirmed that the total cholesterol level in rabbits was above 105 mg/dl. A critical-sized defect was created in the mandible. The defect was closed with xenograft and membrane. Oral 10 mg/kg atorvastatin was started in the experimental group, and no drug was administered in the control group. At 16th week, animals were sacrificed. For histomorphological examination, the new bone area, osteoclast, and osteoblast activities were evaluated. RESULTS: While new bone area (45,924 µm2, p < 0.001) and AP intensities (105.645 ± 16.727, p = 0.006) were higher in the atorvastatin group than in the control group, TRAP intensities in the control group (82.192 ± 5.346, p = 0.021) were higher than that in the atorvastatin group. CONCLUSIONS: It has been found that high blood lipid levels will adversely affect bone graft healing and the use of systemic atorvastatin contributes to bone healing. Clinicians should pay attention to the selection of surgical materials, considering the importance of questioning drug use in their patients and the risks in cases of non-use.

The safety of canakinumab in systemic juvenile idiopathic arthritis and autoinflammatory diseases in pediatric patients: a multicenter study.

OBJECTIVE: To evaluate the safety of canakinumab using real-world data in patients with systemic juvenile idiopathic arthritis (sJIA) and autoinflammatory diseases (AID). RESEARCH DESIGN AND METHODS: This was a cross-sectional observational, multicenter study. Patients diagnosed with AID and sJIA treated with canakinumab were included in the study. The participating 13 centers retrospectively collected their patients' data. RESULTS: A total of 335 patients were involved in the study. Among these patients, 280 were in the AID group and 55 were in the sJIA group. Canakinumab was administered at a median dose of 3 (2.5-4) mg/kg. The median total exposure time to canakinumab was 1.9 (0.8-3.2) years, corresponding to 759.5 patient-years. Seven hundred and seventy-nine total adverse events (AE) were identified. The total incidence of AE, and serious adverse events (SAE) throughout the study period was 1.02 per patient-years. The upper respiratory tract infection rate was 0.7 per patient-years, while the other infection rate was 0.13 per patient-years. While no death was observed in any patient, SAE were observed in 8 patients. Interstitial lung disease, anaphylaxis, or anaphylactoid reactions were not observed in any patient. CONCLUSIONS: Real-life data from a large cohort of patients suggests that canakinumab is as safe as claimed in clinical trials.

Is it possible to extend the dose interval of canakinumab treatment in children with familial Mediterranean fever? PeRA group experience.

BACKGROUND: There is no clear data on the optimal duration of treatment with anti-interleukin-1 drugs in colchicine-resistant familial Mediterranean fever patients, as well as on the dose interval. This study aimed to assess patients whose canakinumab dose interval was adjusted according to a specific protocol, with the objective of evaluating the effectiveness of implementing this protocol for the patient care. METHODS: The files of 45 patients whose canakinumab treatment interval was opened with a standard protocol previously determined by the Delphi method were retrospectively reviewed. RESULTS: Canakinumab treatment was initiated once a month for all patients. In the sixth month of canakinumab treatment, a dose interval extension was introduced; however, 7 patients (15.5%) experienced an attack, and consequently, no further interval extension was administered to them. For 29 patients, the dose interval was successfully extended to once every three months, as they remained attack-free for a year after the first interval extension. Nine patients continued receiving the drug every 2 months, as they had not yet completed one year since the first extension. The study found no significant correlation between experiencing an attack during the dose interval extension protocol and the number, duration of attacks, or autoinflammatory diseases activity index score. CONCLUSION: Extending treatment intervals with canakinumab in colchicine-resistant familial Mediterranean fever shows promise for favorable outcomes.

Macro- and trace elements in plants from Mauritania and risk assessment.

This study aimed to determine the content of elemental levels of eight plants species originating from Mauritania and used as medicinal plant and to assess their risk for human health. The range of elemental content in the studied plant parts of these plant species were 0.51-16.1 mg/g for Ca, 2.63-6.49 mg/g for Mg, 11.2-201 µg/g for Al, 6.5-28.2 µg/g for Zn, 5.6-453 µg/g for Fe, 1.1-6.4 µg/g for Ni, 11.0-302 µg/g for Mn and 0.7-9.0 µg/g for Cu. The Cr, Pb, Cd and Co levels were below the limits of detection in all samples. The estimated weekly intake (EWI) and target hazard quotient (THQ) were calculated. Except for Al the THQ values were below 1, so it could be concluded that these plant species have low probability of causing non-cancer health problems for the consumer.

Do the features of juvenile psoriatic arthritis change according to age? A comprehensive evaluation of the PeRA Research Group Registry.

OBJECTIVES: To describe the clinical features and treatment outcomes of children with juvenile psoriatic arthritis (JPsA) and to compare the distinct patterns of the disease between early-onset and late-onset age groups. METHODS: Patients with JPsA followed regularly for at least 6 months between 2010-2020 in 7 pediatric rheumatology centers in Turkey were included in the study. The demographic features, clinical manifestations, treatment strategies, and outcomes of the patients were evaluated retrospectively. RESULTS: Eighty-seven (46 male/41 female) patients were included in the study. The mean age at diagnosis was 11.9 ± 4.5. Fifty-seven (65.5%) patients had psoriasis at the time of diagnosis, arthritis preceded psoriasis in 10 (11.5%) patients. Thirty (34.5%) patients had dactylitis, 28 (32.2%) had nail pitting, 36 (41.4%) had involvement of the small joints, 20 (23%) had enthesitis. Sacroiliitis was detected in 11 (12.6%) patients by magnetic resonance imaging. Anti-nuclear antibodies (ANA) were positive in 35 (40.2%) patients. Twelve children (%13.8) were in the early-onset (<5 years) group. Uveitis and ANA positivity were more common in the early-onset group. Active joint counts and activity scores of our patients showed significant improvement at 6th month and at the last control compared with baseline. CONCLUSION: About one-third of patients with JPsA do not have psoriasis at the time of diagnosis. In some patients, no skin lesion is seen during the course of the disease. Children with psoriatic arthritis seem to display two different phenotypes. Younger children have female predominance, ANA positivity, and uveitis, while older children have more axial involvement.

The Effects of Sheep Tail Fat, Fat Level, and Cooking Time on the Formation of Nε-(carboxymethyl)lysine and Volatile Compounds in Beef Meatballs.

This study aimed to determine the effects of fat type (sheep tail fat (STF) and beef fat (BF)), fat levels (10, 20, or 30%), and cooking time (0, 2, 4, and 6 min, dry heat cooking at 180 °C) on the carboxymethyl lysine (CML) content in meatballs. pH, thiobarbituric acid reactive substance (TBARS), and volatile compound analyses were also performed on the samples. The use of STF and the fat level had no significant effect on the pH value. The highest TBARS value was observed with the combination of a 30% fat level and STF. CML was not affected by the fat level. The highest CML content was determined in meatballs with STF at a cooking time of 6 min. In the samples cooked for 2 min, no significant difference was observed between STF and BF in terms of the CML content. STF generally increased the abundance of aldehydes. Aldehydes were also affected by the fat level and cooking time. A PCA provided a good distinction between groups containing STF and BF regardless of the fat level or cooking time. Pentanal, octanal, 2,4-decadienal, hexanal, and heptanal were positively correlated with CML.

How common is remission in rheumatoid factor-positive juvenile idiopathic arthritis patients? The multicenter Pediatric Rheumatology Academy (PeRA) research group experience.

OBJECTIVE: Rheumatoid factor (RF)-positive polyarthritis is the least common type of juvenile idiopathic arthritis (JIA). Functional disability in RF-positive polyarthritis patients is much more severe than in patients with other subtypes; but data on this subtype alone is limited. This study aimed to analyze clinical features, long-term follow-up, treatment response, and remission status in a large multicenter cohort of RF-positive polyarthritis patients. METHODS: This retrospective study included RF-positive polyarthritis patients that were followed up for ≥ 6 months between 2017 and 2022 by the Pediatric Rheumatology Academy (PeRA)-Research Group (RG). Data on patient demographics, clinical and laboratory characteristics were obtained from medical charts. JIA treatments and duration of treatment were also recorded. The patients were divided into 2 groups based on methotrexate (MTX) response, as follows: group 1: MTX responsive, group 2: MTX unresponsive. Clinical and laboratory findings were compared between the 2 groups. RESULTS: The study included 56 (45 female and 11 male) patients. The median age at onset of RF-positive polyarthritis was 13.2 years [(interquartile range) (IQR): 9.0-15.0 years] and the median duration of follow-up was 41.5 months (IQR: 19.5-75.7 months). Symmetrical arthritis affecting the metacarpophalangeal and proximal interphalangeal joints of the hands was commonly observed. Subcutaneous MTX was the preferred initial treatment; however, it was ineffective in 39 (69.6%) of the patients. Of 25 patients followed for 24 months, 56% still had active disease at 24 months. CONCLUSION: During 2 years of treatment, 44% of RF-positive polyarthritis patients have inactive disease, and they should be considered as a distinct and important clinical entity requiring aggressive and early treatment.