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 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.

COVID-19-associated multisystem inflammatory syndrome in children: Experiences of three centres in Turkey.

BACKGROUND: The pathogenesis and clinical manifestations of the multisystem inflammatory syndrome in children (MIS-C) has not yet been fully elucidated and there is no clear consensus on its treatment yet. OBJECTIVES: To evaluate our patients diagnosed with MIS-C and present them to the literature in order to contribute to the better understanding of this new disease, which entered paediatric practice with the SARS-CoV-2 peak. METHODS: In this study, 17 MIS-C cases diagnosed according to the Centers for Disease Control and Prevention criteria were included. RESULTS: Of the patients, 7 (41.2%) had a comorbidity. Gastrointestinal system involvement was the most prominent in the patients (70.6%). Laparotomy was performed in 3 patients due to acute abdomen. Two patients had neurological involvement. Of the patients, 15 (88.2%) received intravenous immunoglobulin and 13 (76.5%) received both intravenous immunoglobulin and methylprednisolone. Two patients received invasive mechanical ventilation and 4 patients received high flow rate nasal cannula oxygen therapy. One of our patients who needed invasive mechanical ventilation and high vasoactive-inotrope support died despite all supportive treatments including plasmapheresis and extracorporeal membrane oxygenation. CONCLUSIONS: MIS-C picture can have a fatal course and may present with severe gastrointestinal and neurological signs. Unnecessary laparotomy should be avoided.

Treatment of systemic JIA: When do we need a biologic? Real world data of a single center.

OBJECTIVES: This paper aimed to present real-world data of treatment results of a single center in patients with systemic Juvenile Idiopathic arthritis (SJIA), in which methotrexate (MTX) along with glucocorticoids was preferred as the first-line treatment option. METHODS: The medical records of 50 patients (58 episodes) with SJIA were evaluated. All patients with SJIA were hospitalized and were given high dose glucocorticoid treatment along with subcutaneous MTX. A biological agent was added in which disease activity control was not available with MTX. RESULTS: Forty-one (70.6%) of 58 episodes were controlled by MTX, following discontinuation of steroids, while a biologic drug was needed in the remaining 17 (29.4%) episodes. The patients receiving MTX were divided into two groups: Group I (n = 36) (41 episodes) consisted of patients receiving MTX alone, and Group II (n = 14) (17 episodes) consisted of patients receiving MTX plus a biologic agent. Group I was dominated by the monocyclic course (56.1%), whereas group II was dominated by persistent course (70.6%). The initial erythrocyte sedimentation rate (82 vs 67 mm/h) and neutrophil/lymphocyte ratio (8.6 vs 4.1) were significantly elevated in the Group II (p = .003 and p = .007, respectively). NLR of 5.23 predicted the requirement for biological agents with a sensitivity of 66.7%, specificity 71.4%. Odds ratio for NLR ≥ 5.23 was 5.1 in Group II. CONCLUSION: This study suggested that MTX was highly successful in cases with any SJIA episode, regardless of whether arthritis was present or not, even in cases presenting with MAS. A biologic drug is needed, if NLR is greater than 5.23.Key messagesHigh dose glucocorticoids with high dose SC methotrexate are the initial treatment option in systemic JIA.In glucocorticoid dependent patients, where methotrexate is ineffective, biologic therapy is mandatory.A neutrophil/lymphocyte ratio greater than 5.23 predicts the need for early biologic treatment.High dose S.C. MTX could be an option as an initial treatment in SJIA, especially biologics are not available.

Paediatric Behçet's disease with sinus venous thrombosis: experience from three centres in Turkey.

OBJECTIVES: To report our experiences of the juvenile Behçet's disease (BD) patients with cerebral venous sinus thrombosis (CVST) and to review previous studies reporting the clinical characteristics and outcomes of juvenile BD with CVST. METHODS: Clinical characteristics and outcomes of paediatric patients with CVST who met the Paediatric Behçet's Disease (PEDBD) classification criteria for juvenile BD from 3 referral centres in Turkey were reviewed retrospectively. A systematic review of literature of all published data was conducted. RESULTS: The study group consisted of 12 juvenile BD patients with CVST. At the time of CVST diagnosis, the most common symptom was headache (100%), followed by vomiting (25%), blurred vision (16.7%), and disturbances in eye movements (16.7%). Six (50%) patients presented with CVST. Transverse sinus was the most frequently affected sinus (9/12, 75%) followed by superior sagittal sinus. The mean (±2SD) BDCAF at the CVST diagnosis was 6 (±3.8). Four children (33.3%) had another venous thrombosis apart from CVST. All patients received pulse methylprednisolone for three consecutive days continued with oral prednisolone. Steroid treatment was tapered and discontinued minimum in six months. Eleven patients received azathioprine concomitant to steroid treatment at the time of CVST. All the patients received anticoagulant therapy concomitantly. Only one patient who did not receive azathioprine relapsed. Median follow-up period was 4 years (IQR: 2-5.4). In the literature review, we identified nine articles, describing 35 pediatric CVST patients associated with BD. Thirty patients achieved remission, while five patients had residual neurologic deficit. CONCLUSIONS: Neuroimaging is very important in the diagnosis of NBD. We suggest that treatment with immunosuppressants and steroid treatment is essential to decrease the adverse events of corticosteroids in the pediatric population and decrease relapses. Further multicenter studies with prospective follow-up may guide us in better management of these patients.

Clinical and histopathological prognostic factors affecting the renal outcomes in childhood ANCA-associated vasculitis.

OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are very rare in childhood with an increased risk of morbidity and mortality. We aimed to evaluate renal prognostic factors in childhood AAV from the perspective of ANCA serotype, histopathological classification, and five-factor score (FFS). METHODS: Pediatric AAV patients from 11 referral centers in Turkey had been included to the study. The demographics, clinical findings, AAV subtypes, outcomes, and FFS were evaluated retrospectively. Kidney biopsies were classified histopathologically. RESULTS: Totally, 39 patients were enrolled in the study. Among all patients, 74.4% had renal involvement, 56.4% ear-throat-nose involvement, and 51.3% had musculoskeletal involvement. Proteinase 3 (PR3)-ANCA was positive in 48.7%, and myeloperoxidase (MPO)-ANCA was positive in 30.8%. 69.2% of patients had impaired renal function, and 28.2% had progressed to end-stage renal disease (ESRD) during the follow-up. At the time of diagnosis, FFS was ≥ 2 in 53.8%. The most common histopathologic classifications were as follows: crescentic type in 40.7% and sclerotic type in 25.9%. Gastrointestinal and renal involvement, MPO-ANCA positivity, serum creatinine levels, and impaired renal function during the follow-up were significantly higher in patients with FFS ≥ 2, compared to patients with FFS < 2. Patients with FFS ≥ 2 had more common crescentic, mixed and sclerotic histopathologic findings in biopsies. By logistic regression analysis forward method, the strongest single-risk factor among all the parameters was the initial level of creatinine in patients with ESRD, compared to the other patients (p = 0,007). CONCLUSIONS: Evaluation of the FFS, ANCA serology, and the creatinine levels may help to predict renal prognosis.

A plasmonic biosensor pre-diagnostic tool for Familial Mediterranean Fever.

Familial Mediterranean Fever (FMF) is an autosomal recessive genetic disorder, primarily observed in populations around the Mediterranean Sea, linked to MEFV gene mutations. These mutations disrupt inflammatory responses, increasing pyrin-protein production. Traditional diagnosis relies on clinical symptoms, family history, acute phase reactants, and excluding similar syndromes with MEFV testing, which is expensive and often inconclusive due to heterozygous mutations. Here, we present a biosensor platform that detects differences in pyrin-protein levels between healthy and affected individuals, offering a cost-effective alternative to genetic testing. Our platform uses gold nanoparticle-based plasmonic chips enhanced with anti-pyrin antibodies, achieving a detection limit of 0.24 ng/mL with high specificity. The system integrates an optofluidic system and visible light spectroscopy for real-time analysis, with signal stability maintained for up to six months. Our technology will enhance FMF diagnosis accuracy, enabling early treatment initiation and providing a cost-effective alternative to genetic testing, thus improving patient care.

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.

Pediatric Behçet's disease: Experience of a single tertiary center.

Objectives: The aim of this study was to examine the clinical and phenotypic features of pediatric Behçet's disease (PEDBD) in our clinic and present the rates of fulfilling the diagnostic criteria. Patients and methods: Thirty-four patients (20 males, 14 females; mean age: 16.0±2.1 years; range, 10 to 18 years) diagnosed with PEDBD between January 2010 and December 2019 were retrospectively evaluated. Patients were reclassified according to 1990 International Study Group (ISG) criteria, 2014 International Criteria for Behçet's Disease (ICBD), and PEDBD criteria. Results: The mean age at diagnosis was 12.6±3.1 years, the median diagnosis delay time was 12.0 (range, 4.5 to 27.0) months, and the mean age at symptom onset was 10.8±2.9 years. The mean follow-up period was 31.9±20.9 months. Oral aphthous ulcer was observed in 33 (97.1%), genital ulcer in 16 (47.0%), ocular involvement in 15 (44.1%), skin lesion in 11 (32.3%), joint involvement in nine (26.4%), both vascular and neurological involvement in six (17.6%) patients. The pathergy test was positive in 11 (37.8%) patients, and human leukocyte antigen (HLA)-B51 was positive in 11 (78.5%) of 14 patients. The rates of patients meeting the criteria for ISG, ICBD, and PEDBD were 52.9%, 82.4%, and 50.0%, respectively. Conclusion: Pathergy and HLA-B51 can be used as supportive findings in patients who do not meet the diagnostic criteria. However, expert opinion is still the gold standard in diagnosis.

Yoga versus home exercise program in children with enthesitis related arthritis: A pilot study.

PURPOSE: The aim was to compare the effects of yoga and home exercise program on lower extremity functional status, pain, and quality of life in children with enthesitis related arthritis (ERA). METHODS: Twenty-one children with ERA were divided into two groups as yoga (n = 11) and home exercise (n = 10). Yoga group performed supervised yoga exercises twice a week for eight weeks. Home exercise group performed video-based exercises for the same period. Pain in rest and activity, lower extremity functional status, and quality of life were evaluated at baseline and following eight weeks. RESULTS: The groups were similar at baseline (p > 0.05). All the parameters, except parent reported quality of life, significantly improved in yoga group (p < 0.05), where only stair climb test times significantly improved in home exercise group (p < 0.05). CONCLUSIONS: Yoga seems promising for improving lower extremity functional status, pain, and quality of life as an exercise intervention in rehabilitation programs of children with ERA.

Does Familial Mediterranean Fever Provoke Atherosclerosis in Children? Evaluation of Arterial Stiffness and Serum Endocan Levels.

OBJECTIVES: This study aimed to evaluate the risk for atherosclerosis by using echocardiographic arterial stiffness (AS) parameters and serum endocan levels, as a biomarker of endothelial dysfunction (ED) in children with FMF. METHODS: Seventy-nine children with FMF (12-18 years) and 41 healthy children were included, and clinical features (age at the first attack, age at the time of diagnosis, diagnosis delay time, colchicine dose, biological agent usage, MEFV mutations, and symptoms of attacks) of patients were noted. Arterial stiffness parameters were calculated by using echocardiographic aortic measurements with blood pressure monitoring. Hemogram parameters, acute phase reactants, blood glucose and lipid levels of 12 hours of fasting, and serum endocan levels were evaluated for all participants. RESULTS: There were no statistically significance regarding demographic features, acute phase reactants, and hemogram parameters. Blood glucose and lipid levels were similar, except for HDL (lower in FMF group, p=0.029). Serum endocan levels did not differ in two groups (p=0.906). Only stiffness of descending aorta was lower in FMF group (p=0.028), and the other AS parameters were similar between two groups (p>0.05 for each parameters). CONCLUSION: Good disease control could be preventive for atherosclerosis in children with FMF. On the other hand, screening for cardiovascular diseases is essential, particularly for uncontrolled cases. Distribution of MEFV gene mutations KEY POINTS: • Exaggerated inflammation is the prominent feature of FMF attacks; moreover, it is shown that subclinical inflammation might also continue in attack-free periods. • Chronic inflammation contributes to atherosclerotic process in almost all stages by activating endothelial cells, producing reactive oxygen species, and accelerating foam cell and atherosclerotic plaque formations. • However, the results of this study showed that there was no difference in terms of atherosclerotic markers such as serum endocan levels and arterial stiffness parameters between pediatric FMF patients and healthy peers. • Good disease control in pediatric FMF patients may prevent early atherosclerotic changes during childhood, which then may lead a probable decreased risk of subsequent CVD in adulthood.

Clinical features of children with chronic non-bacterial osteomyelitis: A multicenter retrospective case series from Turkey.

OBJECTIVES: This study aims to evaluate demographic, clinical, and radiological characteristics of Turkish children with chronic non-bacterial osteomyelitis. PATIENTS AND METHODS: Between January 2008 and December 2018, a total of 28 patients (10 males, 18 females; median age: 12.5 years; range, 4.5 to 21 years) who were diagnosed with chronic non-bacterial osteomyelitis in three pediatric rheumatology centers were retrospectively analyzed. The demographic, clinical, and laboratory findings of the patients were recorded. RESULTS: The median age at the time of diagnosis was 10.2 years. The median time from symptom onset to diagnosis was 6.5 months. The median follow-up was 18.5 months. The most frequent symptom at onset was arthralgia (75.0%). The most frequently involved bone was the femur (67.9%). Eight (63.6%) of 12 patients had at least one Mediterranean fever gene (MEFV) mutation, indicating a significantly higher prevalence than the Turkish population (14.8%). Five of these patients fulfilled the diagnostic criteria for familial Mediterranean fever (FMF). All patients received non-steroidal anti-inflammatory drugs. Other drugs were methotrexate (46.4%), sulfasalazine (39%), corticosteroids (25%), anti-tumor necrosis factor (anti-TNF) agents (32%), pamidronate (25%), and colchicine (21.4%). Six of eight patients with MEFV mutations were administered with colchicine, and all of them responded to treatment. CONCLUSION: Clinical evolution and imaging investigations should be carefully performed to prevent any delay in the diagnosis of patients with chronic non-bacterial osteomyelitis. Based on our study results, FMF coexistence is worth investigating in patients with chronic non-bacterial osteomyelitis, particularly in the Turkish population.

Comparison of Different Pharmaceutical Preparations of Colchicine in Children with Familial Mediterranean Fever: Is Colchicine Opocalcium a Good Alternative?

BACKGROUND: Colchicine is an anti-inflammatory agent used for preventing familial Mediterranean fever (FMF) attacks and amyloidosis. A significant number of patients are non-responsive or intolerant to the domestic drug colchicum dispert. AIMS: To compare the efficacy and side effects of colchicum dispert and colchicine opocalcium in children with FMF. STUDY DESIGN: A total of 29 children with FMF who used colchicum dispert for at least 6 months initially and colchicine opocalcium for another consecutive 6 months were included. Sex and gender equity in research was considered. Clinical features, visual analog scale for pain scores, exercise-induced leg pain, and FMF severity scores with laboratory parameters were evaluated for both the treatment periods. Bristol stool chart and number of stools per 24 hours were recorded to compare the gastrointestinal side effects. METHODS: A total of 29 children with FMF who used colchicum dispert for at least 6 months initially and colchicine opocalcium for another consecutive 6 months were included. Sex and gender equity in research was considered. Clinical features, visual analog scale for pain scores, exercise-induced leg pain, and FMF severity scores with laboratory parameters were evaluated for both the treatment periods. Bristol stool chart and number of stools per 24 hours were recorded to compare the gastrointestinal side effects. RESULTS: The major indication was non-responsiveness in 18 patients (62%) and intolerance in 11 patients (38%). Usage of colchicine opocalcium (significantly higher dosage than colchicum dispert) showed statistically significant beneficial effects on the number and duration of attacks, visual analog scale for pain, exercise-induced leg pain scores, and FMF severity scores (p<0.05 for each parameter). Bristol stool chart questionnaire scores decreased from 5.62±1.56 to 4.15±1.73 points, and the scores of daily stool number decreased from 0.46±0.894 to 0.03±0.118 points (p<0.05). There were 12 patients who benefited from the switch without a change in dosage, and the clinical features were significantly better with the colchicine opocalcium treatment. CONCLUSION: Pediatric patients with FMF, who have active disease and/or gastrointestinal complaints during the use of colchicum dispert, may benefit from colchicine opocalcium.

Frequency of juvenile idiopathic arthritis and associated uveitis in pediatric rheumatology clinics in Turkey: A retrospective study, JUPITER.

BACKGROUND: Juvenile idiopathic arthritis (JIA), is the most common pediatric rheumatologic disorder with unknown etiology. Currently, no population-based data are available regarding the distribution of categories and frequency of uveitis in patients with JIA in Turkey. The purpose of this study was to evaluate the frequency of JIA-associated uveitis (JIAU) and distribution of JIA categories in a Turkish JIA cohort. METHODS: This was a retrospective study of 500 randomized patients in four pediatric rheumatology clinics in Turkey. RESULTS: Oligoarticular JIA (oJIA) was the most common JIA disease category in this study cohort (38.8%). The frequencies of the other categories were as follows: enthesitis-related arthritis (ERA), 23.2%; rheumatoid factor (RF)-negative polyarthritis, 15.6%; systemic arthritis, 12.2%; juvenile psoriatic arthritis, 5.2%; undifferentiated arthritis, 2.8%; and RF-positive polyarthritis, 2.2%. JIA-associated uveitis was observed in 6.8% of patients at a mean (Standard Deviation, SD) age of 9.1 (3.8) years over a mean JIA disease duration of 4 (1.9) years. Uveitis developed after joint disease, with a mean (SD) duration of 1.8 (1.9) years. Patients with oJIA had the highest rate of uveitis (12.9%) followed by patients with ERA (5.2%) and polyarticular RF-negative disease (3.8%). Compared with persistent oJIA, the extended oJIA category had a > 3-fold higher risk of uveitis (11.3% vs 27.7%; odds ratio, 3.38 [95% Confidence Interval, 1.09-10.4]). The most frequently administered drug after development of uveitis was tumor necrosis factor-alpha inhibitors (38.2%). Five patients (14.7%) had uveitis-related complications that required surgical intervention. CONCLUSIONS: Turkish pediatric patients with JIA experience a lower frequency of oJIA and higher frequency of ERA than their white European counterparts; the occurrence of uveitis is also somewhat lower than expected. Geographic and ethnic factors may affect these differences and need further investigation.

Effects of familial Mediterranean fever on the middle ear.

OBJECTIVE: To evaluate middle and inner ear function and hearing status of children with familial Mediterranean fever (FMF). METHODS: We assigned 56 patients with FMF to the study group and 52 healthy volunteers to the control group. The mean age of patients in the study and control groups were 10.10 ± 3.70 and 9.77 ± 3.74 years, respectively. Physical examination and hearing evaluation were performed in both groups. The audiological test battery included 226-Hz tympanometry, pure tone audiometric evaluation, acoustic stapedial reflex measurements, distortion product otoacoustic emission recording, and wide-band tympanometry assessment. RESULTS: The groups were similar in age and sex ratio (p > 0.05 for both comparisons). Pure tone audiogram and distortion product otoacoustic emission results were also similar for both groups (p > 0.05). The ipsilateral acoustic stapedial reflex were present in both of the groups and all participants had Type A tympanogram. The contralateral acoustic stapedial reflex thresholds were significantly higher in the familial Mediterranean fever group (p < 0.05 for all comparisons). The ambient and peak pressure absorbance values of wide-band tympanometry were significantly lower at 2000 Hz and significantly higher at 4000 Hz in the familial Mediterranean fever group (p < 0.05 for both comparisons). The severity and duration of disease adversely affected the absorbance values of wide-band tympanometry at 2000 and 4000 Hz (p < 0.05). CONCLUSION: To our knowledge, this is the first study to demonstrate the adverse subclinical effects of familial Mediterranean fever on the middle ear. We recommend that children diagnosed with FMF should be closely monitored for future clinical middle ear pathologies.

Risk factors of intravenous immunoglobulin resistance and coronary arterial lesions in Turkish children with Kawasaki disease.

Kawasaki disease (KD) is the most common cause of childhood coronary artery disease. The incidence of coronary artery lesions (CALs) has declined with the routine use of intravenous immunoglobulin (IVIG) treatment, but there is still considerable risk for resistance to IVIG treatment and development of CALs. The present study was aimed to determine the risk factors in Turkish children with IVIG resistant KD and coronary artery involvement. Clinical, laboratory and echocardiographic data were retrospectively analyzed in 94 Kawasaki patients. IVIG resistant and responsive groups were compared. The IVIG resistant group had a higher rate of CALs compared to the IVIG responsive group (p < 0.05). Duration of fever ≥ 9.5 days, C-reactive protein (CRP) ≥ 88 mg/L and Neutrophil/lymphocyte ratio (NLR) ≥1.69 were the best cutoff values for predicting IVIG resistance before treatment. The criteria for at least two of these three predictors were considered to be statistically significant risk factors for detecting IVIG resistance in KD before treatment (76.47% sensitivity, 71.05% specificity and 95% CI were 50.1-93.19% and 59.51-80.89%, respectively). Based on the clinical and laboratory features, we established a new risk-scoring system for predicting IVIG resistance in a cohort of Turkish children with KD. This may be useful for choosing optimal treatment for KD to prevent coronary artery involvement.

Pediatric mesenteric panniculitis: three cases and a review of the literature.

Açari C, Ünsal E, Hakgüder G, Soylu A, Özer E. Pediatric mesenteric panniculitis: three cases and a review of the literature. Turk J Pediatr 2019; 61: 798-803. Mesenteric panniculitis is an inflammatory and fibrotic process in the mesenteric adipose tissue with unknown etiology. It is rarely seen in general, particularly in children. Etiology is unknown, and pathophysiology is not clear. Factors that trigger the disease are malignancy, tuberculosis, trauma, medications and past surgical interventions. There is no pediatric case series in the literature except single case reports. This paper consists of 3 cases: The first case is a 5-month-old girl, the youngest patient in the literature, who was referred to a pediatric surgeon with vomiting and abdominal distention. She had diffused intraabdominal fluid and mesenteric panniculitis documented by perioperative biopsy. The second case had acute abdominal pain with perforated appendicitis, who eventually had mesenteric panniculitis in the evaluation of the pathological specimen. The last case had a diagnosis of polyarticular juvenile idiopathic arthritis (JIA), successfully treated with etanercept, and has been in remission for 2 years. Interestingly, in one of her routine visits, she had pallor, anemia and renal failure. Bilateral hydronephrosis was detected. Magnetic resonance imaging (MRI) of the abdomen revealed retroperitoneal fibrosis, and mesenteric panniculitis was the histopathological diagnosis.

Current information about vaccination practice in pediatric rheumatic diseases and recommendations for future applications.

Açari C, Ünsal E. Current information about vaccination practice in pediatric rheumatic diseases and recommendations for future applications. Turk J Pediatr 2017; 59: 357-368. Pediatric patients with autoinflammatory or rheumatic diseases are at increased risk of infections; therefore, safe and effective immunizations are crucial in the management of these group of patients. Current aggressive treatments involving the early use of immunosuppressive drugs and biological agents have further increased the susceptibility to infections in this group of patients. Therefore, effective and safe vaccination with adequate serological responses is important. In patients with rheumatic diseases, immunogenicity of a vaccine can differ from the healthy population, because of the disease itself or the immunosuppressive treatment received. Moreover, possible effects of vaccination on the underlying disease should be considered. In general, live attenuated vaccines should not be administered when high-dose immunosuppressive drugs are used for immunosuppressed patients. Inactivated vaccine agents have proven to be generally safe in patients with RD. The immune-modulating of biologic agents effects can last for weeks to months after discontinuation, depending on their half lifes. Also, live virus vaccines are contraindicated during therapy and for weeks to months following discontinuation of the biologics. The aim of this review is to cover the current information about vaccination practice in pediatric rheumatologic diseases and to give recommendations for future applications.