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.

Osteoprotegrin interacts with biomarkers and cytokines that have roles in osteoporosis, skin fibrosis, and vasculopathy in systemic sclerosis: A potential multifaceted relationship between OPG/RANKL/TRAIL and Wnt inhibitors.

Objectives: We explored the interactions of osteoprotegerin (OPG) with biomarkers of bone turnover and cytokines, including soluble receptor activator for nuclear factor kappa beta ligand (sRANKL), tumor necrosis factor-related apoptosis-induced ligand (TRAIL), and Wnt inhibitors in osteoporosis, vasculopathy and fibrosis related to systemic sclerosis (SSc). Methods: The study included 46 SSc patients and 30 healthy controls. Skin thickness, pulmonary fibrosis and/or hypertension, digital ulcers, and calcinosis cutis of SSc patients were assessed. We determined bone mineral density (BMD), and OPG, sRANKL, TRAIL, secreted frizzled-related protein 1 (sFRP-1), Dickkopf-related protein 1 (DKK-1), sclerostin in the serum of both patients and controls. Results: OPG, sclerostin, and sFRP-1 levels were similar between patients and controls (P > 0.05). Femoral neck and lumbar spine BMD and vitamin D levels were lower, and the OC, NTX, sRANKL, DKK1 and TRAIL levels were significantly higher, in patients than in controls (p < 0.05). In subgroup analysis, patients with higher modified Rodnan skin score (mRodnan) had higher DKK-1, sclerostin, and TRAIL levels (p < 0.05); those with diffuse SSc subtype had lower BMD values than those with limited SSc (p < 0.05). Skin and pulmonary fibrosis linked negatively with BMD measures. Conclusion: we showed that sRANKL levels were higher and correlated with bone turnover markers. It may be related to osteoporosis in SSc. The OPG level was unaltered in SSc patients. Higher TRAIL levels associated with skin thickness may indicate vascular dysfunction or injury. Higher DKK-1 and sclerostin levels may be related to a reactive increase in cells and be prominently linked to fibrosis in SSc.

S1000A12, Chitotriosidase, and Resolvin D1 as Potential Biomarkers of Familial Mediterranean Fever.

Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterised by periodic inflammatory attacks. We investigated changes in monocyte-granulocyte derived S10012A and chitotriosidase in both the attack and silent period of FMF for better estimation of inflammation. Endogenous resolvin was determined for utility to restrict inflammation. This study included 29 FMF patients (15 M/14 F) and 30 healthy controls (15 M/15 F). Serum levels of highly sensitive C-reactive protein, serum amiloid A (SAA), S100A12, chitotriosidase, and resolvin D1 were measured. Age, sex, body mass indexes, and lipids were similar between patients and controls. Biomarkers including hs-CRP, SAA, S100A12, chitotriosidase, and resolvin D1 were higher in the attack period of FMF patients compared to controls (P < 0.001). When FMF patients in the silent period were compared with their attack period, hs-CRP, SAA, and chitotriosidase were found elevated in the attack period (P < 0.001, P < 0.001, and P = 0.02 respectively). Serum levels of SAA, S100A12, chitotriosidase, and resolvin D1 in the silent period of FMF patients were still found elevated compared to healthy controls, indicating subclinical inflammation (P < 0.001, P < 0.001, P = 0.009, and P < 0.001 respectively ). In subgroup analysis, patients with M694V homozygote and heterozygote mutations had higher S10012A and hs-CRP compared to other mutation carriers. Our findings indicate that chitotriosidase and S10012A are useful in diagnosis and detection of subclinical inflammation and/or assessment of disease activity in FMF patients. They could be more informative for inflammation in various disease states compared to hsCRP and SAA. Resolvin D1 is elevated in both the attack and silent periods of FMF. It may be helpful to restrict inflammation.

Hyperuricemia and its related factors in an urban population, Izmir, Turkey.

The aim of this study was to examine the prevalence of hyperuricemia and its associated factors in an urban area of Izmir, located in western Turkey. Our study group was selected by computerized sampling from the participants of a larger population-based study searching for the prevalence of rheumatoid arthritis in Balcova and Narlidere districts of Izmir. A total of 132 subjects (69 women and 63 men) were included in this study. Serum uric acid, glucose, creatinine and lipid levels were studied. Body composition along with body fat percentage was determined anthropometrically. A total of 16 subjects had hyperuricemia (4 women and 12 men). The overall prevalence of hyperuricemia was 12.1% and the mean uric acid level was 4.9 +/- 1.3 mg/dl. Males had significantly higher uric acid levels than females (P < 0.05; 5.5 +/- 1.3 vs. 4.3 +/- 1.1 mg/dl, respectively). The prevalence of hypertension, diabetes, obesity and metabolic syndrome was 24.4, 5.3, 28 and 26.5%, respectively. There was no gouty subject. Sum of skinfold thickness (SFT) measurements and creatinine levels were the independent predictors of hyperuricemia (beta = 0.45, 0.47, respectively). Uric acid measurement is important not only for inflammatory rheumatic disorders but also for predicting metabolic syndrome and related coronary artery disease. There is sex difference in uric acid levels in favor of women most probably explained by gonadal hormones. Hyperuricemia is significantly predicted by anthropometric measure of SFT which is a simple clinical screening method along with creatinine levels.

Prevalence of spondyloarthritis in Turkish patients with inflammatory bowel disease.

Rheumatic manifestations are the most common extraintestinal findings of inflammatory bowel disease (IBD), although there are wide variations among different studies. The only previous Turkish study reported a rather high prevalence of spondyloarthritis (SpA) in patients with IBD. We aimed to determine the frequency of SpA and ankylosing spondylitis (AS) in patients with IBD attending a gastroenterology clinic from a referral centre. The study was conducted in 122 patients with established diagnosis of IBD [28 with Crohn's disease (CD) and 94 with ulcerative colitis (UC)]. A detailed medical history was obtained and a complete physical examination was performed in all the patients. Standard pelvic X-rays for examination of the sacroiliac joints were performed only when clinically indicated. The X-rays were read blindly by an experienced rheumatologist and reported according to the established grading system. The modified New York criteria were used to classify AS, and the European Spondyloarthropathy Study Group criteria for SpA. The prevalence of AS and SpA in patients with IBD was 8.2 and 28.7%, respectively. SpA was found to be significantly more common in the patients with CD compared to patients with UC, but the frequency of AS was not different between these two groups. There was no correlation between localisation or extent of the intestinal inflammation and presence of AS and SpA. A higher frequency of women was observed in patients diagnosed as SpA. Almost half of the patients with SpA (45.7%) had not been diagnosed before the study, although they had a history of IBP and/or peripheral arthritis. This study suggests that the prevalences of SpA and AS in Turkish patients with IBD are similar to those in many other populations. There may be a significant female predominance of SpA among patients with IBD.

Prevalence of ankylosing spondylitis and related spondyloarthritides in an urban area of Izmir, Turkey.

OBJECTIVE: To determine the prevalence of ankylosing spondylitis (AS) and related spondyloarthritides (SpA) in an adult urban population of Izmir, Turkey. METHODS: A survey was conducted of 2887 subjects aged 20 years or over, selected by cluster sampling. Those who responded positively to the screening questions were contacted by 2 rheumatologists and evaluated in detail to establish presence of AS (modified New York criteria) or related SpA (ESSG criteria). RESULTS: In the initial screening, 2835 subjects participated; 422 were considered screening-positive and a telephone interview was done with 328 (78%). Based on their clinical history, 145 subjects were invited to the hospital and 120 (83%) agreed to do so. After detailed evaluation, 31 subjects were classified as having SpA (including 14 with AS). The age- and sex-adjusted prevalence was estimated to be 0.49% for AS (95% CI 0.26-0.85), and 1.05% for SpA (95% CI 0.70-1.50). The prevalence of AS was 0.54% in men (95% CI 0.19-1.20) and 0.44% in women (95% CI 0.19-0.88), and that of SpA was 0.88% in men (95% CI 0.42-1.59) and 1.22% in women (95% CI 0.73-1.89). CONCLUSION: This epidemiological study suggests a high prevalence (0.49%) of AS in an adult urban population from western Turkey, which equals that of rheumatoid arthritis in the same population. The overall prevalence of SpA, including AS, was 1.05%. A minimal male predominance was noted among AS patients, which disappeared among the whole group of patients with SpA.

Irritable bowel syndrome in persons who acquired trichinellosis.

BACKGROUND AND AIM: The postinfectious irritable bowel syndrome (PI-IBS) frequently follows bacterial infections. Since people suffering from PI-IBS and Trichinella spiralis-infected mice develop similar findings, this animal model has been successfully used for PI-IBS studies; however, IBS has never been studied in humans after trichinellosis. The aim of this study was to evaluate the IBS symptoms in people who had acquired trichinellosis. PATIENTS AND METHODS: A large outbreak of trichinellosis caused by Trichinella britovi occurred in Izmir, Turkey, in 2003-2004. The diagnosis of trichinellosis was confirmed by serology and muscle biopsy. After clinical and laboratory evaluations, 72 patients (38 women, 34 men, mean age 33.2 +/- 10.4 yr) with trichinellosis without preexisting IBS were enrolled in the study. Noninfected people (N=27) were used as control group. A questionnaire was developed to assess symptoms of IBS and alarm symptoms. The first interview was done face-to-face. After 2, 4, and 6 months of the first interview, the questionnaire was readministered to the patients without any information on the occurrence of a previous trichinellosis syndrome, while it was applied after a year only to the patients who had suffered IBS symptoms according to at least one of the previous interviews. RESULTS: According to Rome II criteria, PI-IBS developed in 10 of 72 (13.9%) people with confirmed trichinellosis, who had no preexisting IBS. The rate of PI-IBS was 13.9% and 13.9% at the 4th and 6th months, respectively. The symptoms of PI-IBS persisted in five of them up to 1 yr. People without trichinellosis did not develop any IBS symptoms. CONCLUSIONS: This is the first report of the development of PI-IBS after T. britovi. Consequently, IBS can be considered as a secondary syndrome induced by trichinellosis.

Circulated activated platelets and increased platelet reactivity in patients with Behçet's disease.

Behçet's disease (BD) is a multisystem disorder. Venous as well as arterial thrombosis is a common complication of BD but exact pathogenetic mechanism of the thrombotic tendency is not well known. This study aimed to evaluate circulating activated platelets and platelet reactivity in Behçet's patients. Twenty-two Behçet's patients (4 female, 18 male; mean age 38.6 +/- 10.9 years) and 20 control subjects (8 female, 12 male; mean age 38.8 +/- 9.4 years) were included. Those patients who had hypertension, hyperlipidemia, peripheral or coronary artery disease, hepatic or renal function abnormality, and who were using aspirin and other platelet-active drugs were excluded. Platelet activity and reactivity to adenosine diphosphate (ADP) were measured by whole blood flow cytometry. We assessed markers of platelet degranulation (P-selectin; CD62P) and the activated glycoprotein IIb/IIIa receptor (PAC1 binding to fibrinogen binding site) before and after stimulation with ADP. Platelet P-selectin expression was not significantly different between patients and control subjects both at baseline (p=0.420) and after stimulation (p=0.56). Baseline (p=0.001) and ADP-stimulated (p=0.003) PAC1 binding was significantly higher in Behçet's patients than in the control group. Clinical activity has no effect on P-selectin expression and PAC1 binding. There is evidence of platelet activity and hyperreactivity in patients with BD and this may contribute to a prothrombotic state. In addition to aspirin, other antiplatelet drugs may be useful in the prevention and treatment of thrombosis in Behçet's patients.

Evaluation of left ventricular diastolic dysfunction with conventional and current Doppler techniques in Behcet's disease.

Behcet's disease (BD) is a systemic vasculitis characterized by genital and oral ulcers, uveitis, and other organs' involvement. Left ventricular (LV) diastolic dysfunction has been documented in BD. However, conventional echocardiographic techniques have serious limitations like its dependence on preload, afterload and heart rate. Recently, new techniques like colour M-mode and tissue Doppler imagining (TDI) have provided additional concept in the assessment of diastolic function. The aim of the present study was to investigate the LV diastolic dysfunction with conventional and new echocardiographic techniques in BD. Forty-eight patients with BD (25 women, 23 men) and 26 healthy volunteers (15 women, 11 men) were enrolled in the study. LV diastolic functions were examined with mitral inflow pulse wave Doppler, TDI and mitral flow propagation rate (MFPR). The following were accepted as diastolic dysfunction: in mitral inflow pulse wave Doppler, E/A<1, isovolumic relaxation time (IVRT)>110 ms and deceleration time of E wave (DT)>240 ms; in TDI of mitral ring with pulse wave, E'/A'<1; and in MFPR, velocity slope (Vp)<45 cm/s. The two groups were comparable in age, sex, heart rate, body mass index, smoking, hyperlipidemia and basic echocardiographic measurements. LV diastolic dysfunction was significantly higher in BD group according to E/A<1 (p<0.05). When echocardiographic measurements were compared one by one for two groups, As' (late diastolic TDI wave in septal wall) was found to be significantly higher in BD group (p<0,0001). IVRT was longer in BD group than in controls, but it did not reach statistical significance (p=0,06). Diastolic dysfunction of LV is more frequent in patients with BD than in control according to E/A and As'. Conventional and current techniques like TDI and colour M-mode Doppler echocardiography could be used to investigate diastolic functions in BD.

Acute trichinellosis in children compared with adults.

OBJECTIVES: Trichinellosis is a cosmopolitan parasite infection caused by Trichinella nematodes that is acquired from consumption of raw meat from several animal species. Knowledge of the clinical pattern and laboratory features of the disease in childhood is limited. The purpose is to study the clinical pattern of trichinellosis caused by Trichinella britovi in children and to compare it in household adults. METHODS: We evaluated all children up to 17 years of age and their adult householders exposed to the consumption of infected meat during an outbreak of trichinellosis. A questionnaire was developed to record clinical data. The blood sample was collected for blood count, muscle enzymes, serum electrolytes, albumin and serology. All exposed children were treated with mebendazole, and severe symptomatic patients received prednisolone. Clinical and laboratory presentations and outcome were recorded. To evaluate the clinical picture of trichinellosis in childhood, clinical and laboratory findings were compared between children and household adults with a confirmed diagnosis who consumed the same amount of infected meat. RESULTS: In 47 (62%) of 76 children with suspected trichinellosis, the diagnosis was serologically confirmed. The main clinical and laboratory findings in children were fever, abdominal pain, myalgia, facial and/or eyelid edema, rash, eosinophilia and increased muscular enzymes. The incubation period was similar in children and adults, but myalgia (66% versus 96%, P < 0.01), facial and/or eyelid edema (57% versus 86%, P < 0.05), eosinophilia (52% versus 96%, P < 0.01) and increased serum creatine kinase (38% versus 79%, P < 0.01) were less common in children than in adults. Seroconversion occurred in fewer children than adults, but the difference was not statistically significant. CONCLUSIONS: T. britovi infection shows a benign course and a milder clinical picture in children than in adults who consumed the same amount of infected meat.

Thyrotoxic periodic paralysis in a Turkish male; the recurrence of the attack after radioiodine treatment.

Thyrotoxic hypokalemic periodic paralysis (THPP) is a rare complication of hyperthyroidism and an uncommon form of hypokalemic periodic paralysis. Its differentiation of more common forms of periodic paralysis is important because aggressive treatment can place the patient at risk for rebound hyperkalemia. Treatment of the underlying thyroid dysfunction cures the muscle symptoms. Here we describe a 37-year-old Turkish male with THPP whose paralysis attack recurred soon after administration of radioactive iodine.