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.

The effects of dietary changes on bone markers in postmenopausal vertebral osteopenia.

BACKGROUND & AIMS: Nutrition is one of the most important environmental factors affecting the formation of osteopenia. The purpose of this study was to investigate the effects of dietary changes on bone formation and bone resorption markers of postmenopausal women with vertebral osteopenia. METHODS: In this study, 108 women with postmenopausal vertebral osteopenia were included. Patients were observed for a month to identify their regular nutritional status. Before intervention, blood and urine samples were taken from all patients. Then, 2-day food consumption records were taken and the patients were divided into 4 groups. Different types of diets (opposite of their regular diets) were prepared for these groups (1: control, 2: reduced-carbohydrate, 3: reduced-protein, 4: reduced-sodium) and followed for 3 months. At the end of follow-ups, blood and urine samples were taken again and changes in osteocalcin (OC) and N-terminal telopeptide (NTX) levels were examined. RESULTS: According to biochemical analysis, there was a significant decrease (p < 0,001) in OC levels in reduced protein group and an increase (p > 0,05) in reduced carbohydrate group. When NTX levels were assessed, a significant decrease (p < 0.001) in the reduced carbohydrate group and a significant increase in the reduced protein group (p < 0.05) were found. CONCLUSION: Our findings show that reduced carbohydrate diet protected whereas, reduced protein diet negatively affected bone health. Osteopenic individuals were thought to be able to improve bone health and their quality of life by early dietary intervention.

Increased epicardial adipose tissue thickness on transthoracic echocardiography in patients with Behçet disease.

OBJECTIVES: Owing to the fact that the potential frequency of endothelial dysfunction and early atherosclerosis might be higher in Behçet disease, characterized by acute and chronic inflammatory attacks, it may lead to impairment in flow-mediated dilatation and an increase in epicardial adipose tissue thickness. Therefore, we aimed to evaluate whether epicardial adipose tissue thickness and brachial artery flow-mediated dilatation as markers of early atherosclerosis and endothelial dysfunction were associated with Behçet disease. METHODS: Thirty-five patients with Behçet disease and 35 healthy volunteers were included in this study. Epicardial adipose tissue was identified as an anechoic space between epicardial layers on 2-dimensional images, and its thickness was measured on the free wall of the right ventricle. Right brachial artery flow-mediated dilatation was assessed according to recent guidelines. RESULTS: Serum γ-glutamyl transferase (GGT) levels and epicardial adipose tissue thickness were significantly higher (P = .001; P < .001 respectively), whereas flow-mediated, endothelium-dependent dilatation was significantly lower in the Behçet disease group than controls (P < .001). There was a significant negative association between epicardial adipose tissue thickness and flow-mediated dilatation (P < .001). Epicardial adipose tissue thickness was also positively correlated with Behçet disease activity (P< .001), Behçet disease duration (P< .001), and waist circumference (P< .001). Flow-mediated dilatation was negatively correlated with GGT (P< .001), Behçet disease activity (P< .001), and age (P< .001). There was also a significant association between GGT and Behçet disease activity (P < .001). CONCLUSIONS: We found that epicardial adipose tissue thickness was significantly higher and flow-mediated dilatation was significantly lower in patients with Behçet disease than in controls. We suggest that identification of increased epicardial adipose tissue might aid in the diagnosis and treatment of possible coronary artery disease in patients with Behçet disease.

Mucopolysaccharidosis type-IS presenting with onset of carpal tunnel syndrome at adolescence.

Mucopolysaccharidosis type I (MPS I) results from deficiency of the lysosomal enzyme alpha-L iduronidase. Three subtypes, based on severity of clinical findings, have been described, of which MPS type IS (also called Scheie syndrome) is the mildest form. A woman (age, 30 years) and her little brother (age, 21 years) presented to our clinic complaining of atrophy of the thenar muscles, numbness in both hands, and contractures in the finger joints. Electrophysiologic examination showed severe carpal tunnel syndrome for both patients. Findings of cardiac and ocular involvements and decreased level of alpha-L iduronidase confirmed the diagnosis of Scheie syndrome. Enzyme replacement therapy was initiated for the further prevention of musculoskeletal and other organ complications. Delayed diagnosis of MPS type-IS and the musculoskeletal findings are discussed in these 2 familial patients.

Unilateral diaphragmatic paralysis and segmental motor paresis following herpes zoster.

We report the case of a 73-year-old woman who complained of acute onset of pain and weakness of her left shoulder and proximal arm muscles 3 weeks after a diagnosis of herpes zoster. Electromyography revealed involvement of the C5-6 myotomes and the upper trunk of the brachial plexus. Chest X-ray and electromyographic studies documented paralysis of the left diaphragm. One year after onset, muscle strength returned to normal, but radiographic and electrophysiologic findings of diaphragm paralysis were unchanged.