Pediatric vasculitis.

Purpose Of Review: The aim of this review is to define childhood vasculitis and to highlight new causative factors and treatment modalities under the guidance of recently published studies.

Recent Findings: Childhood vasculitis is difficult to diagnose because of the wide variation in the symptoms and signs. New nomenclature and classification criteria were proposed for the diagnosis of pediatric vasculitis.

Development of a medication adherence scale for familial Mediterranean fever (MASIF) in a cohort of Turkish children.

Objectives: To develop and assess the validity and reliability of an adherence scale concerning medical treatment in paediatric FMF patients.

Methods: The Medication Adherence Scale in FMF Patients (MASIF) is a 18-item questionnaire that evaluates adherence to medication in four domains. Validation of the instrument was accomplished in paediatric FMF patients (aged 2-18 years) under medication at least for 6 months.

Achromobacter causing a thrombophlebitis and osteomyelitis combination: a rare cause.

Achromobacter xylosoxidans is a Gram-negative, aerobic bacillus, present in normal human flora of the skin and gastrointestinal tract. Infections due to Achromobacter are infrequent and have mostly been reported in immunocompromised patients. Rarely, however, the microorganism can cause soft tissue infections even in healthy subjects with a history of trauma.

Efficacy and safety of canakinumab in adolescents and adults with colchicine-resistant familial Mediterranean fever.

Introduction: This open-label pilot study aimed to investigate the efficacy of canakinumab in colchicine-resistant familial Mediterranean fever (FMF) patients.

Method: Patients with one or more attacks in a month in the preceding 3 months despite colchicine were eligible to enter a 30-day run-in period. Patients who had an attack during the first run-in period advanced to a second 30-day period.

A Difficult Case of Hodgkin Lymphoma with Differential Diagnosis of Tuberculosis and Sarcoidosis.

We report here the case of a 14-year-old boy with history of fever, weight loss, and mediastinal lymphadenopathy. The clinical symptoms and laboratory findings mimicking tuberculosis and sarcoidosis complicated the diagnostic process. He was diagnosed with Hodgkin's lymphoma after several X-rays, computed tomography, positron emission tomography-computed tomography, laboratory tests and three lymph node biopsy.

Chronic recurrent multifocal osteomyelitis: a rare skeletal disorder.

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare non-infectious inflammatory bone disease of unknown aetiology. CRMO mainly affects the metaphyses of long bones and spine in children and young adolescents. It presents with recurrent episodes of bone pain and fever, resembling bacterial osteomyelitis, but cultures of lesions are sterile and it is unresponsive to antibiotic therapy.

Screening Mucopolysaccharidosis Type IX in Patients with Juvenile Idiopathic Arthritis.

Mucopolysaccharidosis is a group of lysosomal disorders of a deficiency of specific enzyme required for glycosaminoglycan degradation. Mucopolysaccharidosis type IX is the rarest form of mucopolysaccharidosis. To date, only four patients have been reported.

Treatment in juvenile rheumatoid arthritis and new treatment options.

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease of the childhood with the highest risk of disability. Active disease persists in the adulthood in a significant portion of children with juvenile rheumatoid arthritis despite many developments in the diagnosis and treatment. Therefore, initiation of efficient treatment in the early period of the disease may provide faster control of the inflammation and prevention of long-term harms.

Association of familial Mediterranean fever in Turkish children with inflammatory bowel disease.

Aim: Familial Mediterranean fever (FMF) and inflammatory bowel disease (IBD) carry similar clinical and biological properties. Both are characterized with chronic inflammation attacks and neutrophil migration and impaired apoptosis mechanism are present in the areas of damage in both conditions. In our study, we aimed to determine the frequency of association of FMF in patients with IBD, to compare the demographic, clinical, laboratory and treatment response properties in these patients with the ones in other IBD patients and to determine association of FMF especially in treatment-resistant patients.

Familial Mediterranean fever with a single MEFV mutation: comparison of rare and common mutations in a Turkish paediatric cohort.

Objectives: Presence of common MEFV gene mutations strengthened the diagnosis of FMF in addition to the typical clinical characteristics of FMF. However, there are also rare mutations. P369S, A744S, R761H, K695R, F479L are the main rare mutations in Turkish population.

Effect of strengthening versus balance-proprioceptive exercises on lower extremity function in patients with juvenile idiopathic arthritis: a randomized, single-blind clinical trial.

Objective: The aim of this study was to investigate the effects of two exercise programs on lower extremity function in patients with juvenile idiopathic arthritis.

Design: Thirty patients with juvenile idiopathic arthritis participated in this study. Pain, passive range of motion, muscle strength, balance, and functional abilities were assessed with the Numeric Rating Scale, goniometer, handheld dynamometer, Flamingo Balance Test, Functional Reach Test, 10-meter walking test, 10-stair climbing test, and Childhood Health Assessment Questionnaire.

Biological agents in familial Mediterranean fever focusing on colchicine resistance and amyloidosis.

Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory syndrome. FMF is caused by mutations in the MEFV gene which encodes the pyrin protein. FMF is characterized by sporadic, paroxysmal attacks of fever and serosal inflammation, lasting 1-3 days.

Health related quality of life measure in systemic pediatric rheumatic diseases and its translation to different languages: an international collaboration.

Background: Rheumatic diseases in children are associated with significant morbidity and poor health-related quality of life (HRQOL). There is no health-related quality of life (HRQOL) scale available specifically for children with less common rheumatic diseases. These diseases share several features with systemic lupus erythematosus (SLE) such as their chronic episodic nature, multi-systemic involvement, and the need for immunosuppressive medications.

Use of tissue Doppler and its comparison with other pulse Doppler echocardiography in the evaluation of diastolic functions in patients with active juvenile idiopathic arthritis.

Juvenile idiopathic arthritis (JIA) is a systemic chronic inflammatory disease. Cardiac involvement as pericarditis, myocarditis and valvular disease is common in JIA. This study aims to assess left ventricular (LV) diastolic functions with tissue Doppler imaging (TDI) and to compare it with conventional Doppler echocardiography (pulse wave Doppler (PWD)) techniques in patients with active JIA.

Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients.

Objective: To describe the clinical, laboratory, and histopathologic features, current treatment, and outcome of patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA).

Methods: In this multinational, multicenter study, pediatric rheumatologists and hemato-oncologists entered patient data collected retrospectively into a web-based database.

Results: A total of 362 patients, 22% of whom had MAS at the onset of systemic JIA, were included in the study by 95 investigators from 33 countries.

Primary hypertrophic osteoarthropathy caused by homozygous deletion in HPGD gene in a family: changing clinical and radiological findings with long-term follow-up.

Autosomal recessive primary hypertrophic osteoarthropathy1 (PHOAR1) is characterized by delayed closure of the fontanels, digital clubbing, arthropathy and periostosis. Homozygous mutations in hydroxyprostaglandin dehydrogenase (HPGD) gene are the underlying pathology of PHOAR1. The aim of this study was to analyze the HPGD gene and the changing clinical and radiological findings with advancing age of two siblings with the diagnosis of PHOAR1.

Novel adenosine deaminase 2 mutations in a child with a fatal vasculopathy.

Unlabelled: Adenosine deaminase 2 (ADA2) deficiency due to CECR1 mutations is a recently defined disorder that involves systemic inflammation and vasculopathy often associated with polyarteritis nodosa. We report on a 5-year-old girl with a severe vasculopathy who carried two novel mutations in CECR1.

Conclusion: Identification of CECR1 mutations in patients with vasculopathy may lead to earlier diagnosis of ADA2 deficiency.

Regular aerobic training combined with range of motion exercises in juvenile idiopathic arthritis.

Objective: To assess the effects of regular aerobic training combined with range of motion (ROM) exercises on aerobic capacity, quality of life, and function in children with juvenile idiopathic arthritis (JIA).

Methods: Thirty patients with JIA and 20 healthy age-matched controls (mean age ± SD, 11.3 ± 2.

FMF50: a score for assessing outcome in familial Mediterranean fever.

Background: Colchicine is the main treatment for familial Mediterranean fever (FMF). However, biological agents and other treatments are available for patients who are unable to receive optimal treatment.

Objective: To develop outcome criteria that define response to treatment.

Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy.

Background: Polyarteritis nodosa is a systemic necrotizing vasculitis with a pathogenesis that is poorly understood. We identified six families with multiple cases of systemic and cutaneous polyarteritis nodosa, consistent with autosomal recessive inheritance. In most cases, onset of the disease occurred during childhood.

Primary headaches in pediatric patients with chronic rheumatic disease.

Objectives: To assess the presence, prevalence and clinical characteristics of primary headaches in pediatric patients with chronic rheumatic diseases such as juvenile idiopathic arthritis (JIA) and familial Mediterranean fever (FMF), and to analyze the common pathophysiological mechanisms.

Study Design: In this noncontrolled, cross-sectional study, a semi-structured 53 item headache questionnaire was administered to subjects with FMF and JIA, and interviewed a total sample size of 601 patients younger than16years of age. The questionnaires were then analyzed according to the International Headache Society's diagnostic criteria.

Severe digital necrosis in a 4-year-old boy: primary Raynaud's or jellyfish sting.

Raynaud's phenomena is a common disorder which may be primary or secondary to some connective tissue disorders such as systemic sclerosis and systemic lupus erythematosus. Jellyfish sting is a rare but life-threatening cause of Raynaud's phenomena. Digital gangrene is reported in 3% of children with secondary Raynaud's phenomena but does not occur in children with primary Raynaud's phenomena.