EEG Microstate Analysis in Patients with Disorders of Consciousness and Its Clinical Significance.

Disorders of Consciousness are divided into two major categories such as vegetative and minimally conscious states. Objective measures that allow correct identification of patients with vegetative and minimally conscious state are needed. EEG microstate analysis is a promising approach that we believe has the potential to be effective in examining the resting state activities of the brain in different stages of consciousness by allowing the proper identification of vegetative and minimally conscious patients. As a result, we try to identify clinical evaluation scales and microstate characteristics with resting state EEGs from individuals with disorders of consciousness. Our prospective observational study included 28 individuals with a disorder of consciousness. Control group included 18 healthy subjects with proper EEG data. We made clinical evaluations using patient behavior scales. We also analyzed the EEGs using microstate analysis. In our study, microstate D coverage differed substantially between vegetative and minimally conscious state patients. Also, there was a strong connection between microstate D characteristics and clinical scale scores. Consequently, we have demonstrated that the most accurate parameter for representing consciousness level is microstate D. Microstate analysis appears to be a strong option for future use in the diagnosis, follow-up, and treatment response of patients with Disorders of Consciousness.

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.

Association of Pediatric Vasculitis Activity Score with immunoglobulin A vasculitis with nephritis.

BACKGROUND: Immunoglobulin A vasculitis with nephritis (IgAVN) is the most serious complication affecting long-term prognosis. Understanding the risk factors and markers for the development of IgAVN is essential. The aim of this study is to identify IgAVN-associated factors and to evaluate the usability of Pediatric Vasculitis Activity Score (PVAS) at diagnosis as an early marker for the development of IgAVN. METHODS: We conducted a retrospective case-control study of 314 patients divided into two groups: those with nephritis (IgAVN) and without nephritis (non-IgAVN). The groups were compared in terms of clinical symptoms, laboratory values, and PVAS values. RESULTS: In total, 18.5% of the patients had IgAVN; they were older than the non-IgAVN patients (median age was 8.8, p < 0.05). Arthritis/arthralgia, abdominal pain, and intestinal bleeding were more common, systolic and diastolic BP were higher in IgAVN (p < 0.05). CRP, serum creatinine, and urine protein/Cr, PVAS were higher, while serum albumin was lower in IgAVN (p < 0.05). The receiver operator characteristic curve (ROC) analysis showed that IgAV patients with a determined cut-off PVAS value greater than 3 had 70.7% sensitivity in predicting whether or not they would develop IgAVN. Logistic regression analysis found that PVAS > 3 and low serum albumin at the time of diagnosis were independent risk factors for IgAVN. CONCLUSION: Our study revealed that PVAS > 3 at diagnosis is an independent predictor of IgAVN. Patients with PVAS > 3 should be followed more closely to ensure early diagnosis and management of IgAVN. A higher resolution version of the Graphical abstract is available as Supplementary information.

Clues for inflammatory diseases in the differential diagnosis of a child with sacroiliitis.

BACKGROUND: The purpose of this study was to compare the demographic, clinical and laboratory characteristics of patients with enthesitis-related arthritis (ERA), familial Mediterranean fever (FMF) and inflammatory bowel disease (IBD), which are inflammatory diseases that may develop sacroiliitis. Thus, it was aimed to reveal various findings that may indicate primary disease in patients with sacroiliitis. METHODS: Pediatric patients aged 6-18 years, who were being followed with a diagnosis of ERA (n = 62), FMF (n = 590), and IBD (n = 56) over the period 2013-2021 were included in the study. Sacroiliitis (n = 55) was diagnosed by magnetic resonance imaging of the sacroiliac joint, obtained from clinically suspected patients. RESULTS: Sacroiliitis was detected in 54.8% of ERA patients, 2.3% of FMF patients, and 12.5% of IBD patients. The mean follow-up period was 4.1 ± 2.8 years (10 months-8 years) for the entire study group. The most common MRI finding for sacroiliitis was bone marrow edema. Peripheral joint involvement (73.5%) and HLA B27 positivity (64.7%) was significantly higher in ERA patients, and ERA was diagnosed more frequently in patients presenting with sacroiliitis. Non-steroidal anti-inflammatory drugs (NSAIDs) were the first choice of treatment agent when sacroiliitis developed in all three patient groups. CONCLUSIONS: The clinical and laboratory findings of ERA, FMF and IBD can sometimes be intertwined or can even coexist. Treatment may differ depending on the disease associated with sacroiliitis, although NSAIDs may be used in the first-line treatment of all three diseases. Sacroiliitis patients with HLA B27 positivity and peripheral arthritis may need to be addressed as ERA.

Childhood-Onset Sacroiliitis: Causes and Correlation Between Clinical Findings and Magnetic Resonance Imaging.

OBJECTIVE: The aims of this study were to describe disease associations of magnetic resonance imaging (MRI)-confirmed and clinically symptomatic sacroiliitis in pediatric patients with rheumatic diseases and to examine the relationship between patient characteristics and MRI findings of the sacroiliac joint (SIJ). METHODS: Demographic and clinical data were extracted from the electronic medical records of the patients with sacroiliitis followed in the last 5 years. Active inflammatory and structural damage lesions of the SIJ-MRI were examined by the modified Spondyloarthritis Research Consortium of Canada scoring system, and correlation analysis of these results with clinical characteristics was evaluated. RESULTS: A total of 46 symptomatic patients were found to have MRI-proven sacroiliitis of 3 different etiologies: juvenile idiopathic arthritis (JIA) (n = 17), familial Mediterranean fever (FMF) (n = 14), and chronic nonbacterial osteomyelitis (CNO) (n = 8). Seven patients, FMF and JIA (n = 6) and FMF and CNO (n = 1), had a co-diagnosis that might cause sacroiliitis. Although inflammation scores and structural damage lesions did not statistically differ between the groups, capsulitis and enthesitis on the MRI were more frequently detected in the CNO group. There was a negative correlation between symptom onset and inflammation scores of bone marrow edema. Disease composite scores and acute phase reactants were correlated with MRI inflammation scores. CONCLUSIONS: We demonstrated that JIA, FMF, and CNO were the major rheumatic causes of sacroiliitis in children originating from the Mediterranean region. Quantitative MRI scoring tools can be used to assess the inflammation and damage of the SIJ in rheumatic diseases, show discrepancies between them, and have an important correlation with various clinical and laboratory features.

Evaluation of Gastrointestinal System Complaints and Comorbidities in Pediatric Familial Mediterranean Fever Patients.

OBJECTIVE: Familial Mediterranean fever (FMF) is the most prevalent hereditary autoinflammatory disease among children. Abdominal pain and various gastrointestinal system (GIS) manifestations may arise directly from FMF or concomitantly with FMF. This study aimed to evaluate GIS complaints and findings other than classic peritonitis attacks in patients with FMF and to interpret concomitant GIS and hepatic disorders in these patients. METHODS: The medical and genetic findings of patients with FMF who attended our clinic between December 2011 and December 2021 were reviewed. Gastrointestinal system symptoms, liver function tests, abdominal images, and endoscopic and histopathological data were extracted from medical records. RESULTS: A total of 576 pediatric patients (female, 52.3%) diagnosed with FMF were included. Among them, almost one-fifth displayed GIS complaints, such as abdominal pain, defecation problems, and dyspepsia, distinct from typical FMF attacks. High serum aminotransferase levels were detected in 18.4% of the patients, with viral infections being the most common cause of moderate/severe hypertransaminasemia. In addition, during follow-up, 26.9% of them were referred to the pediatric gastroenterology department. At least 1 gastroenterological and hepatobiliary disorder was detected in 17.5% of the patients because of organic and functional GIS disorders or hepatobiliary disorders, such as gastroesophageal reflux disease, esophagitis, functional dyspepsia, and inflammatory bowel diseases. CONCLUSION: Various GIS and hepatic disorders can be encountered in children with FMF. The spectrum of these complaints and pathologies can range from frequently observed health problems to more severe diseases.

Delta neutrophil index and C-reactive protein: a potential diagnostic marker of multisystem inflammatory syndrome in children (MIS-C) with COVID-19.

Multisystem inflammatory syndrome in children (MIS-C) is a life-threatening hyperinflammation syndrome emerging after COVID-19. The serum delta neutrophil index (DNI) reflects the fraction of circulating immature granulocytes and is evaluated in infection and inflammation. The aim of this study is to evaluate the usefulness of DNI as a diagnostic marker in patients with MIS-C and to assess its role in determining the severity of MIS-C. This retrospective, observational study included 83 patients with MIS-C and 113 patients with COVID-19, and 102 healthy controls. C-reactive protein (CRP), the absolute neutrophil count (ANC), absolute lymphocyte count (ALC), DNI, and the platelet count were recorded. The DNI levels were 4.60 ± 5.70% in the MIS C group, 0.30 ± 0.99% in the COVID group, and 0.20 ± 0.56% in the control group (p < 0.001). According to the severity of MIS-C, the DNI level was found to be 1.22% in mild MIS-C, 4.3% in moderate MIS-C, and 5.7% in severe MIS-C. There was a statistically significant correlation between DNI levels and the severity of MIS-C. The cutoff value of DNI for predicting MIS-C was 0.45%. In the analysis of the diagnostic performance of DNI compared with CRP, ANC, ALC and platelet counts, sensitivity, specificity, positive predictive value, and negative predictive value were found to be 79.5%, 97.1%, 95.7%, and 85.3%, respectively.Conclusions: The delta neutrophil index was identified as a diagnostic marker for MIS-C such as ANC, ALC, platelet count, and CRP. DNI levels in hemogram analysis may guide clinicians in determining the diagnosis and severity of MIS-C. What is Known: • Although CRP, sedimentation, ALC, ANC, platelet count, sodium, and albumin are used as first step tests, there is no specific laboratory marker used in the diagnosis of MIS C. • The serum delta neutrophil index (DNI) reflects the fraction of circulating immature granulocytes and is elevated in infection and inflammation. What is New: • DNI is a promising and easily accessible marker that can be used with other markers in the diagnosis and determines the severity of MIS C. • DNI is an easily accessible, inexpensive, and dynamic marker and its levels in simple hemogram analysis will guide pediatricians in determining the diagnosis and severity in MIS C.

Performance of diagnostic criteria in pediatric Behçet's disease.

Behçet's disease is an inflammatory vasculitic disease of unknown etiology characterized by recurrent oral and genital ulcers, ocular findings, and multiple organ involvement. Mucocutaneous findings are the most common symptoms. The most used diagnostic criteria are International Criteria for BD (ICBD), International Study Group (ISG) criteria and pediatric Behçet's disease criteria (PEDBD). Although diagnostic criteria have been defined, the diagnosis is still difficult due to clinical findings developed in pediatric patients. The aim of this study was to evaluate the clinical findings, phenotype characteristics, sensitivity and specificity of diagnostic criteria, and the course of pediatric Behçet's disease (BD). We evaluated retrospectively the files of 67 (29 M/38 F) patients diagnosed with BD according to expert opinion. The patients were reclassified according to ISG, ICBD, and PEDBD criteria. The control group consisted of a total of 69 patients with BD-mimicking diseases or presenting at least one major BD sign followed at the same center. Sensitivity and specificity were evaluated for the criteria. The mean age of diagnosis was 13.38 ± 3.2 years. There were oral aphthous ulcers in 98.5%, genital ulcers in 68.7%, skin lesions in 31.3%, vascular involvement in 17.9%, neurological involvement in 11.9%, positive pathergy test in 33.8%, and positive HLA-B51 in 57.1% of patients. The sensitivity of ICBD, ISG and PEDBD criteria was 88.1, 43.3, and 37.3%, respectively. The specificity of ICBD, ISG, and PEDBD criteria was 100%. Diagnosis of BD in childhood is still difficult. ICBD criteria have the highest sensitivity among the diagnostic criteria. These criteria can also be used in childhood.

Chronic non-bacterial osteomyelitis in children: Outcomes, quality of life.

BACKGROUND: Chronic non-bacterial osteomyelitis is a chronic sterile inflammatory bone condition. We aimed to describe patients' clinical and radiographic findings and to evaluate their response to therapy and their quality of life. METHODS: This cross-sectional study included 18 patients from a single center in Turkey whose clinical, radiological features, and outcomes were reviewed retrospectively. The quality of the patients' lives after treatment was compared with healthy controls using the Pediatric Quality of Life Inventory 4.0. RESULTS: The median age of disease onset was 12 years (IQR 10-14 years) and 11 (61.1%) patients were male. The median follow-up duration was 15 months (IQR 12-22 months). The persistent form of chronic non-bacterial osteomyelitis was the most common pattern in 15 (83.3%) patients and a recurrent pattern was defined in three (16.7%) patients. The lesions were multifocal in all patients and 15 (83.3%) patients had symmetric distribution in whole-body magnetic resonance imaging. The most common sites of arthritis were the knee and sacroiliac joints. Methotrexate was used in 16 (88.9%) patients as first-line therapy. However, some patients were unresponsive to the first-line therapy and needed tumor necrosis factor-α inhibitors (55.6%) and bisphosphonates (16.7%). We observed remission in only four (22.2%) patients, and three (16.7%) patients were unresponsive. The patients had a significantly poorer quality of life than controls (P = 0.005). CONCLUSIONS: Chronic non-bacterial osteomyelitis is an insidious disease that requires detailed analysis for diagnosis and whole-body magnetic resonance imaging is an effective tool for its diagnosis. Despite the advanced treatment, patients with chronic non-bacterial osteomyelitis have a poor quality of life.

Role of Biological Agents in the Treatment of SARS-CoV-2-Associated Multisystem Inflammatory Syndrome in Children.

OBJECTIVES: The aims of this study were to evaluate the role of biological agents in the treatment of severe multisystem inflammatory syndrome in children (MIS-C) and to assess the current application, outcomes, and adverse effects in patients who are followed up in a pediatric intensive care unit (PICU). PATIENTS AND METHODS: This observational, descriptive, medical records review study was performed on patients with MIS-C admitted to the PICU between September 1 and November 1, 2020. Through medical records review, we confirmed that patients were positive for current or recent SARS-CoV-2 infection or for COVID-19 exposure history within the 4 weeks before the onset of symptoms. RESULTS: A total of 33 patients with severe MIS-C were included (21 male) with a median age of 9 years. The most common signs and symptoms during disease course were fever (100%) and abdominal pain (75.5%). Clinical features of 63.6% patients were consistent with Kawasaki disease/Kawasaki disease shock syndrome, and 36.4% were consistent with secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome. Myocardial dysfunction and/or coronary artery abnormalities were detected in 18 patients during the PICU stay. Intravenous immunoglobulin and corticosteroids were given to 33 patients. Anakinra was administered to 23 patients (69.6%). There was a significant increase in lymphocyte and platelet counts and a significant decrease in ferritin, B-type natriuretic peptide, and troponin levels at the end of the first week of treatment in patients who were given biological therapy. Two patients were switched to tocilizumab because of an insufficient response to anakinra. The mortality rate of MIS-C patients admitted in PICU was 6.0%. CONCLUSIONS: Management of systemic inflammation and shock is important to decrease mortality and the development of persistent cardiac dysfunction in MIS-C. The aggressive treatment approach, including biological agents, may be required in patients with severe symptoms and cardiac dysfunction.

Impact of cleft-like indentations on procedural outcome of percutaneous edge-to-edge mitral valve repair.

OBJECTIVES AND BACKGROUND: Percutaneous edge-to-edge mitral valve (MV) repair (PMVR) is broadly applied in high-risk patients with relevant mitral regurgitation (MR). We investigated the incidence of cleft-like indentations (CLI) and their impact on PMVR. METHODS AND RESULTS: In a retrospective single center analysis including 263 patients undergoing PMVR with the MitraClip®-system between 11/2012 and 7/2016, MV anatomy was assessed by 3-dimensional transesophageal echocardiography. CLI were present in 37/263 patients (14.1%). Of these, 62.2% had 1 CLI, 27% had 2 CLI, and 10.8% had 3 CLI, mostly concerning segment P2 or P2/3 of the MV. Baseline characteristics were similarly distributed. Interestingly, most patients with CLI suffered from secondary MR (n = 29, 78%). The number of deployed MitraClips was higher in patients with CLI (2 [1.25-2] vs. 2 [1, 2], p = .035), whereas procedural as well as clinical success was similar: MR grade (1.2 vs. 1.5, p = .061), vena contracta width (4.2 vs. 4.5 mm, p = .293), dPmean (4.2 vs. 4.0 mmHg, p = .618) at discharge and NYHA class at 30 days did not differ between groups. Periprocedural complications were rare and equally distributed between groups. At 30 days, MR reduction persisted in patients with CLI (95.8% of these selected patients had a MR grade ≤ 2). CONCLUSIONS: CLI of the MV are common in patients undergoing PMVR, also when presenting mainly with secondary MR. While the number of clips needed to address MR is slightly higher in patients with CLI, procedural success rates appear not to be affected. PMVR seems to be a safe treatment option for patients with CLI.

Therapeutic plasma exchange: A potential management strategy for critically ill MIS-C patients in the pediatric intensive care unit.

This study aimed to evaluate the effectiveness and the role of therapeutic plasma exchange (TPE) in treatment of children with severe MIS-C. In addition, we assessed demographic data, clinical features, laboratory abnormalities, underlying conditions, treatments, and outcomes. Patients with severe MIS-C who were admitted to the pediatric intensive care unit (PICU) between September 01 and October 05, 2020 were included in this observational, descriptive, retrospective study. The data collected included the patients' demographic data, presenting symptoms, clinical features, laboratory parameters, diagnostic investigations, and medications. Of 27 children with MIS-C, 63 % were male. The median age of the patients was nine years. Intravenous immunoglobulin and corticosteroids were used for treatment in 100 % of the patients, anakinra in 51.8 %, vasopressors in 85.1 %, noninvasive mechanical ventilation in 25.9 %, and invasive mechanical ventilation in 18.5 %. Ten of the 27 patients (37 %) underwent TPE. In the patients who underwent TPE, the median PELOD score was 21 (IQR: 11-30.25) before TPE and 10 (IQR: 10-11) after TPE (p < 0.001). Moreover, their median left ventricular ejection fraction (LVEF) was 52 % (IQR: 49.25 %-55 %) before TPE and median LVEF was 66.5 (IQR: 58 %-68.5 %) after TPE (p = 0.012). The median number of TPE sessions was three (IQR: 2-4.75). The mortality rate of the patients with severe MIS-C admitted to the PICU was 7.4 %. We suggest that TPE should be considered as a therapeutic option in children with severe MIS-C. Early initiation of TPE followed by immunomodulatory therapy in critically ill children with MIS-C may help improve clinical and laboratory outcomes.

Performance of the new Eurofever/PRINTO classification criteria in Familial Mediterranean fever patients with a single exon 10 mutation in childhood.

The diagnosis of Familial Mediterranean fever (FMF) based on clinical findings supported by genetic mutation. Recently, the new Eurofever/PRINTO classification criteria including genetic analysis were established. The aim of this study is to evaluate the performance of the new criteria in FMF patients with a single exon 10 mutation in childhood. The study group consisted of FMF patients who had a single exon 10 mutation in a referral center in Turkey. Patients with periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome were included as a control group. The medical charts of all patients were reviewed retrospectively. A total of 106 FMF patients (59 boys) were enrolled in the study group. The median age at first symptom was 5; the median age at diagnosis was 7 years. The mean follow-up was 33 ± 35.4 months. Majority of the patients (n = 58, 54.7%) had heterozygous M694V, 16 (15%) patients had M694V/E148Q and 13 (13.8%) patients had heterozygous M680I mutation. The sensitivity of the Yalcinkaya-Ozen criteria was 98.1% and it was 97.1% for the Eurofever/PRINTO classification criteria. The specificity of the new Eurofever/PRINTO classification criteria was 96.7% and it was 74.1% for the Yalcinkaya-Ozen criteria. The new Eurofever/PRINTO classification criteria have a good sensitivity as the Yalcinkaya-Ozen criteria in patients with a single exon 10 mutation. Additionaly, the new criteria have better specificity. It should be useful to apply the clinical only criteria where the carrier rate is high.

Evaluation of kidney function tests in HIV-positive patients receiving combined antiretroviral therapy.

INTRODUCTION: Human immunodeficiency virus is a chronic infection that attacks the immune system of the human body, particularly CD4 T lymphocytes. Combined antiretroviral therapies are highly effective in virological suppression of human immunodeficiency virus infection. It has been shown that some retroviral therapies have a higher nephrotoxicity potential. As a result of renal injury, serum creatinine increases and the estimated glomerular filtration rate is reduced. The aim of our study was to assess changes in kidney function during a 24-month period in HIV-positive patients who were begun on combined antiretroviral therapy. MATERIAL AND METHODS: A total of 127 HIV-positive patients were enrolled. The patients were divided into five groups; patients who received no therapy were designated as group 1; those who received Dolutegravir/Abacavir/Lamivudine combination as group 2; those who received Elvitegravir/Cobicistat/Emtricitabine/Tenofovir Alafenamide Fumarate combination as group 3; those who received Emtricitabine/Tenofovir Disoproxil Fumarate/Dolutegravir combination as group 4; and those who received Emtricitabine/Tenofovir Disoproxil Fumarate/Raltegravir combination as group 5. We compared the effects of these drugs on estimated glomerular filtration rate during a 24-month follow-up period. RESULTS: At the 24th month of therapy, a significant difference was observed between the estimated glomerular filtration rate (eGFR) levels of the study groups (P < .001). eGFR level was significantly higher in group 4 compared with groups 1, 2 and 3 (P = .009, P < .001, P < .001, respectively), whereas it was significantly lower in group 5 than groups 1, 2 and 3 (P = .005, P < .001, P < .001, respectively). No significant eGFR difference was found between group 4 and group 5 (P > .05). Serum creatinine level was significantly higher in groups 4 and 5 compared with the other groups (P < .001). CONCLUSION: The use of TDF-containing regimens causes renal dysfunction. Therefore, we recommend close monitoring of renal function, especially in patients treated with TDF.

Is the performance of the international severity scoring system for familial mediterranean fever in children better than other scoring systems?

AIMS: Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterised by recurrent episodes of fever and serosal inflammation with elevated acute phase reactants. Assessing the severity of the disease may be useful in identifying colchicine-resistant patients. The aim of this study is to determine the disease severity of FMF patients according to the Pras, Mor, and International Severity Scoring System for Familial Mediterranean Fever (ISSF) scoring systems and to evaluate the consistency of these three systems. METHODS: The medical records of patients with FMF were retrospectively reviewed. Demographic features, family history of FMF, clinical characteristics at disease onset, laboratory features, Mediterranean fever genetic mutations, treatment regimens, and disease courses were recorded. RESULTS: A total of 205 patients (116 girls) were included in the study. The mean age of the patients was 13.3 ± 4.0 years. The Pras, Mor, and ISSF scores were inconsistent with each other, and there was poor fit between them (generalised Kappa: 0.140 ± 0.029; P < .001). In the receiver operating characteristic (ROC) analysis performed by accepting the clinician's opinion as the gold standard, the ISSF was found to be more sensitive and specific than the other two systems. CONCLUSION: Evaluation of disease severity according to the ISSF in paediatric patients is more sensitive and specific than the Pras and Mor scoring systems.

Clinical features and outcomes of childhood polyarteritis nodosa: A single referral center experience.

OBJECTIVES: The aims of this study were to describe the clinical features, comorbidities and outcome of systemic childhood polyarteritis nodosa (PAN) and to evaluate PAN-like diseases in differential diagnosis. METHODS: The study group consisted of patients who were diagnosed as PAN in a referral center in Turkey. The files of all patients were reviewed retrospectively. Disease activity was evaluated with pediatric vasculitis activity score (PVAS). RESULTS: A total of 19 (13 boys/six girls) patients were enrolled in the study. The mean age of patients was 10.37 ± 3.6 years. The mean duration of follow-up was 5.73 ± 3.74 years. Eight patients (42.1%) were also diagnosed with familial Mediterranean fever (FMF). The cutaneous involvement was higher in patients with PAN than those with FMF-associated PAN (p = .03). The median (min-max) PVAS at diagnosis was 5 (3-7). There was no correlation between PVAS scores at the time of diagnosis and age, clinical findings and relapse. CECR1 mutation was detected in one patient leading to deficiency of adenosine deaminase 2. CONCLUSION: The clinical presentation is variable in children with PAN. PAN-like diseases characterized by necrotizing vasculitis should be considered. The possibility of FMF should be kept in mind if inflammation cannot be controlled.

Prediction of inactive disease and relapse in oligoarticular juvenile idiopathic arthritis.

OBJECTIVE: The aim of this study is to describe clinical features of patients with oligoarticular juvenile idiopathic arthritis (JIA) who achieved inactive disease at 3rd month and also to determine the predictors of relapse and extended course. METHODS: In the cohort study, 88 patients with oligoarticular JIA were retrospectively analyzed. The demographic data, clinical features, medications, relapse rates were recorded. Juvenile Arthritis Disease Activity Score (JADAS) and American College of Rheumatology Pediatric criteria were used to measure disease activity and treatment response at 3, 6 and 12 months. RESULTS: Fifty-nine (67%) patients were females and the mean age at diagnosis was 7.9 ± 4.3 years. The odds of achieving inactive disease (JADAS ≤1) at 3rd month were increased by a lower JADAS27 score at admission. Forty-one (48.8%) of 84 patients relapsed. Ankle involvement at onset, high JADAS27 score at admission, increased ESR at admission and presence of synovial hypertrophy in imaging were risk factors for occurrence of relapse. CONCLUSION: Our results show that a significant proportion of oligoarticular JIA patients relapse after inactive period. JADAS is a useful tool to guide the treatment decisions of patients who may be at risk of high disease activity and relapse.

Relationship Between Epicardial Adipose Tissue and Body Composition as Determined by Multi-Frequency Bioelectrical Impedance Analysis in Patients with Stage 5 Chronic Kidney Disease.

BACKGROUND The main cause of mortality among chronic kidney disease (CKD) patients is cardiovascular disease (CVD). Epicardial adipose tissue (EAT) is considered to be novel cardiovascular risk factor. We assessed EAT in non-dialyzed stage 5 CKD patients and explored the association of EAT with body composition as determined by multi-frequency BIA. MATERIAL AND METHODS The present included 70 stage 5 CKD patients who had not undergone dialysis and 40 healthy control subjects. EAT thickness was assessed by echocardiography. Hydration status and body composition were evaluated by multi-frequency bioelectrical impedance analysis. RESULTS Stage 5 CKD patients had significantly higher EAT thickness than healthy subjects (6.56±1.18 vs. 4.05±1.45, p<0.001). Fat tissue mass, systolic blood pressure (SBP), age, fat tissue index, and body mass index were positively correlated with EAT thickness in the CKD patient group (p<0.05). Lean tissue mass, lean tissue index (LTI), and high-density lipoprotein (HDL) were negatively correlated with EAT thickness in the CKD patient group (p<0.05). Stepwise multiple regression analysis showed that age, SBP, and LTI were independently associated with EAT thickness in CKD patients. CONCLUSIONS We found significantly higher EAT thickness in stage 5 CKD patients who were not on dialysis compared to healthy controls. EAT was significantly associated with age, SBP, and LTI in CKD patients. Interventions to reduce the risk factors associated with EAT thickness might protect against CVD disease in CKD patients.

Pre-Treatment and Post-Treatment Demodex Densities in Patients under Immunosuppressive Treatments.

Background and Objectives: Demodex species are common obligatory parasites and normally present in low number in human beings. Immunosuppression was suggested to be associated with increased density of Demodex mites. Systemic glucocorticoids, cyclosporine, methotrexate, and azathioprine are commonly used immunosuppressive agents. We aim to determine the pre- and post-treatment Demodex densities in patients receiving immunosuppressive therapy and compare with those of healthy subjects. Materials and Methods: Demodex density was investigated at the beginning, first, and third months of the immunosuppressive therapy in 45 patients who received methotrexate, cyclosporine, systemic steroid, or azathioprine treatments and in 45 healthy subjects at the same time as the patients. Five standardized skin surface biopsies were taken from cheeks, forehead, nose, and chin of the patients and control group. The presence of five or more parasites in 1 cm2 area was considered as positive. Results: Demodex test was negative at the beginning of the treatment in all patients. Demodex test was positive in one patient in the first and third months of treatment and in three patients only in the third month of treatment. In the control group, Demodex test was determined as positive in just one healthy individual at the beginning, first and third months of the study. When the patient and control groups were evaluated in terms of Demodex number, there was a statistically significant difference in Demodex density in patients treated with immunosuppressive treatment in the first and third months when compared with the control group (p < 0.05). Conclusion: Immunosuppressive treatment might increase the number of Demodex mites and demodicidosis should be kept in mind in patients on immunosuppressive treatment.

Microvascular abnormalities in asymptomatic chronic smokers: A videocapillaroscopic study.

Cigarette smoking is reportedly associated with coronary, cerebral, and peripheral vascular diseases. Nailfold videocapillaroscopy is a noninvasive imaging technique used to examine the microvasculature. In this study we aim to investigate the capillaroscopic abnormalities of asymptomatic chronic smokers (N = 30), and compare findings to those of healthy nonsmokers (N = 30). Nailfold videocapillaroscopy was performed with a videodermatoscope, with images recorded at 40× magnification. Capillary morphologies were assessed as normal, enlargement, tortuosity, and microhemorrhages. Capillaroscopic abnormalities were seen in 16 (53.3%) of subjects within the smoker group and seven (23.3%) within the nonsmoker group (p < 0.05). Six smokers had only capillary enlargement; another 10 had both capillary enlargement and microhemorrhages. In comparison, enlarged capillaries and both enlarged capillaries and microhemorrhages were observed in three and four nonsmokers, respectively. In conclusion, nailfold capillaroscopic abnormalities were more common among asymptomatic chronic smokers than healthy nonsmokers, with the enlargement of nailfold capillaries being the most common abnormality. Nailfold videocapillaroscopic examination may serve as an efficient tool in determining microvascular abnormalities in asymptomatic chronic smokers not only for risk stratification purposes, but also to take the measures needed to preclude future vascular events.