A combination of non-invasive tests for the detection of significant fibrosis in patients with metabolic dysfunction-associated steatotic liver disease is not superior to magnetic resonance elastography alone.

OBJECTIVES: The aims of the present study were to investigate a combination of magnetic resonance elastography (MRE) and vibration-controlled transient elastography (VCTE) or MRE and fibrosis score 4 (FIB-4) in the detection of significant fibrosis in patients with metabolic dysfunction-associated steatotic liver disease (MASLD). METHODS: Between November 5, 2021, and March 4, 2022, a total of 119 consecutive patients with MASLD were included. Liver stiffness was measured using liver biopsy, MRE, VCTE, and FIB-4. Data were collected from outpatient visit charts. Significant fibrosis was defined as ≥ stage 2 fibrosis. RESULTS: All 119 MASLD patients were Caucasian, and their median age was 55 years. MRE, VCTE, and FIB-4 demonstrated significant accuracy in the detection of significant fibrosis with an area under the ROC curve (AUC) of 0.848 ± 0.036 (p < 0.001), 0.632 ± 0.052 (p = 0.012), and 0.664 ± 0.051 (p = 0.001), respectively. However, the diagnostic performance of MRE was superior compared to that of VCTE (AUC difference: 0.216 ± 0.053, p < 0.001) and FIB-4 (AUC difference: 0.184 ± 0.058, p = 0.001). With logistic regression analysis, it was determined that when compared to MRE alone, a combination of MRE and TE (p = 0.880) or MRE and FIB-4 (p = 0.455) were not superior for detecting significant fibrosis. CONCLUSIONS: MRE alone is an accurate and non-invasive method for the identification of MASLD patients with significant fibrosis. CLINICAL RELEVANCE STATEMENT: Magnetic resonance elastography alone accurately detects significant fibrosis in patients with metabolic dysfunction-associated steatotic liver disease. KEY POINTS: • In routine clinical practice, several non-invasive biochemical-based biomarkers and imaging methods are widely used to assess liver fibrosis in patients with metabolic dysfunction-associated steatotic liver disease. • Magnetic resonance elastography (MRE) is more accurate than vibration-controlled transient elastography (VCTE) or fibrosis score 4 (FIB-4) for assessing liver fibrosis and identifying significant fibrosis in patients with metabolic dysfunction-associated steatotic liver disease. • The combination of MRE and VCTE or MRE and FIB-4 was not superior to MRE alone.

Evaluation of Magnetic Resonance Elastography and Transient Elastography for Liver Fibrosis and Steatosis Assessments in the Liver Transplant Setting.

BACKGROUND: Liver graft fibrosis affects long-term graft and patient survival in liver transplant recipients. Transient elastography and magnetic resonance elastography are widely used for the assessment of liver fibrosis in routine clinical practice, but are limited in liver transplant settings. The aims of the present study were to evaluate the accuracy of magnetic resonance elastography and transient elastograph in the assessment of liver fibrosis in liver transplant recipients, and to determine the recurrence rates of post-transplant hepatic steatosis and liver fibrosis. METHODS: A total of 126 consecutive liver transplant recipients were included. Magnetic resonance elastography and transient elastography were performed for to measure liver stiffness. RESULTS: The most common cause of liver transplantation was hepatitis B virus-induced cirrhosis (50%). The mean liver stiffness value with transient elastography was 6.1 ± 3.0 kPa, and the mean magnetic resonance elastography value was 2.7 ± 1.0 kPa. A significant positive correlation was found between magnetic resonance elastography and transient elastography in terms of liver stiffness measurement (r = 0.61, P < .001). Obesity and the underlying etiology of liver diseases did not have any significant negative effect on magnetic resonance elastography and transient elastography measurements. During the follow-up, the post-transplant recurrence rates of hepatic steatosis and hepatic fibrosis were 26% and 37%, respectively. The recurrence rates of post-transplant hepatic steatosis and liver fibrosis were slightly higher in recipients with non-alcoholic fatty liver disease-related cirrhosis than those with viral hepatitisrelated etiologies (44% vs 27%, P = .43; 44% vs 30%, P = .45, respectively). CONCLUSION: Magnetic resonance elastography and transient elastography are accurate in assessing liver fibrosis in the liver transplant setting. Obesity and the underlying etiology of primary liver disease do not influence the measurements.

Use and detailed metric properties of patient-reported outcome measures for rheumatoid arthritis: a systematic review covering two decades.

INTRODUCTION: The importance of patient-reported outcome measures (PROMs) for rheumatoid arthritis (RA) clinical studies has been recognised for many years. The current study aims to describe the RA PROMs used over the past 20 years, and their performance metrics, to underpin appropriate tool selection. METHODS: The study included a systematic search for PROMs that have been in use over the period 2000-2019, with detailed documentation of their psychometric properties, and a user-friendly presentation of the extensive evidence base. RESULTS: 125 PROMs were identified with psychometric evidence available. The domains of pain, fatigue, emotional functions, mobility, physical functioning and work dominated, with self-efficacy and coping as personal factors. Domains such as stiffness and sleep were poorly served. The most frequently used PROMs included the Health Assessment Questionnaire Disability Index (HAQ), the Short Form 36 (SF-36), the EuroQoL and the Modified HAQ which, between them, appeared in more than 3500 papers. Strong psychometric evidence was found for the HAQ, and the SF-36 Physical Functioning and Vitality (fatigue) domains. Otherwise, all domains except stiffness, sleep, education and health utility, had at least one PROM with moderate level of psychometric evidence. CONCLUSION: There is a broad range of PROMs for measuring RA outcomes, but the quality of psychometric evidence varies widely. This work identifies gaps in key RA domains according to the biopsychosocial model.

Cross-diagnostic scale-banking using rasch analysis: Developing a common reference metric for generic and health condition-specific scales in people with rheumatoid arthritis and stroke.

OBJECTIVES: To develop a common reference metric of functioning, incorporating generic and health condition-specific disability instruments, and to test whether this reference metric is invariant across 2 health conditions. DESIGN: Psychometric study using secondary data analysis. Firstly, the International Classification of Functioning, Disability and Health (ICF) Linking Rules were used to examine the concept equivalence between the World Health Organization Disability Assessment Schedule (WHODAS 2.0), Health Assessment Questionnaire (HAQ) and Functional Independence Measure (FIMTM). Secondly, a scale-bank was developed using a reference metric approach to test-equating, based on the Rasch measurement model. PARTICIPANTS: Secondary analysis was performed on data from 487 people; 61.4% with rheumatoid arthritis and 38.6% with stroke. RESULTS: Three sub-domains of the WHODAS 2.0 and all items of the HAQ and FIMTM motor mapped on to the ICF chapters d4 Mobility, d5 Self-care and d6 Domestic life. Test-equating of these scales resulted in good model fit, indicating that a scale bank and associated reference metric across these 3 instruments could be created. CONCLUSION: This study provides a transformation table to enable direct comparisons among instruments measuring physical functioning commonly used in rheumatoid arthritis (HAQ) and stroke (FIMTM motor scale), as well as in people with disability in general (WHODAS 2.0).

Características clínicas y evolución del reflujo vesicoureteral en la infancia / Clinical characteristics and outcome of childhood vesicoureteral reflux

Introducción. El objetivo fue evaluar las características clínicas y la evolución del reflujo vesicoureteral (RVU) según el sexo y grado de RVU.Población y métodos. Se incluyeron pacientes con RVU vistos durante el seguimiento de rutina entre enero de 2014 y enero de 2015. Se registraron las características demográficas, la evolución, los laboratorios y las imágenes.Resultados. Se seleccionó a 220 pacientes, cuya media de edad del diagnóstico era 3,17 ± 3,08 años; en ese momento, los varones eran menores que las niñas (2,00 ± 2,59 vs. 3,81 ± 3,15, p < 0,001). La infección urinaria fue la presentación más frecuente, seguida de hidronefrosis prenatal (HNP). El 22 % de los pacientes tuvo reflujo de grado 1-2; el 51 %, de grado 3; y el 27 %, de grado 4-5. En el reflujo de grado 4-5, las ecografías y gammagrafías con ácido dimercaptosuccínico (DMSA) marcado con 99mTc presentaron más anomalías, y se realizaron más cirugías (p < 0,001). En los varones, fueron más comunes el reflujo de grado 4-5 (43,6 % vs. 18,3 %) y las anomalías ecográficas (77 % vs. 54 %) y en la DMSA (77 % vs. 59 %) (p < 0,05). En las niñas, hubo mayores tasas de infección urinaria, disfunción de las vías urinarias inferiores y resolución espontánea (p < 0,05).Conclusiones. A pesar de la menor edad al momento del diagnóstico, la resolución espontánea fue menor en los varones, y estos presentaron HNP, reflujo grave y anomalías radiológicas más frecuentemente.

Introduction. The aim of the study was to assess the clinical features and outcome parameters of children with vesicoureteral reflux (VUR) based on gender and VUR grade.Population and methods. Patients with VUR who were seen during routine follow-up visits at Ankara University Children's Hospital between January 2014-January 2015 were included in this retrospective study. Patient demographics, clinical course, laboratory investigations, imaging were noted.Results. Two hundred and twenty patients were recruited. Mean age at the time of diagnosis was 3,17 ± 3,08 years. Boys were diagnosed at younger ages as compared to girls (2.00 ± 2,59 vs. 3,81 ± 3.15, p < 0.001). Urinary tract infection (UTI) was the most common presentation. The second presentation form was antenatal hydronephrosis (AHN) which was more common in males (25.6 %, p < 0.001). Twenty-two percent of the patients had grade 1-2, 51 % grade 3 and 27 % grade 4-5 reflux. Patients with grade 4-5 reflux had more abnormal ultrasound (US) and Tech 99m dimercaptosuccinic acid scintigraphy (DMSA) findings and surgery was performed more frequently in this group (p < 0.001). In males, grade 4-5 reflux (43.6 % vs. 18.3 %), abnormal US (77 % vs. 54 %) and DMSA (77 % vs. 59 %) findings were more frequent (p < 0.05). In girls higher rates of UTIs, lower urinary tract dysfunction (LUTD) and spontaneous reflux resolution were seen (p < 0.05).Conclusions: Despite younger age at diagnosis, spontaneous resolution was found lower in boys and they had more frequent AHN, more severe reflux, and radiological abnormalities.

Clinical characteristics and outcome of childhood vesicoureteral reflux. / Características clínicas y evolución del reflujo vesicoureteral en la infancia.

INTRODUCTION: The aim of the study was to assess the clinical features and outcome parameters of children with vesicoureteral reflux (VUR) based on gender and VUR grade. POPULATION AND METHODS: Patients with VUR who were seen during routine follow-up visits at Ankara University Children's Hospital between January 2014-January 2015 were included in this retrospective study. Patient demographics, clinical course, laboratory investigations, imaging were noted. RESULTS: Two hundred and twenty patients were recruited. Mean age at the time of diagnosis was 3,17 ± 3,08 years. Boys were diagnosed at younger ages as compared to girls (2.00 ± 2,59 vs. 3,81 ± 3.15, p < 0.001). Urinary tract infection (UTI) was the most common presentation. The second presentation form was antenatal hydronephrosis (AHN) which was more common in males (25.6 %, p < 0.001). Twenty-two percent of the patients had grade 1-2, 51 % grade 3 and 27 % grade 4-5 reflux. Patients with grade 4-5 reflux had more abnormal ultrasound (US) and Tech 99m dimercaptosuccinic acid scintigraphy (DMSA) findings and surgery was performed more frequently in this group (p < 0.001). In males, grade 4-5 reflux (43.6 % vs. 18.3 %), abnormal US (77 % vs. 54 %) and DMSA (77 % vs. 59 %) findings were more frequent (p < 0.05). In girls higher rates of UTIs, lower urinary tract dysfunction (LUTD) and spontaneous reflux resolution were seen (p < 0.05). CONCLUSIONS: Despite younger age at diagnosis, spontaneous resolution was found lower in boys and they had more frequent AHN, more severe reflux, and radiological abnormalities.

Introducción: El objetivo fue evaluar las características clínicas y la evolución del reflujo vesicoureteral (RVU) según el sexo y grado de RVU. Población y métodos: Se incluyeron pacientes con RVU vistos durante el seguimiento de rutina entre enero de 2014 y enero de 2015. Se registraron las características demográficas, la evolución, los laboratorios y las imágenes. Resultados: Se seleccionó a 220 pacientes, cuya media de edad del diagnóstico era 3,17 ± 3,08 años; en ese momento, los varones eran menores que las niñas (2,00 ± 2,59 vs. 3,81 ± 3,15, p < 0,001). La infección urinaria fue la presentación más frecuente, seguida de hidronefrosis prenatal (HNP). El 22 % de los pacientes tuvo reflujo de grado 1-2; el 51 %, de grado 3; y el 27 %, de grado 4-5. En el reflujo de grado 4-5, las ecografías y gammagrafías con ácido dimercaptosuccínico (DMSA) marcado con 99mTc presentaron más anomalías, y se realizaron más cirugías (p < 0,001). En los varones, fueron más comunes el reflujo de grado 4-5 (43,6 % vs. 18,3 %) y las anomalías ecográficas (77 % vs. 54 %) y en la DMSA (77 % vs. 59 %) (p < 0,05). En las niñas, hubo mayores tasas de infección urinaria, disfunción de las vías urinarias inferiores y resolución espontánea (p < 0,05). Conclusiones: A pesar de la menor edad al momento del diagnóstico, la resolución espontánea fue menor en los varones, y estos presentaron HNP, reflujo grave y anomalías radiológicas más frecuentemente.

Clinical features and disease severity of Turkish FMF children carrying E148Q mutation.

BACKGROUND: Familial Mediterranean fever (FMF) is the most common hereditary monogenic autoinflammatory disease caused by mutations in the MEFV gene. It is controversial whether E148Q alteration is an insignificant variant or a disease-causing mutation. The aim of this study was to evaluate the clinical features and disease severity of FMF patients carrying E148Q mutation. METHODS: Files of FMF patients were retrospectively evaluated. Patients with at least one E148Q mutation were included to the study. The clinical characteristics and disease severity of the patients who were carrying only E148Q mutation were compared with the patients who were compound heterozygous for E148Q and homozygous for M694V mutation. RESULTS: The study group comprised 33 patients who were homozygous or heterozygous for E148Q; 34 with compound heterozygous E148Q mutations and 86 patients who had homozygous M694V mutation. Patients who had only E148Q mutation were found to have the oldest mean age of disease onset and lowest mean disease severity score. Attack frequency and colchicine doses were lower in patients with only E148Q mutation as compared with the other two groups. The frequency of clinical findings such as fever, abdominal pain, arthralgia, and arthritis among the three groups was similar. CONCLUSION: Familial Mediterranean fever patients with only E148Q mutation are presenting with late-onset and milder disease course despite having similar clinical findings as compared with patients who had other mutations. Finally, we imply that E148Q is a mutation and colchicine treatment should be given.

Fatigue in pediatric patients with familial Mediterranean fever.

OBJECTIVES: Familial Mediterranean fever (FMF) is characterized by recurrent, self-limited attacks of fever with serositis involving the peritoneum, pleura and joints. Fatigue is a common problem in many pediatric rheumatic diseases; however, has not been evaluated systematically in FMF patients. Accordingly, the aim of this study was to evaluate fatigue and its possible allied factors in patients with FMF. METHODS: Patients with FMF, aged between 10 and 21 years, were assessed by completed validated fatigue questionnaire (Checklist Individual Strength-20). As a control group, patients with chronic rheumatic diseases and healthy children without any chronic disease were included. RESULTS: The study group comprised 111 patients with FMF, 54 with other chronic rheumatic diseases and 79 healthy subjects. While the CIS-20 total score and subscale scores (including subjective experience of fatigue) were similar between patients with FMF and those with other chronic rheumatic diseases (p > .05); both groups had significantly higher scores when compared with healthy subjects (p < .05). FMF patients with musculoskeletal complaints had significantly higher scores of subjective experiences of fatigue when compared to those without those complaints. CONCLUSIONS: Fatigue is a common but unrecognized complaint in patients with FMF. Familial Mediterranean fever seems to be a chronic disease with inter attack ongoing complaints.

Patient-reported outcome measures in osteoarthritis: a systematic search and review of their use and psychometric properties.

INTRODUCTION: Patient-reported outcome measures (PROM) or self-completed questionnaires have been used to report outcomes in osteoarthritis (OA) for over 35 years. Choices will always need to be made about what should be measured and, if relevant, what would be the most appropriate PROM to use. The current study aims to describe the available PROMs used in OA and their performance quality, so that informed choices can be made about the most appropriate PROM for a particular task. METHODS: The study included a systematic search for PROMs that have been in use over 17 years (period 2000-2016), and to catalogue their psychometric properties, and to present the evidence in a user-friendly fashion. RESULTS: 78 PROMs were identified with psychometric evidence available. The domains of pain, self-care, mobility and work dominated, whereas domains such as cleaning and laundry and leisure, together with psychological and contextual factors, were poorly served. The most frequently used PROMs included the Western Ontario McMaster Osteoarthritis Index, the Short Form 36 and the Knee Disability and Osteoarthritis Outcome Score which, between them, appeared in more than 4000 papers. Most domains had at least one PROM with the highest level of psychometric evidence. CONCLUSION: A broad range of PROMs are available for measuring OA outcomes. Some have good psychometric evidence, others not so. Some important psychological areas such as self-efficacy were poorly served. The study provides a current baseline for what is available, and identifies the shortfall in key domains if the full biopsychosocial model is to be explored.

Late-onset disease is associated with a mild phenotype in children with familial Mediterranean fever.

Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterised by recurrent, self-limited attacks of fever with serositis. Recently, it was shown that patients with early disease onset during childhood period had more severe disease. The aim of this study was to describe the demographic, clinical and genetic features of FMF patients who had late-onset disease during childhood period and to compare them to those with earlier onset patients. Files of patients who had been seen in our department between January 2013 and January 2014 were retrospectively evaluated. Patients were divided into two groups according to age of disease onset (group I, ≤8 years; group II, >8 years), and clinical findings were compared between the two groups. The study group comprised 317 FMF patients. There were 267 patients in group I and 50 patients in Group II. Median attack frequency was 24/year in group I and 12/year in group II (p < 0.05). Fever and M694V homozygosity were less frequently detected in group II (p = 0.003 and p = 0.022). Median delay in diagnosis was 24 months in group I and 12 months in group II (p = 0.002). Disease severity scores and final colchicine dosages were lower in group II (p < 0.001 and p = 0.003). Only a small number of FMF patients had disease onset at older ages in childhood period. It seems that FMF patients with late-onset disease have milder illness. However, more readily expression of their clinical findings in older ages yields earlier diagnosis in this group.

Vesicoureteral Reflux and Renal Scarring Risk in Children after the First Febrile Urinary Tract Infection.

BACKGROUND/AIMS: There are many controversies regarding the best approach for evaluating children after their first febrile urinary tract infection (UTI). The aim of this study was to define the clinical, laboratory, and radiological features of patients with their first febrile UTI and to investigate the factors that might predict the presence of vesicoureteral reflux (VUR) and renal scarring. METHODS: The files of patients who were followed due to their first febrile UTI between 2008 and 2013 were retrospectively reviewed (n = 300). Patients were divided into groups based on their age, the resistance state of microorganisms, the presence of VUR, and scarring on Tc99m dimercaptosuccinic acid scintigraphy. The chi-square test and Mann-Whitney U test were used for analysis. RESULTS: The median age at the first febrile UTI was 11 months and girls constituted 77% of the patient population. VUR and renal scarring were detected in 30.9 and 19.4% of the patients, respectively. C-reactive protein levels and the presence of renal scarring were significantly higher in patients with VUR (p < 0.05). Abnormal ultrasonography findings, VUR and recurrent UTIs were significantly higher in patients with renal scars (p < 0.001). In multivariate analysis, we did not detect any factor that might predict the presence of VUR and renal scarring. CONCLUSION: A majority of children had their first febrile UTI at a young age. Although we could not find any factor that might predict the VUR and scar risk in patients with their first febrile UTI, an abnormal renal scan at 6 months after infection was closely related with the presence of VUR and recurrent UTIs.

Adaptation and validation of the Ankylosing Spondylitis Work Instability Scale (AS-WIS) for use in Turkey.

The Ankylosing Spondylitis Work Instability Scale (AS-WIS) is a recently developed 20-item measure to assess work instability in AS. This study aimed to adapt the AS-WIS to Turkish and to test its reliability and validity. After the translation process, 132 AS patients were assessed by the AS-WIS, Bath AS Disease Activity Index, Bath AS Functional Index and the AS Quality of Life Questionnaire. Reliability was tested by internal consistency, person separation index (PSI) and intra-class correlation coefficient (ICC); internal construct validity by Rasch analysis; external construct validity by associations with comparator scales and cross-cultural validity by comparison with the original UK data. Reliability of the Turkish AS-WIS was good with Cronbach's α and PSI of 0.88 and test-retest ICC of 0.91. Data showed good fit to Rasch model [mean item fit: -0.477 (SD 1.047), Chi-square interaction: 60.9 (df = 40, p = 0.018)]. There was no differential item functioning by age, gender, disease duration or work type. The scale was strictly unidimensional. 51 % of the patients were at moderate risk, and 9 % were at high risk of having to give up their work. External construct validity was confirmed by expected correlations with comparator scales, and a clear gradient of disease activity and functional status across increasing levels of risk. Cross-cultural validity showed some differences in item locations, but this cancelled out at the test level. Turkish version of the AS-WIS is reliable, valid and available for use in routine clinical setting to identify patients who are at risk of having to give up their current job.

Can colchicine response be predicted in familial Mediterranean fever patients?

OBJECTIVES: The aims of this study were to explore whether the demographic and clinical features of paediatric familial Mediterranean fever (FMF) patients with different colchicine response vary or not and to determine whether colchicine response can be predicted in FMF patients. METHODS: Files of patients who have been on colchicine therapy for at least 6 months were retrospectively evaluated. Patients were divided into two groups: group I included patients with no attacks after colchicine and group II comprised patients with ongoing attacks. Thereafter group II was further divided into two groups according to the reduction rate of attack frequency: group IIA (>50%) and group IIB (≤50%). RESULTS: The study group comprised 221 FMF patients (116 females, 105 males). There were 131 patients in group I and 90 patients in group II (54 in group IIA and 36 in group IIB). Leg pain and M694V homozygosity were more frequent in group II (P < 0.05). Final colchicine doses, disease severity scores and number of patients with elevated acute phase reactant levels (attack-free period) were significantly higher and colchicine compliance was lower in group II when compared with group I (P < 0.05). Erysipelas-like erythema (ELE), leg pain and protracted arthritis/protracted febrile myalgia/vasculitis were more frequently detected in group IIB (P < 0.05). CONCLUSION: Colchicine response is excellent in the majority of FMF patients, however, colchicine unresponsiveness cannot be predicted easily at onset. More rarely encountered clinical findings such as ELE, leg pain and protracted complaints and M694V homozygosity may be a clue for less colchicine response.

The prognostic value of quantitative parameters of 18F-FDG PET/CT in the evaluation of response to internal radiation therapy with yttrium-90 in patients with liver metastases of colorectal cancer.

INTRODUCTION: The aim of this study was to evaluate tumor response using fluorine-18-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) in patients who received yttrium-90 selective internal radiation therapy (SIRT) for colorectal liver metastases. The initial and sixth-week tumor lesion glycolysis values were calculated to evaluate the success of the treatment and compare it with patient survival. MATERIALS AND METHODS: Thirty-five patients (15 female, 20 male, mean age: 61.9 ± 9.0 years, range: 33-76 years) who received SIRT treatment for unresectable colorectal cancer liver metastases in our hospital between June 2008 and May 2011 were included in the study. All patients included in the study had liver-only or liver-dominant disease. The treatment response was evaluated by 18F-FDG PET/CT in the sixth week after treatment. Response was evaluated according to the change in total lesion glycolysis (ΔTLG). The ΔTLG was calculated using the following formula: ΔTLG=100 ×[standardized uptake value (SUV) mean1 × total functional tumor volumes (FTVs)1-SUV mean2 × FTV2]/SUV mean1 × FTV1. RESULTS: Mean FTV1 and FTV2 values were calculated to be 235.7 ± 203 and 107.3 ± 67 mm3, respectively (P=0.04). The mean ΔTLG was 43 ± 35 (range: 0-100). Mean overall survival time was 12.7 ± 8.0 months (range: 3-31 months). The cutoff value of ΔTLG was calculated to be 26.5 using receiver operating characteristic analysis (sensitivity 64%; specificity 85%; AUC=0.717 ± 0.087, P=0.034). Patients were allocated into those having values greater than the cutoff value (group 1) and those having values lower than the cutoff value (group 2) in order to calculate the effect of ΔTLG on survival. Survival was 11.32 ± 1.18 (95% CI 9.02-13.62) months in group 2 and 20.76 ± 2.71 (95% CI 15.46-26.06) months in group 1 (P=0.016). ΔTLG was found to be a significant factor in univariate analysis (P=0.01). CONCLUSION: An 18F-FDG PET/CT scan with calculation of ΔSUVmax3, ΔFTV, and ΔTLG before and at the sixth week after SIRT may play an important role in evaluating early tumor response and survival expectancy in these patients and help decide whether these patients should be referred to other treatment modalities or to follow-up.

The reliability and validity of the World Health Organization Disability Assessment Schedule (WHODAS-II) in stroke.

PURPOSE: Psychometric properties of the World Health Organization Disability Assessment Schedule (WHODAS-II) in stroke have received some attention recently, mostly using classical approaches, but there is still an absence of investigation from a modern psychometric perspective. This study aimed to test the reliability and validity of the WHODAS-II in stroke, using modern psychometric analysis. METHODS: A total of 188 community-dwelling poststroke patients were recruited. Internal construct validity was assessed by Rasch analysis, reliability by internal consistency and person separation index (PSI), and external construct validity by associations with Functional Independence Measure (FIM(™)). RESULTS: Rasch analysis indicated that total score (based upon 32 items, omitting the work-related items) was satisfactory, after adjustment for local dependency. The proposed "activities" and "participation" components also satisfied Rasch model expectations. An existing short form was problematic due to inclusion of a work-related item, but an alternative 10-item version was acceptable. Cronbach's α for the WHODAS-II, its domains and components varied between 0.83 and 0.99 and PSI between 0.70 and 0.95. External construct validity was confirmed by expected correlations with FIM(™). CONCLUSIONS: WHODAS-II provides a reliable and valid instrument for measuring disability and components of "activities" and "participation" in stroke survivors. Various combinations of the item set may provide a range of scales to suit most research needs.

Familial Mediterranean fever in siblings.

OBJECTIVE: Genetic and environmental factors have been implicated in disease severity and development of amyloidosis in familial Mediterranean fever (FMF). We investigated similarities in clinical characteristics, disease severity, and treatment response within siblings with FMF. METHODS: The study group consisted of 2 or more siblings who were followed in our center with the diagnosis of FMF. Siblings were evaluated for demographic data, clinical and laboratory disease features, genetic analysis of MEFV mutations, and disease severity score. The intraclass correlation coefficient (ICC), which can be interpreted as the expected correlation between 2 siblings, was used to reflect within-family similarity. RESULTS: The study included 67 pediatric patients from 31 different families. When we investigated the similarity of siblings after adjusting for genetic effects, we found very low ICC with p > 0.05 in the majority of clinical features, disease severity, and colchicine dosages. However, age at disease onset, age at onset of therapy, attack-free acute-phase reactant levels, and presence of amyloidosis were found to be similar within siblings (relatively high ICC with p < 0.05). CONCLUSION: Siblings with FMF had different clinical findings and disease severity. They had similar amyloidogenic potential, proven by both similar presence of amyloid and increased levels of acute-phase reactants between attacks. Our findings strongly support that genetic factors may be more dominant in the development of amyloidosis.

Obesity and postoperative early complications in open heart surgery.

PURPOSE: We investigated the distribution of early clinical outcomes among normal, obese, and morbidly obese patients undergoing open heart surgery. METHODS: Medical records of 1,000 patients undergoing open heart surgery since February 2011 at our hospital were investigated retrospectively after permission was obtained from the Council of Education Planning of the hospital. The comorbidities and perioperative and discharge data were analyzed for 279 patients with a body mass index (BMI) score between 18 and <30 [non-obese reference group (NRG, n = 279)]; 166 patients with BMI between 30 and <35 [obese group (OG, n = 166); and 192 seriously obese patients with BMI ≥35 [extreme obese group (EOG, n = 192)]. Distribution of the patients according to BMI scores was found to represent the BMI distribution of the Turkish population. RESULTS: Pulmonary and infective complications were significantly higher in EOG patients compared to NRG based on crude confidence interval. Based on adjusted multiple logistic regression analysis, by adjusting the effects of age, sex, comorbidities (diabetes mellitus, hypertension, hyperlipidemia, chronic obstructive pulmonary disease), and smoking, the incidence of pulmonary and gastrointestinal complications in EOG was higher compared to NRG. Discharge with morbidity was significantly higher in OG and EOG compared to NRG. CONCLUSIONS: We found that obesity does not increase short-term mortality for open heart surgery; however, it increases the risk of postoperative pulmonary and gastrointestinal complications and discharge with morbidity.

Reliability of basophil activation test using CD203c expression in diagnosis of pollen allergy.

BACKGROUND: CD203c is a basophil surface marker and its expression is rapidly up-regulated after cross-linking of high-affinity immunoglobulin E (IgE) receptor (FcepsilonR1) by an allergen. CD203c basophil activation tests have been studied for the in vitro diagnosis of several allergic conditions. However, there is limited data about its diagnostic usefulness. The optimum allergen concentrations for stimulation and allergen specific cutoff values remain unknown for a number of allergens. This study was designed to investigate the efficacy of basophil activation test via CD203c in the diagnosis of pollen allergy. METHODS: The CD203c basophil activation was determined in 31 allergic rhinitis patients with pollen allergy and 9 healthy nonatopic controls during the off-season. CD203c expression was evaluated using three-color staining protocol by flow cytometry. RESULTS: After an in vitro stimulation with grass pollen extract, the CD203c assay clearly discriminated pollen-allergic patients from controls (p < 0.001). A dose-dependent increase in the percentages of CD203c-activated basophils was shown in rhinitis patients with pollen allergy (p < 0.001). The sensitivity and specificity was 100% and optimal cutoff values were 14.05 and 10.05% with 45.1 and 4.5 µg/mL Phl p 5 stimulation, respectively. Although the specificity was also 100%, the sensitivity was 93 and 87% and the cutoff values were 5.40 and 5.35% with 4.5 × 10(-4) and 4.5 × 10(-5) micrograms/mL Phl p 5 stimulation, respectively. CONCLUSION: The CD203c basophil activation test seems to be a reliable tool in the diagnosis of grass pollen allergy. It could be used when conventional diagnostic tests fail or can not be performed.

Familial Mediterranean fever in small children in Turkey.

OBJECTIVES: Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterised by recurrent, self limited attacks of fever with serositis. The aim of our study was to describe the demographic, clinical and genetic features of FMF patients who had early disease onset and to compare them with late onset patients. Our second aim was to investigate the factors associated with delay in diagnosis. METHODS: The study group consisted of recently diagnosed FMF patients who came to routine follow-up visits between January and July 2009. Patients were divided into two groups according to age of disease onset (Group I: ≤ 3 years of age; Group II: >3 years of age). In the second part, patients were analysed according to the duration of delay in diagnosis. RESULTS: There were 83 patients in group I and 73 patients in Group II. Median delay in diagnosis was 4 years in Group I and 2 years in Group II (p<0.001). The presence of M694V mutation was more frequent in Group I (81%) as compared to Group II (65%), (p=0.034). Mean attack Hb was lower (p<0.01) and mean attack leukocyte count was higher (p=0.017) in Group I. Final colchicine dosages were higher in Group I as compared to Group II. There was a statistically significant negative correlation between the age at disease onset and period of delay in diagnosis (p<0.001). CONCLUSIONS: This study suggests that FMF patients with early disease onset have more severe disease. Moreover, the smaller the age of disease onset, the more likely their diagnoses are delayed.