Evaluation of the Performance of Vasoactive Ventilation Renal Score in Predicting the Duration of Mechanical Ventilation and Intensive Care Hospitalization After Pediatric Cardiac Surgery.

Various methods have been used to evaluate the predictivity of some markers during the recovery process after cardiac surgery in children. The aim herein was to evaluate the vasoactive ventilation renal (VVR) score in predicting the clinical outcomes of children who underwent congenital cardiac surgery within the early period in the pediatric intensive care unit (PICU). Patients who underwent congenital heart surgery (CHS) between November 2016 and March 2020 were enrolled and evaluated prospectively. The VVR score was calculated as follows: vasoactive inotrope score (VIS) + ventilation index + (change in serum creatinine level based on the baseline value × 10). The relationship between the duration of mechanical ventilation and the length of stay (LOS) in the PICU was evaluated via receiver operating characteristic (ROC) curve analysis and the cut-off values were calculated. At all of the time points identified in the study, the VVR score had a higher area under the ROC curve (AUC) when compared to the VIS and serum lactate levels, and the 48-h VVR score had the highest AUC (AUC 0.851, 95% confidence interval (CI) 0.761-0.942/LOS in the PICU; AUC 0.946, 95% CI 0.885-1.000/duration of mechanical ventilation). The 48-h VVR score for the LOS in the PICU was 6.7 (sensitivity 70%, specificity 92%) and that for the duration of mechanical ventilation was 9.1 (sensitivity 87%, specificity 97%). As a result, in our study, it was found that the VVR score is a new and effective predictor of the duration of mechanical ventilation and LOS in the PICU in postoperative CHS patients.

Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry.

BACKGROUND: As outcome data for prune belly syndrome (PBS) complicated by end-stage renal disease are scarce, we analyzed characteristics and outcomes of children with PBS using the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) Registry data. METHODS: Data were available for 88 male PBS patients aged <20 years who started renal replacement therapy (RRT) between 1990 and 2013 in 35 European countries. Patient characteristics, survival, and transplantation outcomes were compared with those of male patients requiring RRT due to congenital obstructive uropathy (COU) and renal hypoplasia or dysplasia (RHD). RESULTS: Median age at onset of RRT in PBS was lower [7.0; interquartile range (IQR) 0.9-12.2 years] than in COU (9.6; IQR: 3.0-14.1 years) and RHD (9.4; IQR: 2.7-14.2 years). Unadjusted 10-year patient survival was 85% for PBS, 94% for COU, and 91% for RHD. After adjustment for country, period, and age, PBS mortality was similar to that of RHD but higher compared with COU [hazard ratio (HR) 1.96, 95% confidence interval (CI) 1.03-3.74]. Seventy-four PBS patients (84%) received a first kidney transplant after a median time on dialysis of 8.4 (IQR 0.0-21.1) months. Outcomes with respect to time on dialysis before transplantation, chance of receiving a first transplant within 2 years after commencing RRT, and death-censored, adjusted risk of graft loss were similar for all groups. CONCLUSIONS: This study in the largest cohort of male patients with PBS receiving RRT to date demonstrates that outcomes are comparable with other congenital anomalies of the kidney and urinary tract, except for a slightly higher mortality risk compared with patients with COU.

Prognostic Importance of Bone Marrow Uptake on Baseline 18F-FDG Positron Emission Tomography in Diffuse Large B Cell Lymphoma.

AIM: To define the role of 18F-flourodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) in the detection of bone marrow (BM) involvement in patients with diffuse large B cell lymphoma (DLBCL). MATERIALS AND METHODS: Fifty-four (mean age: 55.5 ± 18.3 years, 20 female and 34 male) DLBCL patients who underwent pretreatment 18F-FDG PET/CT were included to the study. Focal or diffuse BM 18F-FDG uptake that is higher than mediastinal blood pool uptake was accepted as positive. After staging of disease by CT and 18F-FDG PET/CT, all the patients received R-CHOP treatment after diagnostic blinded bone marrow biopsy (BMB). Presence of positive BM uptake in 18F-FDG PET/CT and histopathological examination results of BMBs were analyzed by Chi-square test. Sensitivity, specificity, accuracy, positive predictive value (PPV), and negative predictive value (NPV) of 18F-FDG PET/CT in the detection of BM involvement were calculated. Prognostic importance of the presence of BM 18F-FDG uptake was analyzed by Kaplan-Meier analysis. RESULTS: BM 18F-FDG uptake was detected in 8 patients. Histopathological examination of BMB revealed BM involvement in 6 out of 8 patients. BMB was negative in all patients who have no 18F-FDG uptake in the evaluation of PET/CT images. Sensitivity, specificity, accuracy, PPV, and NPV of 18F-FDG PET/CT in the detection of BM involvement were calculated as 100%, 96%, 96%, 75%, and 100%, respectively. In the Kaplan-Meier analysis, we found that presence of pretreatment 18F-FDG uptake in BM has a prognostic importance. Whereas mean time to progression (TTP) in patients with BM uptake was 32.25 ± 10.9 months and mean TTP in those without was 51.69 ± 3.6 months (p = 0.013). CONCLUSIONS: BM uptake in pretreatment 18F-FDG PET/CT is an important prognostic factor in DLBCL patients. Moreover, in consideration of high NPV, 18F-FDG PET/CT could eliminate unnecessary BMB in FDG-negative patients.

Foetal biometry in polyhydramnios: Does femur length fall behind?

We aimed to identify the growth patterns in polyhydramnios, and therefore evaluated 108 singleton pregnancies complicated with polyhydramnios according to the changes in biparietal diameter (BPD), abdominal circumference (AC) and femur length (FL) percentiles. The pregnancy outcomes according to the growth features were analysed. In the study population, BPD and AC percentiles exhibited a significant increase (p = 0.023 and 0.05, respectively), although FL percentiles showed a significant decrease (p = 0.006) according to the changes in third trimester relative to second trimester. In the overgrown group (n = 52), the FL/BPD ratio was lower (p < 0.001), with more foetuses with FL/BPD ratios below 71 (p = 0.05). In conclusion, there was a significant increase in BPD and AC percentiles and a decrease in FL percentiles in third trimester relative to second trimester in foetuses with polyhydramnios. However, we observed a shorter FL and a lower FL/BPD ratio without associated skeletal dysplasia in overgrown foetuses.

Repeatability and reproducibility of a new optical biometer in normal and keratoconic eyes.

PURPOSE: To evaluate the repeatability and reproducibility of measurements obtained by a new optical biometer (Nidek AL-Scan) in normal eyes and keratoconic eyes. SETTING: Department of Ophthalmology, Turgut Özal University, Medical School, Ankara, Turkey. DESIGN: Evaluation of diagnostic technology. METHODS: To assess the repeatability and reproducibility of the optical biometer, 2 independent examiners performed 3 consecutive measurements. The following parameters were evaluated: central corneal thickness (CCT), anterior chamber depth, axial length (AL), corneal dioptric power in the flattest meridian (flat keratometry [K]) and in the steepest meridian (steep K), and white-to-white (WTW) distance. Repeatability was assessed using the within-subject standard deviation (S(w)), repeatability, and precision. Reproducibility was evaluated using the 95% limits of agreement proposed by Bland and Altman. RESULTS: Thirty healthy subjects and 27 patients with keratoconus were evaluated. Both examiners achieved high repeatability for all parameters in each group except for the steep K measurement in keratoconic eyes performed by examiner 2 (S(w) = 3.341). The WTW in normal eyes and the CCT and steep K in keratoconic eyes showed less repeatability. In both groups, the smallest range of agreement was in AL measurements whereas the largest was in CCT measurements. In keratoconic eyes, the range of agreement for steep K was also greater (3.766). CONCLUSIONS: The precision of the measurements obtained by the new optical biometer was high. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

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.