Rhupus syndrome in children: A multi-center retrospective cohort study and literature review.

OBJECTIVE: In this study, we aimed to evaluate the characteristics of pediatric rhupus patients including all the related series in the literature. METHODS: Thirty pediatric patients with rhupus syndrome from 12 different centers in Turkey were included in this study. The literature was also reviewed for pediatric patients with rhupus syndrome. RESULTS: The most prominent phenotype of these 30 patients was juvenile idiopathic arthritis (JIA) (60%) at the disease onset and SLE (73.3%) at the last visit. Major SLE-related organ involvements were skin (80%), hematological system (53.3%), and kidney (23.3%). Arthritis was polyarticular (73.3%), asymmetric (66.7%), and erosive (53.3%) in most patients. Hydroxychloroquine (100%), glucocorticoids (86.7%), and mycophenolate mofetil (46.7%) were mostly used for SLE, while glucocorticoids (76.6%), methotrexate (73.3%), and nonsteroidal anti-inflammatory drugs (NSAIDs) (57.6%) were mainly preferred for JIA. Our literature search revealed 20 pediatric patients with rhupus syndrome (75% were RF positive). The most prominent phenotype was JIA (91.7%) at the disease onset and SLE (63.6%) at the last visit. Major SLE-related organ involvements were skin (66.7%), hematological system (58.3%), and kidney (58.3%). Arthritis was polyarticular (77.8%), asymmetric (63.6%), and erosive (83.3%) in most patients. Glucocorticoid (100%), hydroxychloroquine (76.9%), and azathioprine (46.2%) were mostly used for SLE, while methotrexate (76.9%) and NSAIDs (46.2%) were mainly preferred for the JIA phenotype. CONCLUSION: Our study is the largest cohort in the literature evaluating pediatric rhupus cases. Most of the pediatric patients had polyarticular, asymmetric, and erosive arthritis, as well as organ involvements associated with SLE, including the skin, hematological system, and kidney.

Evaluation of quality of life and its associations with clinical parameters in pediatric patients with familial Mediterranean fever.

OBJECTIVE: A favorable quality of life (QoL) is important in children with chronic disease, and it reflects successful disease management. The aim of our study was to evaluate QoL and its association with clinical parameters in pediatric patients with familial Mediterranean fever (FMF). METHODS: The Kinder Lebensqualität Fragebogen (KINDL®) questionnaires (kiddy: 4-7 years; kid: 8-16 years) for children and the proxy version for parents were implemented as a QoL measure. A total of 171 FMF patients, 69 healthy peers and their parents were enrolled in the study. RESULTS: The KINDL QoL scores of the FMF patients were significantly lower than their healthy peers. The physical and emotional well-being KINDL QoL scores of the FMF children were significantly lower than their healthy peers (p=0.017 and p=0.020, respectively). In the evaluation of the KINDL QoL scores between the kiddy and kid groups, only the self-esteem score was higher in the kiddy group (p=0.004), and the school functioning scores were higher in the kid group (p=0.002). The scores in the physical well-being and disease module had significant differences between patients who were adherent and those who were non-adherent to colchicine therapy (p=0.042 and p=0.047, respectively). The scores in the physical well-being and disease module were significantly higher in patients with fewer attacks than those who had many attacks per year (p=0.004 and p=0.014, respectively). CONCLUSION: This study suggests that FMF patients have significantly impaired QoL. The irregular use of colchicine and more frequent attacks affect QoL even more. A QoL assessment with multidisciplinary follow-up and control of the disease activity are essential, and if necessary, individualized support should be given to patients.

Effects of Hemodiafiltration versus Conventional Hemodialysis in Children with ESKD: The HDF, Heart and Height Study.

BACKGROUND: Hypertension and cardiovascular disease are common in children undergoing dialysis. Studies suggest that hemodiafiltration (HDF) may reduce cardiovascular mortality in adults, but data for children are scarce. METHODS: The HDF, Heart and Height study is a nonrandomized observational study comparing outcomes on conventional hemodialysis (HD) versus postdilution online HDF in children. Primary outcome measures were annualized changes in carotid intima-media thickness (cIMT) SD score and height SD score. RESULTS: We enrolled 190 children from 28 centers; 78 on HD and 55 on HDF completed 1-year follow-up. The groups were comparable for age, dialysis vintage, access type, dialysis frequency, blood flow, and residual renal function. At 1 year, cIMT SD score increased significantly in children on HD but remained static in the HDF cohort. On propensity score analysis, HD was associated with a +0.47 higher annualized cIMT SD score compared with HDF. Height SD score increased in HDF but remained static in HD. Mean arterial pressure SD score increased with HD only. Factors associated with higher cIMT and mean arterial pressure SD-scores were HD group, higher ultrafiltration rate, and higher ß2-microglobulin. The HDF cohort had lower ß2-microglobulin, parathyroid hormone, and high-sensitivity C-reactive protein at 1 year; fewer headaches, dizziness, or cramps; and shorter postdialysis recovery time. CONCLUSIONS: HDF is associated with a lack of progression in vascular measures versus progression with HD, as well as an increase in height not seen in the HD cohort. Patient-related outcomes improved among children on HDF correlating with improved BP control and clearances. Confirmation through randomized trials is required.

Evaluation of growth in children and adolescents after renal transplantation.

Büyükkaragöz B, Bakkaloglu SA, Tuncel AF, Kadioglu-Yilmaz B, Karcaaltincaba D, Pasaoglu H. Evaluation of growth in children and adolescents after renal transplantation. Turk J Pediatr 2019; 61: 217-227. Despite the advances in the last decades, it is well-known that optimal growth is usually not achieved in children with chronic kidney disease (CKD) even after successful renal transplantation (RTx). In this study, our aim was to evaluate growth patterns and factors affecting growth in pediatric and adolescent renal transplant recipients (RTR). Thirty-seven prevalent RTR with mean age of 17.0±2.9 years and mean post-RTx duration of 4.2±2.0 years were evaluated. Growth parameters, height velocities and factors affecting growth at the time of RTx (baseline) and in the post-RTx follow-up were also retrospectively assessed. Cumulative corticosteroid (CS) doses were calculated. Mean height and weight standard deviation score (SDS) values were negative (-1.4±1.1 and -1.2±1.5, respectively), whereas height SDS was positive in 16% of the patients. Mean weight, height, and BMI (body mass index) SDS of the RTR were significantly higher than the values at transplantation (p < 0.001 for weight and height SDS; p < 0.05 for BMI SDS). Height SDS was < -2.0 in 19% of the patients while 60% at the baseline. Main factors associated with post-RTx height SDS were pre-RTx height SDS (B: 0.448, p < 0.01) and CKD duration (B: -0.01, p < 0.05). Although it was much better than the pre-RTx period, the present study reveals that post- RTx growth was less than anticipated. As well as minimizing post-RTx CS doses and preserving graft function in the post-RTx follow-up, performing early transplantation and all efforts for minimizing pre-RTx growth deficit are crucial for an optimal post-RTx growth.

The relationship between urinary tract infections and vesicoureteral reflux in Turkish children.

Early determination of renal scar development risk in children following first urinary tract infection (UTI) and early detection and treatment of vesicoureteral reflux (VUR) are important to prevent renal functional impairment. The aim of this study was to determine the prevalence of VUR and associated renal scar formation, in children who had acute pyelonephritis (APN), first afebrile UTI, and recurrent afebrile UTIs. Patient records of 642 children having UTI were scrutinized and 278 out of 642 were enrolled in this study. The patients were divided into three groups: Group 1, patients with APN (n = 73); Group 2, patients with the first afebrile UTI (n = 88); and group 3, patients with recurrent afebrile UTIs (n = 117). Among these groups, VUR prevalence did not differ significantly (group 1: 24.6%, group 2: 22.7%, and group 3: 26.4%. Renal scarring was detected in 19.3% of the patients and was remarkably lower in group 2 when compared with the other two groups (P < 0.05). Renal scarring was found in 37.1% of the patients with VUR, whereas it was found in 14.3% of patients without VUR. Renal scarring incidence was remarkably higher in patients with grades 4-5 VUR (61.5%) compared with those with grades 1-3 VUR (30.6%) (P = 0.055). In conclusion, since VUR was demonstrated in as many as one-quarter of patients with the first afebrile UTI and VUR incidence did not differ significantly among the groups, all UTIs, lower and upper ones, should be carefully evaluated.

Henoch Schonlein purpura in childhood: clinical analysis of 254 cases over a 3-year period.

We aimed to evaluate the patients who were diagnosed as Henoch Schonlein purpura (HSP) for disease characteristics and prognosis of those with joint, gastrointestinal (GI), and renal involvement. Two hundred and fifty-four children who were followed up with the diagnosis of HSP in the Pediatric Nephrology Clinics of Meram Medical Faculty of Selcuk University and Medical Faculty of Gazi University between January 2003 and June 2006 were retrospectively evaluated. The clinical follow-up and treatment regimens of patients in whom renal biopsy was performed were evaluated in detail. The study group consisted of 254 children, 147 boys (57.8%) and 107 girls (42.2%), and the ratio of boys to girls was 1.37. The percentages of skin, joint, GI, and renal manifestations were 100%, 66%, 56%, and 30%, respectively. Eight patients had intussusception. Five of them recovered with steroid treatment only while three patients were operated on. Sixty-four patients (44%) with GI involvement had severe disease and were successfully treated with steroids. Renal biopsy was performed in 26 patients. Among those 26 patients, two of them recovered spontaneously within 3 and 4 weeks. Ten patients improved with only steroid treatment while 12 patients recovered with steroid and cyclophosphamide treatment. Two patients were resistant to steroid and cyclophosphamide treatment and were treated with cyclosporine A. We believe that steroid therapy given to the HSP patients with GI manifestations might be helpful to prevent probable complications such as GI bleeding and intussusception. In addition, combined therapy with steroid and cyclophosphamide can usually be an appropriate treatment for patients with nephrotic proteinuria.

HLA class 1 associations in Henoch Schonlein purpura: increased and decreased frequencies.

Henoch Schonlein purpura (HSP) is the most common vasculitis of childhood. Susceptibility to HSP and associated clinical heterogeneity in HSP may be conferred by a number of genetic loci, including the major histocompatibility complex. We aimed to investigate the implications of the human leukocyte antigen (HLA) class 1 alleles in susceptibility to HSP and determine the possible associations with renal, gastrointestinal (GI), and joint manifestations of the disease. 110 children with HSP (66 boys, 44 girls) and 250 unrelated healthy controls were enrolled in the study. The mean age was 8.65 +/- 3.59 years. HSP was diagnosed on the basis of clinical and laboratory data according to the American College of Rheumatology classification. The diagnosis was supported with skin and/or kidney in most of the patients. Clinical and laboratory findings revealed: skin involvement in 110 (100%), joint manifestations in 82 (74.5%), GI symptoms in 58 (52.7%), and hematuria and/or proteinuria in 36 (32.7%) patients. HLA class 1 alleles were identified by DNA amplification, hybridized with specific primer sequences. Comparison of frequencies between patients and controls were made by using the Fisher's exact test. Odds ratio (OR) was used as the measure of association. HLA A2, A11, and B35 antigens showed an increased risk for predisposition to HSP (OR = 1.714, 95%CI = 1.088-2.700, p = 0.020; OR = 2.185, 95%CI = 1.289-3.703, p = 0.003; and OR = 2.292, 95%CI = 1.451-3.619, p = 0.000, respectively), while HLA A1, B49, and B50 antigens revealed decreased risk for predisposition to HSP (OR = 4.739, 95%CI = 1.828-12.345, p = 0.001; OR = 3.268, 95%CI = 0.955-11.236, p = 0.047; and OR = 7.462, 95%CI = 0.975-55.555, p = 0.024, respectively). Considering the renal involvement and severity of proteinuria, there was no association with HLA class 1 alleles. Our results suggest that the increased frequency of HLA A2, A11, and B35 alleles in unselected pediatric HSP patient population and miscarrying of HLA A1, B49, and B50 could be considered as a risk factor for susceptibility to HSP.