Immunosuppressive Therapy of Antibody-Mediated aHUS and TTP.

The recent classification of pediatric thrombotic microangiopathies (TMA) takes into consideration mechanisms of disease for guidance to targeted therapies. We present our experience with seven patients with antibody mediated atypical hemolytic uremic syndrome (aHUS) and thrombotic thrombocytopenic purpura (TTP). Five children had aHUS with antibodies against complement factor H (CFH-ab) and two with TTP with antibodies against metalloproteinase ADAMTS13.

Glucose Derivative Induced Vasculopathy in Children on Chronic Peritoneal Dialysis.

[Figure: see text].

[De novo inflammatory bowel disease in children after solid organ transplantation].

Összefoglaló. A gyulladásos bélbetegség (inflammatory bowel disease, IBD) incidenciája folyamatosan nő, etiológiája egyelőre ismeretlen. Kezelésében gyakran alkalmazunk immunszuppresszív, illetve immunmoduláns szereket.

Peritoneal Dialysis Vintage and Glucose Exposure but Not Peritonitis Episodes Drive Peritoneal Membrane Transformation During the First Years of PD.

The impact of peritoneal dialysis (PD) associated peritonitis on peritoneal membrane integrity is incompletely understood. Children are particularly suited to address this question, since they are largely devoid of preexisting tissue damage and life-style related alterations. Within the International Peritoneal Biobank, 85 standardized parietal peritoneal tissue samples were obtained from 82 children on neutral pH PD fluids with low glucose degradation product (GDP) content.

Neutral pH and low-glucose degradation product dialysis fluids induce major early alterations of the peritoneal membrane in children on peritoneal dialysis.

The effect of peritoneal dialysates with low-glucose degradation products on peritoneal membrane morphology is largely unknown, with functional relevancy predominantly derived from experimental studies. To investigate this, we performed automated quantitative histomorphometry and molecular analyses on 256 standardized peritoneal and 172 omental specimens from 56 children with normal renal function, 90 children with end-stage kidney disease at time of catheter insertion, and 82 children undergoing peritoneal dialysis using dialysates with low-glucose degradation products. Follow-up biopsies were obtained from 24 children after a median peritoneal dialysis of 13 months.

Reduced Microvascular Density in Omental Biopsies of Children with Chronic Kidney Disease.

Background: Endothelial dysfunction is an early manifestation of cardiovascular disease (CVD) and consistently observed in patients with chronic kidney disease (CKD). We hypothesized that CKD is associated with systemic damage to the microcirculation, preceding macrovascular pathology. To assess the degree of "uremic microangiopathy", we have measured microvascular density in biopsies of the omentum of children with CKD.

Comparison of Interscalene Brachial Plexus Block Performed With and Without Steroids.

This prospective comparative clinical study was performed to evaluate the effect of triamcinolone when added to bupivacaine during brachial plexus blockade in patients undergoing shoulder surgery. Interscalene brachial plexus blocks were performed on 910 patients before shoulder surgery. Of the patients, 574 were randomly allocated to receive steroids added to the injected local anesthetic and 336 patients received local anesthetic without steroids.

Quantitative Histomorphometry of the Healthy Peritoneum.

The peritoneum plays an essential role in preventing abdominal frictions and adhesions and can be utilized as a dialysis membrane. Its physiological ultrastructure, however, has not yet been studied systematically. 106 standardized peritoneal and 69 omental specimens were obtained from 107 patients (0.

Normal 25-Hydroxyvitamin D Levels Are Associated with Less Proteinuria and Attenuate Renal Failure Progression in Children with CKD.

Angiotensin-converting enzyme inhibitors (ACEi) for renin-angiotensin-aldosterone system (RAAS) blockade are routinely used to slow CKD progression. However, vitamin D may also promote renoprotection by suppressing renin transcription through cross-talk between RAAS and vitamin D-fibroblast growth factor-23 (FGF-23)-Klotho pathways. To determine whether vitamin D levels influence proteinuria and CKD progression in children, we performed a post hoc analysis of the Effect of Strict Blood Pressure Control and ACE Inhibition on Progression of CKD in Pediatric Patients (ESCAPE) cohort.

Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the PodoNet registry cohort.

Background And Objectives: Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or genetic alterations of podocyte structure and function. The rare nature, heterogeneity, and slow evolution of the disorder are major obstacles to systematic genotype-phenotype, intervention, and outcome studies, hampering the development of evidence-based diagnostic and therapeutic concepts. To overcome these limitations, the PodoNet Consortium has created an international registry for congenital nephrotic syndrome and childhood-onset steroid-resistant nephrotic syndrome.

Pathogens causing urinary tract infections in infants: a European overview by the ESCAPE study group.

Unlabelled: Knowledge of the distribution spectrum of causative organisms and their resistance patterns has become a core requirement for the rational and effective management of urinary tract infections. In the context of a prospective trial on the use of antibiotic prophylaxis in infants with underling kidney malformations, we conducted an online survey among paediatric nephrologists on positive urine cultures (July 2010-June 2012) from both hospitalized and non-hospitalized infants under 24 months of age. We collected 4745 urine cultures (UCs) at 18 units in 10 European countries.

Continuous glucose monitoring system (CGMS) in kidney-transplanted children.

NODAT and IGT are well-known complications of immunosuppressive therapy after transplantation being a risk factor for cardiovascular disease affecting patient and graft survival. Therefore, early identification and treatment are of high importance. In this study, we examined the glycemic homeostasis of 20 renal-transplanted children using routine laboratory tests and the continuous glucose monitoring system (CGMS).

Ambulatory arterial stiffness index in children after kidney transplantation.

Given the increase in CV morbidity after RTx and the scarcity of CV events in pediatrics, surrogate markers should be assessed to characterize CV damage in this population. AASI is a marker of arterial stiffness in adults, predicting cardio- and cerebrovascular morbidity. Our aim was to assess the determinants of AASI in RTx children (n = 54, 15.

NPHS2 p.V290M mutation in late-onset steroid-resistant nephrotic syndrome.

Background: The most frequently mutated gene of steroid-resistant nephrotic syndrome (SRNS) is NPHS2. Current guidelines propose the sequencing of all NPHS2 exons only in childhood-onset SRNS.

Methods: A cohort of 38 Hungarian patients with childhood-onset nephrotic-range proteinuria was screened for NPHS2 mutations.

Cardiac magnetic resonance imaging in children with chronic kidney disease and renal transplantation.

CV disease is the major cause of death in patients with CKD. Recently, CMR imaging emerges as a complementary method providing advantages in cardiac assessment; however, data on CMR in pediatric CKD are scarce. We performed CMR in 15 children: two with CKD, six on peritoneal dialysis, seven on hemodialysis, and in 18 children 5.

H1N1 vaccination in pediatric renal transplant patients.

Background: Solid organ transplant recipients undergoing immunosuppressive therapy are considered to be at high risk of serious infectious complications. In 2009, a new influenza pandemic caused serious infections and deaths, especially among children and immunocompromised patients. Herein we have reported the safety and efficacy of a single-shot monovalent whole-virus vaccine against H1N1 infection in the pediatric renal transplant population.

A prospective analysis of interscalene brachial plexus blocks performed under general anesthesia.

Background: The purpose of this prospective study was to assess the safety and efficacy of interscalene brachial plexus block anesthesia when performed on patients who were anesthetized with a general anesthetic prior to the performance of the block.

Methods: Patients were assessed postoperatively through surveys, interviews, and physical examinations to document block success, duration of anesthesia, block side effects, and persistent neurological complications. Nine-hundred fifty-one patients were available for the analysis.

Heat shock protein polymorphism predisposes to urinary tract malformations and renal transplantation in children.

Background: Anatomical malformations of the kidney and urinary tract account for 17% of pediatric renal transplantation procedures. Heat shock proteins (HSPs) are molecular chaperones with a protective function that promotes cell survival. HSP72 is an endogenous ligand for toll-like receptor TLR4, thereby stimulating innate immunity.

Strict blood-pressure control and progression of renal failure in children.

Background: Although inhibition of the renin-angiotensin system delays the progression of renal failure in adults with chronic kidney disease, the blood-pressure target for optimal renal protection is controversial. We assessed the long-term renoprotective effect of intensified blood-pressure control among children who were receiving a fixed high dose of an angiotensin-converting-enzyme (ACE) inhibitor.

Methods: After a 6-month run-in period, 385 children, 3 to 18 years of age, with chronic kidney disease (glomerular filtration rate of 15 to 80 ml per minute per 1.

Subjective and functional outcomes following surgical repair of complete ruptures of the proximal hamstring complex.

Complete ruptures of the proximal hamstring tendon insertion are less common than typical muscle tendon junction strain injuries, with a far more debilitating natural history. The present study was designed to assess the subjective and functional outcomes of a group of patients following surgical repair of both acute and chronic complete proximal hamstring tendon ruptures. Twenty-five patients with an average age of 44 years comprised the study cohort.

Post-transplant diabetes mellitus in children following renal transplantation.

PTDM plays a role in chronic allograft nephropathy and decreases graft and patient survival. Considering the serious outcome of chronic hyperglycemia, the importance of early recognition and the few data in children, in this retrospective analysis we studied the characteristics and risk factors of PTDM in 45 pediatric renal transplant recipients receiving Tac or CyA-based immunosuppression. Fasting blood sampling and OGTT were performed.

Frequency of various tear patterns in full-thickness tears of the rotator cuff.

Purpose: We define specific rotator cuff tear patterns, prospectively document their occurrences, and emphasize the importance of tear pattern recognition during arthroscopy.

Methods: We prospectively analyzed 193 full-thickness rotator cuff tears intraoperatively from a single surgeon's practice. We address specific tear patterns, tear size, tissue mobility, tissue quality, and chronicity.

Urinary excretion of endothelin-1 (ET-1), transforming growth factor- beta1 (TGF- beta1) and vascular endothelial growth factor (VEGF165) in paediatric chronic kidney diseases: results of the ESCAPE trial.

Unlabelled: The severity and dynamics of renal tissue damage in chronic kidney disease (CKD) may be reflected by the urinary excretion of vasoactive and growth factors released by the damaged kidney. Urinary excretion of ET-1, TGF-beta1 and VEGF(165) was evaluated in 303 children with CKD stage II-IV (GFR 48 +/- 22 ml/min/1.73 m(2)) and 81 age-matched healthy controls.

A longitudinal study of patients with multidirectional instability of the shoulder with seven- to ten-year follow-up.

The purpose of our study was to evaluate the long-term outcomes of patients with multidirectional instability of the shoulder initially treated with rehabilitation exercises. Sixty-four patients were treated for atraumatic multidirectional instability of the shoulder between 1987 and 1990. Preliminary evaluation was performed 2 years after initiation of treatment, and final evaluation of the patients was performed at a mean of 8 years after initiation of treatment.