Einleitung: Das Pulmonalarterien Banding wird in der Literatur als innovative Strategie zur Unterstützung des linken Ventrikels bei DCMP als Brigde to Transplant oder Recovery vorgeschlagen. Wir berichten über die Anwendung dieses Verfahrens ergänzt durch Erhaltung fetaler Shuntverbindungen und medikamentöser Therapie bei einem Kind mit schwerster bereits intrauteriner Herzinsuffizienz.

Objectives: We report on a 15-year-old patient with failing single ventricle palliation admitted for cardiac transplantation for failing univentricular palliation after tricuspid atresia Ib. So far, a modified Blalock-Taussig shunt and a Glenn anastomosis were performed. Invasive evaluation revealed elevated pulmonary pressure, small pulmonary arteries and extensive pulmonaryarteriovenous fistulas (PAVM) formation in the left lung. Overall, the patient was considered non-transplantable. An alternative treatment strategy with implantation of a Heartware® ventricular assist device together with placement of a systemic shunt to the left lung to promote (1) regression of PAVM by re-establishing hepatic flow to the lung and (2) growth of the pulmonary vasculature, was developed.

Background Unfractionated heparin (UFH) is used for the prophylaxis and treatment of thrombosis in children. Laboratory monitoring of UFH is needed to prevent over-anticoagulation or under-anticoagulation.Objectives To investigate the association between UFH dose and UFH effect as monitored with the anti-activated factor II (FIIa) assay, the relationship between anti-FIIa and anti-activated factor X (FXa) effects, and the influence of patient age and other factors on UFH effect.Patients and methods This was a randomized controlled trial in children during cardiac catheterization, comparing high-dose UFH (100 units kg(-1) bolus) with low-dose UFH (50 units kg(-1) bolus). Blood samples were drawn at baseline, and after 30 min, 60 min, and 90 min. For the purpose of this study, 49 children and 117 blood samples were evaluated.Results The anti-FIIa assay discriminated well between high-dose and low-dose UFH. Multiple regression demonstrated significant influences of UFH dose and age on anti-FIIa levels. Younger children had lower anti-FIIa levels than older children, an effect that was more pronounced with low-dose UFH. Anti-FXa/anti-FIIa ratios were equal with low-dose UFH. However, anti-FXa levels were relatively increased over anti-FIIa levels in infants and after high-dose UFH bolus administration.Conclusion The UFH effect on anti-FIIa levels is lower in infants than in older children. This influence of age appears to be dose-dependent, being more pronounced with low-dose UFH. Anti-FXa and anti-FIIa levels are not equal, particularly in infants and after high-dose UFH. Monitoring UFH solely with anti-FXa assays may not be sufficient in children, and the anti-FIIa assay may provide important complementary information.

Children who participate in competitions or regularly participate in team training should undergo an assessment for capability to partake in sports for early recognition of orthopedic or internistic problems. The assessment of capability to participate in sports should consist of controlling the long-term and family medical history, a comprehensive internistic and orthopedic examination and a 12-lead electrocardiogram (ECG). After the introduction of an obligatory standardized sports capability examination in Italy, including a 12-lead ECG, the incidence of sudden death could be reduced by 89 %. However, in most countries and for most forms of sport a preparticipation physical examination before training and competitions is neither obligatory nor standardized. The Austrian Society for Pediatrics and Adolescent Medicine (A-GKJ) and the Austrian Society for Sports Medicine and Prevention (A-GSMP) have therefore published joint recommendations on the performance of a sports preparticipation physical examination. This should be carried out by an experienced physician (e.g. sports physician, specialist for pediatrics and adolescent medicine or pediatric cardiologist) on starting team training in connection with a competition or before participation in a sports competition and should be repeated at least every 2 years.

Einleitung: Der Cardiac Index (CI) ist ein wichtiger Verlaufsparameter bei Patienten nach Fontan-Operation. Die nicht-invasive Messung des CI bei Fontan-Patienten wurde bislang nur unzureichend untersucht.

Objective: This study reports on the feasibility, efficacy, and outcome of hybrid procedures to close ventricular septal defects (VSD), reflecting the experience of 11 centers in Germany, Austria, and Switzerland. Background: Beating heart closure of VSD has attracted interest in small infants, complex anomalies and postinfarction scenarios where patients are at high risk during surgery. Perventricular or intraoperative device placement allows access to the lesions where percutaneous delivery is limited. Methods: Between December 2001 and April 2009, placement of Amplatzer septal occluders was attempted in 26 patients. The defects were located in the perimembranous (n 5 5) and muscular septum (n 5 21). In 20 patients, a perventricular approach was used, and, in six, the occluders were placed under direct visualization being part of a complex heart surgery. Results: In 23 of 26 procedures, device placement was successful (88.5%). The mean defect size was 7.8 mm (range, 3.5–20). The occluder types were perimembranous VSD occluder (n 5 4), muscular VSD occluder (n 5 20), postinfarct VSD occluder (n 5 1), and ASD occluder (n 5 1) with a ratio device/defect of 0.9–2.4 (median 1.15). Device removal was necessary in three due to arrhythmia, malpositioning, and additional defects. Pericardial effusion occurred once. In the remaining 22 patients, there were no procedure or device-related complications. During mean follow up of 1.4 years (range, 1 day–3.9 years), a residual shunt that was more than trivial was observed in one patient out of 21 successful procedures. Conclusions: Perventricular and intraoperative device closure of VSD is as effective as a surgical patch and averts the increased morbidity of conventional surgical repair in a subgroup of highrisk patients. VC 2010 Wiley-Liss, Inc.

OBJECTIVE:Inhaled nitric oxide (NO) is used as a therapy of pulmonary hypertension in children after cardiac surgery. Hemoglobin binds to NO with great affinity and forms methemoglobin by oxidation in the erythrocyte. Once produced, methemoglobin is unable to transport and unload oxygen in the tissues. The amount of available hemoglobin in the body for oxygen transport is thereby reduced. Anemia, acidosis, respiratory compromise and cardiac disease may render patients more susceptible than expected for a given methemoglobin level. The goal of the present study was to review the cumulative effect of inhaled NO on methemoglobin formation in critically ill children. We therefore looked for methemoglobin levels in children with congenital heart disease after cardiac surgery who were treated with inhaled NO in a range of 5-40 ppm.METHODS:We retrospectively reviewed the medical charts of 38 children with congenital heart disease after cardiac surgery. We extracted demographic data and physiological measurements at the following time points: (1) T0 = before starting inhaled NO therapy, (2) T1 = 24 h after the beginning of inhaled NO therapy, (3) T2 = half-time therapy, (4) T3 = end of therapy, (5) T4 = 24 h after finishing inhaled NO therapy.RESULTS:The median duration of inhaled NO therapy was 5.5 days (interquartile range 6, range 2-29), NO concentrations at T1 and T2 were 16 ppm (10, 5-40) and 12.5 ppm (12.3, 2-40), respectively. The median cumulative dose of inhaled NO was 1699 ppm (2313, 193-7018). Methemoglobin levels increased moderately, but significantly, during therapy ( T0 vs T1 p<0.05 and T0 vs T2 p<0.001). The highest methemoglobin level measured was 3.9%. Methemoglobin levels correlated positively with the inhaled NO doses applied at T1 ( r(2)=0.8376; p<0.01) and at T2 ( r(2)=0.8945; p<0.01). At T1 the methemoglobin level correlated negatively with the T1 blood pH value. The overall mortality rate was 13.2% (5 of 38 study patients died). There was no significant difference in methemoglobin levels between survivors and non-survivors.CONCLUSION:We conclude from our data that the use of inhaled NO therapy for children with congenital heart disease after cardiac surgery in the described range of 5-40 ppm, resulting in a maximum of 4% methemoglobin blood level, is feasible and safe. However, we recommend the use of the minimal effective dose of inhaled NO and continuous monitoring of methemoglobin levels, especially in cases of anemia or sepsis in critically ill children.

A series of impaired metabolic functions in Down Syndrome (DS) including glucose handling has been described. Recent information from positron emission tomography studies in DS patients and our finding of downregulated phosphoglucose isomerase (PGI) in fetal brain with DS by gene hunting using subtractive hybridization, made us investigate PGI, a key enzyme of glucose metabolism, in brain of patients with DS, Alzheimer’s disease (AD) and controls. PGI and phosphofructokinase (PFK) activities were determined in frontal, parietal, temporal, occipital lobe and cerebellum of 9 controls, 9 patients with DS and 9 patients with AD. PGI activity in DS brain was significantly decreased in frontal, temporal lobe and cerebellum, comparable to controls in parietal lobe and elevated in occipital lobe. Brain PGI activity of patients with AD was comparable to controls in all regions tested. PFK, a rate limiting enzyme of glucose metabolism, was comparable between all brain regions of all three groups. Data of this study confirm impaired glucose metabolism in DS proposed in literature and found by positron emission tomography (PET) studies. We show that changes in glucose handling in patients with AD as evaluated by PET studies are not supported by our data, although not contradictory, as determinants other than glucose metabolizing enzymes as e.g. vascular factors and glucose transport may account for these findings. Changes of downregulated PGI found by subtractive hybridization at the transcriptional level in fetal DS brain along with our findings in DS brain regions suggest a strong specific link between glucose metabolism and DS rather than AD.

JunD is a member of the Jun family of transcription factors (TF), recently shown to negatively regulate cell growth and antagonizes transformation by the protooncogene ras: c-jun decreases while junD is accumulating when fibroblasts become quiescent. Furthermore, overexpression of junD resulted in slower growth and an increase in cells in Go/G1. Performing gene hunting on fetal Down syndrome (DS) brain we found a sequence downregulated and homologous to junD. This observation made us examine junD protein levels in adult brain specimens. Western blot experiments were carried out in five brain regions of aged patients with DS (n=9), controls (n=9) and patients with Alzheimer's disease (AD, n=9). We found that junD in AD brains were comparable to controls, whereas junD levels were significantly and remarkably reduced in frontal, temporal lobe and cerebellum of patients with DS. These findings may indicate a specific finding in DS and were not linked to the AD-like-neuropathological changes of plaques and tangles, observed in DS from the fourth decade, which is also suggested by the findings of downregulated junD at the mRNA level revealed by the gene hunting technique (subtractive hybridization) in fetal DS brain. We propose that junD plays a role for the impaired development and wiring of DS brain, maybe already early in life.

The XRCC1 gene was described to play a role in the sensitivity of mammalian cell lines towards ionizing irradiation. Cells with a mutation of this gene present with decreased single-strand break repair and reduced recombination repair, show increased double-strand breaks, and the sister chromatid exchange is increased up to tenfold. The goal of our study was to investigate the transcription of this gene in the kidney following ionizing irradiation in the mouse, as this could be relevant to the pathogenetic mechanisms found in radiation nephropathy. Furthermore, we intended to examine whether radiation-sensitive mice would show a transcriptional pattern different from radiation-resistant mice. Radiation-sensitive BALB/c/J/Him mice and radiation-resistant C3H/He/Him mice were whole body irradiated with X-ray at 2, 4, and 6 Gy and sacrificed 5, 15, and 30 min after irradiation. mRNA was isolated from kidney cortex and hybridized with probes for XRCC1 and β-actin as a housekeeping gene control. Following irradiation at 2 Gy, radiation-resistant mice increased transcriptional levels of mRNA-XRCC1/mRNA-β-actin as early as after 5 min, and 15 and 30 min after irradiation, XRCC1 transcription was still higher than in radiation-sensitive mice. At higher radiation doses, no differences were found. This finding is the first in vivo study on XRCC1 of this kind and may in part explain the differences in the radiation sensitivity between the two strains studied.

BACKGROUND:The aim of our study was to ascertain the complications of chronic peritoneal home dialysis in childhood.PATIENTS:17 children were treated by ambulatory peritoneal home dialysis between 1984 and 1994 at the paediatric dialysis unit of the University Children's Hospital in Vienna, Austria. Their average age was 6.5 years (1 week to 12 years); 7 (41.2%) children were below school age (< 6 years).RESULTS:In our observation period of 369 dialysis months (DM), the average duration of dialysis was 21.7 months (4.0-74.3). In relation to total DM the incidence of peritonitis was 1:23.1 of exit site infection 1:14.8 and of catheter related complications 1:41.0. 5 children developed hernias. 5 children were switched to haemodialysis and 8 children received kidney transplants. 2 children died from non-dialysis-associated causes.CONCLUSION:Peritoneal dialysis, in contrast to haemodialysis, is a home treatment modality applicable even to infants. The most common complication is infection. Our data and the European and North American literature show that by close ambulatory monitoring and special hygenic procedures peritonitis frequency can be markedly reduced.

A current concept for the development of diabetic long-term complications is the involvement of oxidative stress, as, e.g., lipid peroxidation, in the diabetic state. Data published recently show also oxidative damage to DNA, which might be one factor for accelerated aging and diabetic microangiopathy. In our study we tested the hypothesis that l-arginine can reduce lipid peroxidation in patients with diabetes. We performed a blind placebo controlled study with crossing over two treatment periods for 3 months. Thirty patients with diabetes mellitus were randomly assigned to treatment group A (first treatment then placebo) and B (first placebo then treatment). Treatment consisted of two daily dosages of 1 g l-arginine free base. Lipid peroxidation as reflected by malondialdehyde was evaluated in urine using a standard HPLC assay. After 3 months of treatment there was a significant reduction in malondialdehyde levels in group A (p < .0032), whereas there was no difference compared to the baseline values after three months of placebo treatment in group B (p < .97). After crossing over, there was a significant reduction in malondialdehyde levels in group B (p < .0002). Group A showed a significant increase in malondialdehyde levels (p < .0063) returning to baseline values. l-Arginine treatment was able to reduce the lipid peroxidation product malondialdehyde. This provides evidence that treatment with l-arginine may counteract lipid peroxidation and thus reduce microangiopathic long-term complications in diabetes mellitus. Copyright © 1996 Elsevier Science Inc.

L-arginine and taurine are still in the center of physiological and pharmacological research. Although the fate of nitrogen of both compounds and of the 35S-taurine is well-documented, the fate of the carbon skeleton has not been elucidated yet. We studied the organ distribution of 14C arginine and 14C taurine over time in the mouse using whole body autoradiography with densitometric image analysis. We describe different organ distribution patterns. Kidney, heart, lung, the Harderian gland, the central nervous system, intestine and testis showed a comparable pattern of arginine disappearance in contrast to rapid disappearance in the salivary gland and the accumulation pattern in bone and spleen. Data on 14C taurine of liver, kidneys, lung, testis and Harderian gland resembled the arginine pattern; Accumulation of taurine carbon was found in salivary gland, bone, intestine, heart and brain. Our studies challenge and demand further related studies to obtaining more information on the fate of the carbon skeleton of these amino acids.

In diabetic nephropathy a major current concept for pathogenesis is increased collagen accumulation in the glomerulus by increased collagen synthesis and decreased degradation. In the present study, we tested the hypothesis whether arginine is able to influence kidney lipid peroxidation, glycoxidation, collagen accumulation, glucose-mediated cross-linking, hydroxy radical attack, protein oxidation, nitric oxide formation and albuminuria in the diabetic kk mouse. Ten diabetic kk mice were given arginine 50 mg/kg body weight, 10 diabetic kk mice were not treated and used as negative controls and 10 kk mice were kept as healthy controls. Our results show that oral administration of low-dose arginine reduces kidney collagen accumulation as reflected by kidney hydroxyproline, cross-linking as reflected by pentosidine, lipid peroxidation, glycoxidation as reflected by carboxymethyl lysine, kidney weight and albuminuria in the diabetic kk mouse. Albuminuria in untreated animals was closely correlated with lipid peroxidation. Our results in the spontaneously diabetic kk mouse representing type 2 diabetes mellitus therefore confirm and extend recent findings of collagen reduction by arginine in a different animal model. The mechanism of reducing proteinuria can be assigned to the blocking of lipid peroxidation products by L-arginine.

Objective To test the reliability of creatinine clearance in children on peritoneal dialysis (PD). Design Longitudinal, case-controlled. Setting Routine clinic visits at the pediatric dialysis unit of the Universitätskinderklinik of Vienna. Patients: Eleven children (2 -13 years, 10 -55 kg) with end-stage renal disease on PD. Interventions Creatinine clearance (CCr) was determined by measuring creatinine excretion (ECr) over 24 hours in both dialysate and urine. Each child had three to five separate measurements of their CCr. At the same time we also calculated the schwartz formula clearance from the patient's height and serum creatinine, using a modified correlate. Main Outcome Measures Reliability of CCr was assessed by two approaches. First, we compared each serial measurement with the mean value for each patient and thereby assessed the “intramethodical” variability. Second, we compared each CCr with the simultaneous formula clearance and assessed the “intermethodical” disagreement. Results Twenty-seven percent of the measurements of CCr were classified as unreliable based on a comparison with the mean value for each patient. Reliability was closely correlated with residual renal function (p < 0.01); only 12% of the measurements in the an uric patients were classified as unreliable (vs 31% in the patients with residual renal function). The simultaneous formula clearance was less variable than the CCr. The formula clearance had a sensitivity of 93% and a specificity of 60% for detecting unreliable values of CCr. Conclusion Estimation of total CCr is unreliable in pediatric patients on PD. A simultaneous formula clearance can be used to detect which values are unreliable.

intervals. Typically, such monitoring consists of reviewing intake and output charts, and frequent assessment of body weight. However, recording of intake and output is complicated by small volume sizes and frequent "unmeasurable losses" due to "uncooperative" patients and "noninvasive methods." In addition, assessment of body weight is often inaccurate due to small changes in weight of the patient in relation to those of wound dressings, drains, and tubes. Whole-body bioelectrical impedance analysis (BIA) might therefore be a most valuable tool for recording status and changes of hydration in critically ill infants and children as it is simple, noninvasive, easily applicable, and safe (1). Its accuracy had already been perioperatively evaluated in children undergo ing open heart surgery (2). However, recent reports in adults with ascites have shown that BIA might be insensitive to peritoneal third space losses (3,4). The aim of this study was to evaluate whether BIA is a suitable method to quantify peritoneal fluid volume.