Patent ductus venosus: diagnosis by MR angiography.

We report a 15-year-old boy with patent ductus venosus in whom the diagnosis was made by MR angiography. A patent ductus venosus Arantii is a rare form of portosystemic shunt. Only a few cases have been reported in adults and children. The diagnosis is usually made by US and digital subtraction angiography. In our patient, the diagnosis was first made by MR angiography. This demonstrates the excellent diagnostic potential of the method in paediatric patients.

Paediatric thrombo-embolism: the influence of non-genetic factors and the role of activated protein C resistance and protein C deficiency.

UNLABELLED: In many children, the pathogenesis of thrombo-embolism remains unexplained. This study examines the role of non-genetic risk factors in 37 children with venous or arterial thrombosis. Included were 17 patients with portal vein thrombosis following umbilical vein catheterisation, 6 with portal vein thrombosis and an uneventful neonatal period, 4 with deep vein thrombosis, 4 with renal vein thrombosis after kidney transplantation, 1 haemodialysis patient with thromboses of arteriovenous shunts, and 5 with arterial thromboses at various sites. In 25 of these 37 patients (68%) exogenic risk factors and particularly vascular manipulations (24/37) were related to the thrombotic event. Resistance to activated protein C was identified in 5 patients and protein C deficiency in 2 (7/37; 19%). This prevalence was significantly higher than that of the control group (14/243; 5.8%; chi 2, P < 0.008). CONCLUSION: Our data show that non-genetic and particular iatrogenic risk factors can often be identified in children with thrombosis, but activated protein C resistance and protein C deficiency are significant genetic risk factors in this age group.

[Effect of serum and ultrafiltrate (less than 50 k DA) of normal serum and uremic serum from chronic hemodialysis children on lipid peroxidation and vitality of fetal rat hepatocytes in vitro]. / Untersuchung des Einflusses von Serum und Ultrafiltrat (less than 50 k Da) aus Normalserum und urämischem Serum chronisch hämodialysierter Kinder auf die Lipidperoxidation und die Vitalität fetaler Rattenhepatozyten in vitro.

The influence of ultrafiltrate with a molecular exclusion border of less than 50 kDa of hemodialyzed children on viability and lipid peroxidation (LPO) of hepatocytes was investigated in the model of fetal rat hepatocytes. Viability of cells was estimated by trybanblue test and malondialdehyde equivalents (MDA) were estimated by thiobarbiturate method as parameters of LPO. Ultrafiltrate of uremic patients may lead to a concentration-dependent loss of viability and simultaneous reduction of MDA formation of hepatocytes after incubation of 120 min. Uremic serum may reduce the viability, but not the MDA formation of hepatocytes opposite to uremic ultrafiltrate (p less than 0.01). In uremic serum high molecular substances (greater than 50 kDa) may show a cytotoxic effect, but on the other side low and high molecular substances may increase the antioxidative capacity in comparison with serum of healthy volunteers.

[Equivalence values of the antioxidative capacity in serum of children with chronic renal failure, chronic hemodialysis and kidney transplantation]. / Aquivalenzwerte der antioxidativen Kapazität im Serum chronisch niereninsuffizienter, chronisch hämodialysierter und nierentransplantierter Kinder.

It is reported on the results of measurements of the antitoxydative capacity (AC) in the serum of children suffering from chronic renal failure, during hemodialysis and after kidney Transplantation. The results show in comparison with healthy children (19.95 +/- 5.22 mm, n = 19) that children suffering from chronic renal failure may develop an increased AC (66.18 +/- 29.14 mm, n = 24). In dialyzed children different values are found previous and after hemodialysis (58.11 +/- 9.25 vs. 17 +/- 3.6 mm). Children show after transplantation the highest values (78.9 +/- 26.04 mm, n = 13). If the increase of the AC is a compensatory and regulatory mechanism or the result of accumulation of antioxydative substances should realized in further investigations. The measurements of vitamin-E-supplemented sera may show that an oral application of vitamin E increase the AC in vivo.

[Malondialdehyde concentration in the serum of children with chronic renal failure, chronic hemodialysis and kidney transplantation]. / Malondialdehydkonzentration (MDA) im Serum chronisch niereninsuffizienter, chronisch hämodialysierter und nierentransplantierter Kinder.

The malondialdehyde equivalent (MDE) was measured by the thiobarbiturate method quantitatively in serum of healthy children, children suffering from chronic renal failure and after kidney transplantation. In the reference group (n = 19) a serum concentration of 3.98 +/- 0.84 nmol/ml was found. Elevated MDE serum concentrations were measured in all groups suffering from renal disease: groups with various degree of diminished renal function (7.05 +/- 2.12 nmol/ml; n = 24), children treated by chronic hemodialysis (6.38 +/- 0.98 nmol/ml; previous dialysis and 6.27 +/- 0.63 nmol/ml after dialysis, n = 9) and children after renal transplantation (6.59 +/- 1.14 nmol/ml; n = 13). In the hemodialysis group the MDE concentration was not affected by a single dialysis nor by sixth months dialysis course. A cell-damaging lipid peroxidation in children suffering from renal diseases is reflected by the investigations.

[The hepatocyte of the fetal rat as an in vitro model system for testing the antioxidant activity of biological fluids]. / Der Hepatozyt der fetalen Ratte als in vitro-Modellsystem zur Prüfung antioxidativer Aktivität von biologischen Flüssigkeiten.

In case of hepatocytes of the fetal rat at the end of the gestation (H.) the lipid-peroxidation was determined by measuring the malondialdehyde (MDA) and the vitality by means of the trypan-blue test. The H. with an incubation up to 120 minutes in PBS formed a high spontaneous value of MDA with a simultaneous loss of the cell activity (r = -0.89; p less than 0.05; n = 25). On the conditions of an experimentally generated activation of O2-radical-forming processes (addition of Fe2+/vit. C) concentration-depending increasing of the MDA-formation and trypan-blue reception of the H. were induced (p less than 0.01). The addition of vit. C reduced the MDA-formation and the vitality of the H. The results exclude a direct relation of the two parameters. The MDA-formation of the H. in vitro can be used for testing antioxidative effects of biological fluids.

[The effect of pH, temperature and albumin content on the vitality of fetal rat liver cells during in vitro tests]. / Einfluss von pH, Temperatur und Albumingehalt auf die Vitalität von Leberzellen fetaler Ratten bei in vitro-Versuchen.

The influence of pH, temperature, and albumin content in the incubation medium on the vitality incubated hepatocytes was tested in vitro as well as the effects in the trypan-blue exclusion test. pH changes between 6.8 and 7.4 in the incubation medium have not any influence on the vitality of the hepatocytes or the trypan-blue exclusion test. Reduced vitality results occurred in an initial pH 7.8 in the incubation medium. The vital dye exclusion test is not useful in case of incubation temperature of 4 degrees C, since the results regarding the vitality of the hepatocytes are only apparently better. An addition of human serum albumin is necessary for a good survival of the hepatocytes during the incubation.

[Treatment and metaphylaxis of cystine urolithiasis in children]. / Therapie und Metaphylaxe der Zystinurolithiasis im Kindesalter.

Basic ideas about the pathogenesis of cystinuria and calculi formation are shown in this case report. Aspects of a present and effective cystine stone therapy are discussed after that. We present the therapeutic procedure in two patients with recurrent cystine urinary stone formation who were operated on cystine stones in the past. Our experiences with a combination of chemolitholysis with N-acetyl-cystein through percutaneous nephrostomy and an oral drug therapy with alpha-mercaptopropionyl-glycin are reported. This form of treatment was successful in our two cases. A metaphylactic therapy is necessary to prevent recurrent cystine stone formation.

[The behavior of serum "liver enzyme" activity in children with chronic renal failure, hemodialysis and kidney transplantation]. / Zum Aktivitätsverhalten von "Leberenzymen" im Serum bei chronisch niereninsuffizienten, hämodialysierten und nierentransplantierten Kindern.

Liver enzymes were measured in 24 children with different degrees of renal insufficiency, in 6 children treated by chronic hemodialysis and in 13 children after kidney transplantation. The hemodialyzed and transplanted patients have the highest ALAT activity, independently of the presence of a liver infection. The AP activity in these patient groups were not different from the reference value. The activity of ALAT, GGT and CHE, respectively were most pathologic in the hemodialysis group. The enzyme activities have not been influenced by a single dialysis and a 6-month dialysis course. In uremic and chronic hemodialyzed patients the enzyme combination of ALAT, GGT and CHE has been recommended in the diagnosis and follow-up control of a liver cell damage. In the evaluation of enzyme activities in comparison with reference values of healthy volunteers an enzyme inhibition by the uremic serum should considered.

[Management of children with fatal liver diseases with reference to eventual liver transplantation]. / Die Betreuung von Kindern mit schicksalhaft verlaufenden Leberkrankheiten unter dem Aspekt der eventuellen späteren Lebertransplantation.

Children suffering from alpha 1-antitrypsin deficiency hepatopathies, autoimmune hepatitis and from extrahepatic biliary atresia are cared for with the aim of rendering possible a liver transplant in case of liver insufficiency. Among the laboratory parameters signalling hepatic insufficiency the increase in the indirect bilirubin as well as the decrease of cholinesterase activity and the Quick are of great importance. So far it has not been possible to generalize from the data obtained from the longitudinal examination of patients in order to predict the prognosis of severe hepatopathies.

[Effect of human uremic serum on the survival of fetal rat hepatocytes]. / Einfluss von humanem urämischem Serum auf die Vitalität fetaler Rattenhepatozyten.

Children suffering from chronic renal insufficiency frequently show a liver cell damage. It is not clarified, whether this damage is caused immediately by chronic renal insufficiency. In the model of fetal rat hepatocytes, which were isolated by trypsin digestion, we have investigated the influence of normal and uremic serum on the lactate dehydrogenase release and the staining with trypan blue, respectively. Fetal rat hepatocytes under incubation conditions seems to be a useful model in the investigation of the influence of uremic metabolism on the liver cell. In our liver cell model the uremic serum did not influence the membrane permeability of hepatocytes for macromolecules. However, the viability of hepatocytes was impaired by 23% compared with normal serum. The vitality-reducing factor of the uremic serum is not dialysable.

[The percutaneous approach to the pediatric kidney]. / Der perkutane Zugang zur kindlichen Niere.

The percutaneous access also to the infantile kidney increasingly gains significance. It is reported on experience with 16 percutaneous interventions at the kidney in 14 children. Indication, technique and complications are demonstrated, compared with literature and discussed. The percutaneous access to the kidney allows the clarification of certain diagnostic problems, guarantees the protection of the kidney in congenital and acquired obstructions, septic renal diseases, postoperative complications. The drainage of urinomas, the dilation of stenoses of the ureter as well as nephrolitholapaxia and chemolitholysis are with certain restrictions possible also in childhood.

[Oral galactose loading in characterizing the elimination and metabolizing performance of the liver in chronic renal failure]. / Die orale Galaktosebelastung zur Charakterisierung der Eliminations- und Metabolisierungsleistung der Leber bei chronischer Niereninsuffizienz.

For characterising the elimination and metabolisation performance of the liver in chronic renal insufficiency the galactose load test was carried out. 9 children with healthy liver and kidneys, 35 children with chronic renal insufficiency (15 were conservatively treated = group 1, 20 were in the chronic haemodialysis programme = group 2) as well as 5 children after kidney transplantation were examined. In group 1 a normal blood galactose concentration was present. Patients of the 2nd group showed increased concentrations of galactose in the blood which might refer to a decreased redox potential in the liver caused by the uraemic intoxication. In the group of patients who underwent a kidney transplantation in the patients with azathioprine therapy a disturbed use of galactose was present. Children with cyclosporin-A had a normal galactose concentration.