Impact of lipophilic antioxidants and level of antibodies against oxidized low-density lipoprotein in Polish children with phenylketonuria.

The treatment of phenylketonuria (PKU) patients constitutes a phenylalanine (Phe) intake restriction in their diet, which is achieved by adding a special Phe-free amino acid mixture to the diet. It has been reported that this diet could have some micronutrient deficiency. Several authors have also reported an increased oxidative stress or impaired antioxidant status in human and experimental PKU. Our project assessed the concentrations of retinol, alpha-tocopherol, coenzyme Q10, and anti-oxidized low-density lipoprotein (ox-LDL) antibodies in PKU children's plasma. It was found that retinol concentration in PKU children remains within the norm despite a low intake. The lower plasma alpha-tocopherol concentration in PKU children compared with normal children was associated with the lower level of antibodies against ox-LDL. This raises the question whether higher than observed circulatory alpha-tocopherol is indeed beneficial to lower plasma ox-LDL levels. Further studies are needed to explain the genetic factor in PKU patients (e.g., CD36/FAT polymorphism gene). The open clinical question is whether daily supplementation of alpha-tocopherol changes the PKU patients' level of antibodies against ox-LDL.

[Serum leptin levels in children with chronic renal failure on continuous ambulatory peritoneal dialysis and on hemodialysis treatment]. / Ocena stezenia leptyny w surowicy krwi u dzieci z przewlekla niewydolnoscia nerek, leczonych za pomoca ciaglej ambulatoryjnej dializy otrzewnowej oraz hemodializ.

UNLABELLED: Malnutrition and loss of appetite remain a frequent problem in children with chronic renal failure (CRF). Recent studies in human gave much attention for leptin, polypeptide produced by adipocytes, and its influence on appetite and energetic balance regulation. The study aimed at evaluation of serum leptin levels (SLL) in children with CRF with regard to applied method of treatment. Study included 29 children, mean age 13.9 +/- 4.2 years. Among them 10 on peritoneal dialysis (PD, mean age 11.9 +/- 4.3 years), 10 - on maintenance hemodialysis (HD, mean age 16.3 +/- 2.4 years), 9 - on conservative treatment of CRF (CT, 13.0 +/- 4.3 years). SLL was evaluated by RIA. In all groups of children with CRF SLL was high: group I (PD): 34.3 +/- 44.0, group II (HD): 31.5 +/- 57.4, group III (CT): 16.5 +/- 21.1 ng/ml. There was no difference of SLL in children with CRF regarding to method of treatment. Evaluated SLL/BMI index (body mass index) showed no difference in comparison of examined groups of children with CRF (values respectively: PD: 1.5 +/- 1.8; HD: 1.3 +/- 2.1; CT: 0.8 +/- 1.9 ng/ml +/- kg/m2). Also SLL/creatinine index did not differ between examined groups (values respectively: PD: 5.2 +/- 8.2; HD: 4.4+/-8.4; CT: 4.0 +/- 4.6 ng/mg). In both subgroups on dialysis treatment SLL correlated with BMI, additionally in HD SLL correlated with body weight, in PD - with serum ionised calcium. No correlation with age, dialysis treatment duration, creatinine and urea levels was found. CONCLUSIONS: In children on maintenance dialysis high SLL were observed. The highest SLL in PD patients, when considering CRF group could be related to high dialysate glucose concentration and probably decreased clearance through the peritoneal membrane. The correlation between SLL and BMI was preserved in all groups of children with CRF, irrespective of applied method of treatment.

[Treatment of chronic hepatitis with interferon in children with kidney diseases]. / Leczenie interferonem przewleklego zapalenia watroby u dzieci z chorobami nerek.

UNLABELLED: The study aimed at evaluation of chronic hepatitis treatment results in children previously treated from nephrological indications in 1994-2002 years. MATERIAL AND METHODS: Examination was performed in 42 children in the age 10.2 +/- 4.8 years at the onset time of interferon (INF) treatment application. In 30 children (71.4%) chronic HBV infection, in 8 (19.1%) - HCV infection, in 4 (9.5%) - mixed HBV and HCV infection in phase of replication was revealed. Among examined children in 26 (61.9%) symptoms of glomerulonephritis were previously reported, in 17 (40.5%) - symptoms of nephrotic syndrome; in 9 (21.4%) - chronic renal failure was observed. 22 children received prednisone treatment. Concentrations of albumin, gamma- globulin, bilirubin, haemoglobin, creatinine, haematocrit, leukocytosis, activity of alanine aminotransferase (ALT), chronic hepatitis markers, before, during and 6 and 12 months after treatment termination were evaluated. IFN alpha-2a, alpha-2b and human recombined IFN-alpha were applied RESULTS: In 22 (52.4%) children ALT values before treatment not exceeded 100 IU/l. Liver biopsy was performed in 39 children. In 18 (46.2%) - high activity of inflammatory process was revealed; only in 5 of them with ALT activity above 100 IU/l. Higher leukocytosis at the beginning of treatment was accompanied by diminished activity of inflammatory process. In 14/34 children seroconversion was obtained in HBe markers, in 4/12 HCV-RNA elimination occurred after the 1st course of IFN. Only in 5 (11.9%) children treatment was stopped because of side effects (not connected with urinary tract), in 1 - because of relapse of main disease. 2nd course was applied in 13 children. In 2 - seroconversion in HBe markers was obtained. CONCLUSIONS: Considering the small number of examined children full evaluation of chronic hepatitis treatment efficacy is not possible. It seems comparable as observed in population of children without the risk of nephropathies. IFN treatment in children on previous medication of kidney disease, in most cases does not create complications leading to earlier drug cessation. In the case of glomerulonephritis also does not bear an increased risk of relapse of main disease.

[Visual evoked potentials in children with high blood lead level]. / Wzrokowe potencjaly wywolane u dzieci z podwyzszonym poziomem olowiu we krwi.

PURPOSE: The aim of this study is to find the relationship between lead level in blood (Pb-B) and visual evoked potentials (VEP) in children, environmentally exposed to lead. MATERIAL AND METHODS: 32 children (64 eyes) with no clinical signs of lead poisoning were examined. Corrected visual acuity was 5/5 in all eyes. Age of the children was 2-15.5 years (mean 8), Pb-B ranged from 150 to 486 (mean 262) microg/l. For pattern VEP LKC equipment, UTAS E-2000 program and stimulations of 13', 26', 52', 105' were used. P100 amplitude and latency were calculated. RESULTS: P100 latency was significantly delayed in children at the age of seven, with Pb-B above 150 microg/l. The latencies were more delayed in children with higher than in lower Pb-B, but the relation was not linear. P100 amplitudes did not differ significantly from the control group. In some children VEP had abnormal morphology, double P100 peak or P100 without sharp, but extended peak. CONCLUSION: Delayed P100 PVEP latency occurred in children with blood lead level higher than 150 microg/l, after at least 7 years of environmental exposure. Changes in VEP are similar to those which are caused by demyelinization and are found in multiple sclerosis.

[Visual evoked potentials in children cured with sodium-calcium versenate because of high blood lead level--the prospective study]. / Wzrokowe potencjaly wywolane u dzieci leczonych wersenianem sodowo-wapniowym z powodu podwyzszonego poziomu olowiu we krwi--badanie prospektywne.

PURPOSE: To find out if blood lead level (Pb-B) changes have influence on visual evoked potentials. MATERIAL AND METHODS: 18 children (36 eyes) treated with CaNa2EDTA because of chronic environmental lead poisoning were examined two times: first at age 2-15.5 (mean 8) years and second 4 years later. For pattern VEP LKC equipment, UTAS E-2000 program and stimulations of 13', 26', 52', 105' were used. P100 amplitude and latency were calculated. RESULTS: Pb-B was decreased from 150-486 (mean 262) microg/l to 42-248 (mean 103) microg/l. After 4 years, P100 latency was significantly delayed in children with Pb-B above 100 microg/l at second examination. In children with Pb-B below 100 microg/l no significant differences between two consecutive examinations were observed. There were no significant differences in P100 amplitude. CONCLUSIONS: Delayed P100 latency of PVEP in children is permanent but non-progressive if Pb-B is lower than 100 microg/l.