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Biomedical subjects

J Fydryk

Publications and source records attributed to J Fydryk.

At least 19 recordsLinked to original sources

[Renal gene expression of renin and transforming growth factor Beta 1 in children with glomerulonephritis].

Increase of renal expression of transforming growth factor beta 1 (TGF-beta 1) gene caused by activation of the local renin-angiotensin system plays an important role in the pathogenesis of glomerulonephritis (GN). The aim of the present study was to measure the expression of renin and TGF-beta 1 genes (own modification of the RT-PCR method) in the isolated renal glomeruli or in the homogenates of renal biopsy specimens in children with various types of glomerulonephritis. The study enrolled 13 children with glomerulonephritis and 3 boys with Wilm's tumour (control group). The expression of the studied genes was presented using arbitrary units defined as multiplicity of the GAPDH gene. No significant difference was found in expression of mRNA renin in the biopsy specimens of the kidney between GN group and control group. Expression of the TGF-beta 1 gene was found in biopsy specimens in all patients from the control group, and only in one GN child, the sole one who was not treated with converting-enzyme inhibitors. No transcripts of the studied genes were found in all RNA samples obtained from the renal glomeruli using the microdissection method. The RT-PCR method applied in the present study allows evaluation of renal expression of renin and TGF-beta 1 genes. The authors would like to point out that storage of biopsy specimens at -80 degrees C would not prevent the total degradation of RNA during microdissection.

Adolescent↗

[The I/D polymorphism of the ACE gene in children with Henoch-Schoenlein purpura].

The study group consisted of 24 children with Henoch-Schoenlein purpura (HSP) (13 girls and 11 boys, aged 66-233 months) who were treated in the Ist Pediatric Department between 1980 and 1998. The I/D polymorphism of ACE gene was determined by PCR amplification of genomic DNA with primers flanking the polymorphic region. Our preliminary results suggest lack of association between ACE genotype and kidney function. However, further follow-up studies based on sufficient number of participants are necessary to elucidate the role of ACE polymorphism in appearance and progression of renal symptoms in HSP.

Adolescent↗

[Renoscintigraphy applied with etylenedicysteine labeled with technetium 99m and SPECT technique as a method of examining kidneys in children with vesicoureteric reflux].

Vesicoureteric reflux (VUR) can lead to renal parenchymal damage. Renal scarring is an important cause of chronic renal failure and hypertension in children. The significance of possible effects determines the necessity of early diagnosis of urinary tract pathology. The aim of the paper was to evaluate the morphology and function of kidneys with VUR using selected radioisotope techniques, and to compare the sensitivity of planar technique and single-photon emission computed tomography (SPECT) technique in detection of renal scarring. In 45 children with VUR the following test were performed: ultrasonography, renoscintigraphy with technetium-99m-ethylenedicysteine (EC-Tc-99m) and technetium-99m-dimercaptosuccinic acid (DMSA-Tc-99m) scintigraphy with planar and SPECT mode. Stage of VUR correlates with stage of cortical lesions estimated as a value of effective renal plasma flow (ERPF) in kidney, as well as scarring intensity in static scintigraphy. The use of SPECT increases sensitivity of examination for detection small, single scars. It seems that SPECT should be used more frequently in children in the group of scarring risk. That would allow for earlier diagnosis of renal scarring, enabling efficient treatment. Due to the correlation between ERPF and parameters obtained in DMSA scintigraphy, renoscintigraphy with EC-Tc-99m may be applied to monitor the progress of renal scarring.

Adolescent↗

Impact of recurrent nephrotic syndrome after renal transplantation in young patients.

Recurrent disease is a frequent complication of patients transplanted for steroid-resistant nephrotic syndrome associated with focal segmental glomerulosclerosis. Its long-term prognosis has rarely been studied. We examined 39 patients aged 4-25 (mean 13.5) years at the time of first transplantation (TX). Twelve of these (30%) developed nephrotic syndrome after the first TX and 2 of 8 after the second TX. The mean observation period from first TX to last observation with a functioning graft or graft loss was 5.4 (0.1-19.3) years. We confirmed that recurrent disease is associated with older age at onset of the primary disease, shorter time from onset to end-stage renal disease, and diffuse mesangial proliferation in the initial kidney biopsy. Remissions occurred in all 3 children undergoing early repeated plasma exchange and in 1 adolescent following introduction of cyclosporin A 7 years after TX. At last observation 42% of relapsing and 48% of non-relapsing patients with a similar follow-up period had a functioning first graft. Median first graft survival was almost identical in the relapsing and the non-relapsing patients (4.3 vs. 4.2 years). Histological lesions of focal glomerulosclerosis were detected in the posttransplant biopsies of only 3 patients. In conclusion, young patients with nephrotic syndrome associated with focal segmental sclerosis have a similar graft survival with and without recurrence of the nephrotic syndrome.

Adolescent↗

Antioxidant status of children with steroid-sensitive nephrotic syndrome.

Eighteen children with steroid-sensitive nephrotic syndrome (SSNS) were studied. The control group comprised 20 healthy children. The following indirect parameters of reactive oxygen species activity were determined in nephrotic patients during four stages of the disease (full relapse before prednisone administration, disappearance of proteinuria, prednisone cessation, unmaintained remission): plasma malondialdehyde (MDA) levels, copper/zinc superoxide dismutase (CuZn SOD) activity and glutathione peroxidase (GPX) activity in erythrocytes, reduced glutathione (GSH) and vitamin C levels in whole blood, and vitamin E level in serum. Increased MDA levels, reduced vitamin C levels, and enhanced CuZn SOD activity were found in relapse. GSH concentration was high during all four stages. Vitamin E level was also increased, parallel to the pattern of serum lipids. GPX activity remained low during the proteinuria stage and in remission. We conclude that the majority of abnormal findings can be attributed to the hyperlipidemia of NS. Low GPX activity may be a factor limiting the antioxidant capacity in NS. The present study is inconclusive regarding the role of free radicals in the proteinuria of NS.

Adolescent↗

Prevalence and forms of congenital anomalies in twins born in Pomeranian District during the period from 1.07.1997 to 31.12.1998. Polish Register of Congenital Anomalies.

The authors have analysed the frequency and structure of congenital anomalies in children born in the Pomeranian district in the period from 01.07.1997 to 31.12.1998. Among a total of 28.361 births in that area, 748 (2.64%) were affected by congenital anomalies. Among 28.361 births, 620 (2.18%) were from multiple pregnancies. 23 (3.71%) among births from multiple pregnancies were affected by congenital malformations. The prevalence rate of inborn anomalies in births from multiple pregnancy in our area were higher (3.71%) in comparison to births from singleton pregnancy (2.61%). It implies that children born from multiple pregnancy are at higher risk of developing congenital anomalies.

Congenital Abnormalities↗

[DNA index and proliferative activity of blastic cells in childhood ALL].

DNA Index and the pretreatment proliferative activity of blastic cells were assessed in relation to treatment results in 58 children with acute lymphoblastic leukemia. ALL patients were treated with the use of BFM 79 protocol. After the median follow-up time of 32 months p-EFS and p-DFS were 0,425 and 0,495 respectively. There were 35 (56.45%) diploic, 21 (33.81%) hyper-diploic and 2 (3.22%) hypo-diploic patients. There was no statistical difference in p-EFS and p-DFS between diploic and hyper-diploic patients. Patients in I-st remission were characterized by significantly higher percentage of cells remaining in the S-phase of the cell cycle.

Adolescent↗

Mesangial alterations in steroid-responsive minimal change nephrotic syndrome.

Renal biopsies from 25 children with steroid-sensitive minimal change nephrotic syndrome were evaluated retrospectively to determine whether there is any relation between the morphological changes and the frequency of relapses. Biopsy material was examined by light-, immunofluorescence-, and electron microscopy, and by morphometric methods. The patients were divided in a group of 15 children with frequent relapses (FR) and another group of 10 children with an absence of, or only infrequent, relapses (NR/IR). Semiquantitative evaluation of biopsy specimens disclosed no significant differences between groups, but morphometric measurements performed on toluidine stained semi-thin sections showed a significant increase of mesangial nuclei in FR compared with NR/IR (P less than 0.01). Furthermore, the mean area of mesangial nuclei was decreased and the relative frequency of smaller nuclear profiles was higher in patients with FR compared to NR/IR (p less than 0.01). These findings suggest mesangial cell activation in FR which may be related to a longer course of the disease prior to renal biopsy (mean 4.0 years in FR vs. 1.4 years in NR/IR). In our opinion, morphometric assessment of discrete mesangial alterations is a promising method for exploring clinicopathological correlations in minimal change nephrotic syndrome.

Adolescent↗