Bilateral Wilms' tumour. Results of treatment in 29 children

Retrospective analysis of 29 children with bilateral Wilms' Tumour, 14 boys and 15 girls, aged 4m-5 years, from regional centres of paediatric oncology in Poland, treated according to the O1-92 PPGL Protocol of Wilms' tumour, was performed. All patients were treated with two (ACT + VCR) or three (ACT, VCR, DOX) cytostatic drugs preoperatively, and 26 were operated upon after cytoreductive pretreatment. In 12 children nephrectomy was performed. In 28, kidney sparing surgery was possible and made with success. 2 patients who presented disseminated disease and 1 with nephroblastomatosis were given chemotherapy. 19 out of 29 (65.5%) are alive including 1 patient with nephroblastoma. EFS calculated according to Kaplan-Meier analysis for this group, was 58% at 67 months.     

Eleven polish patients with microcephaly,immunodeficiency, and chromosomal instability: The Nijmegen breakage syndrome

We report on 11 patients from 8 independent families (3 pairs of sibs) with a complex clinical pattern including microcephaly, peculiar “bird-like” face, growth retardation, and, in some cases, mild-to-moderate mental deficiency. Most of the patients have recurring respiratory tract infections. One girl has developed B-cell lymphoma. A detailed anthropometric study of 15 physical parameters, including 3 cephalic traits, was performed. It was possible to study the chromosomes of PHA-stimulated lymphocytes in all of the patients. We found structural aberrations with multiple rearrangements, preferentially involving chromosomes 7 and 14 in a proportion of metaphases in all individuals. Profound humoral and cellular immune defects were observed. Serum AFP levels were within normal range. Radioresis-tant DNA synthesis was strongly increased in all 8 patients who were hitherto studied in this respect. Our patients fulfill the criteria of the Nijmegen breakage syndrome, which belongs to the growing category of ataxia telangiectasia-related genetic disorders. In light of the increased predisposition to malignancy in this syndrome, an accurate diagnosis is important for the patient. © 1995 Wiley-Liss, Inc.     

Assessment of the sterilization of medical devices--an important challenge to health care in Poland

The aim of the study was the evaluation of sterilization of medical devices in Polish hospitals. The system that we use to establish which sterilization procedures should be employed to reduce the risk of hospital infections associated with medical devices. Based on inquiries, the conditions for the sterilization of medical devices in 21 Warsaw hospitals were assessed. The following issues were taken into consideration: preparation of medical supplies for sterilization, methods of sterilization and the monitoring system. In order to evaluate hygienic conditions due to sterilization points system was applied. 10% of the hospitals had Central Sterilization Service Department with 3 zones where automatic washing and disinfection, sorting, packaging, sterilization and storage of medical devices had been performed. The other 20% had CSSD without zones. In more than 65% there were common services for sterilization only. Instruments were delivered already prepared for sterilization. In remaining hospitals all steps, including sterilization were performed in words. According preparation of medical devices for sterilization it was established that mainly chemical disinfection just after use and than manual cleaning was used; the automatic cleaning in washer-disinfectors is used mainly in CSSD. Steam was the preferred method of sterilization, but also low temperature methods were used for heat sensitive devices. The monitoring of sterilization processes was satisfactory. There were first trials of the validation of the sterilization processes. There is still a need for improvement in the sterilization of medical devices, especially including: the organisation of CSSD in all Polish hospitals; replacement of manual cleaning processes by automatic cleaning; organisation of advanced training courses for the heads and staff of the CSSD.     

Changes in cardiac adrenergic nervous system after transmyocardial laser revascularisation assessed by I-123-MIBG SPECT. A preliminary report

BACKGROUND: Metaiodobenzylguanidine (MIBG), a noradrenaline analogue which may be labelled with I-123, has been used in the assessment of pre-synaptic activity of the cardiac adrenergic nervous system (Syst(adren)) in several diseases. The effects of transmyocardial laser revascularisation (TMLR) on Syst(adren) have not yet been established. AIM: To examine whether TMLR-induced changes in Syst(adren) may be one of the mechanisms responsible for clinical improvement in patients undergoing this method of revascularisation. METHODS: The study group consisted of 19 patients (mean age 63+/-9 years) who underwent TMLR, by using high-power CO(2) laser; as a single method of cardiac revascularisation. Syst(adren) was assessed before TMLR (STUDY-0), soon after the procedure (mean 13+/-5 days, STUDY-I), and in 12 patients six months after TMLR (STUDY-II). In total, 50 studies using I-123-MIBG SPECT were performed. The regional distribution of tracer was assessed qualitatively, using a 17-segment model of the left ventricle. RESULTS: In 16% of examinations the assessment of the I-123-MIBG uptake was not possible due to the poor quality of images. Thus, 41 SPECT studies (16 - STUDY-0, 16 - STUDY-I, and 9 - STUDY-II) were analysed and compared. In STUDY-0, an impaired uptake of I-123-MIBG was found in 193 of 272 analysed segments. In STUDY-I, the I-123-MIBG uptake increased in 5% of defects (CI(0,95)=3-9%) and deteriorated in 55% (CI(0,95)=48-62%). When STUDY-II was compared with baseline, the uptake was increased in 25% of defects (CI(0,95)=17-34%) and decreased in further 25% of defects. When STUDY-II was compared with STUDY-I, the uptake increased in 67% (CI(0,95)=58-75%) of defects and did not deteriorate in any. The global MIBG uptake in STUDY-I decreased in 15 patients (94%, CI(0,95)=70-100%) when compared with baseline, and increased in all 9 patients with long-term follow-up data available, when STUDY-II to STUDY-I was compared. CONCLUSIONS: TMLR significantly deteriorates Syst(adren) activity which, however, improves 6 months after the procedure to the values similar to those assessed pre-operatively. TMLR-induced impairment of Syst(adren) may contribute to the clinical improvement observed shortly (<6 months) after the procedure.     

Water fungi occurence in the River Supraśl-bath Jurowce near Białystok

Studies on the occurrence of aquatic fungi in the bathing sites of the Supra?l River in Jurowce village were collected in years 2000-2003. Hydrochemical analysis was performed using standard methods. Bait method was used to isolate the fungi. In the Supra?l River at Jurowce village 36 fungi species were identified, among them fish pathogens Achlya orion, Aphanomyces laevis, Dictyuchus monosporus, Saprolegnia ferax, Saprolegnia monoica, S. parasitica, human pathogens Aspergillus flavus, Candida albicans, Lagenidium humanum, Penicillium mycetomagenum, Rhizophydium keratinophilum and Trichosporon cutaneum, phytopathogens Achlya racemosa, Phytophthora gonapodoides, Pythium butleri, P. myriotylum and P. debaryanum. Physicochemical parameters of waters in Supra?l River-bathing sites had no important effect on the occurrence of fungi.     

Carrier frequency of mutation 657del5 in the NBS1 gene in a population of Polish pediatric patients with sporadic lymphoid malignancies

Nijmegen breakage syndrome (NBS) is a human autosomal recessive disease characterized by genomic instability and enhanced cancer predisposition, in particular to lymphoma and leukemia. Recently, significantly higher frequencies of heterozygous carriers of the Slavic founder NBS1 mutation, 657del5, were found in Russian children with sporadic lymphoid malignancies, and in Polish adults with non-Hodgkin lymphoma (NHL). In addition, the substitution 643C>T (R215W) has also been found in excess among children with acute lymphoblastic leukemia (ALL). In an attempt to asses the contribution of both mutations to the development of sporadic lymphoid malignancies, we analyzed DNA samples from a large group of Polish pediatric patients. The NBS1 mutation 657del5 on one allele was found in 3 of 270 patients with ALL and 2 of 212 children and adolescents with NHL; no carrier was found among 63 patients with Hodgkin lymphoma (HL). No carriers of the variant R215W were detected in any studied group. The relative frequency of the 657del5 mutation was calculated from a total of 6,984 controls matched by place of patient residence, of whom 42 were found to be carriers (frequency = 0.006). In the analyzed population with malignancies, an increased odds ratio for the occurrence of mutation 657del5 was found in comparison with the control Polish population (OR range 1.48-1.85, 95% confidence interval 1.18-2.65). This finding indicates that the frequency of the mutation carriers was indeed increased in patients with ALL and NHL (p < 0.05). Nonetheless, NBS1 gene heterozygosity is not a major risk factor for lymphoid malignancies in childhood and adolescence.     

Occurrence of fungi and fungus-like organisms in the Horodnianka River in the vicinity of Białystok, Poland

Studies of fungi and fungus- like organisms in the northeastern Poland have mainly concentrated on running waters in the vicinity of Bia?ystok, including the Horodnianka River. The main objective was to investigate biodiversity of fungi and fungus-like organisms which take part in decomposition of organic matter commonly found in inland waters. To obtain a complete picture of species composition of fungi and fungus-like organisms in running waters we decided to explore representative sites of the Horodnianka River such as Olmonty, Hryniewicze and Horodniany with close localization of landfill. Fungal species were isolated using baiting technique. Baits of onion skin (Alium cepa), hemp-seeds (Cannabis sativa), impregnated cellophane and snake skin (Natrix natrix) were applied to isolate fungi from water of the Horodnianka River. The fungal community consists of 26 species, 10 species of fungi belonging to class Chytridiomycetes (3), anamorphic fungi (6), and Zygomycetes (1). 16 species belong to fungus-like organisms from class Oomycetes. Most of the recognized species have already been found in other running waters. From all the examined habitats the fungi belonging to 26 species of 18 genera Achlya, Alternaria, Aphanomyces, Aspergillus, Catenophlyctis, Dictyuchus, Fusarium, Karlingia, Lagenidium, Leptomitus, Olpidiopsis, Penicillium, Phlyctochytrium, Pythium, Saprolegnia, Scoliognia, Thraustotheca and Zoophagus were obtained. Certain fungal species like Aphanomyces laevis, Fusarium aqueductum, F. moniliforme, F. oxysporum, Leptomitus lacteus, Saprolegnia feax and S. parasitica were found at all the study sites. Among fungi potentially pathogenic and allergogenic for humans the genera Alternaria, Aspergillus, Fusarium, Lagenidium and Penicillium have already been described. However, the species Lagenidium giganteum and Achlya androgyna are new in the fungal biota of Poland. The greatest number of fungal species occurred in Olmonty (24), the smallest in Horodniany (13). Presence of fungi such as Leptomitus lacteus, Fusarium aqueductum in the water of the Horodnianka River offers the possibility of using them as indicators of water quality.     

Homocysteine,adrenergic activity and left ventricular mass in patients with essential hypertension

OBJECTIVE: Assessment of relationship between homocysteine (Hcy) and noradrenaline (NA), adrenaline (A) concentration and left ventricular mass index (LVMI) in patients with essential hypertension (EH). DESIGN AND METHODS: Samples obtained from 37 patients (14 female, 23 male) with mild EH (according to WHO criteria) (mean age 43.6 +/- 13.2 years) and 37 healthy volunteers (18 female, 19 male; mean age 38.2 +/- 10.6 years) were evaluated for Hcy (ELISA), NA and A (HPLC). Each patient underwent echocardiographic investigation with LVMI measurement (Penn convention). The examinations were performed in the outpatient clinic. RESULTS: Hcy was significantly higher in patients with EH (8.7 +/- 2.4 vs 6.6 +/- 1.3 micromol/l; p < 0.01). NA and A levels were significantly elevated in the EH group (A: 43.9 +/- 26.4 vs 36.9 +/- 29.4 pg/ml; NA: 428.5 +/- 148.8 pg/ml vs 314.6 +/- 103.4 pg/ml; both p < 0.05). LVMI was also significantly higher in EH group (96.6 +/- 19.5 vs 83.4 +/- 16.0 g/m2; p < 0.01). There was no significant correlation between Hcy and other analysed parameters in the studied groups. CONCLUSION: High levels of Hcy appear together with increased left ventricular mass and augmented adrenergic activity in patients with EH. Coexistence of high Hcy concentration, left ventricular hypertrophy and increased adrenergic activity increases the risk of atherosclerosis and cardiovascular disease in patients with EH.