[Homocysteine and glutathione metabolism in steroid-treated relapse of idiopathic nephrotic syndrome]. / Metabolizm homocysteiny i glutationu w zaostrzeniu idiopatycznego zespolu nerczycowego leczonym glikokortykosteroidami.

UNLABELLED: Changes in metabolism of aminothiols may have an influence on endothelial function or change the red-ox balance. The aim of study was designed to assess changes in plasma aminothiols': proatherogenic (homocysteine-HCY) and antiatherogenic (glutathione-GSH) metabolism in nephrotic syndrome in children. MATERIAL AND METHODS: The study group included 77 nephrotic children (aged 2-18 years) divided into four groups, i.e. in acute phase of the disease (24), during steroid-induced (24), steroid-free (12) and in long-term remission (17). Twenty five healthy children served as controls. GSH and HCY in plasma were assessed by high-performance liquid chromatography. Fraction of protein-bound and free aminothiols was assessed and albumin saturation was calculated. RESULTS: GSH and its fractions' concentrations were comparable to healthy subject, however in early relapse free fraction was significantly higher than in late remission. The albumin saturation with GSH was significantly higher in early than in late relapse. Total HCY concentration was decreased in early relapse, elevated after 2 week and comparable to controls after 8 week of treatment. HCY free fraction and albumin saturation were elevated within first 2 weeks. Children in long-term remission showed elevated total concentration of HCY and GSH and their protein bound fractions when compared to controls. Albumin saturation with these aminoacids was higher as well. CONCLUSION: The study showed aminothiol imbalance in children in first weeks of relapse of nephrotic syndrome.

Markers of endothelial dysfunction in children with idiopathic nephrotic syndrome.

Endothelial function impairment may constitute a link between nephrotic syndrome and atherosclerosis. We assessed changes in plasma thrombomodulin, von Willebrand factor and plasminogen activator inhibitor-1 at different stages of idiopathic nephrotic syndrome in children and correlated them with clinical and biochemical parameters. The study group included 132 nephrotic children (aged 2-18 years) divided into four groups, i.e. in acute phase of the disease with proteinuria, during steroid-induced remission, steroid-free remission, and in long-term, steroid-free remission. Forty-one healthy children served as controls. Plasma thrombomodulin, plasminogen activator inhibitor-1 and von Willebrand factor activity were increased in children with early nephrotic relapse. They systematically decreased in later stages of the disease but the increase in von Willebrand factor persisted in drug-free remission. These disturbances were dependent on the degree of proteinuria and serum albumin concentration. The study revealed that nephrotic children show markers of endothelial dysfunction that are dependent on the disease activity. This leads to the hypothesis that children with severe clinical course of nephrotic syndrome may be at high risk of accelerated atherogenesis.

[Carriage of Streptococcus pneumoniae in nasopharynx in children with nephrotic syndrome aged 2-5 years in Poland]. / Nosicielstwo nosogardiowe Streptococcus pneumoniae u dzieci w wieku 2-5 lat z zespolem nerczycowym w Polsce.

Children with nephrotic syndrome (NS) are at increased risk of Streptococcus pneumoniae infections. Streptococcus pneumoniae carrier state in the nasopharynx is an early stage of this infections. The aim of the study was to assess carriage rate and characterise bacterial isolates of Streptococcus pneumoniae among children with NS in a prospective multicentre study involving 10 nephrologic centres in Poland. We studied 95 children including 56 boys (59%) i 39 girls (41%) with NS in mean age 3 i 7/12 +/- 11 miesiecy. All children had throat and nasopharynx swabs taken in two epidemiological seasons (Oct-Dec 2006 and Feb-Apr 2007). Streptococcus pneumoniae isolates were serotyped with PCR. Carriage of Streptococcus pneumoniae was diagnosed in 13 children (13.7%) including 13 boys (100%). Following serotypes were found: 6B (38.5%), 9V (15.3%), 23F (15.3%), 19F (7.6%). There were 23.3% isolates without identifiable serotype. Carriage of Streptococcus pneumoniae was diagnosed mostly in boys with NS. Serotype 6B was dominant in this age group.

Ectopic mediastinal parathyroid carcinoma as a cause of dialysis-dependent renal failure.

Carcinoma of the parathyroid gland is infrequent in patients with secondary hyperparathyroidism. Typical clinical symptoms are related to the presence of a neck mass and hypercalcemia. We describe a case of a 55-year-old man in whom primary parathyroid carcinoma led most likely to the development of end-stage dialysis-dependent renal failure, and the diagnosis of the cancer was delayed due to ectopic localization of the tumor, and dramatic complications in the clinical course of the disease, including acute pancreatitis and peritionitis. However, 6 months after successful surgery and subsequent radiotherapy, the patient is well and free from recurrences but remains chronically dialysis-dependent.

[Assessment of the peripheral circulation in children with nephrotic syndrome treated with cyclosporin A]. / Ocena zaburzen krazenia obwodowego u dzieci z idiopatycznym zespolem nerczycowym leczonych cyklosporyna A.

Cyclosporin A (CsA) is an immunosuppressive agent used in children for the treatment of steroid-dependent idiopathic nephrotic syndrome (INS). Despite its benefitial effect on a course of the disease CsA may exert nephrotoxic effects because of its vasoconstrictive properties. CsA-dependent disorders of the peripheral flow (Raynaud phenomenon--RP) have been recently described. The aim of the study was to assess the effect of CsA on the peripheral circulation. The study group comprised 16 children (12 male, 4 female; mean age 9.8 +/- 4.5 years) treated with CsA for at least 6 months due to INS (mean treatment time 39 +/- 27 months). Thirteen age- and sex-matched individuals served as controls. Peripheral circulation disorders were evaluated by means of a cold stress test (both hands were held in lukewarm water (20 degrees C) for 1 minute and thereafter the changes in the hand temperature were recorded with thermographic camera (Inframetrics SC1000). RP assessment was performed according to the method described by Ammer and Ring. The temperature gradient of 4 degrees C or greater maintained between metacarpal and peripheral areas of a hand after 10 minutes was considered diagnostic for RP. According to these criteria RP was confirmed in only 3 patients from the study group and in 2 controls. However, the time of the temperature increase in the first 5 minutes after cooling was considerably shorter in the children with INS (0.26 +/- 0.26 degrees C/min vs 0.51 +/- 0.29 degrees C/min, p=0.02). No correlation between CsA serum concentration, CsA dose and impairment of the hand temperature increase was found. The study confirmed that in children suffering from INS treated with CsA peripheral blood flow disorders can be seen. It seems that impaired vessel reactivity may result from the vasoconstrictive effect of CsA.

[Insulinsensitivity and beta-cell function in children with idiopathic nephrotic syndrome]. / Insulinowrazliwosc i czynnosc komórki beta trzustki u dzieci z idiopatycznym zespolem nerczycowym.

Glucose intolerance which is frequently found in patients with the idiopathic nephrotic syndrome (INS) may be linked to an increased cardiovascular risk in these patients. Recently it has been suggested that proteinuria and steroid treatment may independently affect insulin sensitivity. The aim of the study was to assess insulin resistance (IR) and beta-cell function in children with INS at various stages of the disease. The study group comprised 66 children (32 male, 34 female; age 8.1 +/- 5.0 years). 20 healthy, sex- and age-matched subjects served as controls. The study group was divided into 3 subgroups: A (n=24) children in relapse of INS (steroids 60 mg/m2/48h; proteinuria 1.02 +/- 1.04 g/dl); B (n=20) in remission treated with steroids (30 mg/ m2/48h); C (n=22) in remission but without steroids. Fasting glucose and insulin levels were measured to calculate insulin resistance (HOMA-IR) and beta-cell function (HOMA-beta) using Homeostatic Model Assessment. Fasting glucose was within normal range in all subjects. HOMA-IR was significantly higher in group A (3.2 +/- 3.3) and group B (2.4 +/- 1.7) than in group C (1.45 +/- 1.6 and controls (1.12 +/- 0.6) (p<0.05). HOMA-beta was significantly higher in group A and B than in C and controls (p<0.05). In multivariate analysis HOMA-IR correlated with proteinuria (beta=0.45, p<0.001), steroid dose (beta=0.32) and BMI (beta=0.42). In conclusion, an increase in insulin resistance with compensatory enhanced beta-cell secretion is a typical finding in children with INS treated with steroids. Proteinuria seems to be an independent risk factor for decreased insulin sensitivity.

[Hypertension in pregnancy--risk factors, prevention and treatment]. / Nadcisnienie tetnicze u ciezarnych-ocena czynników ryzyka, zapobieganie i leczenie.

The term hypertension in pregnancy stands either for a high blood pressure, which has already developed before pregnancy (i.e., chronic hypertension in pregnancy), or for a pregnancy-associated disease (i.e., pregnancy-induced hypertension). Each form of hypertension may be an isolated phenomenon or constitute a part of the syndrome of preeclampsia or eclampsia. This review focuses mainly on the risk factor assessment, prevention and treatment of hypertension developing during pregnancy. Despite a frequent occurrence of this disease its prevention and treatment is still a subject of debates, and only a limited number of studies, which fulfill the criteria of "evidence-based medicine" have so far been performed in this field. Although the impressive advances in treatment of hypertension in the general population have been done, the choice of drugs and control of hypertension developing during pregnancy is still far from being satisfactory.

[Analysis of renal replacement therapy in patients with renal failure]. / Analiza leczenia nerkozastepczego u chorych ze schylkowa niewydolnoscia nerek.

The article presents the 10-years experience of renal replacement therapy in a single centre. A total of 158 patients were treated in this period. 77 patients (47 F, 30 M, mean age 18.7 +/- 12 yrs) were treated due to chronic renal failure and 81 (35 F, 46 M, mean age 2.1 +/- 1.5 yrs) due to acute failure. 48 (62%) were treated by haemodialysis, 24 (31%) by peritoneal dialysis and 5 (7%) by both methods. Due to the shortage of dialysis units both children and adults were qualified to the dialysis therapy in our centre. Mean age of haemodialysis patients was 22 yrs and of those treated by peritoneal dialysis 13.5 yrs. 7 patients died (9%) and 30 (39%) were transplanted. 15 (19%) were transferred to other centres. The overall mortality was lower than reported by other authors. 4% of patients were tested HBV positive, 13% HCV positive and 9% both HBV and HCV positive and this rates are lower than the average rate in chronic dialysis patients in Poland. Our experience allows us to conclude that adult patients may be successfully treated in paediatric dialysis centres.

[Dialysis-related complications in peritoneal dialysis patients]. / Powiklania dializy otrzewnowej u chorych na ostra i przewlekla niewydolnosc nerek.

The hospital records of 56 patients (25M, 31F) with acute or chronic renal failure treated by peritoneal dialysis were retrospectively reviewed. Mean dialysis time was 13 +/- 15 days in acute renal failure and 32 +/- 23 months in chronic renal failure. The incidence of infectious (exit site infections and peritonitis) and non-infectious dialysis-related complication was assessed. Exit site infections were significantly more frequent in children aged 5 or less than in older patients (1/9.6 patient-month and 1/26.5 patient-month, respectively, p < 0.001). Such relationship was not found with regard to the incidence of peritonitis. There was a tendency of peritonitis rate to decrease in consecutive years was noted. This can be probably related to an increase in the number of patients, introduction of automated peritoneal dialysis, increasing experience of medical staff and patients themselves.

[Pneumoperitoneum and peritonitis in a child treated by continuous ambulatory peritoneal dialysis]. / Zapalenie otrzewnej i odma otrzewnowa u chorej leczonej ciagla ambulatoryjna dializa otrzewnowa.

Pneumoperitoneum is a rare complication of peritoneal dialysis, which in most cases occurs soon after the implantation of the peritoneal catheter and does not need any specific treatment. In contrast, pneumoperitoneum due to visceral perforation represents a serious clinical problem and usually needs an urgent surgical intervention. We present a case of a 10-year old girl treated by peritoneal dialysis for 7 months who was admitted to hospital with symptoms of peritonitis and pneumoperitoneum. On admission her condition was severe and visceral perforation was strongly suspected. As her parents did not give consent to surgical treatment, only conservative management was introduced. Peritoneal dialysis was continued and the girl responded well to antibacterial therapy. The final outcome was favourable.

[Assessment of children at the beginning of renal replacement therapy in Lodz 1990-2000]. / Ocena stanu dzieci rozpoczynajacych leczenie nerkozastepcze w ICZMP w Lodzi w latach 1990-2000.

The time of patients' referral to dialysis predicts the clinical outcome of the therapy and significantly influences the mortality rate. The aim of the study was to assess the clinical and nutritional status and selected biochemical parameters (serum creatinine, urea, bicarbonate, calcium, albumin concentration) at the beginning of renal replacement therapy. We analysed medical history of 46 children (24 boys; 22 girls) aged 1 month-18 years (mean age 13.1 +/- 5.5 years). We divided them into late-referral and early-referral groups. 56% of the children were under nephrological care before the beginning of dialysis treatment. However, in 44% of the cases renal insufficiency had not been diagnosed before. It was found that the children who received no nephrological care in the past demonstrated significantly worse clinical and biochemical status at the beginning of the renal replacement therapy.

Cyclosporine A - treated nephrotic children show impaired vasodilatation but no autonomic neuropathy.

INTRODUCTION: The aim of the study was to determine the effect of long-term cyclosporine A (CsA) treatment in children with idiopathic nephrotic syndrome on autonomic nerve function and peripheral blood flow assessed with thermography. MATERIAL AND METHODS: The study group consisted of 19 nephrotic children treated with cyclosporine A compared to 16 healthy children. The assessment of blood flow was made based on the results of thermographic measurements during the cold stress test by an infrared radiation registering camera. Ewing's battery of non-invasive tests was used to assess autonomic function. RESULTS: The study showed diminished temperature increase after the cooling test in children with nephrotic syndrome treated with CsA. Sympathetic activity test results were comparable to healthy children. CONCLUSIONS: It may be hypothesized that cyclosporine A administered even in low doses induces small vessel dysfunction measured by the thermographic method. This effect seems to be independent of sympathetic nervous system involvement, which was absent in cyclosporine A treated nephrotic children.

Raynaud's phenomenon and endothelial dysfunction in end-stage renal disease patients treated with hemodialysis.

BACKGROUND/AIMS: Steal syndrome is a well-known complication of arteriovenous shunt placement. Increased frequency of Raynaud's phenomenon (RP) especially concerning shunt limb is reported among hemodialysis (HD) patients. The aim of the study was to assess the relation of impairment of peripheral circulation diagnosed with cold stress test (CST) and thermography to the AV shunt location and markers of endothelial dysfunction in HD patients. METHODS: The study group comprised 21 patients (6 male, 15 female, mean age 32.6 +/- 15.0 years) treated with HD for a mean of 69 +/- 54 months. 10 healthy individuals (4 male, 6 female, mean age 38.6 +/- 14.7 years) served as controls. The diagnosis of RP was made upon the results of thermographic measurements during CST. Von Willebrand factor activity and antigen, endothelin-1 and plasma total homocysteine (tHcy) were measured in all subjects. RESULTS: RP was found significantly more often in HD patients than in controls: 11/21 vs. 1/10 (p = 0.04). RP occurred in both hands in 7/11 (64%) patients. tHcy was higher in HD patients than in the controls (31.7 +/- 13.9 vs. 10.9 +/- 3.2 microg/l, p < 0.0001). tHcy and von Willebrand factor antigen were significantly higher in the RP-positive than RP-negative patients or controls. CONCLUSION: Small vessel dysfunction diagnosed as positive RP is a frequent finding in HD patients. It seems that endothelial injury rather than AV shunt steal syndrome is responsible for development of RP in HD patients.

Peritoneal clearance of homocysteine with icodextrin or standard glucose solution exchange.

BACKGROUND: The aim of the study was to assess plasma homocysteine concentration in peritoneal dialysis patients, and to compare the effect of different peritoneal solutions (glucose-based and icodextrin-based) on peritoneal clearance of homocysteine. METHODS: The study group comprised 10 chronic peritoneal dialysis patients; the control group comprised 15 healthy, age-matched non-obese subjects with normal renal function. Patients with vitamin B(12) or folate deficiency were excluded. In all subjects, plasma homocysteine and dialysis adequacy parameters were assessed at baseline. The clearance study was carried out with 2.27% glucose and 7.5% icodextrin solutions (12-h dwell time). RESULTS: Mean dialysate concentration of homocysteine was similar for both glucose and icodextrin solutions (8.3 +/- 3.2 and 8.4 +/- 1.9 micromol/L, respectively), but homocysteine clearance was significantly higher for icodextrin than glucose solution (1.82 +/- 0.57 vs 1.39 +/- 0.53 mL/min per 1.73 m(2)P = 0.01). Net ultrafiltration after icodextrin solution was also higher than after glucose solution (599 +/- 136 mL vs 134 +/- 337 mL, P < 0.01). A correlation between total plasma level of homocysteine and its peritoneal clearance was found (r = 0.69; P = 0.03). CONCLUSION: It appears that peritoneal elimination of homocysteine depends primarily on its plasma concentration. Icodextrin-based solution for peritoneal dialysis seems to be more efficient in homocysteine elimination than a standard glucose-based solution.