Health-related quality of life in children with immunoglobulin A nephropathy - results of a multicentre national study.

INTRODUCTION: Immunoglobulin A nephropathy (IgAN) may lead to end stage renal disease and severely affect patient functioning and wellbeing. The aim of the study was to evaluate health-related quality of life (HRQoL) in children and adolescents with IgAN, and compare HRQoL in relation to the disease course, social status and psychological factors, such as expressing anger and perceived personal competence. MATERIAL AND METHODS: The multicentre cross-sectional study included 51 patients ≥ 8 years from 7 paediatric nephrology centres in Poland. Psychometric analysis was performed using the Kidscreen-52 questionnaire to evaluate HRQoL, the Anger Expression Scale to evaluate the severity of anger and the Personal Competence Scale to measure general perception of personal competence. RESULTS: Mean age of patients was 14.54 ±3.69 years; duration since the diagnosis of IgAN was 4.98 ±3.9 years. Patients with IgAN rated their psychological wellbeing as significantly worse compared to healthy peers (p < 0.05). The presence of proteinuria was associated with significantly worse physical wellbeing (58.72 ±18.45 vs. 74.44 ±22.97; p < 0.05). Current therapy (steroids/immunosuppressive drugs) had no effect on HRQoL in the study group. Perceived personal competence was rated high by 49% of children in the study group. Children with IgAN were characterized by lower intensity of expressed anger (p < 0.001) and significantly higher intensity of suppressed anger (p < 0.01) compared to reference ranges. Severity of expressed anger correlated positively with the parent relations and school environment dimensions of HRQoL. CONCLUSIONS: We found lower HRQoL in regard to physical and psychological wellbeing in a group of Polish children with IgAN compared to healthy peers. HRQoL should be monitored in this patient group.

Life activity, disease acceptance and quality of life in patients treated with renal replacement therapy since childhood.

BACKGROUND: Advances in the treatment of chronic kidney disease (CKD) resulted in expanding therapy goals from simple prolongation of life to a return to normal social functioning and having an active and satisfactory life after reaching adulthood. OBJECTIVES: The aim of the study was to evaluate life activity, disease acceptance (DA) and quality of life (QOL) in patients with end-stage renal disease (ESRD) treated with renal replacement therapy (RRT) since childhood. MATERIAL AND METHODS: We surveyed 117 patients aged .16 years on RRT since childhood. The control group included 25 healthy subjects. We used questionnaires that included a sociodemographic questionnaire (questions regarding education, work, family, and offspring), Acceptance of Illness Scale (AIS), Satisfaction With Life Scale, and Kidney Disease Quality of Life (KDQOL). RESULTS: A completed survey was returned by 45 respondents aged 27.16 }6.78 years, among whom 82.2% had a transplanted kidney and 17.8% were on hemodialysis (HD). Higher education was reported by 18.18% of respondents, secondary and primary by 63.64% and 18.18%, respectively. Employment was reported by 46.67% of the respondents. A family was started by 35% of women and 4% of men. Good DA was found in 28.9% of the respondents. Satisfaction with life was lower in the study group compared to the control group. We found statistically significant correlations between the age when the kidney disease was diagnosed and satisfaction with life (r = 0.33), and between the time since the last change of RRT modality and emotional well-being (r = 0.34). The number of kidney transplantations correlated negatively with emotional component of QOL (r = .0.66) and emotional well-being (r = .0.73). CONCLUSIONS: Patients treated with RRT were quite well adapted to their chronic disease but showed less ability to live independently. Young age at the diagnosis of kidney disease, loss of kidney transplant and living on social security benefit had a negative effect on their emotional well-being.

Twenty years of growth hormone treatment in dialyzed children in Poland-Results of national multicenter study.

PURPOSE: The aim of the study was to analyze the effect of recombinant human growth hormone (rhGH) therapy and to establish factors influencing growth rate in dialyzed children in Poland. METHODS: We retrospectively analyzed medical records of 81 children with end-stage renal disease (ESRD) on chronic dialysis treated with rhGH for ≥12 months between 1994 and 2014. The following data were recorded: cause of ESRD, dialysis modality, age at the dialysis and rhGH initiation [years]. In addition, growth [cm], [standard deviation score - SDS], body mass index [SDS], skeletal age [years], bone mineral density [SDS], hemoglobin, total protein, albumin, urea, creatinine, calcium, phosphorus, calcium phosphorus product, PTH, and alkaline phosphatase were measured at the baseline and after 12 months. RESULTS: Growth velocity in 81 children during one-year rhGH treatment was 7.33 ± 2.63 cm (ΔSDS 0.36 ± 0.43). Height SDS increased significantly (-3.31 ± 1.12 vs. -2.94 ± 1.15, p < 0.001). Children on peritoneal dialysis (PD) (n = 51) were younger than children on hemodialysis (HD) (n = 30) (9.92 ± 3.72 vs. 12.32 ± 3.11 years, p = 0.003). ΔSDS did not differ between PD and HD children (0.40 ± 0.33 vs. 0.30 ± 0.47, p = 0.311). Growth velocity (ΔSDS) correlated with age at dialysis initiation (r=-0.30, p = 0.009), age at rhGH treatment initiation (r=-0.35, p = 0.002), skeletal age (r=-0.36, p = 0.002), BMI SDS (r=-0.27, p = 0.019), and PTH (r=-0.27, p = 0.017). No correlation between growth velocity and other parameters was observed. CONCLUSIONS: Treatment with rhGH in children with ESRD is effective and safe irrespective of dialysis modality. Early initiation of rhGH therapy is a crucial factor determining response to the treatment in children with ESRD.

Neutrophil Gelatinase-Associated Lipocalin: A Biomarker for Early Diagnosis of Urinary Tract Infections in Infants.

Early diagnosis of urinary tract infection (UTI) is challenging in infants due to unspecific symptoms, difficulty in urine collection and possible contamination. The aim of this study was to assesses the usefulness of serum and urine neutrophil gelatinase-associated lipocalin (sNGAL and uNGAL, respectively) in the diagnosis of febrile and non-febrile UTI in infants. This prospective observational study enrolled 66 infants with the first episode of UTI and 18 healthy controls. At the time of enrollment, sNGAL, uNGAL, urinalysis, urine culture, white blood cell count (WBC), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), procalcitonin (PCT), and serum creatinine (sCr) were assessed. We found that, on average, both sNGAL and uNGAL levels were significantly higher in febrile UTI, compared to non-febrile UTI and controls. In turn, the mean sNGAL level, but not uNGAL, was significantly higher in the non-febrile UTI group compared to controls. sNGAL positively correlated with WBC, CRP, ESR and PCT, and uNGAL with CRP and leukocyturia. The receiver operating curves (ROC) demonstrate that the optimum cut-off of 76.2 ng/ml for sNGAL (sensitivity 92.9%, specificity 94.4%, and the area under the curve (AUC) of 0.98) and of 42.2 ng/ml for uNGAL (sensitivity 73.8%, specificity 72.2%, and AUC of 0.76) for diagnosing febrile UTI and 39.0 ng/ml for sNGAL (sensitivity 83.3%, specificity 55.6%, and AUC of 0.70) for diagnosing non-febrile UTI. In conclusion, serum NGAL is an excellent marker for the early diagnosis of febrile UTI, with sensitivity and specificity higher than those of urine NGAL. Diagnostic sensitivity of serum NGAL is smaller in non-febrile infants suffering from UTI, and urine NGAL is not useful for this purpose at all.

Long-term outcomes in children with chronic kidney disease stage 5 over the last 40 years.

INTRODUCTION: We evaluated outcomes in children with chronic kidney disease stage 5 (CKD 5) treated in the first pediatric dialysis unit in Poland during 1973-2012. MATERIAL AND METHODS: The retrospective analysis included 208 children with CKD 5 undergoing renal replacement therapy (RRT), stratified into four decades of treatment: 1973-1982, 1983-1992, 1993-2002, and 2003-2012. RESULTS: The most common causes of CKD 5 included glomerulonephritis in 27.4% and pyelonephritis secondary to urinary tract anomalies in 25.5% of children. Among 208 children, 172 (82.7%) survived and 17.3% died. Kidney transplantation (KTx) was performed in 47.6% of children, including pre-emptive KTx in 1.92% of children. Chronic dialysis was continued in 34.1% of children, and RRT was withdrawn in 1%. The overall mortality rate was 6.2 per 100 patient-years, and 3-year survival was 83.9%. The highest mortality rate of 23.4 per 100 patient-years was observed among children in whom RRT was initiated in 1973-1982, with subsequent reduction of the mortality rate to 4.5 and 2.1 per 100 patient-years in 1993-2002 and 1983-1992 respectively. No deaths were noted after 2002. Cardiovascular problems were the most common cause of death, found in 36.1% of patients (p < 0.01). Identified risk factors for mortality included young age, low residual diuresis, anemia at the time of RRT initiation, and hypertriglyceridemia and hypoalbuminemia during RRT. CONCLUSIONS: In years 1973-2012 significant improvement in prognosis among children with CKD 5 was achieved. Identified predictors of mortality included young age at initiation of RRT, low residual diuresis, anemia and hypertriglyceridemia.

Markers of bone metabolism in children with nephrotic syndrome treated with corticosteroids.

The aim of the study was to assess bone mineral density, bone metabolism markers, and vitamin D level in children with idiopathic nephrotic syndrome in the course of 1-year observation. Twenty five children with nephrotic syndrome aged 5-17 years were enrolled into the study. The median number of relapses was 6 (range 1-22). All patients were treated with prednisone and vitamin D (800 IU/day). Bone mineral density of total body (TB-BMD) and lumbar spine (L-BMD), evaluated by dual energy X-ray absorptiometry (DXA) expressed as Z-score, and serum calcium, phosphorus, parathormone (iPTH), alkaline phosphatase (ALP), bone alkaline phosphatase (BAP), osteocalcin (OC), albumin, creatinine, 25(OH)D3, 1,25(OH)2D3 and urine calcium/creatinine ratio (uCa/Cr) were evaluated at the enrollment visit and after 1 year of therapy. After 1 year significant decreases of TB-BMD Z-score (from -0.24±1.34 to -0.74±1.31, p<0.05) and 25(OH)D3 serum level (from 31.7±16.3 to 23.7±9.3; p<0.05) were observed. No other appreciable differences were found. At the study onset, negative correlations were found between L-BMD Z-score and serum ALP, BAP, and phosphorus and between TB-BMD Z-score and urine uCa/Cr. After 1 year, L-BMD Z-score correlated negatively with serum BAP and OC, and positively with serum 25(OH)D3. Multivariate analysis showed that BAP was the strongest predictor of L-BMD Z-score (beta=-0.49; p<0.05). We conclude that children with nephrotic syndrome treated with corticosteroids are at risk of bone mass loss. Serum BAP concentration seems to be a good indicator of spongy bone metabolism in these children, who should be supplemented with vitamin D in an adjustable dose, possibly higher than 800 IU/24 h to prevent osteopenia.

BONE MINERAL DENSITY IN CHILDREN WITH IDIOPATHIC HYPERCALCIURIA.

UNLABELLED: The aim of the study was to evaluate bone mineral density (BMD) in the lumbar spine in children with idiopathic hypercalciuria. PATIENTS AND METHODS: The study group included 31 children (14 boys, 17 girls) aged 5 to 17 years (mean age 9.8 ± 4.0 years) with idiopathic hypercalciuria. All children remained on normal calcium diet, without vitamin D and citrate supplementation. We evaluated lumbar spine (L1-L4) BMD (L1-L4 BMD) (expressed as Z-score) and blood serum levels of 25-hydroxyvitamin D3 (250HD3), calcium (Ca), phosphorus (P), alkaline phosphatase (ALP), and intact parathormone (iPTH). We also evaluated 24-hour urinary Ca, P, and sodium (Na) excretion. RESULTS: Reduced L1-L4 BMD Z-score <-1 was found in 25.8% of children, Z-score values from -1 to 1 in 64.5% of children, and Z-score > 1 in 9.7% of children. Reduced 250HD3 level (< 20 ng/mL) was found in 71% of children, levels in the range of 20-30 ng/mL in 22.6% of children, and levels > 30 ng/mL in 6.4% of children. Seven out of 8 children with L1-L4 BMD Z-score <-1 were found to have 250HD3 deficiency (level < 20 ng/mL). Among children with reduced lumbar spine BMD, most were girls at the mean age of 13.8 years. Ca and P levels were normal in all children. We did not find significant differences in 25OHD3, Ca, and P levels in relation to gender and age. We found a positive correlation between L1-L4 BMD Z-score and serum 250HD3 level. Concomitant nephrolithiasis was found in 50% of patients with reduced lumbar spine BMD. CONCLUSIONS: Reduced lumbar spine BMD in patients with idiopathic hypercalciuria seems to be related to vitamin D3 deficiency.

[Congenital laryngeal stridor - an interdisciplinary problem]. / Wrodzony stridor krtaniowy­ problem interdyscyplinarny.

UNLABELLED: The most common causes of laryngeal stridor are laryngomalacia (60%), vocal cord paralysis (VCP) (10 %) and subglottic laryngeal stenosis. Majority of cases of VCP are idiopathic, less frequently it is the effect of abnormalities in central nervous system (Arnold - Chiari syndrome, hydrocephalus, neonatal hypoxia). Differential diagnosis should also include anomalies of aortic arch and its branches (vascular rings). The authors present two cases of neonatal congenital laryngeal stridor. In the first case the girl presented with VCP of unknown etiology. The perinatal period was normal, ultrasound of central nervous system and neurologic examination revealed no abnormalities. Due to sustained VCP in control laryngeal ultrasound examinations, tracheostomy was performed in the third month of life. In the second case, stridor was caused by laryngomalacia and subglottic laryngeal stenosis of first grade according to Meyer-Cotton scale (larynx lumen diameter <4 mm). The diagnosis was established by laryngotracheobronchoscopy. In both children angiotomography was performed and vascular ring was diagnosed (aberrant right subclavian artery). Vascular anomaly was suspected in barium X-ray. In both cases echocardiographic examination did not visualize the fourth vessel of the aortic arch. Chest X- rays were normal. Both children had no symptoms caused by vascular ring. CONCLUSION: Diagnosis of congenital laryngeal stridor is an indication for complete evaluation to establish the cause of the airway obstruction. The differential diagnosis should include laryngomalacia, vocal cord paralysis, subglottic laryngeal stenosis, congenital anomalies of large vessels and abnormalities of the central nervous system.

[Vitamin D status in children with renal diseases]. / Zasoby witaminy D u dzieci chorobami nerek.

AIM: Assessment of vitamin D status in children with selected renal diseases based on serum 25OHD3 level taking into consideration type of kidney disease, vitamin D supplementation and season of the year. MATERIAL AND METHODS: Retrospective analysis of 127 children aged from 0.6 to 17.7 years, median 9.5 [24 with risk status of urolithiasis (RSU), 29 with urolithiasis, 15 with glomerulonephritis, 59 with idiopathic nephrotic syndrome (INS)] was performed. Serum concentrations of 25OHD3, 1,25(OH)2 D3, calcium, and phosphorus were measured. The correlation of 25OHD3 with the type of kidney disease, supplementation of vitamin D, seasons, gender, age, and the dose of glucocorticosteroids (in children with glomerulopathies) were analyzed. RESULTS: In all children serum concentration of 25OHD3 from 4.3 to 72.6 ng/mL (median 21.1 ng/mL). The deficiency or insufficiency of vitamin D were observed in 55 (43.3%) analyzed children with kidney diseases. The deficiency or insufficiency of vitamin D were observed in 49.1% children with RSU and urolithiasis, and in 39.2% with glomerulonephritis and INS: in 36% children supplemented with vitamin D and 54% not supplemented (NS). In winter, 25OHD3 serum concentration was significantly higher in children supplemented with vitamin D compared to not supplemented [median 21.5 ng/mL vs 16.5 ng/mL (p<0.05)]. There were no significant differences in serum concentrations of 25OHD3 , 1,25(OH)2 D3 , calcium, phosphorus and calcium x phophorus product depending on type of kidney disease and gender. The significant negative correlation was found between 25OHD3 and patients' age (r=-0.26 p<0.01) and between calcium (r=0.31, p<0.05) and calcium x phosphorus in children supplemented with vitamin D (r=0.28, p<0.05). CONCLUSIONS: Our results suggest the necessity to develop new strategies in vitamin D supplementation in children with kidney diseases. Futher studies shoud also be performed to evaluate their efficiency.

[Glomerulopathies as causes of end-stage renal disease in children]. / Klebuszkowe choroby nerek jako przyczyna schylkowej niewydolnosci nerek.

UNLABELLED: The aim of the study was assessment of the frequency of glomerular diseases (GD) as a cause of end-stage renal disease (ESRD) in children. PATIENTS AND METHODS: Retrospectively, causes of ESRD, frequency, duration of the disease until diagnosis of ESRD in 195 children treated in 1973-2008 were analysed. RESULTS: Among children with ESRD, GD were diagnosed in 94 (48.2%)--mean age 10.9 +/- 4.3 years, congenital abnormalities of urinary tract in 61 (31.2%) aged 10.6 +/- 4.3 years and other causes in 40 (20.5%). Among 94 children with GD the 49 (52%) patients had primary glomerulonephritis (GN): 24--crescentic GN, 9--focal segmental glomerulosclerosis, 8--mesangioproliferative GN, 5--IgA nephropathy, 3--membranoproliferative GN. In 37 (39.5%) the causes of ESRD were secondary glomerulopathies: 20--amyloidosis, 10--hemolytic-uremic syndrome, 6--Schönlein-Henoch nephropathy, 1--Wegener granulomatosis. In 8 (8.5%) patients causes of ESRD were: Alport syndrome in 2, congenital nephrotic syndrome in 1 and in 5 type of glomerulopathy was unknown. Time between diagnosis of nephropathy and start of dialysis was 0-13.75 years, median 1.1 in patients with GD and was significantly shorter (p<0.003) than this time in the group with congenital abnormalities of the urinary tract (0-14.9 years; median 3.83). There was no difference between primary GN and secondary glomerulopathies. CONCLUSIONS: Chronic glomerulopathies were the most frequent cause of ESRD in investigated group. Time between diagnosis of chronic renal disease and initiation of renal replacement therapy was significantly shorter in children with glomerulopathies than with congenital abnormalities of urinary tract.

[Severe course of typical hemolytic-uremic syndrome in a 14-year-old boy]. / Ciezki przebieg typowego zespolu hemolityczno-mocznicowego u 14-letniego chlopca.

The authors present a 14-year old boy with acute renal failure in the course of hemolytic-uremic syndrome (HUS), preceded by bloody diarrhea of unknown origin. The course of HUS was complicated with hypertensive crisis, pleural effusion. Pleural puncture was complicated with massive hemorrhage which required thoracotomy. Additional risk factor were subendocardial perfusion disorders found in MRI scan of the heart and peripheral peroneal nerve palsy (in neuro-motorical conduction examination--severe neuropathy). Renal replacement therapy was necessary for 11 days (hemodialyses--3 days, continuous hemodiafiltration--9 days). Transfusions of: 3000 mL of packed erythrocyte mass, 2700 mL of fresh frozen plasma, 1000 mL of packed platelet mass were performed. Full parenteric nutrition was needed for 11 days. Full recovery of renal function, gradual improvement of heart muscle function, regression of lung abnormalities have been obtained.

[Fetuin A in children with nephrotic syndrome]. / Fetuina A u dzieci z zespolem nerczycowym.

AIM OF THE STUDY: To estimate concentration of fetuin A in children with nephrotic syndrome (NS). MATERIAL AND METHODS: 53 children with NS aged from 2.5 to 17.2 years (mean age 7.7 +/- 4.4 years), 27 in remission of NS, 26 in relapse of NS were involved into the study. The control group consisted of 22 healthy children, aged from 6.5 to 17.5 years, mean age 10.8 +/- 3.3 years. In all children serum concentration of fetuin A, calcium, phosphorus, total cholesterol, albumin, triglycerides, and total daily urine protein loss were measured. RESULTS: The concentration of fetuin A in children in relapse was significantly lower compared to children in remission and control group (65.9 +/- 28; 87.2 +/- 23.2; 101.9 +/- 11.6 ng/mL, respectively, p < 0.0001). Significant correlation was found between concentrations of fetuin A and albumin level (r = 0.36, p < 0.05) and calcium concentration (r = 0.30, p < 0.05) and negative correlation between concentration of fetuin A and daily proteinuria (r = -0.36, p < 0.05). CONCLUSION: Low concentration of fetuin A in children in relapse of NS it may depends on proteinuria.