Thrombotic complications in children with first-episode steroid-sensitive nephrotic syndrome: A single-center experience.

OBJECTIVE: The aim of the study was to evaluate the clinical course and risk factors of venous thromboembolic complications (VTEC) in children with a first episode of steroid-sensitive nephrotic syndrome (SSNS). MATERIALS AND METHODS: We retrospectively analyzed the medical records of children hospitalized due to SSNS in one pediatric nephrology unit between 2012 and 2019. Demographic data, clinical symptoms at the onset of NS, and laboratory parameters were compared between patients with and without VTEC. RESULTS: Among 106 children (4.7 ± 3.06 years of age) with a first episode of SSNS, 5 VTEC were diagnosed during 2 - 60 days after onset of NS, on the basis of clinical symptoms and/or results of imaging studies. These were thromboses of femoral vein, central part of the kidney, dorsal veins of the hand, venous sinuses of the brain, and superficial vein in the popliteal fossa region. We found significant higher serum fibrinogen level (p = 0.022) and D-dimers (p = 0.0001) in children with VTEC vs. those without VTEC, but AUC analysis showed that only D-dimers significantly differentiate thrombosis. The clinical risk factors of VTEC were vascular cannulation (100%), infections (80%), and diuretics (80%). In children with VTEC, low molecular weight heparin was used. The outcome was a full recovery in all patients. CONCLUSION: VTEC occurs in 4.72% of children with a first episode of SSNS. The course of VTEC in children with SSNS may be asymptomatic. The clinical risk factors of VTEC in children with SSNS are vascular cannulation, infections, and diuretics. High D-dimer levels are a sensitive indicator of thrombosis.

Lactobacillus rhamnosus PL1 and Lactobacillus plantarum PM1 versus placebo as a prophylaxis for recurrence urinary tract infections in children: a study protocol for a randomised controlled trial.

BACKGROUND: Urinary tract infections (UTIs) are one of the most common bacterial infections in children. In children < 7 years of age, the prevalence of one episode of symptomatic UTI has been estimated at 3-7% in girls and 1-2% in boys, whereas 8-30% of them will have one or more episodes of UTI. The use of some probiotics appears to reduce the risk of recurrence of UTIs. Since the effects of probiotics are strain-specific, the efficacy and safety of each strain has to be assessed. The main aim of this study is to determine whether probiotics (containing Lactobacillus rhamnosus PL1 and Lactobacillus plantarum PM1) therapy are effective in preventing UTI in children compared to placebo. METHOD: A superiority, double-blind, randomised, controlled trial is being conducted. One hundred and six patients aged 3 to 18 years with recurrent UTIs in last year (defined as: ≥ 2 episodes of UTI with acute pyelonephritis/upper UTI; or 1 episode of UTI with acute pyelonephritis and ≥ 1 episodes of UTI with cystitis/lower UTI; or ≥ 3 episodes of UTI with cystitis/lower UTI) or children with ≥ 1 infection in the upper urinary tract and ≥ 1 of recurrent UTIs risk factors (congenital anomalies of the kidney and urinary tract, constipation, bladder dysfunction, myelomeningocele, sexual activity in girls) will be randomly assigned to receive a 90-day prophylaxis arm (probiotic containing L. rhamnosus PL1 and L. plantarum PM1) or a 90-day placebo arm. The primary outcome measure will be the frequency of recurrence of UTI during the intervention and in the period 9 months after the intervention. DISCUSSION: The findings of this randomised controlled trial (RCT), whether positive or negative, will contribute to the formulation of further recommendations on prevention of recurrent UTIs in children. TRIAL REGISTRATION NUMBER: NCT03462160, date of trial registration 12th March 2018.

Acute tubulointerstitial nephritis following aciclovir treatment for chickenpox in children with nephrotic syndrome - a report of two cases.

Tubulointerstitial nephritis (TIN) is an inflammatory process primarily involving the renal interstitium and is the cause of acute kidney injury (AKI) in 3-7% of cases confirmed by renal biopsy in children. Aciclovir may have a nephrotoxic effect by crystallization in renal tubules or by inducing an immunologic process that leads to development of TIN. We report 2 male patients, aged 10 and 8 years, with nephrotic syndrome (NS), in whom disease relapse was triggered by varicella zoster infection. The patients received intravenous aciclovir which resulted in AKI due to acute TIN with the glomerular filtration rate 19.5 and 24.9 ml/min/1.73 m2, respectively. The diagnosis was confirmed by kidney biopsy in one of these patients. Initiation of glucocorticosteroids and withdrawal of aciclovir resulted in resolution of proteinuria and symptoms of AKI. In children with active NS treated with intravenous aciclovir, a possibility of AKI due to TIN should be taken into account.

[Cerebral salt wasting syndrome as a complication of subarachnoid hemorrhage in a girl with nephrotic syndrome - a case report]. / Mózgowy zespól utraty soli jako powiklanie krwawienia podpajeczynówkowego u dziewczynki z zespolem nerczycowym ­ opis przypadku.

INTRODUCTION: Cerebral salt wasting syndrome (CSWS), characterized by natriuresis, polyuria, and hypovolemia, is a rare complication of central nervous system injury or disease. A CASE STUDY: 12-year-old girl was admitted with second attack of nephrotic syndrome (NS). On admission she presents with edema, blood pressure 110/60 mm Hg, proteinuria 145 mg/kg/24h, hypoalbuminemia (1.7 g/dL), GFR 94.4 mL/min/1.73m2, sodium 133 mmol/L. On 5th day the patient developed thrombosis of right subclavian and axillary vein and was treated with recombinant tissue plasminogen activator (0.3 mg/kg/h i.v.). 45 minutes after onset of the infusion severe headache appeared. Computed tomography revealed subarachnoid hemorrhage in a region of left occipital lobe and posterior 1/3 part of sickle of the brain. Control ultrasonography examination revealed resolution of the thrombus. No deficits were found on neurologic examination. Proteinuria subsided on 11th day of hospitalization. After the hemorrhage hypovolemia, hypotension (80/40 - 100/60 mm Hg, heart rate 100/min), polyuria, and pathologic natriuresis (up to 13.0 mmol/kg/24h) were observed. Cerebral salt wasting syndrome was recognized. The girl was supplemented with oral and intravenous sodium (up to 10 mmol/ kg/24h). In following days gradual decrease of diuresis and urinary sodium loss was observed. The patient was discharged home after 41 days with normal diuresis (1.5l/24h) and natriuresis (1.44 mmol/kg/24h). CONCLUSIONS: Treatment of thromboembolic complications in children with NS poses a risk of central nervous system bleeding. Serum sodium concentration and diuresis must be strictly monitored in patients with central nervous system lesion, especially in the course of nephrotic syndrome.

Is copeptin a reliable biomarker of primary monosymptomatic nocturnal enuresis?

INTRODUCTION: The position of copeptin (C-terminal fragment of antidiuretin propeptide) as a marker of primary monosymptomatic nocturnal enuresis (PMNE) is under debate, and there are no data on the relation between copeptin and clinical and biochemical parameters in these patients. Aim of the study was to assess the level of serum copeptin in children with PMNE and to look for a relation between copeptin and selected clinical and biochemical parameters in these children. MATERIAL AND METHODS: Twenty-five children recruited for the trial fulfilled the following criteria: clinical diagnosis of PMNE, age 5-15 years, normal creatinine level, normal ultrasonographic image of kidneys and urinary tract. The following parameters were evaluated: serum copeptin, creatinine, sodium, potassium, hematocrit and urine specific gravity. Twenty healthy children were included in the control group. RESULTS: Children from study and control groups did not differ in serum copeptin, sex, age creatinine, sodium, hematocrit and specific gravity. Serum potassium level remained normal in subjects but was significantly higher in the study group. In children with PMNE we found no relation between serum copeptin level and sex, kidney function, sodium, and urinary specific gravity. We found a negative correlation between copeptin and bladder capacity and trends towards positive relations between copeptin and age, as well as hemoglobin. In the subgroup of children with normal bladder capacity a trend towards a positive correlation between copeptin and potassium was found. CONCLUSIONS: Copeptin may be a marker of hydration status in children with PMNE. The relation between potassium and copeptin levels and the clinical significance of the relation require further studies.

[The long-term results of the treatment of renal hypercalciuria - a report of two cases]. / Odlegle wyniki leczenia hiperkalciurii nerkowej ­ prezentacja dwóch przypadków.

In recent years there can be observed a growing number of cases of urolithialis, both in children and in adults. Many pathogenic mechanisms of forming stones have been recognized and there is possible profounder diagnostics. Facing increasing problem of urolithialis in children, it is necessary to seek all possible causes of the disease to prevent forming stones, relapses and its further consequences. In most cases it is possible to diagnose metabolic abnormalities, of which the most frequent is hypercalciuria (excessive excretion of calcium in urine). In children, idiopathic hypercalciuria occurs the most frequently. It is possible to diagnose different types of hypercalciuria (absorptive, renal or resorptive) and apply appropriate treatment. A CASE REPORT: We present two cases of patients with urolithialis and diagnosed renal hypercalciuria. Time of observation of the first patient was more than 6 years, of the second one - 9 years. In both cases there has been applied appropriate treatment, however it was the proper realization of the recommendations that influenced long-term results of the treatment. Even the most detailed examination cannot assure effectiveness if the patient and the parents do not have positive approach to the treatment.

Diagnostic difficulties in a patient with paroxysmal cold haemoglobinuria and acute kidney injury.

Paroxysmal cold haemoglobinuria (PCH) is a form of autoimmune haemolytic anaemia (AIHA) characterised by a sudden onset of haemoglobinuria, either spontaneously or following exposure to cold. In children, it is commonly seen following a viral illness or after immunisation. Diagnosis of PCH is confirmed by a positive Donath Landsteiner (DL) test in which biphasic haemolysins are detected. However, in a real clinical setting, the serological diagnosis of PCH is not always easy. PCH can cause tubular renal injury, which in turn can lead to renal impairment. We describe a case of a two-year-old boy who was admitted to the hospital with pallor, jaundice, dehydration, and dark urine. Two weeks before admission, the child had an upper respiratory tract infection. Laboratory tests showed severe anaemia (haemoglobin 4.5g/dl, haematocrit 11.5%, LDH 8525 U/l), hyperbilirubinaemia (104 µmol/l), haemoglobinuria, and acute kidney injury: GFR 43.9 ml/min/1.73 m2 (grade 2 according to Acute Kidney Injury Network). The direct antiglobulin test was positive for C3c and C3d complement components. The diagnosis of PCH was confirmed by the presence of biphasic antibodies in a DL test on the third day of hospitalisation. The patient received supportive treatment.

Thrombotic thrombocytopenic purpura in the course of systemic lupus erythematosus in a 15-year-old girl.

Systemic lupus erythematosus (SLE) concomitant with thrombotic thrombocytopenic purpura (TTP) in children is rarely diagnosed. We report a case of a 15-year-old girl with butterfly patch, generalized edema, leg pain, anemia (Hb 74 g/l), thrombocytopenia (5 x 109/l), schistocytes in peripheral blood smear, acute kidney injury (eGFR 27 ml/min/1.73 m2), proteinuria, and erythrocyturia. The direct Coombs test was positive, and warm antibodies (IgG) were detected on red blood cells. ANA in titer 1 : 2560, low serum C3 and C4 complement level, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity < 4% and the presence of ADAMTS13 inhibitor were detected. In renal biopsy class IVA/V lupus nephritis was diagnosed. Her clinical symptoms fulfilled criteria for the diagnosis of SLE and the diagnosis of TTP. She was treated with intravenous methylprednisolone and cyclophosphamide 750 mg/1.73 m2/monthly for six months, followed by oral prednisone with azathioprine, chloroquine, and enalapril. The long-term clinical outcome was good. We report a case rare in adolescents, TTP related to SLE, which may suggest the need to test for ADAMTS13 activity and the presence of ADAMTS13 inhibitor in children with SLE and anemia, thrombocytopenia and rapid deterioration of renal function, to make the right therapeutic decisions.

Tuberculosis infection in children with proteinuria/nephrotic syndrome.

Children with nephrotic syndrome (NS) are at greater risk of infections than the general population, due to immunodeficiency in the course of the disease and the treatment. In this study we present 4 children (2 girls, 2 boys), mean age 7.6 ±5.1 years, with NS/proteinuria and latent tuberculosis in 3 children and lymph node tuberculosis in 1 child. The reasons for testing these children for tuberculosis (TB) were the evaluation of the epidemiological status before treatment with corticosteroids (GCS), leukopenia and the relapse of NS, and non-nephrotic proteinuria. The diagnosis of TB infection was based on positive IGRA (Interferon-Gamma Release Assay). Chest X-ray was normal in all the children. Chest CT scan revealed an enlargement of lymph nodes in 1 child. The children were treated with isoniazid (3 children) and isoniazid, rifampicin and pyrazinamide (1 child). Three children with idiopathic nephrotic syndrome were treated with prednisone. The child with non-nephrotic proteinuria was treated with enalapril. Proteinuria disappeared in all children during anti-TB treatment.

Lupus nephritis in children - 10 years' experience.

Systemic lupus erythematosus (SLE) in children is usually more severe than it is in adults and there is a higher incidence of renal involvement. We described 18 children (16 girls, 2 boys) with lupus nephritis (LN), whose average age was 14.4 ±1.81 years. Disease activity was assessed according to SLEDAI (SLE Disease Activity Index). Renal biopsy was classified according to the INS/RPS (International Society of Nephrology/Renal Pathology Society). The patients were treated with steroids (100%) and pulses of cyclophosphamide (88.9%) or mycophenolate mofetil (11.1%), next azathioprine or mycophenolate mofetil with prednisone in reduced doses. In children with renal/multi-organ insufficiency and/or septicaemia, renal replacement therapy (27.8%), and plasmapheresis (22.2%) were used in the initial treatment. The SLEDAI initial activity was high in 44.4% and moderate in 55.6% of children. LN manifested as: nephrotic syndrome (83.3%), microhaematuria (100%), leukocyturia (60%), hypertension (72.2%), and acute renal injury (83.3%); mean GFR was 54.55 ±33.09 ml/min/1.73 m2. In the renal biopsy, class IV LN according to INS/RPS was mainly diagnosed (82%). At the end of follow-up, mean observation time 32.1±23.36 months: mean GFR was 90.87 ±12.13 ml/min/1.73 m2, proteinuria disappeared in 66.7% and decreased in 33.3% of children to the average of 1.7 g/day (range: 0.5-4.0 g/day), hypertension was observed in 83.4% of children. Intensive immunosuppressive treatment with pulses of cyclophosphamide in early stage of LN in children is very effective.

Hemodiafiltration efficacy in treatment of methanol and ethylene glycol poisoning in a 2-year-old girl.

INTRODUCTION: Every year about 2.4 million people in USA are exposed to toxic substances. Many of them are children below 6 years of age. Majority of poisonings in children are incidental and related to household products including for example drugs, cleaning products or antifreeze products. Antifreeze solutions contain ethylene glycol and methanol. Treatment of these toxic substances involves ethanol administration, fomepizole, hemodialysis and correction of metabolic acidosis. PURPOSE: The aim of the study was to check the efficacy of continuous venovenous hemodiagiltration in intoxication with ethylene glycol and methanol. MATERIAL AND METHODS: One year and 7 months old girl after intoxication with ethylene glycol and methanol was treated with continuous venovenous hemodiafiltration instead of hemodialysis because of technical problems (circulatory instability). RESULTS: Intravenous ethanol infusion with hemodialtration resulted in rapid elimination of methanol from the body and significantly reduced blood ethylene glycol level. CONCLUSIONS: Continuous venovenous hemodiafiltration can be helpful in treatment of ethylene glycol and methanol intoxication.

Mycoplasma pneumoniae as a trigger for Henoch-Schönlein purpura in children.

Mycoplasma pneumoniae is one of the most common causes of respiratory tract infections in children. Extrapulmonary manifestations are seen in up to 25% of infected patients. Extrapulmonary complications are associated with the central nervous system, gastrointestinal tract, skin changes, myocarditis, pericarditis, hemolytic anemia, thrombocytopenia and thrombosis. The majority of extrapulmonary symptoms are associated with skin changes such as exanthematous skin eruptions, erythema nodosum, urticaria, Stevens-Jonson syndrome. M. pneumoniae stimulates production of the interleukins and tumor necrosis factor (TNF) α and can cause vasculitis. Henoch-Schönlein purpura (HSP) is a leucoclastic vasculitis that affects small vessels. Clinical manifestations of HSP include typical rash, arthritis, gastrointestinal and sometimes renal involvement. The main feature in HSP is abnormal IgA deposits in vessel walls. Circulating abnormal glycosylated IgA 1 and IgG antibodies form immune complexes: IgA1-IgG and anti-IgA 1. Immune complexes activate cytokines, parts of complement and influence directly the endothelium. We report cases of three children with Henoch-Schönlein purpura with prolonged and recurrent skin and joint changes. The serological analysis (positive serum IgM) confirmed Mycoplasma pneumoniae infection. Treatment with clarithromycin caused complete regression of disease. We suggest that in the case of prolonged symptoms of vasculitis due to Henoch-Schönlein purpura, Mycoplasma pneumonia infection may be a potential cause of exacerbation of the disease.

Lactobacillus rhamnosus PL1 and Lactobacillus plantarum PM1 versus Placebo as Prophylaxis for Recurrence of Urinary Tract Infections in Children.

Urinary tract infections (UTIs) rank among the most prevalent bacterial infections in children. Probiotics appear to reduce the risk of recurrence of UTIs. This study aimed to evaluate whether probiotics containing Lactobacillus rhamnosus PL1 and Lactobacillus plantarum PM1 therapy prevent UTIs in the pediatric population compared to a placebo. A superiority, double-blind, randomized, controlled trial was conducted. In total, 54 children aged 3-18 years with recurrent UTIs or ≥one acute pyelonephritis and ≥one risk factor of recurrence of UTIs were randomly assigned (27 patients in each arm) to a 90-day probiotic or placebo arm. The age, sex, diagnosis, renal function, risk factors, and etiology of UTIs did not vary between the groups. During the intervention, 26% of children taking the probiotic had episodes of UTI, and it was not significantly less than in the placebo group. The number of UTI episodes during the intervention and the follow-up period decreased significantly in both groups, but the difference between them was insignificant. We observed a decrease in UTIs during the study of almost 50% in the probiotic group compared to the placebo group. Probiotics can be used as natural, safe prophylaxis for children with risk factors for UTIs in whom antibiotic prevention is not indicated.

[Steroid-resistant nephrotic syndrome complicated with severe Streptococcus pneumonlae peritonitis in a 10-year-old girl--case report]. / Sterydooporny zespól nerczycowy powiklany ciezkim zapaleniem otrzewnej o etiologii Streptococcus pneumoniae u 10-letniej dzlewczynkl--opis przypadku.

Primary bacterial peritonitis is a rare complication of idiopathic nephrotic syndrome (INS) in children, found in 1.5-3.7% cases. The 10-year-old girl was admitted with INS relapse: generalized edema, proteinuria 630 mg/kg/24 h, hypoalbuminemia 1.8 g/dL, hypogammaglobulinemia 74.0 mg/dL (n: 618-1537 mg/dL), GFR 71.6 mL/min/1.73 m2. She was treated with prednisone 60 mg/24 h. On 5th day severe pain, fever, CRP (15.5 mg/dL) and leukocytosis (19.5 tys/mm3) rise occurred. On 6th day due to suspicion of peritonitis, laparotomy was performed and 400 mL of suppurative exudate was evacuated (Streptococcus pneumoniae was cultured). Postoperative course was complicated with acute kidney injury (GFR 47.7 mL/min/ 1.73 m2), lung edema, arterial hypertension, and separation of the layers of a surgical wound. The patient was treated with: imipenem (9 days), vancomycine i.v. (4 days)/p.o. (11 days) (Clostridium difficile toxin present in stool), fluconazole (14 days), 20% albumins, furosemide, labetalole, cyclosporine A (started on 56th day after the operation due to secondary steroid-resistance of INS). The remission was achieved after 7 days of cyclosporine A treatment. Authors suggest that children with nephrotic syndrome belong to high-risk group of invasive pneumococcal disease, therefore they require careful implementation of mandatory immunization schedule. Peritonitis is a rare and still dangerous infectious complication of nephrotic syndrome in children.

Epidemiology and Risk Factors of UTIs in Children-A Single-Center Observation.

Urinary tract infections (UTIs) are one of childhood's most common bacterial infections. The study aimed to determine the clinical symptoms, laboratory tests, risk factors, and etiology of different UTIs in children admitted to pediatric hospitals for three years. Methods: Patients with positive urine cultures diagnosed with acute pyelonephritis (APN) or cystitis (CYS) were analyzed for clinical symptoms, laboratory tests, risk factors, and etiology, depending on their age and sex. Results: We studied 948 children with UTIs (531 girls and 417 boys), with a median age of 12 (IQR 5−48 months). A total of 789 children had clinical symptoms; the main symptom was fever (63.4% of patients). Specific symptoms of UTIs were presented only in 16.3% of patients. Children with APN had shown significantly more frequent loss of appetite, vomiting, lethargy, seizures, and less frequent dysuria and haematuria than children with CYS. We found significantly higher median WBC, CRP, and leukocyturia in children with APN than with CYS. The risk factors of UTIs were presented in 46.6% of patients, of which 35.6% were children with APN and 61.7% with CYS. The main risk factor was CAKUT, more frequently diagnosed in children with CYS than APN, mainly in children <2 years. The most commonly isolated bacteria were Escherichia coli (74%). There was a higher percentage of urine samples with E. coli in girls than in boys. Other bacteria found were Klebsiella species, Pseudomonas aeruginosa, Proteus mirabilis, and Enterococcus species. Conclusions: Patients with APN were younger and had higher inflammatory markers. Often, fever is the only symptom of UTI in children, and other clinical signs are usually non-specific. The most common UTI etiology is E. coli, regardless of the clinical presentation and risk factors.

Retrospective analysis of clinical and pathomorphological features of lupus nephritis in children.

PURPOSE: The aim of the study was to evaluate the clinical course and pathomorphological correlations in Polish children with the diagnosis of lupus nephritis (LN). METHODS: We retrospectively analyzed the medical records of 39 children hospitalized due to LN in 7 pediatric nephrology units in Poland between 2010 and 2019. Demographic data, clinical symptoms at the onset of LN and laboratory parameters were reviewed. We analyzed Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), histological LN findings with the activity (IA) and chronicity index (IC). RESULTS: We examined 32 girls and 7 boys, median age at LN onset was 14.75 (IQR 13.0-16.0) years, SLEDAI of 22.0 (IQR 18.0-27.0) points; LN histological class: IV (59.4%), III (18.9%), III/V (10.8%), IV/V (8.1%), VI (2.7%); IA 8.0 (IQR 6.0-11.0) points, IC 1.05 (IQR 0-2.0) points. Children with nephrotic (n â€‹= â€‹22) and non-nephrotic (n â€‹= â€‹17) proteinuria differed in median Hb level (9.55, IQR 8.3-11.2 vs 10.9, IQR 10.1-11.6 â€‹g/L; P â€‹< â€‹0.05), albumin level (2.5, IQR 2.1-3.19 vs 3.6, IQR 3.4-4.1 â€‹g/dL; P â€‹< â€‹0.001), proteinuria (5.76, IQR 3.0-7.5 vs 1.08, IQR 0.53-1.50 â€‹g/day; P â€‹< â€‹0.0001), eGFR (53.9, IQR 27.0-68.8 vs 96.7, IQR 73.8-106.2 â€‹mL/min/1.73 â€‹m2; P â€‹< â€‹0.01) and occurrence of hypertension (77% vs 23%; P â€‹< â€‹0.01). In multivariate analysis Hb level (ߠ​= â€‹8.0; 95%CI, 1.90-14.11) was the significant predictor of eGFR<90 â€‹mL/min/1.73 â€‹m2. CONCLUSIONS: Proliferative forms of LN in children may have a varying clinical presentation. Children with LN with nephrotic range proteinuria have lower Hb level, lower eGFR and higher occurrence of hypertension. Hb level is the significant predictor of eGFR<90 â€‹mL/min/1.73 â€‹m2 in children with LN.

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.

[Long-term results of treatment of vesicoureteral refluxes]. / Odlegle wyniki leczenia odplywów pecherzowo-moczowodowych.

AIM OF THE STUDY: To estimate the results of treatment of vesicoureteral refluxes (VUR) in children. 156 children were involved into the study. 68 children aged 10 years and above (mean age 13.1 +/- 2.4 years) were analysed in detail. The patients were divided into groups depending on method of treatment: surgical (26 children), transurethral injection of teflon/collagen (11 children), conservative (31 children). The results of treatment were evaluated on basis of subsidence of VUR, kidney function, renal scarring on DMSA scan and arterial hypertension. RESULTS: Persistence of VUR was observed in 6 patients treated conservatively (19.4%), 5 of them had voiding disturbances, in 1 patient treated with injections (9.1%) and in no operated patients. There was no significant difference between groups in kidney function and in presence of hypertension. Operated patients had significantly higher grade of VUR and grade of renal scarring on DMSA scan. CONCLUSION: Method of treatment of vesicoureteral refluxes does not influence kidney function or occurence of hypertension.

[Voiding dysfunction in children with vesicoureteral reflux]. / Zaburzenia funkcji pecherza moczowego u dzieci z odplywami pecherzowo-moczowodowymi.

UNLABELLED: The aim of the study was to assess the rate of development of voiding dysfunction (VD) in patients with reflux nephropathy. We investigated 150 pts with vesicoureteral reflux (VUR): 33 boys and 117 girls in mean age 9 +/- 3.3 years with normal renal function. In all pts we performed renal scintigraphy (DMSA) and ABPM. Renal scarring by DMSA scan was categorized into grades 1-4. In all pts we assessed high and laterality of VUR, renal function (clearance creatinine--Ccr) and occurency of hypertension (HT). RESULTS: Of 150 pts, 80 (53.3%) developed VD (54.5% boys and 52% girls). DMSA grade: 1--27 pts, 2--85 pts; 3--31 pts; 4--7 pts. No statistically significant difference was found depends of: sex, age, laterality of renal damage in DMSA, unilateral and bilateral reflux for the development of VD. The incidence 3-4 grade in DMSA scan was significantly higher in pts with IV-V grade of VUR in comparison to pts with lower grade of VUR in group with and without voiding dysfunction. IV-V grades of VUR were observed more frequently in pts with bladder/sphincter dysfunction. The occurency of HT in group with VD and without VD was 12 pts (15%) and 8 (11.4%) respectively (ns). Pts with VD have lower Ccr in comparison with pts without VD (129.1 vs 136.3 ml/min/1.73 m2; p<0.05). CONCLUSIONS: Approximately 50% of patients with VUR demonstrated VD. VD occurred independent of age. IV-V degrees VUR and more severe changes in DMSA scan were seen in pts with bladder/sphincter dysfunction.

[Tubulointerstitial nephritis with uveitis (TINU) syndrome]. / Sródmiazszowe zapalenie nerek z zapaleniem blony naczyniowej oka (TINU).

Authors present an 11-year old girl with non-specific clinical symptoms with increase of inflammatory indices and acute non-oliguric renal failure: creatinine clearance - 55 ml/min/1.73 m2. An anterior uveitis was observed after 7 weeks of illness. Diagnosis of tubulointerstitial nephritis with uveitis (TINU) was confirmed by interstitial infiltrate (lymphocytes, neutrophils, eosinophils) and interstitial fibrosis in renal biopsy. Steroid therapy was given for 7 months. Uveitis without kidney involvement relapsed 1 month after discontinuation of steroid therapy. The full recovery of uveitis was achieved after the treatment with topical steroids. After 3 years, the illness remains still in remission.