Clinical profile of a Polish cohort of children and young adults with cystinuria.

BACKGROUND: Cystinuria is an inherited disorder that results in increased excretion of cystine in the urine. It accounts for about 1-2% of pediatric kidney stones. In this study, we sought to identify the clinical characteristics of patients with cystinuria in a national cohort. METHODS: This was a retrospective study involving 30 patients from the Polish Registry of Inherited Tubulopathies. Initial data and that from a 6-month follow-up were analyzed. Mutational analysis was performed by targeted Sanger sequencing and, if applicable, MLPA analysis was used to detect large rearrangements. RESULTS: SLC7A9 mutations were detected in 15 children (50%; 10 males, 5 females), SLC3A1 mutations in 14 children (47%; 5 males, 9 females), and bigenic mutations in one male patient. The first clinical symptoms of the disease were detected at a median of 48 months of age (range 3-233 months). When individuals with different mutations were compared, there were no differences identified in gender, age of diagnosis, presence of UTI or urolithiasis, eGFR, calcium, or cystine excretion. The most common initial symptoms were urolithiasis in 26 patients (88%) and urinary tract infections in 4 patients (13%). Urological procedures were performed in 18 out of 30 (60%). CONCLUSIONS: The clinical course of cystinuria is similar among patients, regardless of the type of genetic mutation. Most patients require surgery before diagnosis or soon after it. Patients require combined urological and pharmacological treatment for prevention of stone recurrence and renal function preservation.

Upper metastable limit osmolality of urine as a predictor of kidney stone formation in children.

High fluid intake has been universally recommended for kidney stone prophylaxis. We evaluated 24-h urine osmolality regarded as the best biomarker of optimal hydration and upper metastable limit osmolality after water evaporation from urine sample to the onset of spontaneous crystallization and its usefulness as a new risk index that would describe an individual lithogenic potential. We collected 24-h urine from 257 pediatric patients with kidney stones and 270 controls. After volume and osmolality assessment, the urine samples were subjected to volume reduction in vacuum rotavapor continued to the onset of an induced urinary crystallization. The upper metastable limit osmolality of urine sample was calculated based on its initial osmolality value and the amount of water reduction. Pediatric stone formers presented with higher urine volume and lower urine osmolality than healthy controls. Despite that, their urine samples required much lower volume reduction to induce the spontaneous crystallization than those of controls. The ROC analysis revealed an AUC for the upper metastable limit osmolality of 0.9300 (95% CI 0.9104-0.9496) for distinguishing between stone formers and healthy subjects. At the cutoff of 2696 mOsm/kg, the test provided sensitivity and specificity of 0.8638 and 0.8189, respectively. 24-h urine osmolality provided the information about current hydration status, whereas evaporation test estimated the urinary potential to crystalize dependent on urine composition. Upper metastable limit osmolality may estimate the individual lithogenic capability and identify people at risk to stone formation when exposed to dehydration.

Citrate usage in the leading causes of blindness: new possibilities for the old metabolite.

INTRODUCTION: Citrate is an old metabolite which is best known for the role in the Krebs cycle. Citrate is widely used in many branches of medicine. In ophthalmology citrate is considered as a therapeutic agent and an useful diagnostic tool-biomarker. OBJECTIVES: To summarize the published literature on citrate usage in the leading causes of blindness and highlight the new possibilities for this old metabolite. METHODS: We conducted a systematic search of the scientific literature about citrate usage in ophthalmology up to January 2018. The reference lists of identified articles were searched for providing in-depth information. RESULTS: This systematic review included 30 articles. The role of citrate in the leading causes of blindness is presented. CONCLUSIONS: Citrate might help inhibit cataract progression, in case of questions confirm glaucoma diagnosis or improve cornea repair treatment as adjuvant agent (therapy of ulcerating cornea after alkali injury, crosslinking procedure). However, the knowledge about possible citrate usage in ophthalmology is not widely known. Promoting recent scientific knowledge about citrate usage in ophthalmology may not only benefit of medical improvement but may also limit economic costs caused by leading causes of blindness. Further studies on citrate usage in ophthalmology should continuously be the field of scientific interest.

Determining normal values of urinary phosphorus excretion in 3913 healthy children aged 2-18 to aid early diagnosis and treatment for urolithiasis.

AIM: This study determined the specific reference values for urinary phosphorus excretion in healthy children and adolescents aged 2-18 years and evaluated whether they changed with age during growth and were gender dependent. METHODS: We enrolled 3913 healthy children and adolescents aged 2-18 years to this study. The study population was divided into age groups, and the analysis was performed in one-year periods, separately for boys and girls. Urinary phosphorus excretion was analysed using four categories: P1 in mmol/24 hour units, P2 in mmol/kg/24 hours, P3 in mmol/1.73 m2 /24 hours and P4 in mmol/mmol creatinine. RESULTS: Clear differences in urinary exertion for girls and boys were observed as well as systematic changes with age. The boys presented with significantly higher daily urinary phosphorus excretion independent of its manner of expression (p < 0.001). The median urinary phosphorus (P1) rose with age (p < 0.001). Percentile tables of phosphorous exertion are presented. CONCLUSION: This was the largest study of urinary phosphate excretion based on a randomly selected sample of girls and boys aged 2-18 years. It highlights the importance of determining phosphorus reference values for children of different ages to provide early diagnosis and treatment for urolithiasis.

Urodynamic Findings and Renal Function in Children with Neurogenic Bladder after Myelomeningocele.

INTRODUCTION: Myelomeningocele (MMC) is a congenital central nervous system malformation caused by a failure of the neurulation process in early pregnancy. Patients with MMC present many abnormalities and the nervous, skeletal and urinary systems are the most affected. The aim of this study was to clinically evaluate patients with MMC, estimate renal and lower urinary tract (LUT) function and to ascertain whether urodynamic findings can predict the deterioration of urinary tract function. MATERIALS AND METHODS: Medical records of 112 patients were gathered from a database and evaluated retrospectively. The data included age, sex, BMI Z-score WHO, physical activity, urodynamic parameters and diagnosis and renal function. RESULTS: A total of 112 patients with MMC were enrolled in the study. There were no differences in age, sex, BMI Z-score WHO, physical activity, renal function and urodynamic findings (apart from cystometric capacity) between boys and girls. Detrusor overactivity was the most frequent urodynamic diagnosis in all groups of physical activity, level of lesion and in catheterized and non-catheterized children. The correlations between urodynamic findings and renal function tests were found. CONCLUSIONS: Patients with neurogenic bladder after MMC most often present detrusor overactivity. LUT function is disturbed in all MMC patients independent of lesion level and physical activity.

Does obesity or hyperuricemia influence lithogenic risk profile in children with urolithiasis?

BACKGROUND: There are indications that obesity and hyperuricemia may influence the formation and composition of urinary stones. The aim of our study was to determine the effect of obesity and hyperuricemia on the urinary lithogenic risk profile in a large cohort of pediatric patients. METHODS: The study population comprised 478 children with urolithiasis and 517 healthy children (reference group). We studied the effects of obesity on the lithogenic profile by dividing the patients with urolithiasis into two groups based on body mass index Z-score (patients who were overweight/obese vs. those with normal weight for age) and comparing the two groups. To study the effect of hyperuricemia on the lithogenic profile, we divided the patients with urolithiasis into two groups based on the presence or not of hyperuricemia (110 patients with urolithiasis accompanied by hyperuricemia vs. 368 patients with urolithiasis and normal serum uric acid levels) and compared the groups. RESULTS: Among the children and adolescents with urolithiasis and hyperuricemia, there was a significantly lower excretion of crystallization inhibitors (citrates, magnesium). We also found significantly negative correlations between serum uric acid levels and the urine citrate/creatinine ratio (citrate/cr.; r = -0.30, p < 0.01), as well as the magnesium/cr. ratio (Mg/cr.; r = -0.33, p < 0.01). There was no statistically significant differences in the urinary excretion of oxalates, citrates, calcium, phosphorus, magnesium and uric acid between children with urolithiasis who were either overweight or obese and children with urolithiasis who had a normal body weight. CONCLUSIONS: In our pediatric patient cohort, hyperuricemia was associated with a decrease in the excretion of crystallization inhibitors in the urine, but the clinical relevance of this observation needs to be confirmed in future studies. Obesity and overweight had no direct influence on the lithogenic risk profile in the urinary stone formers in our study, but there was an indication that higher serum uric acid may be associated with impairment in renal function, which in turn could influence the excretion of lithogenic parameters.

Induced urinary crystal formation as an analytical strategy for the prediction and monitoring of urolithiasis and other metabolism-related disorders.

Crystal formation reflects the entire composition of the surrounding solution. In case of urolithiasis, induced crystal formation in native urine has led to the development of the Bonn-Risk-Index (BRI), a valuable tool to quantify an individual's risk of calcium oxalate urolithiasis. If the progression of a disease is associated with characteristic changes in the activities of urinary components, this leads to an altered urinary crystallisation capacity. Therefore, the results of induced urinary crystal formation can be used to detect and monitor any disease linked to the altered urinary composition. Since crystal formation inherently takes into account the entire urinary composition, the influence of the disease on individual urinary parameters does not have to be known in order to monitor the consequent pathologic alterations. In this paper, we review the background of urinary crystal formation analysis and describe its established application in urolithiasis monitoring as well as potential further fields of clinical application.

Urinary citrate excretion in healthy children depends on age and gender.

BACKGROUND: Hypocitraturia is considered a major risk factor for calcium stone formation. However, there is no widely accepted reference database of urinary citrate excretion in children. The aim of our study was to determine the amount of citrate eliminated in the urine over a 24-h period in a pediatric cohort and to determine an optimal unit reflecting excretion. METHODS: The study cohort comprised 2,334 healthy boys and girls aged 2-18 years. The levels of urinary citrate were assessed by an enzymatic method in 24-hour urine and expressed in absolute values, as urinary concentration, citrate/creatinine ratio, per kilogram of body weight, in relation to 1.73 m2, and as the calcium/citrate index. RESULTS: Similar incremental age-related citraturia rates were observed in both male and female subjects until puberty during which time citrate excretion became significantly higher in girls. Urinary citrate adjusted for creatinine and for body weight showed a significantly decreasing trend with increasing age in both sexes. Urinary citrate corrected for body surface was weakly correlated with age. Thus, the assumption of 180 mg/1.73 m2/24 h for males and 250 mg/1.73 m2/24 h for females as lower cut-off values appeared to be reliable from a practical perspective. CONCLUSIONS: We found distinct sex-dependent differences in citraturia at the start of puberty, with significantly higher values of urinary citrate in girls than in boys. Further prospective studies are warranted to elucidate whether this difference represents a differentiated risk of urolithiasis.

An association between kidney stone composition and urinary metabolic disturbances in children.

OBJECTIVE: To determine kidney stone composition in children and to correlate stone fractions with urinary pH and metabolic urinary risk factors. PATIENTS AND METHODS: We studied 135 pediatric patients with upper urinary tract lithiasis in whom excreted or extracted stones were available for analyses. Composition of stones was analyzed. A 24-hour urine assessment included volume, pH and daily excretions of calcium, oxalate, uric acid, cystine, creatinine, phosphate, magnesium and citrate. RESULTS: Calcium oxalate was the major component of 73% stones, followed by struvite (13%) and calcium phosphate (9%). Uric acid was present in almost half of stones, but in rudimentary amounts. The calcium oxalate content in calculi showed a strong relationship with calciuria, and moderate association with oxaluria, magnesuria and acidification of urine. The percent content of struvite presented reverse and lower correlations with regard to the above parameters. Calcium phosphate stone proportion had low associations with urinary risk factors. CONCLUSIONS: Calciuria, oxaluria, magnesuria and low urine pH exerted the biggest influence on calcium oxalate content in pediatric renal stones. Relationships of urinary risk factors with calculi calcium phosphate content were of unclear significance. Urinary citrate excretion did not significantly correlate with kidney stone composition in children.

Correspondence between Ca²âº and calciuria, citrate level and pH of urine in pediatric urolithiasis.

BACKGROUND: Hypercalciuria and hypocitraturia are considered the most important risk factors for urolithiasis. Citrate binds to urinary calcium to form a soluble complex which decreases the availability of ionized calcium (Ca(2+)) necessary for calcium oxalate formation and phosphate crystallization. The aims of this study were to assess the Ca(2+) fraction in relation to total calciuria, citraturia and urinary pH and to determine whether urinary Ca(2+) concentration is a helpful biomarker in metabolic evaluation of children with urolithiasis. METHODS: We collected 24-h urine samples from 123 stone-forming children and adolescents with hypocitraturia and from 424 healthy controls. Total calciuria (total calcium, Catotal), Ca(2+), pH, citrate, oxalate and Bonn Risk Index (BRI) were assessed and compared between the two groups. RESULTS: Total calciuria and Ca(2+) content were higher in stone-formers than in the healthy children. In both stone-formers and controls, Ca(2+) content was inversely related to citraturia and urinary pH, whereas the Ca(2+)/Catotal ratio differed slightly between the groups. A large variability in Ca(2+) level was found across individuals in both groups. The BRI increased with increasing calciuria and urine acidity. CONCLUSIONS: Compared to controls, stone-formers with hypocitraturia demonstrated a higher urinary Ca(2+) concentration, but this was proportional to calciuria. The large individual variability in urinary Ca(2+) content limits its practical use in metabolic evaluation of children with urolithiasis. However, the Ca/Citrate ratio may be a useful clinical tool in evaluating children with urolithiasis.

Urinary nerve growth factor level in children with neurogenic bladder due to myelomeningocele.

OBJECTIVE: Myelomeningocele is the most common physically disabling birth defect in humans. It is caused by the failure of the neural tube to close and is most common in the lumbosacral area. Because of associated neurogenic bladder dysfunction, children with myelomeningocele have an increased risk of urinary tract infections and, ultimately, of kidney damage. Nerve growth factor (NGF) is an important mediator inducing bladder overactivity in many pathological conditions. The aim of this study was to evaluate urinary NGF excretion in children with neurogenic bladder caused by myelomeningocele. MATERIAL AND METHODS: The investigation was conducted into two groups. Group 1 comprised 28 children with neurogenic bladder, and group 2 comprised 20 healthy children with no abnormalities in the urinary and nervous systems. Urinary NGF levels were measured by enzyme-linked immunosorbent assay. RESULTS: Median urinary NGF concentration in group 1 was higher when compared with healthy controls. Positive correlations between urinary NGF level and detrusor pressure at maximum bladder capacity, and negative correlations between NGF and bladder wall compliance were found. CONCLUSIONS: Urinary NGF levels were significantly elevated in patients with myelomeningocele. Future studies are needed to examine further the significance of urinary NGF levels in the pathogenesis of neurogenic bladder in this clinical condition.

[Metabolic acidosis in children with newly diagnosed type 1 diabetes and risk factors of urolithiasis]. / Kwasica metaboliczna u dzieci z nowo rozpoznana cukrzyca typu 1 a czynniki ryzyka kamicy moczowej.

INTRODUCTION: Urolithiasis is increasingly being diagnosed in children. Some of the major risk factors for kidney stones are hipercalciuria, hiperoxaluria, hipocitraturia and hydrogen ion content measured by pH. Recently, more and more attention is being paid to the impact of diabetes type 1 and 2 on the development of nephrolithiasis especially in periods of poor metabolic control. AIM OF THE STUDY: was to evaluate the BRI (bonn risk index - rate of spontaneous crystallization of calcium oxalate), the concentration of oxalate, citrate and urine pH in children with acid-base balance disturbances occurring in patients with newly diagnosed type 1 diabetes (DMT1). MATERIAL AND METHODS: The study group included 40 patients aged 6 to 17 years (average age ± SD: 12,58 ± 3,33) with newly diagnosed DMT1. The study was performed twice: at the beginning of the disease, immediately after the treatment of diabetic ketoacidosis (study I) and after obtaining satisfactory metabolic control (study II), that is after about two weeks. The control group consisted of 100 children (6-17 years, average age 12,34 ± 3,96) without symptoms suggestive of urolithiasis. In every child, a 24-hour urine sample was collected. BRI was implemented in the urine by his own semi-micro method. RESULTS: Ionized calcium level was significantly higher immediately after the diagnosis of type 1 diabetes compared to the control group (0,68±0,67 vs. 0,40 ± 0,18 mmol/l; p<0,001) and to study II (0,68 ± 0,67 vs. 0,28 ± 0,23 mmol/l; p=0,008). BRI value was significantly higher in early onset compared to the control group and the study II (3,18 ± 5,54 vs. 0,66 ± 0,52, p<0,001; 3,18 ± 5,54 vs. 0,64 ± 1,56; p=0,034). BRI correlated inversely with pH at admission to the hospital (r=-0,53, p=0,0023). CONCLUSIONS: Metabolic alterations occurring during diabetic ketoacidosis at diagnosis of new type 1 diabetes may predispose to the development of the urinary stones, and thereby to the kidney damage.

[Urolithiasis risk factors in obese and overweight children]. / Czynniki ryzyka tworzenia kamieni nerkowych u dzieci z otyloscia i nadwaga

INTRODUCTION: Childhood obesity is becoming a worldwide epidemic and its metabolic and cardiovascular complications may already be evident at a young age. Several epidemiologic studies in adults have clearly demonstrated that obesity and overweight increase the risk of kidney disease and urolithiasis. AIM OF THE STUDY: The purpose of this study was to evaluate the relationship between overweight and obesity and urolithiasis risk factors in children. MATERIALS AND METHODS: The main kidney stones risk factors in urine such as calcium concentration, oxalate concentration, citrate concentration, pH of urine as well as BRI (Bonn Risk Index) were analyzed in 249 overweight and obese children (study group) and in 281 children with normal weight (control) at the age of 3 to 18 years old. RESULTS: In the study group the mean oxalate concentration was significantly higher than in the control (0.52±0.48 vs. 0.26±0.12; p <0.05). The mean calcium concentration of overweight/obese patients was higher than that of normal body weight and the difference was close to statistically significant (3.23±2.55 vs 2.58±1.59; p=0.0537). The mean urine pH in the study group was 6.28±0.46 and was significantly lower (p <0.05) than the mean urine pH in the control, witch was 6.40±0.47. The mean citrate concentration among overweight/obese patients was significantly lower than in control (431,2±309,5 vs. 637,2±310,7; p <0.05). CONCLUSIONS: Our results suggest that obesity or overweight at a young age are associated with an increased risk of kidney stones. Weight loss might be explored as a potential treatment to prevent kidney stone formation.

A potential pathogenic role of oxalate in autism.

BACKGROUND: Although autistic spectrum disorders (ASD) are a strongly genetic condition certain metabolic disturbances may contribute to clinical features. Metabolism of oxalate in children with ASD has not yet been studied. AIM: The objective was to determine oxalate levels in plasma and urine in autistic children in relation to other urinary parameters. METHOD: In this cross-sectional study, plasma oxalate (using enzymatic method with oxalate oxidase) and spontaneous urinary calcium oxalate (CaOx) crystallization (based on the Bonn-Risk-Index, BRI) were determined in 36 children and adolescents with ASD (26 boys, 10 girls) aged 2-18 years and compared with 60 healthy non-autistic children matched by age, gender and anthropometric traits. RESULTS: Children with ASD demonstrated 3-fold greater plasma oxalate levels [5.60 (5th-95th percentile: 3.47-7.51)] compared with reference [(1.84 (5th-95th percentile: 0.50-4.70) µmol/L (p < 0.05)] and 2.5-fold greater urinary oxalate concentrations (p < 0.05). No differences between the two groups were found in urinary pH, citraturia, calciuria or adjusted CaOx crystallization rates based on BRI. Despite significant hyperoxaluria no evidence of kidney stone disease or lithogenic risk was observed in these individuals. CONCLUSIONS: Hyperoxalemia and hyperoxaluria may be involved in the pathogenesis of ASD in children. Whether this is a result of impaired renal excretion or an extensive intestinal absorption, or both, or whether Ox may cross the blood brain barrier and disturb CNS function in the autistic children remains unclear. This appears to be the first report of plasma and urinary oxalate in childhood autism.

[Serum and urinary homocysteine in children with steroid-dependent nephrotic syndrome]. / Homocysteina w surowicy i moczu dzieci ze steroidozaleznym zespolem nerczycowym.

UNLABELLED: Hyperhomocysteinemia is independent risk factor of cardiovascular diseases. Similarly to nephrotic syndrome (NS) predisposes to vein thrombosis. THE AIM OF THE STUDY: To evaluate serum and urinary total homocysteine (stHcy and utHcy) levels in children with the symptoms of SN, and to determine a correlation between its concentration and some parameters of hemostasis, as well as doses and the time of prednisone therapy and serum cortisol level. MATERIAL AND METHODS: The examined group consisted of 18 children with NS, aged 7.64 +/- 5.1 years, divided on two groups: A--in time o proteinuria; B--during treatment with prednisone after regression of proteinuria. Control group (C) consisted of 20 children, aged 8.5 +/- 3.6 years. Serum and urinary tHcy levels were assayed by enzyme-linked immunosorbent assay method using the Axis-Shield set. RESULTS: Serum total Hcy concentration in groups A and B did not differ from the control group (p > 0.05). Urinary total Hcy concentration in groups A and B was significantly higher than that of control (p < 0.05). A positive correlation was observed between stHcy and serum albumin as well as cortisol levels, and between utHcy and serum AT III level. CONCLUSIONS: In children with steroid-dependent NS, subclinical disturbances in hemostasis were independent of serum tHcy concentration. There was no correlation between serum tHcy and cumulated doses, as well as time of prednisone treatment, however positive correlation was found with serum cortisone. Urinary excretion of Hcy significantly increases, in comparison to control, and correlates with serum AT III level.

Assessment of lithogenic risk in children based on a morning spot urine sample.

PURPOSE: The Bonn Risk Index has been used to evaluate the risk of urinary calcium oxalate stone formation. According to the original method, risk should be determined based on 24-hour urine collection. We studied whether the Bonn Risk Index could be measured in spot urine samples and which part of the day is most suitable for this purpose. MATERIALS AND METHODS: We collected total and fractionated 24-hour urine (in a 6-hour nocturnal portion and 9 consecutive 2-hour diurnal samples) in 42 children and adolescents with calcium oxalate urolithiasis and 46 controls. Bonn Risk Index values determined from each of the urine fractions were compared to those obtained from related 24-hour urine collections. RESULTS: Both groups exhibited similar circadian patterns of Bonn Risk Index values. Median Bonn Risk Index for the nighttime portion of urine in the stone group was 1.4 times higher than that obtained from the total 24-hour urine. The morning hours between 08:00 and 10:00 showed the peak lithogenic risk, and this fraction had the highest sensitivity and selectivity regarding discrimination between stone formers and healthy subjects. The afternoon hours demonstrated lower and less fluctuating crystallization risk. Despite diurnal fluctuations in Bonn Risk Index, there was still a well-defined cutoff between the groups. CONCLUSIONS: Bonn Risk Index determined from urine samples collected between 08:00 and 10:00 appears optimal in separating stone formers from healthy subjects, and appears as useful as the value determined from 24-hour urine collection. Investigation of this diurnal sample simplifies diagnosis in pediatric stone disease without loss of clinical information.

Glutathione reductase activity correlates with concentration of extracellular matrix degradation products in synovial fluid from patients with joint diseases.

The mechanisms underlying cartilage matrix degradation in joint diseases is not fully understood but reactive oxygen species are implicated as main causative factors. Comparative studies of glutathione reductase (GR) activity in synovial fluid from patients with rheumatoid arthritis (RA), reactive arthritis (ReA) and osteoarthritis (OA) as well as correlations between GR activity and concentration of the major cartilage components in synovial fluid are presented in this study. We found significantly higher activity of GR in RA (about three-fold) and ReA (about two-fold) than in OA. In RA and ReA patients, GR activity in synovial fluid correlates negatively with the concentrations of collagen and degradation products of sulfated glycosaminoglycans. In OA patients the activity of GR was significantly lower than in RA and ReA, which positively correlated with the concentration of collagen and showed a tendency for positive correlation with the degradation products of sulfated glycosaminoglycans. Our results suggest that in RA and ReA patients increased activity of GR does not prevent the increased degradation of collagen and proteoglycans by ROS.

[Matrix metalloproteinases 2 and 9 and their tissue inhibitors 1 and 2 in the urine of children with pyelonephritis]. / Metaloproteinazy 2 i 9 oraz ich tkankowe inhibitory 1 i 2 w moczu dzieci chorych na odmiedniczkowe zapalenie nerek.

UNLABELLED: In small children, pyelonephritis (PN) is an important cause of scarring in the renal and disturbed in the production and degradation of extracellulare matrix proteins (ECM). Aim of the study was to assess the urinary levels metalloproteinases 2 and 9 (MMP-2 and MMP-9) and their inhibitors 1 and 2 (TIMP-1 and TIMP-2) in children with pyelonephritis (PN). MATERIALS AND METHODS: Study group (I) consisted of 42 children with PN, aged 1-15 years, examined twice: A--prior to treatment (1-3 days of fever), B--after antibacterial treatment (10-14 days). The control group (K) consisted of 30 healthy children. Enzyme-linked immunosorbent assay kits were used for measurements of total human MMP-2, MMP-9, TIMP-1 and TIMP-2 in first morning urine. RESULTS: In children with PN (I) prior to treatment (A), urinary concentration of all parameters were increased as compared to the control (K) (p<0.05). After treatment (B), only the levels of TIMP-1 was still elevated (p = 0.02). In PN before (A) and after (B) treatment MMP-9/TIMP-1 ratio. However MMP-2/TIMP-2 ratio was normal. CONCLUSION: In children with PN the balance MMP-9/TIMP-1 is disturbed, with the predominance of TIMP-1 production over MMP-9. It may lead to renal fibrosis.

Spontaneous urinary calcium oxalate crystallization in hypercalciuric children.

Idiopathic hypercalciuria is the most important predisposing risk factor for calcium oxalate (CaOx) renal stone formation. We assessed the associations between spontaneous CaOx crystallization based on the Bonn Risk Index (BRI), urinary pH, calciuria, oxaluria, and citraturia in 140 Caucasian patients with hypercalciuria, aged 4-17 years, and compared the findings with those in 210 normocalciuric controls. Of the 140 hypercalciuric patients, 58 had renal stones, and 82 had recurrent erythrocyturia, renal colic, or urinary obstructive symptoms-but without stones. Urinary ionized calcium ([Ca(2+)]) levels were measured using a selective electrode, while the onset of crystallization was determined using a photometer and titration with 40 mmol/L ammonium oxalate (Ox(2-)). The calculation of the BRI was based on the [Ca(2+)]:Ox(2-) ratio. The BRI values were 12-fold higher in hypercalciuric children than in healthy controls, but no differences were found in the BRI between subjects with urinary stones and those with urolithiasis-like symptoms. An increased BRI suggested an association with hypercalciuria, lower urinary pH, hypocitraturia, and hypooxaluria. These data indicate that hypercalciuria is an important factor associated with increased urinary CaOx crystallization, although the causal pathways need further investigation. Determination of the BRI in children with hypercalciuria may improve the risk assessment of kidney stones.

[The evaluation of urinary tract dysfunction in children with monosymptomatic primary nocturnal enuresis]. / Ocena czynnosci dolnych dróg moczowych u dzieci z pierwotnym izolowanym moczeniem nocnym (PIMN).

UNLABELLED: The reason for our search was various investigations about urinary tract dysfunctions in enuretic children. AIM: The aim of our study was estimation of lover urinary tract function in children with monosymptomatic primary nocturnal enuresis without positive reaction for a long non pharmacological therapy. MATERIAL AND METHODS: 54 children after 9-12 months behavioral therapy and short pharmacological treatment (desmopresin) was undergoing urodynamic investigation (uroflowmetry and cystometry). RESULTS: Urodynamic disorders was found in 44/54 of estimated children. In 34 of children it was overactive bladder, in 6 patients we found detrusor-sphincter discoordination. Five children had decreased bladder capacity. Next to non pharmacological treatment we used anticholinergic or Baclofen depending on the results of urodynamic tests. The response to the treatment (non bedwetting at all) we observed in 34 children (in 9 of them after 3 months of therapy, in 16 after 6 months of therapy and in 12 after 12 months of therapy). The rest of children had decreased number of wet night per month. CONCLUSION: The pharmacological treatment of urodynamic disorders helps to children with monosymptomatic primary nocturnal enuresis to lost this symptom.