Enzymatic Activity of Candida spp. from Oral Cavity and Urine in Children with Nephrotic Syndrome.

Oral colonization with Candida spp. is not synonymous with a systemic active infection. The aim of the study was to evaluate enzymatic activity of Candida strains isolated from the oral cavity in patients with nephrotic syndrome (NS) and to compare it with the activity determined in urine. We studied 32 children with NS and 26 control healthy children. Children with NS were treated with glucocorticosteroids, cyclosporin A, mycophenolate mofetil or azathioprine. In all children, API-ZYM enzymatic tests were performed to evaluate hydrolytic enzymes of Candida isolated from the oral cavity and in urine. Candida spp. were isolated from the oral cavity in 11 patients with NS (34.4%), all receiving immunosuppressive treatment. All strains produced valine arylamidase, 9 alpha-glucosidase (E16), and 9 N-acetyl-beta-glucosaminidase (E18). A positive correlation between the presence of Candida in the oral cavity and E16 and E18 enzymatic activity in both oral cavity and urine was found. A dose of cyclosporin A had an effect on the enzymatic activity (p < 0.05). We conclude that immunosuppressive treatment of NS in children may predispose to systemic Candida invasion. The results of this study suggest that oral candida infection should be monitored in children with nephrotic syndrome, particularly those treated with immunosuppressive agents.

Effect of perinatal risk factors on neutrophil gelatinase-associated lipocalin (NGAL) level in umbilical and peripheral blood in neonates.

INTRODUCTION: Acute kidney injury biomarkers are opening a new era in diagnosing kidney failure. The requirement for a specific and sensitive marker of kidney function is highly desirable in neonates because the diagnostic possibilities in this age group are not sufficient. Recent research show that neutrophil gelatinase-associated lipocalin (NGAL) can have a great potential but there is a wide range of medical conditions, that may influence their expression. THE AIM OF THE STUDY: was to evaluate the impact of perinatal risk factors on NGAL level in neonates. MATERIAL AND METHODS: NGAL was measured in umbilical cord blood and peripheral blood in full term neonates with perinatal risk factors during the first days of life. RESULTS: We found significantly higher umbilical cord blood NGAL levels in neonates with perinatal risk factors (117.69 ng/ml) compared to the control group (64.37 ng/ml). No significant difference in peripheral blood NGAL level was shown between the two groups. Umbilical cord blood NGAL level correlated positively with peripheral blood NGAL level (r = 0.36, p < 0.01). Umbilical cord blood NGAL level was significantly higher in neonates with fetal distress and infection compared to neonates with other perinatal risk factors. Peripheral blood NGAL level was significantly higher in neonates with infection compared to neonates with other perinatal risk factors. Significantly higher umbilical cord blood NGAL levels were seen in neonates born by operative delivery compared to born by natural delivery.

Treatment Outcomes in Children with Henoch-Schönlein Nephritis.

The aim of the present study was to quantify the effects of treatment of children with Henoch-Schönlein nephritis (HSN) evaluated on the basis of kidney biopsy findings. Data were analyzed from 32 patients with HSN (mean age 9.3 ± 3.5 years, 19 with nephrotic syndrome/nephrotic proteinuria NS/NP, 13 with nephritic syndrome NphS), in whom the diagnosis was confirmed by kidney biopsy. Patients received immunosuppressive treatment (azathioprine or cyclophosphamide) and/or steroids and renoprotection according to a defined protocol. Patients were referred to a specific treatment protocol selected on the basis of clinical symptoms of nephropathy (NS/NP or NphS) and histopathological grade according to the WHO classification. Grade I-II changes were defined as mild HSN, and grade III-V WHO as severe HSN. The follow-up kidney biopsy was performed upon obtaining parental consent in 17 children. Following treatment, proteinuria resolved in 78 % children with mild HSN and 87 % children with severe HSN. In kidney biopsy, histological improvement was seen in 59 % children and no worsening in 35 %. We conclude that a flexible treatment protocol related to clinical symptoms and histological staging may contribute to a reduction of proteinuria and a delay in disease progression in children with HSN.

Polydactyly and obesity - the clinical manifestation of ciliopathy: a boy with Bardet-Biedl syndrome.

UNLABELLED: The prevalence of obesity in children is still rising all over the world. The most common reason for significant weight gain is a high-calorie diet and decreased physical activity. However, apart from environmental factors, genetic predisposition plays a crucial role in the pathomechanism of obesity. We present the case of a boy with pathological obesity and Bardet-Biedl syndrome (BBS). BBS is a ciliopathy, a heterogeneous group of rare disorders associated with defects in primary cilia. Other clinical signs and symptoms of BBS are: polydactyly, hypertension, hyperlipidemia, hypogonadotrophic hypogonadism, intellectual disability, rod-cone dystrophy, genitourinary and renal abnormalities. CONCLUSIONS: genetic factors of rapid weight gain should be taken into consideration in a child with obesity. Polydactyly can be associated with ciliopathy. A patient with Bardet-Biedl syndrome requires multi-specialist care.

[Abdominal symptoms necessitating surgical intervention as the initial presentation of Henoch-Schönlein purpura in children - case reports]. / Objawy brzuszne wymagajace interwencji chirurgicznej inicjujace zespól Schönleina-Henocha u dzieci - opis przypadków.

Henoch-Schönlein purpura (HSP) is the most common pediatric autoimmune vasculitis. Gastrointestinal symptoms of HSP including abdominal pain, diarrhea, and vomiting may precede skin changes by several days. We present diagnostic challenges in two adolescents with HSP and severe abdominal symptoms necessitating surgical intervention before the development of skin changes. CASE REPORT 1: A 15-year old boy with 7 day history of abdominal pain, and bloody vomiting (1-2 x per day) without diarrhea. A suspicion of acute appendicitis was raised and the boy was operated on the 7th day since the initial symptoms. The appendix showed some reactive inflammation and was removed during laparotomy which also revealed enlarged mesenteric lymph nodes and a modest amount of fluid in the pelvic cavity. During the first day after the surgery, skin changes typical for HSP developed on lower limbs and buttocks. CASE REPORT 2: A 12-year old girl with 7 day history of abdominal pain, without diarrhea or vomiting. On the day of admission hemorrhagic rash appeared on lower limbs. Laparotomy was performed on 14th day after onset of abdominal pain - large amounts of serous, blood-stained fluid, massive dilation of small intestine with ecchymoses in mucous membrane, segmental infiltration and stiffening of intestinal wall were found. Normal appendix was removed. CONCLUSIONS: Severe abdominal symptoms may precede skin changes in children with HSP, resulting in diagnostic and therapeutic challenges. When considering laparotomy in children with an atypical "acute abdomen" presentation, other manifestations of HSP should be sought.

Body weight changes in children with idiopathic nephrotic syndrome.

BACKGROUND: Aim of the study was to evaluate factors affecting body mass change in children with idiopathic nephrotic syndrome (INS) during 6-months treatment of initial disease bout with glucocorticoids (GC). MATERIAL AND METHODS: We studied 31 children with INS (22♂, 9♀, 3.6±1.8 years) treated during 6 months with GC due to initial INS bout and 31 control healthy children (18♂, 13♀, 4.0±1.8 years). Following factors were evaluated: body mass, body mass index (BMI), BMI Z-score, gender, age, gestational age at birth, birth weight, GC dose, parental age and BMI, time spent for TV/computer, physical activity, place of residence. RESULTS: Mean initial BMI Z-score was 0.35±1.1 in children with INS and -0.11±1.5 in the control group, after 6 months 0.8±1.2 (P=0.049) and 0.07±1.5 (P=0.629), respectively. Δ0-6 BMI Z-score correlated with initial BMI Z-score (r=-0.45, P=0.001), maternal age (r=0.38, P=0.04), and paternal BMI (r=0.51, P=0.0037). CONCLUSIONS: 1. Initial 6-month GC therapy may result in body mass increase in children with INS. 2. Risk factors for body mass increase in children with INS during the first 6 months of therapy include low initial BMI, older maternal age and paternal obesity.

Relationship between serum IgA/C3 ratio and severity of histological lesions using the Oxford classification in children with IgA nephropathy.

BACKGROUND: The aim of this study was to evaluate the usefulness of serum immunoglobulin A/complement factor 3 (IgA/C3) ratio for predicting histological severity of kidney lesions in children with IgA nephropathy (IgAN) based on World Health Organization (WHO) and the Oxford classification (OC). METHODS: We studied 89 children with IgAN with a mean age of 11.38 ± 4.1 years (range 2-18 years). Based on available medical records, we retrospectively evaluated clinical data, IgA/C3 ratio, and kidney biopsy findings using the five-grade WHO classification and the OC The mesangial hypercellularity (M), endocapillary hypercellularity (E), segmental sclerosis (S), tubular atrophy/interstitial fibrosis (T) (MEST) score (absent = 0, present = 1) calculated as the sum of M+E+S+T ranging from 0 to 4. RESULTS: Mean IgA/C3 ratio values were significantly higher (P < 0.05) in patients with M1, S1, and T1 compared with M0, S0, and T0, respectively (P < 0.05); there were no differences in the WHO classification. We found a significant positive correlation between the IgA/C3 ratio and proteinuria (r = 0.24) and determined optimal cutoff values of the IgA/C3 ratio, with a corresponding confidence interval for specific MEST scores. CONCLUSIONS: The IgA/C3 ratio in children with IgAN may be a useful marker of the severity of lesions found in kidney biopsy as evaluated using the OC.

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.

Candida spp. and gingivitis in children with nephrotic syndrome or type 1 diabetes.

BACKGROUND: Diabetes and Nephrotic syndrome (NS) promote plaque-related gingivitis and yeast-like fungal infections. The study assesses the impact of Candida spp. and general disease- or treatment-related factors on plaque-related gingivitis severity in children and adolescents with Nephrotic syndrome /diabetes. METHODS: Body mass index (BMI), BMI standard deviation score, and oral cavity (Plaque Index--PLI, Gingival Index--GI, mucosa status, presence and Candida enzymatic activity) were assessed in 96 patients (32 with NS: 30- immunosuppressive treatment, 35--type 1 diabetes, and 29 generally healthy), aged; 3-18 years. Laboratory included cholesterol and triglyceride measurements; in diabetic subjects- glycated haemoglobin, in NS: total protein, albumin, creatinine, haemoglobin, haematocrit, white cell count, urinary protein excretion. Medical records supplied information on disease duration and treatment. A statistical analysis was performed; Kendall Tau coefficient, chi-square test, t-test, and multiple regression analysis ( P < 0.05). RESULTS: Candida spp. often occurred in healthy patients, but oral candidiasis was found only in the NS and diabetes groups (9.37% and 11.43%). Gingivitis occurred more frequently in patients with NS/diabetes. Gingivitis severity was correlated with PLI, age, and yeast enzyme activity in NS--to with immunosuppressive treatment with >1 drug, drug doses, treatment duration, lipid disorders, and BMI; in diabetes, with blood glucose and glycated haemoglobin >8%. CONCLUSION: Poor hygiene control is the main cause of gingivitis. Gingivitis severity is most likely related to age, lipid disorders and increase in body mass. Candida spp., in uncompensated diabetes and in those using immunosuppressive treatment, might intensify plaque-related gingivitis.

PROCEEDING IN ACUTE KIDNEY INJURY IN ASPHYXIATED NEONATES--DIFFICULTIES AND SUCCESS.

UNLABELLED: Acute kidney injury (AKI) is a common consequence of perinatal asphyxia reported in 30 to 70% cases. We present 4 full-term neonates with oliguric/anuric AKI caused by perinatal asphyxia requiring renal replacement therapy (RRT) and their long-term outcomes. Patient No. 1 was dialyzed for 12 days (continuous ambulatory peritoneal dialysis (CAPD)/continuous venovenous hemodiafiltration (CWHDF)), then was treated conservatively, and received pre-emptive kidney transplantation (KTx) at the age of 3 3/12 years. Patient No. 2 was treated with CAPD/automated peritoneal dialysis (APD) for 15 months, due to recovery of renal function, dialysis was withdrawn. He is now 8 5/12 years old and has chronic kidney disease (CKD) stage III. Patient No. 3 after 5 days of continuous arteriovenous hemofiltration (CAVH) required CAPD for 17 days. The child is now 8 4/12 years old and has CKD stage III. Patient No. 4, dialyzed from 3rd day of life for 51 months (CAVH followed by CAPD/APD), was given cadaver KTx at the age of 4 3/12 years. Psychomotor development is good in 2 patients, whereas patients No. 3 and 4 have tetraplegic spastic infantile cerebral palsy, severe mental retardation, and epilepsy. CONCLUSIONS: Severe perinatal asphyxia with oliguric/anuric AKI is a risk factor for chronic kidney disease sometimes end-stage renal disease.

VIT. B12 DEFICIENCY IN CHILDREN (IMERSLUND-GRÄSBECK SYNDROME IN TWO PAIRS OF SIBLINGS).

UNLABELLED: Improvement in the quality of life in Europe and North America in last decades caused that economical and social aspects of living conditions of the population have less effect and genetic defects of malabsorption of vitamin B12 became the main reason for cobalamin deficiency in children. Imerslund-Grasbeck syndrome (IGS) is characterized by vitamin B12 deficiency that leads usually to megaloblastic anemia and mild proteinuria. We described two pairs of siblings in two families with IGS. The diagnosis in first family (two brothers) was established at 33 and 22 months of age. The reason for diagnostic tests were proteinuria and anemia. Apart from respiratory tract infections, they didn't present other symptoms of cobalamin deficiency. In the second family IGS was diagnosed in children at 5 and 8 years of age. Diagnostic evaluation procedures wereperformedbecause ofneurologicalsigns, including weakness, loss of appetite, dysmorphia, psychomotor retardation. Laboratory tests revealed megaloblastic anemia, low concentration of vitamin B12 in serum and mild proteinuria. In the first pair low concentration of vitamin B12 was validated by the Schilling test, in the second pair methylomalonate acid was detected in the urinary metabolic test. All children were successfully treated with vitamin B12 and anemia and neurological signs disappeared. Long-term follow up showed failure to thrive in the girl and physical and mental retardation, microcephaly in her brother. Proteinuria in the range: 0.3-1.2 g/24 h was detected in each child, and the other laboratory tests were normal. Clinical symptoms, laboratory tests and good reaction to parenteral treatment with vitamin B12 allowed us to diagnose Imerslund-Grasbeck syndrome, even without genetic tests. CONCLUSION: A delayed diagnosis of congenital malabsorption of cobalamin can lead to physical and mental retardation in children. Children with megaloblastic anemia and proteinuria resistant to classical treatment should be tested for congenital malabsorbtion of cobalamin.

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.

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.

Rare renal ectopia in children - intrathoracic ectopic kidney.

UNLABELLED: Renal ectopia occurs in 1:3000-1:7000 children. Simple ectopia refers to the abnormal kidney location on the same side; in crossed ectopia the ureter crosses the midline of the body. In most cases ectopic kidney is found in the pelvis minor, in 5% of cases in the thoracic cavity. Kidney displacement does not give any clinical symptoms in the majority of patients, sometimes it may cause abdominal pain, urinary tract infection, chest pain or promote nephrolithiasis. Renal ectopia is usually discovered during routine abdominal ultrasound. We describe a boy with prenatal ultrasound diagnosis of left kidney agenesis. Right normal kidney and displaced to the thoracic cavity, smaller, with normal echo-structure left kidney were found in postnatal ultrasound. Left kidney ectopia was confirmed in renal scinthigraphy, radiography and computed tomography of the chest. Voiding cystourethrography (VCUG) excluded vesicoureteral re€ux. CONCLUSIONS: In case of kidney's absence in typical localization in the abdominal ultrasonography, the ectopic kidney should be suspected. Diagnosis of ectopic kidney requires further additional imaging examination to check renal function and other urinary tract anomalies.

Ceftriaxone-associated acute gallbladder enlargement - an unexpected diagnosis in the child with urinary tract infection.

UNLABELLED: Biliary sludge and/or biliary pseudolithiasis occur in patients treated with ceftriaxone with prevalence of 3-57%. Biliary obstruction can be the cause of the acute gallbladder enlargement. It is a minor complication, that usually does not give clinical symptoms and resolves once the drug is discontinued. The authors present a case of a 5-month old boy treated for the acute pyelonephritis. Routine ultrasound, performed on the 5th day of treatment with ceftriaxone, showed gallbladder enlargement. In the consecutive studies small gallblader sludge was visible. Patient had no symptoms related to the gallbladder enlargement. Ultrasound performed 6 weeks from the drug discontinuation was completely normal. CONCLUSIONS: Patients treated with ceftroiaxone should be monitored for biliary sludge and pseudolithiasis.

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.

Long-term outcomes in idiopathic nephrotic syndrome: from childhood to adulthood.

BACKGROUND: The aim of the study was to assess idiopathic nephrotic syndrome (INS) relapse rate, co-morbidities, and social status of adults diagnosed with INS in childhood. MATERIAL AND METHODS: A written questionnaire was sent to 118 adults treated for INS in childhood. In 61 (51.7%) responders (aged 26.0 ± 6.2 years, range 18 - 51.5 years), we used available medical records to evaluate age at the onset of INS, number of INS relapses below 18 years of age, response to corticosteroids (CS), renal biopsy findings, and immunosuppressive treatment as well as questionnaire to evaluate the number and treatment of INS relapses above 18 years of age, co-morbidities, age at menarche, marital status, offspring, educational status, and occupation. RESULTS: In the group of 61 responders, median age at the onset of INS was 3 (range 1.3 - 14.0) years, median number of INS relapses at < 18 years of age was 5 (1 - 20). Steroid-sensitive nephrotic syndrome (SSNS) was diagnosed in 37 (60.7%) patients, steroid-dependent nephrotic syndrome SDNS in 18 (29.5%) patients, and steroid-resistant nephrotic syndrome (SRNS) in 6 (9.8%) patients. Mesangial proliferation was the most common pattern in renal biopsy (35.7%). All patients received CS, 15 were treated with methylprednisolone pulses, 13 with cyclophosphamide, 11 with chlorambucil, 2 with cyclosporine, and 21 with levamisole. All patients achieved remission and had normal renal function at the age of 18. In adulthood, INS relapsed in 10 (16.4%) patients, including 5 (13.5%) patients with SSNS, 4 (22.2%) with SDNS, and 1 (16.7%) with SRNS (p = 0.72). Median number of relapses was 2 (range 1 - 11). Patients with relapses at > 18 years of age had more (p < 0.005) relapses at < 18 years of age. Hypertension was diagnosed in 8 (16.1%), overweight in 14 (23.0%), obesity in 3 (4.9%), and bone fractures in 12 (19.7%) patients. Five patients had height < 3rd percentile, including 4 with INS onset at < 3 years of age. One patient had growth retardation before the treatment. No myocardial infarctions, strokes, severe infections, or malignancies were reported. Mean age at menarche was 12.9 ± 1.4 years, 37 (60.7%) patients were in a steady relationship/ married, 1/18 (5.6%) patients treated with cytostatic agents and 12/43 (24/7%) patients not treated with cytostatic agents had offspring (p < 0.05). Elementary education was reported by 4 (6.6%), secondary education by 32 (52.5%), and higher education by 25 (40.9%) patients, and 34 (55.7%) patients were professionally active. None of the 6 patients with SRNS developed end-stage renal disease. CONCLUSIONS: 1. High number of INS relapses in childhood is a risk factor for recurrences in adulthood. 2. INS relapses in childhood do not preclude active professional life in adulthood.

[The clinical significance of peritoneal transport in children during first year of peritoneal dialysis]. / Znaczenie kliniczne transportu otrzewnowego u dzieci w pierwszym roku leczenia dializa otrzewnowa.

UNLABELLED: Peritoneal transport status is one of main prognostic factors in patients with end-stage renal disease (ESRD) treated with chronic peritoneal dialysis (PD). The aim of study was evaluation of influence of peritoneal transport status on selected clinical and biochemical parameters in children with ESRD in first year of PD treatment. MATERIALS AND METHODS: 52 children (mean age 10.3 ± 4.7 years) with preserved residual renal function (RRF) treated with PD were enrolled into the study. In all patients we evaluated in first 12 months of PD treatment: peritoneal equilibration test (PET): D/P (Crea 4h), D/D0 Glu 4h, age, sex, etiology of ESRD, blood pressure, RRF (daily diuresis [mL/kg/24h], residual GFR [mL/min/1.73 m2]), PD parameters, adequacy, rate of PD-related peritonitis, medications, and biochemical parameters. RESULTS: Mean D/P (Crea 4h) was 0.65 ± 0.13, mean D/D0 glu 4h-0.38 ± 0.13. Patients were divided into 2 groups: H/HA (high/high-average) peritoneal permeability--26 (50.0%) and L/LA (low/low-average)--26 (50.0%) children. Patients with H/HA were significantly (P < 0.05): younger, had slower growth rate, higher systolic blood pressure, worse control of arterial hypertension, slower rates of rGFR and twCCr (total weekly clearance of creatinine) decline, higher incidence of peritonitis, lower total protein and albumin; tendency to lower hemoglobin (P = 0.07) compared to patients with L/LA. CONCLUSIONS: High peritoneal permeability in children with ESRD treated with chronic peritoneal dialysis may be a risk factor for slower growth rate, systolic hypertension, peritonitis and metabolic disturbances: anemia, hypoproteinemia and hypoalbuminemia. Rate of GFR decline is slower in children with high peritoneal transport status.

[False positive urine cultures in children under two years of age - own research].

INTRODUCTION: The basis of the diagnosis of urinary tract infection in children is positive culture of properly collected urine sample. The reliability of the urine cultures depends on the method how the urine sample was taken and sometimes this may increase the risk of misdiagnosis. AIM OF THE STUDY: To determine the frequency of false positive urine cultures taken from midstream to a container or to a plastic collection bag in children under 2 years of age. MATERIAL AND METHODS: The study included 50 children (25 girls, 25 boys) aged 12 days to 24 months (mean age 7.26±6.51months) referred to the hospital with suspicion of urinary tract infection. The most frequent indications for urine analysis were: history of infection and/or abnormalities of urinary tract in 28 (56%) children, failure to thrive in 8 (16%) and fever in 6 (12%). Urine was taken from midstream to a container in 32 (64%) children and collected to a plastic bag in 18 (36%) children. RESULTS: Hospital verifications of urine cultures were performed by suprapubic puncture culture in 24 (48% children) or by catheterization of the urinary bladder in 26 (52%) children. Urinary tract infection was confirmed in 11 (34%) among 32 children who had positive culture of urine form midstream. None of the children with positive urine culture from a plastic collection bag had urinary tract infection confirmed by suprapubic puncture or catheterization. CONCLUSIONS: Correct method of urine collection for bacteriological tests in children under two years of life can avoid the misdiagnosis of urinary tract infection and following unnecessary hospitalization, imaging procedures as well as potentially harmful treatment.

Serum fibroblast growth factor 23 and calcium-phosphorus metabolism parameters in children with chronic kidney disease - preliminary report.

INTRODUCTION: In chronic kidney disease (CKD) the function of all factors regulating mineral metabolism is disturbed, leading inevitably to renal osteodystrophy and vascular calcification. The aimof the study is to assess concentrations of fibroblast growth factor 23 (FGF 23), osteoprotegerin (OPG) and other parameters of calcium-phosphate metabolism in children with CKD. MATERIAL AND METHODS: 37 children with CKD 3-5, aged 1.6-17 years were included in the study. In all children serum levels of calcium (sCa), phosphate (sP), creatinine, alkaline phosphatase (ALP), FGF 23, intact parathormone (PTH), OPG and receptor activator nuclear factor κB ligand (RANKL) were measured. RESULTS: Total calcium concentration was within normal limits in all children included in this study. Hyperphosphatemia was found in 2 children from group CKD 3 (12%), 6 from CKD 4 (54%) and 1 from CKD 5 (11%). FGF 23 level increased consecutively in subsequent CKD stages achieving the highest values in CKD 5 group. In all children with CKD, serum levels of OPG were correlated with FGF 23. In children with CKD 3-4 negative correlation between FGF 23 and PTH (r=-0.45; p=0.02) and positive correlation between FGF 23 and RANKL (r=0,59; p=0.006) has been found. Positive correlation between OPG concentration and HCO3 -and BE levels has been observed, as well as negative correlation between RANKL/OPG ratio and HCO3 -and BE levels. CONCLUSION: Despite maintaining serum calcium, phosphorus and PTH levels within recommended limits, elevated levels of FGF 23 and OPG were observed in children with chronic kidney disease, especially in it's end-stage.