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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.

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

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-Schonlein 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-Schonlein 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-Schonlein purpura, Mycoplasma pneumonia infection may be a potential cause of exacerbation of the disease.

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 (p0.05). 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.

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.

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.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.

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).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.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).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.

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.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.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.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.

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.

The aim of the study was to evaluate bone mineral density (BMD) in the lumbar spine in children with idiopathic hypercalciuria.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.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-score1 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 levels30 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 (level20 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.Reduced lumbar spine BMD in patients with idiopathic hypercalciuria seems to be related to vitamin D3 deficiency.

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.Severe perinatal asphyxia with oliguric/anuric AKI is a risk factor for chronic kidney disease sometimes end-stage renal disease.

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.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.

StreszczenieNieprawidłowości dróg moczowych u płodu są jednym z najczęściej stwierdzanych odchyleń podczas wykonywanych prenatalnie badań ultrasonografi cznych. Bardzo istotne jest takie zaplanowanie postępowania u noworodka i niemowlęcia, aby odpowiednio szybko wyłonić i objąć właściwą opieką grupę dzieci wysokiego ryzyka zakażeń i postępu choroby nerek. Takie działanie wymaga współpracy wielospecjalistycznej i wspólnych dla pediatrów, neonatologów i lekarzy rodzinnych schematów postępowania. Niniejszy artykuł powstał na podstawie opracowanych przez Polskie Towarzystwo Nefrologii Dziecięcej i dotyczy działań, jakie należy podjąć u dziecka z prenatalnie wykrytym poszerzeniem dróg wyprowadzających mocz.AbstractMalformations of urinary tract in fetus are one of the most often abnormalities detected by prenatal ultrasound screening. It is of great importance to establish adequate diagnostic plan of postnatal evaluation and selection of patients at high risk of complications, urinary tract infections or kidney failure progression. Adequate diagnosis requires strict cooperation of specialists: paediatrician, neonatologist and family doctors with common guidelines. The paper is based on Guidelines of Polish Society for Paediatric Nephrology and concerns postnatal evaluation of dilatation of the urinary tract in fetus and newborn.

BACKGROUND Diarrhea-associated hemolytic uremic syndrome (HUS D+) caused by verotoxigenic E. coli strains (VTEC) is a major cause of acute kidney injury in children between 1 and 5 years of age. Because of the short presence of VTEC in the gastrointestinal tract as well as difficulties with the detection of the verotoxigenic strain, identification of HUS etiology might be challenging. OBJECTIVES The aim of the study was to assess the clinical and diagnostic value of serological tests for specific antibodies against verotoxigenic strains of E. coli in patients with HUS. MATERIAL AND METHODS Eight children aged 8 months - 7.1 years (mean 40 ± 29 months) with symptoms of acute kidney injury, hemolytic anemia and thrombocytopenia observed after hemorrhagic diarrhea were included to the study. VTEC presence was detected in a stool culture with subsequent analysis of the ability to produce verotoxin and the presence of VT1 and VT2 as well as intimin and enterohemolysin genes. In addition, the presence of specific IgA, IgM and IgG antibodies against E. coli serogroups O26, O103, O104, O111, O121, O145 and O157 was measured using ELISA. RESULTS In 3 subjects, VTEC O26, O157 and O104 serogroups were cultured in the stool and the specific IgA, IgM and IgG antibodies were detected. In 4 subjects, no VTEC strains were cultured, however, high titers of IgA, IgM and IgG antibodies against E. coli O26, O157 and O111 were detected. In a single patient, the negative results of bacteriological and serological analyses excluded VTEC etiology of HUS. CONCLUSIONS A serological analysis of VTEC can confirm the result of stool culture for verotoxigenic E. coli strains and help to find the cause of HUS in case of negative results of a stool culture.

BackgroundThe aim of the study was to assess risk factors for residual renal function (RRF) decline in children during the first/second year of chronic peritoneal dialysis (PD).MethodsThe study group included 56 children with end-stage renal disease (ESRD) (age 10.13 ± 4.86 years), including 18 on continuous ambulatory PD (CAPD) and 38 on automated PD (APD), in whom we evaluated RRF (daily diuresis [mL/m2/24 h], residual glomerular filtration rate (rGFR) [mL/min/1.73 m2]), etiology of ESRD, PD fluid volume (mL/m2/24 h), glucose load (g/m2/24 h), ultrafiltration (mL/m2/24 h), peritoneal permeability (D/PCrea4h, D/D0Glu4h), dialysis adequacy (twKt/V, twCCr [L/week/1.73 m2]), blood pressure (BP), biochemical parameters, and medications used. Duration of follow-up was 24 months.ResultsMean diuresis before initiation of PD was 1,394.93 ± 698.37 (mL/m2/24 h), and mean rGFR was 7.41 ± 3.96 (mL/min/1.73 m2). The rate of daily diuresis decline was -529.34 ± 546.28 in the first year and -107.10 ± 291.54 (mL/m2/24 h) in the second year ( p = 0.005), and the rate of rGFR decline was -3.35 ± 3.73 in the first year and -1.63 ± 1.85 (mL/min/1.73 m2) in the second year ( p = 0.118). Eleven (19.64%) patients became anuric. In univariate analysis, the rate of daily diuresis decline in the first year was related to baseline diuresis ( r = -0.29, p = 0.031), proteinuria ( r = -0.43, p = 0.001), and systolic BP ( r = -0.31, p = 0.020); 12-month changes (Δ0 - 12) in PD fluid volume ( r = -0.37, p = 0.004), glucose load ( r = -0.28, p = 0.035), and ultrafiltration ( r = -0.38, p = 0.004); serum calcium-phosphorus product ( r = -0.41, p = 0.002); and Δ0 - 12 body mass index (BMI) Z-score ( r = 0.30, p = 0.024); while the rate of rGFR decline in the first year was related only to baseline rGFR ( r = -0.57, p < 0.001). In multivariate analysis, significant predictors of the rate of daily diuresis decline in the first year were baseline diuresis (P = -0.386, p < 0.001) and proteinuria (p = -0.278, p = 0.017), mean systolic BP Z-score (P = -0.237, p = 0.027), and age at the onset of PD (P = -0.224, p = 0.037), while predictors of the rate of rGFR decline were baseline rGFR (P = -0.607, p < 0.001) and baseline proteinuria (P = -0.225, p = 0.046). In the second year, the only predictors of the rate of rGFR decline were D/D0Glu4h( r = 0.44, p = 0.033, univariate analysis) and rGFR at 12 months (P = -0.499, p = 0.044).ConclusionThe most important risk factors for rapid RRF decline in children during the first year of chronic PD include higher baseline daily diuresis and proteinuria, and additional factors are systolic BP and age at the onset of PD; while high baseline GFR and low peritoneal transport status may be the only important factors during the second year.

The aim of this retrospective study was to assess the usefulness of potential predictors of poor prognosis in IgA nephropathy in children. The study population consisted of 55 children aged 11 ± 4 years, diagnosed on the basis of the Oxford classification and MEST score of kidney biopsy findings. Proteinuria, glomerular filtration rate (GFR), and the IgA/C3 serum ratio were assessed in all patients twice: at onset and at follow-up. The patients were treated with steroids, immunosuppressive drugs, and/or angiotensin-converting enzyme inhibitors. Follow-up was at 3.9 ± 2.9 (median 2.7) years. The patients were subdivided into two groups: with GFR

The aim of the present study was to quantify the effects of treatment of children with Henoch-Schonlein 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.

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.Patients treated with ceftroiaxone should be monitored for biliary sludge and pseudolithiasis.

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.The aim of the study was to check the efficacy of continuous venovenous hemodiagiltration in intoxication with ethylene glycol and methanol.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).Intravenous ethanol infusion with hemodialtration resulted in rapid elimination of methanol from the body and significantly reduced blood ethylene glycol level.Continuous venovenous hemodiafiltration can be helpful in treatment of ethylene glycol and methanol intoxication.

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.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.

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

This review summarizes current data on the epidemiology, pathophysiology, and treatment of hypertension (HTN) in children with end-stage renal disease (ESRD). Worldwide prevalence of ESRD ranges from 5.0 to 84.4 per million age-related population. HTN is present in 27-79% of children with ESRD, depending on the modality of renal replacement therapy and the exact definition of hypertension. Ambulatory BP monitoring has been recommended for the detection of HTN and evaluation of treatment effectiveness. HTN in dialyzed patients is mostly related to hypervolemia, sodium overload, activation of the renin-angiotensin-aldosterone system (RAAS) and sympathetic nervous system, impaired nitric oxide synthesis, reduced vitamin D levels, and effects of microRNA. In children undergoing chronic dialysis therapy, important factors include optimization of renal replacement therapy and preservation of residual renal function, allowing reduction of volume- and sodium-overload, along with appropriate drug treatment, particularly with calcium channel blockers, RAAS inhibitors, and loop diuretics.

This study aimed to determine the frequency of associated urological abnormalities in children with unilateral renal agenesis (RA) or multicystic dysplastic kidney (MCDK). In total, 38 children (10 girls, 28 boys) were studied: 21 with RA and 17 with MCDK. In 14 children (37%) anomalies of the urinary tract were suspected prenatally in ultrasound studies. In the remaining 24 children the diagnosis of RA/MCDK was made postnatally: in 13 (34%) in the first 7 days of life, in 11 (29%) at the age of 8 days to 34 months, mean 10.6+/-8.05 months. Voiding cystourethrography was done in 36 (95%) children, the isotopic 99mTc-EC/DMSA scan of the kidney in 29 (67%), and urography in 8. Urological anomalies were present in 11 (29%) children: in 7 (33%) with RA and in 4 (24%) with MCDK. Vesicoureteral reflux was diagnosed in 8 children: grade II in 4, III in 3, and IV in 1 (in 1 child to duplicated, in 1 to ectopic kidney); ureterovesical junction obstruction in 2 (9.5%); and ureteropelvic junction obstruction in 1 (4.8%). Among them, 2 children demanded surgery on the contralateral urinary tract: pyeloplasty in 1, antireflux procedure in 1; while 9 children were treated conservatively. Compensatory hypertrophy of the contralateral kidney was found in 90% of children. Thus due to an increased risk of pathological changes in the single functioning kidney, lifelong nephrological care is recommended in patients with unilateral RA/MCDK.

Patient Male, 0 FINAL DIAGNOSIS: Bland-White-Garland syndrome Symptoms: Cardiomegaly, feeding problems Medication: - Clinical Procedure: Reimplantation of the left coronary artery to the aorta Specialty: Pediatrics and Neonatology. Objective Rare disease. Background Bland-White-Garland syndrome (BWGS) is a very rare disease characterized by anomalous origin of the left coronary artery from the pulmonary trunk (ALCAPA). WBGS affects 1 in every 300 000 live births. Children typically present with dyspnea, pallor, and failure to thrive. Without surgical repair, most of these children die during the first months of life. Case report This case report describes 3-month-old boy admitted to the hospital because of feeding problems. The boy was born at term, with birth weight 3200 g, and was 10 points in Apgar score. He was breast-fed from birth. From the seventh week of age, his mother observed his increasing difficulties with feeding. Physical examination revealed pale skin, diminished heart sounds, tachycardia, cardiomegaly, and hepatomegaly. Results of urine and blood tests and ultrasonography of the central nervous system and abdomen were normal. The chest radiography showed cardiomegaly and electrocardiogram revealed anterolateral myocardial infarction. On echocardiography, an anomalous left coronary artery arising from the pulmonary artery was found. The life-saving treatment of choice was immediate surgical reimplantation of the left coronary artery to the aorta. Conclusions Children with congenital heart disease are often prone to malnutrition, but in rare cases failure to thrive and breast-feeding problems can be the first symptoms of life-threatening diseases like myocardial infarction secondary to Bland-White-Garland syndrome (BWGS).

Objective To review our experience with two types of peritoneal catheters, the standard Tenckhoff catheter and the swan-neck presternal catheter (SNPC). Design A retrospective study was undertaken to compare exit-site infection (ESI) rates using two types of peritoneal catheters in children. Setting Medical University of Warsaw, Warsaw, Poland. Patients During the past 10 years, 60 peritoneal catheters were implanted in 50 children with end-stage renal failure: 46 straight, double-cuffed Tenckhoff in 37 children (mean age 11.8 ± 4.2 years, range 3.1 – 18.5 years), and 14 presternal in 13 children (mean age 10.6 ± 5 years, range 0.3 – 17.7 years). The SNPCs were used in special clinical situations such as recurrent ESI with previous abdominal peritoneal catheters, obesity, presence of ureterocutaneostomies, use of diapers, and young age. For the statistical analysis, only the first catheter placed in each child was chosen: 34 standard Tenckhoff catheters and 9 SNPCs. Intervention In all children, peritoneal catheters were implanted surgically under general anesthesia by one surgeon; uniform operative technique and perioperative management was used. Results The mean observation time for 46 standard Tenckhoff catheters was 23.8 ± 21.1 months, and for 14 SNPCs 25.1 ± 27.0 months. The ESI rate was 1/17.4 patient-months (0.69 episodes/year) for Tenckhoff catheters and 1/70.2 patient-months (0.17 episodes/year) for SNPCs. The observed differences in ESI rates between the groups reported did not achieve statistical significance. Conclusions The risk of ESI may be lower with presternal catheters. Confirmation of these findings requires further prospective clinical investigation in large numbers of patients.

The aim of the study was to determine whether an elevated IgA level at the time of the diagnosis of IgA nephropathy has an effect on the severity of kidney biopsy findings and long-term outcomes in children. We retrospectively studied 89 children with IgA nephropathy who were stratified into Group 1- elevated serum IgA and Group 2 – normal serum IgA at baseline. The level of IgA, proteinuria, hematuria, glomerular filtration rate (GFR) and hypertension (HTN) were compared at baseline and after the end of the follow-up period of 4.0 ± 3.1 years. Kidney biopsy findings were evaluated using the Oxford classification. The evaluation of treatment included immunosuppressive therapy and renoprotection with angiotensin converting-enzyme inhibitor (ACEI) or angiotensin II receptor blocker (ARB), or no treatment. The elevated serum IgA was found in 46 (52 %) patients and normal serum IgA level was found in 43 (48 %) patients. No differences were found between the two groups regarding the mean age of patients, proteinuria, and the number of patients with reduced GFR or HTN at baseline. In kidney biopsy, mesangial proliferation and segmental sclerosis were significantly more common in Group 1 compared with Group 2 (p < 0.05). Immunosuppressive therapy was used in 67 % children in Group 1 and 75 % children in Group 2. The Kaplan-Meier survival curves for renal function (with normal GFR) and persistent proteinuria did not differ significantly depending on the serum IgA level at baseline. We conclude that in IgA nephropathy the elevated serum IgA at baseline may be associated with mesangial proliferation and segmental sclerosis contribute to glomerulosclerosis, but has no effect on the presence of proteinuria or on the worsening of kidney function during several years of disease course.

Purpose To evaluate the effect of hypertension (HTN) and antihypertensive medications (AHM) on residual renal function (RRF) in children on CAPD and APD. Material/methods We retrospectively evaluated underlying kidney disease, systolic and diastolic blood pressure (SBP/DBP), presence and control of HTN (SBP/DBP ≥ 95th percentile), AHM, RRF (daily diuresis, residual glomerular filtration rate [rGFR]), biochemical parameters, BMI Z -score, and dialysis parameters during 12-month follow-up in 87 children (38 CAPD, 49 APD) aged 10.22 ± 4.31 years. The rate of RRF loss was expressed as absolute and relative [%] reduction. Results At baseline, HTN was found in 74.7% patients (CAPD/APD: 84.2%/67.3%, P = 0.06), most commonly in HUS and least frequently in CAKUT. The proportion of CAPD/APD patients with poorly controlled HTN was 70.0%/63.3% ( P = 0.50). Relative daily diuresis loss in children with uncontrolled HTN was higher ( P = 0.017) compared to children with SBP/DBP β = −0.30, P β = −0.31, P = 0.004); absolute rGFR loss to baseline rGFR ( β = −0.73, P β = −0.36, P = 0.02); relative daily diuresis loss to mean BMI Z -score ( β = −0.44, P = 0.04); and relative rGFR to baseline rGFR ( β = −0.37, P β = −0.21, P = 0.045). Conclusions 1. Maintaining blood pressure 2. Risk factors for rapid RRF loss in children on CAPD/APD include elevated SBP, high baseline diuresis/rGFR, proteinuria, and high glucose load in the dialysis fluid.

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.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.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 (P0.05): 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.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.

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 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.

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, p0.05) and 25(OH)D3 serum level (from 31.7±16.3 to 23.7±9.3; p0.05) 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; p0.05). 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.

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.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.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.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.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.

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.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.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.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.

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).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.Mean IgA/C3 ratio values were significantly higher (P0.05) in patients with M1, S1, and T1 compared with M0, S0, and T0, respectively (P0.05); 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.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.

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.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.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 at18 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 at18 years of age had more (p0.005) relapses at18 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 height3rd percentile, including 4 with INS onset at3 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 (p0.05). 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.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 incidence of posterior urethral valves is estimated to be from 3:1000 to 8:1000 and this is one of the most common causes of obstruction of urinary tract in boys. About 13-17% of children with posterior urethral valves develop end stage renal failure. We present a 6-month-old boy with late diagnosis of posterior urtehral valves. Antenatal ultrasound investigation of the urinary tract was normal. A small degree of oligohydramnios was found during delivery. At the age of six months the boy was admitted to hospital because of urinary tract infection, hypertension (130/90 mmHg) and acute kidney injury (urea - 46 mg/dL, creatinine - 1.1 mg/dL, GFR - 35.5 mL/min/1.73 m2 ). Bilateral hydronephrosis and megaureters, low-capacity bladder with hypertrophied wall were seen on ultrasound examination. Voiding cystourethrograhy revealed vesicoureteral refluxes (III/V), hypertrophy of the bladder wall with numerous diverticula and dilated posterior urethra. During urethroscopy urethral valves were resected. Increased intravesical pressure (leak point up to 305 cm H2 O) was found on urodynamic test. Renal scintigraphy (99mTc-EC) revealed decreased intake of isotope in the left kidney (5%), and the right kidney intake was 95% ERPF. The patient was qualified for left-sided nephrectomy, which was postponed because of high leak point and high risk of worsening of vesicoureteral reflux to right kidney after nephrectomy. Anticholinergic and α-blocker treatment was started. At the age of 11 months left-side nephrectomy was performed because of recurrent urinary tract infections. After 3.5-year follow-up blood pressure, physical development, kidney function tests, and urinalysis are normal. Additionally to this investigation the significance of early diagnosis including prenatal (PUV) for further development as well as further therapeutic procedure is discussed.

Assessment of vitamin D status in children with selected renal diseases based on serum 25OHD3 level taking into consideration type of kidney disease, vitamin D supplementation and season of the year.Retrospective analysis of 127 children aged from 0.6 to 17.7 years, median 9.5 [24 with risk status of urolithiasis (RSU), 29 with urolithiasis, 15 with glomerulonephritis, 59 with idiopathic nephrotic syndrome (INS)] was performed. Serum concentrations of 25OHD3, 1,25(OH)2 D3, calcium, and phosphorus were measured. The correlation of 25OHD3 with the type of kidney disease, supplementation of vitamin D, seasons, gender, age, and the dose of glucocorticosteroids (in children with glomerulopathies) were analyzed.In all children serum concentration of 25OHD3 from 4.3 to 72.6 ng/mL (median 21.1 ng/mL). The deficiency or insufficiency of vitamin D were observed in 55 (43.3%) analyzed children with kidney diseases. The deficiency or insufficiency of vitamin D were observed in 49.1% children with RSU and urolithiasis, and in 39.2% with glomerulonephritis and INS: in 36% children supplemented with vitamin D and 54% not supplemented (NS). In winter, 25OHD3 serum concentration was significantly higher in children supplemented with vitamin D compared to not supplemented [median 21.5 ng/mL vs 16.5 ng/mL (p0.05)]. There were no significant differences in serum concentrations of 25OHD3 , 1,25(OH)2 D3 , calcium, phosphorus and calcium x phophorus product depending on type of kidney disease and gender. The significant negative correlation was found between 25OHD3 and patients' age (r=-0.26 p0.01) and between calcium (r=0.31, p0.05) and calcium x phosphorus in children supplemented with vitamin D (r=0.28, p0.05).Our results suggest the necessity to develop new strategies in vitamin D supplementation in children with kidney diseases. Futher studies shoud also be performed to evaluate their efficiency.

Congenital chloride diarrhoea is a rare autosomal recessive disease and the diagnosis is frequently delayed. The disease is most common in Saudi Arabia and Kuwait 1:3200-13 000 births, Finland - 1:30 000-40 000, and in Poland - 1:200 000. Congenital chloride diarrhoea begins in fetal life. The main clinical sign is watery diarrhea that in utero leads to dilated bowel loops, polyhydramnios and often premature birth. Newborns have distended abdomens, absence of meconium, dilated bowel loops in ultrasonography and watery diarrhea which can sometimes be mistaken for urine. The absence of meconium and the distended abdomen suggest meconium ileus or Hirschsprung disease and can lead to unnecessary surgical intervention. The article is a report on a 3-months old boy with the history of dilated bowel loops in prenatal ultrasonograhy, low birth weight and abdominal distention. Because of the suspicion of mechanical bowel obstruction he had laparotomy on the second day of his life. Mechanical obstruction was excluded and enterostomy was performed. Hyponatremia, hypokaliemia and metabolic alkalosis were found in laboratory tests. The electrolyte disturbances were corrected and enterostomy was closed after six weeks. The final diagnosis of congenital chloride diarrhea was established two months later, when the patient was admitted to hospital again with severe watery diarrhea, metabolic alkalosis, hypochloraemia and hypokalemia. The stool chloride concentration was90 mmol/L. Water and electrolyte deficits had been corrected. The patient was discharged home with supplementation of sodium, potassium and chloride. His follow-up was uneventful. He remains under the care of the pediatric clinic.

Methotrexate is a highly nephro- and hepatotoxic drug used in osteosarcoma treatment protocols, in children and adults. High dose methotrexate therapy may lead to kidney injury and decrease of methotrexate clearance, followed by an increase of its serum concentration. As a result, systemic intoxication may develop. Prophylaxis based on intensive fluid therapy and urine alkalization may not be sufficient to prevent the formation of methotrexate crystals in kidney tubules. THE AIM of the study was to present three cases of methotrexate intoxication treated with continuous veno-venous hemodiafiltration.Three children aged 9-16 years old with tibial or fibular osteosarcoma were admitted to the Nephrology Department due to severe methotrexate intoxication. All children presented with multiorgan injury, including liver, kidney, gastrointestinal tract and bone marrow impairment. Methotrexate concentration, 24 hours after drug administration, was 660-1238 µmol/L. Although intensive fluid therapy, urine alkalisation and administration of high doses of folinic acid (leucovorin), methotrexate serum concentration remained toxic. Effective reduction of methotrexate concentration (1.5 μmol/L) was achieved 24-156 hours after CVVHDF initiation. Kidney and liver function recovered completely in all of the patients.Continuous veno-venous hemodiafiltration is an effective supportive method in methotrexate elimination in patients with severe intoxication.

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.

Hypertensive crisis is a sudden rise in blood pressure above 99 c. for sex, age and height +5 mm Hg. Depending on patient's symptoms, hypertensive crisis can be divided into hypertensive emergency severe arterial hypertension with target organ insufficiency and/r damage (central nervous system, heart, kidney, eye), and hypertensive urgency - severe arterial hypertension without target organ insufficiency and damage with non-specific symptoms like: headaches, vertigo, nasal bleeding, nausea, and vomiting. The most common causes of hypertensive crisis in neonates and infants are renal artery thrombosis, broncho-pulmonary dysplasia, and coarctation of aorta; in older children - kidney diseases and renal artery stenosis. In neonates and infants symptoms of cardiac failure predominate, whereas in older children symptoms from central nervous system (headaches, nausea, vomiting, changes in level of consciousness, seizures, focal deficits). Hypertensive crisis is treated with fast- and short-acting medications; 25% reduction of blood pressure within first 8 hours is recommended, with complete normalization within 24-48 hours. Hypertensive emergency should be treated with intravenous agents (labetalol, hydralazine, nicardipine, and sodium nitroprusside), hypertensive urgency with intravenous or oral agents like nifedipine, isradipine, clonidine and minoxidil. Nicardipine is a first-choice medication in neonates.

N. meningitidis wystepuje wylącznie u czlowieka i wywoluje zakazenia o roznej lokalizacji. Posocznica meningokokowa związana jest z wysoką śmiertelnością. Przewlekla meningokokcemia rzadko wystepuje u dzieci. Do grupy ryzyka zakazen meningokokowych nalezą niemowleta. Autorzy przedstawiają przypadek 6-miesiecznego niemowlecia, u ktorego dwukrotnie rozpoznano zakazenie meningokokowe oraz opon mozgowo-rdzeniowych. Po dochodzeniu epidemiologicznym ustalono, ze zakazenia zostalo wywolane tym samym szczepem bakterii pochodzącym od jednej osoby. Potwierdza to koniecznośc przeprowadzania skutecznej profilaktyki u osob z najblizszego otoczenia dziecka, aby zapobiec kolejnym zakazeniom.

We performed a genome-wide association study (GWAS) of IgA nephropathy (IgAN), the most common form of glomerulonephritis, with discovery and follow-up in 20,612 individuals of European and East Asian ancestry. We identified six novel genome-wide significant associations, four in ITGAM-ITGAX, VAV3 and CARD9 and two new independent signals at HLA-DQB1 and DEFA. We replicated the nine previously reported signals, including known SNPs in the HLA-DQB1 and DEFA loci. The cumulative burden of risk alleles is strongly associated with age at disease onset. Most loci are either directly associated with risk of inflammatory bowel disease (IBD) or maintenance of the intestinal epithelial barrier and response to mucosal pathogens. The geo-spatial distribution of risk alleles is highly suggestive of multi-locus adaptation and the genetic risk correlates strongly with variation in local pathogens, particularly helminth diversity, suggesting a possible role for host-intestinal pathogen interactions in shaping the genetic landscape of IgAN.

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.