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

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

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.

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.

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

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

Risk Factors for Decline of Residual Renal Function in Children Treated With Peritoneal Dialysis.

♦ BACKGROUND: The 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). ♦ METHODS: The 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/PCrea 4h, D/D0 Glu 4h), 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. ♦ RESULTS: Mean 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 (ß = -0.386, p < 0.001) and proteinuria (ß = -0.278, p = 0.017), mean systolic BP Z-score (ß = -0.237, p = 0.027), and age at the onset of PD (ß = -0.224, p = 0.037), while predictors of the rate of rGFR decline were baseline rGFR (ß = -0.607, p < 0.001) and baseline proteinuria (ß = -0.225, p = 0.046). In the second year, the only predictors of the rate of rGFR decline were D/D0 Glu 4h (r = 0.44, p = 0.033, univariate analysis) and rGFR at 12 months (ß = -0.499, p = 0.044). ♦ CONCLUSION: The 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.

[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.

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.

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.

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.

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.

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.

Hypertension in children with end-stage renal disease.

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.

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.

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.

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.

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.

Effect of hypertension and antihypertensive medications on residual renal function in children treated with chronic peritoneal dialysis.

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 <95th percentile. No effect of AHM on the rate of RRF loss was found. In multivariate analysis, absolute daily diuresis loss was related to baseline diuresis (ß=-0.30, P<0.001) and proteinuria (ß=-0.31, P=0.004); absolute rGFR loss to baseline rGFR (ß=-0.73, P<0.001) and glucose load after 12 months (ß=-0.36, P=0.02); relative daily diuresis loss to mean BMI Z-score (ß=-0.44, P=0.04); and relative rGFR to baseline rGFR (ß=-0.37, P<0.001) and SBP percentile (ß=-0.21, P=0.045).