Ambulatory blood pressure monitoring parameters in obese children and adolescents with masked hypertension.

OBJECTIVE: We aimed to compare the demographic, laboratory, and ambulatory blood pressure monitoring (ABPM) parameters of patients with masked hypertension (MHT), define factors predicting MHT, and determine the ABPM parameters affecting left ventricular mass index (LVMI) in obese youth. METHODS: Data of obese patients were evaluated retrospectively. Patients with ambulatory hypertension (AHT), white-coat hypertension (WCHT), MHT, or normotension (NT) were determined. Demographic and laboratory findings, office and ABPM measurements, blood pressure variability (BPV), and heart rate variability (HRV) were compared between the groups. The factors predicting MHT and the association between LVMI and ABPM, BPV/HRV parameters were analyzed. RESULTS: None of the 118 patients (M/F: 52/66) had WCHT. Three groups were formed: AHT (n: 60, 51%), MHT (n: 46, 39%), and NT (n: 12, 10%). Striae were significantly more frequent in AHT and MHT groups (P: 0.003). Cut-off levels for office BP measurements predicting MHT were 0.85 and 0.76 for systolic and diastolic BP (SBP and DBP) indexes, respectively. Most of the ABPM parameters of MHT group were as high as those of AHT group. On regression analysis, only daytime MAP had a positive (ß: 0.340; P < 0.01) and diastolic dip (ß: -0.204; P < 0.01) had a significantly negative association with LVMI. CONCLUSION: Stria and cut-off levels for office SBP/DBP indexes, which were defined for the first time in this study, may determine the patients at risk of MHT. Although BPV or HRV had no relation to LVMI, daytime MAP and diastolic dip represented independent associations with LVMI.

Successful Management of a Rare Cause of Hemolytic Uremic Syndrome With Eculizumab in a Child.

BACKGROUND: Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. It very rarely coexists with acute lymphoblastic leukemia (ALL) emerging before, simultaneously, or after the diagnosis has been made, and management of the patient may be difficult. CASE: We present the case of a 7-year-old boy who was diagnosed with HUS and initially managed by hemodialysis (HD). Thereafter, HUS progressed, and neurological findings developed. The patient was treated with eculizumab, agressive blood pressure control, and antiepileptic drugs. At the fifth month of follow-up, the patient was diagnosed with acute B-cell lymphoblastic leukemia with fever, bone pain, hepatosplenomegaly, and pancytopenia. After initiation of ALL treatment, he had no episodes of HUS, despite cessation of eculizumab. CONCLUSION: In conclusion, eculizumab may be a treatment of choice to prevent further systemic damage in recurrent HUS episodes of patients with borderline changes in the bone marrow until ALL is constantly diagnosed.

The evaluation of burnout and job satisfaction levels in residents of pediatrics.

AIM: The aim of the study was to examine the level of job-related burnout and the level of job satisfaction among residents of pediatrics. MATERIAL AND METHODS: A total of 102 residents of pediatrics who were trained in two Ministry of Health teaching and research hospitals and in two university hospitals in Izmir were included in the study. Demographic data (age, sex), lifestyle (living with parents or not, marital status, number of children) and professional characteristics (total time spent in profession, time spent in residency, number of night shifts per month, institution type: teaching hospital/university) were collected. Maslach Burnout Inventory (subscales: emotional exhaustion, desensitization, personal accomplishment) and Minnesota Satisfaction Questionnaire were used. RESULTS: High levels of emotional exhaustion and desensitization, and low levels of personal accomplishment and job satisfaction were found in residents of pediatrics. Low levels of emotional exhaustion in teaching and research hospitals and low levels of desensitization in university hospitals were determined (p<0.05). We found a positive correlation between age and job satisfaction levels and a negative correlation between age and emotional exhaustion levels (p<0.05). There were negative correlations between the length of time in education and desensitization and between the number of night shifts per month and desensitization (p<0.05). CONCLUSIONS: In residents of pediatrics, there is a high-level burnout and low-level of job satisfaction. Emotional exhaustion is more common in teaching and research hospitals and desensitization is more common in universities. Younger age, lower seniority, and the higher number of work-shift increases the burnout.

Pretransplant Stable Systolic Cardiac Functions Play an Important Role in Short-term Systolic Cardiac Functions After Kidney Transplant in Children.

OBJECTIVES: In this study, our aim was to evaluate the systolic cardiac parameters and related risk factors in children within 6 months after kidney transplant. MATERIALS AND METHODS: We retrospectively evaluated 24 children who received kidney transplants. Clinical and laboratory parameters before and after transplant were recorded. Results were evaluated statistically, with a P value less than .05 considered significant. RESULTS: Before transplant, systolic cardiac functions were within normal limits. After transplant, ejection fraction (63.35% ± 5.38% vs 66.95% ± 4.62%; P = .01) was significantly increased and left ventricular mass index (32.63 ± 17.21 g/m2.7 vs 31.29 ± 15.65 g/m2.7; P = .78) was not significantly decreased, whereas fractional shortening (52.16% ± 15.32% vs 59.8% ± 12.94%; P = .54) did not change. Systolic blood pressure, systolic blood pressure index, diastolic blood pressure, and diastolic blood pressure index values were not statistically different before and after transplant (P > .05). The number of antihypertensive agents was significantly decreased (P = .001). Before and after transplant, cardiac geometry was normal in 15 patients (62.5%) and 17 patients (70.8%). CONCLUSIONS: Our patients, who had stable systolic cardiac function before transplant, showed further improvements in systolic cardiac function even within 6 months after transplant. Therefore, strictly monitored and controlled blood pressure, volume, anemia, and nutrition in children before transplant may play important roles in achieving better cardiac systolic function after kidney transplant.

Evaluation of Nutritional Status in Children during Predialysis, or Treated By Peritoneal Dialysis or Hemodialysis.

OBJECTIVE: Malnutrition is one of the major causes of morbidity and mortality in children with chronic kidney disease (CKD). The objective of this study was to evaluate nutritional status of children with stage 3-4 CKD and treated by peritoneal dialysis or hemodialysis using anthropometric measurements, biochemical parameters and bioelectrical impedance analysis. PATIENTS AND METHODS: The study included a total of 52 patients and 46 healthy children. RESULTS: In anthropometric evaluation, the children with CKD had lower values for standard deviation score for weight, height, body mass index, skinfold thickness and mid-arm circumference than those of healthy children (p < 0.05). The fat mass (%) and the body cell mass (%) measurements performed by bioelectrical impedance analysis were lower compared with the control group (p < 0.05). CONCLUSION: It is considered that bioelectrical impedance analysis measurement should be used with anthropometric measurements, which are easy to perform, to achieve more accurate nutritional evaluation in children.

A Rare Cause of Metabolic Acidosis: Fatal Transdermal Methanol Intoxication in an Infant.

Oral methanol intoxication is common, but dermal intoxication is rare. We report a previously healthy 19-month-old female infant admitted to the emergency department (ED) with vomiting and tonic-clonic seizure. On physical examination, she was comatose and presented signs of decompensated shock with Kussmaul breathing. Her left thigh was edematous, with purple coloration. Methanol intoxication was suspected due to high anion gap metabolic acidosis (pH, 6.89; HCO3, <3 meq/L) and exposure to spirit-soaked bandages (%96 methanol) for 24 hours and 3 days. The patient's serum methanol level was 20.4 mg/dL. She was treated with fomepizole and continuous venovenous hemodialysis (CVVHD) in the pediatric intensive care unit, and methanol levels decreased to 0 mg/dL after 12 hours. During follow-up, massive edema and subarachnoid hemorrhage in the occipital lobe were detected by computed tomography of the brain. The patient died after 7 days.Although methanol intoxication occurs predominantly in adults, it must be considered in children with high-anion gap metabolic acidosis. This case report demonstrates that fatal transdermal methanol intoxication can occur in children, and it is the second report in the English literature of transdermal methanol intoxication in an infant.

A Comparison of Bladder Catheterization and Suprapubic Aspiration Methods for Urine Sample Collection From Infants With a Suspected Urinary Tract Infection.

This study compares 2 sampling methods for urine cultures in young infants. We analyzed data on urine samples obtained from 83 infants using 2 sources of urine: suprapubic bladder aspiration (SPA) and bladder catheterization. All specimens were subjected to both urinalysis and culture, and the results compared. Eighty-three infants with positive urine culture results obtained by bladder catheterization were subjected to SPA. Of these, only 24 (28.9%) and 20 (24%) yielded positive urine culture and abnormal urinalysis data, respectively. Samples obtained via catheterization had a high false-positive rate (71.1%). The sensitivity and specificity of urinalysis were 66.7% (95% CI, 44.68% to 84.33%) and 93.22% (95% CI, 83.53% to 98.08%), respectively. In infants younger than 12 months, SPA is the best method to avoid bacterial contamination, showing better results than transurethral catheterization.

Toilet training age and influencing factors: a multicenter study.

To determine toilet training age and the factors influencing this in our country, 1500 children who had completed toilet training were evaluated in a multicenter study. The mean age of toilet training was 22.32 ± 6.57 months. The duration it took to complete toilet training was 6.60 ± 2.20 months on the average. In univariant analysis, toilet training age increased as the parental education level, specifically that of the mother, increased. The training age of children whose mothers had over 12 years of education differed significantly from that of children of mothers with less education. There was no significant difference in toilet training age with regard to the education level of the father, or the employment status of the mother. We also found significant differences with respect to family income level, toilet type and training method. In multivariant analysis, family income >5000 TL and use of a potty chair were determined to be factors affecting toilet training age. In conclusion, toilet training age in Turkey, a developing country, was found to be lower than that in developed countries.

Faecal calprotectin levels in children with Henoch-Schönlein purpura: is this a new marker for gastrointestinal involvement?

AIM: We aimed to investigate the significance of faecal calprotectin (FC) levels in children diagnosed with Henoch-Schönlein purpura (HSP) and examine its relationships with gastrointestinal system (GIS), renal involvement and with clinical findings. PATIENTS AND METHODS: In total, 66 children diagnosed with HSP for the first time and a control group of 25 healthy children were included. The cases were divided into mild and severe groups on the basis of GIS findings. FC was measured twice in all patients with HSP: within 3 days of onset of disease (FC1) and on day 15 (FC2). These results were compared with those of the control group. Faecal occult blood, gastric wall thickness and duodenal wall thickness were measured at the same time as FC1 in all patients, and the presence of renal involvement was recorded. RESULTS: Of the 66 patients, 37 (56%) were females (mean age, 7.5±2.9 years; range, 2.5-14.5 years) and were diagnosed with HSP. Renal involvement was detected in 19 (28%) cases and GIS involvement was found in 28 (43%) cases. GIS involvement was mild in 16 (53%) cases and severe in 12 (43%). A significant difference was detected in FC1 levels between the groups with and without GIS involvement (P=0.01). A marked difference was observed in FC1 levels between the groups with and without renal involvement (P=0.017). CONCLUSION: FC may be a reliable marker for HSP, particularly for identifying GIS and renal involvement as well as disease severity.

The importance of ultrasonographic measurement of peritoneal wall thickness in pediatric chronic peritoneal dialysis patients.

Loss of peritoneal function due to peritoneal fibrosing syndrome (PFS) is a major factor leading to treatment failure in chronic peritoneal dialysis (PD) patients. Although the precise biologic mechanisms responsible for these changes have not been defined, the general assumption is that alterations in peritoneal function are related to structural changes in the peritoneal membrane. Studies of the peritoneal membrane by non-invasive ultrasonography (US) in chronic PD patients are limited. The aim of the present study is to assess the relationship between functional parameters of peritoneum and peritoneal thickness measured by US in children treated by chronic PD. We recruited two groups of patients: 23 subjects (13 females, 10 males) on chronic PD (patient group) and 26 (7 females, 19 males) on predialysis out-patient follow-up (creatinine clearance: 20-60 mL/min/1.73 m(2)) (control group). Age, sex, weight, height, body mass index (BMI), chronic PD duration, episodes of peritonitis and the results of peritoneal equilibration test (PET) were recorded. Hemoglobin (Hb), blood pressure (BP), left ventricular mass index (LVMI) and renal osteodystrophy (ROD) parameters were also obtained. The thickness of the parietal peritoneum was measured by trans-abdominal US in all children. Statistical analyses were performed by using Student's t and Pearson's correlation tests. Mean peritoneal thickness in chronic PD patients (1028.26 ± 157.26 µm) was significantly higher than control patients (786.52 ± 132.33). Mean peritoneal thickness was significantly correlated with mean body height (R(2) = 0.93, p < 0.05), BMI (R(2) = 0.25, p < 0.05), chronic PD duration (R(2) = 0.64, p < 0.05), episodes of peritonitis (R(2) = 0.93, p < 0.05), D/Pcreatinine (R(2) = 0.76, p < 0.05) and D4/D0glucose (R(2) = 0.81, p < 0.05). No correlation was found between peritoneal thickness and Hb, BP, LVMI and ROD parameters. In conclusion, ultrasonographic measurement of peritoneal membrane thickness is a simple and non-invasive method in chronic PD children. This diagnostic tool likely enables to assess peritoneal structure and function in these patients.

The importance of microalbuminuria in predicting patient outcome in a PICU.

OBJECTIVE: To evaluate the prognostic significance of microalbuminuria in critically ill children. DESIGN: Prospective study. SETTING: PICU of a teaching hospital. PATIENTS: Admitted critically ill children. INTERVENTIONS: The urine albumin-creatinine ratio was measured at admission and at 24 hours. Pediatric Risk of Mortality, Pediatric Index of Mortality II, Pediatric Logistic Organ Dysfunction, and Inotrope Score were calculated. MEASUREMENTS AND MAIN RESULTS: In total, 102 patients (median age, 19 mo) were included in the study, among whom were 30 mortalities. Microalbuminuria was identified in 62 patients (64%). The patients were classified into three groups: patients with sepsis, patients with noninfectious systemic inflammatory response syndrome, and patients without systemic inflammatory response syndrome. The highest clinical scores, albumin-creatinine ratio levels, mortality rate, and duration of mechanical ventilation were found in the sepsis group, and the lowest values were seen in patients without systemic inflammatory response syndrome (p < 0.05). Significant correlations were observed between the albumin-creatinine ratio levels and the clinical scores (p < 0.05). The receiver operating characteristics curve analysis showed that the areas under the curves were 0.818 and 0.781, respectively, for albumin-creatinine ratio measured at admission and at 24 hours to identify PICU mortality. At a cutoff value of 34.2 mg/g, albumin-creatinine ratio measured at admission may be able to discriminate between patients a with sensitivity of 63.3%, specificity of 93.3%, positive predictive value of 95%, and negative predictive value of 56%. CONCLUSIONS: Microalbuminuria is a simple, inexpensive, and useful tool for predicting mortality and morbidity in critically ill children in the PICU.

A single-center experience on percutaneously performed partial omentectomy in pediatric peritoneal dialysis patients.

OBJECTIVE: This study describes a single-center experience on percutaneously performed partial omentectomy procedure in pediatric peritoneal dialysis (PD) patients who showed early catheter dysfunction and required catheter replacement due to catheter flow obstruction. MATERIALS AND METHODS: We performed a retrospective review of clinical outcomes from pediatric PD patients who underwent percutaneous catheter replacement by pediatric nephrologists between November 1995 and December 2012. Partial omentectomy was performed in those patients in whom omental or adhesion trapping to the catheter tip was seen. RESULTS: During the study period, catheter dysfunction that eventually required percutaneous catheter replacement occurred in 32 (23.7%) children. Of these, 9 patients were performed partial omentectomy. Mean age at initiation of PD and time of omentectomy was 97.48 ± 46.06 and 98.53 ± 45.55 months, respectively. Catheter dysfunction appeared after a mean 1.20 ± 1.0 months. The causes of catheter dysfunction were omental wrapping and malposition. No peritonitis occurred before omentectomy. Mean total operation time was 60 ± 8.83 min. No complications were encountered during the procedure. After omentectomy, mean catheter survival period was 5.92 ± 6.88 months. A total of five peritonitis episodes occurred. Three patients were transferred to hemodialysis. Six patients were on PD treatment without any problem at the end of the first year of their follow-up. Two patients underwent kidney transplantation. Four patients were still on chronic PD treatment at the end of the study period. CONCLUSION: When performed by an experienced nephrologist, the performance of partial omentectomy by percutaneous route, when required, is an easy, safe and efficient therapeutic procedure in children on chronic PD treatment.

Val2Ala mutation in the Atp6v0a4 gene causes early-onset sensorineural hearing loss in children with recessive distal renal tubular acidosis: a case report.

A young female patient born to consanguineous parents was admitted to our clinic at the age of 3 years with a 5-month history of weight loss and recurrent urinary tract infections. Based on clinical findings (delayed growth and O-bein deformity) and laboratory tests (hypokalemia, hyperchloremia, partially compensated metabolic acidosis, alkaline urine and nephrocalsinosis), a diagnosis of distal renal tubular acidosis (dRTA) was made. Then, the audiogram revealed a bilateral sensorineural hearing loss (SNHL). On follow-up, bilateral SNHL progressively worsened requiring the need for hearing aid. The ATP6V0A4 gene mutation analysis showed homozygote Val2Ala mutation. To the best of our knowledge, this is the first report describing a Turkish girl with dRTA who suffered from early-onset SNHL caused by Val2Ala mutation in the ATP6V0A4 gene.

A retrospective analysis of long-term graft survival in 61 pediatric renal transplant recipients: a single-center experience.

BACKGROUND: Although short-term renal allograft survival in children has improved over the years, long-term graft outcomes remain unclear. In this study we report the characteristics and other variables that impact long-term kidney graft survival in children. MATERIAL AND METHODS: Records of 61 pediatric kidney transplant recipients (mean age: 14±3 years) performed at our institution between 1995 and 2011 were evaluated. Patients were divided into 2 groups (functional and non-functional grafts) to investigate the factors that impact graft survival. The groups were compared in terms of recipient characteristics, underlying disease, HLA status, immunosuppressive therapy, donor characteristics, acute rejection, and delayed graft function (DGF). Statistical significance was detected with the t and chi-squared tests (Pearson and Fisher's exact tests). Kaplan-Meier analysis was performed for graft survival. RESULTS: Overall graft survival at 1, 5, 10, and 15 years were 93%, 66%, 46%, and 41%, respectively. The median graft survival was 128.4 months (range: 3-188 months). Donor age, acute rejection, and DGF strongly predicted the chance of graft survival (p<0.05). CONCLUSIONS: It appears that several modifiable risk factors can partially account for poorer graft survival in pediatric kidney transplant recipients.

The effect of circumcision on the frequency of urinary tract infection, growth and nutrition status in infants with antenatal hydronephrosis.

The objective of this study was to determine the effect of circumcision on the frequency of urinary tract infection (UTI), growth development, and the nutrition status in infants with antenatal hydronephrosis (AH). The data were collected prospectively between 1998 and 2010. Infants with a fetal pelvis diameter of >5 mm identified with antenatal ultrasound were followed-up. Body height and weight were expressed as HZ scores (observed height - median height/standard deviation) and WZ scores (observed weight - median weight/Standard deviation). The nutritional status was evaluated and the body weight was transformed to a weight-for-height index (WHI = weight/median weight for the height age × 100). The HZ and WZ scores or WHI were calculated for each patient at the first and last visits. The chi-square and Student's t tests were used for statistical analysis. A p value <0.05 was considered significant. The study included 178 (134 males, 44 females) patients. Of these, 29 were diagnosed by vesicoureteral reflux (VUR), 87 by obstructive uropathy, and 54 by normal. Of 134 males, 111 infants were circumcised. The mean monitoring time was 45±24.9 months and the mean age of circumcision was 14 ± 16.06 months. The pre-circumcision UTI frequency (2.97 ± 1.14/y) was significantly higher than post-circumcision period (0.25 ± 0.67/y) (p < 0.05). Also, pre-circumcision UTI frequency (2.97 ± 1.14/y) was significantly higher than the UTI frequency observed in female cases (0.85 ± 0.91/y) and in the overall study group (0.73 ± 0.79/y) (p < 0.05). In all patients, the HZ of the circumcised subjects (0.18 ± 1.01) was statistically higher than uncircumcised subjects (-0.26 ± 0.92) (p < 0.05). Although statistically insignificant, the HZ of the circumcised males (0.13 ± 1.24) with VUR was higher than the uncircumcised patients (0.03 ± 0.55) (p > 0.05). In obstructive uropathy groups, the HZ of the circumcised males (-0.13 ± 0.54) was also found to be higher than uncircumcised males (-0.49 ± 0.66) (p < 0.05). Although nutrition scores were found to be better in circumcised males, no statistically significant effect of circumcision on the nutrition status was detected. In conclusion, postnatal early circumcision of infants with AH seems to prevent frequent UTIs and nutritional disturbances enabling normal growth.

An opportunistic pathogen in a peritoneal dialysis patient: Ochrobactrum anthropi.

The authors report a case of chronic peritoneal dialysis-related peritonitis from Ochrobactrum anthropi. O. anthropi is an emerging pathogen in immunocompromised patients. O. anthropi-related peritonitis in peritoneal dialysis patients has rarely been reported. To the authors' knowledge, no pediatric case of O. anthropi peritonitis has been reported to date in the literature.

Severe renal osteodystrophy in a pediatric patient with end-stage renal disease: Sagliker syndrome?

Renal osteodystrophy (ROD) is a multifactorial disorder of bone metabolism in chronic kidney disease (CKD). As CKD progresses, ensuing abnormalities in vitamin D metabolism and parathyroid hormone (PTH) secretion result in distortions in trabecular microarchitecture, thinning of the cortical shell, and increased cortical porosity. The recently described Sagliker syndrome (SS) might be an exaggerated version of ROD and is a very striking and prominent feature of secondary hyperparathyroidism in patients with end-stage renal disease (ESRD). It includes a distorted facial appearance, short stature, extremely severe maxillary and mandibulary changes, soft tissue tumors in the mouth, teeth/dental abnormalities, fingertip changes, knee and scapula deformities, hearing abnormalities, and neurologic and psychological problems. We herein describe an affected 14-year-old girl with severe ROD resulting from ESRD, who had severe peripheral and central neurologic problems caused by bone deformities, mimicking the features of Sagliker syndrome.