Evaluation of the relationship between serum carnitine levels and intradialytic complications in children with kidney failure.

BACKGROUND: Carnitine plays a crucial role in the metabolism of fatty acids as well as energy production. Previous research has suggested a significant decrease in carnitine levels in patients with kidney failure and those undergoing hemodialysis. Therefore, we designed this study to assess the prevalence and characteristics of carnitine deficiency and its association with hemodialysis complications in the pediatric population. METHODS: This research was a pilot study of 29 children undergoing hemodialysis. Before hemodialysis, a 5-mL blood sample was drawn from each patient through a peripheral vein to measure serum-free carnitine levels, complete blood count with differential, blood urea nitrogen (BUN), creatinine, and electrolytes. Each patient was observed for intradialytic complications, including muscle cramps and hypotension, during 12 sessions of hemodialysis. RESULTS: We included 26 participants with a mean age of 14.23 years undergoing hemodialysis. Carnitine deficiency was revealed in 54.8% of our participants. Also, there was no significant correlation between carnitine deficiency and age, gender, and BUN levels (P = 0.698, P = 0.43, and P > 0.05, respectively). Intradialytic complications, including episodes of hypotension and muscle cramps, were more frequent in patients with carnitine deficiency (P = 0.02, P = 0.01, respectively). Other reasons for muscle cramps, such as fluid overload, nutritional status, dialysis regimen, and other important lab results (phosphorus, magnesium, etc.), were ruled out. CONCLUSION: In conclusion, we found a higher prevalence of carnitine deficiency in pediatric hemodialysis patients. Carnitine deficiency was significantly associated with increased intradialytic symptoms, including muscle spasms and hypotension. Our results could support a potential role of carnitine supplementation in pediatric patients with kidney failure for controlling intradialytic complications, but this requires further investigation. A higher resolution version of the Graphical abstract is available as Supplementary information.

Efficacy of rituximab therapy in children with nephrotic syndrome: a 10-year experience from an Iranian pediatric hospital.

BACKGROUND: There are controversy results in the optimal management of children with steroid-dependent and steroid-resistant nephrotic syndrome (SDNS, SRNS). This study aimed to determine the efficacy and safety of rituximab (RTX) in these pediatric patients. METHODS: Medical records of 1-18-year-old Iranian children with SDNS (n = 26) and SRNS (n = 22) with a follow-up for at least 24 months were included from 2009 to 2019. The short- and long-term responses to RTX were respectively evaluated to determine the random protein-to-creatinine ratio after 6 and 24 months and classified as complete (CR) and partial (PR) remission or no response. RESULTS: Male patients (n = 26) were slightly predominate. The median age of patients at the time of RTX therapy was 8.6 ± 4.01 years. At the end of the 6-month follow-up, CR and PR occurred in 23 (47.9%) and 12 (25%) patients, respectively. Of 23 patients with CR, 18 (69.2%) and 5(22.7%) had SDNS and SRNS, respectively (p < 0.005). However, only 18 (37.5%) of patients after 24 months had been in CR. No significant difference in the CR rate was found between the two groups. RTX was more effective when administered during the proteinuria-free period (p = 0.001). CONCLUSION: In the short term, RTX significantly was efficient in inducing complete or PR in SDNS and SRNS patients. However, the favorable response rate in a long-term follow-up was insignificantly lower between the two groups.

Demographic, clinical, and laboratory factors associated with renal parenchymal injury in Iranian children with acute pyelonephritis.

BACKGROUND: The association between renal parenchyma changes on dimercaptosuccinic acid (DMSA) scans and demographic, clinical, and laboratory markers was assessed in pediatric patients with acute pyelonephritis. METHODS: A retrospective study of 67 Iranian babies and children aged 1-month to 12-year with APN was conducted between 2012 and 2018. The presence of renal parenchymal involvement (RPI) during APN was determined using technetium-99m DMSA during the first 2 weeks of hospitalization. The association of DMSA results with demographic data, clinical features (hospitalization stay, fever temperature and duration), and laboratory parameters such as pathogen type, and hematological factors (ESR, CRP, BUN, Cr, Hb, and WBC) was evaluated. RESULTS: 92.5% of children with an average age of 43.76 ± 5.2 months were girls. Twenty-four children (35.8%) did not have renal parenchymal injury (RPI), while 26 (38.8%) and 17 (25.4%) patients showed RPI in one and both kidneys, respectively. There was no significant association between RPI and mean ESR, CRP, BUN, and WBC. However, there were significant associations between RPI and higher mean levels of Cr, Hb, and BMI. CONCLUSIONS: Low BMI and Hb levels and increased Cr levels might be indicative of the presence of RPI in children with APN.

Clinical manifestations, laboratory markers, and renal ultrasonographic examinations in 1-month to 12-year-old Iranian children with pyelonephritis: a six-year cross-sectional retrospective study.

BACKGROUND: Upper urinary tract infection (UTI) or pyelonephritis may increase the pathogenesis rate and risk of severe complications in children due to kidney atrophy. OBJECTIVE: A set of clinical symptoms, laboratory markers, and ultrasound findings were assessed to achieve the early diagnosis and prognosis of pyelonephritis in hospitalized pediatrics. METHODS: A cross-sectional study with 104 Iranian children (95 girls and 9 boys) aged 1 month to 12 years with acute pyelonephritis during 2012-2018 was conducted. The ultrasound examination of kidneys and urinary tract during hospitalization, the incidence of clinical symptoms, and laboratory markers in blood and urine were monitored to identify the best predictive factors of early diagnosis of this bacterial infection. RESULTS: Three-fourth of the patients had one of the four clinical symptoms of abdominal pain, constipation, dysuria, and vomiting, while others were asymptomatic. A much frequency of pyuria (88.46%), Escherichia coli in urine (92.31%), leukocytosis (81.73%), and high ESR (> 10 mm/h, 92.30%) and CRP (> 10 mg/L, 82.82%) was observed. The kidney and urinary tract ultrasonography only in 32.7% of children revealed findings in favor of pyelonephritis (cystitis, ureteral stones, and hydronephrosis). CONCLUSION: There was a high frequency of clinical signs and laboratory markers associated with pyelonephritis. Ultrasound alone was not an efficient tool to track febrile UTI as most patients presented normal sonography.

Chronic Kidney Disease in Iran: First Report of the National Registry in Children and Adolescences.

PURPOSE: Knowing the epidemiological aspects of chronic kidney disease (CKD) in children is crucial for early recognition, identification of reversible causes, and prognosis. Here, we report the epidemiological characteristics of childhood CKD in Iran. MATERIALS AND METHODS: This cross-sectional study was conducted during 1991 - 2009. The data were collected using the information in the Iranian Pediatric Registry of Chronic Kidney Disease (IPRCKD) core dataset. RESULTS: A total of 1247 children were registered. The mean age of the children at registration was 0.69 ± 4.72 years (range, 0.25 -18 years), 7.79 ± 3.18 years for hemodialysis (HD), 4.24 ± 1.86 years for continuous ambulatory peritoneal dialysis (CAPD), and 3.4±1.95 years for the children who underwent the renal transplantation (RT) (P < .001). The mean year of follow-up was 7.19 ± 4.65 years. The mean annual incidence of CKD 2-5 stages was 3.34 per million age-related population (pmarp). The mean prevalence of CKD 2-5 stages was 21.95 (pmarp). The cumulative 1-, 5-, and 10-year patients' survival rates were 98.3%, 90.7%, and 84.8%, respectively. The etiology of the CKD included the congenital anomalies of the kidney and urinary tract (CAKUT) (40.01%), glomerulopathy (19.00%), unknown cause (18.28%), and cystic/hereditary/congenital disease (11.14%). CONCLUSION: The incidence and prevalence rate of pediatric CKD in Iran is relatively lower than those reported in Europe and other similar studies. CAKUT was the main cause of the CKD. Appropriate management of CAKUT including early urological intervention is required to preserve the renal function. Herein, the long-term survival rate was higher among the children with CKD than the literature.

Association of bone mineral density with biochemical markers of bone turnover in hemodialysis children.

INTRODUCTION: Although some descriptive and cross-sectional studies have been reported about bone mass in chronic kidney disease (CKD) children, only a few studies investigated markers of bone turnover and the bone mass measurements. OBJECTIVES: The aim of this study was to evaluate the association between bone mineral density (BMD) and biochemical markers of bone turnover in hemodialysis (HD) children. PATIENTS AND METHODS: The children who had received dialysis for at least the preceding 6-month were included. BMD was measured for total body, the lumbar spine and the femoral neck and the blood samples were tested to assess biochemical bone turnover markers. RESULTS: The study group was comprised of 27 patients with CKD, 9 males (33%) and 18 females (67%) with the mean±SD age of the subjects 14.9±4.5 years. Positive significant correlations of parathyroid hormone (PTH) with total body bone densitometry Z-score, lumbar spine and femoral neck Z-score(r=0.43, P=0.06; r=0.41, P=0.08 and r=0.45, P=0.05, respectively) was noted. In addition, positive significant correlations calcium and total body, lumbar spine and femoral neck Z-score (r=0.52, P=0.02; r=0.28, P=0.23 and r=0.36, P=0.12, respectively) was seen. Interestingly, a positive significant correlation between alkaline phosphatase (ALP) and lumbar spine Z-score was found (r=0.46, P=0.04), while the correlation of this parameter with total body and femoral neck Z-score was not significant (P>0.05). CONCLUSION: In our study, majority of patients with CKD had low level of BMD. In addition, lower levels of calcium (Ca), phosphorus (P), PTH and 25 (OH) vitamins D in patients with abnormal BMD Z-scores were detected.

A Comparison between Clinical and Metabolic Features of Renal Calyceal Microlithiasis and Overt Urolithiasis in Different Pediatric Age Groups.

INTRODUCTION: This study assesses the differences in the presentations, complications and metabolic abnormalities of children with renal calyceal microlithiasis (RCM) and overt urolithiasis in different pediatric ages. MATERIALS AND METHODS: A total of 465 children with urolithiasis were investigated retrospectively. Patients were categorized based on their ages to infancy, early childhood, middle childhood and adolescence. When the hyperechogenic spots on ultrasound imaging were <3 mm, they were considered RCM, and if they were ≥3 mm, they were considered overt urolithiasis. RESULTS: Metabolic abnormalities were detected in 71%; hyperuricosuria in infants, hyperoxaluria in younger children and hypocitraturia in older children were the most common metabolic abnormalities. Hypercalciuria was the only metabolic abnormality that was significantly associated with overt urolithiasis in all pediatric ages (OR 2.25, 95% CI 1.21-4.19). The clinical presentations were not significantly different between RCM and overt urolithiasis; however, complications such as urinary tract infection was significantly higher with overt urolithiasis in infancy (p = 0.01), early childhood (p = 0.02), middle childhood (p = 0.007) and adolescence (p = 0.01). Also, growth retardation was significantly higher with overt urolithiasis in infancy and early childhood (p = 0.02). CONCLUSIONS: Most children with urolithiasis have underlying urinary metabolic abnormalities that differ according to the child's age. Despite these differences, hypercalciuria is significantly associated with overt urolithiasis in all pediatric ages. Clinical and laboratory features cannot differentiate RCM and overt urolithiasis; however, complications are significantly higher with overt urolithiasis.

Comparison between fractional excretions of urea and sodium in children with acute kidney injury.

Fractional excretion of sodium (FENa) has been said to be the most sensitive index for differentiating prerenal failure (PRF) from intrinsic renal failure (IRF). However, there are several instances of high FENa (>2%) in cases of PRF and low FENa (<1%) in IRF patients. In contrast, the fractional excretion of urea nitrogen (FEUN) is primarily dependent on passive forces, and many confounding variables that affect FENa have little effect on FEUN, if any. To compare FEUN with FENa, pediatric patients with acute kidney injury (AKI) were prospectively evaluated by history, physical examination, and obtaining appropriate laboratory data during a 1-year interval. Diagnosis of PRF or IRF was made in each patient, and renal failure indices were compared between two groups using chi-square and t test, as appropriate. Probability value (P value) <0.05 was considered significant. Receiver operating characteristic (ROC) plots for FEUN and FENa were drawn to compare the discriminative power of each index. Forty-three patients were enrolled in the study. There were 27 patients in the PRF and 16 in the IRF group. FENa was 2+/-0.4 in PRF and 4.5+/- 1% in IRF patients (P<0.05), and low FENa (<1%) was only seen in 44.4% of PRF patients, which was not statistically different from those with IRF (P>0.05). FEUN was 23.6+/- 4.9% in PRF and 41.6+/-4.8% in IRF patients (P<0.05), and low FEUN (<35%) was seen in 77.8% of the PRF group (P<0.05). Cutoff values of 30% and 1.6% were reached for FEUN and FENa, respectively. In conclusion, FEUN <35% had higher sensitivity and specificity than FENa <1% for differentiation of PRF from IRF.