Validity of the Adrogué-Madias Formula for the Management of Acute Dysnatremias in Critically Ill Children: A Prospective Multicenter Analysis.

OBJECTIVE: Current conventional formulas do not predict the expected changes in serum sodium after administration of various fluids to correct serum sodium abnormalities. The Adrogué-Madias formula is currently the preferred and widely used fluid prescription for adult patients with dysnatremias, but its therapeutic efficacy has not been validated in pediatric patients. METHODS: In this prospective study, we used the Adrogué-Madias formula for calculating the appropriate rate of various fluids administration to correct serum sodium abnormalities in 7 critically ill children with acute dysnatremias. RESULTS: After administration of various intravenous fluids using the Adrogué-Madias formula, the anticipated as well as the achieved sodium concentrations were almost similar. CONCLUSIONS: This study demonstrates that the use of the Adrogué-Madias quantitative formula allows to calculate the appropriate rate of administration of various fluids. The calculated fluid administration resulted in the subsequent actual laboratory values and clinical changes.

Revisiting the Management of Pediatric Kidney Transplants, A Multicenter Analysis

The newest Kidney Disease Improving Global Outcomes (KDIGO) guideline recommendations were investigated mainly for the care of adult kidney transplant recipients, but no guideline exists for the management of pediatric transplant recipients. This review provides update recommendations in the management of pediatric kidney transplantation. Four electronic databases, PubMed, EMBASE, Google Scholar, and Web of Science were searched systematically for the last two decades, using Mesh terms in English language. The Grades of Recommendation Assessment, Development, and Evaluation (GRADE) approach was used for grading the quality of the overall evidence and the strength of recommendations for each outcome across the studies. The overall quality of evidence categorized as high (A), moderate (B), low (C), or poor (D). The strength of a recommendation was determined as level 1 (recommended) or level 2 (suggested). The ungraded statements were determined on the basis of common sense to provide general advice. Of the 317 citations which were screened for the evidence review, 62 were included in data extraction. The included studies were randomized controlled trials, prospective cohorts and cross-sectional, descriptive, and review studies. Of the 115 statements, 56 (48.6%) were graded 1 (we recommend), 34 (29.5%) were graded 2 (we suggest), and 25 (21.7%) were ungraded statements. Altogether, only 22 (19.1%) of recommendations reached the "A" or "B" levels of quality of evidence. The pediatric kidney transplant recipients are different from adult recipients regarding the primary kidney diseases, surgical techniques, drug metabolism, adherence to medications, growth and neurocognitive development and immunization needs prior to transplantation.  DOI: 10.52547/ijkd.7179.

New Biomarkers in Early Diagnosis of Acute Kidney Injury in Children.

Acute Kidney Injury (AKI) is a common condition with a high risk of mortality and morbidity, so, early diagnosis and management of AKI is very important in clinical practice. Despite significant progress in the management of AKI, it still carries high morbidity and mortality. BUN and serum creatinine are not very sensitive nor specific for the diagnosis of AKI because they are affected by many renal and non-renal factors that are independent of kidney injury or kidney function and change significantly only after significant kidney injury and with a substantial time delay. Detection of biomarkers of AKI made predominantly by the injured kidney tissue are essential for the early diagnosis of AKI. An ideal biomarker should be one that could be easily measured, with no interference with other biologic variables, and be able to clarify early phases of kidney damage. The most common biomarkers studied are Neutrophil Gelatinase-Associated Lipocalin (NGAL), Interleukin-18 (IL-18), Kidney Injury Molecule-1 (KIM-1), Cystatin-C, L type Fatty Acid-Binding Protein (L-FABP), N-Acetyl-ß-D Glucosaminidase (NAG), netrin-1, vanin-1, and Monocyte Chemoattractant Protein-1 (MCP-1) and calprotectin.

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.

The Effect of Oral Cyclophosphamide in the Treatment of Children with Refractory Idiopathic Nephrotic Syndrome.

INTRODUCTION: Nephrotic syndrome (NS) is the most common chronic kidney disease in children. Patients who do not respond to steroids are steroid resistance nephrotic syndrome (SRNS). Cyclophosphamide (CP) has been used in the treatment of SRNS, but its effectiveness has been questioned. The aim of this study was to evaluate the drug response and side effects of CP in the treatment of SRNS. METHODS: This study performed as a historical cohort (1997 to 2017) in idiopathic SRNS patients over one year of age who did not enter remission and used oral. All patients were followed up with CBC and regular visits to control drug side effects. RESULTS: In this study, 52 SRNS patients with a mean age of 5.3 ± 5.3 years were studied, of whom 24 (46%) were male and 22 (54%) were female. The follow-up period of patients was 1 to 264 months. In this study, 38.5% of patients were sensitive to CP and 61.5% of patients were resistant to CP. The response to CP was not significantly different between the ages of higher 6 years and under (P > .05). There was no significant relationship between remission rate and type of pathology and CP addition to treatment. But there was a significant difference between ESRD and CP resistance. CONCLUSION: It can be concluded that CP has no significant effect on the remission of SRNS patients, but has made a significant difference in the development of ESRD in patients.

Urinary Neutrophil Gelatinase-Associated Lipocalin (NGAL) and Cystatin C in Early Detection of Pediatric Acute Kidney Injury; a Diagnostic Accuracy Study.

INTRODUCTION: There is a controversy regarding accuracy of neutrophil gelatinase-associated lipocalin (NGAL) and Cystatin C in early detection of acute kidney injury (AKI). The present study aimed to compare the diagnostic value of two biomarkers in this regard. METHOD: In the present diagnostic accuracy study, all children between the ages of 1 month to 14 years were entered. Pediatric Risk, Injury Failure, Loss, End-stage renal disease (pRIFLE) criteria was used for identification of children with AKI as the reference test. Blood samples were taken from all patients at baseline and 48 hours after admission to assess serum creatinine and Cystatin C level. In addition, a urine sample was obtained within 6 hours of admission in order to measure NGAL level. In the end, area under the receiving operating characteristics (ROC) curve, sensitivity, and specificity of urine NGAL (uNGAL) and Cystatin C in early detection of AKI were compared. RESULTS: Data from 96 children with the mean age of 27.31±36.24 months were entered (56.25% girls). Area under the ROC curve of uNGAL level in diagnosis of AKI in children was 0.91 (95% CI: 0.80 to 1.00) and area under the ROC of Cystatin C was 0.90 (95% CI: 0.77 to 1.00). Both tests had the same value in diagnosis of AKI (p=0.89). The best cut-off point of uNGAL for diagnosing AKI was 125 mg/L. uNGAL had a sensitivity and specificity of 0.92 (0.62 to 0.99) and 0.69 (0.57 to 0.78), respectively. The best cut-off point of serum Cystatin C level was 0.4 mg/L. Cystatin C had a sensitivity and specificity of 0.92 (0.62 to 0.99) and 0.64 (0.52 to 0.74), respectively. CONCLUSION: The present study showed that uNGAL level has the same value as serum Cystatin C level in early diagnosis of AKI.

Angiotensin-Converting Enzyme Gene Polymorphism in Children with Idiopathic Nephrotic Syndrome, Effect on Biopsy Findings.

OBJECTIVE: Angiotensin converting enzyme (ACE) converts angiotensin I into angiotensin II. The ACE gene shows an I/D polymorphism, which correlates with ACE concentrations. The aim of this study is to evaluate the distribution of the ACE I/D genotype in children with idiopathic nephrotic syndrome (INS) and healthy controls and study the effect of this polymorphism on clinical and pathologic findings. METHODS: ACE gene I/D polymorphism of 104 patients with INS and 119 controls were determined. RESULTS: The DD, ID, and II genotypes were found in 58.7%, 22.1%, and 19.2% of the patients, and in 79.8%, 2.5%, and 17.6% of controls, respectively (p > 0.05). The ID genotype was seen more frequently in patients resistant to treatment. CONCLUSION: The observed differences with previous reports suggest the influence of the genetic background on disease course. The ACE I/D gene polymorphism's role seems to be more important in renal disease progression than susceptibility.

Accuracy of cystatin C in prediction of acute kidney injury in children; serum or urine levels: which one works better? A systematic review and meta-analysis.

BACKGROUND: There is still an ongoing discussion on the prognostic value of cystatin C in assessment of kidney function. Accordingly, the present study aimed to conduct a meta-analysis to provide evidence for the prognostic value of this biomarker for acute kidney injury (AKI) in children. METHODS: An extensive search was performed in electronic databases of Medline, Embase, ISI Web of Science, Cochrane library and Scopus until the end of 2015. Standardized mean difference (SMD) with a 95% of confidence interval (95% CI) and the prognostic performance characteristics of cystatin C in prediction of AKI were assessed. Analyses were stratified based on the sample in which the level of cystatin C was measured (serum vs. urine). RESULTS: A total of 24 articles were included in the meta-analysis [1948 children (1302 non-AKI children and 645 AKI cases)]. Serum (SMD = 0.96; 95% CI: 0.68-1.24; p < 0.0001) and urine (SMD = 0.54; 95% CI:0.34-0.75; p < 0.0001) levels of cystatin C were significantly higher in children with AKI. Overall area under the curve of serum cystatin C and urine cystatin C in prediction of AKI were 0.83 (95% CI: 0.80-0.86) and 0.85 (95% CI: 0.81-0.88), respectively. The best sensitivity (value = 0.85; 95% CI: 0.78-0.90) and specificity (value = 0.61; 95% CI: 0.48-0.73), were observed for the serum concentration of this protein and in the cut-off points between 0.4-1.0 mg/L. CONCLUSION: The findings of the present study showed that cystatin C has an acceptable prognostic value for prediction of AKI in children. Since the serum level of cystatin C rises within the first 24 h of admission in patients with AKI, this biomarker can be a suitable alternative for traditional diagnostic measures.

Height-adjusted percentiles evaluated central obesity in children and adolescents more effectively than just waist circumference.

AIM: We compared the prevalence of obesity based on both waist circumference for height and body mass index (BMI) in Iranian children and adolescents. METHODS: Data on 13 120 children with a mean age of 12.45 ± 3.36 years (50.8% male) from the fourth Childhood and Adolescence Surveillance and Prevention of Adult Non-communicable Disease study were included. Measured waist circumference values were modelled according to age, gender and height percentiles. The prevalence of obesity was estimated using the 90th percentiles for both unadjusted and height-adjusted waist circumferences and compared with the World Health Organization BMI cut-offs. They were analysed further for short, average and tall children. RESULTS: Waist circumference values increased steadily with age. For short and average height children, the prevalence of obesity was higher when height-adjusted waist circumference was used. For taller children, the prevalence of obesity using height-adjusted waist circumference and BMI was similar, but lower than the prevalence based on measurements unadjusted for height. Height-adjusted waist circumference and BMI identified different children as having obesity, with overlaps of 69.47% for boys and 68.42% for girls. CONCLUSION: Just using waist circumference underestimated obesity in some Iranian children and measurements should be adjusted for height.

Age-Period-Cohort Analysis of Obesity and Overweight in Iranian Children and Adolescents.

BACKGROUND: To date, few studies looked upon obesity and overweight in children and adolescents through the 3 different temporal dimensions of age, period, and cohort. The current study aimed at evaluating the trends of these health issues among children under 19 years old using the age-period-cohort (APC) analysis. METHODS: Data gathered through 5 cross sectional studies including 2 national health surveillance (1990 - 91 and 1999), and 3 CASPIAN surveys (2003, 2009, and 2011). Subjects were classified by their body mass index (BMI) into 3 groups of normal (BMI < 85th percentile), overweight-obese (85th percentile < BMI < 95th percentile), and obese (95th percentile < BMI). Intrinsic estimator method was used to analyze the effects of age, period, and birth cohort on obesity and overweight among the subjects. RESULTS: A total of 80,698 children and adolescents under 19 years old, including 40,419 (50.09%) males and 40,279 (49.91%) females, were evaluated. The prevalence of obesity decreased progressively by age in males and females with minor discrepancies. It increased from 1990 to 2009 in both genders, but from that point on remained quite constant in males and dropped significantly in females. The prevalence of obesity was steady in earlier birth cohorts, but increased significantly after the birth cohorts from 1986 to 1990. CONCLUSIONS: Environmental factors and social stresses during neonatal and infantile periods (birth cohort effect) along with other variables influencing the children later in their lives (period effect) affect the prevalence of overweight and obesity substantially. Moreover, a decrease in the prevalence of obesity and overweight was observed by age increase (age effect).

Methylmalonic acidemia with emergency hypertension / Acidemia metilmalónica con hipertensión de emergencia

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Early detection of acute kidney injury by serum cystatin C in critically ill children.

BACKGROUND: We prospectively evaluated whether serum cystatin C (CysC) detected acute kidney injury (AKI) earlier than basal serum creatinine (Cr). METHODS: In 107 pediatric patients at high risk of developing AKI, serum Cr and serum CysC were measured upon admission. Baseline estimated creatinine clearance (eCCl) was calculated using a CysC-based glomerular filtration rate (GFR) equation from a serum Cr measured at the pediatric intensive care unit (PICU) entrance. RESULTS: The median age was 10 months (interquartile range, 3-36 months). Serum Cr, serum CysC, and eCCl (mean ± standard deviation [range]) were 0.5 ± 0.18 mg/dl (0.2-1.1 mg/dl), 0.53 ± 0.78 (0.01-3.7 mg/l), and 72.55 ± 28.72 (20.6-176.2) ml/min per 1.73 m(2), respectively. The serum CysC level in patients with AKI was significantly higher than children with normal renal function (p < 0.001). The values for the cut-off point, sensitivity, specificity, and the area under curve (AUC) were determined for CysC as 0.6 mg/l, 73.9 %, 78.9 %, and 0.92 [95 % confidence interval (0.82-1)], respectively, and for Cr the values were 0.4 mg/dl, 68 %, 46.2 %, and 0.39, [95 % confidence interval (0.24-0.54)], respectively. The receiver operating characteristics (ROC) curve analysis revealed that CysC had a significantly higher diagnostic accuracy than eCCl (p < 0.001). CONCLUSIONS: Our results identify that the sensitivity of serum CysC for detecting AKI is higher than that of serum Cr in a heterogeneous pediatric intensive care unit (PICU) population.

Novel NPHS1 Gene Mutation in an Iranian Patient with Congenital Nephrotic Syndrome of the Finnish Type

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