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.

Concomitant BK virus infection and visceral Leishmaniasis in a pediatric liver transplant recipient.

BACKGROUND: Solid organ transplant recipients are vulnerable to various unusual infections. Visceral Leishmaniasis (VL) is a protozoal opportunistic infection, which may affect the immune-suppressed hosts and solid organ transplant recipients. The BK virus infection is an evolving challenge in kidney transplant recipients. However, there are very few reports of BK virus (BKV) nephropathy involving the native kidney in liver transplant recipients. To the best of our knowledge, this is the first report of the simultaneous occurrence of these rare infections in a liver transplant recipient. CASE REPORT: The patient was a 9-year-old girl, a case of liver transplantation who presented with the incidental finding of proteinuria, azotemia, and cytopenia. Investigations revealed that she had concomitant BKV nephropathy and visceral leishmaniasis. Both infections were successfully treated. CONCLUSION: BK virus should be considered as a cause of nephropathy in liver transplant recipients. The presenting features of fever, cytopenia, and splenomegaly in a post-transplant patient should remind of unusual infections such as VL other than the common post-transplant conditions.

Acute necrotizing glomerulonephritis associated with COVID-19 infection: report of two pediatric cases.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is thought to cause kidney injury via a variety of mechanisms. The most common reported kidney injury following COVID-19 infection is acute tubular injury (ATI); however, the procoagulant state induced by the virus may also damage the kidneys. CASE-DIAGNOSIS/TREATMENT: Herein, we report two cases of acute necrotizing glomerulonephritis (GN) with fibrinoid necrosis in the context of COVID-19 infection. The one with more chronic features in the kidney biopsy progressed to permanent kidney failure but the second one had an excellent response to glucocorticoid pulse therapy with subsequent normal kidney function at 2-month follow-up. CONCLUSIONS: Both reported cases had an acute presentation of kidney injury with positive nasopharyngeal PCR test for COVID-19. Based on the data review by the researchers, this is the first report of acute necrotizing GN associated with COVID-19 infection.

Comparison of Appetite-regulating Hormones and Body Composition in Pediatric Patients in Predialysis Stage of Chronic Kidney Disease and Healthy Control Group.

BACKGROUND: Protein-energy malnutrition (PEM) is a common complication in pediatric patients with chronic kidney disease (CKD). Components incorporated in the regulation of appetite and body composition appear to be of the focus in renal insufficiency and may influence the CKD-associated PEM. The purpose of this study was to investigate plasma levels of appetite-regulating hormones and their correlation with the body composition variables in a pediatric in predialysis stage of CKD. METHODS: Thirty children with CKD in predialysis stage were selected and compared with 30 healthy sex- and age-matched controls. Blood samples were collected in fasting. Serum total ghrelin, leptin, and obestatin levels were measured using enzyme immunometric assay methods. Anthropometric parameters measurement and body composition analysis were done using the bioelectric impedance analysis (BIA) method. RESULTS: Patients showed insignificant elevated total ghrelin (105.40±30.83 ng/l), leptin (5.32±1.17 ng/ml) and obestatin (5.07±1.09 ng/ml) levels in comparison with healthy participants. By using BIA, patients had significantly different Dry Lean Weight (P=0.048), Extra Cellular Water (P=0.045), Body Cell Mass (BCM) (P=0.021), Basal Metabolic Rate (P=0.033) and Body Mass Index (P=0.029) compared with controls. Furthermore, the total body water was slightly and the ECW was significantly higher in CKD participants. There were significant negative correlation between obestatin and BCM (r=-0.40, P=0.03) and fat free mass index (FFMI) (r=-0.40, P=0.029) in patients. CONCLUSION: It seems that our results are insufficient to clarify the role of appetite-regulating hormones in PEM in CKD patients. It is apparent that there are still many unknown parameters related to both appetite regulating and CKD-associated PEM.

Exercise test in pediatric renal transplant recipients and its relationship with their cardiac function.

Pediatric kidney transplant recipients are at increased risk of CVD. Exercise test is a good method to evaluate exercise capacity, cardiorespiratory fitness, and risk of potential CVDs. The aim of this study was to assess the exercise capacity in this population and determine its relationship with their cardiac function using conventional and tissue Doppler echocardiography. Exercise test, conventional and tissue Doppler echocardiography were performed on 44 kidney transplant children (age ranging 11-20, 59% male) with acceptable renal function, and the results were compared with their normal healthy counterparts. Our transplant patients achieved significantly lower maximal heart rate, maximal heart rate ratio, total energy expenditure during the exercise, and maximal O2 consumption (Max VO2 ) than the normal group (p < 0.05). No correlation was found between hemoglobin (Hb) level, dialysis duration, kidney function, and the exercise test parameters. Kidney transplant patients had preserved systolic despite diminished diastolic cardiac function compared to the normal children. Our pediatric renal transplant recipients had severely impaired diastolic dysfunction and significantly reduced MaxVO2 compared with their healthy counterparts. No correlation was found between MaxVO2 and measured indices of systolic and diastolic cardiac function.

The evaluation of cisplatin effect on tubular function in children on chemotherapy.

The dose limiting side effect of cisplatin is nephrotoxicity. The aim of this study was to investigate tubular function in children who have received cisplatin and forced diuresis. We performed a cohort study on 20 children under 15 years of age with various type of malignancy on cisplatin-based chemotherapy. Twenty-four-hour urine was collected in three periods: before the first, third, and fifth doses of cisplatin administration to check urine for sodium (Na), magnesium (Mg), uric acid, creatinine (Cr), phosphorus (P), calcium (Ca), beta-2 microglobulin, and N-acetyl-beta-D-glucosaminidase (NAG) levels. At the same time, blood samples were taken to check serum Cr, Na, Mg, Ca, P, and uric acid levels. Then, we compared the mean of glomerular filtration rate (GFR); fraction excretions (FE,%) of Na, Mg, and uric acid; tubular phosphorous reabsorption (TPR,%), 24-hour urine Ca (mg); urine beta-2 microglobulin (mcg/mL); and NAG (IU/L) in three periods of cisplatin administration. The FE of Na, Mg, and urine beta-2 microglobulin increased after administration of cisplatin but TPR, FE, uric acid, and NAG decreased in the 2nd and 3rd period compared to 1st period. GFR revealed a little change that was not significant. Urine calcium was decreased significantly in the second and third periods of cisplatin administration. Since the patients were hydrated for forced diuresis and received magnesium sulfate to prevent nephrotoxicity, we did not see significant tubular dysfunction. But we saw that urine calcium excretion decreased after cisplatin injection without any change in serum calcium in spite of preventive measures.

Relationship between H. pylori infection and some other risk factors in the incidence and recurrence of idiopathic nephrotic syndrome in children.

Background: Idiopathic nephrotic syndrome (INS) is one of the chronic diseases in children and it is important to identify its related factors. The present study aimed at investigating the relationship between H. pylori infection and the incidence and recurrence of idiopathic nephrotic syndrome in children. Methods: The total number of case participants was 40 and the total number of control participants was 41. Based on the number of cases of nephrotic syndrome (NS), the same number of healthy children of the same age and gender were selected as the control group. The information and data collected include demographic characteristics of patients, duration of disease, number of recurrences, blood pressure and blood excretion in urine, height, and weight, and presence of gastrointestinal symptoms on the checklist. The data were entered into SPSS and analyzed at a significance level of 05. Results: From 81 participants in the study, 11 (13.75%) cases were H. pylori positive, of whom 7 (17.5%) cases were in the control group and 4 (10%) cases were in the patients' group. There was no significant difference between the two groups in terms of H. pylori infection rate (P = 0.863). Moreover, there were no significant differences between the patients suffering from the nephrotic syndrome in terms of height, weight, blood pressure, hematuria, duration and recurrence of the disease (p>0.05). Conclusion: According to the evaluations performed in the present study, there was no relationship between low birth weight, blood pressure, disease duration, and disease recurrence with NS.

Cohort Profile: Shiraz Pediatric Liver Cirrhosis Cohort (SPLCCS).

Liver diseases in children and adolescents are a significant and arising public health issue and should be surveyed from different dimensions (clinical and para-clinical, psychological, socio-economic) and in diverse populations. Shiraz Liver Transplant Center, Shiraz, Iran is the only center for pediatric liver transplantation and its pre-operative evaluations. This provides a unique and valuable situation for studying this vulnerable population. The Shiraz Pediatric Liver Cirrhosis Cohort Study (SPLCCS) was established to assess cirrhotic children, the course of their disease, and treatment over time. This cohort study aimed to prospectively evaluate the natural course and factors that contributed to complications and death of children with chronic liver disease in the region. SPLCCS was launched in September 2018 after obtaining ethical approval; until August 2022, 370 children with end-stage liver disease were enrolled and followed every six months. Here, the cohort's features, the included population's baseline characteristics, and primary outcomes are reported.

SMARCAL1 deficiency predisposes to non-Hodgkin lymphoma and hypersensitivity to genotoxic agents in vivo.

Schimke immuno-osseous dysplasia (SIOD) is a multisystemic disorder with prominent skeletal, renal, immunological, and ectodermal abnormalities. It is caused by mutations of SMARCAL1 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1), which encodes a DNA stress response protein. To determine the relationship of this function to the SIOD phenotype, we profiled the cancer prevalence in SIOD and assessed if defects of nucleotide excision repair (NER) and nonhomologous end joining (NHEJ), respectively, explained the ectodermal and immunological features of SIOD. Finally, we determined if Smarcal1(del/del) mice had hypersensitivity to irinotecan (CPT-11), etoposide, and hydroxyurea (HU) and whether exposure to these agents induced features of SIOD. Among 71 SIOD patients, three had non-Hodgkin lymphoma (NHL) and one had osteosarcoma. We did not find evidence of defective NER or NHEJ; however, Smarcal1-deficient mice were hypersensitive to several genotoxic agents. Also, CPT-11, etoposide, and HU caused decreased growth and loss of growth plate chondrocytes. These data, which identify an increased prevalence of NHL in SIOD and confirm hypersensitivity to DNA damaging agents in vivo, provide guidance for the management of SIOD patients.

Bone mineral disorders in pediatric and adolescent renal transplant recipients.

Incomplete resolution of abnormalities of mineral metabolism associated with CRF results in the relatively high prevalence of ROD in pediatric kidney recipients. This non-randomized, cross-sectional, and analytic-descriptive study on bone density, vitamin D, and mineral metabolism was performed in 57 children and adolescents who had received a total of 60 renal allografts in Shiraz, Iran. The height and weight of the patients were measured; their serum calcium (Ca), phosphorus (P), Alk-P, PTH, 25(OH)-vitamin D(3), BUN, creatinine, and electrolyte levels were analyzed, and a complete blood count was performed. In addition, standard radiologic bone assessments, which included conventional left hand-wrist radiography and bone mineral densitometry by the DXA technique, were carried out. Special pediatric software was used for age-related interpretation of the Z-scores of BMD. SPSS(®) software (version 15) was used for statistical analyses. We studied 57 patients (27 males [47.4%]) with a mean age of 18.7 ± 4.25 (9-27) yr and a mean age at transplantation of 13.1 ± 3.46 (4.5-20) yr. They had a post-transplantation follow-up of 67.1 ± 33.8 (6-132) months, and all had well-functioning allografts at enrollment. The mean height age of the patients was 11.9 ± 1.8 (6-15.5), and the mean bone age was 15.6 ± 3.3 (7-19) yr, which corresponded to mean height-age and bone-age retardations of 5.7 ± 2.3 (0.5-10.5) and 1.22 ± 1.47 (0-7) yr, respectively. Hyperphosphatemia and hypercalcemia were each found in nine patients (15.8%), hypophosphatemia in five (8.8%), and hypocalcemia in none of the patients. Seven out of 57 patients (12.3%) had a (Ca×P) product of more than 55 mg(2)/dL(2). Hyperparathyroidism was found in 27 (47.3%) and vitamin D(3) deficiency in four (7%) of the cases. The serum level of Alk-P was higher than the age-related normal range in 20 patients (35%). Left hand-wrist radiography showed no radiologic sign of ROD in any patient. The mean BMD Z-score was -1.77 ± 1.13 (-4.2-1.1) for the lumbar spine and -1.64 ± 0.89 (-3.9 to 1.9) for the femoral neck. "Stepwise backward regression" revealed a significant inverse correlation between the serum level of PTH and the GFR of the transplanted kidney; this correlation was independent from the influence of other variables such as Ca, P, and Alk-P (p = 0.011, ß = -1.556). Bone age and height age both showed significant correlations with age at transplantation and serum levels of P (p < 0.001), but only bone age had a meaningful correlation with Alk-P (p = 0.036). The BMD Z-scores showed statistically meaningful correlations with the serum level of Alk-P, which were independent from the influence of other variables such as Ca, P, and PTH (p ≤ 0.002). Our study revealed a relatively high prevalence of bone mineral disorder in pediatric kidney recipients, which suggests the need for a routine program for periodic screening of these patients to facilitate early diagnosis of either persistent or evolving manifestations of disturbed mineral metabolism, especially ROD.

Blood pressure profile in renal transplant recipients and its relation to diastolic function: tissue Doppler echocardiographic study.

Hypertension is a common complication after renal transplantation and is associated with increased risk of cardiovascular disease. The aim of the current study was to investigate the diurnal blood pressure pattern and its relation to structural and functional cardiac changes in renal transplant recipients. Sixty-six stable renal transplant patients (34 female, 32 male), aged 7 to 25 years (mean 17.4±4.3 years) were enrolled in this study. Cardiac function assessed by tissue Doppler echocardiography and blood pressure measurement performed using both the ambulatory and the casual method. Hypertension was demonstrated in 57% of recipients by the casual method and in 75.7% by ambulatory blood pressure monitoring (ABPM). The efficacy of BP control among patients on antihypertensive drugs was 60%. The prevalence of non-dipping was 73%. There was significant inverse correlation between systolic or diastolic day-time or night-time BP index and post-transplant duration (p<0.001, r=-0.386), but no correlation between ABP parameters and BMI, gender, and eGFR. There was a significant relationship between all ABP parameters and left ventricular mass index (LVMI) (p=0.025-0.007, r=0.28-0.38). LVMI was significantly higher in hypertensive than in normotensive cases (p=0.034). There was no difference in diastolic function between hypertensive and normotensive patients or between patients with and without left ventricular hypertrophy (LVH). In conclusion, our study showed the advantage of ABPM over the casual method of diagnosis of hypertension. LVH is common in transplant patients and is likely associated with arterial hypertension. Hypertension and LVH cannot differentiate transplant patients with diastolic malfunction.

Non-hodgkin lymphoma in a child with schimke immuno-osseous dysplasia.

Schimke immuno-osseous dysplasia is a rare autosomal recessive multisystem disorder characterized by steroid-resistant nephrotic syndrome, immunodeficiency, and spondyloepiphyseal dysplasia. Mutations in SWI/SNF2 related, matrix associated, actin dependent regulator of chromatin, subfamily a-like 1 (SMARCAL1) gene are responsible for the disease. The present report describes, for the first time, a Schimke immuno-osseous dysplasia child with SMARCAL1 missense mutation (R561H) and manifestations of intussusception secondary to Epstein-Barr virus-negative non-Hodgkin lymphoma, who expired due to septicemia following chemotherapy. The report emphasizes the necessity of more limited immunosuppressive protocols in Schimke immuno-osseous dysplasia patients with lymphoproliferative disorders.

ROHHAD Syndrome an Inconspicuous Cause of Hypernatremia, A Case Report.

Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation (ROHHAD) syndrome is a rare, life threatening disease with unknown etiology. Dysnatremia is a common finding in these patients. Here we present a 12-year-old boy with multiple admissions due to hypernatremia and was repeatedly misdiagnosed. An eventual diagnosis of ROHHAD syndrome was made by integration of the previous ignored findings of sleep apnea and obesity. The diagnosis of this rare but potentially fatal syndrome should be considered in patients with dysnatremia associated with obesity and sleep apnea disorders.

Outcome of Mini-Percutaneous Nephrolithotomy in Patients Under the Age of 18: An Experience With 112 Cases.

Purpose: Renal calculi are becoming more common among children. Although, extracorporeal shock wave lithotripsy (ESWL) is the first choice in this age group, minimal invasive surgeries, such as percutaneous nephrolithotomy (PCNL), are indicated for some patients. Recently, PCNL devices have become smaller in size with acceptable efficacy and lower complications. We evaluated the outcomes and complications of mini-PCNL (MPCNL) surgery in our referral training centers. Materials and Methods: Between September 2012 and January 2020, a total of 112 children under the age of 18, who had shown failure of ESWL, and/or their parents refused to do it, underwent MPCNL (15 Fr). The patients' profiles were reviewed for data collection including preoperative and stone data, operation information, and postoperative complications. Results: Of 112 patients, 69 were boys, and 43 were girls. Their mean age was 8.6 years (14 months to 18 years). Mean stone size was 20 mm (14-34 mm). Seventy-four cases had renal pelvic stone, 22 had pelvis and lower pole, and 16 had staghorn. The mean operation time was 65 min (35-100 min), and mean radiation time was 0.6 min (0.2-1.4 min). Low-grade fever was detected in 14 patients (12.5%). Four patients needed blood transfusion and two had increased creatinine, which improved with conservative management. One patient developed urosepsis that resolved with antibiotic therapy. None of the patients had kidney perforation or other organ injury or death. Early stone-free rate (SFR) after operation was 90.2% (101 patients). Six patients had residual fragment <5 mm, which passed spontaneously in 2 weeks after operation (total SFR 95.3%). Three patients underwent second-look nephroscopy, and ureteroscopy was done for two patients due to migrated stone fragments to the distal ureter. Conclusion: MPCNL is recommended as a safe alternative option for treatment of the nephrolithiasis in children with good outcome and acceptable complications.

Subclinical atherosclerosis and related risk factors in renal transplant recipients.

Long-term survival after successful renal transplantation is shortened by cardiovascular disease. Cardiovascular disease is a main cause of morbidity and death among children and young adults after renal transplantation. The aim of our study was to measure the carotid intima media thickness (cIMT) and determine its relationship to the risk factors for early arteriopathy in renal transplant recipients. Sixty-six stable renal transplant patients (36 female and 30 male), 7-25 years of age (mean 18.3 +/- 4.5 years) were enrolled in this study. The cIMT was measured by high-resolution B mode ultrasonography in multiple projections. The results were correlated with clinical and paraclinical parameters, including age, gender, body mass index (BMI), blood pressure, glomerular filtration rate (GFR), duration of dialysis, duration of chronic kidney disease (CKD), post-transplantation interval, calcium-phosphate (CaxP) product, cumulative dose of Ca-based P binder and calcitriol, lipid profile, uric acid, and cyclosporine level. The mean post-transplantation follow-up period was 64 +/- 40 months. The mean cIMT standard deviation score (SDS) of the patients and the control group was 0.60 +/- 0.81 mm (range -1.10 mm to 2.75 mm) and -1.25 +/- 0.95 mm (range -3.23 mm to 0.26 mm), respectively. Renal transplant recipients had a significantly greater cIMT than that of the controls (P < 0.001). Among several risk factors, there were positive correlations between cIMT SDS and gender, and cumulative dose of calcitriol (P = 0.02 and P = 0.02, respectively). In conclusion, subclinical atherosclerosis is present in young transplant recipients. Non-invasive monitoring of cIMT in renal transplant patients for the detection of early vascular lesions might be of value in preventing cardiovascular disease. Further studies are needed to see if proper monitoring of vitamin D therapy before and after transplantation could be helpful in the prevention of arteriopathy in renal transplant recipients.

Risk of nephropathy after consumption of nonionic contrast media by children undergoing cardiac angiography: a prospective study.

Despite increasing reports on nonionic contrast media-induced nephropathy (CIN) in hospitalized adult patients during cardiac procedures, the studies in pediatrics are limited, with even less focus on possible predisposing factors and preventive measures for patients undergoing cardiac angiography. This prospective study determined the incidence of CIN for two nonionic contrast media (CM), iopromide and iohexol, among 80 patients younger than 18 years and compared the rates for this complication in relation to the type and dosage of CM and the presence of cyanosis. The 80 patients in the study consecutively received either iopromide (group A, n = 40) or iohexol (group B, n = 40). Serum sodium (Na), potassium (K), and creatinine (Cr) were measured 24 h before angiography as baseline values, then measured again at 12-, 24-, and 48-h intervals after CM use. Urine samples for Na and Cr also were checked at the same intervals. Risk of renal failure, Injury to the kidney, Failure of kidney function, Loss of kidney function, and End-stage renal damage (RIFLE criteria) were used to define CIN and its incidence in the study population. Accordingly, among the 15 CIN patients (18.75%), 7.5% of the patients in group A had increased risk and 3.75% had renal injury, whereas 5% of group B had increased risk and 2.5% had renal injury. Whereas 33.3% of the patients with CIN were among those who received the proper dosage of CM, the percentage increased to 66.6% among those who received larger doses, with a significant difference in the incidence of CIN related to the different dosages of CM (p = 0.014). Among the 15 patients with CIN, 6 had cyanotic congenital heart diseases, but the incidence did not differ significantly from that for the noncyanotic patients (p = 0.243). Although clinically silent, CIN is not rare in pediatrics. The incidence depends on dosage but not on the type of consumed nonionic CM, nor on the presence of cyanosis, and although CIN usually is reversible, more concern is needed for the prevention of such a complication in children.

Prevalence of Renal Dysfunction Among Pediatric Liver Transplant Recipients.

INTRODUCTION: Renal dysfunction is among the common and considerable complications after liver transplantation and is principally attributable to immunosuppressive medications .The purpose of this study was to define the prevalence of hypertension and renal dysfunction among pediatric liver transplant recipients. METHODS: 46 pediatric liver transplant recipients were assessed for hypertension utilizing ambulatory blood pressure monitoring (ABPM), and glomerular, and tubular renal function at the transplant clinic of Shiraz University of Medical Sciences. Results were analyzed using SPSS 19 and P value < .05 was considered statistically significant. RESULTS: The mean age of the patients was 12.2 ± 3.3 years and 24 of them were female. Considering ABPM measurements 20 patients (43.5%) were hypertensive, 37% were systolic and 36.6% were diastolic non-dippers respectively. eGFR was calculated based on different formulations and Cystatin C-based equation estimated lower GFR than Cr-based equation. Micro-albuminuria was noticed in 26.1%. Additional parameters of tubular dysfunction included hyperuricosuria (4.3%), hypercalciuria (6.5%), abnormal fractional excretion of Mg (FeMg) (43.5%), abnormal tubular reabsorption of phosphate (TRP) (4.3%), and abnormal fractional excretion of uric acid (FEUA) in 13% of the patients. We noticed a statistically significant negative correlation between hypercalciuria, microalbuminuria, FeMg (P < .05) and GFR. CONCLUSION: Renal function impairment and hypertension are frequent complications amongst pediatric liver transplant recipients. Using Cyctatin C instead of Cr based formula for GFR estimation, and blood pressure monitoring by ABPM and regular screening of renal function are essential measures for recognition and treatment of renal dysfunction in these patients.