Protocolized polyoma BK viral load monitoring and high-dose immunoglobulin treatment in children after kidney transplant.

Background: BKPyV virus nephropathy (BKPyVAN) is diagnosed in 5%-16% of pediatric renal transplant recipients (PRTR) and preceded by BKPyV-viruria and DNAemia. Despite the risk of irreversible transplant damage associated with BKPyVAN, evidence-based consensus guidelines for BKPyVAN prevention are still lacking. In this retrospective study, we examined the safety and efficacy of high-dose intravenous immunoglobulin (HD-IVIG) therapy for prevention of BKPyVAN in PRTR with significant BKPyV-viruria/DNAemia. Methods: Between January 2013 and December 2022, all PRTR under our care underwent routine urine and blood testing for BKPyV viral load, using specific polymerase chain reaction (PCR). BKPyV DNAemia, with <103 copies/mL, with BKPyV viruria <107 copies/mL, with no evidence of BKPyVAN, were managed with 50% dose reduction of mycophenolate mofetil (MMF). Patients showing no decline in BKPyV viral load within two months of MMF dose reduction were managed with HD-IVIG (2 g/kg). Results: Seventy patients were recruited during a ten-year period and 31/70 patients (44%) demonstrated significant post-transplantation BKPyV-viruria/DNAemia, while 13/31 (42%) patients were unresponsive to MMF dose reduction, and were administered HD-IVIG. Of these, 12/13 (92%) patients achieved BKPyV viral clearance within six months from completion of HD-IVIG therapy and 1/13 patient (8%) was unresponsive to HD-IVIG therapy, showing increased BKPyV viral load. There were no major adverse events associated with HD-IVIG, and none of our patients developed BKPyVAN during the study period. Conclusions: Prophylactic HD-IVIG therapy in PRTR with significant BKPyV-viruria/DNAemia unresponsive to MMF dose reduction is safe and might be effective in preventing BKPyVAN. Our findings remain to be established by large-scale prospective studies.

Unusual presentation of a five-month-old boy with NaPi2a homozygous mutation without hyperphosphaturia: Case report and review of the literature.

Deletions of the NaPi2a gene and mutations in the SLC34A gene should be considered in patients with atypical presentation, without phosphaturia, with mild hypo to normal phosphatemia, and nephrocalcinosis.

One-month-old girl presenting with pseudohypoaldosteronism leading to the diagnosis of CDK13-related disorder: a case report and review of the literature.

BACKGROUND: It is not uncommon that an infant with a disease of unknown etiology is presented to a physician. Facial dysmorphic features lead to a different diagnosis. It is a challenge to link the presentation to the newfound diagnosis. CASE PRESENTATION: A 37-day-old Yemenite Jewish girl was presented to our institution with a clinical picture of pseudohypoaldosteronism due to abnormal facial features and a psychomotor developmental delay. Further investigation led to the diagnosis of CDK13-related disorder. According to the literature, CDK13 has a key role in the cell cycle, but no interference with the aldosterone signaling pathway or electrolyte balance was described. No mutations in the previously described gene NR3C2 (cytogenetic location 4q31.23), encoding the mineralocorticoid receptor, were found. Although the clinical presentation corresponded to pseudohypoaldosteronism type 1, we could not genetically confirm this. CONCLUSIONS: Probably pseudohypoaldosteronism was a coincidental finding in this girl with a CDK13 mutation, but because only limited information is known about CDK13-related disorders, further investigation could be more informative to clarify this presentation.

Antimicrobial Resistance Among Uropathogens That Cause Childhood Community-acquired Urinary Tract Infections in Central Israel.

In this retrospective study 829 positive urine cultures were analyzed. Escherichia coli bacterium was the leading uropathogen (86%). Almost 60% were resistant to ampicillin and first generation cephalosporins, and about 30% of them resistant to amoxicillin-clavulanic acid and trimethoprim-sulfamethoxazole. Almost none of them were resistant to second and third generation cephalosporins, aminoglycosides, ciprofloxacin or nitrofurantoin.

The Duration of Breastfeeding and Its Association with Metabolic Syndrome among Obese Children.

OBJECTIVE: The objective of this study was to evaluate whether duration of breastfeeding is associated with a lower prevalence of metabolic syndrome in obese children. METHODS: A retrospective analysis of obese children aged 3 to 18 years followed at a pediatric outpatient clinic at a single center between the years 2008 and 2012. The children were divided according to their breastfeeding duration: no breastfeeding, a short period of breastfeeding, and a long term breastfeeding. Also, they were divided into metabolic and nonmetabolic syndrome groups, based on physical examination and laboratory tests. RESULTS: Out of 4642 children who visited the clinic, 123 were obese and were included in the study. About half of them matched the metabolic syndrome criteria. There was no correlation between the prevalence of metabolic syndrome and the duration of breastfeeding. Hypertension, abnormal low levels of HDL, high levels of HbA1c, and high fasting triglyceride levels were very common in our study population, yet no statistical significance was noted among the different breastfeeding groups. CONCLUSION: In this study, breastfeeding was not associated with a reduced risk for metabolic syndrome, compared with formula feeding, in children who are obese.

[Asymptomatic classical hereditary xanthinuria type 1].

We report on a girl who was diagnosed with classical hereditary xanthinuria due to an incidental finding of extremely low Levels of uric acid in the blood. The girl is compLetely asymptomatic. Hereditary xanthinuria is a rare autosomal recessive disease that usually causes early urolithiasis but may cause rheumatoid arthritis-like disease and even be associated with defects in the formation of bone, hair and teeth. In Israel it has mostly been described in patients of Bedouin origin. Throughout the world, only about 150 cases have been described; about two thirds of these patients were asymptomatic. Since the clinical presentation and age of symptom appearance are diverse, the case raises questions as to the required follow-up of these patients and as to whether a low oxalate diet should be initiated.