Primary hyperoxaluria type 1 (PH1) is a rare disease that is challenged by the overproduced oxalate and commonly presented with radiopaque renal stones or obstructive uropathy. This study aimed to report clinical presentations, renal replacement therapy (RRT), and outcome of PH1 in end stage kidney disease (ESKD) children. This is an observational cohort study. Data of 22 patients with ESKD due to PH1 were analyzed at Pediatric Nephrology Unit, Faculty of Medicine Cairo University. Infantile onset patients (n = 10) had worst renal outcome (80% with ESRD at presentation, p = 0.019) and worse patient outcome (mortality 40%, p = 0.016) than juvenile (n = 9) and late onset (PH1 n = 3) patients. RRT modalities include peritoneal dialysis (PD) in 7 (31.8%), hemodialysis (HD) in 11 (50%), and combined liver kidney transplantation (CLKT) in 4 (18.2%) patients. Infectious complications were encountered in 42.8% of PD patients. Better HD adequacy was observed with frequent HD (n = 6) and/or HD via arteriovenous fistula (AVF) than with infrequent dialysis (n = 5) and/or via central venous line (CVL) (p = 0.0001 and 0.0047, respectively). Morbidity and mortality (infection related) rates of the whole cohort were 63.6% and 31.8%, respectively. Clinical presentation of PH1 varies according to the age of onset (infantile onset being the most aggressive form). Aggressive HD (better through AVF) is needed to achieve acceptable HD adequacy, PD was challenged by infection. Infection found to be the main cause of mortality even after successful CLKT.

Introduction: Hearing impairment is frequent in patients with end-stage renal disease (ESRD). We aimed to assess the prevalence of hearing impairment in children on regular hemodialysis and renal transplantation. Materials and Methods: Transient-evoked otoacoustic emissions (TEOAEs) has been done for 80 children on regular hemodialysis and 40 with renal transplant.Results: In hemodialysis group, TEOAEs showed that 53.8% children had hearing affection, it was significantly related to dialysis duration, dialysis adequacy, vascular access infection, hepatitis C virus (HCV) infection, and ototoxic drugs (p = 0.001, 0.037, 0.011, 0.004, 0.030, 0.007, and 0.044, respectively). In renal transplant group hearing impairment was 25%. There was significant relation with period of dialysis before transplantation and biopsy proved rejection (p = 0.008, <0.001, respectively). Conclusion:Hearing impairment is a common finding in ESRD patients.

Background Neonatal sepsis is the major cause of morbidity and mortality in neonates. Frequency of bacterial sepsis is affected by seasonal variations. This study was done to rule out the effects of seasonal variation on neonatal sepsis and to determine isolated bacterial agents and their antibiotic sensitivity profile. Results This cross-sectional study included100 neonates with suspected sepsis, and conducted during summer and winter seasons. High frequencies of neonatal morbidities were observed like poor feeding (98%), Jaundice (82%), pallor (76%), respiratory distress (66%) and convulsions (64%) in winter, whereas fever (40%) and edema (12%) in summer. Statically significant correlation was found between the seasonal variations and different symptomatic expression of bacteremia like jaundice, respiratory distress, convulsions and poor feeding (p value < 0.001) in winter, while pallor, fever and edema (p value < 0.05) in summer. Our study showed high proportion of neonatal infection with Gram negative bacteria, as (40%) Pseudomonas aeruginosa and (16%) Escherichia coli in winter season, while (22%) Enterobacter in summer. Carbapenems was very effective against Pseudomonas aeruginosa isolates, Klebsiella pneumonia, Enterobacter, Acinetobacter, Escherichia coli, Staphylococcus aureus and Listeria, while theses bacterial isolates showed (80–100%) resistance to Unasyn and Cephalosporins (3rd generation). Conclusion This study showed that Seasonal variations had effects on neonatal sepsis regarding their both different symptomatic expressions and bacterial isolates. Most of isolates were sensitive to Carbapenems and resistant to both Unasyn and Cephalosporins (3rd generation).

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