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Background Nasopharyngeal carcinoma is rare in children and adolescents; however, it has a better prognosis than in adults. Nevertheless, its prognostic factors are not clearly documented. Patients and methods A prospective single-arm study was performed in 32 nonmetastatic Stages II–IV nasopharyngeal carcinoma (NPC) patients, below age 18 years. The treatment regimen included three courses of neoadjuvant chemotherapy (cisplatin and fluorouracil) followed by concomitant chemoradiotherapy (simultaneous integrated boost-intensity modulated radiotherapy [SIB-IMRT] with three cycles of cisplatin). Results All patients completed their prescribed treatment with tolerable acute side-effects. The 3-year overall (OS), event-free (EFS), local recurrence-free (LRFS), and distant metastasis-free (DMFS) survival rates were 83 ± 8, 77 ± 8, 92 ± 6 and 77 ± 9 percent, respectively. Although the median initial (pretreatment) gross tumor volume of the primary site (GTVp) increased with increasing stage, yet these differences did not rank to the level of significance. However, GTVp (with a cut-off value of 54.5 mL) was the only significant prognostic factor determining OS, EFS, LRFS and DMFS. On the other hand, age, gender, T stage, AJCC stage and footprint histopathological EBV did not have statistical significant impact on any survival rate. The late toxicity of this regimen was much lower than that reported with the conformal treatment. Conclusion Neoadjuvant chemotherapy and concomitant chemo-radiotherapy using SIB-IMRT led to excellent outcome with reduced late toxicity in juvenile nasopharyngeal carcinoma. GTVp is the sole prognostic factor that showed statistical impact on survival rates when applying this regimen.

Primary hyperoxalurias are rare inborn errors of metabolism with deficiency of hepatic enzymes that lead to excessive urinary oxalate excretion and overproduction of oxalate which is deposited in various organs. Hyperoxaluria results in serious morbid-ity, end stage kidney disease (ESKD), and mortality if left untreated. Combined liver kidney transplantation (CLKT) is recognized as a management of ESKD for children with hyperoxaluria type 1 (PH1). This study aimed to report outcome of CLKT in a pediatric cohort of PH1 patients, through retrospective analysis of data of 8 children (2 girls and 6 boys) who presented by PH1 to Wadi El Nil Pediatric Living Related Liver Transplant Unit during 2001-2017. Mean age at transplant was 8.2 ± 4 years. Only three of the children underwent confirmatory genotyping. Three patients died prior to surgery on waiting list. The first attempt at CLKT was consecutive, and despite initial successful liver transplant, the girl died of biliary peritonitis prior to scheduled renal transplant. Of the four who underwent simultaneous CLKT, only two survived and are well, one with insignificant complications, and other suffered from abdominal Burkitt lymphoma managed by excision and resection anastomosis, four cycles of rituximab, cyclophosphamide, vincristine, and prednisone. The other two died, one due to uncontrollable bleeding within 36 hours of procedure, while the other died awaiting renal transplant after loss of renal graft to recurrent renal oxalosis 6 months post-transplant. PH1 with ESKD is a rare disease; simultaneous CLKT offers good quality of life for afflicted children. Graft shortage and renal graft loss to oxalosis challenge the outcome.