Kimura disease presents as benign subcutaneous swelling predominantly around the head and neck region. It has a high incidence of renal involvement. However, the pathogenesis of this association remains elusive. Only 2 pediatric cases and 11 adult cases of Kimura disease with renal involvement have been reported in the literature. In recent years many immunopathogenetic features suggesting an underlying T-cell and related cytokine defect have been noted in Kimura disease. We describe a unique case of an Asian boy who presented with nephrotic syndrome resistant to steroid and cytotoxic therapy, and 5 years later developed cervical lymphadenopathy consistent with Kimura disease. We also review the literature, summarizing the presentation, differential diagnosis, incidence of renal disease, prognosis, immunopathogenetic features, and therapy.
Neonatal metabolic emergencies require a multidisciplinary team approach for supportive management that has increasingly come to feature renal replacement therapies in addition to nutritional support, the use of pharmaceutical agents, and testing to guide management and provide a definitive diagnosis. An increased appreciation for the mechanisms involved in ammonia neurotoxicity has placed greater emphasis on the need for its rapid yet safe resolution to optimize long-term prognosis. We examined our experience of intermittent hemodialysis (HD) and considered (1) the feasibility of HD in low-weight neonates, (2) the rate of decrease in ammonia, (3) complications during HD in small neonates weighing <4 kg presenting at University Children's Hospital between 1999 and 2002. Additionally, we review the current cellular and molecular mechanism of ammonia-induced brain injury. All patients tolerated intermittent HD and all required pressor agents. We primed all our patients with 20 U/kg of heparin and there was no subsequent need for further heparinization. We also noted that hemodynamic instability persisted during the first 1-2 h of the procedure and improved thereafter, as indicated by a decreased need for pressor agents. All neonates are alive to date. In conclusion, HD remains an effective and practical mode of renal replacement therapy having readily managed complications in preterm neonates weighing <4 kg with metabolic disorders.
Viral infections remain an Achilles heel in solid organ transplant. In recent years, incidence of BK virus infection in the kidney transplant population is on rise. BK virus is known to cause severe renal dysfunction, ureteric stenosis, and hemorrhagic cystitis in renal transplant patients. Most of the reviews and prospective studies on BKV nephropathy pertain to the adult population. Although ureteral stenosis is known to occur in BK infection, urethral stenosis is not being reported in the literature. In this report, we describe a case of BKV nephropathy in a 16-yr-old male presenting with ureteric and urethral stenosis. To our knowledge this is the first case report of its kind in a pediatric transplant population.
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