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Autosomal recessive polycystic kidney disease (ARPKD) is a one of the most common hereditary renal cystic diseases in children. Its clinical spectrum is widely variable with most cases presenting in infancy. Most affected neonates die within the first few hours of life. At present, prenatal diagnosis relies on fetal sonography, which is often imprecise in(More)
OBJECTIVE Our goal is to determine if antimicrobial prophylaxis with trimethoprim/sulfamethoxazole prevents recurrent urinary tract infections and renal scarring in children who are found to have vesicoureteral reflux after a first or second urinary tract infection. DESIGN, PARTICIPANTS, AND METHODS The Randomized Intervention for Children With(More)
The description of Frasier syndrome until now has been restricted to XY females with gonadal dysgenesis, progressive glomerulopathy, and a significant risk of gonadoblastoma. Mutations in the donor splice site in intron 9 of the Wilms' tumor (WT1) gene have been shown to cause Frasier syndrome and are distinct from WT1 exon mutations associated with(More)
OBJECTIVE To identify target areas for interventions to improve communication between pediatric generalists (PCPs) and pediatric subspecialists (SPs) in the outpatient care of children with chronic conditions. METHODS We constructed a 4-page mailed questionnaire probing communication practices, opinions about the role of communication in care, and(More)
The US pediatric nephrology workforce is poorly characterized. This report describes clinical and nonclinical activities, motivations and disincentives to a career in pediatric nephrology, future workforce needs, trainee recruitment, and possible explanations for personnel shortages. An e-mail survey was sent in 2013 to all identified US-trained or(More)
PURPOSE Pediatric patients require special attention from pediatric pharmacists. This is particularly true for pediatric patients with chronic kidney disease (CKD) as the number of their medications and the complexity of their treatment increase with disease progression. However, there is paucity of information describing pediatric cognitive pharmacy(More)
OBJECTIVE Effective communication between primary care and specialty physicians is essential for comanagement when children are referred to specialty care. We sought to determine rates of physician-reported communication between primary care physicians and specialists, the clinical impact of communication or its absence, and patient- and practice(More)
Eight children with autosomal recessive polycystic kidney disease (ARPKD) and recurrent bacteremia with enteric pathogens are described. Typical clinical features of bacterial cholangitis were absent, although in five patients histological and/or microbiological data indicated that the bacteremic episodes originated in the biliary tree. Bacteremia with(More)