Ann P. Guillot

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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)
Sleep disorders have been shown to be more prevalent in adults and children with chronic kidney disease (CKD) and on dialysis. To date, the prevalence and impact of restless legs syndrome (RLS) in various stages of CKD has not been evaluated. The object of this study was to determine the prevalence of RLS in children with CKD in different stages, and to(More)
We investigated the safety and efficacy of magnesium hydroxide as a phosphate binder in patients with end-stage renal disease on maintenance hemodialysis, 9 volunteers participated in a four-phase study during which each ingested (1) no phosphate binders, (2) magnesium hydroxide (Mg(OH)2) alone, (3) Mg(OH)2 and aluminum hydroxide (A1(OH)3) together and (4)(More)
During a 5-year period, we evaluated seven infants and two fetuses who presented with enlarged, hyperechoic kidneys. In each, the initial clinical diagnosis was autosomal recessive polycystic kidney disease (ARPKD). Among the seven unrelated infants were three Caucasian and four African-American infants. No syndromic stigmata were evident in any of these(More)
PURPOSE To describe clinical skills progression during pediatric residency using the distribution of pediatric milestone assessments by subcompetency and year of training and to determine reasonable milestone expectations at time of graduation. METHOD Multi-institutional cohort study of the milestones reported to the Accreditation Council for Graduate(More)
Congenital nephrogenic diabetes insipidus (CNDI) is a rare X-linked disorder in which the renal collecting duct is unresponsive to arginine vasopressin, and thus, the urine is consistently hypotonic to plasma. As a result, affected individuals are unable to concentrate urine and suffer from episodes of severe dehydration and hypernatremia. Recently, the(More)
The angiograms were performed before placement of the retroaortic tissue expander (Panels A and D), with the tissue expander (shown in green) in place before final surgery (Panels B and E), and approximately 9 months after final surgery (Panels C and F). To the Editor: The midaortic syndrome is a rare entity, characterized by coarctation of the abdominal(More)
OBJECTIVE To determine incidence of learning goals by competency area and to assess which goals fall into competency areas with lower self-assessment scores. METHODS Cross-sectional analysis of existing deidentified American Academy of Pediatrics' PediaLink individualized learning plan data for the academic year 2009-2010. Residents self-assessed(More)
We describe a child with circulating anti-epithelial cell antibodies, autoimmune enteropathy with intestinal villous atrophy, and membranous glomerulonephritis. The patient had persistent diarrhea at 6 months of age, and a small bowel biopsy showed active enteritis, villous atrophy, and crypt hyperplasia. When the patient was, 10 months of age, nephrotic(More)