Ann Guillot

The Use of Magnesium-Containing Phosphate Binders in Patients with End-Stage Renal Disease on Maintenance Hemodialysis

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) A1(OH)3 alone. Serum magnesium (SMg) concentrations were maintained at less than 4.5 mEq/1 (2.3 mmol/l) in all subjects while they were ingesting 0.75-3 g Mg(OH)2/day and no magnesium toxicity was noted. In individuals taking a constant daily dose, SMg remained stable over 8-12 weeks. Serum phosphorus (SP) decreased from 9.0 mg/dl (2.9 mmol/l)d during the control period to 8.1 mg/dl (2.6 mmol/l) during the Mg(OH)2 period (p less than 0.05) and increased from 6.1 mg/dl (2.0 mmol/l) during the Mg(OH)2 and A1(OH)3 period to 7.0 mg/dl (2.3 mmol/l) during the Al(OH)3 period (p less than 0.05) indicating that Mg(OH)2 could significantly lower SP. However, SP was best controlled (6.1 mg/dl; 2.0 mmol/l) when Al(OH)3 and Mg(OH)2 were used together and all participants preferred the combination therapy to either of the agents alone. These results indicate that Mg(OH)2 is a potentially useful adjunct to A1(OH)3 for managing hyperphosphatemia in patients on maintenance hemodialysis. In this short-term study Mg(OH)3 for managing hyperphosphatemia in patients on maintenance hemodialysis. In this short-term study Mg(OH)2 was well tolerated and with appropriate monitoring did not cause uncontrolled hypermagnesemia. Further studies are clearly required to determine whether long-term therapy with Mg-containing agents is safe in dialysis patients.

Diffuse renal cystic disease in children: morphologic and genetic correlations

Abstract. 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 infants. At the time of the initial evaluation, the family data were incomplete for four infants. The two fetuses were presumed to be at-risk for ARPKD based on the diagnosis in previous siblings. Renal histopathology was evaluated in all nine cases and revealed a spectrum of cystic disease ranging from ARPKD to glomerulocystic kidney disease to autosomal dominant polycystic kidney disease to diffuse cystic dysplasia. In the eight cases for whom liver histopathology was available, varying degrees of biliary dysgenesis were evident. We present a detailed analysis of the key histopathological features in each case and discuss the histopathological findings in an embryological context. In addition, we address the current role of molecular genetics in the diagnostic evaluation.

Restless legs syndrome in pediatric patients with chronic kidney disease

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 evaluate its impact on sleep and daytime functioning. We conducted a clinic-based or telephone survey of 26 patients in varying stages of CKD to assess for RLS, sleep schedule, and daytime sleepiness. Thirty-five percent of children met criteria for RLS, the majority being in CKD stages 1–4. There were no significant differences seen in sleep schedule and daytime sleepiness between those children with or without RLS. We found that there is an increased prevalence of RLS in children with CKD compared to the general population. This suggests that children with all stages of CKD should be routinely screened for RLS symptoms.

Anaphylactoid reaction to muromonab-CD3 in a pediatric renal transplant recipient.

Muromonab‐CD3 (OKT3), a murine IgG2a antibody directed against the T3 (CD3) complex on mature lymphocytes, triggers adverse immune reactions. Anaphylactic reactions have occurred in patients exposed to OKT3 and are mediated by anti‐OKT3 IgE antibodies. The reactions are not antibody mediated and can occur within seconds of administration of a mast cell secretogogue. A renal transplant recipient became hypotensive and hypoxic immediately after receiving her first dose of OKT3 and required advanced life support. Serum antibody tests were negative for anti‐OKT3 IgG, IgE, and antimouse protein antibodies. To our knowledge, this is the first published report of a patient with an anaphylactoid reaction to the initial infusion of OKT3.

Competent for Unsupervised Practice: Use of Pediatric Residency Training Milestones to Assess Readiness.

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 Medical Education for all 21 pediatric subcompetencies. Most subcompetencies were measured using five milestone levels (1 = novice, 2 = advanced beginner, 3 = competent, 4 = proficient, 5 = master); 3 subcompetencies had only four levels defined. Results Milestone assessments for 2,030 pediatric residents in 47 programs during academic year 2013–2014 were obtained. There was significant variation in end-of-year milestone ratings for residents within each level of training, which decreased as training level increased. Most (78.9%; 434/550) graduating third-year pediatric residents received a milestone rating of ≥ 3 in all 21 subcompetencies; fewer (21.1%; 116/550) received a rating of ≥ 4 in all subcompetencies. Across all training levels, professionalism and interpersonal communication skills were rated highest; quality improvement was rated lowest. Conclusions Trainees entered residency with a wide range of skills. As they advanced, skill variability within a training level decreased. Most graduating pediatric residents were still advancing on the milestone continuum toward proficiency and mastery, and an expectation of milestone ratings ≥ 4 in all categories upon graduation is unrealistic; milestone ratings ≥ 3 upon graduation may be more realistic. Understanding current pediatric residents’ and graduates’ skills can help to identify key areas that should be specifically targeted during training.

Identifying Gaps in the Performance of Pediatric Trainees Who Receive Marginal/Unsatisfactory Ratings.

Purpose To perform a derivation study to determine in which subcompetencies marginal/unsatisfactory pediatric residents had the greatest deficits compared with their satisfactorily performing peers and which subcompetencies best discriminated between marginal/unsatisfactory and satisfactorily performing residents. Method Multi-institutional cohort study of all 21 milestones (rated on four or five levels) reported to the Accreditation Council for Graduate Medical Education, and global marginal/unsatisfactory versus satisfactory performance reported to the American Board of Pediatrics. Data were gathered in 2013–2014. For each level of training (postgraduate year [PGY] 1, 2, and 3), mean differences between milestone levels of residents with marginal/unsatisfactory and satisfactory performance adjusted for clustering by program and C-statistics (area under receiver operating characteristic curve) were calculated. A Bonferroni-corrected significance threshold of .0007963 was used to account for multiple comparisons. Results Milestone and overall performance evaluations for 1,704 pediatric residents in 41 programs were obtained. For PGY1s, two subcompetencies had almost a one-point difference in milestone levels between marginal/unsatisfactory and satisfactory trainees and outstanding discrimination (≥ 0.90): organize/prioritize (0.93; C-statistic: 0.91) and transfer of care (0.97; C-statistic: 0.90). The largest difference between marginal/unsatisfactory and satisfactory PGY2s was trustworthiness (0.78). The largest differences between marginal/unsatisfactory and satisfactory PGY3s were ethical behavior (1.17), incorporating feedback (1.03), and professionalization (0.96). For PGY2s and PGY3s, no subcompetencies had outstanding discrimination. Conclusions Marginal/unsatisfactory pediatric residents had different subcompetency gaps at different training levels. While PGY1s may have global deficits, senior residents may have different performance deficiencies requiring individualized counseling and targeted performance improvement plans.