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Objectives: The objective was to evaluate the accuracy of pediatric emergency physician (EP) sonography for infants with suspected hypertrophic pyloric stenosis (HPS).Methods: This was a prospective observational pilot study in an urban academic pediatric emergency department (PED). Patients were selected if the treating physician ordered an ultrasound (US) in the department of radiology for the evaluation of suspected HPS.Results: Sixty-seven patients were enrolled from August 2009 through April 2012. When identifying the pylorus, pediatric EPs correctly identified all 10 positive cases, with a sensitivity of 100% (95% confidence interval [CI] = 62% to 100%) and specificity of 100% (95% CI = 92% to 100%). There was no statistical difference between the measurements obtained by pediatric EPs and radiology staff for pyloric muscle width or length (p = 0.5 and p = 0.79, respectively).

Uncontrolled or refractory nephrotic syndrome (NS), seen in a variety of glomerular disorders, leads to end-stage renal disease (ESRD). This study describes the use and efficacy of cyclosporine (CSA) for the treatment of refractory NS in 83 children seen over a 10-year period. The histological diagnosis leading to the NS was focal segmental glomerulosclerosis (FSGS) in 51% (n = 42), IgM nephropathy in 20% (n = 17), membranoproliferative glomerulonephritis in 10% (n = 8), lupus nephritis in 6% (n = 5), human immunodeficiency virus (HIV) nephropathy in 5% (n = 4), minimal change disease in 7% (n = 6), and membranous nephropathy in 1% (n = 1) of patients. During CSA therapy the mean proteinuria of the study population decreased from 5.14 g/24 h (4.80 g/m2 per 24 h) to 1.23 g/24 h (0.92 g/m2 per 24 h) (P < 0.001), the mean serum albumin increased from 2.13 g/dl to 3.53 g/dl (P < 0.001), the mean serum cholesterol decreased from 364 mg/dl to 223 mg/dl (P < 0.001), and the mean serum creatinine increased from 0.77 mg/dl to 1.2 mg/dl (P < 0.01). When analyzed by histological diagnosis, similar significant trends of reduction in proteinuria were seen in all but the lupus group. There was a rise in serum creatinine following the use of CSA in patients with FSGS, lupus nephritis, and HIV nephropathy; however the elevated serum creatinine was only significant in patients with FSGS. At the end of the study period, 20 patients had reached ESRD, of which 11 had FSGS, 5 had lupus nephritis, and 4 were patients with HIV nephropathy. Fifty-four patients were in remission at the end of the study period (48 with proteinuria < 100 mg/24 h and 6 with proteinuria < 500 mg/24 h). In conclusion, among children with refractory NS, CSA induced a remission in a large proportion. However toxicity, as noted by the rise in serum creatinine, was observed in several patients. Since this toxicity may be drug induced or a natural progression of the disease, careful monitoring and close follow-up are essential.

A recent review of our center experience revealed that only 38% of our pediatric renal transplants come from living-related donors (LRD), which is 11% lower than the national average. The present study was designed to identify factors that limit the availability of LRD in our population pool. Retrospective chart reviews and subsequent telephone interviews were conducted with parents of all children who received renal replacement therapy (RRT) at our institution from 1990 to 1999. The availability of parents and their willingness to donate a kidney were noted. Self-reported willingness was defined as the verbal expression of a desire to donate. Firm willingness was defined as the completion of the steps necessary for donation, unless excluded by the medical team. Factors that may impact the ability to donate, such as donor age, ethnicity, religion, educational attainment, employment, and presence of other siblings younger than 18 yr of age, were evaluated. Statistical analyses were performed using the Student's t-test and chi-square analysis. Significant results were entered into a single-step multiple regression analysis. Sixty children were identified with RRT, of whom 60% were Blacks, 30% Hispanics, 7% Caucasians, and 3% Asian. Fifty-five mothers were available for interview. Forty-four mothers reported a desire to donate, nine were unwilling to donate, and two were undecided. However, only 35 attended for screening. Only 30 fathers were available and, of these, 27 reported willingness to donate, yet only 20 attended for screening. Seventy-four per cent (26 out of 35) of mothers screened and 55% (11 out of 20) of fathers screened were medically unsuitable for kidney donation. Nineteen potential donors had hypertension, diabetes and/or obesity, seven had renal disease, four had anemia, two had hepatitis C, and five had other conditions. Expressed unwillingness to donate was associated with a greater number of children (3.1 compared to 1.5 children in addition to the child with end-stage renal disease [ESRD]) (odds ratio 2.91, p < 0.05) and employment (26.3% vs. 4.0%, p < 0.05) (odds ratio 31.2, p = 0.05). Comparing mothers who were firmly willing to donate with mothers who did not complete screening and evaluation, unwilling mothers had, likewise, a greater number of children (2.9 vs. 1.2 in addition to the child with ESRD) (odds ratio 3.23, p < 0.01) and a greater number of years of education (12.4 vs. 10.4) (odds ratio 2.14, p < 0.05). Hence, the availability of living kidney donors for our inner city children is severely limited by a high rate of single parenthood and a high rate of comorbid conditions in the parental donor pool. Furthermore, there is a diminished capacity of the available parent, particularly the mother, to donate as she tends to have numerous other dependents.

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