PMC

A. Disease severity curves of medical record donated in Spring 2010 (red) and Spring 2011 (blue). The Spring 2010 case was assessed by four students. The Spring 2011 case was assessed by 30 students. The data were overlaid on disease severity curves obtained by Yanjanin et al., 2010. B. The progression slopes of two curves shown in panel A were determined by linear regression and overlaid onto the progression slope created by Yanjanin et al. 2010.

A. Five students independently scored one NP-C patient's medical record, The disease severity curves were plotted as shown. The correlation among their evaluation is 0.76. The best fit line is shown in black line with a slope of 1.315. In red is the best fit line (with a slope of 1.3 and a correlation of 0.90) obtained by Yanjanin et al. 2010. B. The average of five student scores (show in blue) was overlaid on a summary of disease severity curves obtained by Yanjanin et al 2010 (obtained by combining their historical and current cohorts). C. The progression slope shown in panels A and B was determined by linear regression and overlaid (blue dot) onto the progression slope created by Yanjanin et al. 2010.

The project began in Fall 2008 with discussions with the Center for Social Concerns at the University of Notre Dame, Riley Children's Hospital, Indianapolis, the National Niemann Pick Disease Foundation (NNPDF), the Ara Parseghian Medical Research Foundation (APMRF) and the Niemann-Pick Disease Group (UK), clinical practitioners at Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) and faculty and program staff the Center for Rare and Neglected Diseases (CRND) at the University of Notre Dame.

A. Disease severity curves generated by two different classes of sixteen students in Fall 2009 and Spring 2010. From each class groups of three to five students evaluated four NP-C patient's medical records obtained from Yanjanin et al. 2010. Each group evaluated one patient record. Error bars indicate scoring variation between the two classes. Linear best fit shown as solid black line for each record. Hatched line indicates best fit obtained by Yanjanin et al 2010. Color code: case 1, blue; case 2, red; case 3, purple; case 4 green; B. Disease severity curves generated by thirty students in Spring 2011. Five groups of six students scored the four NP-C medical records from panel A as well as three additional records. Scoring was done by pairs of students and triplicate scores were generated for each medical record as shown. Color code as indicated in panel A as well as case 5, magenta; case 6 orange; case 7, teal. Triplicate curves associated with each case, where each curve indicated pair wise scoring. C. The average of curves shown in panel B overlaid a summary of disease severity curves described in Yanjanin et al. 2010. D. The progression slope shown in panel C was determined by linear regression and overlaid onto the progression slope created by Yanjanin et al. 2010. Red arrows indicate extent of displacement of progression obtained from student assessments. Colors as indicated for panel C. When students' progression values were used, the progression slope was found to be 1.907 +/− 0.2374. The original progression slope calculated by Yanjanin et al. 1.923 +/− 0.2200.