CPKDRC logoCURRENT CPKDRC RESEARCHCPKDRC logo

HLA B40 as a Risk Factor for Hemolytic Uremic Syndrome (HUS)
A. Mackenzie, E. Orrbine, D. Sengar

HUS is the most common cause of sudden kidney failure in childhood. However, the cause of HUS following an Escherichia coli (Hamburger Disease) infection is still unknown. The purpose of this study is to look at the frequency of HLA B40 antigens (inherited characteristics similar to human blood groups), as a possible cause of risk factors for the progression of HUS following E. coli infection. Two earlier research studies have reported that the presence of the antigen HLA B40 may be a risk factor for progression to HUS as well as for severity of this disease. Information from this study will be extremely important in helping us to design future studies to prevent HUS.

The Use of Short-Acting Nifedipine for Acute Blood Pressure Reduction in Hospitalized Children
V. Yiu, F. Harley, P.N. McLaine, E. Orrbine, B. Pham, M. Gowrishankar, P. Goodyer, M. Ogborn, J. Midgley

Much controversy and debate has arisen over the use of short-acting (SA) nifedipine for the treatment of acute hypertension in adults based on publications finding an association between SA nifedipine and an increase in mortality and myocardial infarctions. As a result, both Health Protection Canada and the United States Food and Drug Administration have advised against the use of SA nifedipine for high blood pressure. In pediatric hypertensive patients, however, nifedipine is clearly continuing to be used due to its effectiveness and ease of administration. SA nifedipine is considered a standard medication for the treatment of acute hypertension by 100% of pediatric nephrologists surveyed. This study will attempt to address the safety issues of SA nifedipine use in children, a forgotten subgroup of hypertensive patients. The investigators hypothesize that SA nifedipine has few to no adverse effects in children and that it is an effective treatment for severe hypertension within this group.

Sensitivity and Specificity of ImmunoCard Stat test for the Detection of Enterohemorrhagic Escherichia coli O157:H7 in stools
A. MacKenzie, F. Chan, E. Orrbine, D. Hoban, W. Kennedy, D. Willis, L. Hyde, M. Benoit, and the CPKDRC Co-Investigators

This study examines the performance of the ImmunoCard Stat (ICS), Meridian Diagnostics Inc, a new diagnostic test for the presence of E. coli O157:H7 in stools. ICS incorporates antibodies to O and H antigens. A sensitivity of 85% observed in the direct test of stored specimens collected during the first 5 days of illness indicates excellent performance, and the inclusion of an incubation-amplification step, as recommended by the manufacturer, would probably increase this value. The ICS test will be useful as a rapid diagnostic (10 minutes) for the detection of E. coli O157:H7 in the clinical setting.

Nephropathic Cystinosis, Mutational Analysis
A. Mackenzie, J. Jordan, P. Goodyer, H. Heick, E. Orrbine, P.N. McLaine

This is a molecular genetic study designed to identify causative mutations in the recently identified cystinosis gene. A subsequent multi-site phase of the study will determine whether specific mutations result in associated clinical endpoints.