Complement activation and ADAMTS13 suppression may contribute to the characteristic pathological features in Upshaw-Schulman Syndrome
Not Applicable
- Conditions
- pshaw-Schulman syndrome
- Registration Number
- JPRN-UMIN000027482
- Lead Sponsor
- ara medical university
- Brief Summary
Chronic glomerular sclerotic changes in the majority of USS cases (4 of 5), with minor glomerular pathology in the remaining case. In two of these four severe cases, more than half of the glomerular segmental sclerosis area was localized in the perihilar region. The average number of ADAMTS13-positive cells per glomerulus was significantly lower in USS cases than controls (p < 0.05). C4d staining was significantly more prevalent in the glomerular capillary walls of USS cases than controls (p < 0.05).
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Complete: follow-up complete
- Sex
- All
- Target Recruitment
- 5
Inclusion Criteria
Not provided
Exclusion Criteria
Transplanted kidney
Study & Design
- Study Type
- Observational
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method Immunohistochemistry of ADAMTS13, C4d and C5b-9 of Upshaw-Schulman syndrome renal biopsies
- Secondary Outcome Measures
Name Time Method