UMIN-CTR Clinical Trial
| Unique ID issued by UMIN | UMIN000027482 |
|---|---|
| Receipt number | R000031482 |
| Scientific Title | Complement activation and ADAMTS13 suppression may contribute to the characteristic pathological features in Upshaw-Schulman Syndrome |
| Date of disclosure of the study information | 2017/06/01 |
| Last modified on | 2020/11/25 09:40:19 |
* This page includes information on clinical trials registered in UMIN clinical trial registed system.
* We don't aim to advertise certain products or treatments
Basic information
Public title
Complement activation and ADAMTS13 suppression may contribute to the characteristic pathological features in Upshaw-Schulman Syndrome
Acronym
Complement activation and ADAMTS13 suppression in USS
Scientific Title
Complement activation and ADAMTS13 suppression may contribute to the characteristic pathological features in Upshaw-Schulman Syndrome
Scientific Title:Acronym
Complement activation and ADAMTS13 suppression in USS
Region
| Japan |
Condition
Condition
Upshaw-Schulman syndrome
Classification by specialty
| Hematology and clinical oncology | Nephrology |
Classification by malignancy
Others
Genomic information
NO
Objectives
Narrative objectives1
To investigate the glomerular localization of ADAMTS13 and the commitment of complement activation of Upshaw-Schulman syndrome renal biopsies
Basic objectives2
Bio-availability
Basic objectives -Others
Trial characteristics_1
Trial characteristics_2
Developmental phase
Assessment
Primary outcomes
Immunohistochemistry of ADAMTS13, C4d and C5b-9 of Upshaw-Schulman syndrome renal biopsies
Key secondary outcomes
Base
Study type
Observational
Study design
Basic design
Randomization
Randomization unit
Blinding
Control
Stratification
Dynamic allocation
Institution consideration
Blocking
Concealment
Intervention
No. of arms
Purpose of intervention
Type of intervention
Interventions/Control_1
Interventions/Control_2
Interventions/Control_3
Interventions/Control_4
Interventions/Control_5
Interventions/Control_6
Interventions/Control_7
Interventions/Control_8
Interventions/Control_9
Interventions/Control_10
Eligibility
Age-lower limit
| Not applicable |
Age-upper limit
| Not applicable |
Gender
Male and Female
Key inclusion criteria
Severe deficient ADAMTS13 activity
(<10%), negative for ADAMTS13 inhibitor and mutations in the ADAMTS13 gene
Key exclusion criteria
Transplanted kidney
Target sample size
10
Research contact person
Name of lead principal investigator
| 1st name | Hiroe |
| Middle name | |
| Last name | Itami |
Organization
Nara medical university
Division name
Department of diagnostic pathology
Zip code
634-8521
Address
840 Shijo-Cho, Kashihara, Nara, Japan
TEL
0744-29-8910
hritami@naramed-u.ac.jp.
Public contact
Name of contact person
| 1st name | Hiroe |
| Middle name | |
| Last name | Itami |
Organization
Nara medical university
Division name
Department of diagnostic pathology
Zip code
634-8521
Address
840 Shijo-Cho, Kashihara, Nara, Japan
TEL
0744-29-8910
Homepage URL
hritami@naramed-u.ac.jp.
Sponsor or person
Institute
Nara medical university
Institute
Department
Personal name
Funding Source
Organization
Nothing
Organization
Division
Category of Funding Organization
Other
Nationality of Funding Organization
Other related organizations
Co-sponsor
Name of secondary funder(s)
IRB Contact (For public release)
Organization
Nara medical university
Address
840 Shijo-Cho, Kashihara, Nara, Japan
Tel
0744-29-8910
hritami@naramed-u.ac.jp.
Secondary IDs
Secondary IDs
NO
Study ID_1
Org. issuing International ID_1
Study ID_2
Org. issuing International ID_2
IND to MHLW
Institutions
Institutions
Other administrative information
Date of disclosure of the study information
| 2017 | Year | 06 | Month | 01 | Day |
Related information
URL releasing protocol
None
Publication of results
Published
Result
URL related to results and publications
https://doi.org/10.1016/j.thromres.2018.08.020
Number of participants that the trial has enrolled
5
Results
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).
Results date posted
| 2020 | Year | 11 | Month | 25 | Day |
Results Delayed
Results Delay Reason
Date of the first journal publication of results
Baseline Characteristics
Upshaw-Schulman syndrome (USS) is a congenital form of thrombotic thrombocytopenic purpura (TTP) associated with loss-of-function mutations in the ADAMTS13 gene, possibly leading to aberrant complement activation and vascular injury. However, USS is extremely rare, and there have been no systematic studies correlating histopathological severity with local ADAMTS13 expression and complement activation.
Participant flow
We compared histopathological features, ADAMTS13 immunoreactivity, and immunoreactivity of complement proteins C4d and C5b-9 among renal biopsy tissues from five USS cases, ten acquired TTP cases, and eleven controls.
Adverse events
None
Outcome measures
Immunohistochemistry(ADAMTS13, C4d, C5b-9)
Plan to share IPD
IPD sharing Plan description
Progress
Recruitment status
Completed
Date of protocol fixation
| 2017 | Year | 06 | Month | 01 | Day |
Date of IRB
| 2018 | Year | 03 | Month | 28 | Day |
Anticipated trial start date
| 2017 | Year | 06 | Month | 01 | Day |
Last follow-up date
| 2018 | Year | 06 | Month | 01 | Day |
Date of closure to data entry
| 2018 | Year | 06 | Month | 01 | Day |
Date trial data considered complete
| 2018 | Year | 06 | Month | 01 | Day |
Date analysis concluded
| 2018 | Year | 06 | Month | 01 | Day |
Other
Other related information
Nothing in particular
Management information
Registered date
| 2017 | Year | 05 | Month | 24 | Day |
Last modified on
| 2020 | Year | 11 | Month | 25 | Day |
Link to view the page
Value
https://center6.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000031482
