Fibrillary Glomerulonephritis Confirmation, Mass Spectrometry, Paraffin Tissue
Use
Fibrillary glomerulonephritis (FGN) is a rare kidney disease characterized by fibrillary deposits in the glomeruli that include polyclonal IgG and complement, suggesting immune complex deposition. This test is useful in diagnosing FGN as it uses liquid chromatography tandem mass spectrometry (LC-MS/MS) to identify a unique proteomic profile that includes the protein DNAJB9. The presence of DNAJB9 is highly sensitive and specific for FGN, allowing for differentiation from other glomerular diseases such as amyloid, immunotactoid glomerulopathy, and immune complex-mediated proliferative glomerulonephritis. Accurate diagnosis of FGN can guide clinical and pathological management.
Special Instructions
Preliminary pathology report and history are required. A brief explanatory note or consultative letter is recommended with each submission. No fixed tissue slides should be sent, as testing can only be performed on paraffin-embedded tissue blocks. Attach the green pathology address label included in the kit to the outside of the transport container.
Limitations
There are no significant diagnostic cautions mentioned. However, improper specimen collection or handling, inappropriate test selection, or interfering substances could potentially cause diagnostic confusion. The test's accuracy is reliant on the proper handling of the paraffin-embedded blocks and the preservative methods used. Cases demonstrating weak Congo-red positivity may pose a challenge in distinction from amyloid.
New York Approved
Methodology
Mass Spectrometry (LC-MS/MS)
Biomarkers
LOINC Codes
- 59465-5
- 19139-5
- 81178-6
- 62364-5
- 80398-1
Result Turnaround Time
7-15 days
Related Documents
For more information, please review the documents below
Specimen
Tissue (FFPE)
Volume
Not provided
Minimum Volume
Not provided
Container
Paraffin-embedded tissue block
Collection Instructions
1. Do not send fixed tissue slides. Testing can only be done on paraffin-embedded tissue blocks. 2. Attach the green pathology address label included in the kit to the outside of the transport container.
Causes for Rejection
Reject fixed tissue slides, wet or frozen tissue, cytological smears, nonformalin fixed tissue, nonparaffin embedded tissue.
Stability Requirements
| Temperature | Period |
|---|---|
| Room Temperature | Ambient (preferred) |
| Refrigerated | Refrigerated (acceptable) |