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Renal function in familial amyloidosis with polyneuropathy
Acta Medica Scandinavica
|January 1, 1982
Summary
Familial amyloidosis with polyneuropathy (FAP) type 1 impairs kidney function, affecting glomerular clearance and urine concentrating ability. Amyloid deposition in glomeruli is the likely cause of this renal dysfunction.
Area of Science:
- Nephrology
- Genetics
- Internal Medicine
Background:
- Familial amyloidosis with polyneuropathy (FAP) type 1 is a hereditary condition.
- Renal function is often presumed normal in FAP type 1 patients.
- Early detection of renal involvement is crucial for patient management.
Purpose of the Study:
- To investigate renal function in patients with FAP type 1.
- To assess glomerular clearance and urinary concentrating capacity.
- To determine the underlying cause of renal dysfunction in FAP type 1.
Main Methods:
- Studied 24 patients diagnosed with FAP type 1.
- Measured urinary concentrating capacity.
- Assessed glomerular filtration using 51Cr-EDTA clearance.
- Performed urine electrophoresis to analyze proteinuria.
Main Results:
- Most patients exhibited impaired glomerular clearance and reduced urinary concentrating capacity.
- A significant correlation was observed between concentrating capacity and clearance values.
- Urine electrophoresis suggested a glomerular origin for proteinuria.
Conclusions:
- Renal dysfunction is common in FAP type 1 patients.
- Amyloid substance deposition in the glomeruli is the probable cause of renal impairment.
- Kidney function assessment should be a routine part of FAP type 1 management.