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[Primary ureteral amyloidosis]
H Fernández1, A R Rovegno, R Marrugar
1Servicio de Urología, Centro de Educación Médica e Investigaciones Clínicas (C.E.M.I.C.), Buenos Aires, Argentina.
Archivos Espanoles De Urologia
|July 24, 1998
Summary
Primary ureteral amyloidosis is a rare cause of ureteral obstruction. Early diagnosis and characterization of amyloid proteins, like immunoglobulin lambda light chains, are crucial for appropriate management and avoiding unnecessary surgeries.
Area of Science:
- Nephrology
- Urology
- Pathology
Background:
- Primary ureteral amyloidosis is a rare condition.
- Ureteral amyloidosis can present as organic ureteral stenosis.
- Literature review and clinical classification of the disease are essential.
Observation:
- Two cases of difficult-to-diagnose organic ureteral stenosis were observed over 18 months.
- One patient underwent nephroureterectomy due to urinary tract compromise.
- The second patient, with a single kidney, received conservative surgery after preoperative diagnosis.
Findings:
- Histological analysis confirmed ureteral amyloidosis in the first case.
- Immunochemical study by Western Blot identified immunoglobulin lambda light chain fragments in both patients.
- This is the first reported instance of ureteral amyloidosis with immunochemical characterization of the amyloid protein.
Implications:
- Ureteral amyloidosis should be considered in the differential diagnosis of organic ureteral stenosis.
- Accurate diagnosis can prevent unnecessary extensive surgeries.
- Immunochemical characterization aids in understanding the disease and guiding treatment.