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Secondary amyloidosis with severe autonomic dysfunctions
1First Department of Internal Medicine, Kumamoto University School of Medicine, Japan.
Journal of the Autonomic Nervous System
|March 18, 1995
Summary
This study reports a case of secondary amyloidosis in a hemodialysis patient, presenting with severe gastrointestinal issues and widespread autonomic dysfunction, including reduced glandular secretions and cardiac MIBG uptake.
Area of Science:
- Nephrology
- Pathology
- Autonomic Neurology
Background:
- A 46-year-old male with end-stage renal disease due to glomerulonephritis required hemodialysis.
- The patient experienced severe diarrhea, prompting further gastrointestinal investigation.
Observation:
- Biopsies of the stomach and colon revealed significant amyloid deposition in the submucosal layers.
- Amyloid was identified as AA-protein, confirmed by antibody staining and solubility in potassium permanganate, indicating secondary amyloidosis.
Findings:
- Despite normal blood pressure, the patient exhibited profound glandular and visceral autonomic dysfunction.
- Key indicators included reduced lacrimal and salivary secretions, anhidrosis, decreased heart rate variability, and diminished cardiac uptake of MIBG (meta-iodobenzylguanidine).
Implications:
- This case highlights a rare presentation of secondary amyloidosis with extensive autonomic neuropathy in a hemodialysis patient.
- The findings underscore the importance of considering autonomic dysfunction in patients with chronic kidney disease and unexplained gastrointestinal symptoms.