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Published on: June 20, 2018
Acute intermittent porphyria and chronic renal failure
J T Marsden1, P Chowdhury, J Wang
1Department of Clinical Biochemistry, King's College Hospital, London, UK. joanne.marsden@kch.nhs.uk
Clinical Nephrology
|June 10, 2008
Summary
Acute intermittent porphyria (AIP) patients show tubulointerstitial renal disease, not glomerular damage. Impaired erythropoietin production suggests porphyrin precursors may cause kidney issues.
Area of Science:
- Nephrology
- Biochemistry
- Genetics
Background:
- The link between acute porphyrias and renal damage is poorly understood.
- Previous research identified hypertension as a primary cause of kidney disease in these patients.
Observation:
- This study examined nine patients with acute intermittent porphyria (AIP) experiencing renal disease.
- Renal histology revealed tubulointerstitial disease, with no glomerular lesions observed.
- Impaired erythropoietin production was noted in the AIP patients.
Findings:
- Acute intermittent porphyria (AIP) is associated with tubulointerstitial nephritis, not glomerulopathy.
- Evidence suggests impaired erythropoietin production in AIP patients with renal disease.
- Hypertension and NSAID use were common comorbidities but may not be the sole causes.
Implications:
- Porphyrin precursors may have direct nephrotoxic effects, contributing to renal pathology in AIP.
- Understanding the pathogenesis of renal disease in AIP is crucial for patient management.
- Further research is needed to elucidate the mechanisms of kidney damage in acute porphyrias.
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