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The Pediatric Forum |

The Possible Link Between Erythropoietic Protoporphyria and Alport Syndrome

Carl F. Needles, MD
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Copyright 1998 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.

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Arch Pediatr Adolesc Med. 1998;152(12):1248-1248. doi:
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The June issue of the ARCHIVES features a "Pathologic Case of the Month" on erythropoietic protoporphyria and outlines the progressive damage that can be caused by an overabundance of type IV collagen.1 Repeated episodes lead to fibrogenic changes in the skin and, sometimes, to cirrhosis in the liver. I would like to ask whether this disease is mostly inflammation involving primarily the fibrosis limb of inflammation?

I would also like to suggest there may be a link to another disease that features type IV collagen abnormalities, ie, Alport syndrome. In that condition, basement membranes in glomeruli and other structures are abnormal and there may be progressive glomerular incapacity. Might this condition also involve recurrent bouts of remote inflammation and consequent fibrogenesis?

REFERENCES

Mather  MK, Sau  P. Pathological case of the month: erythropoietic protoporphyria. Arch Pediatr Adolesc Med. 1998;152603- 604

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Mather  MK, Sau  P. Pathological case of the month: erythropoietic protoporphyria. Arch Pediatr Adolesc Med. 1998;152603- 604

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