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2018 ; 11
(2
): 191-197
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Porphyria and kidney diseases
#MMPMID29644058
Pallet N
; Karras A
; Thervet E
; Gouya L
; Karim Z
; Puy H
Clin Kidney J
2018[Apr]; 11
(2
): 191-197
PMID29644058
show ga
The kidneys, after the bone marrow and liver, are third in terms of the amounts
of haem synthesized daily. Haem is incorporated into haemoproteins that are
critical to renal physiology. In turn, disturbances in haem metabolism interfere
with renal physiology and are tightly interrelated with kidney diseases. Acute
intermittent porphyria causes kidney injury, whereas medical situations
associated with end-stage renal disease, such as porphyrin accumulation, iron
overload and hepatitis C, participate in the inhibition of uroporphyrinogen
decarboxylase and predispose the individual to porphyria cutanea tarda. Even if
some of these interactions have been known for a long time, the clinical
situations associated with these interrelations have strikingly evolved over time
with the advent of new therapeutic strategies for dialysis therapy and a better
understanding of the pathophysiological mechanisms of porphyria-associated kidney
disease. Physicians should be aware of these interactions. The aim of this review
is to summarize the complex interactions between kidney physiology and pathology
in the settings of porphyria and to emphasize their often-underestimated
importance.
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