H. Puy, L. Gouya, J. C. Deybach, and . Porphyrias, Lancet, vol.375, pp.924-937, 2010.

M. Siegesmund, A. M. Van-tuyll-van-serooskerken, and P. Poblete-gutierrez, The acute hepatic porphyrias: current status and future challenges, Best Pract Res Clin Gastroenterol, vol.24, pp.593-605, 2010.

G. Elder, P. Harper, and M. Badminton, The incidence of inherited porphyrias in Europe, J Inherit Metab Dis, vol.36, pp.849-857, 2013.

Y. Nordmann, H. Puy, D. Silva, and V. , Acute intermittent porphyria: prevalence of mutations in the porphobilinogen deaminase gene in blood donors in France, J Intern Med, vol.242, pp.213-217, 1997.

A. L. Herrick and K. E. Mccoll, Acute intermittent porphyria, Best Pract Res Clin Gastroenterol, vol.19, pp.235-249, 2005.

K. E. Anderson, J. R. Bloomer, and H. L. Bonkovsky, Recommendations for the diagnosis and treatment of the acute porphyrias, Ann Intern Med, vol.142, pp.439-450, 2005.

C. Andant, H. Puy, and C. Bogard, Hepatocellular carcinoma in patients with acute hepatic porphyria: frequency of occurrence and related factors, J Hepatol, vol.32, pp.933-939, 2000.

C. Andant, H. Puy, and J. Faivre, Acute hepatic porphyrias and primary liver cancer, N Engl J Med, vol.338, pp.1853-1854, 1998.

M. F. Stewart, Review of hepatocellular cancer, hypertension and renal impairment as late complications of acute porphyria and recommendations for patient follow-up, J Clin Pathol, vol.65, pp.976-980, 2012.

C. Andersson, A. Wikberg, and B. Stegmayr, Renal symptomatology in patients with acute intermittent porphyria. A population-based study, J Intern Med, vol.248, pp.319-325, 2000.

J. T. Marsden, P. Chowdhury, and J. Wang, Acute intermittent porphyria and chronic renal failure, Clin Nephrol, vol.69, pp.339-346, 2008.

C. Andersson and F. Lithner, Hypertension and renal disease in patients with acute intermittent porphyria, J Intern Med, vol.236, pp.169-175, 1994.

E. Sardh, D. E. Andersson, and A. Henrichson, Porphyrin precursors and porphyrins in three patients with acute intermittent porphyria and endstage renal disease under different therapy regimes, Cell Mol Biol, vol.55, pp.66-71, 2009.

A. F. Castro and J. Coresh, CKD surveillance using laboratory data from the population-based National Health and Nutrition Examination Survey (NHANES), Am J Kidney Dis, vol.53, pp.46-55, 2009.

A. D. Rule, H. M. Gussak, and G. R. Pond, Measured and estimated GFR in healthy potential kidney donors, Am J Kidney Dis, vol.43, pp.112-119, 2004.

M. Hazzan, A. Hertig, and D. Buob, Epithelial-to-mesenchymal transition predicts cyclosporine nephrotoxicity in renal transplant recipients, J Am Soc Nephrol, vol.22, pp.1375-1381, 2011.

A. Hertig, D. Anglicheau, and J. Verine, Early epithelial phenotypic changes predict graft fibrosis, J Am Soc Nephrol, vol.19, pp.1584-1591, 2008.

J. T. Marsden and D. C. Rees, Urinary excretion of porphyrins, porphobilinogen and delta-aminolaevulinic acid following an attack of acute intermittent porphyria, J Clin Pathol, vol.67, pp.60-65, 2014.

O. Kepp, L. Galluzzi, and M. Lipinski, Cell death assays for drug discovery, Nat Rev Drug Discov, vol.10, pp.221-237, 2011.

N. Pallet, N. Bouvier, and A. Bendjallabah, Cyclosporine-induced endoplasmic reticulum stress triggers tubular phenotypic changes and death, Am J Transplant, vol.8, pp.2283-2296, 2008.

J. P. Thiery and J. P. Sleeman, Complex networks orchestrate epithelialmesenchymal transitions, Nat Rev Mol Cell Biol, vol.7, pp.131-142, 2006.

D. Nochy, E. Daugas, and D. Droz, The intrarenal vascular lesions associated with primary antiphospholipid syndrome, J Am Soc Nephrol, vol.10, pp.507-518, 1999.

C. J. Chang, Y. H. Lee, and J. Y. Yang, Pilot in vitro toxicity study of 5-ALA and Photofrin in microvascular endothelial cell cultures, J Clin Laser Med Surg, vol.15, pp.83-87, 1997.

T. A. Middelburg, H. S. De-bruijn, and L. Tettero, Topical hexylaminolevulinate and aminolevulinic acid photodynamic therapy: complete arteriole vasoconstriction occurs frequently and depends on protoporphyrin IX concentration in vessel wall, J Photochem Photobiol B, vol.126, pp.26-32, 2013.

F. Lithner, Could attacks of abdominal pain in cases of acute intermittent porphyria be due to intestinal angina?, J Intern Med, vol.247, pp.407-409, 2000.

H. Kupferschmidt, A. Bont, and H. Schnorf, Transient cortical blindness and bioccipital brain lesions in two patients with acute intermittent porphyria, Ann Intern Med, vol.123, pp.598-600, 1995.

C. W. Lai, T. P. Hung, and W. S. Lin, Blindness of cerebral origin in acute intermittent porphyria, Arch Neurol, vol.34, pp.310-312, 1977.

M. Celik, H. Forta, and T. Dalkilic, MRI reveals reversible lesions resembling posterior reversible encephalopathy in porphyria, Neuroradiology, vol.44, pp.839-841, 2002.

A. Soysal, P. Dogan, and C. Dayan, Reversible MRI findings of porphyric encephalopathy. A report of two cases, Neuroradiol J, vol.21, pp.655-659, 2008.

R. L. Lindberg, C. Porcher, and B. Grandchamp, Porphobilinogen deaminase deficiency in mice causes a neuropathy resembling that of human hepatic porphyria, Nat Genet, vol.12, pp.195-199, 1996.

C. Unzu, A. Sampedro, and E. Sardh, Renal failure affects the enzymatic activities of the three first steps in hepatic heme biosynthesis in the acute intermittent porphyria mouse, PLoS One, vol.7, p.32978, 2012.

A. Viau, K. El-karoui, and D. Laouari, Lipocalin 2 is essential for chronic kidney disease progression in mice and humans, J Clin Invest, vol.120, pp.4065-4076, 2010.

D. Siervi, A. Vazquez, E. S. Rezaval, and C. , Delta-aminolevulinic acid cytotoxic effects on human hepatocarcinoma cell lines, BMC Cancer, vol.2, p.6, 2002.

X. L. Zhang, N. Topley, and T. Ito, Interleukin-6 regulation of transforming growth factor (TGF)-beta receptor compartmentalization and turnover enhances TGF-beta1 signaling, J Biol Chem, vol.280, pp.12239-12245, 2005.

Y. Dai, W. Zhang, and J. Wen, A2B adenosine receptor-mediated induction of IL-6 promotes CKD, J Am Soc Nephrol, vol.22, pp.890-901, 2011.

W. Zhang, W. Wang, and H. Yu, Interleukin 6 underlies angiotensin IIinduced hypertension and chronic renal damage, Hypertension, vol.59, pp.136-144, 2012.

G. He, D. Dhar, and H. Nakagawa, Identification of liver cancer progenitors whose malignant progression depends on autocrine IL-6 signaling, Cell, vol.155, pp.384-396, 2013.

M. C. Tollanes, A. K. Aarsand, and J. H. Villanger, Establishing a network of specialist Porphyria centres -effects on diagnostic activities and services, Orphanet J Rare Dis, vol.7, p.93, 2012.

H. Puy, J. C. Deybach, and J. Lamoril, Molecular epidemiology and diagnosis of PBG deaminase gene defects in acute intermittent porphyria, Am J Hum Genet, vol.60, pp.1373-1383, 1997.

S. Tang, J. C. Leung, and A. K. , Albumin stimulates interleukin-8 expression in proximal tubular epithelial cells in vitro and in vivo, J Clin Invest, vol.111, pp.515-527, 2003.

N. Pallet, E. Thervet, L. Corre, and D. , Rapamycin inhibits human renal epithelial cell proliferation: effect on cyclin D3 mRNA expression and stability, Kidney Int, vol.67, pp.2422-2433, 2005.

K. J. Livak and T. D. Schmittgen, Analysis of relative gene expression data using real-time quantitative PCR and the 2(-Delta Delta C(T)) Method, Methods, vol.25, pp.402-408, 2001.

I. Sirois, J. Groleau, and N. Pallet, Caspase activation regulates the extracellular export of autophagic vacuoles, Autophagy, vol.8, pp.927-937, 2012.