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The difference in liver pathology between sporadic and familial forms of porphyria cutanea tarda: the role of iron.
J Hepatol 1995; 23(3):259-67JH

Abstract

Porphyria cutanea tarda is a disorder of porphyrin metabolism, of which familial and sporadic forms have been described. Factors such as iron seem necessary for porphyria cutanea tarda to become clinically manifest. To study the relationship between iron and uroporphyrins in hepatocytes of patients with porphyria cutanea tarda, a morphological and morphometrical study was performed in 13 liver biopsies of patients with porphyria cutanea tarda (eight with sporadic porphyria cutanea tarda and five with familial porphyria cutanea tarda). In addition, possible differences in clinical and biochemical features and in histopathological findings between patients with sporadic porphyria cutanea tarda and familial porphyria cutanea tarda were investigated. Familial porphyria cutanea tarda patients presented at a younger age than sporadic porphyria cutanea tarda patients (42.4 +/- 5.3 vs. 57.3 +/- 8.6 years). Biochemical features were not different between sporadic porphyria cutanea tarda and familial porphyria cutanea tarda patients. Uroporphyrin crystals and a variable degree of liver siderosis were detected in the biopsies of all 13 patients. Uroporphyrin crystals were often found close to ferritin-like iron deposits. The morphometrical analysis showed that an increased mean area fraction of ferritin iron was associated with an increased mean area fraction of uroporphyrin crystals in hepatocytes of sporadic porphyria cutanea tarda and familial porphyria cutanea tarda patients. Moreover, the amount of uroporphyrin crystals was significantly higher in livers of familial porphyria cutanea tarda than sporadic porphyria cutanea tarda patients. These findings are consistent with the hypothesis that uroporphyria is precipitated by an iron-dependent process.(

ABSTRACT

TRUNCATED AT 250 WORDS)

Authors+Show Affiliations

Department of Internal Medicine II, University Hospital Rotterdam-Dijkzigt, The Netherlands.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

8550989

Citation

Siersema, P D., et al. "The Difference in Liver Pathology Between Sporadic and Familial Forms of Porphyria Cutanea Tarda: the Role of Iron." Journal of Hepatology, vol. 23, no. 3, 1995, pp. 259-67.
Siersema PD, Rademakers LH, Cleton MI, et al. The difference in liver pathology between sporadic and familial forms of porphyria cutanea tarda: the role of iron. J Hepatol. 1995;23(3):259-67.
Siersema, P. D., Rademakers, L. H., Cleton, M. I., ten Kate, F. J., de Bruijn, W. C., Marx, J. J., & Wilson, J. H. (1995). The difference in liver pathology between sporadic and familial forms of porphyria cutanea tarda: the role of iron. Journal of Hepatology, 23(3), pp. 259-67.
Siersema PD, et al. The Difference in Liver Pathology Between Sporadic and Familial Forms of Porphyria Cutanea Tarda: the Role of Iron. J Hepatol. 1995;23(3):259-67. PubMed PMID: 8550989.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The difference in liver pathology between sporadic and familial forms of porphyria cutanea tarda: the role of iron. AU - Siersema,P D, AU - Rademakers,L H, AU - Cleton,M I, AU - ten Kate,F J, AU - de Bruijn,W C, AU - Marx,J J, AU - Wilson,J H, PY - 1995/9/1/pubmed PY - 1995/9/1/medline PY - 1995/9/1/entrez SP - 259 EP - 67 JF - Journal of hepatology JO - J. Hepatol. VL - 23 IS - 3 N2 - Porphyria cutanea tarda is a disorder of porphyrin metabolism, of which familial and sporadic forms have been described. Factors such as iron seem necessary for porphyria cutanea tarda to become clinically manifest. To study the relationship between iron and uroporphyrins in hepatocytes of patients with porphyria cutanea tarda, a morphological and morphometrical study was performed in 13 liver biopsies of patients with porphyria cutanea tarda (eight with sporadic porphyria cutanea tarda and five with familial porphyria cutanea tarda). In addition, possible differences in clinical and biochemical features and in histopathological findings between patients with sporadic porphyria cutanea tarda and familial porphyria cutanea tarda were investigated. Familial porphyria cutanea tarda patients presented at a younger age than sporadic porphyria cutanea tarda patients (42.4 +/- 5.3 vs. 57.3 +/- 8.6 years). Biochemical features were not different between sporadic porphyria cutanea tarda and familial porphyria cutanea tarda patients. Uroporphyrin crystals and a variable degree of liver siderosis were detected in the biopsies of all 13 patients. Uroporphyrin crystals were often found close to ferritin-like iron deposits. The morphometrical analysis showed that an increased mean area fraction of ferritin iron was associated with an increased mean area fraction of uroporphyrin crystals in hepatocytes of sporadic porphyria cutanea tarda and familial porphyria cutanea tarda patients. Moreover, the amount of uroporphyrin crystals was significantly higher in livers of familial porphyria cutanea tarda than sporadic porphyria cutanea tarda patients. These findings are consistent with the hypothesis that uroporphyria is precipitated by an iron-dependent process.(ABSTRACT TRUNCATED AT 250 WORDS) SN - 0168-8278 UR - https://www.unboundmedicine.com/medline/citation/8550989/The_difference_in_liver_pathology_between_sporadic_and_familial_forms_of_porphyria_cutanea_tarda:_the_role_of_iron_ L2 - https://linkinghub.elsevier.com/retrieve/pii/0168827895804692 DB - PRIME DP - Unbound Medicine ER -