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Porphyria cutanea tarda and liver disease: a retrospective analysis of 17 cases from a single centre and review of the literature

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Organization
Abstract
Background/aims : Sporadic Porphyria Cutanea Tarda (sPCT) is associated with liver disease, e.g. HCV infection, haemochromatosis and especially alcoholic liver disease. We conducted a retrospective analysis on the prevalence of liver disorders in association with Porphyria Cutanea Tarda (PCT), in a university referral centre. Methods : The PCT cases were retrieved from computerized databases. Patient files lacking information on the presence of concomitant liver disease were excluded from further analysis. Results : 29 PCT patients were retrieved from our databases, of which 17 patients with sPCT were retained for further analysis. Patients were middle aged (mean age: 43 3) and there was no gender difference (10 males vs. 7 females). Almost all patients had iron overload (14/17). 5 patients had chronic HCV, with type 1b in 3 of them, 7 abused alcohol, 4 patients had hereditary haemochromatosis (3 homozygous C282Y - 1 heterozygous H63D/C282Y). In 3 patients sPCT was associated with medication intake and one patient had chronic hepatitis B (HBV). 13 patients were treated with phlebotomies, with success in 11/13. 4 patients were treated with chloroquine, 3 of which also underwent phlebotomies. Of the 5 patients with HCV, 3 were successfully treated with combined antiviral therapy; one of them is planned to be treated; one patient never received therapy and was lost from follow-up. One patient developed hepatocellular carcinoma (HCC) during a median follow-up of 24 years. Conclusions : We found a significant association between sPCT and liver disorders, such as chronic HCV infection, alcohol abuse, iron overload and hereditary haemochromatosis. Therefore, patients presenting with PCT should be screened for concomitant liver disease. Iron overload is present in a majority of patients, the majority of patients can be successfully treated with phlebotomies. The risk of developing HCC in our sPCT patients and in literature is low.
Keywords
INTERFERON-ALPHA THERAPY, CHRONIC HEPATITIS-C, HEPATOCELLULAR-CARCINOMA, EXTRAHEPATIC MANIFESTATIONS, ITALIAN PATIENTS, VIRUS-INFECTION, UROPORPHYRINOGEN DECARBOXYLASE, VIRAL-INFECTION, HIGH PREVALENCE, SKIN-LESIONS

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Chicago
Cassiman, David, Jaarke Vannoote, Rik Roelandts, Louis Libbrecht, Tania Roskams, Joost Van den Oord, Johan Fevery, Marjan Garmyn, and Frederik Nevens. 2008. “Porphyria Cutanea Tarda and Liver Disease: a Retrospective Analysis of 17 Cases from a Single Centre and Review of the Literature.” Acta Gastro-enterologica Belgica 71 (2): 237–242.
APA
Cassiman, D., Vannoote, J., Roelandts, R., Libbrecht, L., Roskams, T., Van den Oord, J., Fevery, J., et al. (2008). Porphyria cutanea tarda and liver disease: a retrospective analysis of 17 cases from a single centre and review of the literature. ACTA GASTRO-ENTEROLOGICA BELGICA, 71(2), 237–242.
Vancouver
1.
Cassiman D, Vannoote J, Roelandts R, Libbrecht L, Roskams T, Van den Oord J, et al. Porphyria cutanea tarda and liver disease: a retrospective analysis of 17 cases from a single centre and review of the literature. ACTA GASTRO-ENTEROLOGICA BELGICA. 2008;71(2):237–42.
MLA
Cassiman, David, Jaarke Vannoote, Rik Roelandts, et al. “Porphyria Cutanea Tarda and Liver Disease: a Retrospective Analysis of 17 Cases from a Single Centre and Review of the Literature.” ACTA GASTRO-ENTEROLOGICA BELGICA 71.2 (2008): 237–242. Print.
@article{3183715,
  abstract     = {Background/aims : Sporadic Porphyria Cutanea Tarda (sPCT) is associated with liver disease, e.g. HCV infection, haemochromatosis and especially alcoholic liver disease. We conducted a retrospective analysis on the prevalence of liver disorders in association with Porphyria Cutanea Tarda (PCT), in a university referral centre. 
Methods : The PCT cases were retrieved from computerized databases. Patient files lacking information on the presence of concomitant liver disease were excluded from further analysis. 
Results : 29 PCT patients were retrieved from our databases, of which 17 patients with sPCT were retained for further analysis. Patients were middle aged (mean age: 43 3) and there was no gender difference (10 males vs. 7 females). Almost all patients had iron overload (14/17). 5 patients had chronic HCV, with type 1b in 3 of them, 7 abused alcohol, 4 patients had hereditary haemochromatosis (3 homozygous C282Y - 1 heterozygous H63D/C282Y). In 3 patients sPCT was associated with medication intake and one patient had chronic hepatitis B (HBV). 13 patients were treated with phlebotomies, with success in 11/13. 4 patients were treated with chloroquine, 3 of which also underwent phlebotomies. Of the 5 patients with HCV, 3 were successfully treated with combined antiviral therapy; one of them is planned to be treated; one patient never received therapy and was lost from follow-up. One patient developed hepatocellular carcinoma (HCC) during a median follow-up of 24 years. 
Conclusions : We found a significant association between sPCT and liver disorders, such as chronic HCV infection, alcohol abuse, iron overload and hereditary haemochromatosis. Therefore, patients presenting with PCT should be screened for concomitant liver disease. Iron overload is present in a majority of patients, the majority of patients can be successfully treated with phlebotomies. The risk of developing HCC in our sPCT patients and in literature is low.},
  author       = {Cassiman, David and Vannoote, Jaarke and Roelandts, Rik and Libbrecht, Louis and Roskams, Tania and Van den Oord, Joost and Fevery, Johan and Garmyn, Marjan and Nevens, Frederik},
  issn         = {0001-5644},
  journal      = {ACTA GASTRO-ENTEROLOGICA BELGICA},
  keywords     = {INTERFERON-ALPHA THERAPY,CHRONIC HEPATITIS-C,HEPATOCELLULAR-CARCINOMA,EXTRAHEPATIC MANIFESTATIONS,ITALIAN PATIENTS,VIRUS-INFECTION,UROPORPHYRINOGEN DECARBOXYLASE,VIRAL-INFECTION,HIGH PREVALENCE,SKIN-LESIONS},
  language     = {eng},
  number       = {2},
  pages        = {237--242},
  title        = {Porphyria cutanea tarda and liver disease: a retrospective analysis of 17 cases from a single centre and review of the literature},
  volume       = {71},
  year         = {2008},
}

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