The Porphyrias

Anderson, Karl E.

doi:10.1016/b978-1-4160-3258-8.50077-2

Cited by 4 publications

(5 citation statements)

References 468 publications

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“…In most studies, there is a strong association between HCV infection and PCT (Table ) . Furthermore, it has been observed that HCV‐infected individuals develop PCT at an earlier age than do uninfected persons with PCT .…”

Section: Epidemiology/association Of Porphyria Cutanea Tarda With Hepmentioning

confidence: 98%

“…These lesions are not unique to PCT and are found in other porphyrias of the bullous type. Similarly, liver histopathology is mostly non specific and includes red fluorescence of unfixed hepatic tissue (which is seen in various types of porphyria), necrosis, inflammation, varying siderosis and steatosis (Fig. ).…”

Section: Major Clinical Laboratory Histopathological Features and Rmentioning

confidence: 99%

“…Furthermore, it has been observed that HCV‐infected individuals develop PCT at an earlier age than do uninfected persons with PCT . Although the presence of any single risk factor is likely not sufficient to cause PCT, HCV is thought to be a strong trigger for the development of deranged porphyrin metabolism in those with other known predisposition . O'Reilly et al .…”

Section: Epidemiology/association Of Porphyria Cutanea Tarda With Hepmentioning

confidence: 99%

“…It has been known for many years that (i) most patients with PCT have some degree of iron overload; (ii) iron removal ameliorates porphyrin overproduction and clinical features and (iii) administration of iron produces relapse of PCT . Serum iron indices and iron content of the liver are often elevated in patients with CHC . The precise molecular mechanisms by which iron may influence HCV‐induced liver disease are not fully understood.…”

Section: Factors That Underlie the Association Of Hepatitis C Virus Amentioning

confidence: 99%

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Hepatitis C, porphyria cutanea tarda and liver iron: an update

Caballes

Sendi

Bonkovsky

2012

Liver International

105

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Porphyria cutanea tarda (PCT) is the most common form of porphyria across the world. Unlike other forms of porphyria, which are inborn errors of metabolism, PCT is usually an acquired liver disease caused by exogenous factors, chief among which are excess alcohol intake, iron overload, chronic hepatitis C, estrogen therapy, and cigarette smoking. The pathogenesis of PCT is complex and varied, but hereditary or acquired factors that lead to hepatic iron loading and increased oxidative stress are of central importance. Iron loading is usually only mild or moderate in degree (less than that associated with full-blown hemochromatosis) and is usually acquired and/or due to mutations in HFE. Among acquired factors are excessive alcohol intake and chronic hepatitis C infection, which, like mutations in HFE, decrease hepcidin production by hepatocytes. The decrease in hepcidin leads to increased iron absorption from the gut. In the liver, iron-loading and increased oxidative stress leads to the formation of non-porphyrin inhibitor(s) of uroporphyrinogen decarboxylase and to oxidation of porphyrinogens to porphyrins. The treatment of choice of active PCT is iron reduction by phlebotomy and maintenance of a mildly iron-reduced state without anemia. Low-dose anti-malarials (cinchona alkaloids) are also useful as additional therapy or as alternative therapy for active PCT in those without hemochromatosis or chronic hepatitis C. In this review, we provide an update of PCT with special emphasis upon the important role often played by the hepatitis C virus.

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Section: Epidemiology/association Of Porphyria Cutanea Tarda With Hepmentioning

confidence: 98%

Section: Major Clinical Laboratory Histopathological Features and Rmentioning

confidence: 99%

Section: Epidemiology/association Of Porphyria Cutanea Tarda With Hepmentioning

confidence: 99%

Section: Factors That Underlie the Association Of Hepatitis C Virus Amentioning

confidence: 99%

See 2 more Smart Citations

Hepatitis C, porphyria cutanea tarda and liver iron: an update

Caballes

Sendi

Bonkovsky

2012

Liver International

105

View full text Add to dashboard Cite

show abstract

“…Massive accumulation of porphyrins in the liver also is associated with low-grade hepatocellular damage. Certain susceptibility factors themselves cause liver damage, and risks of progressive liver disease, cirrhosis and hepatocellular carcinoma are increased 2, 3.…”

Section: Introductionmentioning

confidence: 99%