The difference in liver pathology between sporadic and familial forms of porphyria cutanea tarda: the role of iron

Siersema, Peter D.; Rademakers, L. H. P. M.; Cleton, Maud L.; Kate, Fiebo J.W. ten; Bruijn, W. C. de; Marx, Joannes J.M.; Wilson, J. H. P.

doi:10.1016/s0168-8278(95)80004-2

Cited by 52 publications

(32 citation statements)

References 62 publications

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“…Der intrazelluläre Nachweis dieser Strukturen in unmittelbarer Nachbarschaft zu Ferritin-ähnlichen Eisenablagerungen spricht für eine mögliche pathogenetische Verbindung (44). Der frische Biopsiezylinder der Leber zeigt eine für Porphyrinakkumulation charakteristische Rotfluoreszenz unter langwelligem UV-Licht (366 nm), die bereits vor der kutanen Manifestation der Erkrankung auftreten kann (11,12 …”

Section: Pct Und Leberunclassified

Hepatitis C und Hämochromatose bei Porphyria cutanea tarda

Teubner

Richter²,

Schuppan³

et al. 2006

Dtsch med Wochenschr

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Porphyria cutanea tarda (PCT) is characterized by decreased activity of the enzyme uroporphyrinogen decarboxylase (URO-D) and the accumulation of uro- and heptaporphyrins in the liver. Apart from increased alcohol exposure and certain drugs, PCT is associated with antibodies to the hepatitis C virus (HCV), with its prevalence increasing from Northern (8-10%) to Southern Europe (71 to 91%). Chronic HCV-infection is thus considered to be a major trigger for PCT and PCT is said to be an important extrahepatic manifestation of HCV-infection in predisposed individuals. Iron overload is common in PCT. Iron is an inhibitory co-factor of URO-D activity in hepatocytes. Accordingly, in support of the critical role of iron, the clinical efficacy of iron removal is coupled to an improvement of hepatic URO-D activities. Up to two thirds of Saxon patients with PCT carry the classical hemochromatosis (HFE) mutations (C282Y and/or H63D). HFE genotyping can help to further classify patients with PCT and associated hemochromatosis. Simple or compound heterozygosity of HFE mutations does not affect the therapeutic response to chloroquine in PCT. Since Patients carrying homozygous mutations (C282Y/C282Y) with hemochromatosis and PCT do not respond to chloroquine, phlebotomy should be first-line treatment to remove toxic iron.

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Section: Pct Und Leberunclassified

Hepatitis C und Hämochromatose bei Porphyria cutanea tarda

Teubner

Richter²,

Schuppan³

et al. 2006

Dtsch med Wochenschr

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“…Eine Siderämie, eine Erhöhung des Ferritins und eine Hepatosiderose treten häufig auf [17]. Pathogenetisch ist eine massive Induktion der Kollagenase mit folgendem Abbau der unterschiedlichen Kollagentypen durch Uroporphyrin anzunehmen, das durch UV-Licht aktiviert wird [1,18].…”

Section: Laborbefundeunclassified

Unklare Hypertrichosis lanuginosa im Gesichtsbereich

Schulz¹,

Moll²

2002

Der Hautarzt

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Eine 59-jährige Patientin stellte sich mit seit 9 Monaten bestehendem vermehrtem Wachstum von weißen, flaumartigen Haaren sowie Hyperpigmentierungen im Gesicht und an den Unterarmen vor. Es bestünde kein Gewichtsverlust, aber reduzierter Appetit und gelegentlicher Nachtschweiß. Ein erhöhter Prolaktinspiegel sei festgestellt worden. Die Patientin rauche 30 Zigaretten am Tag und konsumiere regelmäßig Alkohol. Auf-

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“…We consider that the skin manifestations in this case might have been a result of iron overload in a patient predisposed to PCT, rather than secondary to the haematological malignancy per se . Abnormalities in iron metabolism are commonly observed in patients with PCT, and most patients with PCT have some degree of iron overload 2 . In liver biopsies uroporphyrin crystals are often found close to ferritin‐like iron deposits 2 and iron may reduce the activity of uroporphyrinogen decarboxylase, which causes an accumulation of uroporphyrins and PCT in predisposed individuals.…”

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confidence: 99%

“…Abnormalities in iron metabolism are commonly observed in patients with PCT, and most patients with PCT have some degree of iron overload 2 . In liver biopsies uroporphyrin crystals are often found close to ferritin‐like iron deposits 2 and iron may reduce the activity of uroporphyrinogen decarboxylase, which causes an accumulation of uroporphyrins and PCT in predisposed individuals. In recent years considerable interest has focused on the relationship of PCT to hereditary haemochromatosis and mutations in the haemochromatosis (HFE) gene 3 .…”

mentioning

confidence: 99%