Allgemeinsymptome der späten Hautporphyrie (Porphyria cutanea tarda) als Hinweise für deren Behandlung

Ippen, H

doi:10.1055/s-0028-1112755

Cited by 86 publications

(20 citation statements)

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“…11,111 Phlebotomy -Several reports stress the efficacy of this treatment, 111 which was introduced by Ippen, in 1961. 112 Phlebotomy is an outpatient procedure in which approximately 500ml (one unit) of blood are removed weekly or at every two weeks, until hemoglobin reaches 10g/dl or serum iron reaches 50 to 60 ìg/dl. 23 The goal of this treatment is to reduce the iron stores to a level lower than the normal limit.…”

Section: Treatmentmentioning

confidence: 99%

Porfiria cutânea tardia

Vieira

Martins

2006

An. Bras. Dermatol.

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Section: Treatmentmentioning

confidence: 99%

Porfiria cutânea tardia

Vieira

Martins

2006

An. Bras. Dermatol.

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“…Porphyria cutanea tarda (PCT) is charac terized by (1) cutaneous expression, (2) hepat ic accumulation and urinary excretion o f high-carboxylated porphyrins, (3) associated liver disease such as fatty liver, chronic hepa titis, fibrosis, cirrhosis or siderosis (PC T liver syndrome), (4) manifestation mainly due to alcohol and estrogens, and (5) inherited pre-disposition in about 50% o f cases based on decreased uroporphyrinogen decarboxylase (UD ) activity o f around 53% o f correspond ing mean value for normal subjects [1], The prevalence o f P C T including subclinical phases (chronic hepatic porphyria, table 1) is estimated at 20-50 cases in 100,000 persons. Prior to therapy, P C T patients usually show an accumulation o f hepatocellular uro-and heptacarboxyporphyrins o f >500 nmol/g (normal <1) as well as elevated urinary por phyrins o f >2.5 pmol/24 h (normal <0.2).…”

Section: Introductionmentioning

confidence: 99%

Treatment of Porphyria cutanea tarda by the Effect of Chloroquine on the Liver

Freesemann¹,

Frank

Sieg³

et al. 1995

Skin Pharmacol Physiol

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Porphyria cutanea tarda (PCX) is characterized by cutaneous symptoms in association with hepatic accumulation and urinary excretion of mainly uro- and heptacarboxyporphyrins. 24 PCX patients excreting urinary porphyrins between 1.9 and 5.8 µmol/24 h (normal < 0.2) were treated with chloroquine at a dose of 125 mg twice a week. During the first phase of therapy urinary porphyrin excretion transiently increased 1.1- to 2.9-fold indicating a phase of mobilization. A slight initial elevation was also found regarding the activities of serum amino-transferases reflecting the hepatotoxic effect of chloroquine. Xhe clinical symptoms disappeared after a period ranging from 2 to 6 months, and after an average of 12 months the porphyrin excretion in all patients had returned to nearly normal values.

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“…This illness is characterized by in vivo biochemical abnormalities similar to those observed in our in vitro system; namely, excessive total porphyrin production and a predominance of type I isomers. Iron overload accompanies the disturbance, and measures directed at removal of the iron relieve it (7)(8)(9)(10)(11)(12)(13). It is reasonable to conclude that these observations are explained by an inhibitory effect of iron on COSYN.…”

Section: Resultsmentioning

confidence: 79%

“…First, hepatic cell iron loading is almost invariably found in patients with the disorder (5,6). Second, renoval of iron by phlebotomy (7)(8)(9)(10) or the administration of iron-chelating agents (11) induces both clinical and biochemical remissions in such patients. Finally, the administration of iron is followed by relapse in patients in whom a remission has been induced by phlebotomy therapy (12,13).…”

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confidence: 99%