Effect of hematin administration to patients with protoporphyria and liver disease

Bloomer, Joseph R.; Pierach, Claus A.

doi:10.1002/hep.1840020613

Cited by 60 publications

(22 citation statements)

References 24 publications

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“…Many therapeutic regimens have been proposed for liver disease like vitamin E [36], cholestyramine [37], chenodeoxycholic acid [38], blood transfusion [39] and hematin infusion [40], Their efficacy in the prevention of liver failure has not been clear ly established so far. These therapies can produce serious side effects or discomfort for the patient.…”

Section: Avoidance O F Possible Risk Factors For Liver Disease In Eppmentioning

confidence: 99%

Hepatic Disease in Erythropoietic Protoporphyria

Mooyaart¹,

Jong²,

Veen³

et al. 1986

Dermatology

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A standardized hepatological investigation was performed in 9 unselected patients with erythropoietic protoporphyria (EPP). The aim of this study was to detect early liver involvement due to EPP and to determine the significance of several diagnostic procedures. Scintigraphy revealed slight enlargement of liver and spleen in all cases. Light microscopic examination of liver tissue in 7 patients showed protoporphyrin deposition in 4 and signs of fibrosis in 3 cases. Cirrhosis was not found. Electron microscopical examination (EM) of all 7 cases was negative and needle-shaped crystals were not found. Therefore we regard EM of little diagnostic value in the detection of early liver involvement. The literature on fatal and asymptomatic cases of liver involvement in EPP is discussed with emphasis on possible predictive and provocative factors. A proposal for EPP patients intending to reduce possible risk factors is made.

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Section: Avoidance O F Possible Risk Factors For Liver Disease In Eppmentioning

confidence: 99%

Hepatic Disease in Erythropoietic Protoporphyria

Mooyaart¹,

Jong²,

Veen³

et al. 1986

Dermatology

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“…Since this enhancement only seemed to occur in those experiments in which griseofulvininduced porphyrogenesis was continuously stimulated, it may be speculated that "newer" protoporphyrin in the hepatocyte is more readily mobilized for such accelerated secretion, while "older" deposits are less accessible. Since large amounts of protoporphyrin in livers of patients with protoporphyria are thought to be cleared from the plasma and transported into bile relatively efficiently (as reflected by the large amounts of fecal protoporphyrin excreted daily) (1,3,4,6,8,9,13,15,16,30), an important fraction of total intrahepatic protoporphyrin of these patients may be of such a relatively labile type. Other explanations may be speculated as well.…”

Section: Discussionmentioning

confidence: 99%

“…Hematin infusion (10,16) and hypertransfusion (17) may dimninish protoporphyrin overproduction, but are not suitable for chronic use in the general population of patients with protoporphyria.…”

Section: Introductionmentioning

confidence: 99%

Protoporphyrin hepatopathy. Effects of cholic acid ingestion in murine griseofulvin-induced protoporphyria.

Poh–Fitzpatrick¹,

Sklar²,

Goldsman³

et al. 1983

J. Clin. Invest.

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A B S T R A C T Short-term effects of cholic acid ingestion on hepatic accumulation, fecal excretion, and blood levels of protoporphyrin were studied in vivo in griseofulvin-induced protoporphyric mice. Experimental mice that received feed with 2% griseofulvin and 0.5% cholic acid were compared with control mice that received feed with 2% griseofulvin for 4 wk. Five mice from each group were assessed each week for liver and blood porphyrin levels. Fecal protoporphyrin was compared weekly in the total pooled output of each population. Mean protoporphyrin levels were significantly lower for liver (P < 0.0001), erythrocytes (P < 0.05), and plasma (P < 0.05), and higher for feces (P < 0.001) for the mice that were fed cholic acid. Microscopic protoporphyrin deposits, inflammation, necrosis, and dysplasia were more severe in livers of control mice. A second experimental design compared four regimens in the feed given to all mice after 1-wk induction with 2% griseofulvin: (a) 0.5% cholic acid, (b) no adulterant, (c) 2% griseofulvin and 0.5% cholic acid, and (d) 2% griseofulvin. No difference in protoporphyrin removal from livers of mice in groups 1 and 2 was observed after 1 and 2 wk of these regimens. The apparent reduction in hepatic protoporphyrin content in mice of group 3 as compared with group 4 at weeks 2 and 3 was not significant at P < 0.05.These data suggest that in selected circumstances, Drs. Sklar and Goldsman participated in these studies during student research elective periods at Columbia University College of Physicians and Surgeons. Address all correspondence to Dr. Poh-Fitzpatrick.

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“…Liver damage can progress slowly, as indicated by mild and often unexplained abnormal liver function tests, or advance rapidly to hepatic failure with evidence of both acute and chronic liver disease (Bruguera and Herrero, 2005;Anstey and Hift, 2007;Ma et al, 2010). Treatment with a combination of plasmapheresis to remove PPIX from plasma, hemin infusions to reduce marrow production of PPIX (Bloomer and Pierach, 1982;Gordeuk et al, 1986), cholestyramine (or activated charcoal) to interrupt the enterohepatic circulation of PPIX (Gorchein and Foster, 1999), bile acids such as ursodeoxycholic acid to enhance bile formation and flow (Van Hattum et al, 1986;Gross et al, 1998), and vitamin E to reduce oxidative stress (Komatsu et al, 2000;Singal et al, 2014) may achieve remission or slow the progression of liver disease and bridge the patient to hepatic transplantation. Such approaches are depicted in Fig.…”

Section: Clinical Manifestations and Management Of Ppix Toxicitymentioning

confidence: 99%

Protoporphyrin IX: the Good, the Bad, and the Ugly

Sachar

Anderson

2015

Journal of Pharmacology and Experimental Therapeutics

162

148

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Protoporphyrin IX (PPIX) is ubiquitously present in all living cells in small amounts as a precursor of heme. PPIX has some biologic functions of its own, and PPIX-based strategies have been used for cancer diagnosis and treatment (the good). PPIX serves as the substrate for ferrochelatase, the final enzyme in heme biosynthesis, and its homeostasis is tightly regulated during heme synthesis. Accumulation of PPIX in human porphyrias can cause skin photosensitivity, biliary stones, hepatobiliary damage, and even liver failure (the bad and the ugly). In this work, we review the mechanisms that are associated with the broad aspects of PPIX. Because PPIX is a hydrophobic molecule, its disposition is by hepatic rather than renal excretion. Large amounts of PPIX are toxic to the liver and can cause cholestatic liver injury. Application of PPIX in cancer diagnosis and treatment is based on its photodynamic effects.

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Effect of hematin administration to patients with protoporphyria and liver disease

Cited by 60 publications

References 24 publications

Hepatic Disease in Erythropoietic Protoporphyria

Hepatic Disease in Erythropoietic Protoporphyria

Protoporphyrin hepatopathy. Effects of cholic acid ingestion in murine griseofulvin-induced protoporphyria.

Protoporphyrin IX: the Good, the Bad, and the Ugly

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