Page last updated: 2024-08-23

heme and Hepatic Porphyria

heme has been researched along with Hepatic Porphyria in 62 studies

Research

Studies (62)

TimeframeStudies, this research(%)All Research%
pre-19904 (6.45)18.7374
1990's15 (24.19)18.2507
2000's15 (24.19)29.6817
2010's15 (24.19)24.3611
2020's13 (20.97)2.80

Authors

AuthorsStudies
Anderson, KE; Bonkovsky, HL; Desnick, RJ; Stewart, MF; Ventura, P1
Harper, P; Sardh, E1
Larmann, J; Lederer, D; Weigand, MA1
Bishop, C; Bonkovsky, HL; Ma, CD; Van Horn, CG; Wan, M1
Testai, FD; Wylie, K1
Balwani, M; Fanelli, MJ; Gouya, L; Longo, N; Petrides, PE; Phillips, J; Plutzky, J; Rhyee, S; Sardh, E; Sweetser, MT; Ventura, P1
Balwani, M; Bonkovsky, HL; Lim, JK; Wang, B1
Balwani, M; Keel, S; Yasuda, M1
Anderson, KE; Dickey, A; Erwin, A; Leaf, RK; Moghe, A; O'Brien, A; Quigley, JG; Thapar, M1
Badawy, AA2
Anderson, KE; Chan, A; Desnick, RJ; Lahiji, AP; Ramanujam, VMS; Simon, A1
Batlle, A; Cerbino, GN; Granata, BX; Martinez, MDC; Parera, VE; Rossetti, MV1
Sardh, E; Vassiliou, D1
Handler, GA; Handler, MZ; Handler, NS; Schwartz, RA; Stephany, MP1
Deybach, JC; Gouya, L; Karim, Z; Manceau, H; Peoc'h, K; Puy, H; Wahlin, S1
Chen, B; Desnick, RJ; Yasuda, M1
Balwani, M1
Friesema, EC; Koole-Lesuis, R; Langendonk, JG; Neeleman, RA; van Beers, EJ; van der Pol, WL; Wilson, JHP1
Bonkovsky, HL; Dixon, N; Rudnick, S1
Anderson, KE; Ávila, MA; Deybach, JC; Fontanellas, A1
Anderson, KE1
Phillips, JD1
Krishnan, AV; Lin, CS; Park, SB1
Huang, J; Lin, S; Tang, L; Wang, H; Wu, J; Xu, M; Zhang, S; Zhang, Y1
Nascimbeni, F; Rocchi, E; Roveri, G; Ventura, P1
Balwani, M; Desnick, RJ; Naik, H; Sanderson, SC; Stoecker, M1
Gouya, L; Manceau, H; Puy, H1
Anstey, AV; Badminton, MN; Beaumont, C; Corrigall, AV; Deybach, JC; Ducamp, S; Elder, GH; Gouya, L; Grandchamp, B; Hift, RJ; Holme, SA; Ma, Y; Marsden, JT; Meissner, PN; Mieli-Vergani, G; Parker, M; Puy, H; Whatley, SD1
Iwadate, R; Kawai, H; Kawashima, Y; Kudo, N; Mitsumoto, A; Satoh, Y; Watanabe, Y1
Biolcati, G; Cappellini, MD; Danielli, MG; Di Pierro, E; Martinez di Montemuros, F; Patti, E; Rocchi, E; Tavazzi, D1
Herrero, C; Lecha, M; Ozalla, D1
Bonkovsky, HL; Chemmanur, AT1
Beer, R; Engelhardt, K; Franz, G; Kampfl, A; Pfausler, B; Schmutzhard, E; Spiegel, M; Trinka, E; Unterberger, I1
Bloomer, JR; Brenner, DA; Scarlett, YV1
Brand, HS; Kooijman, MM1
Beaumont, C; Boileau, C; Camadro, JM; Da Silva, V; Deybach, JC; Flamme, M; Gouya, L; Grandchamp, B; Lamoril, J; Lyoumi, S; Malonova, E; Puy, H; Rose, C; Schmitt, C1
Sassa, S1
Bloomer, JR; McGuire, BM1
Romeo, G1
Aderka, D; Dotan, I; Levo, Y; Mamet, R; Schoenfeld, N; Sztern, M1
Bonkovsky, HL; Cable, EE; Donohue, SE; Lambrecht, RW; Pepe, JA1
Deybach, JC; Nordmann, Y; Puy, H1
Arvela, P; Mustajoki, P; Mustajoki, S; Pelkonen, O; Rautio, A1
Brady, AM; De Matteis, F; Elcombe, BM; Hasmall, RL; Lock, EA1
Dover, SB; Fitzsimmons, EJ; Graham, A; McColl, KE; Moore, MR1
Deybach, JC; Nordmann, Y1
Reynolds, NC1
Schreiber, WE1
Clothier, B; De Matteis, F; Francis, JE; Gibbs, AH; Hider, RC; Smith, AG1
Gorchein, A1
Mustajoki, P; Tenhunen, R1
Andant, C; Bogard, C; Deybach, JC; Faivre, J; Nordmann, Y; Puy, H; Soulé, JC1
Doss, MO; Gross, U; Kühnel, A1
Hoffmann, F; Lindberg, RL; Meyer, UA; Schuurmans, MM1
Abe, K; Fukada, K; Kaido, M; Kondo, M; Moriya, M; Sakoda, S; Yanagihara, T1
Doss, M; Doss, MO; Gross, U; Schurek, JO1
Elder, GH; Evans, JO; Thomas, N1
Armas, R; Chaná, P; Krause, P; Parraguez, A; Soto, J; Wolff, C1
Paslin, DA1
Hindmarsh, JT1
Rimington, C1

Reviews

28 review(s) available for heme and Hepatic Porphyria

ArticleYear
Acute Hepatic Porphyrias: "Purple Flags"-Clinical Features That Should Prompt Specific Diagnostic Testing.
    The American journal of the medical sciences, 2022, Volume: 363, Issue:1

    Topics: Diagnostic Techniques and Procedures; Heme; Humans; Hyponatremia; Pain; Porphobilinogen Synthase; Porphyrias, Hepatic

2022
RNAi therapy with givosiran significantly reduces attack rates in acute intermittent porphyria.
    Journal of internal medicine, 2022, Volume: 291, Issue:5

    Topics: Acetylgalactosamine; Adolescent; Adult; Heme; Humans; Incidence; Porphobilinogen Synthase; Porphyria, Acute Intermittent; Porphyrias, Hepatic; Pyrrolidines; Quality of Life; RNAi Therapeutics

2022
Neurological Manifestations of Acute Porphyrias.
    Current neurology and neuroscience reports, 2022, Volume: 22, Issue:7

    Topics: Heme; Humans; Neuralgia; Porphyria, Acute Intermittent; Porphyrias; Porphyrias, Hepatic; Posterior Leukoencephalopathy Syndrome

2022
AGA Clinical Practice Update on Diagnosis and Management of Acute Hepatic Porphyrias: Expert Review.
    Gastroenterology, 2023, Volume: 164, Issue:3

    Topics: Abdominal Pain; Aminolevulinic Acid; Antiemetics; Carcinoma, Hepatocellular; Creatinine; Female; Heme; Hemin; Humans; Hypertension; Liver Neoplasms; Middle Aged; Porphobilinogen; Porphobilinogen Synthase; Porphyria, Acute Intermittent; Porphyrias, Hepatic; Quality of Life; Renal Insufficiency, Chronic; United States

2023
Hypothesis: Metabolic targeting of 5-aminolevulinate synthase by tryptophan and inhibitors of heme utilisation by tryptophan 2,3-dioxygenase as potential therapies of acute hepatic porphyrias.
    Medical hypotheses, 2019, Volume: 131

    Topics: 5-Aminolevulinate Synthetase; Allopurinol; Animals; Enzyme Induction; Enzyme Inhibitors; Fasting; Feedback, Physiological; Gene Silencing; Glucose; Guinea Pigs; Heme; Humans; Kynurenine; Liver; Models, Biological; Molecular Targeted Therapy; Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; Porphyrias, Hepatic; Rodentia; Species Specificity; Tryptophan; Tryptophan Oxygenase

2019
Porphyria cutanea tarda: an intriguing genetic disease and marker.
    International journal of dermatology, 2017, Volume: 56, Issue:6

    Topics: Diagnosis, Differential; Disease Progression; Heme; Humans; Porphyria Cutanea Tarda; Porphyrias, Hepatic; Risk Factors

2017
Recent advances on porphyria genetics: Inheritance, penetrance & molecular heterogeneity, including new modifying/causative genes.
    Molecular genetics and metabolism, 2019, Volume: 128, Issue:3

    Topics: Biosynthetic Pathways; Gain of Function Mutation; Gene Expression Regulation; Heme; Humans; Loss of Function Mutation; Penetrance; Porphobilinogen Synthase; Porphyria, Acute Intermittent; Porphyrias; Porphyrias, Hepatic; Protoporphyria, Erythropoietic

2019
Erythropoietic Protoporphyria and X-Linked Protoporphyria: pathophysiology, genetics, clinical manifestations, and management.
    Molecular genetics and metabolism, 2019, Volume: 128, Issue:3

    Topics: 5-Aminolevulinate Synthetase; Anemia; Clinical Trials as Topic; Dermatitis, Phototoxic; Disease Management; Genes, X-Linked; Heme; Humans; Liver Diseases; Porphyrias, Hepatic; Protoporphyria, Erythropoietic

2019
Pathogenesis and clinical features of the acute hepatic porphyrias (AHPs).
    Molecular genetics and metabolism, 2019, Volume: 128, Issue:3

    Topics: Anxiety; Heme; Humans; Mutation; Neuralgia; Porphobilinogen; Porphobilinogen Synthase; Porphyrias, Hepatic; Sleep Initiation and Maintenance Disorders

2019
Current and innovative emerging therapies for porphyrias with hepatic involvement.
    Journal of hepatology, 2019, Volume: 71, Issue:2

    Topics: 5-Aminolevulinate Synthetase; Acetylgalactosamine; alpha-MSH; Bone Marrow Transplantation; Cholestyramine Resin; Genetic Therapy; Heme; Humans; Liver; Liver Transplantation; Porphyrias, Hepatic; Porphyrins; Pyrrolidines; Receptor, Melanocortin, Type 1

2019
Acute hepatic porphyrias: Current diagnosis & management.
    Molecular genetics and metabolism, 2019, Volume: 128, Issue:3

    Topics: Animals; Biosynthetic Pathways; Clinical Trials as Topic; Disease Management; Heme; Hemin; Humans; Mice; Porphobilinogen; Porphobilinogen Synthase; Porphyrias, Hepatic

2019
Heme biosynthesis and the porphyrias.
    Molecular genetics and metabolism, 2019, Volume: 128, Issue:3

    Topics: Animals; Biosynthetic Pathways; Environment; Heme; Humans; Liver; Mice; Mutation; Porphobilinogen Synthase; Porphyria, Acute Intermittent; Porphyrias; Porphyrias, Hepatic; Uroporphyrinogen Decarboxylase

2019
Porphyric neuropathy.
    Handbook of clinical neurology, 2013, Volume: 115

    Topics: Heme; Humans; Peripheral Nervous System Diseases; Porphobilinogen Synthase; Porphyrias, Hepatic; Signal Transduction

2013
Drugs and acute porphyrias: reasons for a hazardous relationship.
    Postgraduate medicine, 2014, Volume: 126, Issue:7

    Topics: Carrier State; Cytochromes; Heme; Humans; Metabolism; Porphyrias; Porphyrias, Hepatic

2014
Acute hepatic and erythropoietic porphyrias: from ALA synthases 1 and 2 to new molecular bases and treatments.
    Current opinion in hematology, 2017, Volume: 24, Issue:3

    Topics: 5-Aminolevulinate Synthetase; Age Factors; Animals; Biomarkers; Chronic Pain; Enzyme Activation; Erythrocytes; Gene Expression Regulation; Genes, X-Linked; Genetic Association Studies; Genetic Predisposition to Disease; Heme; Humans; Mutation; Phenotype; Porphobilinogen Synthase; Porphyria, Erythropoietic; Porphyrias, Hepatic; RNA, Small Interfering

2017
Diagnosis and treatment of the hepatic porphyrias.
    Dermatologic therapy, 2003, Volume: 16, Issue:1

    Topics: Acute Disease; Antimalarials; Chloroquine; Heme; Humans; Photosensitivity Disorders; Porphyrias, Hepatic; Porphyrins

2003
Hepatic porphyrias: diagnosis and management.
    Clinics in liver disease, 2004, Volume: 8, Issue:4

    Topics: Diagnosis, Differential; Heme; Humans; Pain; Porphyrias, Hepatic; Porphyrins; Recurrence

2004
Hepatic porphyrias.
    Clinics in liver disease, 1998, Volume: 2, Issue:1

    Topics: Heme; Humans; Liver; Porphyria Cutanea Tarda; Porphyrias, Hepatic

1998
Oral aspects of porphyria.
    International dental journal, 2005, Volume: 55, Issue:2

    Topics: Algorithms; Anesthesia, Dental; Arginine; Blister; Carbohydrates; Contraindications; Heme; Humans; Mouth Diseases; Photophobia; Porphobilinogen Synthase; Porphyria, Erythropoietic; Porphyrias, Hepatic; Tooth Discoloration

2005
Modern diagnosis and management of the porphyrias.
    British journal of haematology, 2006, Volume: 135, Issue:3

    Topics: Acute Disease; Chronic Disease; Coproporphyria, Hereditary; Heme; Humans; Porphobilinogen Synthase; Porphyria Cutanea Tarda; Porphyria, Erythropoietic; Porphyria, Hepatoerythropoietic; Porphyria, Variegate; Porphyrias; Porphyrias, Hepatic; Protoporphyria, Erythropoietic

2006
The hepatic porphyrias.
    Progress in medical genetics, 1980, Volume: 4

    Topics: 5-Aminolevulinate Synthetase; Acute Disease; Adolescent; Adult; Aminolevulinic Acid; Chemical Phenomena; Chemistry; Child; Child, Preschool; Female; Gene Expression Regulation; Heme; Humans; Infant; Liver; Liver Diseases; Male; Middle Aged; Photosensitivity Disorders; Porphobilinogen; Porphyria, Acute Intermittent; Porphyrias; Porphyrias, Hepatic; Porphyrins; Prenatal Diagnosis; Protoporphyria, Erythropoietic; Recurrence; Skin Diseases

1980
[Hepatic porphyrias and drugs].
    Gastroenterologie clinique et biologique, 1994, Volume: 18, Issue:4

    Topics: Acute Disease; Alanine; Arginine; Carbohydrates; Heme; Humans; Ligases; Porphyrias, Hepatic

1994
Acute intermittent porphyria: laboratory diagnosis by molecular methods.
    Clinics in laboratory medicine, 1995, Volume: 15, Issue:4

    Topics: DNA Mutational Analysis; Erythrocytes; Genetic Linkage; Genetic Testing; Heme; Humans; Hydroxymethylbilane Synthase; Mutation; Polymorphism, Genetic; Porphyria, Acute Intermittent; Porphyrias, Hepatic; Specimen Handling

1995
Drug treatment in acute porphyria.
    British journal of clinical pharmacology, 1997, Volume: 44, Issue:5

    Topics: Acute Disease; Animals; Heme; Humans; Porphyrias, Hepatic

1997
[The hepatic porphyrias: experience with 105 cases].
    Revista medica de Chile, 1992, Volume: 120, Issue:3

    Topics: Adolescent; Adult; Child; Chile; Female; Heme; Humans; Male; Middle Aged; Porphyrias, Hepatic; Pregnancy; Pregnancy Complications; Retrospective Studies

1992
The porphyrias.
    International journal of dermatology, 1992, Volume: 31, Issue:8

    Topics: Acute Disease; Heme; Humans; Plasmapheresis; Porphyria, Erythropoietic; Porphyrias; Porphyrias, Hepatic; Porphyrinogens; Porphyrins

1992
The porphyrias: recent advances.
    Clinical chemistry, 1986, Volume: 32, Issue:7

    Topics: Acute Disease; Adult; Child; Chromatography, High Pressure Liquid; Diagnosis, Differential; Erythrocytes; Heme; Humans; Infant, Newborn; Lead Poisoning; Liver Diseases; Porphobilinogen Synthase; Porphyria, Erythropoietic; Porphyrias; Porphyrias, Hepatic; Porphyrins; Protoporphyria, Erythropoietic; Skin Diseases; Uroporphyrinogen Decarboxylase

1986
A review of the enzymic errors in the various porphyrias.
    Scandinavian journal of clinical and laboratory investigation, 1985, Volume: 45, Issue:4

    Topics: 5-Aminolevulinate Synthetase; Flavoproteins; Heme; Humans; Liver Diseases; Mitochondrial Proteins; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Porphobilinogen Synthase; Porphyria, Acute Intermittent; Porphyria, Erythropoietic; Porphyrias; Porphyrias, Hepatic; Porphyrins; Protoporphyria, Erythropoietic; Protoporphyrinogen Oxidase; Skin Diseases; Uroporphyrinogen Decarboxylase

1985

Trials

1 trial(s) available for heme and Hepatic Porphyria

ArticleYear
Tin protoporphyrin prolongs the biochemical remission produced by heme arginate in acute hepatic porphyria.
    Gastroenterology, 1993, Volume: 105, Issue:2

    Topics: Acute Disease; Adult; Arginine; Drug Synergism; Drug Therapy, Combination; Female; Heme; Humans; Male; Metalloporphyrins; Porphyrias, Hepatic; Protoporphyrins

1993

Other Studies

33 other study(ies) available for heme and Hepatic Porphyria

ArticleYear
[Anesthesia in patients with acute porphyria].
    Der Anaesthesist, 2022, Volume: 71, Issue:4

    Topics: Anesthesia; Heme; Humans; Porphyria, Acute Intermittent; Porphyrias; Porphyrias, Hepatic

2022
Assessment of porphyrogenicity of drugs and chemicals in selected hepatic cell culture models through a fluorescence-based screening assay.
    Pharmacology research & perspectives, 2022, Volume: 10, Issue:3

    Topics: Cell Culture Techniques; Heme; Hepatocytes; Humans; Liver; Norgestrel; Phenobarbital; Phenytoin; Porphobilinogen Synthase; Porphyrias, Hepatic; Valproic Acid

2022
Hyperhomocysteinemia in acute hepatic porphyria (AHP) and implications for treatment with givosiran.
    Expert review of gastroenterology & hepatology, 2022, Volume: 16, Issue:9

    Topics: Clinical Trials as Topic; Cystathionine beta-Synthase; Folic Acid; Heme; Homocysteine; Humans; Hyperhomocysteinemia; Methionine; Porphyrias, Hepatic; Pyridoxine; RNA, Small Interfering; Sulfur; Vitamin B 6

2022
RNA interference therapy in acute hepatic porphyrias.
    Blood, 2023, Nov-09, Volume: 142, Issue:19

    Topics: Aminolevulinic Acid; Heme; Humans; Pain; Porphyrias; Porphyrias, Hepatic; Quality of Life; RNA Interference

2023
Acute hepatic porphyrias: Recommendations for diagnosis and management with real-world examples.
    Molecular genetics and metabolism, 2023, Volume: 140, Issue:3

    Topics: Heme; Humans; Porphobilinogen; Porphobilinogen Synthase; Porphyrias, Hepatic

2023
5-Aminolevulinate dehydratase porphyria: Update on hepatic 5-aminolevulinic acid synthase induction and long-term response to hemin.
    Molecular genetics and metabolism, 2020, Volume: 131, Issue:4

    Topics: 5-Aminolevulinate Synthetase; Adolescent; Adult; Child; Child, Preschool; Female; Heme; Hemin; Humans; Infant; Infant, Newborn; Liver; Male; Middle Aged; Mutation; Porphobilinogen; Porphobilinogen Synthase; Porphyria, Acute Intermittent; Porphyrias, Hepatic; RNA, Messenger; Young Adult

2020
Clinical, biochemical, and genetic characterization of acute hepatic porphyrias in a cohort of Argentine patients.
    Molecular genetics & genomic medicine, 2021, Volume: 9, Issue:5

    Topics: Argentina; Female; Heme; Homocysteine; Humans; Male; Mutation; Oxidative Stress; Porphyrias, Hepatic

2021
Multiple roles of haem in cystathionine β-synthase activity: implications for hemin and other therapies of acute hepatic porphyria.
    Bioscience reports, 2021, 07-30, Volume: 41, Issue:7

    Topics: Animals; Cystathionine beta-Synthase; Heme; Hemin; Homocysteine; Humans; Kynurenine; Nutritional Status; Porphyrias, Hepatic; Treatment Outcome; Tryptophan; Vitamin B Complex

2021
Acute hepatic porphyria and maternal health: Clinical and biochemical follow-up of 44 pregnancies.
    Journal of internal medicine, 2022, Volume: 291, Issue:1

    Topics: Female; Follow-Up Studies; Heme; Humans; Maternal Health; Porphobilinogen Synthase; Porphyrias, Hepatic; Pregnancy; Pregnancy Outcome; Sweden

2022
Hepatocellular carcinoma in acute hepatic porphyrias: A Damocles Sword.
    Molecular genetics and metabolism, 2019, Volume: 128, Issue:3

    Topics: Biomarkers; Carcinoma, Hepatocellular; Female; Heme; Humans; Incidence; Liver Neoplasms; Male; Middle Aged; Norway; Porphobilinogen Synthase; Porphyria, Acute Intermittent; Porphyrias, Hepatic; Risk Factors; Sweden

2019
Clinical Remission of Delta-Aminolevulinic Acid Dehydratase Deficiency Through Suppression of Erythroid Heme Synthesis.
    Hepatology (Baltimore, Md.), 2019, Volume: 70, Issue:1

    Topics: Adult; Erythroid Cells; Heme; Humans; Hydroxyurea; Infant, Newborn; Male; Nucleic Acid Synthesis Inhibitors; Porphobilinogen Synthase; Porphyrias, Hepatic

2019
Heme acts through the Bach1b/Nrf2a-MafK pathway to regulate exocrine peptidase precursor genes in porphyric zebrafish.
    Disease models & mechanisms, 2014, Volume: 7, Issue:7

    Topics: Amino Acid Motifs; Amino Acid Sequence; Animals; Chromatin Immunoprecipitation; Enzyme Precursors; Gene Expression Regulation; Gene Knockdown Techniques; Heme; In Situ Hybridization; Mice; Models, Biological; Molecular Sequence Data; NIH 3T3 Cells; Nucleotide Motifs; Pancreas, Exocrine; Peptide Hydrolases; Porphyrias, Hepatic; Promoter Regions, Genetic; Protein Multimerization; RNA, Messenger; Signal Transduction; Zebrafish; Zebrafish Proteins

2014
Experiences and concerns of patients with recurrent attacks of acute hepatic porphyria: A qualitative study.
    Molecular genetics and metabolism, 2016, Volume: 119, Issue:3

    Topics: Adult; Aged; Female; Heme; Humans; Male; Middle Aged; Patients; Physician-Patient Relations; Porphobilinogen Synthase; Porphyrias, Hepatic; Quality of Life

2016
C-terminal deletions in the ALAS2 gene lead to gain of function and cause X-linked dominant protoporphyria without anemia or iron overload.
    American journal of human genetics, 2008, Volume: 83, Issue:3

    Topics: 5-Aminolevulinate Synthetase; Chromosomes, Human, X; Erythrocytes; Female; Heme; Humans; Male; Mutation; Porphyrias, Hepatic; Protoporphyrins

2008
Manifestation of psychiatric behaviors in a mouse model of griseofulvin-induced hepatic porphyria.
    The Journal of toxicological sciences, 2008, Volume: 33, Issue:5

    Topics: Animals; Anxiety; Behavior, Animal; Cytochrome P-450 Enzyme System; Depression; Disease Models, Animal; Enzymes; Feces; Griseofulvin; Heme; Liver; Male; Mice; Mice, Inbred ICR; Muscle, Skeletal; Porphyrias, Hepatic; Swimming

2008
Molecular characterization of porphyrias in Italy: a diagnostic flow-chart.
    Cellular and molecular biology (Noisy-le-Grand, France), 2002, Volume: 48, Issue:8

    Topics: DNA; Ferrochelatase; Flavoproteins; Heme; Humans; Hydroxymethylbilane Synthase; Italy; Mitochondrial Proteins; Mutation; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Polymerase Chain Reaction; Polymorphism, Single-Stranded Conformational; Porphyria Cutanea Tarda; Porphyria, Acute Intermittent; Porphyria, Hepatoerythropoietic; Porphyrias; Porphyrias, Hepatic; Porphyrins; Protoporphyrinogen Oxidase; Reverse Transcriptase Polymerase Chain Reaction; Risk Factors; Sensitivity and Specificity; Spectrometry, Fluorescence; Uroporphyrinogen Decarboxylase

2002
Refractory status epilepticus due to acute hepatic porphyria in a pregnant woman: induced abortion as the sole therapeutic option?
    European journal of neurology, 2004, Volume: 11, Issue:10

    Topics: Abortion, Induced; Adult; Arginine; Brain Mapping; Diffusion Magnetic Resonance Imaging; Electroencephalography; Female; Frontal Lobe; Heme; Humans; Porphyrias, Hepatic; Porphyrins; Pregnancy; Status Epilepticus; Time Factors

2004
Mutations in human CPO gene predict clinical expression of either hepatic hereditary coproporphyria or erythropoietic harderoporphyria.
    Human molecular genetics, 2005, Oct-15, Volume: 14, Issue:20

    Topics: Amino Acid Sequence; Coproporphyria, Hereditary; Coproporphyrinogen Oxidase; Exons; Gene Expression; Heme; Humans; Iron Overload; Molecular Sequence Data; Mutation; Mutation, Missense; Pedigree; Porphyrias, Hepatic; Protein Structure, Secondary; Sequence Homology

2005
Intermittent unexplained abdominal pain: is it porphyria?
    Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association, 2007, Volume: 5, Issue:11

    Topics: Abdominal Pain; Acute Disease; Adult; Aminolevulinic Acid; Anti-Infective Agents; Cimetidine; Contraceptives, Oral; Diet, Reducing; Enzyme Inhibitors; Female; Heme; Hemin; Humans; Liver; Liver Transplantation; Porphobilinogen; Porphyrias, Hepatic; Sulfamethoxazole; Trimethoprim; Urinary Tract Infections

2007
Relation between uroporphyrin excretion, acute attacks of hereditary coproporphyria and successful treatment with haem arginate.
    Clinical science (London, England : 1979), 1995, Volume: 88, Issue:3

    Topics: Acute Disease; Adult; Arginine; Erythrocytes; Heme; Humans; Hydroxymethylbilane Synthase; Male; Porphyrias, Hepatic; Porphyrins; Spectrometry, Fluorescence; Uroporphyrins

1995
Effects of mifepristone (RU-486) on heme metabolism and cytochromes P-450 in cultured chick embryo liver cells, possible implications for acute porphyria.
    European journal of biochemistry, 1994, Oct-15, Volume: 225, Issue:2

    Topics: 5-Aminolevulinate Synthetase; Animals; Cells, Cultured; Chick Embryo; Cytochrome P-450 Enzyme System; Deferoxamine; Heme; Liver; Mifepristone; Porphyrias, Hepatic; Porphyrins; RNA, Messenger

1994
Effects of heme arginate on cytochrome P450-mediated metabolism of drugs in patients with variegate porphyria and in healthy men.
    Clinical pharmacology and therapeutics, 1994, Volume: 56, Issue:1

    Topics: Adult; Arginine; Caffeine; Coumarins; Cytochrome P-450 Enzyme System; Debrisoquin; Female; Heme; Humans; Isoenzymes; Lidocaine; Male; Middle Aged; Porphyrias, Hepatic; Reference Values

1994
Effects of 2-[1-(ethoxyimino)propyl]-3-hydroxy-5-(2,4,6-trimethylphenyl) cyclohex-2-enone on hepatic haem biosynthesis: species differences in hepatic porphyria.
    Toxicology, 1993, Nov-12, Volume: 84, Issue:1-3

    Topics: Administration, Oral; Animals; Cricetinae; Cyclohexanones; Female; Ferrochelatase; Heme; Herbicides; Liver; Male; Mesocricetus; Mice; Porphyrias, Hepatic; Porphyrins; Rats; Rats, Wistar; Species Specificity

1993
[Acute attacks of hepatic porphyria: specific treatment with heme arginate].
    Annales de medecine interne, 1993, Volume: 144, Issue:3

    Topics: Acute Disease; Arginine; Female; Heme; Hospitalization; Humans; Infusions, Intravenous; Male; Porphyrias, Hepatic; Time Factors

1993
Epilepsy in porphyria.
    Neurology, 1996, Volume: 46, Issue:5

    Topics: 5-Aminolevulinate Synthetase; Acetates; Adult; Amines; Anticonvulsants; Cyclohexanecarboxylic Acids; Disease-Free Survival; Epilepsy; Gabapentin; gamma-Aminobutyric Acid; Heme; Humans; Liver; Male; Porphyrias; Porphyrias, Hepatic

1996
Protoporphyria induced by the orally active iron chelator 1,2-diethyl-3-hydroxypyridin-4-one in C57BL/10ScSn mice.
    Blood, 1997, Feb-01, Volume: 89, Issue:3

    Topics: Administration, Oral; Animals; Cytochrome P-450 Enzyme System; Ferrochelatase; Heme; Iron; Iron Chelating Agents; Liver; Male; Mice; Mice, Inbred C57BL; Porphyrias, Hepatic; Pyridones

1997
Acute porphyria: treatment with heme.
    Seminars in liver disease, 1998, Volume: 18, Issue:1

    Topics: Acute Disease; Arginine; Heme; Hemin; Humans; Porphyrias, Hepatic

1998
Hepatocellular carcinoma in patients with acute hepatic porphyria: frequency of occurrence and related factors.
    Journal of hepatology, 2000, Volume: 32, Issue:6

    Topics: Acute Disease; Adult; Carcinoma, Hepatocellular; Cohort Studies; Female; Heme; Humans; Incidence; Liver Neoplasms; Male; Melatonin; Middle Aged; Porphyrias; Porphyrias, Hepatic; Prospective Studies; Risk Factors; Survival Analysis

2000
Hereditary coproporphyria in Germany: clinical-biochemical studies in 53 patients.
    Clinical biochemistry, 2000, Volume: 33, Issue:6

    Topics: Adolescent; Adult; Aged; Aged, 80 and over; Aminolevulinic Acid; Arginine; Child; Chromatography, High Pressure Liquid; Coproporphyrinogen Oxidase; Coproporphyrins; Feces; Female; Germany; Heme; Heterozygote; Humans; Isomerism; Male; Middle Aged; Porphobilinogen; Porphyrias, Hepatic; Uroporphyrins

2000
Zinc mesoporphyrin represses induced hepatic 5-aminolevulinic acid synthase and reduces heme oxygenase activity in a mouse model of acute hepatic porphyria.
    Hepatology (Baltimore, Md.), 2001, Volume: 33, Issue:5

    Topics: 5-Aminolevulinate Synthetase; Acute Disease; Aminolevulinic Acid; Animals; Arginine; Cytosol; Drug Combinations; Female; Heme; Heme Oxygenase (Decyclizing); Injections, Intraperitoneal; Liver; Metalloporphyrins; Mice; Mice, Inbred C57BL; Mitochondria, Liver; Phenobarbital; Porphyria, Acute Intermittent; Porphyrias, Hepatic; Reference Values; RNA, Messenger

2001
Porphyria with double errors in the heme biosynthetic pathway.
    Journal of neurology, 2001, Volume: 248, Issue:4

    Topics: Acute Disease; Adult; Encephalitis, Viral; Female; Heme; Humans; Pedigree; Phenotype; Porphobilinogen Synthase; Porphyrias, Hepatic; Seizures

2001
Haem arginate interferes with estimation of carcinoembryonic antigen.
    Annals of clinical biochemistry, 2001, Volume: 38, Issue:Pt 3

    Topics: Adult; alpha-Fetoproteins; Arginine; Carcinoembryonic Antigen; Carcinoma, Hepatocellular; Chemistry, Clinical; Erythrocytes; False Positive Reactions; Female; Heme; Hemoglobins; Humans; Porphyrias, Hepatic; Prostate-Specific Antigen

2001
The primary enzyme defect in hereditary coproporphyria.
    Lancet (London, England), 1976, Dec-04, Volume: 2, Issue:7997

    Topics: Adult; Aged; Chromosome Aberrations; Chromosome Disorders; Coproporphyrinogens; Coproporphyrins; Feces; Female; Fibroblasts; Heme; Humans; Hydroxymethylbilane Synthase; Liver; Male; Oxidoreductases; Porphyrias; Porphyrias, Hepatic; Skin

1976