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aminolevulinic acid and Hepatic Porphyria

aminolevulinic acid has been researched along with Hepatic Porphyria in 20 studies

Aminolevulinic Acid: A compound produced from succinyl-CoA and GLYCINE as an intermediate in heme synthesis. It is used as a PHOTOCHEMOTHERAPY for actinic KERATOSIS.
5-aminolevulinic acid : The simplest delta-amino acid in which the hydrogens at the gamma position are replaced by an oxo group. It is metabolised to protoporphyrin IX, a photoactive compound which accumulates in the skin. Used (in the form of the hydrochloride salt)in combination with blue light illumination for the treatment of minimally to moderately thick actinic keratosis of the face or scalp.

Research Excerpts

ExcerptRelevanceReference
" As a small interfering RNA that is selectively taken up in the liver, both the mechanism and targeted delivery create a complex relationship between givosiran pharmacokinetics (PK) and the pharmacodynamic (PD) response."5.91Pharmacokinetic-pharmacodynamic model of urinary δ-aminolevulinic acid reduction after givosiran treatment in patients with acute hepatic porphyria. ( Lee, J; Melch, M; Robbie, GJ, 2023)
" The final model was used to assess the adequacy of the recommended givosiran dosing regimen across demographic and clinical subgroups."5.91Pharmacokinetic-pharmacodynamic model of urinary δ-aminolevulinic acid reduction after givosiran treatment in patients with acute hepatic porphyria. ( Lee, J; Melch, M; Robbie, GJ, 2023)
"The acute hepatic porphyrias (AHP) are rare, inborn errors of heme-metabolism and include acute intermittent porphyria, hereditary coproporphyria, variegate porphyria, and porphyria due to severe deficiency of 5-aminolevulinic acid dehydratase."5.41AGA Clinical Practice Update on Diagnosis and Management of Acute Hepatic Porphyrias: Expert Review. ( Balwani, M; Bonkovsky, HL; Lim, JK; Wang, B, 2023)
"The acute hepatic porphyrias (AHPs) are inherited disorders of heme biosynthesis characterized by life-threatening acute neurovisceral attacks precipitated by factors that upregulate hepatic 5-aminolevulinic acid synthase 1 (ALAS1) activity."4.31RNA interference therapy in acute hepatic porphyrias. ( Balwani, M; Keel, S; Yasuda, M, 2023)
"The quantification of delta-aminolevulinic acid (ALA) and porphobilinogen (PBG) in urine are the first-line tests for diagnosis and monitoring of acute hepatic porphyrias (AHP)."4.31Quantification of Urine and Plasma Porphyrin Precursors Using LC-MS in Acute Hepatic Porphyrias: Improvement in Routine Diagnosis and in the Monitoring of Kidney Failure Patients. ( Dessendier, N; Gouya, L; Junot, C; Lefebvre, T; Manceau, H; Moulouel, B; Nguyen, AL; Poli, A; Puy, H; Schmitt, C; Talbi, N, 2023)
"A new form of acute hepatic porphyria with double genetic defect--deficiency of porphobilinogen deaminase and coproporphyrinogen oxidase--is described."3.69Coexistence of hereditary coproporphyria with acute intermittent porphyria. ( Gregor, A; Kostrzewska, E; Stachurska, H; Tarczynska-Nosal, S, 1994)
"Acute intermittent porphyria is the most common type of AHP, with an estimated prevalence of patients with symptoms of approximately 1 in 100,000."3.01AGA Clinical Practice Update on Diagnosis and Management of Acute Hepatic Porphyrias: Expert Review. ( Balwani, M; Bonkovsky, HL; Lim, JK; Wang, B, 2023)
"The acute hepatic porphyrias (AHP) are rare, inborn errors of heme-metabolism and include acute intermittent porphyria, hereditary coproporphyria, variegate porphyria, and porphyria due to severe deficiency of 5-aminolevulinic acid dehydratase."3.01AGA Clinical Practice Update on Diagnosis and Management of Acute Hepatic Porphyrias: Expert Review. ( Balwani, M; Bonkovsky, HL; Lim, JK; Wang, B, 2023)
"Acute hepatic porphyrias are inherited metabolic disorders that may present with polyneuropathy, which if not diagnosed early can lead to quadriparesis, respiratory weakness, and death."2.72Porphyric neuropathy. ( Bonkovsky, HL; Caress, JB; Gandhi Mehta, RK; Rudnick, SR, 2021)
" As a small interfering RNA that is selectively taken up in the liver, both the mechanism and targeted delivery create a complex relationship between givosiran pharmacokinetics (PK) and the pharmacodynamic (PD) response."1.91Pharmacokinetic-pharmacodynamic model of urinary δ-aminolevulinic acid reduction after givosiran treatment in patients with acute hepatic porphyria. ( Lee, J; Melch, M; Robbie, GJ, 2023)
" The final model was used to assess the adequacy of the recommended givosiran dosing regimen across demographic and clinical subgroups."1.91Pharmacokinetic-pharmacodynamic model of urinary δ-aminolevulinic acid reduction after givosiran treatment in patients with acute hepatic porphyria. ( Lee, J; Melch, M; Robbie, GJ, 2023)
"The acute hepatic porphyrias (AHPs) are inherited disorders of heme biosynthesis characterized by life-threatening acute neurovisceral attacks precipitated by factors that upregulate hepatic 5-aminolevulinic acid synthase 1 (ALAS1) activity."1.91RNA interference therapy in acute hepatic porphyrias. ( Balwani, M; Keel, S; Yasuda, M, 2023)
"Patients with acute hepatic porphyria are denied essential operations because of concern that general anaesthesia and surgery will precipitate a life threatening porphyric crisis."1.29Safety of general anaesthesia and surgery in acute hepatic porphyria. ( Dover, SB; McColl, KE; Moore, MR; Plenderleith, L, 1994)
"A new form of acute hepatic porphyria with double genetic defect--deficiency of porphobilinogen deaminase and coproporphyrinogen oxidase--is described."1.29Coexistence of hereditary coproporphyria with acute intermittent porphyria. ( Gregor, A; Kostrzewska, E; Stachurska, H; Tarczynska-Nosal, S, 1994)

Research

Studies (20)

TimeframeStudies, this research(%)All Research%
pre-19902 (10.00)18.7374
1990's8 (40.00)18.2507
2000's5 (25.00)29.6817
2010's0 (0.00)24.3611
2020's5 (25.00)2.80

Authors

AuthorsStudies
Wang, B1
Bonkovsky, HL2
Lim, JK1
Balwani, M2
Lee, J1
Melch, M1
Robbie, GJ1
Yasuda, M1
Keel, S1
Poli, A1
Manceau, H1
Nguyen, AL1
Moulouel, B1
Dessendier, N1
Talbi, N1
Puy, H1
Junot, C1
Gouya, L1
Schmitt, C1
Lefebvre, T1
Gandhi Mehta, RK1
Caress, JB1
Rudnick, SR1
Hift, RJ1
Todd, G1
Meissner, PN1
Kirsch, RE1
López Montes, A1
Lorenzo, I1
Pérez Martínez, J1
Bloomer, JR1
McGuire, BM1
Romeo, G1
Böhrer, H1
Schmidt, H1
Martin, E1
Lux, R1
Bolsen, K1
Goerz, G1
Sieg, I1
Bhutani, LK1
Doss, MO3
Gross, U2
Honcamp, M1
Daume, E1
Frank, M1
Düsterberg, B1
Dover, SB1
Plenderleith, L1
Moore, MR1
McColl, KE1
Gregor, A1
Kostrzewska, E1
Tarczynska-Nosal, S1
Stachurska, H1
Yano, Y1
Kondo, M1
Kühnel, A1
Schuurmans, MM1
Hoffmann, F1
Lindberg, RL1
Meyer, UA1
Hermes-Lima, M1
Castilho, RF1
Valle, VG1
Bechara, EJ1
Vercesi, AE1
Akagi, R1
Prchal, JT1
Eberhart, CE1
Sassa, S1
Rojas, F1
Aguilera, S1

Reviews

4 reviews available for aminolevulinic acid and Hepatic Porphyria

ArticleYear
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; Hem

2023
Porphyric neuropathy.
    Muscle & nerve, 2021, Volume: 64, Issue:2

    Topics: Aminolevulinic Acid; Guillain-Barre Syndrome; Humans; Peripheral Nervous System Diseases; Polyneurop

2021
The hepatic porphyrias.
    Progress in medical genetics, 1980, Volume: 4

    Topics: 5-Aminolevulinate Synthetase; Acute Disease; Adolescent; Adult; Aminolevulinic Acid; Chemical Phenom

1980
[Hereditary coproporphyria (HCP)].
    Ryoikibetsu shokogun shirizu, 1998, Issue:19 Pt 2

    Topics: Aminolevulinic Acid; Biomarkers; Diagnosis, Differential; Mutation; Porphobilinogen; Porphobilinogen

1998

Trials

1 trial available for aminolevulinic acid and Hepatic Porphyria

ArticleYear
Administration of oral activated charcoal in variegate porphyria results in a paradoxical clinical and biochemical deterioration.
    The British journal of dermatology, 2003, Volume: 149, Issue:6

    Topics: Administration, Oral; Adolescent; Adult; Aminolevulinic Acid; Analysis of Variance; Charcoal; Female

2003

Other Studies

15 other studies available for aminolevulinic acid and Hepatic Porphyria

ArticleYear
Pharmacokinetic-pharmacodynamic model of urinary δ-aminolevulinic acid reduction after givosiran treatment in patients with acute hepatic porphyria.
    CPT: pharmacometrics & systems pharmacology, 2023, Volume: 12, Issue:6

    Topics: Adolescent; Adult; Aminolevulinic Acid; Humans; Porphobilinogen Synthase; Porphyrias, Hepatic

2023
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 Inter

2023
Quantification of Urine and Plasma Porphyrin Precursors Using LC-MS in Acute Hepatic Porphyrias: Improvement in Routine Diagnosis and in the Monitoring of Kidney Failure Patients.
    Clinical chemistry, 2023, 10-03, Volume: 69, Issue:10

    Topics: Aminolevulinic Acid; Chromatography, Liquid; Humans; Porphobilinogen; Porphyrias; Porphyrias, Hepati

2023
[Porphyria and inappropriate antidiuretic hormone syndrome].
    Nefrologia : publicacion oficial de la Sociedad Espanola Nefrologia, 2004, Volume: 24 Suppl 3

    Topics: Abdominal Pain; Adult; Aminolevulinic Acid; Coproporphyrins; Diuretics; Endometriosis; Female; Furos

2004
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; Contra

2007
Testing the porphyrinogenicity of propofol in a primed rat model.
    British journal of anaesthesia, 1995, Volume: 75, Issue:3

    Topics: 5-Aminolevulinate Synthetase; Allylisopropylacetamide; Aminolevulinic Acid; Anesthetics, Intravenous

1995
Dual porphyria of coexisting variegata and cutanea tarda.
    European journal of clinical chemistry and clinical biochemistry : journal of the Forum of European Clinical Chemistry Societies, 1995, Volume: 33, Issue:7

    Topics: Adult; Aged; Aminolevulinic Acid; Feces; Female; Humans; Male; Porphobilinogen; Porphyria Cutanea Ta

1995
Hormonal oral contraceptives, urinary porphyrin excretion and porphyrias.
    Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme, 1995, Volume: 27, Issue:8

    Topics: Adult; Aminolevulinic Acid; Contraceptives, Oral, Combined; Contraceptives, Oral, Hormonal; Copropor

1995
Safety of general anaesthesia and surgery in acute hepatic porphyria.
    Gut, 1994, Volume: 35, Issue:8

    Topics: Acute Disease; Adult; Aged; Aminolevulinic Acid; Anesthesia, General; Child, Preschool; Contraindica

1994
Coexistence of hereditary coproporphyria with acute intermittent porphyria.
    Annals of medicine, 1994, Volume: 26, Issue:2

    Topics: Adolescent; Adult; Aged; Aminolevulinic Acid; Child; Female; Humans; Male; Middle Aged; Pedigree; Po

1994
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, Hi

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 C

2001
Calcium-dependent mitochondrial oxidative damage promoted by 5-aminolevulinic acid.
    Biochimica et biophysica acta, 1992, Dec-10, Volume: 1180, Issue:2

    Topics: Aminolevulinic Acid; Animals; Calcium; Cell Membrane Permeability; Cell Size; Egtazic Acid; Magnesiu

1992
An acquired acute hepatic porphyria: a novel type of delta-aminolevulinate dehydratase inhibition.
    Clinica chimica acta; international journal of clinical chemistry, 1992, Nov-16, Volume: 212, Issue:1-2

    Topics: Acute Disease; Aminolevulinic Acid; Blotting, Western; Colorimetry; Dithiothreitol; Humans; Male; Mi

1992
[Ion-exchange chromatography in the diagnosis of hepatic porphyria].
    Revista medica de Chile, 1974, Volume: 102, Issue:2

    Topics: Amines; Aminolevulinic Acid; Anion Exchange Resins; Chromatography, Ion Exchange; Humans; Ketones; P

1974