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Reduced erythrocyte free porphyrin concentration

MedGen UID:
1841815
•Concept ID:
C5826723
•
Finding
HPO: HP:0034869

Definition

Concentration of porphyrin in erythrocytes below the lower limit of normal. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVReduced erythrocyte free porphyrin concentration

Recent clinical studies

Etiology

Elevated heme impairs macrophage phagocytosis in endometriosis.
Liu YY, Liu YK, Hu WT, Tang LL, Sheng YR, Wei CY, Li MQ, Zhu XY
Reproduction 2019 Sep;158(3):257-266. doi: 10.1530/REP-19-0028. PMID: 31299634
Effects of vitamin E administration on the hemorheological status and redox homeostasis of patients with porphyria cutanea tarda treated with phlebotomy.
Székely E, Vereckei A, Almási A, Rapavi E, Tasnádi G, Várnai K, Pallai Z, Lugasi A, Blázovics A
Clin Hemorheol Microcirc 2007;36(1):13-23. PMID: 17211058
Hemorheological status and redox homeostasis of phlebotomised porphyria cutanea tarda patients with diabetes mellitus and in moderate alcohol consumer.
Székely E, Bor M, Tasnádi G, Várnai K, Almási A, Blázovics A
Clin Hemorheol Microcirc 2006;35(3):387-96. PMID: 16899961
5-Aminolevulinic acid dehydratase deficiency porphyria: a twenty-year clinical and biochemical follow-up.
Gross U, Sassa S, Jacob K, Deybach JC, Nordmann Y, Frank M, Doss MO
Clin Chem 1998 Sep;44(9):1892-6. PMID: 9732973
Porphyrin metabolism in some malignant diseases.
el-Sharabasy MM, el-Waseef AM, Hafez MM, Salim SA
Br J Cancer 1992 Mar;65(3):409-12. doi: 10.1038/bjc.1992.83. PMID: 1558795Free PMC Article

Diagnosis

Exacerbation of erythropoietic protoporphyria by hyperthyroidism.
Minder EI, Haldemann AR, Schneider-Yin X
J Inherit Metab Dis 2010 Dec;33 Suppl 3:S465-9. Epub 2010 Nov 11 doi: 10.1007/s10545-010-9234-z. PMID: 21069463
Hypoxia inducible factor-1 modulates hemin-induced IL-8 secretion in microvascular endothelium.
Natarajan R, Fisher BJ, Fowler AA 3rd
Microvasc Res 2007 May;73(3):163-72. Epub 2007 Feb 8 doi: 10.1016/j.mvr.2007.01.002. PMID: 17336340
Evolution of lead toxicity in a population of children.
Calderón-Salinas JV, Hernandez-Luna C, Valdez-Anaya B, Maldonado-Vega M, Lopez-Miranda A
Hum Exp Toxicol 1996 May;15(5):376-82. doi: 10.1177/096032719601500502. PMID: 8735459
Biosynthesis of heme in immature erythroid cells. The regulatory step for heme formation in the human erythron.
Gardner LC, Cox TM
J Biol Chem 1988 May 15;263(14):6676-82. PMID: 3360800
Industrial lead poisoning in China over the past 33 years.
Wang YL
Ecotoxicol Environ Saf 1984 Dec;8(6):526-30. doi: 10.1016/0147-6513(84)90012-5. PMID: 6391901

Therapy

Exacerbation of erythropoietic protoporphyria by hyperthyroidism.
Minder EI, Haldemann AR, Schneider-Yin X
J Inherit Metab Dis 2010 Dec;33 Suppl 3:S465-9. Epub 2010 Nov 11 doi: 10.1007/s10545-010-9234-z. PMID: 21069463
Effects of vitamin E administration on the hemorheological status and redox homeostasis of patients with porphyria cutanea tarda treated with phlebotomy.
Székely E, Vereckei A, Almási A, Rapavi E, Tasnádi G, Várnai K, Pallai Z, Lugasi A, Blázovics A
Clin Hemorheol Microcirc 2007;36(1):13-23. PMID: 17211058
Anaesthetic management of a patient with erythropoietic protoporphyria for ventricular septal defect closure.
Asokumar B, Kierney C, James TW, Amato J, Tumanmd KJ
Paediatr Anaesth 1999;9(4):356-8. doi: 10.1046/j.1460-9592.1999.00338.x. PMID: 10411776
5-Aminolevulinic acid dehydratase deficiency porphyria: a twenty-year clinical and biochemical follow-up.
Gross U, Sassa S, Jacob K, Deybach JC, Nordmann Y, Frank M, Doss MO
Clin Chem 1998 Sep;44(9):1892-6. PMID: 9732973
Evolution of lead toxicity in a population of children.
Calderón-Salinas JV, Hernandez-Luna C, Valdez-Anaya B, Maldonado-Vega M, Lopez-Miranda A
Hum Exp Toxicol 1996 May;15(5):376-82. doi: 10.1177/096032719601500502. PMID: 8735459

Prognosis

5-Aminolevulinic acid dehydratase deficiency porphyria: a twenty-year clinical and biochemical follow-up.
Gross U, Sassa S, Jacob K, Deybach JC, Nordmann Y, Frank M, Doss MO
Clin Chem 1998 Sep;44(9):1892-6. PMID: 9732973

Clinical prediction guides

Elevated heme impairs macrophage phagocytosis in endometriosis.
Liu YY, Liu YK, Hu WT, Tang LL, Sheng YR, Wei CY, Li MQ, Zhu XY
Reproduction 2019 Sep;158(3):257-266. doi: 10.1530/REP-19-0028. PMID: 31299634
Hemorheological status and redox homeostasis of phlebotomised porphyria cutanea tarda patients with diabetes mellitus and in moderate alcohol consumer.
Székely E, Bor M, Tasnádi G, Várnai K, Almási A, Blázovics A
Clin Hemorheol Microcirc 2006;35(3):387-96. PMID: 16899961
Prooxidant activity of beta-hematin (synthetic malaria pigment) in arachidonic acid micelles and phospholipid large unilamellar vesicles.
Omodeo-Salè F, Monti D, Olliaro P, Taramelli D
Biochem Pharmacol 2001 Apr 15;61(8):999-1009. doi: 10.1016/s0006-2952(01)00558-5. PMID: 11286991
Magnetic field effects on the photohemolysis of human erythrocytes by ketoprofen and protoporphyrin IX.
Chignell CF, Sik RH
Photochem Photobiol 1995 Jul;62(1):205-7. doi: 10.1111/j.1751-1097.1995.tb05260.x. PMID: 7638268
Inhibition of membrane erythrocyte (Ca2+ + Mg2+)-ATPase by hemin.
Leclerc L, Vasseur C, Bursaux E, Marden M, Poyart C
Biochim Biophys Acta 1988 Dec 8;946(1):49-56. doi: 10.1016/0005-2736(88)90456-7. PMID: 2974727

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