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Favism

MedGen UID:
4667
•Concept ID:
C0015702
•
Disease or Syndrome
Synonym: Favisms
SNOMED CT: Favism (191172001)
 
Monarch Initiative: MONDO:0001761

Definition

A condition associated with glucose-6-phosphate dehydrogenase deficiency, which is characterized by hemolysis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFavism

Professional guidelines

PubMed

Glucose-6-phosphate dehydrogenase deficiency in Tunisia: molecular data and phenotype-genotype association.
Laouini N, Bibi A, Ammar H, Kazdaghli K, Ouali F, Othmani R, Amdouni S, Haloui S, Sahli CA, Jouini L, Hadj Fredj S, Siala H, Ben Romdhane N, Toumi NE, Fattoum S, Messsaoud T
Mol Biol Rep 2013 Feb;40(2):851-6. Epub 2012 Oct 14 doi: 10.1007/s11033-012-2124-8. PMID: 23065279
Marked decline of favism after neonatal glucose-6-phosphate dehydrogenase screening and health education: the northern Sardinian experience.
Meloni T, Forteleoni G, Meloni GF
Acta Haematol 1992;87(1-2):29-31. doi: 10.1159/000204709. PMID: 1585769
Treatment of favism with cortisone.
BECKER AH
J Am Med Assoc 1954 Jul 24;155(13):1158. doi: 10.1001/jama.1954.73690310008007g. PMID: 13174371

Recent clinical studies

Etiology

Favism: Clinical Features at Different Ages.
Beretta A, Manuelli M, Cena H
Nutrients 2023 Jan 10;15(2) doi: 10.3390/nu15020343. PMID: 36678214Free PMC Article
G6PD deficiency: An update.
Harcke SJ, Rizzolo D, Harcke HT
JAAPA 2019 Nov;32(11):21-26. doi: 10.1097/01.JAA.0000586304.65429.a7. PMID: 31609781
Perioperative management of the glucose-6-phosphate dehydrogenase deficient patient: a review of literature.
Elyassi AR, Rowshan HH
Anesth Prog 2009 Autumn;56(3):86-91. doi: 10.2344/0003-3006-56.3.86. PMID: 19769422Free PMC Article
Glucose-6-phosphate dehydrogenase deficiency.
Mehta A, Mason PJ, Vulliamy TJ
Baillieres Best Pract Res Clin Haematol 2000 Mar;13(1):21-38. doi: 10.1053/beha.1999.0055. PMID: 10916676
Glucose-6-phosphate: a key compound in glycogenosis I and favism leading to hyper- or hypolipidaemia.
Schmitz G, Hohage H, Ullrich K
Eur J Pediatr 1993;152 Suppl 1:S77-84. doi: 10.1007/BF02072094. PMID: 8319730

Diagnosis

Favism: Clinical Features at Different Ages.
Beretta A, Manuelli M, Cena H
Nutrients 2023 Jan 10;15(2) doi: 10.3390/nu15020343. PMID: 36678214Free PMC Article
Review and drug therapy implications of glucose-6-phosphate dehydrogenase deficiency.
Belfield KD, Tichy EM
Am J Health Syst Pharm 2018 Feb 1;75(3):97-104. Epub 2018 Jan 5 doi: 10.2146/ajhp160961. PMID: 29305344
Glucose-6-Phosphate Dehydrogenase Deficiency.
Luzzatto L, Nannelli C, Notaro R
Hematol Oncol Clin North Am 2016 Apr;30(2):373-93. doi: 10.1016/j.hoc.2015.11.006. PMID: 27040960
Acid phosphatases.
Bull H, Murray PG, Thomas D, Fraser AM, Nelson PN
Mol Pathol 2002 Apr;55(2):65-72. doi: 10.1136/mp.55.2.65. PMID: 11950951Free PMC Article
Glucose-6-phosphate dehydrogenase deficiency.
Mehta A, Mason PJ, Vulliamy TJ
Baillieres Best Pract Res Clin Haematol 2000 Mar;13(1):21-38. doi: 10.1053/beha.1999.0055. PMID: 10916676

Therapy

Review and drug therapy implications of glucose-6-phosphate dehydrogenase deficiency.
Belfield KD, Tichy EM
Am J Health Syst Pharm 2018 Feb 1;75(3):97-104. Epub 2018 Jan 5 doi: 10.2146/ajhp160961. PMID: 29305344
Favism and Glucose-6-Phosphate Dehydrogenase Deficiency.
Luzzatto L, Arese P
N Engl J Med 2018 Jan 4;378(1):60-71. doi: 10.1056/nejmra1708111. PMID: 29298156
New legume sources as therapeutic agents.
Madar Z, Stark AH
Br J Nutr 2002 Dec;88 Suppl 3:S287-92. doi: 10.1079/BJN2002719. PMID: 12498629
Hemolysis in glucose-6-phosphate dehydrogenase deficiency.
Motulsky AG
Fed Proc 1972 Jul-Aug;31(4):1286-92. PMID: 4556779
L-dopa and favism.
Braham J, Sarova-Pinhas I
Blood 1971 Jun;37(6):744-5. PMID: 5162316

Prognosis

A novel G6PD gene variant in a Chinese girl with favism.
Shen S, Xiong Q, Cai W, Xiong H, Hu X
J Clin Lab Anal 2020 Sep;34(9):e23402. Epub 2020 Jun 17 doi: 10.1002/jcla.23402. PMID: 32557798Free PMC Article
Clinical complications of G6PD deficiency in Latin American and Caribbean populations: systematic review and implications for malaria elimination programmes.
Monteiro WM, Franca GP, Melo GC, Queiroz AL, Brito M, Peixoto HM, Oliveira MR, Romero GA, Bassat Q, Lacerda MV
Malar J 2014 Feb 25;13:70. doi: 10.1186/1475-2875-13-70. PMID: 24568147Free PMC Article
At least five polymorphic mutants account for the prevalence of glucose-6-phosphate dehydrogenase deficiency in Algeria.
Nafa K, Reghis A, Osmani N, Baghli L, Aït-Abbes H, Benabadji M, Kaplan JC, Vulliamy T, Luzzatto L
Hum Genet 1994 Nov;94(5):513-7. doi: 10.1007/BF00211017. PMID: 7959686
Glucose-6-phosphate dehydrogenase enzyme activity in normal, hemizygote and heterozygote Kelantanese Malays.
Normah J, Choo KE, Oppenheimer SJ, Selamah G
J Paediatr Child Health 1991 Dec;27(6):376-9. doi: 10.1111/j.1440-1754.1991.tb00423.x. PMID: 1756082
Favism: impairment of proteolytic systems in red blood cells.
Morelli A, Grasso M, Meloni T, Forteleoni G, Zocchi E, De Flora A
Blood 1987 Jun;69(6):1753-8. PMID: 3034353

Clinical prediction guides

Molecular mediators of favism-induced acute kidney injury.
García-Camín RM, Goma M, Osuna RG, Rubio-Navarro A, Buendía I, Ortiz A, Egido J, Manzarbeitia F, Chevarria JL, Gluksmann MC, Moreno JA
Clin Nephrol 2014 Mar;81(3):203-9. doi: 10.5414/CN107520. PMID: 23006341
Perioperative management of the glucose-6-phosphate dehydrogenase deficient patient: a review of literature.
Elyassi AR, Rowshan HH
Anesth Prog 2009 Autumn;56(3):86-91. doi: 10.2344/0003-3006-56.3.86. PMID: 19769422Free PMC Article
Pathophysiology of favism.
Arese P, Mannuzzu L, Turrini F
Folia Haematol Int Mag Klin Morphol Blutforsch 1989;116(5):745-52. PMID: 2481620
Favism: disordered erythrocyte calcium homeostasis.
De Flora A, Benatti U, Guida L, Forteleoni G, Meloni T
Blood 1985 Aug;66(2):294-7. PMID: 2410063
The epidemiology of favism.
Belsey MA
Bull World Health Organ 1973;48(1):1-13. PMID: 4541143Free PMC Article

Recent systematic reviews

Laboratory diagnosis of G6PD deficiency. A British Society for Haematology Guideline.
Roper D, Layton M, Rees D, Lambert C, Vulliamy T, De la Salle B, D'Souza C; British Society for Haematology
Br J Haematol 2020 Apr;189(1):24-38. Epub 2020 Jan 28 doi: 10.1111/bjh.16366. PMID: 31991476
Clinical complications of G6PD deficiency in Latin American and Caribbean populations: systematic review and implications for malaria elimination programmes.
Monteiro WM, Franca GP, Melo GC, Queiroz AL, Brito M, Peixoto HM, Oliveira MR, Romero GA, Bassat Q, Lacerda MV
Malar J 2014 Feb 25;13:70. doi: 10.1186/1475-2875-13-70. PMID: 24568147Free PMC Article

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