The prevalence of phenylketonuria (PKU) in the US is estimated to be about 1 in 25,000 newborns.[3]Elhawary NA, AlJahdali IA, Abumansour IS, et al. Genetic etiology and clinical challenges of phenylketonuria. Hum Genomics. 2022 Jul 19;16(1):22.
https://humgenomics.biomedcentral.com/articles/10.1186/s40246-022-00398-9
http://www.ncbi.nlm.nih.gov/pubmed/35854334?tool=bestpractice.com
[4]Hillert A, Anikster Y, Belanger-Quintana A, et al. The Genetic Landscape and Epidemiology of Phenylketonuria. Am J Hum Genet. 2020 Aug 6;107(2):234-50.
https://www.cell.com/ajhg/fulltext/S0002-9297(20)30194-4
http://www.ncbi.nlm.nih.gov/pubmed/32668217?tool=bestpractice.com
Black, Asian, and Hispanic infants are less commonly affected due to decreased carrier frequency.[5]Kaye CI, Accurso F; Committee on Genetics, et al. Newborn screening fact sheets. Pediatrics. 2006 Sep;118(3):e934-63.
https://publications.aap.org/pediatrics/article/118/3/e934/69364/Newborn-Screening-Fact-Sheets
http://www.ncbi.nlm.nih.gov/pubmed/16950973?tool=bestpractice.com
Global data parallel what is observed in the US, with a global prevalence 1:23,930 live births.[4]Hillert A, Anikster Y, Belanger-Quintana A, et al. The Genetic Landscape and Epidemiology of Phenylketonuria. Am J Hum Genet. 2020 Aug 6;107(2):234-50.
https://www.cell.com/ajhg/fulltext/S0002-9297(20)30194-4
http://www.ncbi.nlm.nih.gov/pubmed/32668217?tool=bestpractice.com
PKU prevalence is highest in Europe, with most European countries having an incidence of between 1 in 5000 and 1 in 15,000 births.[3]Elhawary NA, AlJahdali IA, Abumansour IS, et al. Genetic etiology and clinical challenges of phenylketonuria. Hum Genomics. 2022 Jul 19;16(1):22.
https://humgenomics.biomedcentral.com/articles/10.1186/s40246-022-00398-9
http://www.ncbi.nlm.nih.gov/pubmed/35854334?tool=bestpractice.com
[4]Hillert A, Anikster Y, Belanger-Quintana A, et al. The Genetic Landscape and Epidemiology of Phenylketonuria. Am J Hum Genet. 2020 Aug 6;107(2):234-50.
https://www.cell.com/ajhg/fulltext/S0002-9297(20)30194-4
http://www.ncbi.nlm.nih.gov/pubmed/32668217?tool=bestpractice.com
An exception is Finland, where the disorder is extremely rare (1 in 112,000 births).[4]Hillert A, Anikster Y, Belanger-Quintana A, et al. The Genetic Landscape and Epidemiology of Phenylketonuria. Am J Hum Genet. 2020 Aug 6;107(2):234-50.
https://www.cell.com/ajhg/fulltext/S0002-9297(20)30194-4
http://www.ncbi.nlm.nih.gov/pubmed/32668217?tool=bestpractice.com
Asian countries have the lowest reported PKU such as Japan (1 in 125,000), Philippines (1 in 116,006), Singapore (1 in 83,333), and Thailand (1 in 227,273).[3]Elhawary NA, AlJahdali IA, Abumansour IS, et al. Genetic etiology and clinical challenges of phenylketonuria. Hum Genomics. 2022 Jul 19;16(1):22.
https://humgenomics.biomedcentral.com/articles/10.1186/s40246-022-00398-9
http://www.ncbi.nlm.nih.gov/pubmed/35854334?tool=bestpractice.com
[4]Hillert A, Anikster Y, Belanger-Quintana A, et al. The Genetic Landscape and Epidemiology of Phenylketonuria. Am J Hum Genet. 2020 Aug 6;107(2):234-50.
https://www.cell.com/ajhg/fulltext/S0002-9297(20)30194-4
http://www.ncbi.nlm.nih.gov/pubmed/32668217?tool=bestpractice.com
China is the exception (1 in 15,924), where PKU prevalence is comparable to that in Europe.[3]Elhawary NA, AlJahdali IA, Abumansour IS, et al. Genetic etiology and clinical challenges of phenylketonuria. Hum Genomics. 2022 Jul 19;16(1):22.
https://humgenomics.biomedcentral.com/articles/10.1186/s40246-022-00398-9
http://www.ncbi.nlm.nih.gov/pubmed/35854334?tool=bestpractice.com
[4]Hillert A, Anikster Y, Belanger-Quintana A, et al. The Genetic Landscape and Epidemiology of Phenylketonuria. Am J Hum Genet. 2020 Aug 6;107(2):234-50.
https://www.cell.com/ajhg/fulltext/S0002-9297(20)30194-4
http://www.ncbi.nlm.nih.gov/pubmed/32668217?tool=bestpractice.com
Data are not available from parts of Africa, Asia, South America, and Caribbean populations.[4]Hillert A, Anikster Y, Belanger-Quintana A, et al. The Genetic Landscape and Epidemiology of Phenylketonuria. Am J Hum Genet. 2020 Aug 6;107(2):234-50.
https://www.cell.com/ajhg/fulltext/S0002-9297(20)30194-4
http://www.ncbi.nlm.nih.gov/pubmed/32668217?tool=bestpractice.com
This is an autosomal-recessive disorder with males and females being equally affected. Newborn screening programmes were initiated in the US in the mid- to late 1960s.[6]Berry SA, Brown C, Grant M, et al. Newborn screening 50 years later: access issues faced by adults with PKU. Genet Med. 2013 Aug;15(8):591-9.
https://www.gimjournal.org/article/S1098-3600(21)02736-2/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/23470838?tool=bestpractice.com
Most individuals with PKU born before that time have intellectual disability.[3]Elhawary NA, AlJahdali IA, Abumansour IS, et al. Genetic etiology and clinical challenges of phenylketonuria. Hum Genomics. 2022 Jul 19;16(1):22.
https://humgenomics.biomedcentral.com/articles/10.1186/s40246-022-00398-9
http://www.ncbi.nlm.nih.gov/pubmed/35854334?tool=bestpractice.com