Background
Classification | Pretreatment blood phenylalanine concentration | Treatment recommended? | |
---|---|---|---|
European guidelinesa | ACMGb | ||
Classical PKU | > 1200 µmol/L (> 20 mg/dL) | Yes | Yes |
Moderate PKU | 900–1200 µmol/L (15–20 mg/dL) | Yes | Yes |
Mild PKU | 600–900 µmol/L (10–15 mg/dL) | Yes | Yes |
Mild HPA-gray zone | 360–600 µmol/L (6–10 mg/dL) | Yes (only if < 12 years or in women before/during pregnancy) | Yesc |
PAH deficiency not requiring treatment | 120–360 µmol/L (2–6 mg/dL) | No | No |
Methods
Birth prevalence
Literature search
Study selection
Data extraction and quality assessment
Scoring domain | Score | ||
---|---|---|---|
Strong | Moderate | Weak | |
Case definitiona | The case definition is complete (including both screening positive and confirmed cases) | The case definition is partially complete (lacks either the definition of screening positive or of confirmed cases) | The case definition is incomplete for both screening positive and confirmed cases |
Study setting/source population | Mandatory population-wide newborn screening program General population from a well-defined region and time | Catchment area of a hospital or other medical facility Hospital or laboratory records or disease registry Surveys (e.g., to health care providers) | Personal communication Unclear or not reported |
Statistical methods | The denominator is the number of newborns screened, and cases in the numerator arise from the population in the denominator If any quantity is estimated rather than directly measured, estimations are in line with the criteria described here | The denominator is the overall number of births rather than the number screened | Cases in the numerator do not arise from the population in the denominator Unclear or not reported |
Precision of prevalence estimateb | Half the width of the 95% confidence interval is less than half of the prevalence | Half the width of the 95% confidence interval is between half of the prevalence and the prevalencec | Half the width of the 95% confidence interval is greater than the prevalence Confidence interval is not estimable |
Diagnostic method used for case confirmationd | Tandem mass spectrometry, high-performance liquid chromatography, column chromatography, (rapid) ion exchange chromatography, quantitative amino acid analyzer, positive mutational analysis, or enzymatic assay (including colorimetric, fluorimetric, and ELISA) | Guthrie test, bacterial inhibition assay, thin layer or paper chromatography | Other methods, or those where urine is used as the assay substrate Unclear or not reported |
Meta-analyses
Results
Literature search and review
Meta-analysis results
Country | Birth prevalence per 10,000 newborns (95% CI) | Phe cutoff value for confirmatory diagnosis (µmol/L) | Score for additional quality of evidence domainse | References | |||
---|---|---|---|---|---|---|---|
Case definition | Study setting source population | Statistical methods | Precision of prevalence estimate | ||||
Europe | |||||||
Austria (Eastern, PKUf) | 1.3 (0.92–1.83)d | NR | Weak | Strong | Strong | Strong | Thalhammer [49] |
Austria (Eastern, HPAf) | 0.49 (0.28–0.85)d | NR | Weak | Strong | Strong | Moderate | Thalhammer [49] |
Austria (Western, PKUf) | 0.45 (0.23–0.88)d | NR | Weak | Strong | Strong | Moderate | Thalhammer [49] |
Austria (Western, HPAf) | 0.5 (0.26–0.96)d | NR | Weak | Strong | Strong | Moderate | Thalhammer [49] |
Estonia | 0 (0–1.02)d | < 600 | Strong | Strong | Strong | Weak | Ounap et al. [38] |
Estonia | 1.66 (0.76–3.63)c,d | 1000 | Strong | Strong | Strong | Moderate | Ounap et al. [38] |
Finland | 0 (0–0.52)a,d | 363 | Moderate | Strong | Strong | Weak | Visakorpi et al. [39] |
Germany | 0.81 (0.66–1)d | NR | Weak | Strong | Strong | Strong | Lindner et al. [50] |
Germany | 0.78 (0.63–0.97)b,d | 600 | Moderate | Strong | Strong | Strong | Lindner et al. [50] |
Germany | 0.81 (0.65–1.01)a,d | 363– < 908 | Strong | Strong | Strong | Strong | Mathias and Bickel [51] |
Germany | 0.99 (0.81–1.21)a,d | 908 | Strong | Strong | Strong | Strong | Mathias and Bickel [51] |
Germany | 0.96 (0.65–1.43) | 600 | Strong | Strong | Strong | Strong | Schulze et al. [52] |
Germany | 1.24 (0.87–1.76) | 150–600 | Strong | Strong | Strong | Strong | Schulze et al. [52] |
Greece | 0.44 (0.05–1.61)d | NR | Moderate | Moderate | Strong | Weak | Loukas et al. [53] |
Greece | 0.41 (0.31–0.56)c,d | 1211 | Strong | Strong | Strong | Strong | Missiou-Tsagaraki et al. (1988) [54] |
Hungary | 0.85 (0.39–1.86)d | NR | Weak | Strong | Strong | Moderate | Mehes et al. [55] |
Italy (PKUf) | 1.38 (0.85–2.23)d | NR | Moderate | Weak | Strong | Moderate | Antonozzi et al. [56] |
Italy (HPAf) | 0.26 (0.05–0.75)d | NR | Moderate | Weak | Strong | Weak | Antonozzi et al. [56] |
Italy | 0.22 (0.15–0.32)c,d | 1211 | Strong | Strong | Strong | Strong | Zaffanello et al. [57] |
Italy | 0.78 (0.63–0.96)d | NR | Moderate | Strong | Strong | Strong | Zaffanello et al. [57] |
Macedonia | 2.46 (0.06–13.68)d | 151 | Moderate | Strong | Strong | Weak | Kocova and Anastasovska [41] |
Poland | 0.28 (0.22–0.34)a,d | 363–1211 | Strong | Moderate | Strong | Strong | Cabalska et al. [58] |
Poland | 1.29 (1.16–1.42)a,c,d | 1211 | Strong | Moderate | Strong | Strong | Cabalska et al. [58] |
Portugal | 0.82 (0.56–1.2)a,d | 360 | Strong | Strong | Strong | Strong | Vilarinho et al. [59] |
Portugal | 0.38 (0.22–0.66)d | 150–360 | Strong | Strong | Strong | Moderate | Vilarinho et al. [59] |
Slovakia | 1.69 (1.45–1.98)d | NR | Weak | Strong | Strong | Strong | Dluholucký et al. [60] |
Slovenia | 0.98 (0.72–1.35)b,c,d | 1200 | Strong | Strong | Moderate | Strong | Smon et al. [61] |
Slovenia | 0.39 (0.24–0.64)b,d | 600–900 | Strong | Strong | Moderate | Moderate | Smon et al. [61] |
Slovenia | 0.1 (0.03–0.27)b,d | 900–1200 | Strong | Strong | Moderate | Weak | Smon et al. [61] |
Spain | 0.66 (0.22–1.55)d | 240 | Strong | Strong | Strong | Weak | Fernández-Iglesias et al. [62] |
USSR/Russia | 1.5 (0.98–2.3)b,c,d | 1200 | Moderate | Strong | Strong | Strong | Gerasimova et al. [63] |
USSR/Russia | 0.36 (0.12–0.84)b,d | 600–1200 | Moderate | Strong | Strong | Weak | Gerasimova et al. [63] |
United Kingdom | 0.49 (0.36–0.67)c,d | 1200 | Strong | Moderate | Strong | Strong | Walker et al. [64] |
United Kingdom | 0.19 (0.11–0.31)d | 240 | Strong | Moderate | Strong | Moderate | Walker et al. [64] |
Yugoslavia | 0.22 (0.1–0.48)b,d | 605–902 | Strong | Moderate | Strong | Moderate | Mardesic et al. [65] |
Yugoslavia | 0.69 (0.44–1.08)b,d | 908 | Strong | Moderate | Strong | Strong | Mardesic et al. [65] |
Latin America | |||||||
Brazil (Laboratory A, 2005) | 0.36 (0.12–0.84)b,d | 605 | Strong | Strong | Strong | Weak | Botler et al. [66] |
Brazil (Laboratory A, 2006) | 0.59 (0.31–1.12)b,d | 605 | Strong | Strong | Strong | Intermediate | Botler et al. [66] |
Brazil (Laboratory A, 2007) | 0.35 (0.11–0.82)b,d | 605 | Strong | Strong | Strong | Weak | Botler et al. [66] |
Brazil (Laboratory B, 2005) | 0.52 (0.06–1.9)b,d | 605 | Strong | Strong | Strong | Weak | Botler et al. [66] |
Brazil (Laboratory B, 2006) | 0.84 (0.17–2.45)b,d | 605 | Strong | Strong | Strong | Weak | Botler et al. [66] |
Brazil (Laboratory B, 2007) | 0.91 (0.11–3.28)b,d | 605 | Strong | Strong | Strong | Weak | Botler et al. [66] |
Brazil | 0.92 (0.25–2.36)a–d | 1211 | Strong | Strong | Strong | Weak | Ramalho et al. [67] |
Brazil | 0.23 (0.01–1.28)a,d | 302–604 | Strong | Strong | Strong | Weak | Ramalho et al. [67] |
Brazil | 0.23 (0.01–1.28)a,b,d | 606–1210 | Strong | Strong | Strong | Weak | Ramalho et al. [67] |
Chile | 0.53 (0.45–0.63)c,d | 1211 | Strong | Strong | Strong | Strong | Cornejo et al. [68] |
Chile | 0.98 (0.86–1.11)d | NR | Intermediate | Strong | Strong | Strong | Cornejo et al. [68] |
Middle East/North Africa | |||||||
Iran | 0.15 (0.07–0.32)b,c,d | 1211 | Strong | Strong | Strong | Intermediate | Abbaskhanian et al. [69] |
Iran | 0.29 (0.17–0.52)d | 121–605 | Strong | Strong | Strong | Intermediate | Abbaskhanian et al. [69] |
Iran | 0.22 (0.12–0.42)b,d | 606–1210 | Strong | Strong | Strong | Intermediate | Abbaskhanian et al. [69] |
Iran | 1.6 (1.11–2.31)a,d | 424 | Strong | Strong | Strong | Strong | Habib et al. [44] |
Iran | 0.52 (0.14–1.33)d | 121– < 1211 | Strong | Strong | Strong | Weak | Karamifar et al. [70] |
Iran | 0.39 (0.08–1.14)c,d | 1211 | Strong | Strong | Strong | Weak | Karamifar et al. [70] |
Iran | 1.92 (1.53–2.41)d | NR | Intermediate | Strong | Intermediate | Strong | Motamedi et al. [71] |
Saudi Arabia | 0.68 (0.52–0.89)d | 180 | Strong | Strong | Strong | Strong | Alfadhel et al. [72] |
Turkey (classical PKUf) | 1.35 (1.18–1.54)d | NR | Weak | Strong | Strong | Strong | Ozalp et al. [73] |
Turkey (mild PKUf) | 0.64 (0.52–0.77)d | NR | Weak | Strong | Strong | Strong | Ozalp et al. [73] |
Turkey (mild HPAf) | 0.36 (0.28–0.47)d | NR | Weak | Strong | Strong | Strong | Ozalp et al. [73] |
UAE | 0.76 (0.57–0.99)c,d | 1211 | Strong | Strong | Strong | Strong | Al Hosani et al. [74] |
North America | |||||||
Canada (Alberta) | 0.50 (CI not estimable) | NR | Weak | Intermediate | Strong | Weak | Somers and Favreau [37] |
Canada (Ontario, PKUf) | 0.60 (CI not estimable) | NR | Weak | Intermediate | Strong | Weak | Somers and Favreau [37] |
Canada (Ontario, HPAf) | 0.29 (CI not estimable) | NR | Weak | Intermediate | Strong | Weak | Somers and Favreau [37] |
US (NC) | 0.08 (0.01–0.3) | 157 | Intermediate | Strong | Strong | Weak | Frazier et al. [42] |
US (NC) | 0.46 (0.26–0.82) | 250 | Intermediate | Strong | Strong | Intermediate | Frazier et al. [42] |
US (NC) | 0.52 (0.39–0.69)a,d | 300 | Intermediate | Strong | Strong | Strong | Frazier et al. [42] |
US (NY) | 0.1 (0.05–0.2)d | 908– < 1211 | Strong | Strong | Intermediate | Intermediate | Hansen et al. [75] |
US (NY) | 0.53 (0.39–0.72)c,d | 1211 | Strong | Strong | Intermediate | Strong | Hansen et al. [75] |
US (NY) | 0.14 (0.07–0.25)d | NR | Intermediate | Strong | Intermediate | Intermediate | Hansen et al. [75] |
US (NY) | 0.7 (0.52–0.93)d | NR | Intermediate | Strong | Intermediate | Strong | Kelly and Palombi [76] |
US (MA) | 1.04 (0.62–1.75)d | NR | Intermediate | Strong | Strong | Intermediate | Maccready and Hussey [28] |
US (CT) | 0.83 (CI not estimable) | NR | Weak | Intermediate | Strong | Weak | Somers and Favreau [37] |
US (FL) | 1.00 (CI not estimable) | NR | Weak | Intermediate | Strong | Weak | Somers and Favreau [37] |
US (KS) | 0.80 (CI not estimable) | NR | Weak | Intermediate | Strong | Weak | Somers and Favreau [37] |
US (KY) | 0.87 (CI not estimable) | NR | Weak | Intermediate | Strong | Weak | Somers and Favreau [37] |
US (OK) | 0.59 (CI not estimable) | NR | Weak | Intermediate | Strong | Weak | Somers and Favreau [37] |
US (PA, PKUf) | 0.78 (CI not estimable) | NR | Weak | Intermediate | Strong | Weak | Somers and Favreau [37] |
US (PA, HPAf) | 0.18 (CI not estimable) | NR | Weak | Intermediate | Strong | Weak | Somers and Favreau [37] |
US (TX) | 0.38 (CI not estimable) | NR | Weak | Intermediate | Strong | Weak | Somers and Favreau [37] |
US (VA) | 0.57 (CI not estimable) | NR | Weak | Intermediate | Strong | Weak | Somers and Favreau [37] |
US (WV) | 0.67 (CI not estimable) | NR | Weak | Intermediate | Strong | Weak | Somers and Favreau [37] |
US (PA) | 0.43 (0.29–0.64)a,d | 363 | Strong | Strong | Strong | Strong | Wainer and Sideman [43] |
US (PA) | 0.9 (0.68–1.19)d | NR | Intermediate | Strong | Strong | Strong | Wainer and Sideman [43] |
US (New England) | 0.27 (0.13–0.56)d | NR | Intermediate | Strong | Strong | Intermediate | Zytkovicz et al. [77] |
US (New England) | 0.43 (0.24–0.77)d | NR | Intermediate | Strong | Strong | Intermediate | Zytkovicz et al. [77] |
Southeast Asia | |||||||
Thailand | 0.04 (0.01–0.08) | NR | Intermediate | Strong | Strong | Weak | Charoensiriwatana et al. [78] |
Thailand | 0.05 (0.02–0.11) | 1211 | Strong | Strong | Strong | Weak | Pangkanon et al. [79] |
Thailand | 0.03 (0.02–0.05)c,d | 1200 | Intermediate | Strong | Strong | Intermediate | Pangkanon et al. [80] |
Thailand | 0 (0–2.12) | NR | Intermediate | Intermediate | Strong | Weak | Ratrisawadi et al. [40] |
Thailand | 0.04 (0.01–0.1) | NR | Intermediate | Strong | Strong | Weak | Sutivijit et al. [81] |
West Pacific | |||||||
Australia | 0.26 (0.09–0.61)d | 200–300 | Intermediate | Intermediate | Strong | Weak | Boneh et al. [34] |
Australia | 0.37 (0.18–0.76)b,d | 600–1200 | Intermediate | Intermediate | Strong | Intermediate | Boneh et al. [34] |
Australia | 0.05 (0–0.29)b,d | 2600 | Intermediate | Intermediate | Strong | Weak | Boneh et al. [34] |
China | 0.17 (0.08–0.36) | 242–1211 | Strong | Strong | Strong | Intermediate | Chen et al. [82] |
China | 0.59 (0.38–0.89)c | 1211 | Strong | Strong | Strong | Strong | Chen et al. [82] |
China | 0.38 (0.23–0.64)d | NR | Weak | Strong | Strong | Intermediate | Lin et al. [29] |
China | 0.1 (0.01–0.36)a,d | 363– < 908 | Strong | Strong | Strong | Weak | Liu and Zuo [83] |
China | 0.5 (0.27–0.93)a,d | 908 or 1211 | Strong | Strong | Strong | Intermediate | Liu and Zuo [83] |
China | 0.4 (0.34–0.47) | NR | Intermediate | Strong | Strong | Strong | Maitusong et al. [84] |
China | 0.91 (0.65–1.28) | NR | Weak | Intermediate | Strong | Strong | Shi et al. [85] |
China | 0.65 (0.48–0.9)a,c,d | 1200 | Strong | Strong | Weak | Strong | Su et al. [86] |
China | 0.28 (0.17–0.45)d | 120–360 | Strong | Strong | Weak | Intermediate | Su et al. [86] |
China | 0.98 (0.76–1.27)a,d | 360–1200 | Strong | Strong | Weak | Strong | Su et al. [86] |
China | 0.88 (0.46–1.67) | NR | Weak | Strong | Strong | Intermediate | Tu et al. [87] |
China | 0.07 (0.01–0.21)a–d | 1200 | Strong | Strong | Strong | Weak | Wang et al. [88] |
China | 0.1 (0.03–0.24)d | 120–360 | Strong | Strong | Strong | Weak | Wang et al. [88] |
China | 0.05 (0.01–0.17)a,d | 360–600 | Strong | Strong | Strong | Weak | Wang et al. [88] |
China | 0.14 (0.07–0.31)a,b,d | 600–1200 | Strong | Strong | Strong | Intermediate | Wang et al. [88] |
China | 0.86 (0.82–0.91)d | NR | Intermediate | Strong | Strong | Strong | Zhan et al. [89] |
South Korea | 0.51 (0.14–1.29)c,d | 1200 | Strong | Strong | Strong | Weak | Yoon et al. [90] |
Taiwan | 0.27 (0.2–0.36)d | 120– < 600 | Strong | Strong | Strong | Strong | Niu et al. [91] |
Taiwan | 0.13 (0.09–0.21)b,d | 600– < 1200 | Strong | Strong | Strong | Strong | Niu et al. [91] |
Taiwan | 0.03 (0.01–0.08)b–d | 1200 | Strong | Strong | Strong | Weak | Niu et al. [91] |
Region | Birth prevalence per 10,000 screened (95% CI) | I2 (%) | Number of estimates | Reference(s) | Country |
---|---|---|---|---|---|
Confirmatory test phenylalanine cutoff value of 360 ± 100 µmol/L | |||||
Europe | 0.97 (0.52–1.53) | 93.8 | 4 | Cabalska et al. [58] | Poland |
Mathias and Bickel [51] | Germany | ||||
Vilarinho et al. [59] | Portugal | ||||
Visakorpi et al. [39] | Finland | ||||
Latin America | 1.38 (0.51–3.01) | NA | 1 | Ramalho et al. [67] | Brazil |
Middle East/North Africa | 1.60 (1.06–2.31) | NA | 1 | Habib et al. [44] | Iran |
North America | 0.49 (0.38–0.61) | 0.0 | 2 | Frazier et al. [42] | United States |
Wainer and Sideman[43] | United States | ||||
West Pacific | 0.63 (0.03–1.75) | 96.5 | 3 | Liu and Zuo [83] | China |
Su et al. [86] | China | ||||
Wang et al. [88] | China | ||||
Global (non-regionally weighted) | 0.85 (0.51–1.26) | 95.9 | 11 | – | – |
Global (regionally weighted)b | 0.96 (0.50–1.42) | NA | 11 | – | – |
Confirmatory test phenylalanine cutoff value of 600 ± 100 µmol/L | |||||
Europe | 1.18 (0.75–1.69) | 85.8 | 4 | Lindner et al. [50] | Germany |
Gerasimova et al. [63] | USSR/Russia | ||||
Mardesic et al. [65] | Yugoslavia | ||||
Smon et al. [61] | Slovenia | ||||
Latin America | 0.65 (0.14–1.46) | 64.2 | 2 | Botler et al. [66] | Brazil |
Ramalho et al. [67] | Brazil | ||||
Middle East/North Africa | 0.37 (0.21–0.61) | NA | 1 | Abbaskhanian et al. [69] | Iran |
West Pacific | 0.23 (0.12–0.36) | 55.9 | 3 | Boneh et al. [34] | Australia |
Niu et al. [91] | Taiwan | ||||
Wang et al. [88] | China | ||||
Global (non-regionally weighted) | 0.66 (0.38–1.02) | 94.1 | 10 | – | – |
Global (regionally weighted)b | 0.50 (0.37–0.64) | NA | 10 | – | – |
Confirmatory test phenylalanine cutoff value of 1200 ± 200 µmol/L | |||||
Europe | 0.78 (0.40–1.3) | 96.9 | 7 | Cabalska et al. [58] | Poland |
Gerasimova et al. [63] | USSR/Russia | ||||
Missiou-Tsagaraki et al. [54] | Greece | ||||
Ounap et al. [38] | Estonia | ||||
Smon et al. [61] | Slovenia | ||||
Walker et al. [64] | United Kingdom | ||||
Zaffanello et al. [57] | Italy | ||||
Latin America | 0.58 (0.30–0.94) | 29.2 | 2 | Cornejo et al. [68] | Chile |
Ramalho et al. [67] | Brazil | ||||
Middle East/North Africa | 0.36 (0.04–0.94) | 91.2 | 3 | Abbaskhanian et al. [69] | Iran |
Karamifar et al. [70] | Iran | ||||
Al Hosani et al. [74] | United Arab Emirates | ||||
North America | 0.53 (0.38–0.72) | NA | 1 | Hansen et al. [75] | United States |
Southeast Asia | 0.03 (0.02–0.05) | NA | 1 | Pangkanon et al. [80] | Thailand |
West Pacific | 0.22 (0.03–0.56) | 94.6 | 6 | Boneh et al. [34] | Australia |
Chen et al. [82] | China | ||||
Niu et al.[91] | Taiwan | ||||
Su et al. [86] | China | ||||
Yoon et al. [90] | South Korea | ||||
Wang (2019) [88] | China | ||||
Global (non-regionally weighted) | 0.47 (0.26–0.74) | 98.0 | 20 | – | – |
Global (regionally weighted)b | 0.30 (0.20–0.40) | NA | 20 | – | – |
Overall analysisc | |||||
Europe | 1.14 (0.89–1.41) | 92.2 | 19 | Antonozzi et al. [56] | Italy |
Cabalska et al. [58] | Poland | ||||
Dluholucký and Knapková [60] | Slovakia | ||||
Fernández-Iglesias et al. [62] | Spain | ||||
Gerasimova et al. [63] | USSR/Russia | ||||
Kocova and Anastasovska [41] | Macedonia | ||||
Lindner et al. [50] | Germany | ||||
Loukas et al. [53] | Greece | ||||
Mardesic et al. [65] | Yugoslavia | ||||
Mathias and Bickel [51] | Germany | ||||
Mehes et al. [55] | Hungary | ||||
Missiou-Tsagaraki et al. [54] | Greece | ||||
Ounap et al. [38] | Estonia | ||||
Smon et al. [61] | Slovenia | ||||
Thalhammer [49] | Austria | ||||
Vilarinho et al. [59] | Portugal | ||||
Visakorpi et al. [39] | Finland | ||||
Walker et al. [64] | United Kingdom | ||||
Zaffanello et al. [57] | Italy | ||||
Latin America | 0.98 (0.29–2.03) | 95.8 | 3 | Botler et al. [66] | Brazil |
Cornejo et al. [68] | Chile | ||||
Ramalho et al. [67] | Brazil | ||||
Middle East/North Africa | 1.18 (0.64–1.87) | 96.5 | 7 | Abbaskhanian et al. [69] | Iran |
Alfadhel et al. [72] | Saudi Arabia | ||||
Al Hosani et al. [74] | United Arab Emirates | ||||
Habib et al. [44] | Iran | ||||
Karamifar et al. [70] | Iran | ||||
Motamedi et al. [71] | Iran | ||||
Ozalp et al. [73] | Turkey | ||||
North America | 0.81 (0.58–1.07) | 82.3 | 6 | Frazier et al. [42] | United States |
Hansen et al. [75] | United States | ||||
Kelly and Palombi [76] | United States | ||||
Maccready and Hussey [28] | United States | ||||
Wainer and Sideman [43] | United States | ||||
Zytkovicz et al. [77] | United States | ||||
Southeast Asia | 0.03 (0.02–0.05) | NA | 1 | Pangkanon et al. [80] | Thailand |
West Pacific | 0.68 (0.43–0.98) | 94.2 | 8 | Boneh et al. [34] | Australia |
Lin et al. [29] | China | ||||
Liu and Zuo [83] | China | ||||
Niu et al. [91] | Taiwan | ||||
Su et al. [86] | China | ||||
Wang et al. [88] | China | ||||
Yoon et al. [90] | South Korea | ||||
Zhan et al. [89] | China | ||||
Global (non-regionally weighted) | 0.96 (0.75–1.19) | 98.0 | 44 | – | – |
Global (regionally weighted)b | 0.64 (0.53–0.75) | NA | 44 | – | – |
Discussion
Region | Birth prevalence estimate per 10,000 (95% CI) | ||
---|---|---|---|
Hillert et al. [46] | Shoraka et al. [47] | This studyb | |
Europea | NR | 0.81 (0.65–0.97) | 1.14 (0.89–1.41) |
Middle East/North Africa | NR | NR | 1.18 (0.64–1.87) |
Eastern Mediterranean | NR | 0.98 (0.62–1.35) | NR |
Pan America | NR | 0.53 (0.46–0.61) | NR |
Latin America | NR | NR | 0.98 (0.29–2.03) |
North America | NR | NR | 0.81 (0.58–1.07) |
Southeast Asia | NR | 0.03 (0.02–0.05) | 0.03 (0.02–0.05) |
West Pacific | NR | 0.29 (0.09–0.50) | 0.68 (0.43–0.98) |
Global (non-regionally weighted) | NR | 0.60 (0.51–0.69) | 0.96 (0.75–1.19) |
Global (regionally weighted) | 0.42 (NR) | NR | 0.64 (0.53–0.75)c |