Table 1.
Summary of the data from newborn screening surveys in Africa and the Middle East.
ID | Reference | Study period | Country | N | Observed | Expected (HWE) | Exact test (LLR) | F IS (%) | ||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
%AA | %AS | %SS | %AC | %SC | %CC | %AA | %AS | %SS | %AC | %SC | %CC | |||||||
Sub-Saharan Africa
| ||||||||||||||||||
1 | McGann et al, 2013 | Jul 2011 - Jun 2013 | Angola | 36,453 | 77.4 | 21.0 | 1.5 | 0.1 | 0.0 | 0.0 | 77.3 | 21.2 | 1.4 | 0.1 | 0.0 | 0.0 | 0.103 | 0.6 |
2 | Kafando et al, 2005 | 2000, 2003, 2004 | Burkina Faso | 2,341 | 72.7 | 7.3 | 0.6 | 16.9 | 1.2 | 1.4 | 71.9 | 8.2 | 0.2 | 17.6 | 1.0 | 1.1 | 0.002 * | 5.5 |
3 | Moreno et al, 1994 | Not specified | Burundi | 260 | 91.5 | 8.5 | 0.0 | / | / | / | 91.7 | 8.1 | 0.2 | / | / | / | 0.696 | −4.4 |
4 | Moreno et al, 1994 | Not specified | Burundi | 82 | 73.2 | 24.4 | 2.4 | / | / | / | 72.9 | 25.0 | 2.1 | / | / | / | 1.000 | 2.4 |
5 | Moreno et al, 1994 | Not specified | Burundi | 40 | 80.0 | 20.0 | 0.0 | / | / | / | 81.0 | 18.0 | 1.0 | / | / | / | 0.712 | −11.1 |
Moreno et al, 1994 | Not specified | Burundi | 382 | 86.4 | 13.1 | 0.5 | / | / | / | 86.4 | 13.1 | 0.5 | / | / | / | 1.000 | 0.4 | |
6 | Mutesa et al, 2007 | Jul 2004 - Jul 2006 | Burundi | 637 | 95.4 | 4.4 | 0.2 | 0.0 | 0.0 | 0.0 | 95.3 | 4.6 | 0.1 | 0.0 | 0.0 | 0.0 | 0.295 | 4.4 |
7 | Le Hesran et al, 1999 | Jan 1993 - Dec 1995 | Cameroon | 156 | 75.6 | 21.8 | 2.6 | / | / | / | 74.9 | 23.3 | 1.8 | / | / | / | 0.484 | 6.5 |
8 | Diallo et al, 2008 | Feb 2005 - Mar 2005 | Congo | 1,186 | 80.3 | 19.0 | 0.8 | / | / | / | 80.6 | 18.4 | 1.0 | / | / | / | 0.281 | −3.2 |
9 | Van Baelen et al, 1969 | Nov 1964 - Apr 1965 | DRC | 500 | 73.4 | 24.0 | 2.6 | / | / | / | 72.9 | 24.9 | 2.1 | / | / | / | 0.471 | 3.8 |
10 | Lallemant et al, 1986 | Not specified | DRC | 146 | 80.8 | 18.5 | 0.7 | / | / | / | 81.1 | 17.9 | 1.0 | / | / | / | 1.000 | −3.4 |
11 | Mutesa et al, 2007 | Jul 2004 - Jul 2006 | DRC | 84 | 92.9 | 4.8 | 0.0 | 2.4 | 0.0 | 0.0 | 93.0 | 4.6 | 0.1 | 2.3 | 0.1 | 0.0 | 1.000 | −2.9 |
12 | Tshilolo et al, 2008 | Oct 2000 - Aug 2002 | DRC | 4,116 | 77.3 | 20.4 | 2.3 | 0.0 | 0.0 | 0.0 | 76.5 | 21.9 | 1.6 | 0.0 | 0.0 | 0.0 | 0.000 * | 7.0 |
13 | Tshilolo et al, 2009 | Jan 2006 - ? | DRC | 70 | 91.4 | 8.6 | 0.0 | 0.0 | 0.0 | 0.0 | 91.6 | 8.2 | 0.2 | 0.0 | 0.0 | 0.0 | 1.000 | −4.5 |
14 | Tshilolo et al, 2009 | Jan 2006 - ? | DRC | 579 | 81.7 | 17.3 | 1.0 | 0.0 | 0.0 | 0.0 | 81.6 | 17.5 | 0.9 | 0.0 | 0.0 | 0.0 | 0.810 | 1.2 |
15 | Tshilolo et al, 2009 | Jan 2006 - ? | DRC | 717 | 84.9 | 14.2 | 0.8 | 0.0 | 0.0 | 0.0 | 84.7 | 14.6 | 0.6 | 0.0 | 0.0 | 0.0 | 0.610 | 2.8 |
16 | Tshilolo et al, 2009 | Jan 2006 - ? | DRC | 559 | 78.7 | 19.7 | 1.6 | 0.0 | 0.0 | 0.0 | 78.4 | 20.3 | 1.3 | 0.0 | 0.0 | 0.0 | 0.528 | 3.0 |
17 | Tshilolo et al, 2009 | Jan 2006 - ? | DRC | 932 | 84.9 | 13.9 | 1.2 | 0.0 | 0.0 | 0.0 | 84.4 | 15.0 | 0.7 | 0.0 | 0.0 | 0.0 | 0.078 | 6.9 |
18 | Tshilolo et al, 2009 | Jan 2006 - ? | DRC | 1,016 | 80.6 | 17.6 | 1.8 | 0.0 | 0.0 | 0.0 | 80.0 | 18.9 | 1.1 | 0.0 | 0.0 | 0.0 | 0.044 * | 6.9 |
19 | Tshilolo et al, 2009 | Jan 2006 - ? | DRC | 3,069 | 81.2 | 17.2 | 1.7 | 0.0 | 0.0 | 0.0 | 80.6 | 18.4 | 1.1 | 0.0 | 0.0 | 0.0 | 0.001 * | 6.7 |
20 | Tshilolo et al, 2009 | Jan 2006 - ? | DRC | 4,217 | 81.8 | 16.9 | 1.3 | 0.0 | 0.0 | 0.0 | 81.4 | 17.6 | 1.0 | 0.0 | 0.0 | 0.0 | 0.014 * | 4.0 |
21 | Tshilolo et al, 2009 | Jan 2006 - ? | DRC | 10,445 | 81.9 | 16.8 | 1.3 | 0.0 | 0.0 | 0.0 | 81.5 | 17.5 | 0.9 | 0.0 | 0.0 | 0.0 | 0.000 * | 4.0 |
22 | Tshilolo et al, 2009 | Jan 2006 - ? | DRC | 9,600 | 81.4 | 17.2 | 1.4 | 0.0 | 0.0 | 0.0 | 80.9 | 18.1 | 1.0 | 0.0 | 0.0 | 0.0 | 0.000 * | 4.6 |
Tshilolo et al, 2009 | Jan 2006 - ? | DRC | 31,204 | 81.7 | 16.9 | 1.4 | 0.0 | 0.0 | 0.0 | 81.3 | 17.7 | 1.0 | 0.0 | 0.0 | 0.0 | 0.000 * | 4.6 | |
23 | Agasa et al, 2010 | Dec 2006 - Feb 2007 | DRC | 520 | 75.8 | 23.3 | 1.0 | / | / | / | 76.4 | 22.0 | 1.6 | / | / | / | 0.236 | −5.7 |
24 | Richard-Lenoble et al, 1980 | Not specified | Gabon | 96 | 89.6 | 8.3 | 2.1 | / | / | / | 87.9 | 11.7 | 0.4 | / | / | / | 0.037 * | 28.9 |
25 | Ohene-Frempong (Pers.com.) | Feb 1995 - Dec 2011 | Ghana | 343,355 | 75.6 | 13.3 | 0.9 | 8.8 | 0.8 | 0.5 | 75.2 | 13.9 | 0.6 | 9.2 | 0.8 | 0.3 | 0.000 * | 3.9 |
26 | Williams (Pers. Com.) | May 2006 - May 2014 | Kenya | 15,444 | 84.1 | 15.1 | 0.8 | / | / | / | 84.0 | 15.3 | 0.7 | / | / | / | 0.093 | 1.4 |
27 | Diallo et al, 2008 | Feb 2005 - Apr 2005 | Mali | 1,029 | 78.4 | 9.9 | 0.4 | 10.2 | 0.4 | 0.7 | 78.3 | 9.8 | 0.3 | 10.6 | 0.7 | 0.4 | 0.263 | 2.5 |
28 | Kulkarni et al, 1986 | Not specified | Nigeria | 700 | 75.1 | 19.7 | 2.9 | 2.1 | 0.0 | 0.1 | 74.1 | 21.9 | 1.6 | 2.1 | 0.3 | 0.0 | 0.001 * | 10.0 |
29 | Odunvbun et al, 2008 | Jun 2000 - Sep 2000 | Nigeria | 644 | 75.3 | 20.7 | 2.8 | 1.1 | 0.2 | 0.0 | 74.3 | 22.7 | 1.7 | 1.1 | 0.2 | 0.0 | 0.094 | 8.7 |
30 | Munyanganizi et al, 2006 | 2005 | Rwanda | 987 | 97.3 | 2.7 | 0.0 | 0.0 | 0.0 | 0.0 | 97.3 | 2.7 | 0.0 | 0.0 | 0.0 | 0.0 | 1.000 | −1.4 |
31 | Mutesa et al, 2007 | July 2004 - July 2006 | Rwanda | 1,104 | 97.2 | 2.5 | 0.1 | 0.2 | 0.0 | 0.0 | 97.1 | 2.7 | 0.0 | 0.2 | 0.0 | 0.0 | 0.204 | 5.0 |
32 | Oudart et al, 1968 | Not specified | Senegal | 345 | 90.7 | 7.2 | 0.3 | 1.2 | 0.6 | 0.0 | 90.1 | 8.0 | 0.2 | 1.7 | 0.1 | 0.0 | 0.061 | 7.4 |
33 | Mbodji et al, 2003 | Oct 1996 - Jun 1997 | Senegal | 479 | 88.9 | 7.9 | 1.9 | 1.0 | 0.2 | 0.0 | 87.3 | 11.1 | 0.4 | 1.2 | 0.1 | 0.0 | 0.000 * | 25.7 |
34 | Tchamago et al, 2006 | Mar 2005 - Aug 2005 | Senegal | 944 | 88.9 | 9.5 | 0.4 | 1.0 | 0.0 | 0.0 | 88.6 | 9.8 | 0.3 | 0.9 | 0.0 | 0.0 | 0.513 | 5.9 |
35 | Diallo et al, 2008 | Not specified | Senegal | 1,000 | 91.0 | 8.5 | 0.5 | 0.0 | 0.0 | 0.0 | 90.7 | 9.0 | 0.2 | 0.0 | 0.0 | 0.0 | 0.163 | 6.1 |
36 | Aebischer et al, 1990 | Not specified | Tanzania | 92 | 89.1 | 10.9 | 0.0 | / | / | / | 89.4 | 10.3 | 0.3 | / | / | / | 1.000 | −5.7 |
37 | Aebischer et al, 1990 | Not specified | Tanzania | 101 | 86.1 | 12.9 | 1.0 | / | / | / | 85.7 | 13.7 | 0.6 | / | / | / | 1.000 | 6.4 |
38 | Aebischer et al, 1990 | Not specified | Tanzania | 30 | 96.7 | 3.3 | 0.0 | / | / | / | 96.7 | 3.3 | 0.0 | / | / | / | 1.000 | −1.7 |
Aebischer et al, 1990 | Not specified | Tanzania | 223 | 88.8 | 10.8 | 0.4 | / | / | / | 88.7 | 11.0 | 0.3 | / | / | / | 1.000 | 2.0 | |
39 | North et al, 1988 | Not specified | Togo | 385 | 69.9 | 18.7 | 1.0 | 8.8 | 1.3 | 0.3 | 70.0 | 18.5 | 1.2 | 8.9 | 1.2 | 0.3 | 0.970 | −1.0 |
40 | Segbena et al, 2002 | Not specified | Togo | 171 | 64.3 | 16.4 | 1.2 | 15.8 | 2.3 | 0.0 | 64.7 | 16.9 | 1.1 | 14.6 | 1.9 | 0.8 | 0.485 | −3.3 |
North Africa
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41 | Diallo et al, 2005 | Not specified | Tunisia | 1,329 | 98.5 | 1.5 | 0.0 | 0.0 | 0.0 | 0.0 | 98.5 | 1.5 | 0.0 | 0.0 | 0.0 | 0.0 | 1.000 | −0.8 |
42 | Fattoum et al, 2006 | Not specified | Tunisia | 335 | 97.0 | 2.2 | 0.0 | 0.8 | 0.0 | 0.0 | 97.0 | 2.1 | 0.0 | 0.8 | 0.0 | 0.0 | 1.000 | −1.2 |
43 | Fattoum et al, 2006 | Not specified | Tunisia | 335 | 99.1 | 0.6 | 0.0 | 0.3 | 0.0 | 0.0 | 99.1 | 0.6 | 0.0 | 0.3 | 0.0 | 0.0 | 1.000 | −0.3 |
44 | Fattoum et al, 2006 | Not specified | Tunisia | 264 | 94.3 | 5.7 | 0.0 | 0.0 | 0.0 | 0.0 | 94.4 | 5.5 | 0.1 | 0.0 | 0.0 | 0.0 | 1.000 | −2.9 |
Fattoum et al, 2006 | Not specified | Tunisia | 2,628 | 97.0 | 2.3 | 0.0 | 0.7 | 0.0 | 0.0 | 97.0 | 2.3 | 0.0 | 0.7 | 0.0 | 0.0 | 1.000 | −1.3 | |
45 | Hajer et al, 2012 | Not specified | Tunisia | 9,148 | 97.7 | 1.9 | 0.0 | 0.4 | 0.0 | 0.0 | 97.7 | 1.9 | 0.0 | 0.4 | 0.0 | 0.0 | 0.000 * | 1.3 |
Middle East
| ||||||||||||||||||
46 | Mohammed et al, 1992 | Oct 1984 - Dec 1985 | Bahrain | 10,327 | 86.7 | 11.2 | 2.1 | 0.0 | 0.0 | 0.0 | 85.2 | 14.2 | 0.6 | 0.0 | 0.0 | 0.0 | 0.000 * | 21.2 |
47 | Al Arrayed et al, 2005 | Feb 2002 - April 2002 | Bahrain | 1,995 | 82.8 | 16.3 | 0.9 | / | / | / | 82.7 | 16.5 | 0.8 | / | / | / | 0.682 | 1.0 |
48 | Al Arrayed et al, 2012 | Jan 2008 - Dec 2010 | Bahrain | 38,940 | 86.1 | 13.5 | 0.6 | / | / | / | 86.1 | 13.6 | 0.5 | / | / | / | 0.156 | −0.9 |
49 | Talafih et al, 1996 | Not specified | Jordan | 181 | 95.0 | 5.0 | 0.0 | / | / | / | 95.1 | 4.8 | 0.1 | / | / | / | 1.000 | −2.5 |
50 | Khoriaty et al, 2014 | Dec 2010 - Mar 2013 | Lebanon | 10,095 | 98.2 | 1.7 | 0.1 | 0.0 | 0.0 | 0.0 | 98.1 | 1.9 | 0.0 | 0.1 | 0.0 | 0.0 | 0.000 * | 9.9 |
51 | Alkindi et al, 2010 | Apr 2005 - Mar 2007 | Oman | 7,837 | 94.5 | 5.2 | 0.3 | / | / | / | 94.3 | 5.6 | 0.1 | / | / | / | 0.000 * | 7.0 |
52 | Al-Awamy et al, 1984 | Dec 1981 - Apr 1983 | Saudi Arabia | 1,697 | 95.0 | 4.4 | 0.6 | / | / | / | 94.5 | 5.4 | 0.1 | / | / | / | 0.000 * | 18.8 |
53 | Al-Awamy et al, 1984 | Dec 1981 - Apr 1983 | Saudi Arabia | 2,800 | 92.8 | 6.7 | 0.5 | / | / | / | 92.4 | 7.5 | 0.2 | / | / | / | 0.000 * | 10.3 |
54 | Al-Awamy et al, 1984 | Mar 1982 - Apr | Saudi Arabia | 1,124 | 80.1 | 18.0 | 2.0 | / | / | / | 79.3 | 19.5 | 1.2 | / | / | / | 0.021 * | 7.8 |
Al-Awamy et al, 1984 | Dec 1981 - Apr 1983 | Saudi Arabia | 5,621 | 90.9 | 8.3 | 0.8 | / | / | / | 90.3 | 9.5 | 0.2 | / | / | / | 0.000 * | 12.5 | |
55 | Nasserullah et al, 2003 | Dec 1992 - Dec 2001 | Saudi Arabia | 2,149 | 97.5 | 2.4 | 0.1 | / | / | / | 97.5 | 2.5 | 0.0 | / | / | / | 0.046 * | 6.1 |
56 | Nasserullah et al, 2003 | Dec 1992 - Dec 2001 | Saudi Arabia | 21,829 | 76.3 | 21.2 | 2.6 | / | / | / | 75.4 | 22.9 | 1.7 | / | / | / | 0.000 * | 7.4 |
Nasserullah et al, 2003 | Dec 1992 - Dec 2001 | Saudi Arabia | 23,978 | 78.2 | 19.5 | 2.4 | / | / | / | 77.3 | 21.3 | 1.5 | / | / | / | 0.000 * | 8.4 | |
57 | Al Hosani et al, 2005 | Jan 2002 - Dec 2002 | UAE | 9,165 | 98.4 | 1.5 | 0.1 | 0.0 | 0.0 | 0.0 | 98.3 | 1.6 | 0.0 | 0.0 | 0.0 | 0.0 | 0.000 * | 7.1 |
58 | Al Hosani et al, 2005 | Jan 2002 - Dec 2002 | UAE | 13,035 | 99.1 | 0.8 | 0.0 | 0.1 | 0.0 | 0.0 | 99.1 | 0.8 | 0.0 | 0.1 | 0.0 | 0.0 | 0.000 * | 6.3 |
Al Hosani et al, 2005 | Jan 2002 - Dec 2002 | UAE | 22,200 | 98.8 | 1.1 | 0.0 | 0.0 | 0.0 | 0.0 | 98.8 | 1.2 | 0.0 | 0.1 | 0.0 | 0.0 | 0.000 * | 6.8 | |
59 | Al Hosani et al, 2014 | Jan 2002 - Dec 2011 | UAE | 542,286 | 99.1 | 0.8 | 0.0 | 0.0 | 0.0 | 0.0 | 99.1 | 0.9 | 0.0 | 0.0 | 0.0 | 0.0 | 0.000 * | 8.1 |
60 | Al-Nood et al, 2004 | July 2001 - Dec 2001 | Yemen | 1,500 | 97.8 | 2.2 | 0.0 | 0.0 | 0.0 | 0.0 | 97.8 | 2.2 | 0.0 | 0.0 | 0.0 | 0.0 | 1.000 | −1.1 |
N = sample size. HWE = Hardy-Weinberg equilibrium. LLR = likelihood ratio. F = fixation index.
indicate surveys for which observed and expected counts differ significantly (p<0.05).