Table 3 Reported lifetime risk at birth estimates in studies included > 1 subtype of Sanfilippo syndrome
From: Epidemiology of Sanfilippo syndrome: results of a systematic literature review
First author, year | Country | Study period | Reference population | Size of the reference population | Patients per 100,000 live births | |||||
|---|---|---|---|---|---|---|---|---|---|---|
Sum of all MPS III | Type A | Type B | Type C | Type D | Estimation method | |||||
Baehner, 2005 [17] | Germany | 1980–1995 | General population (live births in the study period) | 13,410,924 | 1.57 | 1.11 | 0.37 | 0.10 | 0 | Dx |
Héron, 2011 [19] | France | 1990–2006 | General population (live births per year) | No data | 0.82a | 0.48 | 0.15 | 0.15 | 0.04 | Dx |
Hult, 2014 [20] | Sweden | 1990–2009 | General population (live births in the study period) | 2,080,791 | 1.01 | 0.62 | 0.05 | 0.34 | 0 | Dx |
Lin, 2009 [23] | Taiwan | 1984–2004 | General population (live births in the study period) | 6,377,299 | 0.39 | 0.08 | 0.28 | 0.03 | 0 | Dx |
Meikle, 1999 [29] | Australia | 1980–1996 | General population (live births in the study period) | No data | 1.37b | 0.78 | 0.43 | 0.07 | 0.09 | Dx |
Nelson, 1997 [25] | Great Britain | 1958–1985 | General population (live births in the study period in Northern Ireland) | 839,517 | 0.36 | 0.24 | 0.12 | 0 | 0 | Dx |
Nelson, 2003 [26] | Australia | 1969–1996 | General population (live births in the study period in Western Australia) | 641,179 | 1.71 | 0.62 | 0.78 | 0.16 | 0 | Dx |
Al-Jasmi, 2013 [30] | United Arab Emirates | 1995–2010 | General population | No data | – | 0 | 1.05 | 0.25 | – | DoB |
Krabbi, 2012 [31] | Estonia | 1985–2006 | General population (live births in the study period) | 370,298 | 1.62b | 1.62 | 0 | 0 | 0 | DoB |
Pinto, 2004 [27] | Portugal | 1982–2001 | General population | No data | 0.84 | 0 | 0.72 | 0.12 | – | DoB |
Poorthuis, 1999 [28] | The Netherlands | 1970–1996 | General population (live births in the study period) | Depends on the subtype of Sanfilippo: A: 6,972,344 (1960–1993); B: 11,131,609 (1940–1991); C: 7,119,276 (1949–1980); D: 2,994,743 (1970–1985) | 1.89 | 1.16 | 0.42 | 0.21 | 0.10 | DoB |