Table 3.

Reported lifetime risk at birth estimates in studies included > 1 subtype of Sanfilippo syndrome

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

^aOriginal paper reported 0.73, which is possibly a misprint, as sum of subtypes is 0.82

^bCalculated from the reported numbers of all subtypes

DoB date-of-birth method, Dx diagnosis period method, MPS mucopolysaccharidosis