Table 6 Population-based epidemiological investigations of C1-INH-HAE
Reference | Roche et al. | Stray-Pedersen et al. | Bygum A | Nordenfelt et al. | Zanicchelli et al. | Psarros et al. |
|---|---|---|---|---|---|---|
Region | Spain | Norway | Denmark | Sweden | Italy | Greece |
Time period | 1999–2004 | 1998/1999 | 1999, 2003/4 and 2009 | 2011 | 1973–2013 | 2010–2013 |
Design | Doctor, hospital and patient survey | Hospital & patient survey, telephone follow-up | Comprehensive national survey (hospitals, doctors, laboratories, media and patient incl. families) | Sweha-Reg, a population-based census of HAE in Sweden | Survey of 17 dedicated tertiary referral centers | Prospective HAE registry |
Reference population | Spain 2001, 40.476 Millions | Norway 1999, 4.462 Millions | Denmark 2009, 5.523 Millions | Sweden 2010, 9.348 Millions | Italy 2013, 60.783 Millions | Greece, 2012 10.815 Millions |
Cases | 444 | 67 | 76 | 146 | 983 | 116 |
Diagn. criteria | Criteria & C1-INH | Diagnosis as per record | Criteria & C1-INH | Criteria & C1-INH/C4 | Criteria & C1-INH | Criteria & C1-INH |
Calculated prevalence | 1.10 per 100,000 / 1 in 91,162 | 1.50 per 100,000 / 1 in 66,597 | 1.38 per 100,000 / 1 in 72,671 | 1.56 per 100,000 / 1 in 64,028 | 1.51 per 100,000 / 1 in 66,284 | 1.07 per 100,000 / 1 in 93,235 |