Andersson, Nadine GAuerswald, GünterBarnes, ChrisCarcao, ManuelDunn, Amy LFijnvandraat, KarinHoffmann, MarianneKavakli, KaanKenet, GiliKobelt, RainerKurnik, KarinLiesner, RiMäkipernaa, AnneManco-Johnson, Marilyn JMancuso, Maria EMolinari, Angelo CNolan, BeatricePerez Garrido, RosarioPetrini, PiaPlatokouki, Helen EShapiro, Amy DWu, RunhuiLjung, Rolf2023-01-252023-01-252017-07-12http://hdl.handle.net/10668/11396The discussion of prophylactic therapy in haemophilia is largely focused on joint outcomes. The impact of prophylactic therapy on intracranial haemorrhage (ICH) is less known. This study aimed to analyse ICH in children with haemophilia, with a focus on different prophylaxis regimens and sequelae of ICH. We conducted a multicentre retrospective and prospective study that included 33 haemophilia centres from 20 countries. Inclusion criteria were children and adolescents born between 1993 and 2014, with severe haemophilia A or B without inhibitors. Participants were categorized by prophylaxis regimen: full, partial or none, based on dose and dose frequency of regular infusions. The cohort study included 1515 children: 29 cases of ICH over 8038 patient years were reported. The incidence of ICH in the prophylaxis group, 0·00033 cases of ICH/patient year, was significantly lower compared to the no prophylaxis group, 0·017 cases of ICH/patient year (RR 50·06; P enchildrenfactor IXfactor VIIIhaemophilia Ahaemophilia BAdolescentAdultChildChild, PreschoolFemaleHemophilia AHemophilia BHumansInfantIntracranial HemorrhagesMaleProspective StudiesRetrospective StudiesSeverity of Illness Indexresearch article2869967510.1111/bjh.148441365-2141