4S: Crises in relation to spleen status (excluding patients who underwent partial followed by full splenectomy) Spleen Crises in long bones (mean events per Crises in weight bearing bones (mean Crises in other bones (mean Crises in small bones of hands and status patient) events per patient) events per patient) feet (mean events per patient) N Events Events Events after N Events Events Events after N Events N Events Events Events after before same yr >1 yr before same >1 yr after >1 before same >1 yr yr same yr yr yr Intact 10 21 (2.1) NA NA 1 2 (2.0) NA NA 1 1 NA NA 4 9 (2.3) NA NA Partial 6 4 (0.6) 1 (0.2) 20 (3.3) 2 0 1 (0.5) 2 (1) 0 0 0 0 6 0 0 16 (2.7) splenectomy Mean 5.1 Mean 4 yrs Mean 13.9 yrs; median later yrs later; 2.5 yrs; (untreated) median 14 range 1-27 yrs; range yrs after yrs later. surgery. 6 14 events events occurred occurred while treated while treated (mean 9.4 (mean 2.7 yrs after ERT yrs after initiation; surgery median 7 (median 2 yrs; range yrs, range yrs) Full 4 1 (0.25) 3 (0.75) 15 (3.75) 1 0 1 1 0 0 3 3 0 0 12 (4.0) splenectomy Mean 6 yrs 13 yrs after Mean 17 Mean 20.4 after surgery in yrs after yrs; median surgery; same year surgery; 15 yrs; range median 5 as starting median 12-46 yrs yrs; range treatment 15 yrs; later. 11 yrs) range events 1 in 1 14-22 yrs occurred patient All after ERT occurred treated : initiation while on + 1, 2 (mean 6.7 ERT (1 yr and 9 yrs yrs; median after ERT after ERT 3 yrs; range initiation) start 2-21yrs). Abbreviation: N: number of patients with date-documented crises
4S: Crises in relation to spleen status (excluding patients who underwent partial followed by full splenectomy) Spleen status Crises in long bones (mean events per patient) Crises in weight bearing bones (mean events per patient) Crises in other bones (mean events per patient) Crises in small bones of hands and feet (mean events per patient) N Events before Events same yr Events after >1 yr N Events before Events same yr Events after >1 yr N same yr Events after >1 yr N Events before Events same yr Events after >1 yr Intact 10 21 (2.1) NA NA 1 2 (2.0) NA NA 1 1 NA NA 4 9 (2.3) NA NA Partial splenectomy 6 4 (0.6) 1 (0.2) 20 (3.3) Mean 5.1 yrs; median 2.5 yrs; range 1-27 yrs after surgery. 6 events occurred while treated (mean 2.7 yrs after surgery (median 2 yrs, range yrs) 2 0 1 (0.5) 2 (1) Mean 4 yrs later (untreated) 0 0 0 0 6 0 0 16 (2.7) Mean 13.9 yrs later; median 14 yrs; range yrs later. 14 events occurred while treated (mean 9.4 yrs after ERT initiation; median 7 yrs; range Full splenectomy 4 1 (0.25) 3 (0.75) 15 (3.75) Mean 6 yrs after surgery; median 5 yrs; range yrs) 1 in 1 patient occurred while on ERT (1 yr after ERT initiation) 1 0 1 13 yrs after surgery in same year as starting treatment 1 0 0 3 Mean 17 yrs after surgery; median 15 yrs; range 14-22 yrs All treated : + 1, 2 and 9 yrs after ERT start 3 0 0 12 (4.0) Mean 20.4 yrs; median 15 yrs; range 12-46 yrs later. 11 events occurred after ERT initiation (mean 6.7 yrs; median 3 yrs; range 2-21yrs). Abbreviation: N: number of patients with date-documented crises
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