Birgitta Versluijs

169 Discussion TABLE 2. Overview of publications on treatment outcome for IPS and BOS in children after HCT — continued Versluys (37) 2018w N=53. Median age 6.9 (0.3-18.8) IPS (30) BOS (23) MP pulse 10 mg/kg/day, 3 days, every 4 wks, plus Imatinib and azithromycin in BOS Initial response: 55% (IPS 53%, BOS 57%) Death from Allo-LS: 38% (IPS 43%, BOS 30%), NRM: 9% Overall survival: 47% (IPS 40%, BOS 57%  Chronic Allo-LS: 6% (IPS 3%, BOS 9%  Complete response: 42%, normalization of PFT Uniform treatment Ratjen (38) 2005 N=9 Mean age 8.8 (±5.6) BOS MP pulse 10 mg/kg/ day, 3 days, every 4 wks, plus Inhaled steroids (budesonide) Death from BOS: 22%, NRM 0% Overall survival: 78%  Stable lung disease: 78%  NO normalization of PFT Uniform treatment Duncan (13) 2008 N=18 Median age 10.7 BOS Corticosteroids (dose?) , plus CsA, MMF, rapamycin, azithromycin, pravastatin Death from BOS: 28%, NRM: 11% Overall survival: 61%  Progressive lung disease: 17  Stable lung disease: 11% / Partial response: 33%  Resolved BOS: 0% Treatment unclear No uniform treatment Duncan (39) 2010 N=5 (+5 off study) Median age 13 (8-15) BOS Pravastatin (statin), plus Corticosteroids (dose?) Death from BOS: 20%, NRM: 10% Overall survival: 70%  Partial response/Stable disease: 70% Unclear, uniform treat- ment. Study closure: slow accrual. No follow up on PFT Uhlving (40) 2012 N=9 Median age 10.5 (4-13) BOS MP pulse 15 mg/kg/day, 2-3 days, every 4-6 wks Death from BOS: 11%, NRM:11% Overall survival: 66%  Partial/complete response: 66%  almost normalization of PFT Uniform treatment Nagasawa (11) 2017 N=9. Median age 11.6(0.6-18.6) BOS (Inhaled) steroids (dose?), plus leukotriene receptor antagonist and MMF Death from BOS: 44%, NRM 11% Overall survival: 66% Treatment unclear No follow up on PFT 9

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