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A multi-centre study in Singapore and Canada found variations in perioperative practices around CVAD placement in children with haemophilia (CwH) with and without inhibitors. Further studies are recommended to support the development of guidelines for optimal CVAD placement in CwH.© Shutterstock/NINUN
A multi-centre study in Singapore and Canada found variations in perioperative practices around CVAD placement in children with haemophilia (CwH) with and without inhibitors. Further studies are recommended to support the development of guidelines for optimal CVAD placement in CwH.© Shutterstock/NINUN

Comparison of perioperative practices between CwH with and without inhibitors

CWH WITHOUT INHIBITORS CWH WITH INHIBITORS P-VALUE

Number of CwH 14 7

Preoperative investigations performed
• Full blood count (%) 7 (50%) 7 (100%) 0.047
• APTT (%) 5 (36%) 2 (29%) 1.000
• Factor assay (%) 9 (64%) 1 (14%) 0.063
• Recovery study (%) 0 (0%) 2 (29%) 0.100
• Inhibitor screen (%) 9 (64%) 7 (100%) 0.123

Antifibrinolytic use (%) 6 (43%) 4 (57%) 0.659

Mean length of stay in days (range) 4.9 (3–7) 8.6 (3–25) 0.096

Postoperative complications (%) 0 (0%) 4 (57%) 0.006

Need for CVAD revision (%) 0 (0%) 1 (14%) 0.333

Characteristics and operative details of CwH included in the study, from all centres

Number of CwH 21

Median age at insertion, months (range)* 20.5 (12–110)

Haemophilia A (%) 20 (95%)

Haemophilia B (%) 1 (5%)

Deficient factor <1% (%) 21 (100%)

Number of patients with inhibitors (%) 7 (33%)
Median preoperative inhibitor titres (range) 5 BU (1.5–210 BU)

Preoperative treatment**
• Prophylaxis (%) 9 (64%)
• On-demand (%) 5 (36%)

Indication for CVAD insertion
• Prophylaxis (%) 13 (62%)
• ITI (%) 4 (19%)
• Revision (%) 2 (9.5%)
• Difficult venous access (on-demand treatment) (%) 2 (9.5%)

Type of CVAD
• Portacath (%) 21 (100%)

Vessel used for CVAD
• Internal jugular vein (%) 9 (43%)
• Subclavian vein (%) 9 (43%)
• External jugular vein (%) 3 (14%)

Mean number of CVAD insertion attempts, including successful attempt (range) 1.4 (1–4)

Preoperative investigations performed prior to CVAD surgery and details of perioperative factor VIII cover for CVAD surgery amongst CwH without inhibitors

SINGAPORE (% PERFORMED) HAMILTON (% PERFORMED) P-VALUE
Investigations
Full blood count 100% 0% 0.000
APTT 83% 0% 0.001
Factor assay 25% 100% 0.003
Inhibitor screen 25% 100% 0.003
Factor VIII cover
Mean preoperative factor VIII dose (±SD) 50.0 IU/kg (±7.6) 72.4 IU/kg (±12.5) 0.002
Mean total dose used (±SD) 8642.9 IU (±6011.9)425.0 IU/kg (±114.9) 8924.1 IU (±2488.6)646.8 IU/kg (±118.1) 0.9110.004
Mean duration of factor VIII cover (±SD) 5.3 days (±0.9) 6.9 days (±0.7) 0.004

Details of perioperative rFVIIa cover for CVAD surgery in CwH with inhibitors

RFVIIA COVER SINGAPORE HAMILTON P-VALUE
Mean preoperative rFVIIa dose (±SD) 160.5 mcg/kg (±99.9) 88.2 mcg/kg (±3.8) 0.244
Mean total dose used from preoperative dose to discharge (±SD) 3008.0 mcg/kg (±2305.9) 2640.2 mcg/kg (±134.1) 0.842
Mean duration of rFVIIa cover (±SD) 5.3 days (±1.7) 9.5 days (±2.1) 0.054
eISSN:
2055-3390
Język:
Angielski
Częstotliwość wydawania:
Volume Open
Dziedziny czasopisma:
Medicine, Basic Medical Science, other, Clinical Medicine, Pharmacy, Pharmacology