TY - JOUR
T1 - Vascular access for lipid apheresis
T2 - a challenge in young children with homozygous familial hypercholesterolemia
AU - Lischka, Julia
AU - Arbeiter, Klaus
AU - de Gier, Charlotte
AU - Willfort-Ehringer, Andrea
AU - Walleczek, Nina-Katharina
AU - Gellai, Renata
AU - Boehm, Michael
AU - Wiegman, Albert
AU - Greber-Platzer, Susanne
N1 - Lischka: externe Aff.
PY - 2022/3/12
Y1 - 2022/3/12
N2 - BACKGROUND: Homozygous familial hypercholesterolemia (hoFH) is a rare genetic disorder leading to extremely increased LDL-cholesterol (LDL-C), resulting in high cardiovascular risk in early childhood. Lipid apheresis (LA) is an effective treatment and should be started as early as possible to prevent premature cardiovascular events. As peripheral punctures in children can be challenging due to small vessels and anxiety, this study aimed to evaluate feasibility and safety of central venous catheters (CVCs) as vascular access for LA in young children with hoFH.METHODS: Retrospective analysis (2016-2019) on four children with hoFH aged 3-5 years, performing weekly or biweekly LA with a CVC.RESULTS: LDL-C decreased by> 60%. In three children, the use of a permanent CVC for 698, 595, and 411 days, respectively, avoided difficult peripheral access, without the occurrence of occlusion or thrombosis. Unfortunately, one child had recurrent CVC-related infections and needed an arteriovenous fistula from the age of 5. Although the mean dwell time per catheter was 212 days, there were, as expected, severe side effects of early catheter infections with sepsis and accidental self-removal. Starting LA at an early age improved or stabilized carotid intima-media thickness (IMT) in three children. However, IMT did increase in one child caused by intolerance to peripheral punctures and LA interruption.CONCLUSIONS: Permanent CVCs are a viable temporary access choice for LA in young children with hoFH until peripheral venipuncture is practicable. The risk of CVC-related infections needs to be taken into account.
AB - BACKGROUND: Homozygous familial hypercholesterolemia (hoFH) is a rare genetic disorder leading to extremely increased LDL-cholesterol (LDL-C), resulting in high cardiovascular risk in early childhood. Lipid apheresis (LA) is an effective treatment and should be started as early as possible to prevent premature cardiovascular events. As peripheral punctures in children can be challenging due to small vessels and anxiety, this study aimed to evaluate feasibility and safety of central venous catheters (CVCs) as vascular access for LA in young children with hoFH.METHODS: Retrospective analysis (2016-2019) on four children with hoFH aged 3-5 years, performing weekly or biweekly LA with a CVC.RESULTS: LDL-C decreased by> 60%. In three children, the use of a permanent CVC for 698, 595, and 411 days, respectively, avoided difficult peripheral access, without the occurrence of occlusion or thrombosis. Unfortunately, one child had recurrent CVC-related infections and needed an arteriovenous fistula from the age of 5. Although the mean dwell time per catheter was 212 days, there were, as expected, severe side effects of early catheter infections with sepsis and accidental self-removal. Starting LA at an early age improved or stabilized carotid intima-media thickness (IMT) in three children. However, IMT did increase in one child caused by intolerance to peripheral punctures and LA interruption.CONCLUSIONS: Permanent CVCs are a viable temporary access choice for LA in young children with hoFH until peripheral venipuncture is practicable. The risk of CVC-related infections needs to be taken into account.
KW - Blood Component Removal/methods
KW - Carotid Intima-Media Thickness
KW - Child, Preschool
KW - Cholesterol, LDL
KW - Homozygote
KW - Homozygous Familial Hypercholesterolemia
KW - Humans
KW - Hyperlipoproteinemia Type II/genetics
KW - Retrospective Studies
U2 - 10.1186/s12887-022-03192-7
DO - 10.1186/s12887-022-03192-7
M3 - Original Article
C2 - 35279116
SN - 1471-2431
VL - 22
SP - 131
JO - BMC PEDIATRICS
JF - BMC PEDIATRICS
IS - 1
ER -