Abstract
Tunneled central venous catheters (TCVCs) provide prolonged intravenous access for pediatric patients with severe primary immunodeficiency disease (PID) undergoing hematopoietic stem cell transplantation (HSCT). However, little is known about the epidemiology and clinical significance of TCVC-related morbidity in this particular patient group. We conducted the retrospective analysis of patients with severe PID who received percutaneous landmark-guided TCVC implantation prior to HSCT. We analyzed 92 consecutive TCVC implantations in 69 patients (median [interquartile range] age 3.0 [0-11] years) with severe combined immune deficiency (n = 39, 42.4%), chronic granulomatous disease (n = 17, 18.4%), and other rare PID syndromes (n = 36, 39.2%). The median length of TCVC observation was 144.1 (85.5-194.6) days with a total of 14,040 catheter days at risk (cdr). The overall rate of adverse events during catheter insertion was 17.4% (n = 16) and 25.0% during catheter dwell period (n = 23, catheter risk [CR] per 1000 cdr = 1.64). The most common complication was TCVC-related infection with an overall prevalence of 9.8% (n = 9, CR = 0.64), followed by late dislocation (n = 6, 6.5%, CR = 0.43), early dislocation (n = 4, 4.3%) and catheter dysfunction (n = 4, 4.3%, CR = 0.28). TCVCs are safe in children with severe PID undergoing HSCT with relatively low rates of TCVC-related infection.
Highlights
Primary immunodeficiency diseases (PID) comprise a wide spectrum of disorders, including more than 350 genetically defined inborn errors of adaptive and innate immunity [1]
This study included 92 consecutive catheter positioning procedures in 69 patients with severe PID considered for hematopoietic stem cell transplantation (HSCT)
Ultrasound guidance has been shown to reduce the incidence of failures and inadvertent arterial punctures in pediatric central venous catheterization [15], percutaneous landmark technique was predominantly utilized in our study as this approach is traditional at our Complications during catheter dwell period Early dislocation, n (%) time to surgical revision (TTR), d, Median (IQR) Late dislocation, n (%) CR TTR, d, Median (IQR) Infection, n (%) CR TTR, d, Median (IQR) Dysfunction, n (%) CR TTR, d, Median (IQR) Total, n (%) CR Time to surgical revision (TTR), Median (IQR)
Summary
Primary immunodeficiency diseases (PID) comprise a wide spectrum of disorders, including more than 350 genetically defined inborn errors of adaptive and innate immunity [1]. The clinical presentation of PID is highly variable, many disorders are characterized by recurrent, serious infections, autoimmune dysregulation, and aberrant inflammation if not treated appropriately [2]. The management of PID depends on the type and severity of the underlying defect.
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