Introduction
Sinusoidal obstruction syndrome (SOS), formerly known as veno-occlusive
disease (VOD), is a serious complication encountered in the early phase
post hematopoietic cell transplant (HCT). The pathogenesis of SOS
involves injury to the sinusoidal endothelial cells of the liver,
resulting in increased vascular permeability and narrowing of the
sinusoids, slowing the blood flow within the vascular lumen (1, 2). The
incidence of SOS is typically 5% to 13% but is reported to be higher
in the pediatric high-risk population (20-60%) (1-3). Severe SOS is
associated with multi-organ dysfunction (MOD) and a higher rate of
mortality. Acute kidney injury (AKI) seen in SOS is secondary to kidney
hypoperfusion, vasoconstriction, portal hypertension, and fluid overload
(3-5). In addition, intra-abdominal hypertension can exacerbate
pre-existing kidney injury (3). SOS increases the risk of AKI by 6.02
times in these patients (4). Despite treatment advances, such as the
introduction of defibrotide that contributed to reduced mortality and
reversal of organ dysfunction, mortality continues to be high if MOD is
present, with a 100-day estimated survival rate of only 28% in
defibrotide-treated patients (adults and children) who had both
ventilator and dialysis dependence (6).
Continuous kidney replacement therapy (CKRT) is used to manage severe
AKI and to mitigate the effects of fluid overload (FO), both of which
occur in children with SOS. Recent guidelines by the
HCT subgroup
of the
Pediatric Acute
Lung Injury and Sepsis Investigators (PALISI) and the Supportive Care
Committee of Pediatric Blood and Marrow Transplant Consortium (PBMTC)
recommended consideration of CKRT in children with SOS when FO is ≥10%
(7). Studies that evaluate the use of CKRT in children with SOS
following HCT are lacking. Therefore, we sought to examine the use of
CKRT in a cohort of critically ill children with SOS following HCT. The
primary objective of this study was to assess the outcome of critically
ill children post-HCT with severe SOS who received CKRT therapy. In
addition, our aim was to assess factors associated with survival and
liberation post-CKRT.