.png){kind=icon}
.png){kind=icon}
Return
Contemporary outcomes of isolated liver and combined liver-lung transplantation for cystic fibrosis in children
.png){kind=icon}
PRE
AI
I
V
D
A
S
M
B
M
S
W
M
DOI：10.1002/jpn3.70392.png)
.png){kind=icon}
Original request for help.png){kind=icon}
Share.png){kind=icon}
Save
Abstract
En 中文
Objective To compare isolated liver transplantation (LT) for cystic fibrosis (CF) versus other indications and versus combined liver-lung transplantation (CLLT) for CF in children and identify factors associated with survival. Methods We compared clinical and survival data after first isolated LT for CF versus other indications and versus CLLT for CF in children (<18 years) using United Network for Organ Sharing data (02/2002-12/2024). Results A total of 157 pediatric CF transplant recipients were included (LT: 145; CLLT: 12). Isolated CF LT recipients had higher total bilirubin (TB) than CLLT (median 1.6 vs. 0.7 mg/dL, p = 0.02). A higher proportion of CF transplant recipients with high TB levels (>= 1.5 mg/dL) had ascites, encephalopathy, and required life support compared to those with low TB levels (<1.5 mg/dL). CF LT demonstrated superior patient survival versus CF CLLT (log-rank test, p = 0.02; 5-year: 89.1% vs. 72.2%), but inferior versus non-CF LT (log-rank test, p < 0.001; 5-year: 91.5%). Multivariable Cox regression showed increased risk of patient mortality and liver graft loss in CF CLLT recipients compared to isolated CF LT recipients (hazard ratio [HR] = 2.92, 95% confidence interval [95% CI]: 1.20-7.07, p = 0.02 and HR = 2.56, 95% CI: 1.09-5.98, p = 0.03, respectively) and recipients with higher TB levels (HR = 1.05, 95% CI: 1.01-1.10, p = 0.008 and HR = 1.05, 95% CI: 1.01-1.09, p = 0.008, respectively), when adjusting for recipient age, albumin and international normalized ratio (INR) at time of LT, ICU status, and liver graft type. Multivariable Cox regression of isolated LT recipients showed increased risk of patient mortality (HR = 2.03, 95% CI: 1.41-2.93, p < 0.001) and liver graft loss (HR = 1.54, 95% CI: 1.13-2.11, p = 0.006) for CF compared to non-CF etiologies, when adjusting for recipient age, albumin, INR, and TB at time of LT, ICU status, and liver graft type. Conclusion Isolated LT for CF was associated with superior survival compared to CLLT for CF, but inferior survival compared to LT for non-CF indications. Higher TB in CF may be a marker of inferior outcomes post-LT.
Keywords:
bilirubin
cirrhosis
lung transplantation
pediatric liver transplantation
united network for organ sharing
Journal
IF:
2.6
Papers:
250
Citations:
1.4W
Organization
Cited Papers