Pediatric Transplantation最新文献

Background: Solid organ transplantation has evolved in recent decades, resulting in a rise in patient and graft survival. Frequent hospitalizations affect graft function, patients' health, and quality of life. This study characterizes the frequency and causes of post-transplant hospitalizations among pediatric recipients.

Methods: This is a retrospective observational study evaluating pediatric kidney transplant recipients (KTR) and liver transplant recipients (LTR) aged 0-21 years, followed at a tertiary pediatric center in Israel from 2012 to 2017. Data were collected starting at 60 days post-transplantation. Diagnoses of admissions were based on clinical, laboratory, and radiographic findings.

Results: Forty-nine KTR experienced 199 all-cause re-hospitalizations (median number of re-hospitalizations per patient - 3 (IQR [interquartile range] 1-5.5), while 351 re-hospitalizations were recorded in 56 LTR (median - 5 [IQR 2-8.8]). Median follow-up time was 2.2 years for KTR (IQR 1-3.9) and 3 years for LTR (IQR 2.1-4.1). The most common cause for hospitalization for both cohorts was infection (50.8% and 62%, respectively). Gram-negative bacteria were the most common pathogens identified in KTR, while viral pathogens were more common in LTR (51% and 57% of pathogen-identified cases, respectively).

Conclusions: This is the largest study to describe rehospitalizations for pediatric solid organ recipients. The hospital admission rate was higher in LTR in comparison to KTR. Infections were the most common cause of hospitalization throughout the whole study period in both populations. Frequent hospitalizations impose a heavy burden on patients and their families; better understanding of hospitalization causes may help to minimize their frequency.

Background: Renal function is reduced in patients undergoing heart transplant due to hemodynamic compromise, cardiorenal syndrome, and nephrotoxin exposure. No current studies evaluate renal function in retransplants.

Methods: We reviewed all heart transplants at our center from 1995 to 2021 and matched first-time heart transplants with retransplants, based on age at transplant, sex, and race. Estimated glomerular filtration rate (eGFR) was derived from CKiD-U25 calculator using creatinine and measured prior to transplant, 1-week post-transplant, 1-3, 6, and 12 months post-transplant, and recent follow-up. Changes in eGFR were measured within and between patients using a piecewise linear mixed effect model with matching. Exploratory univariate analysis was performed to evaluate pre-transplant risk factors for decreased eGFR.

Results: The unmatched cohort included 393 heart transplant recipients, with 47 being retransplants. Thirty-eight patients in both groups with at least 1 year of follow-up underwent matching. Both retransplants and first-time transplants had an initial decline in eGFR. eGFR rebounded to baseline or above baseline at 1-3 months post-transplant, but eGFR in retransplants remained significantly lower. At 1-year post-transplant, the average eGFR was 67.8 ± 4.3 mL/min/1.73 m² versus 104.7 ± 4.3 mL/min/1.73 m² (p < .001) in the retransplants and first-time transplants group, respectively.

Conclusion: This study provides data on anticipated renal trajectory following retransplantation.

Background: Hepatic undifferentiated embryonal sarcoma (HUES) is the third most common primary hepatic malignancy in children. If unresectable, liver transplantation (LT) is the only curative option. Historically, HUES LT outcomes were not favorable; however, modern-era data are lacking. We aimed to describe LT outcomes in children with HUES and compared with LT outcomes in children transplanted for hepatoblastoma (HBL) and non-malignancy indications.

Methods: Children 18 years or younger with HUES who underwent LT from 1987 to 2021 were identified from the Scientific Registry of Transplant Recipients database. Graft and patient survival were studied in HUES and LT recipients with HBL and non-malignancy indications using Kaplan-Meier analysis. Cox regression was used to compare patient and graft survival among groups, controlling for confounders.

Results: Twenty-one children with HUES underwent LT during the study period with a median age at LT of 10 years (IQR: 8-12 years). One and five-year patient survival for HUES recipients was not significantly different from that of recipients with HBL (p = .3) or non-malignancy diagnoses (p = .6). There were no deaths due to HUES recurrence. In multivariable Cox regression, HUES did not increase risk of either patient or graft loss as compared to HBL (HR 2.36, p = .2) or non-malignancy indications (HR 0.74, p = .7).

Conclusion: LT outcomes are more favorable in patients with HUES than historically described, and similar to LT outcomes of patients with HBL and non-malignancy indications. Transplant should be considered for HUES patients with unresectable localized tumors.

Background: Hepatic artery thrombosis (HAT) is a reported complication of 5%-10% of pediatric liver transplantations, rates 3-4 times that seen in adults. Early HAT (seen within 14 days after transplant) can lead to severe allograft damage and possible urgent re-transplantation. In this report, we present our analysis of HAT in pediatric liver transplant from a national clinical database and examine the association of HAT with anticoagulant or antiplatelet medication administered in the post-operative period.

Methods: Data were obtained from the Pediatric Health Information System database maintained by the Children's Hospital Association. For each liver transplant recipient identified in a 10-year period, diagnosis, demographic, and medication data were collected and analyzed.

Results: Our findings showed an average rate of HAT of 6.3% across 31 centers. Anticoagulant and antiplatelet medication strategies varied distinctly among and even within centers, likely due to the fact there are no consensus guidelines. Notably, in centers with similar medication usage, HAT rates continue to vary. At the patient level, use of aspirin within the first 72 h of transplantation was associated with a decreased risk of HAT, consistent with other reports in the literature.

Conclusion: We suggest that concerted efforts to standardize anticoagulation approaches in pediatric liver transplant may be of benefit in the prevention of HAT. A prospective multi-institutional study of regimen-possibly including aspirin-following transplantation could have significant value.

Background: Kidney transplant (KT) was initially associated with poor outcomes, especially in smaller recipients. However, pediatric transplantation has evolved considerably over time. We investigated the impact of weight at the time of transplant and whether outcomes changed over 25 years for <10 kg recipients.

Methods: Using the UNOS database, pediatric recipient outcomes were analyzed between 1/1/99 and 12/31/14. KT weight was stratified: <8.6 kg (mean weight of recipients <10 kg), 8.6-9.9 kg, 10-14.9 kg, 15-29.9 kg, and ≥30 kg. Outcomes in recipients <10 kg were then compared between 1990-1999 and 2000-2014.

Results: 17 314 pediatric KT recipients were included; 518 (3%) had a transplant weight <10 kg. The highest rates of allograft loss and death were in recipients <8.6 kg and ≥30 kg. Recipients <8.6 kg also had higher rates of delayed graft function, rejection, and longer hospital length of stay. In the multivariable Cox regression model, transplant weight was not a predictor of allograft loss. When compared with recipients <8.6 kg, patient survival hazard ratios associated with recipient weight of 10-14.9 kg, 15-29.9 kg, and ≥30 kg were 0.61 (95%CI: 0.4, 1), 0.42 (95%CI: 0.3, 0.7) and 0.32 (95%CI: 0.2, 0.6), respectively. In the later era of transplant, recipients <10 kg had improved outcomes on univariate analysis; however, the era of transplantation was not an independent predictor of allograft loss or patient survival in Cox regression models.

Conclusions: Outcomes in children weighing 8.6-9.9 kg at the time of KT were similar to higher weight groups and improved over time; however, special precautions should be taken for recipients <8.6 kg at the time of transplant.

Background: Children at high risk for prolonged mechanical ventilation (PMV) after liver transplantation (LT) need to be identified early to optimize pulmonary support, allocate resources, and improve surgical outcomes. We aimed to develop and validate a metric that can estimate risk for Prolonged Ventilation After LT (PROVE-ALT).

Methods: We identified preoperative risk factors for PMV by univariable analysis in a retrospective cohort of pediatric LT recipients between 2011 and 2017 (n = 205; derivation cohort). We created the PROVE-ALT score by mapping multivariable logistic regression coefficients as integers, with cutoff values using the Youden Index. We validated the score by C-statistic in a retrospectively collected separate cohort of pediatric LT recipients between 2018 and 2021 (n = 133, validation cohort).

Results: Among total 338 patients, 21% (n = 72) were infants; 49% (n = 167) had cirrhosis; 8% (n = 27) required continuous renal replacement therapy (CRRT); and 32% (n = 111) required management in hospital (MIH) before LT. Incidence of PMV post-LT was 20% (n = 69) and 3% (n = 12) required tracheostomy. Independent risk factors (OR [95% CI]) for PMV were cirrhosis (3.8 [1-14], p = .04); age <1-year (8.2 [2-30], p = .001); need for preoperative CRRT (6.3 [1.2-32], p = .02); and MIH before LT (12.4 [2.1-71], p = .004). PROVE-ALT score ≥8 [Range = 0-21] accurately predicted PMV in the validation cohort with 73% sensitivity and 80% specificity (AUC: 0.81; 95% CI: 0.71-0.91).

Conclusion: PROVE-ALT can predict PMV after pediatric LT with a high degree of sensitivity and specificity. Once externally validated in other centers, PROVE-ALT will empower clinicians to plan patient-specific ventilation strategies, provide parental anticipatory guidance, and optimize hospital resources.

Background: The optimal age of kidney transplantation for infants and toddlers with kidney failure is unclear. We aimed to evaluate the patient survival associated with kidney transplantation before 2 years of age versus remaining on the waitlist until ≥2 years.

Method: We used the Scientific Registry of Transplant Recipients to identify all children added to the deceased-donor waitlist before 2 years of age between 1/1/2000 and 4/30/2020. For each case aged <2 years at transplant, we created a control group comprising all candidates on the waitlist on the case's transplant date. Patient survival was evaluated using sequential Cox regression. Dialysis-free time was defined as graft survival time for cases and the sum of dialysis-free time on the waitlist and graft survival time for controls.

Results: We observed similar patient survival for posttransplant periods 0-3 and 4-12 months but higher survival for period >12 months for <2-year decreased-donor recipients (aHR: 0.32; 95% CI: 0.13-0.78; p = .01) compared with controls. Similarly, patient survival was higher for <2-year living-donor recipients for posttransplant period >12 months (aHR: 0.21; 95% CI: 0.06-0.73; p = .01). The 5-year dialysis-free survival was higher for <2-year deceased- (difference: 0.59 years; 95% CI: 0.23-0.93) and living-donor (difference: 1.84 years; 95% CI: 1.31-2.25) recipients.

Conclusion: Kidney transplantation in children <2 years of age is associated with improved patient survival and reduced dialysis exposure compared with remaining on the waitlist until ≥2 years.

Introduction: In pediatric patients with Budd-Chiari syndrome (BCS), living donor liver transplantation (LDLT) raises substantial challenges regarding IVC reconstruction.

Case presentation: We present a case of an 8-year-old girl with BCS caused by myeloproliferative syndrome with JAK2 V617F mutation. She had a complete thrombosis of the inferior vena cava (IVC) with multiple collaterals, developing a Budd-Chiari syndrome. She underwent LDLT with IVC reconstruction with a cryopreserved pulmonary vein graft obtained from a provincial biobank. The living donor underwent a laparoscopic-assisted left lateral hepatectomy. The reconstruction of the vena cava took place on the back table and the liver was implanted en bloc with the reconstructed IVC in the recipient. Anticoagulation was immediately restarted after the surgery because of her pro-thrombotic state. Her postoperative course was complicated by a biliary anastomotic leak and an infected biloma. The patient recovered progressively and remained well on outpatient clinic follow-up 32 weeks after the procedure.

Conclusion: IVC reconstruction using a cryopreserved pulmonary vein graft is a valid option during LDLT for pediatric patients with BCS where reconstruction of the IVC entails considerable challenges. Early referral to a pediatric liver transplant facility with a multidisciplinary team is also important in the management of pediatric patients with BCS.