Pediatric Transplantation最新文献

Background: Normothermic regional perfusion (NRP) is gaining rapid popularity in adult donation after circulatory death (DCD) to increase organ utilization and improve outcomes. However, literature is lacking for the pediatric population. We therefore present the youngest DCD donor in the United States from whom a liver was recovered with NRP and subsequently transplanted.

Methods: The donor was a 5-year-old male who underwent thoraco-abdominal NRP for kidney and liver procurement. In total, 72 min passed from the withdrawal of life-sustaining treatment to the start of NRP, resulting in 10 min of functional warm ischemia time. The donor was perfused for 80 min, with lactate levels decreasing from 8.29 at the start of perfusion to 5.40 mmol/L at the end of perfusion. The procured graft weighed 480 g and was subsequently transplanted in an adult female recipient with decompensated cirrhosis due to alcohol-associated liver disease.

Results: The liver was successfully utilized and functioned immediately with no graft-specific complications. The patient was discharged on postoperative day 39.

Conclusions: This case demonstrates that NRP can be applied effectively in small pediatric donors, yielding excellent early graft function. Our experience adds to the emerging literature on pediatric NRP. We conclude that broader adoption of NRP could help increase the donor pool and ease the strain on the pediatric waiting list.

Background: Changes to the calculation of the Kidney Donor Profile Index (KDPI) have lowered the KDPI of hepatitis C (HCV+) donor kidneys; therefore, increasing the proportion of pediatric-prioritized kidneys that are HCV+. We aimed to study consent rates for HCV+ kidneys among pediatric kidney transplant candidates.

Methods: We identified pediatric candidates waitlisted from 2019 to 2024 and excluded those who received a living donor transplant. We used logistic regression to identify candidate characteristics associated with HCV+ offer consent and Cox proportional hazards models to determine the association between HCV+ offer consent and the rate of deceased donor transplantation.

Results: Among 3202 candidates included in the analysis, 124 (4%) consented to receive HCV+ deceased donor kidney offers, and 3077 (96%) did not. In adjusted logistic regression, higher candidate age (OR 1.09 per year, 95% CI 1.03-1.15, p = 0.002) and high PRA status (OR 2.76, 95% CI 1.42-5.37, p = 0.003) were associated with a higher odds of consenting to receive HCV+ donor offers, whereas Hispanic ethnicity was associated with lower odds (OR 0.44, 95% CI 0.28-0.72, p = 0.001) of consenting to receive these offers. 2773 candidates (87%) received a transplant. There was no significant association between HCV+ donor offer consent status and transplant rate after adjusting for candidate characteristics. Only 1 received a kidney from a HCV+ donor.

Conclusions: Consent to receive HCV+ donor kidney offers was rare among pediatric kidney transplant candidates. Allocation changes that increase the proportion of pediatric-prioritized kidneys that are HCV+ may decrease access to transplant for pediatric candidates.

Transition of care from pediatric to adult providers occurs during a time of increased risk, especially in the liver transplant population where close follow-up and adherence to immunologic therapies are critical to patient and graft survival. While there is a substantial body of literature supporting the need for improved transition from pediatric to adult care, concrete steps on how to implement a clinic and partner with adult colleagues are lacking. This article aims to provide a flexible pathway for transition clinics that can be tailored to an institution's unique needs while also advocating for system-wide change to better support transitions in care, specifically through the lens of the United States health care system.

Background: Cardiac allograft vasculopathy (CAV) is the leading cause of late graft loss in children after pediatric heart transplantation (HTx). Coronary angiography, the reference standard for diagnosis, is invasive and carries a risk of complications. Noninvasive echocardiographic methods to reliably identify CAV in children have not yet been defined.

Methods: This study assessed the utility of functional echocardiography (FE) in the diagnosis of CAV in children. We prospectively assessed all children (< 18 years old) post-HTx during routine clinical surveillance between 2005 and 2020. For this study, we compared patients diagnosed with CAV (angiographic ISHLT criteria) with transplanted children without CAV, with FE at the time of diagnosis (d-CAV) and 6-12 months before diagnosis (pre-CAV), and evaluated clinical risk factors.

Results: We identified 10 children with CAV and 11 matched controls (CON). Right ventricular longitudinal strain (RV LS) and strain rate (RV LSR) were reduced at d-CAV (d-CAV RV LS, 12.1% [9.2,16.1] vs. CON RV LS, 21.5% [18.2,25.7]; d-CAV RV LSR, 0.75%/s [0.48, 0.98] vs. CON RV LSR, 1.35%/s [0.95,1.50], p < 0.01 for all) and pre-CAV (pre-CAV RV LS, 17.3% [10.8,20.2] vs. CON RV LS, 21.5% [18.2,25.7]; pre-CAV RV LSR 0.95%/s [0.80,1.10] vs. CON RV LSR, 1.35%/s [0.95,1.50], p ≤ 0.05 for all). Patients with CAV were more likely to have class II donor-specific antibodies (p = 0.01).

Conclusion: Right ventricle systolic strain parameters were reduced as early as 12 months prior to CAV diagnosis, while the left ventricle functional parameters remained preserved. FE of the right ventricle may be a useful noninvasive tool for early recognition of developing CAV in transplanted children.

Background: Filamin A (FLNA) deficiency is a known cause of progressive lung disease and need for pediatric lung transplant; however, what may be less well known to lung transplant providers are the extrapulmonary complications of FLNA deficiencies, such as wandering spleen. We present a patient who underwent a lung transplant for FLNA deficiency and later developed posttransplant abdominal pain.

Case presentation: An 11-year-old female who had previously undergone a bilateral lung transplant due to FLNA deficiency, causing progressive lung disease, presented with abdominal pain and diarrhea. The patient's stool was tested for causes of gastroenteritis using a gastrointestinal pathogen panel (GIPP). Additionally, an initial abdominal ultrasound was obtained to rule out surgical causes of acute abdomen. The initial abdominal ultrasound showed the spleen in the correct anatomical location. However, subsequent abdominal ultrasounds revealed an incidental finding of wandering spleen in multiple locations in the abdomen. As she has remained stable despite the migration of her spleen, the decision was made not to pursue surgical intervention and to continue monitoring with medical and surgical follow-up.

Conclusions: There are multiple complications caused by FLNA deficiency besides progressive respiratory failure, which include gastrointestinal (GI) complications such as wandering spleen. Wandering spleen is a rare clinical entity and, to our knowledge, this is the first case report of it being identified in a pediatric lung transplant patient. This case highlights the importance of transplant providers remaining vigilant when evaluating seemingly benign complaints such as abdominal pain in this population.

Background: The first 35 years of pediatric heart transplantation (pHTx) in Sweden were investigated to determine outcomes following listing and transplantation, investigate sub-populations of recipients, and describe the presence of donor-specific antibodies (DSA) in a contemporary cohort.

Methods: Swedish children < 18 years, listed from 1/1/1989 to 31/12/2023, were included. The cohort was split based on the era of transplantation (ERA I: 1989-2008, ERA II: 2009-2023).

Results: A total of 254 children were listed and 185 (72.8%) reached pHTx, with no loss to follow-up. Waiting list duration was 62 days and increased over time, while mortality on the waiting list decreased (30.5% in ERA I, 8.8% in ERA II). Congenital heart disease was the etiology of heart failure in 36.2% of recipients, including 24.9% with univentricular physiology. The frequency of ABO-incompatible transplantations was 9.3% and 8.0% were considered to be at high immunological risk pre-pHTx due to pre-formed HLA-antibodies with mean fluorescence intensity ≥ 5000. Ventricular assist device (VAD) was used in 26.9% of recipients. Long-term survival was not affected by age, heart failure etiology, the use of pre-transplant VAD, or elevated baseline indexed pulmonary vascular resistance. Era of transplantation was a determinant of listing, but not post-pHTx outcome. Survival at 1-, 10-, and 30-year follow-up was 94.5%, 79.4%, and 57.1%, respectively. Of the total de novo DSA burden, 45.9% were HLA-DQ-type specific. Re-transplantation was performed in 5.9% of recipients.

Conclusions: A high quality of care has been achieved in Sweden, despite modest pHTx numbers, in cooperation with the Scandiatransplant organization.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by the aberrant activation of macrophages. Liver dysfunction is often observed in patients with HLH and has the potential to advance to acute liver failure (ALF). However, limited data exist regarding the application of liver transplantation (LTx) for the management of HLH-associated acute liver failure (HLH-ALF).

Methods: A retrospective analysis was conducted on a pediatric case of LTx for HLH-ALF at our center, and previously documented cases of liver transplantation for this condition were summarized.

Results: A 4-year-old girl was admitted with intermittent fever that had persisted for more than 2 months. She exhibited recurrent high fever (40°C), accompanied by hepatosplenomegaly and superficial lymphadenopathy. The patient's condition rapidly progressed and fulfilled the HLH-2004 diagnostic criteria. The liver function progressively deteriorated. The patient was diagnosed with secondary HLH-ALF. Given the patient's critical condition, she underwent a living donor liver transplantation from her mother (left lobe). Despite experiencing rejection and human herpes virus 6 (HHV 6) infection, the patient recovered well and achieved stable disease.

Conclusion: This experience underscores the potential effectiveness of LTx in the management of HLH-ALF. Prompt investigation and intervention are crucial for patients with HLH exhibiting ALF. Although complex post-transplantation complications may arise, LTx remains a viable treatment option for HLH-ALF.

Background: Childhood overweight and obesity have been hypothesized to pose challenges in pediatric kidney transplantation due to potential complications and resultant worse outcomes. Herein, we aimed to determine if pediatric patients with an elevated body mass index (BMI) (> 85th percentile) are at higher risk for short-term complications following kidney transplantation.

Methods: Following a review of our transplant database (2010-2020), patients > 2-18 years were assigned to groups based on BMI percentiles at the time of surgery: normal (5th-85th percentile; n = 120) and obese/overweight (> 85th percentile; n = 60). Patients were matched using a 1-to-2-ratio nearest-neighbor propensity score matching adjusting for underlying diagnosis, sex, and age. The primary outcome was postoperative complications classified according to Clavien-Dindo, and secondary outcomes included postoperative creatinine and kidney graft rejection.

Results: One hundred eighty patients (n = 120 in the control and n = 60 in the overweight and obese group) were included. There was no significant difference in postoperative complications (72/120 vs. 40/60, p = 0.42). We noted no difference in nadir creatinine (7 days, [interquartile range (IQR) 1, 679] and 9 days, [IQR 1, 690], p = 0.11), postoperative creatinine levels at 3-, 6-, and 12-months post-transplantation, or rejection rates (8.4% and 8.4%, p > 0.99) between normal weighed and obese/overweight patients. However, more overweight and obese patients required intraoperative blood transfusions (55% and 33.4%, p = 0.006) and longer hospital stays (18.0 [IQR 9, 133] and 15.0 [IQR 7, 49], p = 0.02).

Conclusions: It appears as though overweight and obese pediatric kidney transplant patients do not experience more postoperative complications. We propose that overweight and obesity should not be considered criteria to exclude pediatric patients from undergoing kidney transplantations.

Background: Antibody-mediated rejection (AMR) remains a significant complication following heart transplantation, contributing to graft dysfunction and reduced survival. Donor-derived cell-free DNA (dd-cfDNA) is emerging as a non-invasive biomarker for detecting and monitoring graft injury, correlating with episodes of rejection and response to treatment. Daratumumab, an anti-CD38 monoclonal antibody targeting plasma cells, has shown promise in treating AMR. We present a case series of pediatric and young adult heart transplant recipients demonstrating donor-derived cell-free DNA's potential utility in monitoring for AMR and the effect of therapies including daratumumab.

Case descriptions: We report five cases showing that elevated dd-cfDNA correlated with pathological AMR (pAMR), and treatment with daratumumab improved both pAMR and dd-cfDNA levels. Most of our patients had persistently elevated donor-specific antibody (DSA) as observed by MFI values; however, there was a reduction in DSA titer that corresponded with improvement in pAMR and dd-cfDNA levels. Recurrent increases in dd-cfDNA were also useful in guiding the need for repeat treatment with daratumumab. Although DSA levels often remained elevated despite histologic improvement, decreasing dd-cfDNA levels correlated more closely with the resolution of AMR.

Conclusion: In this case series of pediatric and young adult heart transplant recipients, our findings suggest that dd-cfDNA can serve as a valuable biomarker for diagnosing AMR and treatment response, which are not often reflected by DSA MFI alone. Our dd-cfDNA data supports the efficacy of daratumumab in treating AMR and may guide the need for ongoing treatment. Further studies are warranted to validate these findings and establish guidance for the use of daratumumab and dd-cfDNA in this patient population.

Pediatric liver transplantation (PLT) is challenged by anatomical variability and small vessel size, especially in partial grafts. This review outlines strategies to minimize and manage complications of hepatic vein (HV) and portal vein (PV) reconstruction. HV reconstruction employs vein unification, direct caval implantation, or interposition grafts to prevent outflow obstruction, with diagnosis and management guided by imaging and interventional radiology. PV reconstruction in small recipients requires direct anastomosis, interposition grafts, or venoplasty, with intraoperative assessment of flow and pressure. PV thrombosis and stenosis are managed endovascularly or surgically, including Meso-Rex bypass. Individualized approaches and multidisciplinary care have improved outcomes in PLT.