Familial hypertriglyceridemia (FHTG) is a rare but important cause of recurrent acute pancreatitis (RAP) in children. Liver transplantation (LT) may be the only definitive metabolic cure for this condition, although such an approach has not been reported previously. In the current report, we describe a 8-year-old girl suffering from RAP secondary to FHTG for whom an LT led to complete resolution of her metabolic defect. An 8-year-old girl with FHTG had severe RAP since the age of 6 years. Her serum triglyceride (TG) levels ranged between 2000 and 5439 mg/dL during the episodes of acute pancreatitis severe enough to cause hospitalisations. Acute episodes were managed with insulin-dextrose infusion and therapeutic plasma exchange. She was maintained on hypolipidemic agents along with a fat-restricted diet, despite which the TG levels continued to remain elevated, triggering further attacks of pancreatitis. In due course, she developed progressive wasting and stunting. After multidisciplinary evaluation and based on a strong scientific rationale, a living donor liver transplant (LDLT) was offered with a left lateral segment from her father. Post-transplant, the child had an uneventful recovery, without any medical or surgical complications, and her serum TG levels normalized within 6 weeks. At 11 months follow-up, her TG levels are 75 mg/dL, and she is free of any further episodes of pancreatitis. This is the first report of a successful cure of FHTG via LDLT, highlighting its potential as a definitive therapeutic option for selected pediatric patients with severe, refractory HTG.

Heart transplant is a life-saving treatment for children with advanced heart failure. Despite improvements in overall survival, the quality of life of both patients and their family caregivers can be negatively impacted by lifelong post-transplant care. The purpose of this study is to understand the experience of pediatric patients and their caregivers with their overall health, well-being, and quality of life after heart transplantation. This is a qualitative study using semi-structured interviews and inductive thematic analysis, conducted at a single center in the United States. Adolescent heart transplant recipients (HTRs) and their family caregivers were included. Semi-structured interviews were conducted with 12 HTRs and 13 parents. Several themes appeared from the interviews: (1) heart transplant recipients experience overall better physical health after transplant, despite post-transplant complications. Both parents and HTRs (2) have difficulty reintegrating into social life and adjusting to new activity restrictions, (3) experience a reorganization of social support networks, (4) and experience high emotional burden from multiple stressors. Finally, (5) heart transplant recipients and their parents suggested greater social resources, mental health resources, and telehealth options to improve post-transplant care. Findings from this study paint a complex picture of the overall quality of life for adolescent HTRs and their parents. Clinical recommendations generated from results include leveraging care that keeps families close to home whenever possible, assessing communication preferences of the HTR and their family, validating mental health struggles for HTRs and their family, and longitudinally screening for anxiety, PTSD, and depression.

China's growing healthcare system, serving over 1.4 billion people, has witnessed remarkable progress in organ transplantation, including among pediatric patients. This review provides a comprehensive overview of pediatric transplantation in China, summarizing national trends, regulatory frameworks, clinical outcomes, and ongoing challenges. Data were obtained from the Report on Organ Donation and Transplantation in China (RODTC) between 2015 and 2024. Pediatric patients were defined as those under 18 years of age. Following the 2015 reforms establishing voluntary citizen donation as the only legitimate organ source, China has developed a robust national regulatory and ethical framework under the National Health Commission. The China Organ Transplant Response System (COTRS) ensures transparent allocation, with a 2018 policy granting priority to pediatric recipients and mandating that organs from donors under 18 be preferentially allocated to pediatric patients. From 2015 to 2024, pediatric kidney, liver, and lung transplants increased 4.9-, 2.1-, and 7.5-fold, respectively; pediatric heart transplantation increased 2.5-fold from 2019 to 2024. In 2019, child donors represented 8.20% of deceased donors. Leading indications included non-ischemic cardiomyopathy for heart, bronchiolitis obliterans for lung, biliary atresia and metabolic liver disease for liver, and glomerulonephritis for kidney transplantation. Reported survival rates were favorable, with 5-year patient survival of 70.0% for heart and 95.5% for kidney transplants. Major transplant centers are concentrated in Shanghai, Guangzhou, Zhengzhou, and Beijing. Four national registries ensure lifelong follow-up and quality monitoring. Pediatric organ transplantation in China has advanced rapidly under strengthened ethical oversight and allocation reforms prioritizing children. Despite significant growth, challenges remain in data accessibility, regional disparities, and donor shortages. Continued policy refinement, registry expansion, and regional collaboration-particularly with Hong Kong and Macao-are essential to further improve access and outcomes for pediatric transplant recipients.

Clinical practice variation in the management of recurrent idiopathic focal segmental glomerulosclerosis (rFSGS) is poorly defined and likely hinders successful clinical trial design. We conducted an online survey between June 2024 and September 2024 of Pediatric Nephrology Research Consortium (PNRC) and European Society for Pediatric Nephrology (ESPN) members to evaluate global clinical practice patterns in the management of pediatric rFSGS and physician perceptions of barriers to rFSGS clinical trial participation. Of 120 responses (50 PNRC members from the US and Canada and 70 ESPN members from Europe, Asia, and South America), all respondents were physicians and 50% were transplant program medical directors. Pretransplant mutation analysis was routine in 104 (87%) centers, and native nephrectomy was routine for patients with and without hypoalbuminemia in 77 (66%) and 23 (19%) centers, respectively. Forty-two (35%) centers reported routine therapeutic interventions to prevent rFSGS prior to kidney transplantation; this increased to 79 centers (66%) in patients with previous recurrence. Plasmapheresis was the first therapeutic intervention in most centers, although it was used significantly more often in North America (96%) than Europe (77%; p = 0.005). Rituximab was administered in 102 (85%) centers as treatment for rFSGS. Common barriers to recruitment and inclusion of patients in clinical trials of rFSGS were limited site resources (36%), recipient/family reluctance (10%), and operational and logistical issues (26%). Heterogeneity of practice patterns in the management of pediatric rFSGS and barriers to clinical trials requires collaborative efforts to study and reach consensus on prevention and management of rFSGS.

Portal vein (PV) reconstruction is a crucial step in pediatric liver transplantation (LT). Pediatric recipients with a hypoplastic or sclerotic PV often require an interposition vein graft. Allogeneic grafts from donors are generally preferred. However, if they are not available, the use of autologous vessels is necessary. An 8-month-old girl with biliary atresia (BA) underwent living donor LT with a left lateral segment graft. During the operation, portal vein thrombosis (PVT) developed after direct anastomosis between the recipient PV and graft left PV. We used several techniques to resolve this problem, including ligation of collateral circulation and use of interposition grafts from the left internal jugular vein (IJV) and left renal vein (LRV). On postoperative day 1, PVT reoccurred. Emergency exploratory laparotomy was performed for thrombectomy, using the retrohepatic inferior vena cava (IVC) as an additional vascular graft. The final reconstruction successfully utilized a combination of LRV and IVC grafts between the superior mesenteric vein and graft left PV. Postoperatively, the patient received thrombolytic therapy followed by anticoagulants for thrombus prevention, along with immunosuppressive drugs. The patient's postoperative clinical course was uneventful. Although the IJV and LRV are established options for PV reconstruction in pediatric LT, we propose retrohepatic IVC as a feasible and effective alternative option. This approach maximizes the availability of autologous vessels; however, selection must be individualized based on graft availability and the patient condition.

Lung transplantation (LTx) is a life-saving intervention for pediatric patients with end-stage lung diseases. Understanding its impact on renal function is essential for improving patient management and achieving the primary goals of LTx: enhancing respiratory function, quality of life, and survival. This study analyzes the effect of LTx on renal function in pediatric patients. This retrospective study assessed renal function in 49 children (age range: 1-204 months; median 144 months, IQR 72 months; 26 females, 23 males) who underwent LTx. Renal function was evaluated quantitatively (using glomerular filtration rate, GFR) and qualitatively (using renal scans) before and after LTx. The lowest post-LTx GFR (post-LTx LGFR) was the assessment endpoint. GFR decline was analyzed as a percentage reduction from pre- to post-LTx LGFR. The impact of age, sex, primary lung disease, and nephrotoxic medications on GFR decline was also examined. The mean pre-LTx GFR was 151.5 mL/min/1.73 m2, while the mean post-LTx GFR was 81.24 mL/min/1.73 m2. A decrease in GFR of more than 25% was observed in 72.09% of patients, with 40.48% experiencing a reduction of more than 50%. The prevalence of impaired renal function (GFR < 90 mL/min/1.73 m2) increased from 11.63% pre-LTx to 65.31% post-LTx. Nephrotoxic agents (p = 0.006) and infections (p = 0.001) were significant risk factors. Lung transplantation significantly reduces renal function in pediatric patients. Frequent renal assessments are recommended for early detection and management of renal impairment, focusing on mitigating nephrotoxic medication and infection risks.

ABSTRACTBackgroundIn order to reduce the waiting time, new strategies have been developed to safely transplant donor and recipient pairs with mismatched blood groups. The present study examined the safety of the preparatory treatments and the midterm outcome of AB0 incompatible (AB0i) kidney transplantation in children.MethodsWe retrospectively analyzed 10 children who received a kidney transplant from an AB0i donor from 2012 to 2024 and 30 patients matched by sex, height, and weight who received an AB0 compatible (AB0c) living kidney transplant in the same period.ResultsIn the AB0i group, preparatory treatment before KTx was well tolerated. The number of rejection episodes was comparable in the AB0i group and in the AB0c group (three episodes in three patients vs. seven episodes in six patients) during the observation period of 36 months, with rejections occurring earlier in the AB0c group. Infections were more frequent in the AB0c group than in the AB0i group (30 episodes in 23 patients [76%] vs. eight episodes in six patients [60%]). In the AB0i group, the 3‐year graft survival rate was 90%; the 3‐year patient survival was 100%. In the AB0c group, the 3‐year graft survival was 86.8%, and the 3‐year patient survival was 93.3%. There was no difference in graft survival (p = 0.08) and patient survival (p = 0.24) between the AB0i and AB0c groups.ConclusionsAB0 incompatible kidney transplants can be safely performed in children with equivalent midterm graft and patient survival.

Over the last decades, long-term survival after pediatric liver transplantation (LT) has improved substantially, highlighting the importance of long-term graft and recipient outcomes. Metabolic syndrome, a combination of components associated with increased cardiovascular risk, is a well-defined concept in the general adult population. The same components can be present after LT leading to post-transplant metabolic syndrome (PTMS). In children, PTMS is estimated to be prevalent in around 14%-20% of LT recipients. However, a univocal definition is lacking as well as consensus on specific cut-off values for the different components. The latter comprise: low HDL-cholesterol, high triglycerides (both falling under the heading of "dyslipidemia"), hypertension, impaired fasting glucose and/or impaired glucose tolerance, and (abdominal) obesity. Although most studies have a retrospective or cross-sectional nature and are of relatively small sample size, there seems to be an accumulation of risk factors in the first years after transplantation, declining thereafter and rising again in the long term. Sustained exposure to immunosuppression is identified as a major contributor, as well as accumulating general cardiometabolic risk factors throughout life.

To describe an exceedingly rare vascular anatomical variant of the splenic artery arising from the superior mesenteric artery (SMA), termed a splenomesenteric trunk, with angiographic imaging in a pediatric patient. Case review of a 6 year-old boy, status post liver transplantation for biliary atresia, who presented with ongoing hematemesis, melena, portal hypertension and chronic thrombocytopenia from hypersplenism. He underwent portal venous stenting, and we decided to perform a partial splenic embolization to reduce portal pressure and improve platelet counts. CT and angiographic imaging findings are presented, with literature correlation. CT 3D and DSA demonstrated the absence of the splenic artery on injection of the celiac trunk. Selective SMA angiography revealed a variant origin of the splenic artery from the SMA, with normal caliber and no aneurysm or stenosis. Recognition of this rare vascular variant is essential to avoid procedural complications. This represents, to our knowledge, the first angiographically documented case of a splenomesenteric trunk in a pediatric patient.

End-stage renal disease (ESRD) is a critical global health concern, with kidney transplantation being the preferred treatment for children with stage 5 chronic kidney disease. However, ABO-incompatibility (ABOi) poses a major barrier to successful transplantation. While advances in antibody removal techniques, including immunoadsorption (IA), have enabled ABOi kidney transplantation, cost remains a significant challenge, especially in low-resource settings. Most available data focus on adults, with limited pediatric reports, particularly using reusable IA columns. We report the case of a 7-year-old girl who underwent ABOi kidney transplantation from her mother, despite an exceptionally high pretransplant anti-B IgG antibody titer of 1:1 024. She received a single dose of Rituximab (200 mg) 14 days pretransplant, followed by two IA sessions using a reusable Vitrosorb IA column. The first session reduced titers to 1:16, but a rebound increase to 1:128 necessitated a second session, bringing titers down to 1:8. A final plasmapheresis session stabilized titers before transplantation. The surgery was uneventful, and post-transplant antibody levels remained controlled. The patient demonstrated good graft function and was discharged in stable condition. This is the highest reported pretransplant titer (1:1 024) in a pediatric ABOi kidney transplant case. The use of a reusable Vitrosorb IA column proved both effective and cost-efficient, making it a viable option in resource-limited settings. Our findings suggest that IA reuse is safe, reduces financial burden, and warrants further investigation for broader clinical application.