Progressive familial intrahepatic cholestasis represents a group of disorders characterized by defective bile excretion, which causes a multitude of clinical symptoms of variable severity and usually begins in childhood. During the past few decades, a number of gene sequence variants have been shown to be associated with progressive familial intrahepatic cholestasis, and new subtypes continue to be discovered. Sequence variants of the ubiquitinspecific peptidase 53 gene have previously been associated with a novel autosomal recessive form of cholestasis with coincident normal or low γ-glutamyl transferase, with mild phenotypes. Here, we present 2 siblings with novel homozygous sequence variants in the ubiquitin-specific peptidase 53 gene with acute-on-chronic liver failure who underwent liver transplant.

Invasive aspergillosis is a rare but serious complication that can occur in solid-organ transplant recipients. This condition arises from an infection caused by Aspergillus, a widespread mold typically transmitted through the inhalation of its spores (conidia). This report presents 3 unique cases of invasive aspergillosis in patients who received either kidney or liver transplants from a 17-year-old deceased pediatric donor. The first case was a 27-yearold male kidney transplant recipient who developed symptoms at posttransplant day 65, leading to graft nephrectomy despite antifungal therapy. The second case, a 17-year-old male kidney transplant recipient with a posttransplant course similar to the first case, also required nephrectomy due to worsening invasive aspergillosis. The third case involved a 28-year-old female liver transplant recipient who presented with symptoms at posttransplant day 45 but recovered without the need for graft explant. The occurrence of Aspergillus in graft organs is generally rare and is often associated with systemic hematogenous dissemination before transplant. Clinicians should maintain a high index of suspicion for invasive mycotic infections in solid-organ transplant recipients, especially for patients who present with clump-like lesions on diagnostic imaging. Such awareness is crucial for timely diagnosis and management and may thereby potentially improve patient outcomes in this vulnerable population.

We investigated hepatoblastoma with invasion into the vessels of the portal vein and widespread thrombosis of the portal vein, where tumor resection is impossible, that was treated with liver transplant.

Abernethy syndrome is a rare congenital vascular anomaly. In this condition, blood from the portal system is diverted to the caval system through a portocaval shunt, entirely or partially bypassing the liver. Prevalence figures in the literature range from 1 per 30000 population up to 1 per 50000 population. Abernethy syndrome is classified into 2 types based on the absence (type 1) or presence (type 2) of an intrahepatic portal vein. Abernethy syndrome can manifest with hepatic encephalopathy, pulmonary hypertension, severe osteoporosis, gastrointestinal bleeding, cirrhosis, hepatopulmonary syndrome, and abdominal pain. Hepatocellular carcinoma is a less common presentation. Type 1 Abernethy syndrome is usually treated with a liver transplant, whereas type 2 is addressed by closing the shunt either endovascularly or surgically. Here, we present cases to highlight that surgical shunt ligation is an effective treatment for type 2 Abernethy malformation when the shunt cannot be closed endovascularly.

Organ transplant is a vital treatment for pediatric patients. Kidney, liver, heart, and other organ transplants can significantly improve the quality of life for children with various chronic diseases and can improve long-term survival rates. However, the effects of transplant on cognitive and educational aspects should be considered, including the effects of pre- and posttransplant treatment protocols, medications, psychosocial stress, and surgical interventions. Learning disabilities can negatively affect the child's educational life, social relationships, and overall quality of life. We aimed to examine the prevalence of learning difficulties after organ transplant, the influencing factors, and the interventions aimed at solving these problems by conducting a systematic review of existing research on learning difficulties associated with pediatric organ transplant.

Hepatitis delta virus infection poses a significant challenge in solid-organ transplant recipients due to its aggressive nature and limited therapeutic options. Bulevirtide is a novel antiviral agent approved by the European Medicines Agency in 2020 for the treatment of hepatitis delta virus infection, but limited data are available on its use in solid-organ transplant recipients.

End-stage renal disease is a health concern around the world. Kidney transplant substantially improves survival compared with dialysis. Although kidney transplant is the better choice for treatment of end-stage renal disease, most patients refuse this option. In this study, we aimed to find the most common reasons why patients did not undergo a kidney transplant.

This study investigated the level of organ donation awareness among students at vocational schools of different sectors in order to measure of awareness of organ donation among young population of our society.

There have been conflicting reports detailing the effect of sex match between transplant donor and recipient on the outcome of solid-organ transplants. We investigated the effect of donor-recipient sex match on allograft function and survival outcomes after simultaneous pancreas-kidney transplant at a single transplant center in China.

Chemokines are inflammatory mediators directly involved in immunological mechanisms that mediate alloimmune responses; recently, gene expression analysis studies have aroused great interest in the field of transplantation. We aimed to evaluate the predictive role of chemokine gene expression in rejection in renal transplant patients.

The right posterior segment graft can be selected in cases where neither the right nor left lobe graft satisfies the selection criteria for adult living donor liver transplant. However, vascular and biliary anatomy may cause technical difficulties in procurement of posterior segments in donors of living donor liver transplant and may require specific attention in vascular and biliary reconstruction in the recipient. In this study, we examined the feasibility of right posterior segment grafts in adult living donor liver transplants through donor safety and recipient outcomes and clarified the anatomic points of the surgical technique.

Portal inflow modulation is currently an essential step in living donor liver transplantation, particularly among patients with portal hypertension who receive small grafts. This study compared splenic artery ligation with splenectomy as a method for portal inflow modulation.

Hematopoietic stem cell transplant is increasingly being used as a curative therapeutic option for patients with hematologic malignancies and nonmalignant diseases. Here, we aimed to determine the frequency and features of typical and atypical central variants of posterior reversible encephalopathy syndrome in children who had undergone an allogeneic hematopoietic stem cell transplant procedure for any indication at a single center.

Patients with liver transplant can present to a pediatric emergency department with short- and long-term complications of transplant. Here, we described clinical features of pediatric liver transplant patients admitted to the pediatric emergency department and identified risk factors that may lead to pediatric intensive care unit admission.

After renal transplant, identification of early graft dysfunction is crucial for graft survival. We aimed to assess the perfusion of the transplanted kidney using near-infrared spectroscopy and to see any correlations between regional saturation and serum creatinine and urine output.

Despite increased rates of survival, pediatric organ transplant is characterized by clinical complexities and psychosocial challenges. Understanding and addressing the psychosocial issues inherent in this population are crucial for optimizing their overall well-being and transplant outcomes. In this systematic review, we thus aimed to provide a comprehensive analysis of the psychosocial issues encountered by pediatric patients undergoing or awaiting organ transplant.

Although thrombocytopenia is a frequent complication that develops after liver transplantation, immune thrombocytopenia rarely occurs post-transplant. We present an 8-year-old boy who experienced 2 immune thrombocytopenia episodes that occurred 72 and 93 months after liver transplant while the patient was on tacrolimus therapy. The patient, who had primary diagnosis being deoxyguanosine kinase deficiency, received a liver transplant from his father at 1 year of age. Parents were first-degree relatives. In both episodes, the patient was admitted because of diffuse petechial lesions that developed 7 to 14 days after upper respiratory tract infection. Laboratory findings were unremarkable except for low platelet count (13 × 109/L and 6 × 109/L) and increased immature megakaryocytes in the bone marrow. The first episode responded to a mega-level dose of methylprednisolone; platelet count remained stable at >150 × 109/L until immune thrombocytopenia recurred 21 months later. The second episode was resistant to standard treatment modalities, including steroid, intravenous immunoglobulin G, and rituximab administration but responded to eltrombopag (starting at 25 mg/day and gradually increased to 75 mg/day) as a bridging regimen for partial splenic embolization and as maintenance therapy for 45 days. Platelet count has remained normal and stable over 3 years. In our literature review, we found 9 children who developed immune thrombocytopenia after liver transplant. In our patient, with response to eltrombopag as a bridging regimen for partial splenic embolization and maintenance therapy and primary liver pathology showing deoxyguanosine kinase deficiency, the posttransplant period before diagnosis of immune thrombocytopenia was longer than periods reported in the literature. These factors make our case unique. Our case highlights the need for clinicians to be aware of posttransplant immune thrombocytopenia, which can develop despite ongoing immunosuppressive therapy, and to differentiate this entity from other causes of thrombocytopenia.

Extrahepatic portal vein obstruction is the most common cause of portal hypertension in children. This study aimed to evaluate the causes, clinical, laboratory and endoscopic findings, treatment approaches, long-term results, and prognosis of extrahepatic portal vein obstruction in children.

The implantable Doppler probe can monitor the patency of vascular anastomosis, thereby facilitating early detection of vascular thrombosis critical to reducing graft loss. In this study, we examined the effectiveness of this technology compared with standard clinical care in reducing thrombosis-related graft loss and the requirement for color duplex ultrasonography in the first 24 hours postoperatively by a medical literature search of the application of this technology in kidney transplantation.

Giant cell hepatitis is an important diagnostic consideration in early childhood, especially for patients who present with jaundice. Different diseases may play a role in their etiology. In this study, we presented pediatric patients in our center diagnosed with giant cell hepatitis.

Renal transplantation offers dialysis-dependent patients a new lease on life. Compared with chronic dialysis, kidney transplant recipients benefit from a substantially improved quality and quantity of life. Despite increases in age and comorbidity among contemporary transplant recipients, recipients can expect substantial decreases in all-cause mortality, hospitalizations from infections (despite the required immunosuppression), and cardiovascular events. Invariably, the benefits of transplantation require the ongoing survival and function of the graft. Long-term graft function and patient survival are correlated with graft function in the immediate postoperative period. However, in up to 30% of cases, a wide range of clinical factors contribute to insufficient graft function, requiring transplant recipients to undergo further dialysis in a complication referred to as delayed graft function. An intermediate phenotype, slow graft function, is characterized by slower postoperative declines in creatinine without the need for dialysis and is similarly associated with long-term graft failure. These postoperative complications also confer additional costs to the health care system, with more intensive and protracted hospital stays. Delayed graft function and slow graft function continue to be major obstacles in allograft and patient survival; however, a possible contributing factor that requires further attention is perioperative sleep.