• Hepatorenal transplant
• Kidney transplant
• Liver-kidney
• Pediatrics

• combined liver–kidney transplantation
• kidney transplant
• liver transplant
• postoperative complications
• simultaneous organ transplantation
• transplant outcomes

• Nephrectomy
• Primary hyperoxaluria
• Sequential liver-kidney transplantation
• Simultaneous liver-kidney transplantation

• Allocation
• Heart-kidney transplantation
• Kidney-pancreas transplantation
• Liver-kidney transplantation
• Multi-organ transplantation
• Pediatric

• Humans
• Child
• Kidney Transplantation/adverse effects
• Organ Transplantation/adverse effects
• Multiple Organ Failure/etiology
• Graft Rejection/prevention & control
• Graft Rejection/etiology
• Graft Rejection/immunology

• Humans
• Liver Transplantation/standards
• Germany
• Gastroenterology/standards
• Practice Guidelines as Topic
• Societies, Medical

• heart transplant
• kidney transplant
• liver transplant
• multi-organ transplant
• paediatric

• Humans
• Quality of Life
• Child
• Male
• Female
• Graft Survival
• Child, Preschool
• Adolescent
• Organ Transplantation
• Cohort Studies
• Infant
• Kidney Transplantation
• Treatment Outcome
• Graft Rejection
• Registries
• Liver Transplantation
• United Kingdom
• Surveys and Questionnaires

• Biological sciences
• Cell biology
• Molecular genetics

• enzyme defect
• living donor
• metabolic liver disease
• renal replacement therapy
• sequential liver-kidney transplantation

• Adult
• Child
• Humans
• Kidney Transplantation/methods
• Liver Transplantation/methods
• Treatment Outcome
• Kidney Diseases
• Liver Failure/surgery
• Hypertension, Portal
• Kidney

• autosomal recessive polycystic kidney disease
• children
• isolated
• kidney transplantation

Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive disease stemming from a deficiency in liver-specific alanine-glyoxylate aminotransferase, resulting in increased endogenous oxalate deposition and end-stage renal disease. Organ transplantation is the only effective treatment. However, its approach and timing remain controversial.

We retrospectively analyzed 5 patients diagnosed with PH1 from the Liver Transplant Center of the Beijing Friendship Hospital from March 2017 to December 2020. Our cohort included 4 males and 1 female. The median age at onset was 4.0 years (range: 1.0-5.0), age at diagnosis was 12.2 years (range: 6.7-23.5), age at liver transplantation (LT) was 12.2 years (range: 7.0-25.1), and the follow-up time was 26.3 mo (range: 12.8-40.1). All patients had delayed diagnosis, and 3 patients had progressed to end-stage renal disease by the time they were diagnosed. Two patients received preemptive LT; their estimated glomerular filtration rate was maintained at > 120 mL/min/1.73 m², indicating a better prognosis. Three patients received sequential liver and kidney transplantation. After transplantation, serum and urinary oxalate decreased, and liver function recovered. At the last follow-up, the estimated glomerular filtration rates of the latter 3 patients were 179, 52 and 21 mL/min/1.73 m².

Different transplantation strategies should be adopted for patients based on their renal function stage. Preemptive-LT offers a good therapeutic approach for PH1.

• Primary hyperoxaluria type 1
• Liver transplantation
• Combined liver and kidney transplantation
• Sequential liver and kidney transplantation
• Renal calculi
• End-stage renal disease
• Case reports

• Infant
• Humans
• Female
• Hyperoxaluria, Primary/diagnosis
• Kidney
• Kidney Transplantation/adverse effects
• Kidney Failure, Chronic/complications

Both Caroli disease (CD) and autosomal recessive polycystic kidney disease (ARPKD) are autosomal recessive disorders, which are more commonly found in infants and children, for whom surviving to adulthood is rare. Early diagnosis and intervention can improve the survival rate to some extent. This study adopted the case of a 26-year-old pregnant woman to explore the clinical and imaging manifestations and progress of CD concomitant with ARPKD to enable a better understanding of the disease.

A 26-year-old pregnant woman was admitted to our hospital for more than 2 months following the discovery of pancytopenia and increased creatinine. Ultrasonography detected an enlarged left liver lobe, widened hepatic portal vein, splenomegaly, and dilated splenic vein. In addition, both kidneys were obviously enlarged and sonolucent areas of varying sizes were visible, but color Doppler flow imaging revealed no abnormal blood flow signals. The gestational age was approximately 25 weeks, which was consistent with the actual fetal age. Polyhydramnios was detected but no other abnormalities were identified. Magnetic resonance imaging revealed that the liver was plump, and polycystic liver disease was observed near the top of the diaphragm. The T1 and T2 weighted images were the low and high signals, respectively. The bile duct was slightly dilated; the portal vein was widened; and the spleen volume was enlarged. Moreover, the volume of both kidneys had increased to an abnormal shape, with multiple, long, roundish T1 and T2 abnormal signals being observed. Magnetic resonance cholangiopancreatography revealed that intrahepatic cystic lesions were connected with intrahepatic bile ducts. The patient underwent a genetic testing, the result showed she carried two heterozygous mutations in PKHD1. The patient was finally diagnosed with CD with concomitant ARPKD. The baby underwent a genetic test three months after birth, the result showed that the patient carried one heterozygous mutations in PKHD1, which indicated the baby was a PKHD1 carrier.

This case demonstrates that imaging examinations are of great significance for the diagnosis and evaluation of CD with concomitant ARPKD.

• Autosomal recessive polycystic kidney disease
• Caroli disease
• Diagnosis
• Magnetic resonance imaging
• Ultrasound

• Liver transplantation
• children
• hepatic neoplasms
• liver graft
• living-donor liver transplantation
• organ shortage.

• Atypical hemolytic uremic syndrome
• Combined liver-kidney transplantation
• Donor-specific antibodies
• Glycogen storage diseases
• Hereditary complement C3 deficiency
• Homozygous protein C deficiency
• Methylmalonic aciduria
• Primary hyperoxaluria
• Sensitization

Combined liver-kidney transplantation (CLKT) is a rarely performed complex surgical procedure in children and involves transplantation of kidney and either whole or part of liver donated by the same individual (usually a cadaver) to the same recipient during a single surgical procedure. Most common indications for CLKT in children are autosomal recessive polycystic kidney disease and primary hyperoxaluria type 1. Atypical haemolytic uremic syndrome, methylmalonic academia, and conditions where liver and renal failure co-exists may be indications for CLKT. CLKT is often preferred over sequential liver-kidney transplantation due to immunoprotective effects of transplanted liver on renal allograft; however, liver survival has no significant impact. Since CLKT is a major surgical procedure which involves multiple and complex anastomosis surgeries, acute complications are not uncommon. Bleeding, thrombosis, haemodynamic instability, infections, acute cellular rejections, renal and liver dysfunction are acute complications. The long-term outlook is promising with over 80% 5-year survival rates among those children who survive the initial six-month postoperative period.

• Combined liver-kidney transplantation
• Immunoprotection
• Long-term outcomes
• Renal allograft survival
• Acute cellular rejection
• Autosomal recessive polycystic kidney disease

Primary hyperoxaluria type 1(PH 1) is the most common indication for a paediatric combined liver-kidney transplant. It is a technically challenging procedure. We describe the challenges in managing a 21-month-old female child weighing 7 kg for a combined liver-kidney transplant from two related living donors.

• Anesthesia for combined liver and kidney transplant
• combined liver-kidney transplantation
• primary hyperoxaluria type 1 (PH 1)

• autosomal recessive polycystic kidney disease
• cholangitis
• liver-kidney transplantation
• pancytopenia
• splenectomy

Non-invasively monitoring allogeneic graft rejection with a specific marker is of great importance for prognosis of patients. Recently, data revealed that IL-27Rα was up-regulated in alloreactive CD4⁺ T cells and participated in inflammatory diseases. Here, we evaluated whether IL-27Rα could be used in monitoring allogeneic graft rejection both in vitro and in vivo. Allogeneic (C57BL/6 donor to BALB/c recipient) and syngeneic (BALB/c both as donor and recipient) skin grafted mouse models were established. The expression of IL-27Rα in grafts was detected. The radio-probe, ¹²⁵I-anti-IL-27Rα mAb, was prepared. Dynamic whole-body phosphor-autoradiography, ex vivo biodistribution and immunofluorescence staining were performed. The results showed that the highest expression of IL-27Rα was detected in allogeneic grafts on day 10 post transplantation (top period of allorejection). ¹²⁵I-anti-IL-27Rα mAb was successfully prepared with higher specificity and affinity. Whole-body phosphor-autoradiography showed higher radioactivity accumulation in allogeneic grafts than syngeneic grafts on day 10. The uptake of ¹²⁵I-anti-IL-27Rα mAb in allogeneic grafts could be almost totally blocked by pre-injection with excess unlabeled anti-IL-27Rα mAb. Interestingly, we found that ¹²⁵I-anti-IL-27Rα mAb accumulated in allogeneic grafts, along with weaker inflammation earlier on day 6. The high uptake of ¹²⁵I-anti-IL-27Rα mAb was correlated with the higher infiltrated IL-27Rα positive cells (CD3⁺/CD68⁺) in allogeneic grafts. In conclusion, IL-27Rα may be a novel molecular imaging marker to predict allorejection.

• IL-27Rα 1
• allorejection 2
• autoradiography imaging 3
• biomarker 4
• radioiodine 5

In conditions of limited experience of pediatric simultaneous liver‐kidney transplantation (SLKT) using grafts from living and deceased donors, there is a certain need to validate the approach.

The retrospective study of 18 pediatric patients who received SLKT between 2008 and 2019.

Grafts were obtained from both living and deceased donors. The patients’ age ranged from 2 to 16 years (9 years ±4). The body weight of the children varied from 9.5 to 39 kg (22 kg ±9). The follow‐up period lasted from 1 to 109 months (median 38 months ±35). The various graft combinations were used in both groups. There was no mortality during the follow‐up. There was no significant difference in baseline parameters in recipients who received grafts from living and deceased donors except age (7.5 years ±2.2 vs 11.8 years ±4.1; P = .038). Rate of complications > grade II was higher among recipients of deceased donor SLKT (7.7% vs 60%; OR, 7.8; 95% CI, 1.04‐58.48; P = .044). All the patients are alive with both grafts functioning. All the living donors returned to the normal life.

SLKT is a safe and effective procedure for children with both simultaneous end‐stage liver disease and end‐stage renal disease. Both living donor partial liver and kidney transplantation and deceased donor liver‐kidney transplantation can be considered as safe and feasible options.

• autosomal recessive polycystic kidney disease/congenital hepatic fibrosis
• living donor
• long-term outcomes
• simultaneous liver-kidney transplantation
• split-liver transplantation

• autosomal recessive polycystic kidney disease
• endoscopic variceal ligation
• liver fibrosis
• molecular confirmation
• portal hypertension
• transient elastography

• Doppler ultrasound
• combined liver-kidney transplantation
• hepatic flow velocity
• liver transplantation
• pediatric

• ABO incompatibility
• clinical research/practice
• kidney transplantation/nephrology
• kidney transplantation: living donor
• liver transplantation/hepatology
• liver transplantation: living donor
• minimally invasive surgery
• pediatrics

• ABO Blood-Group System
• Adult
• Child, Preschool
• End Stage Liver Disease/complications
• End Stage Liver Disease/surgery
• Female
• Hepatectomy/methods
• Humans
• Kidney Transplantation/methods
• Laparoscopy/methods
• Liver Transplantation/methods
• Living Donors
• Male
• Nephrectomy/methods
• Polycystic Kidney, Autosomal Recessive/complications
• Polycystic Kidney, Autosomal Recessive/surgery
• Tissue and Organ Harvesting/methods

• Primary hyperoxaluria
• kidney transplantation
• liver transplantation
• living donor

• cystatin C
• eGFR
• hyperammonemia
• kidney disease
• methylmalonic academia

• 4-hydroxy-2-oxoglutarate aldolase enzyme
• Primary hyperoxaluria type 1 (PH1)
• combined liver-kidney transplantation
• end-stage kidney disease (ESKD)
• glyoxylate reductase
• hydroxypyruvate reductase
• liver-specific alanine-glyoxylate aminotransferase

• Cardiovascular risk
• Endothelium
• Hyperoxaluria type I
• Microcirculation
• Renal transplantation