Digestive System Diseases

Symptoms and General Pathology

All Conditions

Infections

Neoplasms

Diseases and Abnormalities at or Before Birth

Immune System Diseases

Rare Diseases

Liver Cirrhosis

Hepatitis A

Hepatitis

Cholangitis

Choledochal Cyst

Cysts

Hepatitis, Autoimmune

Cholangitis, Sclerosing

Biliary Atresia

Fibrosis

Liver Diseases

Gastrointestinal Diseases

Bile Duct Diseases

Biliary Tract Diseases

Congenital Abnormalities

Digestive System Abnormalities

Hepatitis, Chronic

Autoimmune Diseases

Bile Duct Cysts

Autoimmune Hepatitis

Primary Sclerosing Cholangitis

Chronic Graft Versus Host Disease

Hematinics

All Drugs and Chemicals

Liver Extracts

The internal cohort was retrospectively enrolled in West China Hospital, Sichuan University from June 2010 and December 2020. It is a training and internal validation cohort.

The same inclusion/exclusion criteria were applied for the same center prospectively. It is a external validation cohort.

Clinical models were developed and validated to predict liver-related complications and survival outcomes in pediatric patients with biopsy-proven with cirrhosis.

Prediction model

Yuhan Yang, MD

The aim of this study was to developed and validated models to predict hepatic decompensation and survivals in pediatric patients with cirrhosis and compared these models with currently available models.

Noninvasive liver fibrosis tests such as the NAFLD fibrosis score (NFS), Hepascore, and transient elastography were specifically developed to predict fibrosis and can help predict patients with NAFLD at the highest risk of developing liver-related complications. These tests have been widely applied in adult cirrhosis. The accuracy of these models, however, may be influenced by patient factors including age, body mass index, and diabetes, potentially limiting their prognostic accuracy and clinical practicability in children. Therefore, it is currently unknown how to best predict hepatic decompensation and survival outcomes among pediatric patients with cirrhosis. To fill this knowledge gap, the investigators performed a retrospective-prospective cohort study with the aim of developing and validating a clinical model to predict liver-related complications and survival outcomes in pediatric patients with biopsy-proven with cirrhosis. Secondly, the investigators aimed to compare the predictive accuracy with currently available noninvasive model.

Predictive Models of Hepatic Decompensation and Survival Outcomes in Pediatric Patients With Cirrhosis

The primary outcome was the first event of hepatic decompensation, defined by the occurrence of any of the following: ascites (identified or confirmed by abdominal ultrasound), upper gastrointestinal bleeding secondary to portal hypertension (confirmed by endoscopy in the presence of gastroesophageal varices or portal hypertensive gastropathy), or hepatic encephalopathy (established by clinical parameters, neuropsychological tests, or electroencephalogram)

The endpoint was defined as the occurrence of death or the last follow-up. Patients were followed from the day of liver biopsy until the occurrence of death, liver transplant, or last visit. The outcome was evaluated by an experienced hepatologist in each center every 3-6 months. At each visit, a medical history, physical examination, and standard laboratory tests were performed.

West China Hospital

Associate Professor

Nutritional and Metabolic Diseases

Metabolic Diseases

Liver Neoplasms

Hepatic Insufficiency

Liver Failure

Liver Failure, Acute

Gastrointestinal Neoplasms

Digestive System Neoplasms

Acute Graft Versus Host Disease

Zhifeng Xi, MD

Qiang Xia, MD, PhD

Background: In mainland China, the development of pediatric liver transplantation (LT) has lagged behind that of adult LT during the past two decades, but it has been progressing immensely in recent years. Renji hospital(shanghai) is currently the largest pediatric transplant center in mainland China.

Aim and method: This study is performed for establishment of key techniques for pediatric LT in mainland China, including the indications and timing for pediatric LT, the criteria for donor selections, living donor LT planning, prevention and treatment for posttransplant complications, long-term follow-up management et al.

Outcome measures:

* Annual caseloads of pediatric liver transplantation
* Patient survival: 1-, 3- and 5-year patient survival after liver transplantation
* Graft survival: 1-, 3- and 5-year graft survival after liver transplantation
* Posttransplant complications, such as biliary complications, vascular complications, acute rejections, infections et al.
* Perioperative data, such as duration of operations, postoperative hospital stay et al.
* Clinical data for living donors.

Clinical and Basic Study for Pediatric Liver Transplantation

RenJi Hospital

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization	Link
Predictive Models of Hepatic Decompensation and Survival Outcomes in Pediatric Patients With Cirrhosis	NCT05181332	Cirrhosis Biliary Atresia Choledochal Cys... Primary Scleros... HBV HCV Autoimmune Hepa...	Prediction mode...	0 Years - 18 Years	West China Hospital
Clinical and Basic Study for Pediatric Liver Transplantation	NCT02503384	Biliary Atresia Metabolic Disor... Fulminant Liver... Hepatic Tumors		1 Month - 17 Years	RenJi Hospital

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