Wilson disease（WD）is an inherited disorder caused by mutations in the ATP7B gene.It is characterized by pathological accumulation of copper in the organs throughout the body，especially the liver and brain. This article reviews the latest progress of existing and emerging therapies. At present，the most commonly used treatment methods are oral chelators of copper and zinc agents. Liver transplantation can be used as a treatment for end-stage patients. However，these treatment methods have some shortcomings：many side effects of drugs，poor treatment effect，lifelong treatment，poor patient compliance and so on. At present，the choice of drugs is still in the exploratory stage，and there are no guidelines to specify which patients should receive which treatment. In addition，there are many emerging therapies in different experimental stages，such as new chelators of copper，targeted molecular therapy，gene therapy and cell transplantation. The old methods have many disadvantages and lack large controlled clinical trials，and emerging research is still in its infancy，which are urgent questions.

Infliximab（IFX）is one of anti-tumor necrosis factor-α（anti-TNF-α）and is currently biological preparations with indications for the use of inflammatory bowel disease（IBD）of children in China.With the clinical application of IFX，the treatment goal of IBD has shifted from clinical remission to mucosal healing，thereby further improving the prognosis of the disease.However，in clinical work，it has been found that some children have primary or secondary unresponsiveness to IFX treatment，which is a major challenge during the IFX treatment process.Research has found that this phenomenon may be related to a decrease in blood drug concentration and the production of anti drug antibodies.Therefore，IFX therapeutic drug monitoring（TDM）is very important in the treatment of pediatric IBD.This article reviews the current application status of TDM in the treatment of IBD in children with IFX，and puts forward prospects for future research directions of TDM.

Biliary atresia（BA）is the most common cause of liver disease in children leading to liver transplantation.Kasai operation is the main treatment method.Earlier diagnosis and younger age of operation can greatly improve the prognosis.Although the diagnosis and treatment of BA have been gradually improved in recent years，some patients still need liver transplantation or even die due to cirrhosis.There is still a lack of non-invasive prognostic evaluation methods.This article reviews the value of serum matrix metalloproteinase-7（MMP-7）level in the early diagnosis of biliary atresia，and discusses the relationship between MMP-7 level and the prognosis of children with biliary atresia，and how MMP-7 participates in the mechanism of the occurrence and development of biliary atresia，so as to provide reference for clinical diagnosis and treatment.

Coagulopathy in patients with liver disease is considered to be a fragile "rebalancing" state.Conventional coagulation test commonly used in clinic can not reflect the whole process of coagulation, so its application is limited.In recent years, thromboelastography has developed rapidly as a rapid and real-time detection method to evaluate the coagulation status of whole blood, and has unique advantages in the application of liver disease.The article reviews the application in transfusion guidance of thromboelastography and its association with severity of liver disease, bleeding, thrombosis, mortality and conventional coagulation testing.Although thromboelastography has some shortcomings, it can better reflect the real coagulation state of patients with liver disease and help reduce the use of blood products.Pediatric data are scarce, but the use of thromboelastography alone or in combination with conventional coagulation test will have broad prospects.

Matrix metalloproteinases(MMPs)are a large family of zinc-dependent endopeptidases, which are mainly synthesized by connective tissues, it can degrade the extracellular matrix(ECM)and basement membrane, affect the regeneration and reconstruction of normal tissues, and participate in the pathological process of malignant tumors.Matrix metalloproteinase-7(MMP-7)is the smallest member of the metalloproteinase family.It is expressed in many tissues of the body, such as thyroid, breast, lung, digestive tract, reproductive system and hepatobiliary system.In recent years, the expression of MMP-7 in hepatobiliary diseases has attracted more and more attention.MMP-7 is not only involved in the growth, metastasis and invasion process of hepatobiliary malignant tumors, but also highly expressed in liver fibrosis, biliary atresia and other diseases.This paper reviews the expression of MMP-7 in the above diseases.

Tumor-related diarrhea is a rare cause of chronic diarrhea in children, which is usually one of the clinical manifestations of specific functional syndrome of neuroendocrine neoplasms.Diarrhea may appear as the initial symptom or accompany symptom of disease progression, and it is generally relieved or cured after surgical resection.The present review mainly introduces several common neuroendocrine neoplasms which cause chronic diarrhea in children, and summarizes their different diarrhea features and mechanisms, as well as key points in diagnosis and treatment, in order to improve the rate of early diagnosis and prognosis of children.

Inflammatory bowel disease(IBD) is a complex multifactorial disease characterized by chronic recurrent intestinal inflammation.The etiology of IBD has not yet been determined, and relevant studies have focused on the genetic susceptibility, immune response, intestinal microbiome and environmental factors of the host.Studies have found that interleukin-10 receptor A(IL-10RA)plays important roles in the pathogenesis of IBD.IL-10RA can not only affect the intestinal barrier, but also affect the intestinal immune system.Furthermore, the mutation of IL-10RA itself is highly correlated with very early-onset IBD.

Inflammatory bowel disease is a chronic and nonspecific intestinal disease that is mainly treated with drugs.At present, Infliximab (IFX) is the only biological agent applied for children with inflammatory bowel disease in China.It is essentially a biosynthetic chimeric monoclonal antibody, and can be combined with tumor necrosis factor-alpha(TNF-α) on T cells inhibition of inflammatory response through cytotoxicity and induction of lymphocyte apoptosis.IFX can induce remission in the short term and maintain remission in the long term.It can also promote mucosal repair, improve the quality of life and reduce complications.Clinical studies revealed that there were a proportion of patients who respond poorly or ineffectively to IFX treatment.Furthermore, due to the high cost of biological agents, bio generic drugs are gradually starting to be developed and applied.This article provided an overview of the application of IFX on inflammatory bowel disease in children.

Progressive familial intrahepatic cholestasis (PFIC) is a rare heterogenous groups of autosomal recessive monogenic disease, resulting in abnormal bile acid formation and secretion.According to the type of gene mutation, PFIC was classified as PFIC 1-6 and more new mutated genes have been identified, such as ABCC12, VPS33B, et al, making diagnosis and treatment more accurate.PFIC is characterized by progressive jaundice and pruritus, growth and development disorders, and fat-soluble vitamin deficiency.Except for PFIC3, the level of γ-glutamyl transpeptidase in serum of PFIC is normal, which is an important clue for clinical diagnosis.However, each type of PFIC has its own characteristics such as the age of onset, disease severity, extrahepatic symptoms, and prognosis.With the progress of the disease, it eventually develops into fibrosis, cirrhosis and liver failure.Treatment includes nutrition, medication, partial external/internal biliary diversion, and liver transplantation.Recent studies on transporters and gene therapy have brought new hope to patients with PFIC.

Acute pancreatitis in children is a common acute and critical disease in clinical practice,and its incidence is increasing.The motality of moderate severe acute pancreatitis or severe acute pancreatitis is up to 10％-15％.Proper nutrition support can reduce the infection rate and mortality and improve the disease outcome under the condition of high metabolism and negative nitrogen balance,especially early enteral nutrition can benefit the patients significantly.Proper nutrition support methods and approaches,proper enteral nutrition initiation timing,optimized enteral nutrition formulations and quantity are all the hot spots at present.