Advances in the pathogenesis and treatment of infantile liver failure syndrome-2 / 国际儿科学杂志

Infantile liver failure syndrome-2(ILFS2)is a rare autosomal recessive disorder caused by neuroblastoma amplified sequence(NBAS)gene mutation, manifested as recurrent acute liver failure(ALF)with fever/infection-related pathogenesis.First-onset ALF is common in infants or early childhood(8 months to 3 years of age). The main characteristic of this disease is that the liver function can be recovered completely in the interval, and the definitive diagnosis is based on the identification of NBAS gene mutation in gene analysis.Until now, the pathogenesis of ILFS2 is not yet fully understood.Patients can be treated by supportive treatment clinically, while liver transplantation is the only treatment option currently available for patients with end-stage ALF.This review will focus on the recent progress in the pathogenesis and treatment of ILFS2.

Advances in the effect and mechanism of Faecalibacterium prausnitzii on immune regulation of inflammatory bowel disease / 国际儿科学杂志

Inflammatory bowel disease is a common digestive tract disease, and its incidence rate is increasing year by year.Changes of intestinal microecology in patients with inflammatory bowel disease are mainly manifested by the decreased species richness of intestinal microorganisms, among which the significant reduction of Faecalibacterium prausnitzii is one of its important characteristics.In addition, Faecalibacterium prausnitzii participates in the immune regulation of inflammatory bowel disease and plays an important role in the occurrence and development of inflammatory bowel disease.In this article, we reviewed the role and mechanism of Faecalibacterium prausnitzii involved in the immune regulation of inflammatory bowel disease by inhibiting inflammatory pathways, regulating the differentiation and function of Treg cells, and repairing intestinal mucosal barrier.By summarizing the specific mechanisms and roles of Faecalibacterium prausnitzii in the intestine, we hope to provide a basis for clinical treatment and research of inflammatory bowel disease.

Research advances in animal models of cow's milk protein allergy / 国际儿科学杂志

Cow's milk protein allergy (CMPA) is the most common food allergy among infants and young children,which has an increasing incidence in recent years.Performing suitable animal experiments is a key to study CMPA,but currently there aren't proper models consistent with human physiological sensitization process.Ideal animal models can serve as critical tools to reveal the mechanisms of CMPA and develop preventive and therapeutic approaches.Animal species and strains,different types of the models,biological detection methods and evaluation are reviewed in this paper.It also summarizes characteristics of animal models,which will provide a reference for basic and applied researches of CMPA.