Research advance in autosomal recessive polycystic kidney disease / 临床儿科杂志

Autosomal recessive polycystic kidney disease (ARPKD) had a low incidence, and mainly in neonates or infants. It is caused by mutations of the polycystic kidney and hepatic disease 1 gene (PKHD1). The pathogenesis of ARPKD is still not clear. The principal of treatment is focused on the control of complications and slow down the progression. In this article, the research advances in the pathogenesis and treatment of ARPKD was reviewed.

Regulation of CD4 + T cell subsets on pathogenic mechanism in children with Henoch - Schonlein purpura / 中华实用儿科临床杂志

Objective To explore the alternations of CD4 + T cell subsets and their cytokines and transcription factors in children with Henoch - Schonlein purpura(HSP). Methods Forty - one children were enrolled in this stud-y,including 41 in acute stage of HSP,35 cases of HSP in clinical remission stage and 30 healthy children as healthy control group. The percentages of helper T lymphocytes(Th)1,Th2,regulatory T cells(Treg)and Th17 subsets were determined by flow cytometry(FCM). The concentrations of plasma interferon - γ( INF - γ),interleukin( IL) - 4, transforming growth factor - β1(TGF - β1 )and IL - 17 were examined by ways of enzyme - linked immunosorbent assay(ELISA). The expressions of T - bet,GATA3,Foxp3 and ROR - γt mRNA in peripheral blood mononuclear cells were examined by means of reverse transcription - polymerase chain reaction(RT - PCR). Results (1)During the a-cute and recovery stage of HSP,compared with the control group,the percentages of Th2 and Th17 cell subsets,the le-vels of plasma IL - 4 and IL - 17,and the expressions of GATA3 and ROR - γt mRNA were significantly higher(all P ﹤ 0. 05). The percentages of Th1 and Treg cell subsets,the levels of plasma INF - γ and TGF - β1 and the expres-sions of T - bet and Foxp3 mRNA were lower(all P ﹤ 0. 05). The imbalances of Th1 / Th2 and Treg/ Th17 appeared during the acute stage of HSP.(2)During the recovery stage of HSP,the percentages of Th1,Th2,Th17 and Treg,and the ratio of Treg / Th17 were of no difference compared with those in acute stage( all P ﹥ 0. 05). The ratio of Th1 / Th2,and the expressions of T - bet and Foxp3 mRNA were increased(all P ﹤ 0. 05),while the levels of plasma IL - 4 and IL - 17,the expressions of GATA3 and ROR - γt mRNA were decreased(all P ﹤ 0. 05)compared with those in acute stage. In the recovery stage of HSP,the imbalances of Th1 / Th2 and Treg/ Th17 were still obvious .(3)There was a positive correlation in every 2 cytokines of Th1,INF - γ and T - bet. And the same correlation existed in Th2, IL - 4 and GATA3,in Treg,TGF - β1 and Foxp3,and in Th17,IL - 17 and ROR - γt(P ﹤ 0. 05). Conclusions The imbalance of Th1 / Th2 and Treg/ Th17 is critical in pathological mechanism of HSP. The disturbance of immune tole-rance induced by Treg cells is important in HSP.

Intestinal microbiota and children gastrointestinal diseases / 中华实用儿科临床杂志

The microbiota of the human gastrointestinal tract inhabits a complex ecosystem.Intestinal normal flora is obtained by newborn after birth,and suffers influences on the type of delivery,the type of feeding and contamination from the environment.There is emerging evidence indicating that quantitative and qualitative changes on gut microbiota contribute to alterations in the mucosal activation of immune system leading to intra-or extra-intestinal diseases.A balance between pathogenic and beneficial microbiota throughout childhood is important to gastrointestinal health,including protection against pathogens,inhibition of pathogens,nutrient processing,and regulation of host fat storage.Probiotics can promote an intentional modulation of intestinal microbiota favoring the health of the host.This paper is a review about modulation of intestinal microbiota on prevention and adjuvant treatment of intra-and extra intestinal pediatric diseases.