It is an important turning point that early diagnosis and prompts initiation of adequate treatment improve the outcomes of primary or secondary hemophagocytic lymphohistiocytosis (HLH) which is a rare life-threatening syndrome.Even though the survival rate of the patients with HLH has been improved because of the immunochemotherapy or targeted immunotherapy based on new findings in the immunopathogenesis and the genetic defects,reducing the mortality will be main challenge for the pediatricians in pediatric intensive care unit.All of the clinical features are significant clues for the diagnosis,including continuing fever,specific liver dysfunction with coagulation abnormalities,pancytopenia,hypertriglyceridemia and hyperferritinemia.Defining patients' natural killer cells status during the different stages of sepsis and autoimmune diseases will be guiding innovative immunointerventions.

Child ; Dermatomyositis ; diagnosis ; pathology ; therapy ; Diagnosis, Differential ; Electromyography ; Glucocorticoids ; administration & dosage ; Humans ; Immunosuppressive Agents ; administration & dosage ; Magnetic Resonance Imaging

Child ; Humans ; Macrophage Activation Syndrome ; diagnosis

This article mainly discusses the relationship between the active treatment concept of the systemic lupus erythematosus (SLE) and its positive outcome in long-term prognosis,which is the overall prognosis of SLE benefits from the improvement on the liying conditions of patients with lupus nephritis (LN).Although more argumentations are still required in regards to whether to use the different general conclusions of the meta analysis between the childhood-onset SLE (cSLE) and the early adulthood-onset SLE patients at the same age as more proactive induction therapy in early phase,yet the time point for early therapy before clinical symptoms appearing can be determined and thereby positive anticipation can be achieved through keeping the difference between the long-term prognosis of the cSLE and the adult-onset patients as evidences,and making in-depth study on the innate laws of the heterogeneity of cSLE,meanwhile giving biological markers more application which can be highly reliable in reflecting the morbid status,supported by the study outcomes of pharmacogenetics/pharmacogenomics and further optimization of current immunoregulation/immune inhibitors approaches (as evidences required by long-term effects).

The common mucosal immunological system is an important anatomical basis of the allergic disorders.Its function is not only regulated by the susceptibility gene,but also affected the development because of the early life exposure to antibiotics.It is the tangible that early life antibiotic-driven changes in mucosal immune enhance susceptibility to allergic disorders of respiratory system.A holistic approach to study the mucosal immune system as an integrated global organ is imperative for future advances in understanding mucosal immunology and for future treatment of the allergic disorders.This review will be focus on the latest evidence and the mechanisms in driving the lung-gutcross-talk.

As the hallmark sequelae of juvenile dermatomyositis(JDM),the calcinosis still bothers approxi-mately one third of the patients,but it was considered a good prognostic sign. Today,however,it is universally acknow-ledged that the calcinosis is instead deemed a marker of the special state and inappropriate treatment for JDM. The treatment of based on disease process,including the biological agents and other immunomodulator,which have been not the radical therapy. However,it is a crucial to alleviate the calcinosis that controlling the long - term chronic inflamma-tion for JDM.

Good standardized training of resident physicians is indispensable for high quality medical personnel building.This paper gives a brief introduction to the experiences of endocrinology fellowship trainings in three top hospitals in USA.On the basis of current simation in China,a new and improve the quality'is proposed,and a new approach is explored,which is a valuable reference for the endocrine resident trainings in China.

Objective To draw up a satisfaction rating scale utilized by outpatients in general hospitals,for the quality evaluation of the out-patient service.Methods Outpatients at four municipal general hospitals in Hangzhou City were as the subjects.The validity and reliability of the rating scale were estimated.Results The response rate to items was 98.82% ,and valid rate reached 96.8% ,the internal consistency coefficient was in the range of 0.76 to 0.87.The composition of the scale conforms well to the management practice,the relativity inside the factor was stronger than the relativity among the factors,which supported the contents validity.Factor analysis results demonstrated that both the factor loads and structures were consistent with the structures of the rating scale,which supported the structure validity.Conclusion All of validity indexes of the rating scale meet the requirements of psychometrics.This scale is practicable to estimate the satisfaction of outpatients to the service they received,and provides valuable evidence to estimate the quality of hospital therapeutic services.

Protein degradation mediated by ubiquitin-proteasome pathway is involved in many biological processes.SCF complex is a sort of important E3 ubiquitin-ligase enzyme.Fbx4 protein is one of F-box protein family members,an important component of substrate-specific ScF Fbx4/αβ-crystallin ligase,and a subunit of SCF complex.Inactivation of Fbx4 protein or alteration of its expression level will influence activity of SCF Fbx4/αβ-crystallin ligase,thus will influence expression level of Cyclin D1.And Fbx4 protein is also an important regulatory factor for telomere maintenance.

Glutathione ; metabolism ; Humans ; Lipoxygenase ; metabolism ; Oxidation-Reduction ; Xenobiotics ; metabolism