Autoimmune Diseases ; Child ; Humans

The Wiskott-Aldrich syndrome(WAS)is a rare X-linked recessive immunodeficiency disorder characterized by thrombocytopenia and small platelets,eczema,recurrent infections,and increased risk for autoimmunity and malignancy.The gene responsible for this syndrome is WAS protein(WASP)gene.Six mutational hotspots have been identified now.Flow cytometric analysis of the WASP expression in lymphocytes is simple,rapid,and applicable for screening of WAS.WASP gene sequencing analysis can confirm the diagnosis.Hemato-poietic stem cell transplantation(HSCT)is the mainstay of treatment for WAS.So far more than 40 WAS patients have been diagnosed in China.Due to the few reports and varied clinical manifestations,WAS is often misdiagnosed as idiopathic thrombocytopenic purpura.This review will focus on recent progress in understanding the clinical presentations associated with mutations of the WASP gene,the genotype-phenotype correlation and management of this lethal disease.

Humans ; Mutation ; Wiskott-Aldrich Syndrome ; genetics ; Wiskott-Aldrich Syndrome Protein ; genetics

Animals ; Astragalus membranaceus ; chemistry ; CD4-CD8 Ratio ; Carcinoma, Lewis Lung ; drug therapy ; pathology ; Cell Line, Tumor ; Drugs, Chinese Herbal ; administration & dosage ; therapeutic use ; Female ; Injections ; Mice ; Mice, Inbred C57BL ; Neoplasm Transplantation ; Plants, Medicinal ; chemistry

Objective To investigate the mechanisms of attachment of respiratory syncytial virus(RSV) to human airway epithelial cells.Methods Attachment of RSV to CFBE was quantitatively assessed by flow cytometry.Various polyclonal and monoclonal antibodies to RSV and heparin were pre-incubated with RSV.The blocking effects of these antibodies and heparin on attachment were evaluated.Results CFBE cells reduced capacity being bound by RSV.All the antibodies used were failed to block attachment of RSV on CFBE,whereas heparin blocked RSV attachment in a dose-response manner and the blokade by heparin was almost complete at the concentration of 0.4 mg/L.Conclusions Flow cytometry provides direct evaluation of attachment without growth of virus.Heparin-like molecules on cell surface of CFBE appears to be involved in attachment between RSV and human epithelial cells.

China ; Humans ; Immunologic Deficiency Syndromes ; diagnosis ; therapy

Child ; China ; epidemiology ; Female ; Human bocavirus ; Humans ; Infant ; Infant, Newborn ; Male ; Parvoviridae Infections ; epidemiology ; virology ; Respiratory Tract Infections ; epidemiology ; virology

Adjuvants, Immunologic ; administration & dosage ; adverse effects ; Child ; Humans ; Immunosuppressive Agents ; administration & dosage ; adverse effects ; Pediatrics ; Practice Guidelines as Topic ; Treatment Outcome