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

China ; Humans ; Immunologic Deficiency Syndromes ; diagnosis ; therapy

Congenital median dermoid is an uncommon disease. Surgery is the main curative treatment. To review a clinical case and to summarise the characteristics and treatment experience of this disease, referring to the related literature, it is expected that we can provide more clinical thought and therapeutic method for congenital median dermoid fistula of nasal dorsum.
Dermoid Cyst ; congenital ; therapy ; Fistula ; congenital ; therapy ; Humans ; Nose ; pathology ; Nose Neoplasms ; congenital ; therapy

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

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