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

BACKGROUND: The grafted human hair keratin(HHK) artificial tendon,being degradable and absorbable, can induce formation of neotendon. In the present study, the main biomechanical indexes of HHK artificial tendon and its components were measured so as to determine a proper ratio for the artificial tendon composition according to different needs of the body sites and make it possess tensile stress required for functional activities.OBJECTIVE: To determine the main biomechanical indexes of HHK artificial tendon and its components.DESIGN: An observational study based on HHK artificial tendon.SETTING: Department of Biochemistry and Molecular Biology, Medical Biomechanical Key Laboratory of Chinese PLA, and Department of Histology and Embryology, a military medical university; Foshan Kangxing Medical Technology Co., Ltd.MATERIALS: The experiment was completed at the Medical Biomechanical Key Laboratory of the First Military Medical University of Chinese PLA in October 2001. The material of HHK artificial tendons was uncontaminated black hair collected from healthy young women in remote mountainous areas free from endemic diseases. There were three components of HHK artificial tendons with different in vivo absorption velocities Z, B and F. The in vivo absorption was the slowest for Z, faster for B, and the fastest for F. Different kinds of HHK artificial tendon were the mixture of Z, B and F at a proper ratio. Each group had 3 kinds, namely, Z3.0-20, B3.0-20, and F3.0-20. HHK artificial tendon consisted of 6 kinds: ZBF1. 0-20,ZBF1.5-20, ZBF3.0-20, ZBF6. 0-20, ZBF9. 0-20, and ZBF12.0-20.METHODS: Both ends of an HHK artificial tendon or each of its components were fixed on MTS 858 Moni Bionix checker to determine such biomechanical indexes as fracturing pull and tensile stress.MAIN OUTCOME MEASURES: Fracturing pull and tensile stress of HHK artificial tendon of different kinds and its components.RESULTS: Fracturing pull in Z3.0-20, B3.0-20 and F3.0-20 groups was (211.8 ± 12. 1 ), ( 178.8 ± 8.2), and ( 151.3 ± 6. 7) N, respectively; tensile stress was(71.3 ±3.9), (59.9 ±2. 7), and(50. 3 ±2.3) Mpa, respectively. Fracturing pull and tensile stress were decreased gradually in the same cross-section area of the 3 kinds of materials; Fracturing pull in ZBF1.0-20,ZBF1.5-20, ZBF3.0-20, ZBF6.0-20, ZBF9.0-20, and ZBF12.0-20 groups was(75.0 ± 3.0), (107.8±4.2), (194.1±5.3), (375.9±12.7),(508.2 ±21.3), and(670.8 ±25.4) N, respectively, while tensile stress was(75.0 ±3.0), (72.0 ±2.8), (65.1 ± 1.8), (62.9 ±2.2), (56. 3 ±2.4),and(55.8 ± 1.5) Mpa, respectively.CONCLUSION: Fracturing pull of HHK artificial tendon is increased with the increase of cross-section area, whereas tensile stress is slightly decreased with the increase of cross section area. With the increased extent of human hair treatment, its absorption in vivo is accelerated, and fracturing pull and tensile stress of HHK artificial tendon are decreased.

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

China ; Humans ; Immunologic Deficiency Syndromes ; diagnosis ; therapy