Congenital heart disease (CHD) refers to abnormal development of the heart in the foetus during the embryonic period, usually accompanied by abnormal development of the large blood vessels. The pathogenesis of CHD is complex and has not yet been elucidated. Studies have shown that the Notch signaling pathway is involved in the whole process of cardiac development, including the formation and development of the primitive heart, and the maintenance of normal cardiac function after birth, and the abnormalities in this pathway can lead to the development of CHD. In this paper, we review the mechanisms of Notch signaling pathway involved in cardiac growth and development, with a view of providing reference for the early diagnosis of CHD.

Chronic active EBV (CAEBV) infection presents with persistent or recurring IM-like symptoms including fever, hepatosplenomegaly, lymphadenopathy, and liver dysfunction without definite underlying diseases at the diagnosis. No causative pathogenetic mechanisms have been sufficiently clarified, these patients have high EBV-DNA load in the peripheral blood and systemic clonal expansion of EBV-infected T cells, NK cells and B cells. Their prognosis is poor with life-threatening complications including hemophagocytic lymphohistiocytosis, diverse organ failure, malignant lymphomas and so on. Allogeneic hematopoietic stem cell transplantation(Allo-HSCT)is the only effective therapy at present. This review outlines the recent progress of CAEBV clinical and fundamental research at home and abroad.

Objective To evaluate the clinical application of pulmonary high-resolution CT (HRCT) and radionuclide pulmonary V/Q scan in pediatric patients with bronchiolitis obliterans (BO).Methods From February 2005 to April 2011,30 BO pediatric patients (18 males,12 females,age range:7 months-14 years) in Beijing Children's Hospital Affiliated to Capital Medical University were recruited for pulmonary HRCT and radionuclide pulmonary V/Q scan.Kappa test was used to describe the consistency between "Mosaic perfusion pattern" in pulmonary HRCT and radionuclide pulmonary ventilation abnormalities.Results "Mosaic perfusion patterns" in pulmonary HRCT were showed in 30 cases (100％).Ventilation defects were found in 27 cases (90.0％) and perfusion defects in 25 cases (83.3％).The Kappa value between "Mosaic perfusion pattern" in pulmonary HRCT and pulmonary ventilation defects was 0.201 (0.1340.268),which was slightly higher than that between "Mosaic perfusion pattern" in pulmonary HRCT and pulmonary perfusion defects (0.147 (0.061-0.233)),but there was no statistical difference.Conclusions " Mosaic perfusion pattern" in pulmonary HRCT cannot distinguish between pulmonary ventilation and perfusion abnormalities.V/Q scan could be a useful tool for BO.

Objective To investigate the clinical application of radionuclide pulmonary V/Q scan in the diagnosis,evaluation of the severity and prognosis of pediatric patients with bronchiolitis obliterans (BO).Methods From February 2005 to April 2011,30 BO pediatric patients (18 males,12 females,age range:7 months-14 years) were prospectively recruited for radionuclide pulmonary V/Q scan.The relationship between the radionuclide pulmonary V/Q scan and clinical presentations was analyzed.Results Perfusion defects were seen in 25 cases (83.3％) and ventilation defects in 27 cases(90.0％).Among the 27patients with abnormal V/Q scan,matched abnormalities were seen in 13 cases (48.1％),mismatched in 1 case (3.7％) and reverse mismatched in 13 cases (48.1％).Of the 3 patients with mild disease,1 had normal V/Q scan while 2 showed V/Q defect in 1 segment.In the 10 patients with moderate disease,the mean number of segments having perfusion and ventilation defects was 3.7 and 5.6,respectively.In the 17 patients with severe disease,the mean number of segments having perfusion and ventilation defects was 6.0 and 8.2,respectively.During follow-up,all 8 patients with progressive disease presented with severe impairment of pulmonary perfusion and ventilation;while the 16 patients with improvement had mild impairment of pulmonary perfusion and ventilation or normal V/Q scan.Conclusion Radionuclide pulmonary V/Q scan is valuable for diagnosis,evaluation of the disease severity,and prognosis in pediatric patients with BO.