ABSTRACT
Clinical data of 2 children with cystic fibrosis (CF) and Pseudomonas aeruginosa infection in the Department of Pediatrics of Sichuan Provincial People′s Hospital from April 2018 to June 2019 were retrospectively analyzed.Patient 1 was an 11-year-old girl with no history of recurrent respiratory infections. Pseudomonas aeruginosa was found in the first sputum culture.A large number of yellow and white secretions were visible under repeated fiberoptic bronchoscopy.Chest CT showed multiple spots and tree-in-bud signs around the bronchi of both lungs. CFTR gene test results revealed 3 heterozygous mutations: c.2909G>A (chr7: 117246728), c.*133T>A (chr7: 117307295) and c. *125delT (chr7: 117307285). The other patient was a 7-year-old boy with a history of recurrent respiratory infections.His parents were close relatives.Multiple cultures of sputum and bronchoalveolar lavage fluid of the boy were Pseudomonas aeruginosa, and chest CT suggested dilation and inflammation in bronchi of both lungs.Gene detection showed that the c. 380T>G homozygous mutation at chromosome chr7-117171059 resulted in an amino acid change p. leu127stop (nonsense mutation). This article suggests that CF should be considered for Pseudomonas aeruginosa infected children having signs of bronchiectasis on chest CT and a large number of secretions under bronchoscopy.Besides, it is necessary for such kind of children to perform genetic testing in time to confirm the diagnosis as soon as possible.
ABSTRACT
Objective To analyze comparatively the outcome of modified Manipal and DeVega tricuspid annuloplaaty.Methods From Oct.2001 to Aug.2004,the consecutive 123 patients operated with modified Manipal tricuspid annuloplasty for tricuspid re- gurgitation at the time of left cardiac valve replacement(group A)were elected in this study.The other 174 patients operated with De Vega tricuspid annuloplasty at the time of left cardiac valve replacement were elected randomly for control(group B).There were no significant differences of the patient data before surgery between two groups.Results There were overall 11 early deaths(4 in group A,7 in group B),The overall in-hospital mortality rate was 3.7 %.215 of the 286 surviving patients were followed;the overall follow up was 75.2 %.Mean follow-up was(28.4?9.1)months(range from 13 to 49 months).There were ten late deaths and the late mortality rate was 4.7%.There was no significant differences in the patients with 3+～4+ tricuspid regurgitation at follow-up 18,30 and 42 months in group A;but there was an increase of the patients with 3+～4+ tricuspid regurgitation with an incremental follow up term in group B(P