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Objective: To evaluate the incidence of chronic thromboembolic pulmonary hypertension (CTEPH) secondary to acute pulmonary thromboembolism (PTE) and the serum level of growth differentiation factor-15(GDF-15). Methods: Ninety-six acute PTE patients were recruited in the study. Clinical data, Wells score, blood gas analysis, D-dimmer level, GDF-15 level, atrial and ventricular sizes, pulmonary arterial systolic pressure (PASP) and pulmonary artery CT (CTPA) data were collected. Patients were followed up to evaluate the cardiac function (WHO class), ultrasonic cardiogram and CTPA to confirm the incidence of CTEPH. Results: Eighty-fivepatients were followed for 5 months to 58 months (average 26.5±14.7 months). The incidence of CTEPH was 12.9% (11/85). Between CTEPH patients and non-CTEPH patients, PASP, right atrial and ventricular sizes, and GDF-15in the acute stage were significantly different( P <0.05). According to binary logistic regression analysis, the incidence of CTEPH was correlated positively with acute PASP and the serum level of GDF-15. Conclusions: The incidence of CTEPH in acute PTE patients was 12.9% in this study. Acute PASP and higher level of GDF-15 are predictive factors for CTEPH secondary to acute PTE.

Objective: To report a case of the pulmonary surfactant protein(SP) adenosine triphosphate-binding-cassette-A3 (ABCA3) gene mutations in infant congenital interstitial lung disease(ILD), and review the related literature, to investigate the relationships of ABCA3 gene mutation associated with ILD in infants. Method: A 6-months-old boy was hospitalized in the department of Pediatrics of the First Affiliated Hospital of Guangzhou Medical University. The clinical, radiological, histological information from transbronchial lung biopsy (TBLB) and genetic testing in this case was analyzed; 12 reports retrieved on literature search at Pubmed, OVID databases from 2004 to 2015 by using the ABCA3 as keyword were reviewed and analyzed. Result: (1)The patient, a 6-months-old boy, had progressive tachypnea and dyspnea since 4 months old. Physical examination on admission revealed respiratory rate of 78 times/min , heart rate of 187 times/min, SpO 2 0.93(mask oxygen-inspiration with 6 L/min), scattered fine moist crackles could be heard over the both lungs, clubbing fingers were found. High-resolution computed tomography(HRCT) revealed diffuse ground-glass opacity, interlobular and intralobular septal thickening. Lung biopsies showed evidences of the alveolar cavity atelectatic changes and interstitial fibrosis. SP-A and SP-B were negative in immunohistochemical stainting. SP-related gene sequence analysis found that there was compound heterozygous missense mutation of ABCA3 gene in c. 1942A>G, c.2701-33G>C and c. 991-105C>A. (2)The review of related literature found that totally 12 cases were reported. The main manifestations were progressive tachypnea and dyspnea, age of onset was between birth and 4 years of age. The imaging characteristics of chest HRCT revealed diffuse infiltration or diffuse ground-glass pattern in the lung., Prognosis: 6 cases died, and 6 cases survived, including 4 cases with pulmonary function disturbance to different degrees; 12 cases had ABCA3 gene mutations, 9 cases had composite ABCA3 gene mutations, in 11 cases the mutation occured in the exon of coding region, in 1 case in the intron, 9 cases had heterozygous mutations, 3 cases had homozygous mutations. Conclusion: The main phenotypes of ABCA3 mutation associated with ILD were full term neonatal respiratory distress syndrome or progressive tachypnea or dyspnea unexplained in infants. The chest HRCT showed two diffuse pulmonary interstitial changes. ABCA3 mutation mainly was multi-site composite mutations and heterozygous mutations in the exon of coding region.

Twenty one patients with bronchial adenoma, treated surgically in our hospital, include three different neoplasms: carcinoid, cylindroma and mucoepidermoid adenoma. In this series, clinical characteristics were: bronchial obstruction when the tumor protrudes into the lumen and infection, first in the bronchus (bronchitis or bronchiectasis) then in the parenchyma (acute, recurrent or chronic pneumonia, tension abscess). Ball-valve action of the tumor may result in lobar or segmental emphysema. Preoperatively, most of the patients had been misdiagnosed as bronchitis, carcinoma of lung, bronchiectasis or acute tension abscess. In our series, all the patients were alive in a follow up of 2-8 years. Yet one patient is living with local recurrence and distant metastasis. To our experience, pneumonia recurring in the same area of the lung, localized wheezing, with or without endocrine symptoms, lobar or segmental emphysema may suggest bronchial adenoma. Tomography and endoscopy are important for diagnosis. For the treatment, sleeve resection of the main bronchus was done in 2, bronchoplastic lobectomy in 7, lobectomy in 10, and pneumonectomy in 2. Sleeve resection of the main bronchus or bronchoplastic lobectomy is recommended as a reliable procedure for this disease.