In sum, twenty-seven patients were registered for hemoptysis with pulmonary vascular malformations where 88.9% of patients were with abnormal BAs and 11.1% were with PAVFs. The age distribution was recorded as 2 patients were in preschool-group, and 20 patients were in school-group in addition to 5 patients in adolescent-group. Meanwhile, it also demonstrated that the distribution of hemoptysis degree affected by age that almost all the moderate and severe conditions were observed in elder children, including 76.2% in school-group and 23.8% in adolescent-group. Additionally, findings indicated that hemoptysis was with a presence of moderate to massive condition when related to abnormal BAs, mostly. When associated with PAVFs, however, symptomatic hemoptysis was unobvious with little blood in sputum or vomitus. At the initial point of hemoptysis, associated with which the common symptoms with the highest frequency were cough (74.1%), fever (29.6%), abdominal pain (14.8%), vomit (7.4%) and circulation manifestations (22.2%). And the mean interval duration between the antecedent symptoms and hemoptysis was calculated as 3.9±3.0 days. In these series, pathogen test revealed no specific bacteria except for MP. Serum level of MP-Ab were tested in 18 individuals showing a positive rate of 83.3%. And it indicated a level of ≥ 1:320 titers in 12 children and a level of ≥ 1:160 titers in 3 children where these 15 children were diagnosed with a current MP infection according to the diagnostic criteria and rolled in Group1, eventually. Furthermore, MP-DNA-PCR identified 37.5% patients were detected as a high copy in Group 1. The other 12 patients without proofs for MP infection were defined as Group 2. The demographic characteristics between groups were recorded in Table 1. It showed there existed no obvious difference in distribution of age (P=.439) and gender (P=.420). However, in terms of hemoptysis degree, we found that most severe conditions of massive hemoptysis existed in patients suffered from MP infection at that point, showing a statistical significance (P=.008). Blood routine examinations were conducted in each individual, presenting similar outcomes between groups in WBC count (P=.733), in neutrophil accounting (P=.107), and in hemoglobin level (P=.884). Meanwhile, we found that five episodes, about 62.5%, were observed an abnormal FVC or V25-V75. And it showed three patients from Group 1 with a significant decrease of pulmonary ventilation function, who manifested a massive hemoptysis.
Graphic findings indicated diagnostic yields. As to CT, this procedure helped a 30% higher detectable rate of pulmonary lesions than initial radiographs. Currently, a sum of 25 children were detected with diagnostic fields where most of locations were presented in the right lung, especially in Group 1 (P=.006). All patients in patients with MP infection were detected with definitive lesions, including glassy degeneration (20.0%), flocculent shadows (20.0%), inflammatory infiltration (20.0%), pulmonary atelectasis (13.3%), consolidation (13.3%), diffuse patchy shadow (6.7%) and bronchial arterial enlargement (6.7%). Furthermore, the distribution of lesions was focused on the right inferior lobe. Totally, 41 detectable anomalous BAs were found in 24 children, and single or multiple PAVFs were detected in 3 children by angiography. As golden criteria of pulmonary vascular malformations, it hinted smoking phenomenon locating at the sites of vascular ruptures and bleeding. About 50% of patients had more than two abnormal bronchial vessels corresponding to each other. These abnormal arteries were shown to originate from different sites of systemic circulation, which connected and jointly delivered systemic-to-pulmonary blood perfusion. Twenty-six, about 63.4%, tortuous or hypertrophy arteries were from T5-T6 level of thoracic aorta, along the bronchial tree entering the pulmonary fields. Eight BAs (19.5%) were from descending aorta beyond T5-T6 level, and two BAs (4.9%) were involved in the aortic arch, four BAs (9.8%) were the branches of subclavian arteries in four independent cases. One abnormal artery was considered from the hepatic aorta. As to patients with MP infection in Group1, 14 patients were with abnormal BAs, as shown in Fig.1. Meanwhile, the counts of abnormal arteries were different with or without MP infection (P=.026).
Twenty-three patients were treated transcatheter plug occlusion, including 20 patients with abnormal BAs and 3 patients with PAVFs. In Group 1, 14 patients received interventional therapy and one patient received the lung resection for an aberrant artery originating from the hepatic artery. In Group 2, two patients chose traditional medicine (antibiotics and hemostatic drugs) for a mild hemoptysis and one patient underwent a ligation of the abnormal BA from right subclavian artery using a thoracoscopic guidance. The others were undertaken a treatment of transcatheter plug occlusion. After the transcatheter plug occlusion, findings showed an obvious reduction of contrast medium shunts according to an instant angiogram, indicating an effective procedure, while there existed 14 recurrence episodes in 8 patients with abnormal BAs in Group1 (P=.002). Additionally, all of cases with recurrent hemoptysis were categorized as a massive hemoptysis, with a titer of MP-Ab≥1:320 in serum, primarily. Furthermore, a lasting high level of MP-Ab was observed in a recurrent state, and details of recurrent cases were shown in Table 2. One patient received the lung resection died after surgery for one week. The OR of clinical worsening in patients with MP infection was about 2:1. Moreover, the even-free survival rate of patients was portrayed in Fig.2.