Objective To investigate the clinical characteristics and treatment for Type II pulmonary vascular anomaly ( pulmonary arteriovenous malformations) of hepatopulmonary syndrome ( HPS)presenting with hemothorax. Methods A case of Type II pulmonary vascular anomaly of HPS presenting with recurrent hemothorax was described. The clinical data was analyzed and the related literature was reviewed. Results A 72-year-old male patient with Type II pulmonary vascular dilatations of HPS was described to present with recurrent dyspnea and encapsulated pleural effusions. After 4 procedures of thoracentesis, a total of 2510 mL of bloody pleural effusions was drained. The routine analysis of pleural fluid showed the count of red cells exceeded 100 ×109 / L, whereas cytologic examination and tumor biomarkers were negative. Then CTPA and pulmonary angiogramrevealed a Type II pulmonary vascular anomaly of HPS combined with hemothorax. The PaO2 of arterial blood in upright and supine position was 58. 3 mm Hg and 66. 3 mm Hg, respectively. Hypoxemia was alleviated and hemothorax was controlled after embolization of malformed blood vessels. Fromliterature review, similar cases of hemothorax resulted fromrupture of Type II pulmonary vascular anomaly of HPS were not reported. The primary clinical manifestations of HPS were dyspnea and cyanosis. Orthodeoxia and platypnea were most consistent with HPS. The best screening tool for hypoxemia in patients with HPS was P( A-a) O2. The characteristic findings of HPS on chest CT was a lesion or reticulonodular opacities occurring predominantly in the bases of the lungs, which could be enhanced by contrast medium. Pulmonary angiogram was necessary to identify the types of pulmonary vascular dilatations. Hepoxemia of patients with Type II HPS often responded poorly to oxygen therapy, whereas embolization of the pulmonary arteriovenous fistulas was helpful to improve anoxia. Conclusions Rupture ofType II pulmonary vascular malformations in HPS was a rare cause of hemothorax. Thrombosis of pulmonary arteriovenous malformations may result in significant improvement in oxygen saturations as well as control of hemothorax. In the setting of liver disease, intrapulmonary vascular dilatations and hypoxemia often suggestthe existence of HPS.
Objective To study the clinical application of the GOLD/GINA criteria and the Spanish guideline in the diagnosis of asthma-COPD overlap syndrome (ACOS). Methods Patients with stable COPD were consecutively enrolled in the study. Clinical data were collected, lung function with bronchodilator test and peak expiratory flow (PEF) were performed, and peripheral blood eosinophils, total IgE, and sputum inflammatory cells were measured. Those overlap with asthma were identified by the 2 different criteria, and the prevalence and features of ACOS were compared. Results Among 104 cases of stable COPD, 24 (23.1%) and 10 (9.6%) were identified as ACOS by the GOLD/GINA criteria and the Spanish guideline, respectively; the latter 10 cases were all included in the former 24. For the GOLD/GINA criteria, the most common features were symptoms triggered by exercise or emotions, variable airflow limitation, family history of asthma, and other allergic conditions. Mean diurnal PEF variation≥10% was evident in 11 cases (45.8%, 11/24), while bronchodilator test was positive in 16 cases (66.7%, 16/24). For the Spanish guideline, the most common features were diagnosis of asthma before 40, other allergic diseases, positive bronchodilator test on 2 occasions. Conclusions The GOLD/GINA criteria may be more sensitive for the diagnosis of ACOS, and do not need sophisticated lab tests, which may be more applicable for clinical use. The Spanish guideline is restrictive, and therefore may lead to under-diagnosis.
Objective To improve the knowledge of pulmonary sclerosing hemangioma ( PSH)especially with bilateral multiple lesions of the lung. Methods The clinical data of 3 cases of PSH ( 1 case with bilateral multiple lesions in the lung) were analyzed, and the related literatures were reviewed. Results All of the 3 cases were females. A 22-year-old female patientwith bilateral multiple nodules in the lungs was complicated with thyroid multiple nodular goiter ( with hypothyroidism) , dysfunctional uterine bleeding ( with anemia) , nodular hyperplasia of the breast, and arteriovenous malformation over forearm. Thoracoscopicbiopsy of left lung and resection of the right pulmonary mass were performed and both the lesions were confirmed as PSH. The clinical manifestations of multiorgan diseases and the presence of PSH suggested Cowden syndrome in this patient. The other 2 cases aged 50 and 53 were asymptomatic with solitary pulmonary nodules identified incidentally. The accessory examinations for malignancies, infections, and autoimmune diseases showed no specific findings. Resection of the lesions were performed by thoracoscopic surgery and thoracotomy respectively, and the histopathological results proved to be PSH. Literature review showed that PSH typically occurred in middle-aged women without clinical symptoms and signs, often presenting as a pulmonary solitary nodule/mass identified incidentally. The differential diagnosis should include peripheral carcinoma, hamartoma, inflammatory pseudotumor and tuberculoma. Multiple PSH, which mainly presented as multiple well-defined nodules /masses of different size in the lungs, was rather rare, but easily confused with metastatic neoplasm. Lung biopsy by surgical operation was a common way to confirm the diagnosis, while FDP-PET and fine needle aspiration biopsy showed some defects. Surgical resection was an effective method of treatment, the residual lesions of multiple PSH should be monitored. Cowden syndrome may be considered if a PSH coexisting with abnormity of multiple organs such as thyoid, breast and vessels. Conclusions PSH should be considered during the differential diagnosis for solitary or multiple nodules /masses in the lung. Surgical biopsy is a common way to confirm the diagnosis. Local excision andnecessary follow-up are effective methods of treatment.