Objective To investigate the diagnosis and treatment of pulmonary arterial hypertension ( PAH) due to rare causes. Methods The clinical presentation, laboratory testing, diagnosis and treatment of 4 patients with PAH associated with rare causes in Beijing Anzhen Hospital from January 2001 to March 2008 were analysed retrospectively. Results Primary biliary cirrhosis, hyperthyroidism, antiphospholipid syndrome and pulmonary artery sarcoma may cause PAH, which were improved after corresponding diagnosis and management. Conclusion PAH can result from rare causes. The enhancement of its recognition will help earlier diagnosis and treatment and improve the prognosis.
Objective To study the clinical features and prognosis of recurrent pneumonia associated with myotonic dystrophy. Methods A case of recurrent pneumonia related to myotonic dystrophy was retrospectively analyzed and the related literatures were reviewed. Results The patient was a 32-year-old man with recurrent fever, cough and expectoration for more than 10 years. He was diagnosed as " pneumonia” in the local hospital at every relapse, and improved after antibiotic therapy. Nine months ago, the symptoms of fever, cough and expectoration aggravated. The chest X-ray examination showed consolidation in right middle and lower lobe. After 2-month antibiotic treatment, the symptoms relieved. Then he was admitted to Beijing Anzhen Hospital for further diagnosis and treatment. Physical examination revealed " hatchet-faced” appearance with neck muscles atrophy and slightly flexion. Bilateral sternocleidomastoid muscle symmetrically rised. Few moist rales of bilateral lower lung were found. Lateral elbow and femoral quadriceps muscles symmetrically rised. The muscle strength of his four distal limbs became weakness, and the squat and standing movement was difficult. Chest CT showed pathy effusion and consolidation in right middle lobe and the lower left lobe. Electromyography showed myogenic damage in left extensor digitorum, left deltoid, right anterior tibial muscle, femoral quadriceps muscle, and left sternocleidomastoid, and spontaneous myotonic discharges in left extensor digitorum, right anterior tibial muscle and left sternocleidomastoid. Pathologic examination of left femoral quadriceps muscle showed inflammatory myopathy. The final diagnosis was myotonic dystrophy associated recurrent pneumonia. Two articles revolving 2 cases were retrieved in English literature. No case was reported in Chinese literature. Conclusion The possibility of myotonic dystrophy should be considered in the case of recurrent pneumonia complicated with muscle atrophy, weakness and myotonia.
Objective To invesitgate the prevalence of pulmonary thromboembolism （PTE） in hospitalized patients with obstructive sleep apnea-hypopnea syndrome （OSAHS）from January 2004 to December 2008，and analyse its impact on the severity of OSAHS. Methods Demographic and clinical characteristics of 24 OSAHS patients complicated with PTE were analyzed. 30 OSAHS patients without PTE were served as controls. Results PTE was detected in 2.44% （31/1268） of the OSAHS patients. When compared with the OSAHS patients without PTE，the OSAHS patients with PTE had a significantly higher apnea hypopnea index （AHI） [（27.8±11.6）/h vs. （18.2±8.1）/h，P=0.038] and a lower LSpO2 （lowest saturated pulse arterial oxygen level） [（78.4±8.5）% vs. （85.2±7.9）%，P=0.035]. Both groups received continuous positive airway pressure （CPAP） ventilation. Anticoagulation and/or thrombolysis treatment were used in the OSAHS patients with PTE. Conclusions We found a higher prevalence of PTE in patients with OSAHS. Compared with those without PTE，OSAHS patients with PTE have more severe sleep apnea-hypopnea and hypoxemia in sleep. Comprehensive treatments including anticoagulation and CPAP should be used in these patients.