Torii Nobuyuki, Ichihara Atsuhiro, Mizuguchi Yuki, Seki Yasufumi, Hashimoto Etsuko, Tokushige Katsutoshi
Department of Internal Medicine and Gastroenterology, Tokyo Women's Medical University, Japan.
Department of Medicine II, Endocrinology and Hypertension, Tokyo Women's Medical University, Japan.
Intern Med. 2018;57(12):1741-1745. doi: 10.2169/internalmedicine.0027-17. Epub 2018 Jun 15.
The patient was a 23-year-old man who was diagnosed with severe hypoxemia and liver dysfunction after suffering from sudden difficulty breathing. At 2 years of age, he had been diagnosed with hypopituitarism, and had received hormone-replacement until he was 18 years of age. Echocardiography using micro bubbles and pulmonary scintigraphy indicated intrapulmonary shunt and a liver biopsy showed steatohepatitis. He was diagnosed with hepatopulmonary syndrome associated with nonalcoholic steatohepatitis. Hormone-replacement therapy was re-started. After 5 months, a second liver biopsy revealed the amelioration of nonalcoholic steatohepatitis, which improved his respiratory condition. This case suggested that early effective therapy for chronic liver diseases might improve the pathological and clinical conditions of hepatopulmonary syndrome.
该患者为一名23岁男性,在突发呼吸困难后被诊断为严重低氧血症和肝功能不全。他在2岁时被诊断为垂体功能减退,并接受了激素替代治疗直至18岁。使用微泡的超声心动图和肺闪烁扫描显示存在肺内分流,肝脏活检显示脂肪性肝炎。他被诊断为与非酒精性脂肪性肝炎相关的肝肺综合征。重新开始激素替代治疗。5个月后,第二次肝脏活检显示非酒精性脂肪性肝炎有所改善,这改善了他的呼吸状况。该病例表明,对慢性肝病进行早期有效治疗可能会改善肝肺综合征的病理和临床状况。