SELF-ASSESSMENT QUESTIONS
A case of refractory hypoxaemia
1 Scottish Pulmonary Vascular Unit, Western Infirmary, Glasgow, UK
2 Respiratory Medicine, Department of Medicine, University of Cambridge, Addenbrookes Hospital, Cambridge, UK
Correspondence to:
Dr A C Church, Scottish Pulmonary Vascular Unit, Western Infirmary, Dumbarton Road, Glasgow, G11 6NT, UK; colinchurch@doctors.org.uk
Accepted 7 July 2008
Keywords: hepatopulmonary syndrome; pulmonary arteriovenous shunts; orthodeoxia
| The first 150 words of the full text of this article appear below. |
A 61-year-old man, who is a lifelong non-smoker, was found on routine testing in 2002 to have abnormal liver function tests. Ultrasound examination demonstrated abnormal liver texture. Shortly afterwards he had a significant bleed from an oesophageal varicoele and as a consequence underwent computed tomography (CT) scanning of his thorax and abdomen. A liver biopsy was performed at this time, which showed granulomatous hepatitis. On the basis of this and widespread hilar and mediastinal lymphadenopathy, a clinical diagnosis of sarcoidosis was made. Treatment with systemic corticosteroids resulted in a modest improvement in his liver function and general well being.
Over the subsequent 4 years he developed finger clubbing and progressive exertional dyspnoea with a final exercise tolerance of just 20 m on the flat. He was aware that his dyspnoea improved when lying down. Clinical examination at this time revealed cyanosis, an elevated jugular venous pressure, and a degree of
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