Abstract. Kjeldsen AD, Oxhùj H, Andersen PE, Green A, Vase P (Odense University and Odense University Hospital, Odense; University of Aarhus, Aarhus; and the Department of Otorhinolaryngology, Svendborg, Denmark). Prevalence of pulmonary arteriovenous malformations (PAVMs) and occurrence of neurological symptoms in patients with hereditary haemorrhagic telangiectasia (HHT). J Intern Med 2000; 248: 255±262.Background. Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease. HHT is characterized by a wide variety of clinical manifestations, including pulmonary arteriovenous malformations (PAVMs) and neurological symptoms. Objective. To estimate (i) the prevalence of PAVM, and (ii) the occurrence of neurological symptoms in a geographical well-defined population of HHT patients. Methods. HHT family members were invited to a clinical examination including registration of HHT manifestations, screening for pulmonary arteriovenous malformations and neurological evaluation. Two groups served as controls: (i) first-degree relatives without any signs of HHT; and (ii) age-and gendermatched controls.

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The validity of peak expiratory flow (PEF) recordings and the sensitivity of PEF variability indices in asthma and asthma-like conditions were examined in an adolescent population. Recordings from 245 randomly selected subjects and from an additional 181 subjects reporting or considered at risk for developing asthma were analyzed. Subjects recorded PEF twice daily for 2 wk using Mini-Wright meters, completed a symptoms questionnaire, and were tested for airway responsiveness to methacholine. The first three recording days showed significant accumulation of lowest PEF values recorded (41%) and were excluded from further analysis. Among nine PEF variability indices, the Two-lowest%mean (the mean of the two lowest PEF values as a percentage of the period mean) had the best sensitivity for physician-diagnosed asthma (28%). The sensitivity of the methacholine dose-response slope (DRS) was 69%. Combining these indices, a sensitivity of 77% for diagnosed asthma was obtained. Among subjects with asthma-like symptoms but no diagnosis of asthma, 14% had increased Two-lowest%mean and the DRS was increased in 17%, but only 3% were identified by both tests. In conclusion, inhomogeneity of PEF data could be corrected by disregarding the first three recording days. PEF variability indices identified some diagnosed asthmatics, and particularly some symptomatic "nonasthmatics," not identified by the DRS, suggesting that the combined use of these indices might be helpful.

Respiratory symptoms, spirometry, forced expiratory flows, and the nitrogen closing volume test were studied in 119 welders and 90 controls, matched with respect to age, height, and smoking habits. Respiratory symptoms according to a questionnaire were more prevalent in the welders. No short-term changes of the measured variables during the day or week attributable to welding were found in twenty-one nonsmoking welders. Compared to the controls, closing volume and closing capacity (i. e., closing volume + residual volume) were significantly higher, and total lung capacity and the amplitude of the cardiogenic oscillations in the nitrogen curve were significantly lower in the welders who were nonsmokers or exsmokers, whereas there were no differences among smokers. These findings in welders may be attributable to deposition of welding fume particles in peripheral small airways or alveoli.