Sökning: L773:0040 6376 OR L773:1468 3296
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Clinical course and...
Clinical course and prognosis of never-smokers with severe alpha-1-antitrypsin deficiency (PiZZ).
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- Tanash, Hanan (författare)
- Lund University,Lunds universitet,Lungmedicin, allergologi och palliativ medicin,Sektion II,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Respiratory Medicine, Allergology, and Palliative Medicine,Section II,Department of Clinical Sciences, Lund,Faculty of Medicine
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Nilsson, Peter M (författare)
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- Nilsson, Jan-Åke (författare)
- Lund University,Lunds universitet,Ortopedi - klinisk och molekylär osteoporosforskning,Forskargrupper vid Lunds universitet,Orthopedics - Clinical and Molecular Osteoporosis Research,Lund University Research Groups
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- Piitulainen, Eeva (författare)
- Lund University,Lunds universitet,Lungmedicin, allergologi och palliativ medicin,Sektion II,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Respiratory Medicine, Allergology, and Palliative Medicine,Section II,Department of Clinical Sciences, Lund,Faculty of Medicine
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(creator_code:org_t)
- 2008-05-20
- 2008
- Engelska.
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Ingår i: Thorax. - : BMJ. - 1468-3296 .- 0040-6376. ; 63, s. 1091-1095
- Relaterad länk:
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http://www.ncbi.nlm....
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http://dx.doi.org/10...
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https://thorax.bmj.c...
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https://lup.lub.lu.s...
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https://doi.org/10.1...
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Abstract
Ämnesord
Stäng
- BACKGROUND: Previous studies of non-smoking individuals with severe alpha-1-antitrypsin deficiency (PiZZ) have been sparse and included only a limited number of individuals, mostly identified by respiratory symptoms. The aim of this study was to estimate the prognosis of non-smoking PiZZ individuals and to analyze the most common causes of death by including a large number of individuals who had been identified due to reasons other than respiratory symptoms. METHODS: The study included 568 non-smoking PiZZ subjects who were selected from the Swedish National AAT Deficiency Registry and followed-up from 1991 to September 2007. Of these, 156 (27%) were identified by respiratory symptoms (respiratory cases) and 412 were identified by extra pulmonary symptoms or screening (non-respiratory cases). RESULTS: Ninety-three subjects (16%) died during the follow-up time. The specific Standardized Mortality Rate (SMR) for the whole study population was 2.32 (95% confidence interval (CI) 1.87-2.83) with no significant difference between men and women. The SMR was 2.55 (95% CI 1.91-2.83) for the respiratory cases and 2.07 (95% CI 1.49-2.81) for the non-respiratory cases. Further calculation of SMR for subgroups in the non-respiratory cases showed that the SMR was 0.70 (95% CI 0.14-2.04) for individuals identified by family/population screening. Emphysema and liver cirrhosis were the most common causes of death (45% and 28%, respectively). Malignant transformation was found in 38% of the cirrhotic cases. CONCLUSION: Non-smoking PiZZ individuals identified by screening do not have an increased mortality risk compared with the Swedish general population.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Lungmedicin och allergi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Respiratory Medicine and Allergy (hsv//eng)
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