Studien zum Alpha1-PI-Mangelemphysem
|Dirksen-A; Friis-M; Olesen-KP; Skovgaard-LT; Sorensen-K
Department of Respiratory Medicine, Bispebjerg Hospital, Copenhagen, Denmark.
Acta-Radiol. 1997 Sep; 38(5): 826-32
Progress of emphysema in severe alpha 1-antitrypsin deficiency as assessed by annual CT .
PURPOSE: To assess serial CT as a measure of the progress of emphysema in patients with
severe alpha 1-antitrypsin deficiency (phenotype PiZ).
MATERIAL AND METHODS: In a randomized placebo-controlled study of alpha 1-antitrypsin
augmentation therapy, 22 patients with moderate emphysema were fol1owed for 2-4 years with
an annual lung CT. The images were analysed by means of semiautomatic lung detection, and
the degree of emphysema was quantitated by the density-mask and the percentile methods.
The influence of lung volume was standardised by a regression model. RESULTS: A highly
significant decline in Hounsfield units (HU) was found in low-density areas, corresponding
to a mean (SE) annualloss of lung tissue of 2.1 (0.4) g/l lung volume. Analysis of a
single slice at 5 cm below the level of the carina gave comparable results: 2.4 (0.4) g/l.
CONCLUSION: Serial CT is a sensitive measure of the progress of emphysema in patients with
severe alpha 1-antitrypsin deficiency.
Abteilung Pneumologie, Medizinische Univ. Klinik Freiburg.
Versicherungsmedizin. 1995 Dec 1; 47(6): 201-3
The most frequent form of lung emphysema leading to respiratory failure is the tobacco
bronchitis-induced type of emphysema the so called chronic obstructive pulmonary (lung)
disease ( COPD). Histologically the centrilobular or centriacinar emphysema is believed
to develop due to elastase and oxidant overload with concomitant antiprotease deficiency.
The alphal-antitrypsin deficiency is a rare genetic defect leading also in non-smoking
patients to early death due to panlobular or panacinar emphysema. The functional pattern
of both emphysema types shows irreversible lung overinf1ation with severe mainly expiratory
bronchial obstruction with various degrees of pulmonary hypertension alphal-proteinase
inhibitor deficiency emphysema is prophylactically treated with Prolastine and if hypoxia
(Pa02 < 55 mm/Hg) is present with long term oxygen therapy. If hypercapnia develops
02-Therapy is combined with non invasive pressure supported ventilation. Volume reducing
surgery may precede. In nonsmoking emphysema patients long term oxygen therapy and later
unilateral lung transplantation improves quality of life as weIl as life expectancy.
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