Swiss Registry for alpha-1 Antitrypsin Deficiency

Several years ago we established a National Registry for alpha-1 antitrypsin deficiency at the Pulmonary Division of the University Hospital of Zurich (http://www.unispital.ch/lungenthorax). The purpose is to collect information on patients with this inherited condition within our country. Data are reported by the patients physicians, provided the patient has given his consent. Our registry comprises about 70 patients, mostly with a Pi ZZ or Pi SZ phenotype.

Prof. E.W. Russi, head of the Pulmonary Division is a member of the Task Force AIR, which is a multinational group of experts on alpha 1-antitrypsin deficiency.

A working group of the Swiss Pulmonary Society made statements several years ago on intravenous augmentation therapy with the human plasma derived product Prolastinâ. Due to a lack of controlled prospective, randomised clinical trials convincingly showing a positive effect on the progression of pulmonary emphysema, this type of treatment is not covered by health insurance in Switzerland.

Patients with alpha-1 antitrypsin deficiency get advice on their disease and the current treatment options by our group. For patients suffering from an advanced stage of their disease we recommend VAT lung volume reduction surgery or eventually lung transplantation.

At present we are involved in a multinational, EU supported study. The aim of this investigation is to study reproducibility and variability of radiological lung density assessment for quantifying pulmonary emphysema by CT scans of the lung. Other planned studies in co-operation with other centres of the AIR group: the effect of various genetic factors on the ion of emphysema; novel therapeutic strategies.

For information and contact:

Dr. E. Grebski, Pulmonary Division
University Hospital Zurich
Rämistrasse 100
CH-8091 Zürich
Tel.: 01 255 88 74
Fax: 01 255 88 20
elzbieta.grebski@dim.usz.ch

Prof. Dr. E.W. Russi, Head of the Pulmonary Division
University Hospital Zurich
Rämistrasse 100
CH-8091 Zürich
Tel.: 01 255 39 28
Fax: 01 255 44 51
erich.russi@dim.usz.ch