TY - JOUR T1 - Sex differences in alpha-1-antitrypsin deficiency JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2015.PA1134 VL - 46 IS - suppl 59 SP - PA1134 AU - Sebastian Faehndrich AU - Christian Herr AU - Timm Greulich AU - Martina Seibert AU - Philipp M. Lepper AU - Nikolas Bernhard AU - Cindy Lützow AU - Claus Vogelmeier AU - Robert Bals Y1 - 2015/09/01 UR - http://erj.ersjournals.com/content/46/suppl_59/PA1134.abstract N2 - Alpha-1-Antitrypsin deficiency (AATD) is a rare disease which leads to early development of pulmonary emphysema or to liver cirrhosis. With the aim to learn more details about the natural history and the quality of care for these patients the German AATD registry maintained in Marburg and Homburg/Saar was established in 2004. Methods, Patients, Results: Up to December 2014, 1066 patients (male 482; female 584) were registered, 822 with the genotype PiZZ and 109 with PiSZ. The age of the registered individuals is age (male: Mean 51.91 years [SD=13.86]; female: Mean 52.76 years [SD=13.39] years). Most patients are affected by chronic obstructive pulmonary disease (COPD, distribution to the GOLD stages: GOLD 0-I= 47 (male: 19; female: 28), GOLD II =160 (male: 83; female: 77), GOLD III =163 (male: 111; female: 52), GOLD IV =51 (male: 37; female: 14). Currently 465 subjects are under substitution therapy. The time period between the first symptom and the establishment of the correct diagnosis was on average 7 years (SD=13 years). Conclusions: The data of the registry allow a detailed characterization of the natural course of the disease and the levels of the patient care. ER -