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Long-term efficacy and safety of alpha1 proteinase inhibitor treatment for emphysema caused by severe alpha1 antitrypsin deficiency: an open-label extension trial (RAPID-OLE).
McElvaney NG, Burdon J, Holmes M, Glanville A, Wark PA, Thompson PJ, Hernandez P, Chlumsky J, Teschler H, Ficker JH, Seersholm N, Altraja A, Mäkitaro R, Chorostowska-Wynimko J, Sanak M, Stoicescu PI, Piitulainen E, Vit O, Wencker M, Tortorici MA, Fries M, Edelman JM, Chapman KR; RAPID Extension Trial Group. McElvaney NG, et al. Among authors: wencker m. Lancet Respir Med. 2017 Jan;5(1):51-60. doi: 10.1016/S2213-2600(16)30430-1. Epub 2016 Dec 2. Lancet Respir Med. 2017. PMID: 27916480 Clinical Trial.
The effects of weekly augmentation therapy in patients with PiZZ α1-antitrypsin deficiency.
Schmid ST, Koepke J, Dresel M, Hattesohl A, Frenzel E, Perez J, Lomas DA, Miranda E, Greulich T, Noeske S, Wencker M, Teschler H, Vogelmeier C, Janciauskiene S, Koczulla AR. Schmid ST, et al. Among authors: wencker m. Int J Chron Obstruct Pulmon Dis. 2012;7:687-96. doi: 10.2147/COPD.S34560. Epub 2012 Sep 28. Int J Chron Obstruct Pulmon Dis. 2012. PMID: 23055718 Free PMC article.
BACKGROUND: The major concept behind augmentation therapy with human alpha(1)-antitrypsin (AAT) is to raise the levels of AAT in patients with protease inhibitor phenotype ZZ (Glu342Lys)-inherited AAT deficiency and to protect lung tissues from proteolysis and progression of emph …
BACKGROUND: The major concept behind augmentation therapy with human alpha(1)-antitrypsin (AAT) is to raise the levels of AAT in patients wi …
New Findings in PiZZ alpha1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy.
Gross B, Grebe M, Wencker M, Stoller JK, Bjursten LM, Janciauskiene S. Gross B, et al. Among authors: wencker m. Dermatology. 2009;218(4):370-5. doi: 10.1159/000202982. Epub 2009 Feb 16. Dermatology. 2009. PMID: 19218787 Free PMC article.
Our case extends current knowledge of AAT-associated panniculitis in 2 ways: (1) we demonstrate Z-type AAT polymers in the skin, which supports the inflammatory pathogenesis of panniculitis and the potential pro-inflammatory role of polymers; (2) we show that a high dose and long …
Our case extends current knowledge of AAT-associated panniculitis in 2 ways: (1) we demonstrate Z-type AAT polymers in the skin, which suppo …
[Long-term therapy of alpha 1-antitrypsin-deficiency-associated pulmonary emphysema with human alpha 1-antitrypsin].
Wencker M, Banik N, Buhl R, Seidel R, Konietzko N. Wencker M, et al. Pneumologie. 1998 Oct;52(10):545-52. Pneumologie. 1998. PMID: 9847632 German.

However, it is not clear if affected patients benefit from long-term augmentation therapy and no long-term safety data are available. ...Stratified for baseline FEV1, the decline was 35.6 +/- 21.3 ml in the 108 patients with an initial FEV1 < 30% and 64.0 +/- 26.

…
However, it is not clear if affected patients benefit from long-term augmentation therapy and no long-term safety data are ava …
Long-term treatment of alpha1-antitrypsin deficiency-related pulmonary emphysema with human alpha1-antitrypsin. Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL)-alpha1-AT-study group.
Wencker M, Banik N, Buhl R, Seidel R, Konietzko N. Wencker M, et al. Eur Respir J. 1998 Feb;11(2):428-33. doi: 10.1183/09031936.98.11020428. Eur Respir J. 1998. PMID: 9551749 Free article. Clinical Trial.
However, it is not clear if affected patients benefit from long-term augmentation therapy and no long-term safety data are available. ...The remaining 15 patients had an initial FEV1 >65% pred. Long-term treatment with i.v. alpha1-antitrypsin in patients w …
However, it is not clear if affected patients benefit from long-term augmentation therapy and no long-term safety data are ava …