Alpha1 Germany eV.

After the long-awaited initial results of the study conducted by Aachen University Hospital for the 2015 Annual General Meeting in Rostock reached our members' households between January and February 2016, the Berlin-Brandenburg Self-Help Group met on March 16, 2016, at the Charité's Benjamin Franklin Campus in Berlin to continue the study. Of the 13 participating members, 7 underwent repeat measurements and 6 underwent initial assessments. Each participant received an individual appointment and study information via email in advance. Following a detailed explanation and blood draw by Dr. Pavel Strnad (Aachen University Hospital), the elastographic liver fibrosis measurements were performed by Dr. Karim Hamesch (Aachen University Hospital) and Martin Wetzel (Charité Berlin). Elastography uses various ultrasound-based technologies to measure liver stiffness. A healthy liver is "soft," while fibrosis or scarring of the liver is associated with increasing liver stiffness. In certain cases, this modern method now allows doctors to forgo a liver biopsy. In Berlin, three different elastographic devices were used. One was a state-of-the-art ultrasound machine used for a familiar abdominal ultrasound. Two different units were used, which looked like ordinary ultrasound machines from the outside but had a special integrated elastographic measurement unit. The other was the FibroScan examination, which many of us are already familiar with from Rostock. The Aachen study group, which now includes several other cooperating liver centers, aims to directly compare fibrosis assessments using different devices.

The aim of this study is to systematically record and evaluate liver involvement in patients with alpha-1 antitrypsin deficiency in Germany for the first time. Furthermore, the various measurement methods will be examined to enable the future monitoring of patients with a standardized, informative examination procedure throughout their disease progression. Ultimately, this should lead to the development of a preventative care plan for liver involvement, which is currently entirely lacking. From the physicians' perspective, a fundamental problem is that while liver involvement is common in alpha-1 antitrypsin deficiency, liver function tests are often normal despite advanced liver damage, resulting in missed or delayed diagnoses. Consequently, the complications of liver cirrhosis often cannot be prevented early on. The study group hopes that measuring liver stiffness and identifying new blood markers will enable the early detection of liver damage. Further information can be found on the study group's new website: www.alpha1-leber.de

The study group will also be present at our members' meeting in Bremen and will be using various methods for determining liver fibrosis there as well. The Charité Campus Benjamin Franklin, a partner clinic of the study, is also available to all patients with AAT deficiency as a liver diagnostic center, with Mr. Martin Wetzel as the contact person. Interested members can have a free MRI examination of the liver as part of the study there or at the University Hospital Aachen. This was the first and very satisfactory examination conducted by a self-help group – other self-help groups can arrange an appointment with the study group (contact details: Alpha1-leber@ukaachen.de, +49 241 80 80865)., www.alpha1-leber.de).