{"id":1948,"date":"2014-09-15T12:22:07","date_gmt":"2014-09-15T10:22:07","guid":{"rendered":"http:\/\/www.alpha1-deutschland.org\/?p=1948"},"modified":"2024-01-18T13:30:36","modified_gmt":"2024-01-18T12:30:36","slug":"pimz-ich-bin-doch-nur-traeger","status":"publish","type":"post","link":"https:\/\/alpha1-deutschland.org\/en\/pimz-ich-bin-doch-nur-traeger","title":{"rendered":"PiMZ \u2013 I'm just a carrier"},"content":{"rendered":"
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PiMZ \u2013 I'm just a carrier<\/span><\/h1>\n<\/header>\n<\/div>\n\n\n\n
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Published in Alpha1 Journal 02\/2014<\/p>\n<\/div>\n\n\n\n

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PiMZ is one of the most important topics of the year for us at Alpha 1 Germany. Dr. Steveling from the Ruhrland Clinic in Essen already gave a presentation on this subject at our members' meeting. People with MZ must not be overlooked. This topic is personally important to me, as there are many people with MZ in my family. Therefore, I would like to explain what a diagnosis of heterozygous alpha-1 antitrypsin deficiency (PiMZ) means for each individual.<\/p>\n

\u201e"I'm just an MZ and therefore just a carrier," many of those affected who came to the children's day event think. We're talking about affected children, and by that we almost always mean ZZ. Many questions quickly arise: What about the siblings? Were they even tested? Did the parents get tested? Shouldn't we even be thinking a generation further ahead \u2013 what about grandparents, aunts, and uncles \u2013 were they all tested?<\/p>\n

Alpha-1 antitrypsin deficiency is a familial disease<\/span><\/h2>\n

It's easy to forget that we have affected individuals in our families. That was the case in my family, too. When children are affected, the parents also carry a Z allele. It took time to explain to the family that they, too, have a gene mutation. Many affected individuals only perceive themselves as PiMZ carriers. And they might even be bakers, grinders, or smokers. We want to emphasize that this is not a good thing. As Dr. Kardorff explained, even a PiMZ has a slightly higher risk of lung disease (i.e., higher than in MM) because the AAT level in the blood is lower than in the normal variant, PiMM. AAT levels can be high or low within the reference range, but a PiMZ can also have a quite low concentration of AAT in their blood. That's why it's important for PiMZ individuals to know their serum levels.<\/p>\n

In Germany, approximately 1,538,000 people with multiple myeloma (MZ) and 12,000 people with multiple myeloma (ZZ) are affected. To illustrate: Statistically, on a bus with 50 people, there is one person with multiple myeloma (PiMZ). The number of people with multiple myeloma (PiMZ) is increasing worldwide. And even if only a few of these individuals develop pulmonary emphysema, this still represents more than the total number of people with multiple myeloma (PiZZ).<\/p>\n

The health significance for PiMZ<\/span><\/h2>\n