Publications by authors named "J Hammermann"

Cystic Fibrosis (CF) is the most common autosomal recessive genetic multisystemic disease. In Germany, it affects at least 8000 people. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel.

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Article Synopsis
  • Many clinical guidelines suggest chronic inhaled therapy for people with cystic fibrosis (pwCF) who have chronic Pseudomonas aeruginosa lung infections.
  • A study analyzed inhaled antibiotic therapies in Germany's CF Registry in 2020, finding that nearly 90% of pwCF with chronic P. aeruginosa infections received inhaled antibiotics, with colistin being the most commonly used.
  • In both children and adults, many patients alternated between different antibiotics, with a notable interest in how new modulators like elexacaftor/tezacaftor/ivacaftor may impact future therapy choices.
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Objectives: Sweat chloride testing (SCT) is the mainstay for the diagnosis of cystic fibrosis (CF) and biomarker in the evaluation of CFTR-modifying drugs. To be a reliable and valid tool, analytical variance (CVA) must be minimized. However, external quality assessments have revealed significant deviations in routine clinical practice.

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There are currently four countries and one local region in Europe that use PAP in their newborn screening programme. The first country to employ PAP at a national level was the Netherlands, which started using IRT/PAP/DNA/EGA in 2011. Germany followed in 2016 with a slightly different IRT/PAP/DNA strategy.

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