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The purpose of this study was to assess the frequency, severity, and clinical associations of dural ectasia (DE) in Loeys-Dietz syndrome (LDS). Database analysis of three German metropolitan regions identified 30 patients with LDS and TGFBR1 mutation in 6 and a TGFBR2 mutation in 24 individuals (17 men; mean age: 31 ± 19 years), as well as 60 age and sex-matched control patients with Marfan syndrome carrying a FBN1 mutation. DE was present in 22 patients with LDS (73%), and it related to skeletal score points (p = 0.008), non-skeletal score points (p < 0.001), and to the presence of ≥7 systemic score points (p = 0.010). Similarly, the severity of DE was related to body height (p = 0.010) and non-skeletal score points (p = 0.004). Frequency (p = 0.131) and severity of DE (p = 0.567) was similar in LDS and Marfan syndrome. DE is a manifestation of LDS that occurs with similar frequency and severity as in Marfan syndrome. Severity of DE may serve as a marker of the overall connective tissue disease severity. LDS may be considered in patients with DE. [ABSTRACT FROM AUTHOR]

Approximately 15,000 tons of wild boar meats ( Sus scrofa) are consumed per year in Germany. Boar meat therefore plays a definite role in regard to human diet. Because they are omnivores and because of their high body fat quotient, wild boar may accumulate large concentrations of persistent organic compounds, such as halogenated hydrocarbons, and could thus possibly serve as bioindicators for persistent xenobiotics. In addition, consumption of wild boar meat and liver could lead to increased contaminant levels in humans. Between 2007 and 2009, we tested a total of 529 livers and 506 muscle tissue samples from wild boar for the presence of perfluorooctanoic acid (PFOA) and perfluorooctane sulfonate (PFOS). PFOA concentrations ≤45 μg/kg and PFOS concentrations ≤1,780 μg/kg were detected in the liver samples. PFOA concentrations ≤7.4 μg/kg and PFOS concentrations ≤28.6 μg/kg were detected in muscle tissue. Our results show that PFOS may be detected in considerably greater concentrations than PFOA in organs and tissues, which is in agreement with results from other published studies. The comparisons between both organs for the same substance, as well as the comparisons between the substances within an organ, showed clear and statistically significant differences at P < 0.0001. Assuming a tolerable daily intake value of PFOA (1.5 μg/kg bw/d) and PFOS (0.15 μg/kg bw/d) as recommended by the European Food Safety Authority, the results of model calculations based on the maximum concentrations of PFOA and PFOS found in wild boar indicate that there should be no PFC-related health danger resulting from moderate consumption of wild boar meat or liver. [ABSTRACT FROM AUTHOR]

The prevalence of varicocele was estimated among pupils of forms 4 and 8 in the city of Hamburg and the severity of the associated venous reflux was analysed. In the school year of 1998/99, a genital examination was performed on 2756 children (median age 10.2 years) and 2008 adolescents (14.6 years). The varicocele degree was determined according to the WHO. Venous reflux was proven by CW-Doppler sonography, distinguishing between Valsalva-induced reflux (VR) and continuous reflux (CR). As a result, varicoceles were detected in 18.0% of the children (1.2% bilaterally) and 42.7% of the adolescents (7.2%). High-graded forms (palpable/visible) occurred with increasing rate (from 7% to 22.9%) on the left side. Subtle forms (subclinical/during Valsalva) counted for >90% of all right-sided findings, whilst an age-related shift towards higher degrees was noted left-sided. VR occurred bilaterally, CR was almost only established left-sided. VR was mainly associated with subtle varicoceles, CR was predominantly found in the high-graded forms. These results suggest that even in children varicoceles are not a rare phenomenon. However, adolescence is the main period of manifestation. A major venous malfunction is already evident in maturing boys, which seems to be associated with the formation of high-graded varicoceles. [ABSTRACT FROM AUTHOR]

Plum decline was associated with Pseudomonas syringae pathovars syringae and morsprunorum in Baden-Württemberg. The trunks of affected plum trees ( Prunus domestica) were girdled by bacterial cankers resulting in sudden death of infected trees. Copper compounds that were applied extensively during leaf fall and bud burst, were not effective. A minority of P. syringae strains isolated from cankers on plum trees were moderately resistant, while most strains were sensitive to cupric ions. Invasions through blossoms, leaves and wounds during the vegetation period were limited to the infection sites and plum trees coped effectively with both P. syringae pathovars eliminating them eventually. Infections after dormancy including very rare leaf scar infections did not induce cankers on the trunk. However, infections of dormant trees through frost injuries, (pruning) wounds or non-injurious ingress by freezing and thawing were serious, because they led to cankers girdling the trunk. Control strategies to manage plum decline have to be adapted to the disease cycle. They should concentrate on the dormant period beginning with early frosts in autumn and ending with bud burst. [ABSTRACT FROM AUTHOR]

Recent studies have found a higher prevalence of allergic rhinitis and atopic sensitization among adults living in eastern than those living in western Germany. We hypothesize that prevalence rates were similar before Germany was divided and diverged after the division. Because there are no historical data comparing atopic status between the two parts of Germany, we tested this hypothesis by comparing the prevalence of atopy among persons who were born during different decades. As part of the EC Respiratory Health Survey, a respiratory health questionnaire was mailed to a population-based sample of 8363 subjects aged 20-44 years from a city in the former West Germany (Hamburg) and a city in the former East Germany (Erfurt). Of the target population. 6428 (77%) subjects responded. Subsamples of 731 subjects from Erfurt and 1159 subjects from Hamburg participated in medical examinations, including skin prick tests and specific IgE measurements. Prevalence rates of allergic sensitization were similar in Hamburg and Erfurt for those born in the periods 1946-51 and 1952-61. respectively, but differed between Hamburg and Erfurt subjects born in the period 1962-71. After adjustment for several potential predictors, the younger subjects from Hamburg had a higher odds ratio (OR) of sensitization than those Hamburg subjects born before 1952 (skin prick test reactivity: OR 2.06. any specific IgE > 0.35 kU/1: OR 1.61). The younger subjects from Erfurt were not more frequently sensitized than the older subjects (skin prick test reactivity: OR 1.05. any specific IgE > 0.35 kU/1: OR 0.79). No single allergen could be identified as responsible for the observed difference. We conclude that factors related to a "Western lifestyle", which were prevalent in the West German city during the 1960s and 1970s. may be responsible for the higher prevalence of allergic sensitization observed in Hamburg. [ABSTRACT FROM AUTHOR]

Atypical EGFR mutations occur in 10%-30% of non-small-cell lung cancer (NSCLC) patients with EGFR mutations and their sensitivity to classical epidermal growth factor receptor (EGFR)-tyrosine kinase inhibitors (TKI) is highly heterogeneous. Patients harboring one group of uncommon, recurrent EGFR mutations (G719X, S768I, L861Q) respond to EGFR-TKI. Exon 20 insertions are mostly insensitive to EGFR-TKI but display sensitivity to exon 20 inhibitors. Clinical outcome data of patients with very rare point and compound mutations upon systemic treatments are still sparse to date. In this retrospective, multicenter study of the national Network Genomic Medicine (nNGM) in Germany, 856 NSCLC cases with atypical EGFR mutations including co-occurring mutations were reported from 12 centers. Clinical follow-up data after treatment with different EGFR-TKIs, chemotherapy and immune checkpoint inhibitors were available from 260 patients. Response to treatment was analyzed in three major groups: (i) uncommon mutations (G719X, S7681, L861Q and combinations), (ii) exon 20 insertions and (iii) very rare EGFR mutations (very rare single point mutations, compound mutations, exon 18 deletions, exon 19 insertions). Our study comprises the largest thus far reported real-world cohort of very rare EGFR single point and compound mutations treated with different systemic treatments. We validated higher efficacy of EGFR-TKI in comparison to chemotherapy in group 1 (uncommon), while most exon 20 insertions (group 2) were not EGFR-TKI responsive. In addition, we found TKI sensitivity of very rare point mutations (group 3) and of complex EGFR mutations containing exon 19 deletions or L858R mutations independent of the combination partner. Notably, treatment responses in group 3 (very rare) were highly heterogeneous. Co-occurring TP53 mutations exerted a non-significant trend for a detrimental effect on outcome in EGFR-TKI-treated patients in groups 2 and 3 but not in group 1. Based on our findings, we propose a novel nNGM classification of atypical EGFR mutations. • The EGFR-TKI efficacy of in uncommon EGFR mutations generally considered TKI sensitive was validated. • Complex EGFR mutations containing exon19del or L858R were EGFR-TKI sensitive, independent of the combination partner. • Very rare single and complex mutations, comprising 40% of atypical mutations, show a high heterogeneity of TKI sensitivity. • TP53 co-mutations may confer a detrimental outcome in TKI-treated patients with very rare EGFR mutations and exon20ins. • We propose a novel nNGM classification for atypical EGFR mutations. [ABSTRACT FROM AUTHOR]