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ZusammenfassungDie Tuberkulose ist in Deutschland eine seltene, überwiegend gut behandelbare Erkrankung. Weltweit ist sie eine der häufigsten Infektionserkrankungen mit ca. 10 Millionen Neuerkrankungen/Jahr. Auch bei einer niedrigen Inzidenz in Deutschland bleibt Tuberkulose insbesondere aufgrund der internationalen Entwicklungen und Migrationsbewegungen eine wichtige Differenzialdiagnose. In Deutschland besteht, aufgrund der niedrigen Prävalenz der Erkrankung und der damit verbundenen abnehmenden klinischen Erfahrung, ein Informationsbedarf zu allen Aspekten der Tuberkulose und ihrer Kontrolle. Diese Leitlinie umfasst die mikrobiologische Diagnostik, die Grundprinzipien der Standardtherapie, die Behandlung verschiedener Organmanifestationen, den Umgang mit typischen unerwünschten Arzneimittelwirkungen, die Besonderheiten in der Diagnostik und Therapie resistenter Tuberkulose sowie die Behandlung bei TB-HIV-Koinfektion. Sie geht darüber hinaus auf Versorgungsaspekte und gesetzliche Regelungen wie auch auf die Diagnosestellung und präventive Therapie einer latenten tuberkulösen Infektion ein. Es wird ausgeführt, wann es der Behandlung durch spezialisierte Zentren bedarf.Die Aktualisierung der S2k-Leitlinie „Tuberkulose im Erwachsenenalter“ soll allen in der Tuberkuloseversorgung Tätigen als Richtschnur für die Prävention, die Diagnose und die Therapie der Tuberkulose dienen und helfen, den heutigen Herausforderungen im Umgang mit Tuberkulose in Deutschland gewachsen zu sein.

RationaleIn 2016, a new interferon-gamma release assay (IGRA) was introduced, QuantiFERON-TB Gold Plus (QFT-Plus), claimed to have improved sensitivity in active tuberculosis (TB).ObjectivesThis study aimed to determine the performance of QFT-Plus, compared with previous generation IGRAs and the tuberculin skin test (TST), in children with TB in Europe.MethodsMulticentre, ambispective cohort study within the Paediatric Tuberculosis Network European Trials Group (ptbnet), a dedicated paediatric TB research network comprising >300 members, capturing TB cases Measurements and main results1001 TB cases from 16 countries were included (mean age (IQR) 5.6 (2.4–12.1) years). QFT-Plus was performed in 358, QFT Gold in-Tube (QFT-GIT) in 600, T-SPOT.TBin 58 and TST in 636 cases. The overall test sensitivities were: QFT-Plus 83.8% (95% CI 80.2% to 87.8%), QFT-GIT 85.5% (95% CI 82.7% to 88.3%), T-SPOT.TB77.6% (95% CI 66.9% to 88.3%) and TST (cut-off ≥10 mm) 83.3% (95% CI 83.3% to 86.2%). There was a trend for tests to have lower sensitivity in patients with miliary and/or central nervous system (CNS) TB (73.1%, 70.9%, 63.6% and 43.5%, respectively), and in immunocompromised patients (75.0%, 59.6%, 45.5% and 59.1%, respectively).ConclusionsThe results indicate that the latest generation IGRA assay, QFT-Plus, does not perform better than previous generation IGRAs or the TST in children with TB disease. Overall, tests performed worse in CNS and miliary TB, and in immunocompromised children. None of the tests evaluated had sufficiently high sensitivity to be used as a rule-out test in children with suspected TB.

ZusammenfassungDas vorliegende Konsensuspapier bietet in Ergänzung zur AWMF-S1-Leitlinie eine Übersicht über die verschiedenen klinischen Aspekte von Long COVID im Kindes- und Jugendalter. Es wurde von Vertreter:innen aus 19 Fachgesellschaften des DGKJ-Konvents und kooperierenden Fachgesellschaften erstellt und bietet Expertenempfehlungen für die Praxis auf Grundlage der bisherigen, noch geringen studienbasierten Evidenz zu Long COVID im Kindes- und Jugendalter. Es enthält Screeningfragen zu Long COVID sowie einen Vorschlag zur strukturierten, standardisierten pädiatrischen Anamnese und zur diagnostischen Evaluation bei V. a. Long COVID. Dazu werden ein zeit- und ressourcensparender Erfassungsbogen, der die Komplexität des Krankheitsbildes berücksichtigt, über die Internetseiten der DGKJ und DGPI zur Verfügung gestellt und weitere Fragebögen zur Abklärung von spezifischen neurokognitiven und/oder psychischen Störungen sowie post-exertioneller Malaise (PEM) und myalgischer Enzephalomyelitis/chronischem Fatigue-Syndrom (ME/CFS) benannt. Anhand der jeweiligen anamnestisch und klinisch ermittelten Hauptsymptome werden ein gestuftes, diagnostisches Vorgehen und eine multidisziplinäre Betreuung empfohlen.

Zusammenfassung. Mit der COVID-19-Pandemie ergeben sich nach aktuellem Wissensstand vermutlich weniger prävalent somatische als vielmehr psychische Folgeerkrankungen im Kindes- und Jugendalter. Diese sind jedoch umso alarmierender, da sie auf ein schon vor der Pandemie stark belastetes System treffen. Auch zu beachten sind die bislang unklaren Folgen für die allgemeine psychische, soziale und emotionale Entwicklung, da beispielsweise Lernräume mit Gleichaltrigen langfristig fehlten oder nur eingeschränkt zugänglich waren. Mit der Betrachtung von Kindern und Jugendlichen selbst ist auch das nähere Bezugssystem von Eltern und Familie relevant, um dort Schutz- und Risikofaktoren zu identifizieren. In der Gesamtschau des aktuellen Themenschwerpunkts wie auch weiterer Studien ergibt sich die Notwendigkeit, Prävention und Frühintervention auszubauen und den Zugang zum Gesundheitssystem für betroffene Kinder, Jugendliche und Familien zu verbessern.

Background: Given the crucial role of vaccination in managing and halting the COVID-19 pandemic, it is imperative to understand the factors that motivate adolescents to get vaccinated. However, data on this topic are currently limited. This study aimed to characterize the adolescent population willing to receive COVID-19 vaccination in mid-2021 in an urban region in Germany and to identify the factors that motivated those who accepted vaccination with the mRNA vaccine Comirnaty, which was not yet nationally recommended. Methods: We surveyed adolescents and their accompanying guardians who were scheduled to receive COVID-19 vaccination and collected data on sociodemographic characteristics, the adolescent's medical history, the family's vaccination status, and any history of COVID-19 infection in the family. We also queried information strategies related to the SARS-CoV-2 pandemic and reasons for vaccinating the adolescents. Results: Motivations for getting vaccinated were similar among adolescents and their parents. The primary reasons for vaccination were to protect against SARS-CoV-2-related illness and to gain access to leisure facilities. The adolescents' motivation for vaccination was not influenced by gender, health status, migration background, or presence of chronic or acute diseases. The percentage of parents who had received SARS-CoV-2 immunization and the proportion of parents with a high level of education were higher among study participants than in the general population. Conclusions: Adolescents are especially willing to be vaccinated if they have a better-educated environment and a high vaccination rate in the family. Emphasizing the importance of vaccination among all segments of population and removing barriers to vaccines, may lead to an ameliorated acceptance of COVID-19 vaccines.

Most individuals with rare diseases initially consult their primary care physician. For a subset of rare diseases, efficient diagnostic pathways are available. However, ultra-rare diseases often require both expert clinical knowledge and comprehensive genetic diagnostics, which poses structural challenges for public healthcare systems. To address these challenges within Germany, a novel structured diagnostic concept, based on multidisciplinary expertise at established university hospital centers for rare diseases (CRDs), was evaluated in the three year prospective study TRANSLATE NAMSE. A key goal of TRANSLATE NAMSE was to assess the clinical value of exome sequencing (ES) in the ultra-rare disease population. The aims of the present study were to perform a systematic investigation of the phenotypic and molecular genetic data of TRANSLATE NAMSE patients who had undergone ES in order to determine the yield of both ultra-rare diagnoses and novel gene-disease associations; and determine whether the complementary use of machine learning and artificial intelligence (AI) tools improved diagnostic effectiveness and efficiency.ES was performed for 1,577 patients (268 adult and 1,309 pediatric). Molecular genetic diagnoses were established in 499 patients (74 adult and 425 pediatric). A total of 370 distinct molecular genetic causes were established. The majority of these concerned known disorders, most of which were ultra-rare. During the diagnostic process, 34 novel and 23 candidate genotype-phenotype associations were delineated, mainly in individuals with neurodevelopmental disorders.To determine the likelihood that ES will lead to a molecular diagnosis in a given patient, based on the respective clinical features only, we developed a statistical framework called YieldPred. The genetic data of a subcohort of 224 individuals that also gave consent to the computer-assisted analysis of their facial images were processed with the AI tool Prioritization of Exome Data by Image Analysis (PEDIA) and showed superior performance in variant prioritization.The present analyses demonstrated that the novel structured diagnostic concept facilitated the identification of ultra-rare genetic disorders and novel gene-disease associations on a national level and that the machine learning and AI tools improved diagnostic effectiveness and efficiency for ultra-rare genetic disorders.

BackgroundAvailable data on aerosol emissions among children and adolescents during spontaneous breathing are limited. Our aim was to gain insight into the role of children in the spread of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and whether aerosol measurements among children can be used to help detect so-called superspreaders—infected individuals with extremely high numbers of exhaled aerosol particles.MethodsIn this prospective study, the aerosol concentrations of SARS-CoV-2 PCR-positive and SARS-CoV-2 PCR-negative children and adolescents (2–17 years) were investigated. All subjects were asked about their current health status and medical history. The exhaled aerosol particle counts of PCR-negative and PCR-positive subjects were measured using the Resp-Aer-Meter (Palas GmbH, Karlsruhe, Germany) and compared using linear regression.ResultsA total of 250 children and adolescents were included in this study, 105 of whom were SARS-CoV-2 positive and 145 of whom were SARS-CoV-2 negative. The median age in both groups was 9 years (IQR 7–11 years). A total of 124 (49.6%) participants were female, and 126 (50.4%) participants were male. A total of 81.9% of the SARS-CoV-2-positive group had symptoms of viral infection. The median particle count of all individuals was 79.55 particles/liter (IQR 44.55–141.15). There was a tendency for older children to exhale more particles (1–5 years: 79.54 p/L; 6–11 years: 77.96 p/L; 12–17 years: 98.63 p/L). SARS-CoV-2 PCR status was not a bivariate predictor (t = 0.82, p = 0.415) of exhaled aerosol particle count; however, SARS-CoV-2 status was shown to be a significant predictor in a multiple regression model together with age, body mass index (BMI), COVID-19 vaccination, and past SARS-CoV-2 infection (t = 2.81, p = 0.005). COVID-19 vaccination status was a highly significant predictor of exhaled aerosol particles (p ConclusionDuring SARS-CoV-2 infection, children and adolescents did not have elevated aerosol levels. In addition, no superspreaders were found.

Zusammenfassung Hintergrund Zystische Fibrose (CF) ist eine der häufigsten vererbbaren Stoffwechselerkrankungen in Deutschland. Sie betrifft verschiedene Organsysteme und führt häufig zu Schmerzen, die meist unterschätzt werden. Im deutschsprachigen Raum existieren bis dato keinerlei Leitlinien zu diesem relevanten Thema. Methoden Es erfolgte eine systematische Literaturrecherche in PubMed sowie der deutschsprachigen Leitlinien und Fachliteratur zum Thema CF und Schmerzen. Als relevant wurden Arbeiten gewertet, die Daten zu CF, zu Schmerzen oder deren Behandlung bei der CF enthielten. Insgesamt fanden sich 1690 Artikel, von denen 41 Arbeiten unsererseits aufgenommen wurden. Ergebnisse Die Recherche ergab, dass Schmerzen unterschiedlichster Art aufgrund der diversen Organbeteiligungen weit verbreitet sind. Zur Diagnostik und Therapie von Schmerzen bei CF existieren aber keine deutschsprachigen Leitlinien oder Konsenspapiere. In europäischen und amerikanischen Studien spiegelt sich die klinische Relevanz und Bandbreite des Themas wider, Daten zur Effektivität der Schmerztherapie enthalten diese jedoch nicht. Neben einer klassischen Schmerzmedikation mit Analgetika setzen viele Patienten auch auf alternative Heilversuche wie physikalische Therapien, Akupunktur oder Tetrahydrocannabinol (THC). Angesichts der multiplen Organdysfunktionen ist der Einsatz von Analgetika mit besonderen Risiken verbunden. Diskussion Eine Schmerztherapie ist bei Patienten mit zystischer Fibrose und Multiorganbeteiligung häufig erforderlich und muss der Progression der Erkrankung angepasst werden. Kontraindikationen und Nebenwirkungen der Analgetika sollten individuell ermittelt werden. Die Aufnahme der Behandlung von Schmerzen in deutschsprachige CF-Leitlinien ist dringend erforderlich.

Despite the need to generate valid and reliable estimates of protection against SARS-CoV-2 infection and severe course of COVID-19 for the German population in summer 2022, there was a lack of systematically collected population-based data allowing for the assessment of the protection level in real-time.In the IMMUNEBRIDGE project, we harmonised data and biosamples for nine population-/hospital-based studies (total number of participants n=33,637) to provide estimates for protection levels against SARS-CoV-2 infection and severe COVID-19 between June and November 2022. Based on evidence synthesis, we formed a combined endpoint of protection levels based on the number of self-reported infections/vaccinations in combination with nucleocapsid/spike antibody responses (“confirmed exposures”). Four confirmed exposures represented the highest protection level, and no exposure represented the lowest.Most participants were seropositive against the spike antigen; 37% of the participants ≥79 years had less than four confirmed exposures (highest level of protection) and 5% less than three. In the subgroup of participants with comorbidities, 46-56% had less than four confirmed exposures. We found major heterogeneity across federal states, with 4%-28% of participants having less than three confirmed exposures.Using serological analyses, literature synthesis and infection dynamics during the survey period, we observed moderate to high levels of protection against severe COVID-19, whereas the protection against SARS-CoV-2 infection was low across all age groups. We found relevant protection gaps in the oldest age group and amongst individuals with comorbidities, indicating a need for additional protective measures in these groups.

[Background]: Currently, data on treatment, outcome, and prognostic factors in children with tuberculous meningitis (TBM) in Europe are limited. To date, most existing data on TBM originate from adult studies, or studies conducted in low-resource settings., [Methods]: We designed a multicenter, retrospective study involving 27 pediatric healthcare institutions in 9 European countries via an established pediatric TB research network, before and after the 2014 revision of World Health Organization (WHO) dosing recommendations., [Results]: Of 118 children, 39 (33.1%) had TBM grade 1, 68 (57.6%) grade 2, and 11 (9.3%) grade 3. Fifty-eight (49.1%) children received a standard 4-drug treatment regimen; other commonly used drugs included streptomycin, prothionamide, and amikacin. Almost half of the patients (48.3%; 56/116) were admitted to intensive care unit, with a median stay of 10 (interquartile range [IQR] 4.5–21.0) days. Of 104 children with complete outcome data, 9.6% (10/104) died, and only 47.1% (49/104) recovered fully. Main long-term sequelae included spasticity of 1 or more limbs and developmental delay both in 19.2% (20/104), and seizure disorder in 17.3% (18/104). Multivariate regression analyses identified microbiological confirmation of TBM, the need for neurosurgical intervention, and mechanical ventilation as risk factors for unfavorable outcome., [Conclusions]: There was considerable heterogeneity in the use of TB drugs in this cohort. Despite few children presenting with advanced disease and the study being conducted in a high-resource setting, morbidity and mortality were high. Several risk factors for poor outcome were identified, which may aid prognostic predictions in children with TBM in the future., TPtbnet is supported by the Deutsche Gesellschaft für Internationale Zusammenarbeit. M. Te. was supported by a Clinical Lectureship provided by the UK National Institute for Health Research (NIHR), and by a grant from the Technology Strategy Board/Innovate UK. R. B. R. is funded by an NIHR Academic Clinical Lectureship at the London School of Hygiene & Tropical Medicine (grant number CL-2018-20-001). A. N.-J. was supported by Subvencions per a la Intensificació de Facultatius Especialistes (Departament de Salut de la Generalitat de Catalunya, Programa PERIS 2016–2020; ref. SLT008/18/00193). O. N. is supported by Consejeria de Salud y Familias, Servicio Andaluz de Salud 2020 (grant number A1-0049-2020).

Zusammenfassung Hintergrund Die zystische Fibrose (CF) ist eine multisystemische progrediente Stoffwechselerkrankung mit vorwiegend abdomineller und pulmonaler Beteiligung. Schmerzen sind für Betroffene ein weiteres komplexes und von den Behandlern unterschätztes Problem. Methoden Eine Literaturrecherche deutschsprachiger Leitlinien und englischsprachiger Studien zum Thema CF und Schmerzen wurde durchgeführt, zusätzlich die Beobachtungen zur Diagnostik und Therapie eines CF-Patienten mit progredienten thorakalen Schmerzen ausgewertet. Ergebnisse Die Recherche ergab, dass zur Diagnostik und Therapie thorakaler Schmerzen bei CF keine deutschsprachigen Leitlinien oder Konsenspapiere existieren. Die europäischen und amerikanischen Erhebungen zeigen aber die große Relevanz des Themas und postulieren einen Zusammenhang von Schmerzintensität mit einer erhöhten Mortalität. Sie enthalten jedoch keine Daten zur Effektivität der Schmerztherapie. Anhand dieser Daten und des Fallberichts eines jungen CF-Patienten mit stärksten Thoraxschmerzen bei pulmonalen Exazerbationen lassen sich die CF-spezifischen Herausforderungen der Schmerztherapie illustrieren. Neben den Schmerzen an sich sind auch Analgetika angesichts der multiplen Organdysfunktionen mit besonderen Risiken wie gastrointestinalen Blutungen, opioidinduzierter Atemdepression oder opioidinduzierter Obstipation verbunden. Diskussion Schmerztherapie bei Patienten mit zystischer Fibrose und Multiorganbeteiligung erfordert ein sorgfältiges Monitoring und interdisziplinäres Handeln. Empfehlungen zum Schmerzmanagement sollten in die deutschsprachigen CF-Leitlinien aufgenommen werden.

ZusammenfassungDie Deutsche Gesellschaft für Pneumologie hat die AWMFS1-Leitlinie Post-COVID/Long-COVID initiiert. In einem breiten interdisziplinären Ansatz wurde diese S1-Leitlinie basierend auf dem aktuellen Wissensstand gestaltet.Die klinische Empfehlung beschreibt die aktuellen Post-COVID/Long-COVID-Symptome, diagnostische Ansätze und Therapien.Neben der allgemeinen und konsentierten Einführung wurde ein fachspezifischer Zugang gewählt, der den aktuellen Wissensstand zusammenfasst.Die Leitlinie hat einen expilzit praktischen Anspruch und wird basierend auf dem aktuellen Wissenszugewinn vom Autorenteam stetig weiterentwickelt und adaptiert.

Background: Nowadays physical activity (PA)/exercise is an important component of cystic fibrosis (CF) therapy. The aim of the study was to assess the barriers to PA and the barrier management and to explore the effect of supervision on the barriers and barrier management during an exercise program. Methods: In total, 88 people with CF (pwCF) of the ages 6 to 50 years old (mean 24.2 ± 7.9 yrs) participated in the partially supervised 12-month exercise program and filled in a structured and validated questionnaire about barriers to sports and barrier management at baseline. Additionally, 23 pwCF filled in the questionnaire after 6 months and 12 months. The items were clustered into physical and psychosocial barriers and into preventive counter strategies and situational counter strategies and analyzed at baseline and over time. Results: Physical barriers were more relevant than psychosocial barriers and no trend could be seen in the situational and preventive counter strategies. When divided in subgroups, the less active pwCF (7500 steps/day), physical barriers, and psychosocial barriers showed no significant differences. However physical barriers showed a tendency to have a higher value in the less active group compared to the more active group (p > 0.05). Stratified by age or FEV1%pred between the subgroups, no differences could be seen regarding barriers and counter strategies. Conclusions: Physical barriers seemed to have a higher priority when it comes to not participating in PA/exercise. Supervision over 6 months during an exercise program did not show a beneficial effect on barriers and barrier management. Besides the motivational aspect of sport counselling, the volitional aspect seemed to be more important to incorporate more PA into daily life. Individual barriers and their concrete counter strategies should be discussed with the patient with CF. Sport counselling is needed permanently and should be part of the CF routine care.

Introduction Socio-economic and ethnic background have been discussed as possible risk factors for SARS-CoV-2 infections in children. Improved knowledge could lead to tailored prevention strategies and help improve infection control. Aim We aimed to identify risk factors for SARS-CoV-2 infections in children in the first and second wave of the pandemic. Methods We performed an observational population-based cohort study in children (6 months–18 years) scheduled for legally required preventive examination and their parents in a metropolitan region in Germany. Primary endpoint was the SARS-CoV-2 seroconversion rate during the study period. Risk factors assessed included age, pre-existing medical conditions, socio-economic factors and ethnicity. Results We included 2,124 children and their parents. Seroconversion rates among children in all age groups increased 3–4-fold from June 2020 to February 2021. Only 24 of 58 (41%) seropositive children reported symptoms. In 51% of infected children, at least one parent was also SARS-CoV-2-positive. Low level of parental education (OR = 3.13; 95% CI: 0.72–13.69) non-significantly increased the risk of infection. Of the total cohort, 38.5% had a migration background, 9% of Turkish and 5% of Middle Eastern origin, and had the highest risk for SARS-CoV-2 infections (OR = 6.24; 95% CI: 1.38–28.12 and OR = 6.44 (95% CI: 1.14–36.45) after adjustment for other risk factors. Conclusion In the second half of 2020, seroprevalence for SARS-CoV-2 in children increased especially in families with lower-socioeconomic status. Culture-sensitive approaches are essential to limit transmission and could serve as a blueprint for vaccination strategies.

Previous studies showed that the lung clearance index (LCI) determined by multiple-breath washout (MBW) is sensitive to detecting early lung disease in preschool children with cystic fibrosis (CF). In preschool children with primary ciliary dyskinesia (PCD), data on the onset and severity of lung disease and on the sensitivity of the LCI as a noninvasive quantitative outcome measure remain limited.Is MBW feasible and sensitive to detect ventilation inhomogeneity in preschool children with PCD?This was a prospective, cross-sectional, multicenter study and included preschoolers with PCD, preschoolers with CF, and healthy control (HC) participants. LCI was determined using nitrogen MBW and was compared among the three groups.LCI was determined in 27 children with PCD, 34 children with CF, and 30 HC participants (mean age, 4.8 years; range, 2.2-6.9 years). The LCI in preschool children with PCD was increased (median, 9.1; 95% CI, 8.6-10.3) compared with HC participants (median, 7.0; 95% CI, 6.7-7.1; P .0001), but did not differ from preschool children with CF (median, 8.6; 95% CI, 8.4-9.7; P = .71). The feasibility in the PCD group was 93.1% and was similar to that in the CF group (91.9%) and in HC participants (85.7%; P = .55).This study demonstrated early onset of lung disease in preschool children with PCD and indicated that lung disease severity in PCD may be similar to that in CF during preschool years. These data support a need for early diagnostic monitoring and therapy and suggest the LCI as a noninvasive diagnostic tool and as a potential end point in clinical trials testing early interventions in children with PCD.

Studies in recent years and especially since the beginning of the COVID-19 pandemic have shown a significant increase in the problematic use of computer games and social media. Adolescents having difficulties in regulating their unpleasant emotions are especially prone to Problematic Internet Use (PIU), which is why emotion dysregulation has been considered a risk factor for PIU. The aim of the present study was to assess problematic internet use (PIU) in adolescents after the third wave (nearly 1.5 years after the onset in Europe) of the COVID-19 pandemic. In the German region of Siegen-Wittgenstein, all students 12 years and older from secondary-level schools, vocational schools and universities were offered a prioritized vaccination in August 2021 with an approved vaccine against COVID-19. In this context, the participants filled out the Short Compulsive Internet Use Scale (SCIUS) and two additional items to capture a possible change in digital media usage time and regulation of negative affect due to the COVID-19 pandemic. A multiple regression analysis was performed to identify predictors of PIU. The original sample consisted of 1477 participants, and after excluding invalid cases the final sample size amounted to 1268 adolescents aged 12–17 (x = 14.37 years, SD = 1.64). The average prevalence of PIU was 43.69%. Gender, age, digital media usage time and the intensity of negative emotions during the COVID-19 pandemic were all found to be significant predictors of PIU: female gender, increasing age, longer digital media usage time and higher intensity of negative emotions during the COVID-19 pandemic were associated with higher SCIUS total scores. This study found a very high prevalence of PIU among 12- to 17-year-olds for the period after the third wave of the COVID-19 pandemic, which has increased significantly compared to pre-pandemic prevalence rates. PIU is emerging as a serious problem among young people in the pandemic. Besides gender and age, pandemic-associated time of digital media use and emotion regulation have an impact on PIU, which provides starting points for preventive interventions.

ZusammenfassungDie vorliegende Fassung der deutschsprachigen AWMF-Leitlinie soll unter Berücksichtigung der vorliegenden Evidenz die medizinische Versorgung von Kindern und Jugendlichen mit ambulant erworbener Pneumonie (pediatric community acquired pneumonia, pCAP) verbessern. In Mitteleuropa steht einer Prävalenz von ca. 300 Fällen pro 100 000 Kinder/Jahr eine sehr geringe Mortalität gegenüber, die Prävention umfasst Hygiene-Maßnahmen und Impfung z. B. gegen Pneumokokken, Hämophilus, Masern und Influenza. Hauptsymptome der pCAP sind Fieber und Tachypnoe, die Diagnosestellung erfolgt primär klinisch durch Anamnese, körperliche Untersuchung und Pulsoxymetrie. Das zusätzliche Vorliegen von Warnsymptomen wie stark reduzierter Allgemeinzustand, Nahrungsverweigerung, Dehydratation, Bewusstseinsstörung oder Krampfanfälle definiert die schwere pCAP in Abgrenzung zur nicht-schweren pCAP. Das Erregerspektrum ist altersabhängig, zur Differenzierung zwischen viraler, bakterieller oder gemischt viral-bakterieller Infektion stehen jedoch keine zuverlässigen Biomarker zur Verfügung. Die meisten Kinder und Jugendlichen mit nicht-schwerer pCAP und O2-Sättigung > 92 % können ohne weitere Röntgen-, Labor- und Erreger-Diagnostik ambulant betreut werden. Der Einsatz von Antiinfektiva ist nicht grundsätzlich indiziert, vor allem bei jungen Kindern, bronchialer Obstruktion und anderen Hinweisen auf virale Genese kann darauf i. d. R. verzichtet werden. Zur kalkulierten Antibiotika-Therapie sind Aminopenicilline Mittel der Wahl, bei gewährleisteter Einnahme und Resorption sind die orale (Amoxicillin) und intravenöse Verabreichung (Ampicillin) von vergleichbarer Wirksamkeit. Nach 48 – 72 Stunden ist eine Verlaufsbeurteilung notwendig, um den Behandlungserfolg und mögliche Komplikationen wie z. B. parapneumonische Ergüsse oder Pleuraempyeme, die eine Erweiterung bzw. Änderung der Therapie erforderlich machen, rechtzeitig zu erfassen.

BackgroundPersistent respiratory symptoms after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in adults are frequent, and there can be long-term impairment of pulmonary function. To date, only preliminary evidence is available on persistent respiratory sequelae of SARS-CoV-2 in children and adolescents. Our objective was to examine the long-term effects of symptomatic and asymptomatic SARS-CoV-2 infections on pulmonary function in this age group in a single-center, controlled, prospective study.MethodsParticipants with serological or polymerase chain reaction-based evidence of SARS-CoV-2 infection were recruited from a population-based study of seroconversion rates. Multiple-breath washout (MBW), body plethysmography, and diffusion capacity testing were performed for children and adolescents. Participants were interviewed about their symptoms during the acute phase of infection and long-lasting symptoms. Cases were compared with SARS-CoV-2 seronegative controls from the same population-based study with and without history of respiratory infection within 6 months prior to assessment. Primary endpoints were differences in pulmonary function, including diffusion capacity and MBW, between participants with and without evidence of SARS-CoV-2 infection. Secondary endpoints included correlation between lung function and long-lasting symptoms as well as disease severity.FindingsIn total, 73 seropositive children and adolescents (5–18 years) were recruited after an average of 2.6 months (range 0.4–6.0) following SARS-CoV-2 infection. Among 19 patients (27.1%) who complained of persistent or newly emerged symptoms since SARS-CoV-2, 8 (11.4%) reported respiratory symptoms. No significant differences were detected in frequency of abnormal pulmonary function when comparing cases with 45 controls, including 14 (31.1%) with a history of previous infection (SARS-CoV-2: 12, 16.4%; controls: 12, 27.7%; odds ratio 0.54, 95% confidence interval 0.22–1.34). Only two patients with persistent respiratory symptoms showed abnormal pulmonary function. Multivariate analysis revealed reduced forced vital capacity (p = 0.012) in patients with severe SARS-CoV-2 infection.InterpretationPulmonary function is rarely impaired in children and adolescents after SARS-CoV-2 infection, except from those with severe infection, and did not differ between SARS-CoV-2 and other previous infections, suggesting that SARS-CoV-2 is not more likely to cause pulmonary sequelae than other infections. The discrepancy between persisting respiratory symptoms and normal pulmonary function suggests a different underlying pathology such as dysfunctional breathing.

Background: From March – June 2021, the third wave of the COVID-19 pandemic occurred in Germany. First and second wave evaluations showed an increase in mental health problems and a reduction of quality of life in adolescents. In August 2021, two vaccines against COVID-19 were approved for adolescents aged 12-17 years in Germany. Aim of the study was to assess mental health and health-related quality of life (HRQoL) after 1.5 years of COVID-19 pandemic in adolescents who decided to receive a vaccination. Methods: In the German region of Siegen-Wittgenstein, all students from 12-17 years from secondary level schools, vocational schools and universities were offered a prioritized vaccination against COVID-19. Mental health and HRQoL were assessed at the first vaccination appointment by self- and parental report. Adolescents and their parents completed the Strength and Difficulties Questionnaire (SDQ) and the KIDSCREEN-10. Results: Data from 1412 adolescents (49.2% female, 47.7% male; mean age 14.3 years, SD=1.64) and 908 parents were collected. Mean self-reported HRQoL was T=53.7 (SD=11.2), in boys significantly higher than in girls (56.0 vs. 51.3), and significantly higher in younger (12-14 years) than in older (15-17 years) adolescents (56.2 vs. 51.5). Parental-reported HRQoL was T=51.8 (SD=12.8). In total, 18.5% of adolescents reported clinically relevant psychological symptoms, especially peer problems (23.7%), emotional problems (16.7%) and hyperactivity (17.2%). Girls reported significantly higher scores on emotional, peer and total problems than boys, whereas younger adolescents reported more hyperactivity and less emotional and peer problems than older ones. Comparing the present data to evaluations after the first and second wave of COVID-19 in Germany, adolescents facing vaccination after the third wave rated a higher HRQoL and reported less mental health problems. The data from the present study are comparable to those from pre-COVID evaluations. Conclusion: After 1.5 years of living with the pandemic, adolescents may have adapted to the changes in everyday life which has lowered the anticipated stress. Further, the relaxation of restrictions, better school organization and the prospect of the vaccination may have increased optimism, wellbeing and contentment, leading to declining but still alarming rates of psychological symptoms.

The German government initiated the Network University Medicine (NUM) in early 2020 to improve national research activities on the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic. To this end, 36 German Academic Medical Centers started to collaborate on 13 projects, with the largest being the National Pandemic Cohort Network (NAPKON). The NAPKON’s goal is creating the most comprehensive Coronavirus Disease 2019 (COVID-19) cohort in Germany. Within NAPKON, adult and pediatric patients are observed in three complementary cohort platforms (Cross-Sectoral, High-Resolution and Population-Based) from the initial infection until up to three years of follow-up. Study procedures comprise comprehensive clinical and imaging diagnostics, quality-of-life assessment, patient-reported outcomes and biosampling. The three cohort platforms build on four infrastructure core units (Interaction, Biosampling, Epidemiology, and Integration) and collaborations with NUM projects. Key components of the data capture, regulatory, and data privacy are based on the German Centre for Cardiovascular Research. By December 01, 2021, 34 university and 34 non-university hospitals have enrolled 4,241 patients with local data quality reviews performed on 2,812 (66%). 47% were female, the median age was 53 (IQR: 38-63)) and 3 pediatric cases were included. 30% of patients were hospitalized, 11% admitted to an intensive care unit, and 4% of patients deceased while enrolled. 7,143 visits with biosampling in 3,595 patients were conducted by November 29, 2021. In this overview article, we summarize NAPKON’s design, relevant milestones including first study population characteristics, and outline the potential of NAPKON for German and international research activities.Trial registration:· https://clinicaltrials.gov/ct2/show/NCT04768998· https://clinicaltrials.gov/ct2/show/NCT04747366· https://clinicaltrials.gov/ct2/show/NCT04679584

The German government initiated the Network University Medicine (NUM) in early 2020 to improve national research activities on the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic. To this end, 36 German Academic Medical Centers started to collaborate on 13 projects, with the largest being the National Pandemic Cohort Network (NAPKON). The NAPKON’s goal is creating the most comprehensive Coronavirus Disease 2019 (COVID-19) cohort in Germany. Within NAPKON, adult and pediatric patients are observed in three complementary cohort platforms (Cross-Sectoral, High-Resolution and Population-Based) from the initial infection until up to three years of follow-up. Study procedures comprise comprehensive clinical and imaging diagnostics, quality-of-life assessment, patient-reported outcomes and biosampling. The three cohort platforms build on four infrastructure core units (Interaction, Biosampling, Epidemiology, and Integration) and collaborations with NUM projects. Key components of the data capture, regulatory, and data privacy are based on the German Centre for Cardiovascular Research. By April 01, 2022, 34 university and 40 non-university hospitals have enrolled 5298 patients with local data quality reviews performed on 4727 (89%). 47% were female, the median age was 52 (IQR 36–62-) and 50 pediatric cases were included. 44% of patients were hospitalized, 15% admitted to an intensive care unit, and 12% of patients deceased while enrolled. 8845 visits with biosampling in 4349 patients were conducted by April 03, 2022. In this overview article, we summarize NAPKON’s design, relevant milestones including first study population characteristics, and outline the potential of NAPKON for German and international research activities.Trial registrationhttps://clinicaltrials.gov/ct2/show/NCT04768998.https://clinicaltrials.gov/ct2/show/NCT04747366.https://clinicaltrials.gov/ct2/show/NCT04679584

IntroductionSocioeconomic and ethnic background have been discussed as possible risk factors for SARS-CoV-2 infections in children. Improved knowledge could lead way to tailored prevention strategies and help to improve infection control.MethodsObservational population-based cohort study in children (6mo. - 18 ys.) scheduled for legally required preventive examination and their parents in a metropolitan region in Germany. Primary endpoint was the SARS-CoV-2 seroconversion rate during study period. Risk factors assessed included age, pre-existing medical conditions, socioeconomic factors, and ethnicity.Results2124 children and their parents were included. Seroconversion rates among children in all age groups increased by 3-4-fold from 06/2020 to 02/2021. Only 41% of seropositive children were symptomatic. In 51% of infected children at least one parent was also SARS-CoV-2 positive.Low level of parental education (OR 3.13 (0.72-13.69)) significantly increased the risk of infection. Of the total cohort, 38.5% had a migration background. Specifically, 9% were of Turkish and 5% of Middle Eastern origin. These children had the highest risk for SARS-CoV-2 infections (OR 6.24 (1.38-28.12) and 6.44 (1.14-36.45) after adjustment for other risk factors.DiscussionSeroprevalence of SARS-CoV-2 infections in children increased by 3-4-fold within the study period. Frequently, more than one family member was infected. Children from families with lower socioeconomic status were at higher risk. The highest risk for SARS-CoV-2 infection was identified in families with Turkish or Middle Eastern background. Culture sensitive approaches are essential to improve infection control and serve as a blueprint for vaccination strategies in this population.Trial RegistrationBMBF funding registration 01KI20173 (Corkid)

RationaleIn adults, personalised asthma treatment targets patients with type 2 (T2)-high and eosinophilic asthma phenotypes. It is unclear whether such classification is achievable in children.ObjectivesTo define T2-high asthma with easily accessible biomarkers and compare resulting phenotypes across all ages.MethodsIn the multicentre clinical All Age Asthma Cohort (ALLIANCE), 1125 participants (n=776 asthmatics, n=349 controls) were recruited and followed for 2 years (1 year in adults). Extensive clinical characterisation (questionnaires, blood differential count, allergy testing, lung function and sputum induction (in adults)) was performed at baseline and follow-ups. Interleukin (IL)-4, IL-5 and IL-13 were measured after stimulation of whole blood with lipopolysaccharide (LPS) or anti-CD3/CD28.Measurements and main resultsBased on blood eosinophil counts and allergen-specific serum IgE antibodies, patients were categorised into four mutually exclusive phenotypes: “atopy-only”, “eosinophils-only”, “T2-high” (eosinophilia + atopy) and “T2-low” (neither eosinophilia nor atopy). The T2-high phenotype was found across all ages, even in very young children in whom it persisted to a large degree even after 2 years of follow-up. T2-high asthma in adults was associated with childhood onset, suggesting early origins of this asthma phenotype. In both children and adults, the T2-high phenotype was characterised by excessive production of specific IgE to allergens (pConclusionsUsing easily accessible biomarkers, patients with T2-high asthma can be identified across all ages delineating a distinct phenotype. These patients may benefit from therapy with biologicals even at a younger age.

Comprehensive studies investigated the role of T-cells in asthma which led to personalised treatment options targeting severe eosinophilic asthma. However, little is known about the contribution of B-cells to this chronic inflammatory disease. In this study we investigated the contribution of various B-cell populations to specific clinical features in asthma.In the All Age Asthma Cohort (ALLIANCE), a subgroup of 154 adult asthma patients and 28 healthy controls were included for B-cell characterisation by flow cytometry. Questionnaires, lung function measurements, blood differential counts and allergy testing of participants were analysed together with comprehensive data on B-cells using association studies and multivariate linear models.Patients with severe asthma showed decreased immature B-cell populations while memory B-cells were significantly increased compared with both mild-moderate asthma patients and healthy controls. Furthermore, increased frequencies of IgAWith this study we demonstrate for the first time a significant association of increased IgA

BackgroundThe frequency of persistent symptoms after coronavirus disease 2019 (COVID-19) in adults varies from 4.5% to 87%. Pulmonary function can also show long-term impairment in adults: 10% of hospitalised adults had reduced spirometry values, and 24% had decreased diffusion capacity. To date, only preliminary evidence is available on persistent respiratory sequelae in children and adolescents, therefore our objective was to examine the long-term effects of COVID-19 on pulmonary function in this age group.MethodsMultiple-breath washout, body plethysmography, and diffusion capacity testing were performed after an average of 2.6 months (range 0.4–6.0) following COVID-19 in 73 children and adolescents (age 5–18 years) with different disease severity. Cases were compared to 45 controls with and without infection within six months prior to assessment after exclusion of severe acute respiratory coronavirus-2 infection (SARS-CoV-2).ResultsOf the 19 patients (27.1%) who complained about persistent or newly emerged symptoms since COVID-19, 8 (11.4%) reported respiratory symptoms. Comparing patients with COVID-19 to controls, no significant differences were detected in frequency of abnormal pulmonary function (COVID-19: 12, 16.4%; controls: 12, 27.7%; OR 0.54, 95% CI 0.22–1.34). Only two patients with persistent respiratory symptoms showed abnormal pulmonary function. Multivariate analysis revealed reduced forced vital capacity (p=0.045) in patients with severe infection regardless of SARS-CoV-2 infection.DiscussionPulmonary function is rarely impaired in children and adolescents after COVID-19, except of those with severe infection. The discrepancy between persistent respiratory symptoms and normal pulmonary function suggests a different underlying pathology such as dysfunctional breathing.

Background: Once daily intravenous (iv) treatment with tobramycin for Pseudomonas aeruginosa infection in patients with cystic fibrosis (CF) is usually monitored by measuring tobramycin trough levels. Although the necessity of these trough levels is recently questioned in CF patients without renal impairment, no study has evaluated this so far. The aim of this observational study was to evaluate the frequency of increased tobramycin trough levels in 278 courses of iv tobramycin in CF children and adolescents. Methods: Patient records of all consecutive once daily iv tobramycin courses in 35 CF patients between 07/2009 and 07/2019 were analyzed for tobramycin level, renal function, co-medication and comorbidity. Results: Eight elevated tobramycin levels (2.9% of 278 courses) were recorded in four patients, two with normal renal function. One of these did resolve without adjustment of tobramycin dosages suggesting laboratory error. In the other patient elevated tobramycin levels occurred after recently being started on lumacaftor/ivacaftor and decreased after dosage adjustment. Six of the elevated levels occurred in two patients with chronic renal failure. In 15 other patients with reduced glomerular filtration rate (GFR) (36 courses) no case of an elevated tobramycin trough level was detected. Cumulative tobramycin dosages were neither a risk factor for elevated trough levels nor were diabetes or nutritional status. Conclusion: In CF patients with normal GFR (GFR >100 ml/min) and in absence of additional risk factors (e.g. recently started CFTR modulator therapy) a monitoring of the once daily iv treatment by tobramycin trough levels seems not to be necessary.

Introduction: Following exposure to infectious tuberculosis e.g. in a kindergarden setting preventative therapy (IPT) with isoniazide (INH) is recommended for all children under the age of five at least 8 weeks followed by immunological tests (TST, IGRA) for TB infection. Compliance with IPT is often poor an infection rates very from 5-10%. Methods: Children who were exposed to infectious tuberculosis in their kindergarden but showed no signs of infection (TST and IGRA negative) were started on IPT with INH for minimum 8 weeks. One group received medication and regular follow up care, the other one more detailed and repetitive parent information, teaching and a close follow up by paediatricians and health care authorities. At the end of treatment all children were retested for TB infection (TST, IGRA). Adherence with treatment as well as reasons for interruption of therapy were recorded. Results: Two groups of 44 and 46 children between one and five years of age were examined. In the group without specific intervention 25/46 (54%) were adherent, in the intervention group 37/44(89%) reported good adherence. In the first group 4/46 (8,6%) of the children developed TB infection (positive TST/IGRA) after 8 weeks, whereas none of the children in the intervention group developed infection. Method: Timely and detailed information of families as well as a close follow up increases adherence with IPT in TB exposed small children and therefore significantly reduces the risk of TB infection.

Introduction: Aminoglykosides have been part of standard treatment for pseudomonas infection in patients with cystic fibrosis for decades. Nephro- and ototoxicity are the most common side effects. Therefore monitoring of tobramycin levels is recommended. The aim of the study was to evaluate the impact of measuring tobramycin levels in once daily iv tobramycin therapy. Methods: All once daily tobramycin treatments between 07/2010 and 07/2019 in CF patients were included and patient data analysed for tobramycin levels, renal function and comorbities. Results: Out of 278 iv tobramycin courses seven elevated tobramycin levels were recorded (2.5%). Six of the measurements (86%) were performed in patients with chronic renal failure. Method: In absence of additional comorbitites and risk factors the monitoring of tobramycin levels in once daily iv treamtent in CF patients is not necessary.

Background To date, few data on paediatric COVID-19 have been published, and most reports originate from China. This study aimed to capture key data on children and adolescents with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection across Europe to inform physicians and health-care service planning during the ongoing pandemic. Methods This multicentre cohort study involved 82 participating health-care institutions across 25 European countries, using a well established research network—the Paediatric Tuberculosis Network European Trials Group (ptbnet)—that mainly comprises paediatric infectious diseases specialists and paediatric pulmonologists. We included all individuals aged 18 years or younger with confirmed SARS-CoV-2 infection, detected at any anatomical site by RT-PCR, between April 1 and April 24, 2020, during the initial peak of the European COVID-19 pandemic. We explored factors associated with need for intensive care unit (ICU) admission and initiation of drug treatment for COVID-19 using univariable analysis, and applied multivariable logistic regression with backwards stepwise analysis to further explore those factors significantly associated with ICU admission. Findings 582 individuals with PCR-confirmed SARS-CoV-2 infection were included, with a median age of 5·0 years (IQR 0·5–12·0) and a sex ratio of 1·15 males per female. 145 (25%) had pre-existing medical conditions. 363 (62%) individuals were admitted to hospital. 48 (8%) individuals required ICU admission, 25 (4%) mechanical ventilation (median duration 7 days, IQR 2–11, range 1–34), 19 (3%) inotropic support, and one ( Interpretation COVID-19 is generally a mild disease in children, including infants. However, a small proportion develop severe disease requiring ICU admission and prolonged ventilation, although fatal outcome is overall rare. The data also reflect the current uncertainties regarding specific treatment options, highlighting that additional data on antiviral and immunomodulatory drugs are urgently needed. Funding ptbnet is supported by Deutsche Gesellschaft für Internationale Zusammenarbeit.

Cystic fibrosis (CF, OMIM 219700) is a rare genetic disorder caused by a chloride channel defect, resulting in lung disease, pancreas insufficiency and liver impairment. Altered L-arginine (Arg)/nitric oxide (NO) metabolism has been observed in CF patients&rsquo, lungs and in connection with malnutrition. The aim of the present study was to investigate markers of the Arg/NO pathway in the plasma and urine of CF patients and to identify possible risk factors, especially associated with malnutrition. We measured the major NO metabolites nitrite and nitrate, Arg, a semi-essential amino acid and NO precursor, the NO synthesis inhibitor asymmetric dimethylarginine (ADMA) and its major urinary metabolite dimethylamine (DMA) in plasma and urine samples of 70 pediatric CF patients and 78 age-matched healthy controls. Biomarkers were determined by gas chromatography&ndash, mass spectrometry and high-performance liquid chromatography. We observed higher plasma Arg (90.3 vs. 75.6 &micro, M, p &lt, 0.0001), ADMA (0.62 vs. 0.57 &micro, M, p = 0.03), Arg/ADMA ratio (148 vs. 135, p = 0.01), nitrite (2.07 vs. 1.95 &micro, M, p = 0.03) and nitrate (43.3 vs. 33.1 &micro, 0.001) concentrations, as well as higher urinary DMA (57.9 vs. 40.7 &micro, M/mM creatinine, p &lt, 0.001) and nitrate (159 vs. 115 &micro, M/mM creatinine, p = 0.001) excretion rates in the CF patients compared to healthy controls. CF patients with pancreatic sufficiency showed plasma concentrations of the biomarkers comparable to those of healthy controls. Malnourished CF patients had lower Arg/ADMA ratios (p = 0.02), indicating a higher NO synthesis capacity in sufficiently nourished CF patients. We conclude that NO production, protein-arginine dimethylation, and ADMA metabolism is increased in pediatric CF patients. Pancreas and liver function influence Arg/NO metabolism. Good nutritional status is associated with higher NO synthesis capacity and lower protein-arginine dimethylation.

ZusammenfassungAktuelle epidemiologische Daten zeigen in den letzten Jahren einen Anstieg der Tuberkulose im Kindes- und Jugendalter. Es findet sich zudem auch in Deutschland ein Anstieg von Infektionen mit gegenüber Tuberkulosemedikamenten resistenten Erregern. In diesem Zusammenhang stellt die Diagnose, Prävention und Therapie der Tuberkulose vor allem im Kindesalter eine Herausforderung dar.Leitlinien für die Diagnostik und Therapie der Tuberkulose im Erwachsenenalter können nicht generell auf das Kindesalter übertragen werden, da hier relevante altersabhängige Unterschiede bzgl. der Krankheitsprogression, Krankheitsmanifestation, Unterschiede in der Anwendung von diagnostischen Maßnahmen und der Therapie bestehen.Unter Federführung der Deutschen Gesellschaft für Pädiatrische Infektiologie (DGPI) e. V. wurde die S2k-Leitlinie für die Diagnostik, Prävention und Therapie der Tuberkulose im Kindes- und Jugendalter verfasst, um die adäquate Versorgung von Kindern und Jugendlichen mit Tuberkulose-Exposition, Infektion oder Erkrankung nach neuesten wissenschaftlichen Erkenntnissen zu sichern.Aktualisierte Dosierungsempfehlungen berücksichtigen die altersabhängige Pharmakokinetik in der Therapie der medikamentensensiblen, aber auch resistenten Tuberkulose. Darüber hinaus werden die Themen perinatale Exposition und Erkrankung sowie die im Kindesalter häufigeren extrapulmonalen Manifestationsformen gesondert behandelt.

ZusammenfassungIn Deutschland ist seit 2015 ein deutlicher Anstieg der gemeldeten Tuberkulosefälle zu verzeichnen. Zurückzuführen ist dieser vor allem auf die aktuell vermehrte Migration. Durch die niedrige Inzidenz in den vorhergehenden Jahren konzentriert sich die Erfahrung im Umgang mit Tuberkulose immer mehr auf spezialisierte Zentren. Pneumologen wie auch andere Fachbereiche sind nun aber wieder häufiger an der Behandlung einer Tuberkulose beteiligt, sodass Fachwissen zur Standardtherapie wie auch zu selteneren Therapiesituationen benötigt wird. Die aktuelle Leitlinie zur Diagnostik und Therapie, einschließlich Chemoprävention und -prophylaxe im Erwachsenenalter fasst den derzeitigen Wissensstand zusammen und passt die Empfehlungen an die Situation in Deutschland an. Realisiert wurde die AWMF S2k-Leitlinie durch das Deutsche Zentralkomitee zur Bekämpfung der Tuberkulose e. V. (DZK) im Auftrag der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin e. V. (DGP). Zur Behandlung der Tuberkulose im pädiatrischen Bereich wird in Kürze eine eigenständige Leitlinie der entsprechenden Fachgesellschaften veröffentlicht. Im Vergleich zu den Empfehlungen von 2012 sind eigenständige Kapitel zur Labordiagnostik und zum therapeutischen Medikamentenmanagement entstanden. Die Kapitel Mehrfachresistenzen gegen Medikamente der Standardtherapie, HIV-Koinfektion und die Übersicht der Arzneimittel wurden erweitert. Die umfangreiche Überarbeitung der Empfehlungen soll Ärzten und auch anderen Beschäftigten im Gesundheitswesen helfen, den aktuellen Herausforderungen im Umgang mit dem selten gewordenen Tuberkuloseerreger zu begegnen.

Primary Ciliary Dyskinesia (PCD, MIM 242650) is a rare, hereditary multiorgan disease characterized by malfunction of motile cilia. Hallmark symptom is a chronic airway infection due to mucostasis leading to irreversible lung damage that may progress to respiratory failure. There is no cure for this genetic disease and evidence-based treatment is limited. Until recently, there were no randomized controlled trials performed in PCD, but this year, data of the first placebo-controlled trial on pharmacotherapy in PCD were published. This cornerstone in the management of PCD was decisive for reviewing currently used treatment strategies. This article is a consensus of patient representatives and clinicians, which are highly experienced in care of PCD-patients and provides an overview of the management of PCD. Treatments are mainly based on expert opinions, personal experiences, or are deduced from other lung diseases, notably cystic fibrosis (CF), COPD or bronchiectasis. Most strategies focus on routine airway clearance and treatment of recurrent respiratory tract infections. Non-respiratory symptoms are treated organ specific. To generate further evidence-based knowledge, other projects are under way, e. g. the International PCD-Registry. Participating in patient registries facilitates access to clinical and research studies and strengthens networks between centers. In addition, knowledge of genotype-specific course of the disease will offer the opportunity to further improve and individualize patient care.Die Primäre Ciliäre Dyskinesie (PCD, MIM 242650) ist eine seltene hereditäre Multisystemerkrankung mit klinisch heterogenem Phänotyp. Leitsymptom ist eine chronische Sekretretention der oberen und unteren Atemwege, welche durch die Dysfunktion motiler respiratorischer Zilien entsteht. In der Folge kommt es zur Ausbildung von Bronchiektasen, häufig zu einer Infektion durch Pseudomonas aeruginosa sowie einer abnehmenden Lungenfunktion bis hin zum Lungenversagen.Bislang gibt es kaum evidenzbasierte Therapieempfehlungen, da randomisierte Langzeitstudien zur Behandlung der PCD fehlten. In diesem Jahr wurden die Daten einer ersten placebokontrollierten Medikamentenstudie bei PCD veröffentlicht. Anlässlich dieses Meilensteins im Management der PCD wurde der vorliegende Übersichtsartikel als Konsens von Patientenvertretern sowie Klinikern, die langjährige Erfahrung in der Behandlung der PCD haben, verfasst. Diese Arbeit bietet eine Zusammenfassung aktuell eingesetzter Behandlungsverfahren, die überwiegend auf persönlichen Erfahrungen und Expertenmeinungen beruhen oder von anderen Atemwegserkrankungen wie der Cystischen Fibrose (CF), COPD oder Bronchiektasen-Erkrankung abgeleitet werden. Da es derzeit keine kurative Therapie für PCD gibt, stehen symptomatische Maßnahmen wie die regelmäßige Reinigung der Atemwege und die Behandlung von rezidivierenden Atemwegsinfektionen im Fokus. Nicht respiratorische Manifestationen werden organspezifisch behandelt. Um neben der ersten Medikamentenstudie mehr evidenzbasiertes Wissen zu generieren, werden weitere Projekte etabliert, u. a. ein internationales PCD-Register. Hierüber wird Patienten der Zugang zu klinischen und wissenschaftlichen Studien erleichtert und die Vernetzung behandelnder Zentren gefördert. Des Weiteren können Erkenntnisse über eine Genotyp-spezifische Erkrankungsschwere erlangt werden, um folglich die therapeutische Versorgung der Patienten zu verbessern und somit zu individualisieren.

Die Bestatigung einer Tuberkuloseerkrankung ist im Kindesalter erschwert. Nicht nur Diagnostik, sondern auch Pravention und Behandlung stellen weiterhin eine Herausforderung dar. Einige wichtige Besonderheiten mussen dabei beachtet werden.