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1. Weekly SARS-CoV-2 Sentinel Surveillance in Primary Schools, Kindergartens, and Nurseries, Germany, June-November 2020

Author

Hoch, Martin, Vogel, Sebastian, Kolberg, Laura, Dick, Elisabeth, Fingerle, Volker, Eberle, Ute, Ackermann, Nikolaus, Sing, Andreas, Huebner, Johannes, Rack-Hoch, Anita, Schober, Tilmann, and von Both, Ulrich

Subjects
Epidemics -- Statistics -- Risk factors -- Control, Sentinel health events -- Methods, Elementary schools -- Statistics -- Health aspects -- Safety and security measures -- Germany, Nursery schools -- Statistics -- Health aspects -- Safety and security measures, Health
Abstract

We investigated severe acute respiratory syndrome coronavirus 2 infections in primary schools, kindergartens, and nurseries in Germany. Of 3,169 oropharyngeal swab specimens, only 2 were positive by real-time reverse transcription [...]

Published
2021
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2. Weekly SARS-CoV-2 Sentinel Surveillance in Primary Schools, Kindergartens, and Nurseries, Germany, June‒November 2020

Author

Martin Hoch, Sebastian Vogel, Laura Kolberg, Elisabeth Dick, Volker Fingerle, Ute Eberle, Nikolaus Ackermann, Andreas Sing, Johannes Huebner, Anita Rack-Hoch, Tilmann Schober, and Ulrich von Both

Subjects
severe acute respiratory syndrome coronavirus 2, SARS-CoV-2, coronaviruses, viruses, coronavirus disease, COVID-19, Medicine, Infectious and parasitic diseases, RC109-216
Abstract

We investigated severe acute respiratory syndrome coronavirus 2 infections in primary schools, kindergartens, and nurseries in Germany. Of 3,169 oropharyngeal swab specimens, only 2 were positive by real-time reverse transcription PCR. Asymptomatic children attending these institutions do not appear to be driving the pandemic when appropriate infection control measures are used.

Published
2021
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3. SARS-CoV-2 Saliva Mass Screening in Primary Schools: A 10-Week Sentinel Surveillance Study in Munich, Germany

Author

Sebastian Vogel, Ulrich von Both, Elisabeth Nowak, Janina Ludwig, Alexandra Köhler, Noah Lee, Elisabeth Dick, Anita Rack-Hoch, Bernd Wicklein, Jessica Neusser, Tobias Wagner, Alexandra Schubö, Maxim Ustinov, Werner Schimana, Stephan Busche, Laura Kolberg, and Martin Hoch

Subjects
SARS-CoV-2, saliva, mass screening, primary school, Salivette®, RT-qPCR, Medicine (General), R5-920
Abstract

Representative, actively collected surveillance data on asymptomatic SARS-CoV-2 infections in primary schoolchildren remain scarce. We evaluated the feasibility of a saliva mass screening concept and assessed infectious activity in primary schools. During a 10-week period from 3 March to 21 May 2021, schoolchildren and staff from 17 primary schools in Munich participated in the sentinel surveillance, cohort study. Participants were tested using the Salivette® system, testing was supervised by trained school staff, and samples were processed via reverse transcription quantitative polymerase chain reaction (RT-qPCR). We included 4433 participants: 3752 children (median age, 8 [range, 6–13] years; 1926 girls [51%]) and 681 staff members (median age, 41 [range, 14–71] years; 592 women [87%]). In total, 23,905 samples were processed (4640 from staff), with participants representing 8.3% of all primary schoolchildren in Munich. Only eight cases were detected: Five out of 3752 participating children (0.13%) and three out of 681 staff members (0.44%). There were no secondary cases. In conclusion, supervised Salivette® self-sampling was feasible, reliable, and safe and thus constituted an ideal method for SARS-CoV-2 mass screenings in primary schoolchildren. Our findings suggest that infectious activity among asymptomatic primary schoolchildren and staff was low. Primary schools appear to continue to play a minor role in the spread of SARS-CoV-2 despite high community incidence rates.

Published
2022
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4. Feasibility and Diagnostic Accuracy of Saliva-Based SARS-CoV-2 Screening in Educational Settings and Children Aged

Author

Martin Hoch, Sebastian Vogel, Ute Eberle, Laura Kolberg, Valerie Gruenthaler, Volker Fingerle, Nikolaus Ackermann, Andreas Sing, Bernhard Liebl, Johannes Huebner, Simone Kuttiadan, Anita Rack-Hoch, Melanie Meyer-Buehn, Tilmann Schober, and Ulrich von Both

Subjects
SARS-CoV-2, Salivette®, saliva sampling, primary school, childcare facilities, Medicine (General), R5-920
Abstract

Children have been disproportionately affected during the COVID-19 pandemic. We aimed to assess a saliva-based algorithm for SARS-CoV-2 testing to be used in schools and childcare institutions under pandemic conditions. A weekly SARS-CoV-2 sentinel study in primary schools, kindergartens, and childcare facilities was conducted over a 12-week-period. In a sub-study covering 7 weeks, 1895 paired oropharyngeal and saliva samples were processed for SARS-CoV-2 rRT-PCR testing in both asymptomatic children (n = 1243) and staff (n = 652). Forty-nine additional concurrent swab and saliva samples were collected from SARS-CoV-2 infected patients (patient cohort). The Salivette® system was used for saliva collection and assessed for feasibility and diagnostic performance. For children, a mean of 1.18 mL saliva could be obtained. Based on results from both cohorts, the Salivette® testing algorithm demonstrated the specificity of 100% (95% CI 99.7–100) and sensitivity of 94.9% (95% CI 81.4–99.1) with oropharyngeal swabs as reference. Agreement between sampling systems was 100% for moderate to high viral load situations (defined as Ct-values ® system proved to be an easy-to-use, safe and feasible saliva collection method and a more pleasant alternative to oropharyngeal swabs for SARS-CoV-2 testing in children aged 3 years and above.

Published
2021
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5. The German National Registry of Primary Immunodeficiencies (2012–2017)

Author

Sabine M. El-Helou, Anika-Kerstin Biegner, Sebastian Bode, Stephan R. Ehl, Maximilian Heeg, Maria E. Maccari, Henrike Ritterbusch, Carsten Speckmann, Stephan Rusch, Raphael Scheible, Klaus Warnatz, Faranaz Atschekzei, Renata Beider, Diana Ernst, Stev Gerschmann, Alexandra Jablonka, Gudrun Mielke, Reinhold E. Schmidt, Gesine Schürmann, Georgios Sogkas, Ulrich H. Baumann, Christian Klemann, Dorothee Viemann, Horst von Bernuth, Renate Krüger, Leif G. Hanitsch, Carmen M. Scheibenbogen, Kirsten Wittke, Michael H. Albert, Anna Eichinger, Fabian Hauck, Christoph Klein, Anita Rack-Hoch, Franz M. Sollinger, Anne Avila, Michael Borte, Stephan Borte, Maria Fasshauer, Anja Hauenherm, Nils Kellner, Anna H. Müller, Anett Ülzen, Peter Bader, Shahrzad Bakhtiar, Jae-Yun Lee, Ursula Heß, Ralf Schubert, Sandra Wölke, Stefan Zielen, Sujal Ghosh, Hans-Juergen Laws, Jennifer Neubert, Prasad T. Oommen, Manfred Hönig, Ansgar Schulz, Sandra Steinmann, Klaus Schwarz, Gregor Dückers, Beate Lamers, Vanessa Langemeyer, Tim Niehues, Sonu Shai, Dagmar Graf, Carmen Müglich, Marc T. Schmalzing, Eva C. Schwaneck, Hans-Peter Tony, Johannes Dirks, Gabriele Haase, Johannes G. Liese, Henner Morbach, Dirk Foell, Antje Hellige, Helmut Wittkowski, Katja Masjosthusmann, Michael Mohr, Linda Geberzahn, Christian M. Hedrich, Christiane Müller, Angela Rösen-Wolff, Joachim Roesler, Antje Zimmermann, Uta Behrends, Nikolaus Rieber, Uwe Schauer, Rupert Handgretinger, Ursula Holzer, Jörg Henes, Lothar Kanz, Christoph Boesecke, Jürgen K. Rockstroh, Carolynne Schwarze-Zander, Jan-Christian Wasmuth, Dagmar Dilloo, Brigitte Hülsmann, Stefan Schönberger, Stefan Schreiber, Rainald Zeuner, Tobias Ankermann, Philipp von Bismarck, Hans-Iko Huppertz, Petra Kaiser-Labusch, Johann Greil, Donate Jakoby, Andreas E. Kulozik, Markus Metzler, Nora Naumann-Bartsch, Bettina Sobik, Norbert Graf, Sabine Heine, Robin Kobbe, Kai Lehmberg, Ingo Müller, Friedrich Herrmann, Gerd Horneff, Ariane Klein, Joachim Peitz, Nadine Schmidt, Stefan Bielack, Ute Groß-Wieltsch, Carl F. Classen, Jessica Klasen, Peter Deutz, Dirk Kamitz, Lisa Lassay, Klaus Tenbrock, Norbert Wagner, Benedikt Bernbeck, Bastian Brummel, Eusebia Lara-Villacanas, Esther Münstermann, Dominik T. Schneider, Nadine Tietsch, Marco Westkemper, Michael Weiß, Christof Kramm, Ingrid Kühnle, Silke Kullmann, Hermann Girschick, Christof Specker, Elisabeth Vinnemeier-Laubenthal, Henriette Haenicke, Claudia Schulz, Lothar Schweigerer, Thomas G. Müller, Martina Stiefel, Bernd H. Belohradsky, Veronika Soetedjo, Gerhard Kindle, and Bodo Grimbacher

Subjects
registry for primary immunodeficiency, primary immunodeficiency (PID), German PID-NET registry, PID prevalence, European Society for Immunodeficiencies (ESID), IgG substitution therapy, Immunologic diseases. Allergy, RC581-607
Abstract

Introduction: The German PID-NET registry was founded in 2009, serving as the first national registry of patients with primary immunodeficiencies (PID) in Germany. It is part of the European Society for Immunodeficiencies (ESID) registry. The primary purpose of the registry is to gather data on the epidemiology, diagnostic delay, diagnosis, and treatment of PIDs.Methods: Clinical and laboratory data was collected from 2,453 patients from 36 German PID centres in an online registry. Data was analysed with the software Stata® and Excel.Results: The minimum prevalence of PID in Germany is 2.72 per 100,000 inhabitants. Among patients aged 1–25, there was a clear predominance of males. The median age of living patients ranged between 7 and 40 years, depending on the respective PID. Predominantly antibody disorders were the most prevalent group with 57% of all 2,453 PID patients (including 728 CVID patients). A gene defect was identified in 36% of patients. Familial cases were observed in 21% of patients. The age of onset for presenting symptoms ranged from birth to late adulthood (range 0–88 years). Presenting symptoms comprised infections (74%) and immune dysregulation (22%). Ninety-three patients were diagnosed without prior clinical symptoms. Regarding the general and clinical diagnostic delay, no PID had undergone a slight decrease within the last decade. However, both, SCID and hyper IgE- syndrome showed a substantial improvement in shortening the time between onset of symptoms and genetic diagnosis. Regarding treatment, 49% of all patients received immunoglobulin G (IgG) substitution (70%—subcutaneous; 29%—intravenous; 1%—unknown). Three-hundred patients underwent at least one hematopoietic stem cell transplantation (HSCT). Five patients had gene therapy.Conclusion: The German PID-NET registry is a precious tool for physicians, researchers, the pharmaceutical industry, politicians, and ultimately the patients, for whom the outcomes will eventually lead to a more timely diagnosis and better treatment.

Published
2019
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6. Human CARMIL2 deficiency underlies a broader immunological and clinical phenotype than CD28 deficiency

Author

Lévy, Romain, Gothe, Florian, Momenilandi, Mana, Magg, Thomas, Materna, Marie, Peters, Philipp, Raedler, Johannes, Philippot, Quentin, Rack-Hoch, Anita, Langlais, David, Bourgey, Mathieu, Lanz, Anna-Lisa, Ogishi, Masato, Rosain, Jérémie, Martin, Emmanuel, Latour, Sylvain, Vladikine, Natasha, Distefano, Marco, Khan, Taushif, Rapaport, Franck, Schulz, Marian, Holzer, Ursula, Fasth, Anders, Sogkas, Georgios, Speckmann, Carsten, Troilo, Arianna, Bigley, Venetia, Roppelt, Anna, Dinur-Schejter, Yael, Toker, Ori, Bronken Martinsen, Karen, Sherkat, Roya, Somekh, Ido, Somech, Raz, Shouval, Dror, Kühl, Jörn-Sven, Ip, Winnie, Mcdermott, Elizabeth, Cliffe, Lucy, Ozen, Ahmet, Baris, Safa, Rangarajan, Hemalatha, Jouanguy, Emmanuelle, Puel, Anne, Bustamante, Jacinta, Alyanakian, Marie-Alexandra, Fusaro, Mathieu, Wang, Yi, Kong, Xiao-Fei, Cobat, Aurélie, Boutboul, David, Castelle, Martin, Aguilar, Claire, Hermine, Olivier, Cheminant, Morgane, Suarez, Felipe, Yildiran, Alisan, Bousfiha, Aziz, Al-Mousa, Hamoud, Alsohime, Fahad, Cagdas, Deniz, Abraham, Roshini, Knutsen, Alan, Fevang, Borre, Bhattad, Sagar, Kiykim, Ayca, Erman, Baran, Arikoglu, Tugba, Unal, Ekrem, Kumar, Ashish, Geier, Christoph, Baumann, Ulrich, Neven, Bénédicte, Calas, Julie, Feuille, Elizabeth, Chan, Angela, Yesil, Gozde, Nammour, Justine, Bandet, Élise, Picard, Capucine, Benhsaien, Ibtihal, Lang, Peter, Atschekzei, Faranaz, Warnatz, Klaus, Hambleton, Sophie, Desai, Mukesh, Karakoc-Aydiner, Elif, Kolukisa, Burcu, Al-Muhsen, Saleh, Alosaimi, Mohammed, Cipe, Funda, Alazami, Anas, Hancioglu, Gonca, Can Meydan, Bilge, Sorte, Hanne, Stray-Pedersen, Asbjørg, Mammayil, Geetha, Tökmeci, Nazan, Shcherbina, Anna, Stepensky, Polina, Nasereddin, Adeeb, Rouzaud, Claire, Hoshino, Akihiro, Shamriz, Oded, Ledder, Oren, Maccari, Maria, Castro, Carla, Grimbacher, Bodo, Schmidt, Reinhold, Collin, Matthew, Zakharova, Victorya, Rohlfs, Meino, Walz, Christoph, Abel, Laurent, Malissen, Bernard, Marr, Nico, Klein, Christoph, Casanova, Jean-Laurent, Hauck, Fabian, Béziat, Vivien, Lévy R., Gothe F., Momenilandi M., Magg T., Materna M., Peters P., Raedler J., Philippot Q., Rack-Hoch A. L., Langlais D., et al., Centre d'Immunologie de Marseille - Luminy (CIML), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre d'Immunophénomique (CIPHE), ANR-10-LABX-0062,IBEID,Integrative Biology of Emerging Infectious Diseases(2010), ANR-10-IAHU-0003,Méditerranée Infection,I.H.U. Méditerranée Infection(2010), and ANR-21-CE15-0034,CARMIL2,Bases moléculaires, cellulaires et immunologiques de la déficience combinée résultant de mutations dans CARMIL2(2021)

Subjects
CD4-Positive T-Lymphocytes, Phenotype, CD28 Antigens, [SDV]Life Sciences [q-bio], Microfilament Proteins, Mutation, Immunology, Humans, Immunology and Allergy
Abstract

International audience; Patients with inherited CARMIL2 or CD28 deficiency have defective T cell CD28 signaling, but their immunological and clinical phenotypes remain largely unknown. We show that only one of three CARMIL2 isoforms is produced and functional across leukocyte subsets. Tested mutant CARMIL2 alleles from 89 patients and 52 families impair canonical NF-κB but not AP-1 and NFAT activation in T cells stimulated via CD28. Like CD28-deficient patients, CARMIL2-deficient patients display recalcitrant warts and low blood counts of CD4 + and CD8 + memory T cells and CD4 + T REG s. Unlike CD28-deficient patients, they have low counts of NK cells and memory B cells, and their antibody responses are weak. CARMIL2 deficiency is fully penetrant by the age of 10 yr and is characterized by numerous infections, EBV + smooth muscle tumors, and mucocutaneous inflammation, including inflammatory bowel disease. Patients with somatic reversions of a mutant allele in CD4 + T cells have milder phenotypes. Our study suggests that CARMIL2 governs immunological pathways beyond CD28.

Published
2022
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8. Weekly SARS-CoV-2 Sentinel Surveillance in Primary Schools, Kindergartens, and Nurseries, Germany, June‒November 2020

Author

Sebastian Vogel, Andreas Sing, Ulrich von Both, Anita Rack-Hoch, Johannes Huebner, Elisabeth Dick, Laura Kolberg, Volker Fingerle, Tilmann Schober, Martin Hoch, Ute Eberle, and Nikolaus Ackermann

Subjects
Microbiology (medical), 2019-20 coronavirus outbreak, Oropharyngeal swab, Coronavirus disease 2019 (COVID-19), Epidemiology, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Expedited, coronaviruses, education, childcare, Infectious and parasitic diseases, RC109-216, Asymptomatic, primary schools, respiratory infections, nurseries, Germany, Pandemic, Medicine, Infection control, Humans, viruses, sentinel, Child, Schools, business.industry, SARS-CoV-2, Dispatch, Infant, COVID-19, Virology, zoonoses, Infectious Diseases, coronavirus disease, Weekly SARS-CoV-2 Sentinel Surveillance in Primary Schools, Kindergartens, and Nurseries, Germany, June‒November 2020, surveillance, kindergartens, medicine.symptom, business, Nurseries, Infant, Sentinel Surveillance, severe acute respiratory syndrome coronavirus 2
Abstract

We investigated severe acute respiratory syndrome coronavirus 2 infections in primary schools, kindergartens, and nurseries in Germany. Of 3,169 oropharyngeal swab specimens, only 2 were positive by real-time reverse transcription PCR. Asymptomatic children attending these institutions do not appear to be driving the pandemic when appropriate infection control measures are used.

Published
2021

9. Brief Report: HIV-Positive and Breastfeeding in High-Income Settings: 5-Year Experience From a Perinatal Center in Germany

Author

Fabian Weiss, Ulrich von Both, Anita Rack-Hoch, Franz Sollinger, Josef Eberle, Sven Mahner, Ralph Kaestner, and Irene Alba Alejandre

Subjects
Infectious Diseases, Breast Feeding, Pregnancy, Infant, Humans, Pharmacology (medical), Female, HIV Infections, Zidovudine, Infectious Disease Transmission, Vertical, Retrospective Studies
Abstract

Exclusive breastfeeding is recommended for women living with HIV (WLWH) in low-income-but not in high-income-countries, where milk substitutes are preferred. Some guidelines for high-income countries opted for a shared decision-making process regarding breastfeeding in optimal scenarios with adherence to antiretroviral therapy (cART), suppressed maternal viral load (mVL), and clinical monitoring. Although vertical transmission (VT) risk under cART is estimated below 1% in low-income settings, data from high-income countries are rare.We retrospectively analyzed all 181 live births from WLWH at the LMU Munich university hospital perinatal center in Germany between January 2016 and December 2020. We focused on VT, suppressed mVL and optimal scenario rates, breastfeeding frequency, cART regimens, and infant prophylaxis. All women were counseled according to current guidelines, foremost recommending avoidance of breastfeeding.In the 5-year cohort, no VT was observed. One hundred fifty-one WLWH (83.4%) decided not to breastfeed, even in optimal scenarios. Thrity infants (16.6%) were nursed, of which 25 were within an optimal scenario, whereas in 5 cases, breastfeeding was performed with a detectable VL in pregnancy or the postpartum period. All WLWH were treated with cART at delivery, and 91.7% sustained suppressed mVL. Zidovudine infant prophylaxis was given between 2 and 8 weeks but not necessarily over the whole breastfeeding duration and was declined from 5 breastfeeding WLWH.Although the cohort is too small to assess VT risk through breastfeeding with cART-suppressed mVL, breastfeeding might be an alternative even in high-income countries, but further studies are needed.

Published
2022

10. SARS-CoV-2 Saliva Mass Screening in Primary Schools: A 10-Week Sentinel Surveillance Study in Munich, Germany

Author

Vogel, Sebastian, primary, von Both, Ulrich, additional, Nowak, Elisabeth, additional, Ludwig, Janina, additional, Köhler, Alexandra, additional, Lee, Noah, additional, Dick, Elisabeth, additional, Rack-Hoch, Anita, additional, Wicklein, Bernd, additional, Neusser, Jessica, additional, Wagner, Tobias, additional, Schubö, Alexandra, additional, Ustinov, Maxim, additional, Schimana, Werner, additional, Busche, Stephan, additional, Kolberg, Laura, additional, and Hoch, Martin, additional

Published
2022
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11. Feasibility and Diagnostic Accuracy of Saliva-Based SARS-CoV-2 Screening in Educational Settings and Children Aged &lt

Author

Hoch, Martin, Vogel, Sebastian, Eberle, Ute, Kolberg, Laura, Gruenthaler, Valerie, Fingerle, Volker, Ackermann, Nikolaus, Sing, Andreas, Liebl, Bernhard, Huebner, Johannes, Kuttiadan, Simone, Rack-Hoch, Anita, Meyer-Buehn, Melanie, Schober, Tilmann, and Both, Ulrich von

Subjects
saliva sampling, SARS-CoV-2, childcare facilities, Salivette®, primary school
Abstract

Children have been disproportionately affected during the COVID-19 pandemic. We aimed to assess a saliva-based algorithm for SARS-CoV-2 testing to be used in schools and childcare institutions under pandemic conditions. A weekly SARS-CoV-2 sentinel study in primary schools, kindergartens, and childcare facilities was conducted over a 12-week-period. In a sub-study covering 7 weeks, 1895 paired oropharyngeal and saliva samples were processed for SARS-CoV-2 rRT-PCR testing in both asymptomatic children (n = 1243) and staff (n = 652). Forty-nine additional concurrent swab and saliva samples were collected from SARS-CoV-2 infected patients (patient cohort). The Salivette® system was used for saliva collection and assessed for feasibility and diagnostic performance. For children, a mean of 1.18 mL saliva could be obtained. Based on results from both cohorts, the Salivette® testing algorithm demonstrated the specificity of 100% (95% CI 99.7–100) and sensitivity of 94.9% (95% CI 81.4–99.1) with oropharyngeal swabs as reference. Agreement between sampling systems was 100% for moderate to high viral load situations (defined as Ct-values &lt, 33 from oropharyngeal swabs). Comparative analysis of Ct-values derived from saliva vs. oropharyngeal swabs demonstrated a significant difference (mean 4.23, 95% CI 2.48–6.00). In conclusion, the Salivette® system proved to be an easy-to-use, safe and feasible saliva collection method and a more pleasant alternative to oropharyngeal swabs for SARS-CoV-2 testing in children aged 3 years and above.

Published
2021
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12. Feasibility and Diagnostic Accuracy of Saliva-Based SARS-CoV-2 Screening in Educational Settings and Children Aged <12 Years

Author

Valerie Gruenthaler, Andreas Sing, Ulrich von Both, Johannes Huebner, Melanie Meyer-Buehn, Bernhard Liebl, Martin Hoch, Anita Rack-Hoch, Nikolaus Ackermann, Sebastian Vogel, Simone Kuttiadan, Laura Kolberg, Volker Fingerle, Tilmann Schober, and Ute Eberle

Subjects
medicine.medical_specialty, Saliva, Medicine (General), Coronavirus disease 2019 (COVID-19), business.industry, Salivette®, SARS-CoV-2, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Clinical Biochemistry, Diagnostic accuracy, Asymptomatic, Article, primary school, saliva sampling, R5-920, stomatognathic system, childcare facilities, Internal medicine, Cohort, Medicine, Sampling (medicine), medicine.symptom, business, Viral load
Abstract

Children have been disproportionately affected during the COVID-19 pandemic. We aimed to assess a saliva-based algorithm for SARS-CoV-2 testing to be used in schools and childcare institutions under pandemic conditions. A weekly SARS-CoV-2 sentinel study in primary schools, kindergartens, and childcare facilities was conducted over a 12-week-period. In a sub-study covering 7 weeks, 1895 paired oropharyngeal and saliva samples were processed for SARS-CoV-2 rRT-PCR testing in both asymptomatic children (n = 1243) and staff (n = 652). Forty-nine additional concurrent swab and saliva samples were collected from SARS-CoV-2 infected patients (patient cohort). The Salivette® system was used for saliva collection and assessed for feasibility and diagnostic performance. For children, a mean of 1.18 mL saliva could be obtained. Based on results from both cohorts, the Salivette® testing algorithm demonstrated the specificity of 100% (95% CI 99.7–100) and sensitivity of 94.9% (95% CI 81.4–99.1) with oropharyngeal swabs as reference. Agreement between sampling systems was 100% for moderate to high viral load situations (defined as Ct-values ® system proved to be an easy-to-use, safe and feasible saliva collection method and a more pleasant alternative to oropharyngeal swabs for SARS-CoV-2 testing in children aged 3 years and above.

Published
2021

13. Feasibility and Diagnostic Accuracy of Saliva-Based SARS-CoV-2 Screening in Educational Settings and Children Aged <12 Years

Author

Hoch, Martin, primary, Vogel, Sebastian, additional, Eberle, Ute, additional, Kolberg, Laura, additional, Gruenthaler, Valerie, additional, Fingerle, Volker, additional, Ackermann, Nikolaus, additional, Sing, Andreas, additional, Liebl, Bernhard, additional, Huebner, Johannes, additional, Kuttiadan, Simone, additional, Rack-Hoch, Anita, additional, Meyer-Buehn, Melanie, additional, Schober, Tilmann, additional, and von Both, Ulrich, additional

Published
2021
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14. Feasibility and diagnostic accuracy of saliva-based SARS-CoV-2 screening in educational settings and children aged < 12 years

Author

Laura Kolberg, Gruenthaler, Kuttiadan S, Bernhard Liebl, Tilmann Schober, Johannes Huebner, von Both U, Martin Hoch, Anita Rack-Hoch, Fingerle, Sebastian Vogel, Andreas Sing, Melanie Meyer-Buehn, Nikolaus Ackermann, and Ute Eberle

Subjects
Test strategy, Sampling system, Saliva, Pediatrics, medicine.medical_specialty, Coronavirus disease 2019 (COVID-19), business.industry, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Cohort, Medicine, Sampling (statistics), Positive control, business
Abstract

ObjectivesChildren have been disproportionately affected during the COVID-19 pandemic. Novel test strategies are urgently needed to ensure safe operation of schools and childcare institutions and to avoid prolonged closures. MethodsA weekly SARS-CoV-2 sentinel study in primary schools, kindergartens and childcare facilities over a 12-week-period was conducted. In total, 3123 concurrent oropharyngeal and saliva samples were processed for SARS-CoV-2 rRT-PCR testing in both children (n=2104) and staff (n=1019). Saliva sampling was optimised, and a novel sampling system was introduced and assessed for feasibility, the Salivette(R) system. ResultsFor children across all age groups a mean of 1,18 ml saliva could be obtained with this easy-to-handle system. Using 1293 concurrent oropharyngeal swabs from children, staff and participants of a positive control cohort as reference, the Salivette testing method could be assigned an overall specificity of 99.8% and sensititity of 95.1%. Of note, clinical sensitivity, defined as detection of positive cases with an oropharyngeal-swab derived Ct-value < 33, was 100%. Comparative analysis of Ct-values derived from saliva vs. oropharyngeal swabs demonstrated a significant difference (mean difference 4.23 (95% CI 2.48-6.00). ConclusionsThe Salivette system is an easy-to-use, safe and feasible collection method for saliva sampling and subsequent SARS-CoV-2 testing in children aged 3 years and above. In view of the excellent sensitivity and specificity documented in our study, this novel testing approach is a very reliable and much more pleasant alternative to oropharyngeal swab based testing, particularly in children and for self-testing in the home setting.

Published
2021
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15. Weekly SARS-CoV-2 sentinel in primary schools, kindergartens and nurseries, June to November 2020, Germany

Author

Martin Hoch, Andreas Sing, Nikolaus Ackermann, Elisabeth Dick, Anita Rack-Hoch, Laura Kolberg, Ulrich von Both, Tilmann Schober, Johannes Huebner, Sebastian Vogel, Ute Eberle, and Volker Fingerle

Subjects
2019-20 coronavirus outbreak, Coronavirus disease 2019 (COVID-19), business.industry, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Environmental health, Pandemic, Medicine, Infection control, business
Abstract

A 12-week sentinel programme monitored SARS-CoV-2 in primary schools, kindergartens and nurseries. Out of 3169 oropharyngeal swabs, only two tested positive on rRT-PCR while general incidence rates were surging. Thus, children attending respective institutions are not significantly contributing to the pandemic spread when appropriate infection control measures are in place.

Published
2021
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17. Reduced Immunoglobulin (Ig) G Response to Staphylococcus aureus in STAT3 Hyper-IgE Syndrome

Author

Barbara M. Bröker, Felicitas Abel, Anita Rack-Hoch, Barbara C. Kahl, Ellen D. Renner, Sebastian Stentzel, and Beate Hagl

Subjects
Adult, Male, 0301 basic medicine, Microbiology (medical), Staphylococcus aureus, Adolescent, Cystic Fibrosis, medicine.disease_cause, Immunoglobulin E, Cystic fibrosis, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Immune system, Humans, Medicine, 030212 general & internal medicine, Child, biology, business.industry, Middle Aged, Staphylococcal Infections, Acquired immune system, medicine.disease, Antibodies, Bacterial, S. Aureus, Stat3, Hyper-ige Syndrome, Hies, Th17, Adaptive Immunity, Specific Igg, 030104 developmental biology, Infectious Diseases, Child, Preschool, Immunoglobulin G, Immunology, Humoral immunity, biology.protein, Female, Antibody, business, Job Syndrome, Staphylococcus
Abstract

STAT3 hyper-IgE syndrome (STAT3-HIES) patients presented with significantly lower Staphylococcus aureus-specific serum IgG compared to cystic fibrosis patients despite recurrent S. aureus infections. Immunoglobulin replacement therapy increased S. aureus-specific IgG in STAT3-HIES patients and attenuated the clinical course of disease suggesting a role of humoral immunity in S. aureus clearance.

Published
2017
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18. The German National Registry of Primary Immunodeficiencies (2012–2017)

Author

El-Helou, Sabine M., primary, Biegner, Anika-Kerstin, additional, Bode, Sebastian, additional, Ehl, Stephan R., additional, Heeg, Maximilian, additional, Maccari, Maria E., additional, Ritterbusch, Henrike, additional, Speckmann, Carsten, additional, Rusch, Stephan, additional, Scheible, Raphael, additional, Warnatz, Klaus, additional, Atschekzei, Faranaz, additional, Beider, Renata, additional, Ernst, Diana, additional, Gerschmann, Stev, additional, Jablonka, Alexandra, additional, Mielke, Gudrun, additional, Schmidt, Reinhold E., additional, Schürmann, Gesine, additional, Sogkas, Georgios, additional, Baumann, Ulrich H., additional, Klemann, Christian, additional, Viemann, Dorothee, additional, von Bernuth, Horst, additional, Krüger, Renate, additional, Hanitsch, Leif G., additional, Scheibenbogen, Carmen M., additional, Wittke, Kirsten, additional, Albert, Michael H., additional, Eichinger, Anna, additional, Hauck, Fabian, additional, Klein, Christoph, additional, Rack-Hoch, Anita, additional, Sollinger, Franz M., additional, Avila, Anne, additional, Borte, Michael, additional, Borte, Stephan, additional, Fasshauer, Maria, additional, Hauenherm, Anja, additional, Kellner, Nils, additional, Müller, Anna H., additional, Ülzen, Anett, additional, Bader, Peter, additional, Bakhtiar, Shahrzad, additional, Lee, Jae-Yun, additional, Heß, Ursula, additional, Schubert, Ralf, additional, Wölke, Sandra, additional, Zielen, Stefan, additional, Ghosh, Sujal, additional, Laws, Hans-Juergen, additional, Neubert, Jennifer, additional, Oommen, Prasad T., additional, Hönig, Manfred, additional, Schulz, Ansgar, additional, Steinmann, Sandra, additional, Schwarz, Klaus, additional, Dückers, Gregor, additional, Lamers, Beate, additional, Langemeyer, Vanessa, additional, Niehues, Tim, additional, Shai, Sonu, additional, Graf, Dagmar, additional, Müglich, Carmen, additional, Schmalzing, Marc T., additional, Schwaneck, Eva C., additional, Tony, Hans-Peter, additional, Dirks, Johannes, additional, Haase, Gabriele, additional, Liese, Johannes G., additional, Morbach, Henner, additional, Foell, Dirk, additional, Hellige, Antje, additional, Wittkowski, Helmut, additional, Masjosthusmann, Katja, additional, Mohr, Michael, additional, Geberzahn, Linda, additional, Hedrich, Christian M., additional, Müller, Christiane, additional, Rösen-Wolff, Angela, additional, Roesler, Joachim, additional, Zimmermann, Antje, additional, Behrends, Uta, additional, Rieber, Nikolaus, additional, Schauer, Uwe, additional, Handgretinger, Rupert, additional, Holzer, Ursula, additional, Henes, Jörg, additional, Kanz, Lothar, additional, Boesecke, Christoph, additional, Rockstroh, Jürgen K., additional, Schwarze-Zander, Carolynne, additional, Wasmuth, Jan-Christian, additional, Dilloo, Dagmar, additional, Hülsmann, Brigitte, additional, Schönberger, Stefan, additional, Schreiber, Stefan, additional, Zeuner, Rainald, additional, Ankermann, Tobias, additional, Bismarck, Philipp von, additional, Huppertz, Hans-Iko, additional, Kaiser-Labusch, Petra, additional, Greil, Johann, additional, Jakoby, Donate, additional, Kulozik, Andreas E., additional, Metzler, Markus, additional, Naumann-Bartsch, Nora, additional, Sobik, Bettina, additional, Graf, Norbert, additional, Heine, Sabine, additional, Kobbe, Robin, additional, Lehmberg, Kai, additional, Müller, Ingo, additional, Herrmann, Friedrich, additional, Horneff, Gerd, additional, Klein, Ariane, additional, Peitz, Joachim, additional, Schmidt, Nadine, additional, Bielack, Stefan, additional, Groß-Wieltsch, Ute, additional, Classen, Carl F., additional, Klasen, Jessica, additional, Deutz, Peter, additional, Kamitz, Dirk, additional, Lassay, Lisa, additional, Tenbrock, Klaus, additional, Wagner, Norbert, additional, Bernbeck, Benedikt, additional, Brummel, Bastian, additional, Lara-Villacanas, Eusebia, additional, Münstermann, Esther, additional, Schneider, Dominik T., additional, Tietsch, Nadine, additional, Westkemper, Marco, additional, Weiß, Michael, additional, Kramm, Christof, additional, Kühnle, Ingrid, additional, Kullmann, Silke, additional, Girschick, Hermann, additional, Specker, Christof, additional, Vinnemeier-Laubenthal, Elisabeth, additional, Haenicke, Henriette, additional, Schulz, Claudia, additional, Schweigerer, Lothar, additional, Müller, Thomas G., additional, Stiefel, Martina, additional, Belohradsky, Bernd H., additional, Soetedjo, Veronika, additional, Kindle, Gerhard, additional, and Grimbacher, Bodo, additional

Published
2019
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19. The German National Registry of Primary Immunodeficiencies (2012-2017)

Author

El-Helou, Sabine M., Biegner, Anika-Kerstin, Bode, Sebastian, Ehl, Stephan R., Heeg, Maximilian, Maccari, Maria E., Ritterbusch, Henrike, Speckmann, Carsten, Rusch, Stephan, Scheible, Raphael, Warnatz, Klaus, Atschekzei, Faranaz, Beider, Renata, Ernst, Diana, Gerschmann, Stev, Jablonka, Alexandra, Mielke, Gudrun, Schmidt, Reinhold E., Schuermann, Gesine, Sogkas, Georgios, Baumann, Ulrich H., Klemann, Christian, Viemann, Dorothee, von Bernuth, Horst, Krueger, Renate, Hanitsch, Leif G., Scheibenbogen, Carmen M., Wittke, Kirsten, Albert, Michael H., Eichinger, Anna, Hauck, Fabian, Klein, Christoph, Rack-Hoch, Anita, Sollinger, Franz M., Avila, Anne, Borte, Michael, Borte, Stephan, Fasshauer, Maria, Hauenherm, Anja, Kellner, Nils, Mueller, Anna H., Uelzen, Anett, Bader, Peter, Bakhtiar, Shahrzad, Lee, Jae-Yun, Hess, Ursula, Schubert, Ralf, Woelke, Sandra, Zielen, Stefan, Ghosh, Sujal, Laws, Hans-Juergen, Neubert, Jennifer, Oommen, Prasad T., Hoenig, Manfred, Schulz, Ansgar, Steinmann, Sandra, Schwarz, Klaus, Dueckers, Gregor, Lamers, Beate, Langemeyer, Vanessa, Niehues, Tim, Shai, Sonu, Graf, Dagmar, Mueglich, Carmen, Schmalzing, Marc T., Schwaneck, Eva C., Tony, Hans-Peter, Dirks, Johannes, Haase, Gabriele, Liese, Johannes G., Morbach, Henner, Foell, Dirk, Hellige, Antje, Wittkowski, Helmut, Masjosthusmann, Katja, Mohr, Michael, Geberzahn, Linda, Hedrich, Christian M., Mueller, Christiane, Roesen-Wolff, Angela, Roesler, Joachim, Zimmermann, Antje, Behrends, Uta, Rieber, Nikolaus, Schauer, Uwe, Handgretinger, Rupert, Holzer, Ursula, Henes, Joerg, Kanz, Lothar, Boesecke, Christoph, Rockstroh, Juergen K., Schwarze-Zander, Carolynne, Wasmuth, Jan-Christian, Dilloo, Dagmar, Huelsmann, Brigitte, Schoenberger, Stefan, Schreiber, Stefan, Zeuner, Rainald, Ankermann, Tobias, von Bismarck, Philipp, Huppertz, Hans-Iko, Kaiser-Labusch, Petra, Greil, Johann, Jakoby, Donate, Kulozik, Andreas E., Metzler, Markus, Naumann-Bartsch, Nora, Sobik, Bettina, Graf, Norbert, Heine, Sabine, Kobbe, Robin, Lehmberg, Kai, Mueller, Ingo, Herrmann, Friedrich, Horneff, Gerd, Klein, Ariane, Peitz, Joachim, Schmidt, Nadine, Bielack, Stefan, Gross-Wieltsch, Ute, Classen, Carl F., Klasen, Jessica, Deutz, Peter, Kamitz, Dirk, Lassay, Lisa, Tenbrock, Klaus, Wagner, Norbert, Bernbeck, Benedikt, Brummel, Bastian, Lara-Villacanas, Eusebia, Muenstermann, Esther, Schneider, Dominik T., Tietsch, Nadine, Westkemper, Marco, Weiss, Michael, Kramm, Christof, Kuehnle, Ingrid, Kullmann, Silke, Girschick, Hermann, Specker, Christof, Vinnemeier-Laubenthal, Elisabeth, Haenicke, Henriette, Schulz, Claudia, Schweigerer, Lothar, Mueller, Thomas G., Stiefel, Martina, Belohradsky, Bernd H., Soetedjo, Veronika, Kindle, Gerhard, Grimbacher, Bodo, El-Helou, Sabine M., Biegner, Anika-Kerstin, Bode, Sebastian, Ehl, Stephan R., Heeg, Maximilian, Maccari, Maria E., Ritterbusch, Henrike, Speckmann, Carsten, Rusch, Stephan, Scheible, Raphael, Warnatz, Klaus, Atschekzei, Faranaz, Beider, Renata, Ernst, Diana, Gerschmann, Stev, Jablonka, Alexandra, Mielke, Gudrun, Schmidt, Reinhold E., Schuermann, Gesine, Sogkas, Georgios, Baumann, Ulrich H., Klemann, Christian, Viemann, Dorothee, von Bernuth, Horst, Krueger, Renate, Hanitsch, Leif G., Scheibenbogen, Carmen M., Wittke, Kirsten, Albert, Michael H., Eichinger, Anna, Hauck, Fabian, Klein, Christoph, Rack-Hoch, Anita, Sollinger, Franz M., Avila, Anne, Borte, Michael, Borte, Stephan, Fasshauer, Maria, Hauenherm, Anja, Kellner, Nils, Mueller, Anna H., Uelzen, Anett, Bader, Peter, Bakhtiar, Shahrzad, Lee, Jae-Yun, Hess, Ursula, Schubert, Ralf, Woelke, Sandra, Zielen, Stefan, Ghosh, Sujal, Laws, Hans-Juergen, Neubert, Jennifer, Oommen, Prasad T., Hoenig, Manfred, Schulz, Ansgar, Steinmann, Sandra, Schwarz, Klaus, Dueckers, Gregor, Lamers, Beate, Langemeyer, Vanessa, Niehues, Tim, Shai, Sonu, Graf, Dagmar, Mueglich, Carmen, Schmalzing, Marc T., Schwaneck, Eva C., Tony, Hans-Peter, Dirks, Johannes, Haase, Gabriele, Liese, Johannes G., Morbach, Henner, Foell, Dirk, Hellige, Antje, Wittkowski, Helmut, Masjosthusmann, Katja, Mohr, Michael, Geberzahn, Linda, Hedrich, Christian M., Mueller, Christiane, Roesen-Wolff, Angela, Roesler, Joachim, Zimmermann, Antje, Behrends, Uta, Rieber, Nikolaus, Schauer, Uwe, Handgretinger, Rupert, Holzer, Ursula, Henes, Joerg, Kanz, Lothar, Boesecke, Christoph, Rockstroh, Juergen K., Schwarze-Zander, Carolynne, Wasmuth, Jan-Christian, Dilloo, Dagmar, Huelsmann, Brigitte, Schoenberger, Stefan, Schreiber, Stefan, Zeuner, Rainald, Ankermann, Tobias, von Bismarck, Philipp, Huppertz, Hans-Iko, Kaiser-Labusch, Petra, Greil, Johann, Jakoby, Donate, Kulozik, Andreas E., Metzler, Markus, Naumann-Bartsch, Nora, Sobik, Bettina, Graf, Norbert, Heine, Sabine, Kobbe, Robin, Lehmberg, Kai, Mueller, Ingo, Herrmann, Friedrich, Horneff, Gerd, Klein, Ariane, Peitz, Joachim, Schmidt, Nadine, Bielack, Stefan, Gross-Wieltsch, Ute, Classen, Carl F., Klasen, Jessica, Deutz, Peter, Kamitz, Dirk, Lassay, Lisa, Tenbrock, Klaus, Wagner, Norbert, Bernbeck, Benedikt, Brummel, Bastian, Lara-Villacanas, Eusebia, Muenstermann, Esther, Schneider, Dominik T., Tietsch, Nadine, Westkemper, Marco, Weiss, Michael, Kramm, Christof, Kuehnle, Ingrid, Kullmann, Silke, Girschick, Hermann, Specker, Christof, Vinnemeier-Laubenthal, Elisabeth, Haenicke, Henriette, Schulz, Claudia, Schweigerer, Lothar, Mueller, Thomas G., Stiefel, Martina, Belohradsky, Bernd H., Soetedjo, Veronika, Kindle, Gerhard, and Grimbacher, Bodo

Abstract

Introduction: The German PID-NET registry was founded in 2009, serving as the first national registry of patients with primary immunodeficiencies (PID) in Germany. It is part of the European Society for Immunodeficiencies (ESID) registry. The primary purpose of the registry is to gather data on the epidemiology, diagnostic delay, diagnosis, and treatment of PIDs. Methods: Clinical and laboratory data was collected from 2,453 patients from 36 German PID centres in an online registry. Data was analysed with the software Stata((R)) and Excel. Results: The minimum prevalence of PID in Germany is 2.72 per 100,000 inhabitants. Among patients aged 1-25, there was a clear predominance of males. The median age of living patients ranged between 7 and 40 years, depending on the respective PID. Predominantly antibody disorders were the most prevalent group with 57% of all 2,453 PID patients (including 728 CVID patients). A gene defect was identified in 36% of patients. Familial cases were observed in 21% of patients. The age of onset for presenting symptoms ranged from birth to late adulthood (range 0-88 years). Presenting symptoms comprised infections (74%) and immune dysregulation (22%). Ninety-three patients were diagnosed without prior clinical symptoms. Regarding the general and clinical diagnostic delay, no PID had undergone a slight decrease within the last decade. However, both, SCID and hyper IgE-syndrome showed a substantial improvement in shortening the time between onset of symptoms and genetic diagnosis. Regarding treatment, 49% of all patients received immunoglobulin G (IgG) substitution (70%-subcutaneous; 29%-intravenous; 1%-unknown). Three-hundred patients underwent at least one hematopoietic stem cell transplantation (HSCT). Five patients had gene therapy. Conclusion: The German PID-NET registry is a precious tool for physicians, researchers, the pharmaceutical industry, politicians, and ultimately the patients, for whom the outcomes will eventually lead to a m

Published
2019

20. The German national registry of primary immunodeficiencies (2012–2017)

Author

Al-Herz, Waleed, El-Helou, Sabine M., Biegner, Anika-Kerstin, Bode, Sebastian, Ehl, Stephan, Heeg, Maximilian, Maccari, Maria E., Ritterbusch, Henrike, Speckmann, Carsten, Rusch, Stephan, Scheible, Raphael, Warnatz, Klaus, Atschekzei, Faranaz, Beider, Renata, Ernst, Diana, Gerschmann, Stev, Jablonka, Alexandra, Mielke, Gudrun, Schmidt, Reinhold E., Schürmann, Gesine, Sogkas, Georgios, Baumann, Ulrich, Klemann, Christian, Viemann, Dorothee, Bernuth, Horst von, Krüger, Renate, Hanitsch, Leif Gunnar, Scheibenbogen, Carmen, Wittke, Kirsten, Albert, Michael H., Eichinger, Anna, Hauck, Fabian, Klein, Christoph, Rack-Hoch, Anita, Sollinger, Franz, Avila, Anne, Borte, Michael, Borte, Stephan, Fasshauer, Maria, Hauenherm, Anja, Kellner, Nils, Müller, Anna H., Ülzen, Anett, Bader, Peter, Bakhtiar, Shahrzad, Lee, Jae-Yun, Heß, Ursula, Schubert, Ralf, Wölke, Sandra, Zielen, Stefan, Ghosh, Sujal, Laws, Hans-Jürgen, Neubert, Jennifer, Oommen, Prasad Thomas, Hönig, Manfred, Schulz, Ansgar, Steinmann, Sandra, Schwarz, Klaus, Dückers, Gregor, Lamers, Beate, Langemeyer, Vanessa, Niehues, Tim, Shai, Sonu, Graf, Dagmar, Müglich, Carmen, Schmalzing, Marc, Schwaneck, Eva C., Tony, Hans-Peter, Dirks, Johannes, Haase, Gabriele, Liese, Johannes G., Morbach, Henner, Föll, Dirk, Hellige, Antje, Wittkowski, Helmut, Masjosthusmann, Katja, Mohr, Michael, Geberzahn, Linda, Hedrich, Christian Michael, Müller, Christiane, Rösen-Wolff, Angela, Roesler, Joachim, Zimmermann, Antje, Behrends, Uta, Rieber, Nikolaus, Schauer, Uwe, Handgretinger, Rupert, Holzer, Ursula, Henes, Jörg Christoph, Kanz, Lothar, Boesecke, Christoph, Rockstroh, Jürgen, Schwarze-Zander, Carolynne, Wasmuth, Jan-Christian, Dilloo, Dagmar, Hülsmann, Brigitte, Schönberger, Stefan, Schreiber, Stefan, Zeuner, Rainald, Ankermann, Tobias, Bismarck, Philipp von, Huppertz, Hans-Iko, Kaiser-Labusch, Petra, Greil, Johann, Jakoby, Donate, Kulozik, Andreas, Metzler, Markus, Naumann-Bartsch, Nora, Sobik, Bettina, Graf, Norbert, Heine, Sabine, Kobbe, Robin, Lehmberg, Kai, Müller, Ingo, Herrmann, Friedrich, Horneff, Gerd, Klein, Ariane, Peitz, Joachim, Schmidt, Nadine, Bielack, Stefan S., Groß-Wieltsch, Ute, Classen, Carl Friedrich, Klasen, Jessica, Deutz, Peter, Kamitz, Dirk, Lassay, Lisa, Tenbrock, Klaus, Wagner, Norbert, Bernbeck, Benedikt, Brummel, Bastian, Lara-Villacanas, Eusebia, Münstermann, Esther, Schneider, Dominik T., Tietsch, Nadine, Westkemper, Marco, Weiß, Michael, Kramm, Christof M., Kühnle, Ingrid, Kullman, Silke, Girschick, Hermann, Specker, Christof, Vinnemeier-Laubenthal, Elisabeth, Haenicke, Henriette, Schulz, Claudia, Schweigerer, Lothar, Müller, Thomas G., Stiefel, Martina, Belohradsky, Bernd H., Soetedjo, Veronika, Kindle, Gerhard, Grimbacher, Bodo, Al-Herz, Waleed, El-Helou, Sabine M., Biegner, Anika-Kerstin, Bode, Sebastian, Ehl, Stephan, Heeg, Maximilian, Maccari, Maria E., Ritterbusch, Henrike, Speckmann, Carsten, Rusch, Stephan, Scheible, Raphael, Warnatz, Klaus, Atschekzei, Faranaz, Beider, Renata, Ernst, Diana, Gerschmann, Stev, Jablonka, Alexandra, Mielke, Gudrun, Schmidt, Reinhold E., Schürmann, Gesine, Sogkas, Georgios, Baumann, Ulrich, Klemann, Christian, Viemann, Dorothee, Bernuth, Horst von, Krüger, Renate, Hanitsch, Leif Gunnar, Scheibenbogen, Carmen, Wittke, Kirsten, Albert, Michael H., Eichinger, Anna, Hauck, Fabian, Klein, Christoph, Rack-Hoch, Anita, Sollinger, Franz, Avila, Anne, Borte, Michael, Borte, Stephan, Fasshauer, Maria, Hauenherm, Anja, Kellner, Nils, Müller, Anna H., Ülzen, Anett, Bader, Peter, Bakhtiar, Shahrzad, Lee, Jae-Yun, Heß, Ursula, Schubert, Ralf, Wölke, Sandra, Zielen, Stefan, Ghosh, Sujal, Laws, Hans-Jürgen, Neubert, Jennifer, Oommen, Prasad Thomas, Hönig, Manfred, Schulz, Ansgar, Steinmann, Sandra, Schwarz, Klaus, Dückers, Gregor, Lamers, Beate, Langemeyer, Vanessa, Niehues, Tim, Shai, Sonu, Graf, Dagmar, Müglich, Carmen, Schmalzing, Marc, Schwaneck, Eva C., Tony, Hans-Peter, Dirks, Johannes, Haase, Gabriele, Liese, Johannes G., Morbach, Henner, Föll, Dirk, Hellige, Antje, Wittkowski, Helmut, Masjosthusmann, Katja, Mohr, Michael, Geberzahn, Linda, Hedrich, Christian Michael, Müller, Christiane, Rösen-Wolff, Angela, Roesler, Joachim, Zimmermann, Antje, Behrends, Uta, Rieber, Nikolaus, Schauer, Uwe, Handgretinger, Rupert, Holzer, Ursula, Henes, Jörg Christoph, Kanz, Lothar, Boesecke, Christoph, Rockstroh, Jürgen, Schwarze-Zander, Carolynne, Wasmuth, Jan-Christian, Dilloo, Dagmar, Hülsmann, Brigitte, Schönberger, Stefan, Schreiber, Stefan, Zeuner, Rainald, Ankermann, Tobias, Bismarck, Philipp von, Huppertz, Hans-Iko, Kaiser-Labusch, Petra, Greil, Johann, Jakoby, Donate, Kulozik, Andreas, Metzler, Markus, Naumann-Bartsch, Nora, Sobik, Bettina, Graf, Norbert, Heine, Sabine, Kobbe, Robin, Lehmberg, Kai, Müller, Ingo, Herrmann, Friedrich, Horneff, Gerd, Klein, Ariane, Peitz, Joachim, Schmidt, Nadine, Bielack, Stefan S., Groß-Wieltsch, Ute, Classen, Carl Friedrich, Klasen, Jessica, Deutz, Peter, Kamitz, Dirk, Lassay, Lisa, Tenbrock, Klaus, Wagner, Norbert, Bernbeck, Benedikt, Brummel, Bastian, Lara-Villacanas, Eusebia, Münstermann, Esther, Schneider, Dominik T., Tietsch, Nadine, Westkemper, Marco, Weiß, Michael, Kramm, Christof M., Kühnle, Ingrid, Kullman, Silke, Girschick, Hermann, Specker, Christof, Vinnemeier-Laubenthal, Elisabeth, Haenicke, Henriette, Schulz, Claudia, Schweigerer, Lothar, Müller, Thomas G., Stiefel, Martina, Belohradsky, Bernd H., Soetedjo, Veronika, Kindle, Gerhard, and Grimbacher, Bodo

Abstract

Introduction: The German PID-NET registry was founded in 2009, serving as the first national registry of patients with primary immunodeficiencies (PID) in Germany. It is part of the European Society for Immunodeficiencies (ESID) registry. The primary purpose of the registry is to gather data on the epidemiology, diagnostic delay, diagnosis, and treatment of PIDs. Methods: Clinical and laboratory data was collected from 2,453 patients from 36 German PID centres in an online registry. Data was analysed with the software Stata® and Excel. Results: The minimum prevalence of PID in Germany is 2.72 per 100,000 inhabitants. Among patients aged 1–25, there was a clear predominance of males. The median age of living patients ranged between 7 and 40 years, depending on the respective PID. Predominantly antibody disorders were the most prevalent group with 57% of all 2,453 PID patients (including 728 CVID patients). A gene defect was identified in 36% of patients. Familial cases were observed in 21% of patients. The age of onset for presenting symptoms ranged from birth to late adulthood (range 0–88 years). Presenting symptoms comprised infections (74%) and immune dysregulation (22%). Ninety-three patients were diagnosed without prior clinical symptoms. Regarding the general and clinical diagnostic delay, no PID had undergone a slight decrease within the last decade. However, both, SCID and hyper IgE- syndrome showed a substantial improvement in shortening the time between onset of symptoms and genetic diagnosis. Regarding treatment, 49% of all patients received immunoglobulin G (IgG) substitution (70%—subcutaneous; 29%—intravenous; 1%—unknown). Three-hundred patients underwent at least one hematopoietic stem cell transplantation (HSCT). Five patients had gene therapy. Conclusion: The German PID-NET registry is a precious tool for physicians, researchers, the pharmaceutical industry, politicians, and ultimately the patients, for whom the outcomes will eventually lead to a more

Published
2019

21. Louse-borne relapsing fever (Borrelia recurrentis) diagnosed in 15 refugees from northeast Africa: epidemiology and preventive control measures, Bavaria, Germany, July to October 2015

Author

Andreas Sing, Katja Hauptvogel, Gabriele Margos, Jürgen Zühl, W Hautmann, Wolfgang Guggemos, Thomas Löscher, Martin Hoch, Lukas Balzer, M Seilmaier, Anita Rack-Hoch, Andreas Wieser, Ulrich von Both, Friedrich Pürner, Volker Fingerle, and Katharina Schönberger

Subjects
Adult, DNA, Bacterial, Male, medicine.medical_specialty, Veterinary medicine, Adolescent, relapsing fever, Epidemiology, Somalia, Refugee, Preventive control, Eritrea, Young Adult, Germany, Virology, Environmental health, medicine, Humans, Infection control, Louse-Borne Relapsing Fever, Young male, Refugees, Travel, biology, business.industry, Borrelia, Relapsing Fever, Public Health, Environmental and Occupational Health, Lice Infestations, medicine.disease, biology.organism_classification, Treatment Outcome, Doxycycline, Communicable Disease Control, Ethiopia, business, Borrelia recurrentis
Abstract

We report 15 imported louse-borne relapsing fever (LBRF) cases in refugees in Bavaria, Germany. One patient died. Epidemiological findings confirmed that all were young males from the Horn of Africa (12 from Somalia), who had similar migration routes converging in Sudan continuing through Libya and Italy. The majority likely acquired their infection during migration. Healthcare workers should be aware of LBRF in refugees passing through north Africa to ensure correct treatment and preventive measures.

Published
2015
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22. Verringerung des Antibiotika- und Antimykotikaverbrauchs und Kosteneinsparung durch Antibiotic Stewardship (ABS) in der Kinderklinik

Author

Rack-Hoch, A. L., Pecar, A., and Hübner, J.

Subjects
ddc: 610, 610 Medical sciences, Medicine
Abstract

Hintergrund: Der unbedachte und häufig unnötige Einsatz von antiinfektiven Substanzen hat schwerwiegende Folgen für den individuell behandelten Patienten und die gesamte Gesundheitsökonomie. Sowohl die hohen Kosten als auch das Auftreten multiresistenter Erreger und unerwünschte[for full text, please go to the a.m. URL], 21. Jahrestagung der Deutschen Gesellschaft für Pädiatrische Infektiologie (DGPI)

Published
2013
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23. Louse-borne relapsing fever (Borrelia recurrentis) diagnosed in 15 refugees from northeast Africa: epidemiology and preventive control measures, Bavaria, Germany, July to October 2015

Author

Hoch, Martin, primary, Wieser, Andreas, additional, Löscher, Thomas, additional, Margos, Gabriele, additional, Pürner, Friedrich, additional, Zühl, Jürgen, additional, Seilmaier, Michael, additional, Balzer, Lukas, additional, Guggemos, Wolfgang, additional, Rack-Hoch, Anita, additional, von Both, Ulrich, additional, Hauptvogel, Katja, additional, Schönberger, Katharina, additional, Hautmann, Wolfgang, additional, Sing, Andreas, additional, and Fingerle, Volker, additional

Published
2015
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25. Human CARMIL2 deficiency underlies a broader immunological and clinical phenotype than CD28 deficiency

Author

Lévy, Romain, Gothe, Florian, Momenilandi, Mana, Magg, Thomas, Materna, Marie, Peters, Philipp, Raedler, Johannes, Philippot, Quentin, Rack-Hoch, Anita Lena, Langlais, David, Bourgey, Mathieu, Lanz, Anna-Lisa, Ogishi, Masato, Rosain, Jérémie, Martin, Emmanuel, Latour, Sylvain, Vladikine, Natasha, Distefano, Marco, Khan, Taushif, Rapaport, Franck, Schulz, Marian S., Holzer, Ursula, Fasth, Anders, Sogkas, Georgios, Speckmann, Carsten, Troilo, Arianna, Bigley, Venetia, Roppelt, Anna, Dinur-Schejter, Yael, Toker, Ori, Bronken Martinsen, Karen Helene, Sherkat, Roya, Somekh, Ido, Somech, Raz, Shouval, Dror S., Kühl, Jörn-Sven, Ip, Winnie, McDermott, Elizabeth M., Cliffe, Lucy, Ozen, Ahmet, Baris, Safa, Rangarajan, Hemalatha G., Jouanguy, Emmanuelle, Puel, Anne, Bustamante, Jacinta, Alyanakian, Marie-Alexandra, Fusaro, Mathieu, Wang, Yi, Kong, Xiao-Fei, Cobat, Aurélie, Boutboul, David, Castelle, Martin, Aguilar, Claire, Hermine, Olivier, Cheminant, Morgane, Suarez, Felipe, Yildiran, Alisan, Bousfiha, Aziz, Al-Mousa, Hamoud, Alsohime, Fahad, Cagdas, Deniz, Abraham, Roshini S., Knutsen, Alan P., Fevang, Borre, Bhattad, Sagar, Kiykim, Ayca, Erman, Baran, Arikoglu, Tugba, Unal, Ekrem, Kumar, Ashish, Geier, Christoph B., Baumann, Ulrich, Neven, Bénédicte, Rohlfs, Meino, Walz, Christoph, Abel, Laurent, Malissen, Bernard, Marr, Nico, Klein, Christoph, Casanova, Jean-Laurent, Hauck, Fabian, and Béziat, Vivien

Abstract

Patients with inherited CARMIL2 or CD28 deficiency have defective T cell CD28 signaling, but their immunological and clinical phenotypes remain largely unknown. We show that only one of three CARMIL2 isoforms is produced and functional across leukocyte subsets. Tested mutant CARMIL2 alleles from 89 patients and 52 families impair canonical NF-κB but not AP-1 and NFAT activation in T cells stimulated via CD28. Like CD28-deficient patients, CARMIL2-deficient patients display recalcitrant warts and low blood counts of CD4+ and CD8+ memory T cells and CD4+ TREGs. Unlike CD28-deficient patients, they have low counts of NK cells and memory B cells, and their antibody responses are weak. CARMIL2 deficiency is fully penetrant by the age of 10 yr and is characterized by numerous infections, EBV+ smooth muscle tumors, and mucocutaneous inflammation, including inflammatory bowel disease. Patients with somatic reversions of a mutant allele in CD4+ T cells have milder phenotypes. Our study suggests that CARMIL2 governs immunological pathways beyond CD28.

Published
2023
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26. Brief Report: HIV-Positive and Breastfeeding in High-Income Settings: 5-Year Experience From a Perinatal Center in Germany.

Author

Weiss F, von Both U, Rack-Hoch A, Sollinger F, Eberle J, Mahner S, Kaestner R, and Alba Alejandre I

Subjects
Infant, Pregnancy, Female, Humans, Zidovudine therapeutic use, Retrospective Studies, Infectious Disease Transmission, Vertical prevention & control, Breast Feeding, HIV Infections drug therapy, HIV Infections prevention & control
Abstract

Background: Exclusive breastfeeding is recommended for women living with HIV (WLWH) in low-income-but not in high-income-countries, where milk substitutes are preferred. Some guidelines for high-income countries opted for a shared decision-making process regarding breastfeeding in optimal scenarios with adherence to antiretroviral therapy (cART), suppressed maternal viral load (mVL), and clinical monitoring. Although vertical transmission (VT) risk under cART is estimated below 1% in low-income settings, data from high-income countries are rare., Methods: We retrospectively analyzed all 181 live births from WLWH at the LMU Munich university hospital perinatal center in Germany between January 2016 and December 2020. We focused on VT, suppressed mVL and optimal scenario rates, breastfeeding frequency, cART regimens, and infant prophylaxis. All women were counseled according to current guidelines, foremost recommending avoidance of breastfeeding., Results: In the 5-year cohort, no VT was observed. One hundred fifty-one WLWH (83.4%) decided not to breastfeed, even in optimal scenarios. Thrity infants (16.6%) were nursed, of which 25 were within an optimal scenario, whereas in 5 cases, breastfeeding was performed with a detectable VL in pregnancy or the postpartum period. All WLWH were treated with cART at delivery, and 91.7% sustained suppressed mVL. Zidovudine infant prophylaxis was given between 2 and 8 weeks but not necessarily over the whole breastfeeding duration and was declined from 5 breastfeeding WLWH., Conclusions: Although the cohort is too small to assess VT risk through breastfeeding with cART-suppressed mVL, breastfeeding might be an alternative even in high-income countries, but further studies are needed., Competing Interests: S.M. is on multiple advisory boards and has received research support, honoraria, and travel expenses from AbbVie, AstraZeneca, Clovis, Eisai, GlaxoSmithKline, Medac, MSD, Novartis, Olympus, PharmaMar, Pfizer, Roche, Sensor Kinesis, Teva, and Tesaro.The remaining authors have no funding or conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Published
2022
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