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2. Impact of lung morphology on clinical outcomes with riociguat in patients with pulmonary hypertension and idiopathic interstitial pneumonia: A post hoc subgroup analysis of the RISE-IIP study

Author

Nathan, Steven D., Cottin, Vincent, Behr, Juergen, Hoeper, Marius M., Martinez, Fernando J., Corte, Tamera J., Keogh, Anne M., Leuchte, Hanno, Mogulkoc, Nesrin, Ulrich, Silvia, Wuyts, Wim A., Yao, Zhen, Ley-Zaporozhan, Julia, Müller-Lisse, Ullrich G., Scholle, Frank-Detlef, Brüggenwerth, Günther, Busse, Dennis, Nikkho, Sylvia, and Wells, Athol U.

Published
2021
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4. Chest CT Imaging in Children

Author

Ley, Sebastian, Ley-Zaporozhan, Julia, Kauczor, Hans-Ulrich, Series Editor, Parizel, Paul M., Series Editor, Peh, Wilfred C. G., Series Editor, Brady, Luther W., Honorary Editor, Lu, Jiade J., Series Editor, Nikolaou, Konstantin, editor, Bamberg, Fabian, editor, Laghi, Andrea, editor, and Rubin, Geoffrey D., editor

Published
2019
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5. Erratum: S3-Leitlinie: Lungenerkrankung bei Mukoviszidose – Pseudomonas aeruginosa

Author

Schwarz, Carsten, additional, Bend, Jutta, additional, Hebestreit, Helge, additional, Hogardt, Michael, additional, Hügel, Christian, additional, Illing, Stephan, additional, Mainz, Jochen G., additional, Rietschel, Ernst, additional, Schmidt, Sebastian, additional, Schulte-Hubbert, Bernhard, additional, Sitter, Helmut, additional, Wielpütz, Marc Oliver, additional, Hammermann, Jutta, additional, Baumann, Ingo, additional, Brunsmann, Frank, additional, Dieninghoff, Doris, additional, Eber, Ernst, additional, Ellemunter, Helmut, additional, Eschenhagen, Patience, additional, Evers, Caroline, additional, Gruber, Saskia, additional, Koitschev, Assen, additional, Ley-Zaporozhan, Julia, additional, Düesberg, Uta, additional, Mentzel, Hans-Joachim, additional, Nüßlein, Thomas, additional, Ringshausen, Felix C., additional, Sedlacek, Ludwig, additional, Smaczny, Christina, additional, Sommerburg, Olaf, additional, Sutharsan, Sivagurunathan, additional, Vonberg, Ralf-Peter, additional, Weber, Ann-Katrin, additional, and Zerlik, Jovita, additional

Published
2024
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6. S3-Leitlinie: Lungenerkrankung bei Mukoviszidose – Pseudomonas aeruginosa

Author

Schwarz, Carsten, additional, Bend, Jutta, additional, Hebestreit, Helge, additional, Hogardt, Michael, additional, Hügel, Christian, additional, Illing, Stephan, additional, Mainz, Jochen G., additional, Rietschel, Ernst, additional, Schmidt, Sebastian, additional, Schulte-Hubbert, Bernhard, additional, Sitter, Helmut, additional, Wielpütz, Marc Oliver, additional, Hammermann, Jutta, additional, Baumann, Ingo, additional, Brunsmann, Frank, additional, Dieninghoff, Doris, additional, Eber, Ernst, additional, Ellemunter, Helmut, additional, Eschenhagen, Patience, additional, Evers, Caroline, additional, Gruber, Saskia, additional, Koitschev, Assen, additional, Ley-Zaporozhan, Julia, additional, Düesberg, Uta, additional, Mentzel, Hans-Joachim, additional, Nüßlein, Thomas, additional, Ringshausen, Felix C., additional, Sedlacek, Ludwig, additional, Smaczny, Christina, additional, Sommerburg, Olaf, additional, Sutharsan, Sivagurunathan, additional, Vonberg, Ralf-Peter, additional, Weber, Ann-Katrin, additional, and Zerlik, Jovita, additional

Published
2024
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8. Vascular Anomalies and Diseases

Author

Ley, Sebastian, Ley-Zaporozhan, Julia, Kauczor, Hans-Ulrich, Series Editor, Parizel, Paul M., Series Editor, Peh, Wilfred C. G., Series Editor, and Wielpütz, Mark Oliver, editor

Published
2018
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11. MRI of Lung Morphology and Perfusion

Author

Ley, Sebastian, Ley-Zaporozhan, Julia, Reiser, Maximilian F, Series editor, Kauczor, Hans-Ulrich, Series editor, Hricak, Hedvig, Series editor, Knauth, Michael, Series editor, Garcia-Peña, Pilar, editor, and Guillerman, R. Paul, editor

Published
2014
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13. ABCA3-related interstitial lung disease beyond infancy

Author

Li, Yang, primary, Seidl, Elias, additional, Knoflach, Katrin, additional, Gothe, Florian, additional, Forstner, Maria Elisabeth, additional, Michel, Katarzyna, additional, Pawlita, Ingo, additional, Gesenhues, Florian, additional, Sattler, Franziska, additional, Yang, Xiaohua, additional, Kroener, Carolin, additional, Reu-Hofer, Simone, additional, Ley-Zaporozhan, Julia, additional, Kammer, Birgit, additional, Krüger-Stollfuß, Ingrid, additional, Dinkel, Julien, additional, Carlens, Julia, additional, Wetzke, Martin, additional, Moreno-Galdó, Antonio, additional, Torrent-Vernetta, Alba, additional, Lange, Joanna, additional, Krenke, Katarzyna, additional, Rumman, Nisreen, additional, Mayell, Sarah, additional, Sismanlar, Tugba, additional, Aslan, Ayse, additional, Regamey, Nicolas, additional, Proesmans, Marijke, additional, Stehling, Florian, additional, Naehrlich, Lutz, additional, Ayse, Kilinc, additional, Becker, Sebastian, additional, Koerner-Rettberg, Cordula, additional, Plattner, Erika, additional, Manali, Effrosyni D, additional, Papiris, Spyridon A, additional, Campo, Ilaria, additional, Kappler, Matthias, additional, Schwerk, Nicolaus, additional, and Griese, Matthias, additional

Published
2023
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16. CT of the Airways

Author

Kauczor, Hans-Ulrich, Owsijewitsch, Michael, Ley-Zaporozhan, Julia, Reiser, Maximilian F., editor, Becker, C.R., editor, Nikolaou, Konstantin, editor, and Glazer, Gary, editor

Published
2009
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22. Kommentar zu: Bekanntmachung der aktualisierten diagnostischen Referenzwerte für diagnostische und interventionelle Röntgenuntersuchungen des Bundesamtes für Strahlenschutz

Author

Hamer, Okka Wilkea, additional, Biederer, Jürgen, additional, Das, Marco, additional, Kauczor, Hans-Ulrich, additional, Wielpütz, Mark Oliver, additional, Ley-Zaporozhan, Julia, additional, Vogel-Claussen, Jens, additional, Wormanns, Dag, additional, and Ley, Sebastian, additional

Published
2022
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24. Dunkelfeld-Radiographie: Erste Ergebnisse in gesunden Probanden und Patienten mit Lungenemphysem

Author

Pfeiffer, Daniela (Prof. Dr.), Pfeiffer, Daniela (Prof. Dr.);Herzen, Julia (Prof. Dr.);Ley-Zaporozhan, Julia (Prof. Dr.), Gassert, Florian Tilman, Pfeiffer, Daniela (Prof. Dr.), Pfeiffer, Daniela (Prof. Dr.);Herzen, Julia (Prof. Dr.);Ley-Zaporozhan, Julia (Prof. Dr.), and Gassert, Florian Tilman

Abstract

In der vorliegenden Arbeit konnte gezeigt werden, dass die neuartige Dunkelfeld-Radiographie, welche nicht die Abschwächung sondern die Kleinwinkelstreuung beim Auftreffen der Röntgenstrahlen auf Gewebe misst, sich für die Bildgebung der menschlichen Lunge eignet und die Signalstärke dabei unabhängig von demographischen Parametern Alter, Geschlecht, Gewicht und Größe ist. Insbesondere für die Diagnostik eines Lungenemphysems konnte gezeigt werden, dass die Technik großes Potenzial aufweist., In the present work, it could be shown that the novel X-ray dark-field radiography, which measures the small-angle scattering occurring when the X-rays hit tissue rather than its attenuation, is suitable for imaging of the human lung and that the signal strength is independent of demographic parameters such as age, gender, weight and height. The technique has been shown to have great potential, particularly for diagnosing emphysema.

Published
2022

26. ABCA3-related interstitial lung disease beyond infancy.

Author

Yang Li, Seidl, Elias, Knoflach, Katrin, Gothe, Florian, Forstner, Maria Elisabeth, Michel, Katarzyna, Pawlita, Ingo, Gesenhues, Florian, Sattler, Franziska, Xiaohua Yang, Kroener, Carolin, Reu-Hofer, Simone, Ley-Zaporozhan, Julia, Kammer, Birgit, Krüger-Stollfuß, Ingrid, Dinkel, Julien, Carlens, Julia, Wetzke, Martin, Moreno-Galdó, Antonio, and Torrent-Vernetta, Alba

Subjects
INTERSTITIAL lung diseases, INFANTS, IDIOPATHIC pulmonary fibrosis, FORCED expiratory volume
Published
2023
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29. Persistent Tachypnea of Infancy. Usual and Aberrant

Author

Rauch, Daniela, Wetzke, Martin, Reu, Simone, Wesselak, Waltraud, Schams, Andrea, Hengst, Meike, Kammer, Birgit, Ley-Zaporozhan, Julia, Kappler, Matthias, Proesmans, Marijke, Lange, Joanna, Escribano, Amparo, Kerem, Eitan, Ahrens, Frank, Brasch, Frank, Schwerk, Nicolaus, and Griese, Matthias

Published
2016
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31. Total Psoas Muscle Area as a Marker for Sarcopenia Is Related to Outcome in Children With Neuroblastoma

Author

Ritz, Annika, Froeba-Pohl, Alexandra, Kolorz, Julian, Vigodski, Victor, Hubertus, Jochen, Ley-Zaporozhan, Julia, von Schweinitz, Dietrich, Häberle, Beate, Schmid, Irene, Kappler, Roland, Lurz, Eberhard, and Berger, Michael

Subjects
Medizin, Surgery
Abstract

Background: Sarcopenia describes a generalized loss of skeletal muscle mass, strength, or function. Determined by measuring the total psoas muscle area (tPMA) on cross-sectional imaging, sarcopenia is an independent marker for poor post-surgical outcomes in adults and children. Children with cancer are at high risk for sarcopenia due to immobility, chemotherapy, and cachexia. We hypothesize that sarcopenic children with neuroblastoma are at higher risk for poor post-operative outcomes. Patients and Methods: Retrospective analysis of children with neuroblastoma ages 1–15 years who were treated at our hospital from 2008 to 2016 with follow-up through March 2021. Psoas muscle area (PMA) was measured from cross-sectional images, using computed tomography (CT) and magnetic resonance imaging (MRI) scans at lumbar disc levels L3-4 and L4-5. tPMA is the sum of the left and right PMA. Z-scores were calculated using age- and gender-specific reference values. Sarcopenia was defined as a tPMA z-score below −2. A correlation of tPMA z-scores and sarcopenia with clinical variables and outcome was performed. Results: One hundred and sixty-four children with workup for neuroblastoma were identified, and 101 children fulfilled inclusion criteria for further analysis, with a mean age of 3.92 years (SD 2.71 years). Mean tPMA z-score at L4-5 was −2.37 (SD 1.02). Correlation of tPMA z-score at L4-5 with weight-for-age z-score was moderate (r = 0.54; 95% CI, 0.38, 0.66). No association between sarcopenia and short-term outcome was observed. Sarcopenia had a sensitivity of 0.82 (95% CI, 0.62–0.93) and a specificity of 0.48 (95% CI 0.36–0.61) in predicting 5-year survival. In a multiple regression analysis, pre-operative sarcopenia, pre-operative chemotherapy in the NB2004 high-risk group, unfavorable tumor histology, and age at diagnosis were associated with 5-year survival after surgery, with hazard ratios of 4.18 (95% CI 1.01–17.26), 2.46 (95% CI 1.02–5.92), 2.39 (95% CI 1.03–5.54), and 1.01 (95% CI 1.00–1.03), respectively. Conclusion: In this study, the majority of children had low tPMA z-scores and sarcopenia was a risk factor for decreased 5-year survival in children with neuroblastoma. Therefore, we suggest measuring the tPMA from pre-surgical cross-sectional imaging as a biomarker for additional risk stratification in children with neuroblastoma. CA Berger

Published
2021
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39. Early‐onset, fatal interstitial lung disease in STAT3 gain‐of‐function patients

Author

Gothe, Florian, primary, Gehrig, Jonathan, additional, Rapp, Christina K., additional, Knoflach, Katrin, additional, Reu‐Hofer, Simone, additional, Länger, Florian, additional, Schramm, Dirk, additional, Ley‐Zaporozhan, Julia, additional, Ehl, Stephan, additional, Schwerk, Nicolaus, additional, Faletti, Laura, additional, and Griese, Matthias, additional

Published
2021
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46. SARS-CoV-2 triggering severe ARDS and secondary HLH in a 3-year-old child with Down syndrome

Author

Kim-Hellmuth, Sarah, Hermann, Matthias, Eilenberger, Julia, Ley-Zaporozhan, Julia, Fischer, Marcus, Hauck, Fabian, Klein, Christoph, Haas, Nikolaus, Kappler, Matthias, Huebner, Johannes, Jakob, André, and von Both, Ulrich

Subjects
Male, Alanine, Critical Care, SARS-CoV-2, Down syndrome, Prednisolone, hyperinflammation, Remdesivir, COVID-19, Immunoglobulins, Intravenous, Case Report, Antiviral Agents, Adenosine Monophosphate, Systemic Inflammatory Response Syndrome, Lymphohistiocytosis, Hemophagocytic, COVID-19 Drug Treatment, paediatrics, AcademicSubjects/MED00290, COVID-19 Testing, Child, Preschool, Humans, Genetic Predisposition to Disease, AcademicSubjects/MED00670, sHLH, Glucocorticoids
Abstract

Down syndrome (DS) predisposes to severe immunologic reaction secondary to infectious triggers. Here, we report a pediatric DS patient with coronavirus disease 2019 (COVID-19) who developed a hyperinflammatory syndrome, severe acute respiratory distress syndrome, and secondary hemophagocytic lymphohistiocytosis requiring pediatric intensive care unit admission and treatment with steroids, intravenous immunoglobulin, and remdesivir. Investigations into genetic susceptibilities for COVID-19 and severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2)-associated complications warrant systematic clinical and scientific studies. We report a pediatric Down syndrome patient with coronavirus disease 2019 (COVID-19) who developed secondary hemophagocytic lymphohistiocytosis requiring treatment with steroids, intravenous immunoglobulin, and remdesivir. Investigations into genetic susceptibilities for COVID-19-associated complications warrant systematic clinical and scientific studies.

Published
2020

47. Impact of lung morphology on clinical outcomes with riociguat in patients with pulmonary hypertension and idiopathic interstitial pneumonia: A post hoc subgroup analysis of the RISE-IIP study

Author

Nathan, Steven D, Cottin, Vincent, Behr, Juergen, Hoeper, Marius M, Martinez, Fernando J, Corte, Tamera J, Keogh, Anne M, Leuchte, Hanno, Mogulkoc, Nesrin, Ulrich, Silvia, Wuyts, Wim A, Yao, Zhen, Ley-Zaporozhan, Julia, Müller-Lisse, Ullrich G, Scholle, Frank-Detlef, Brüggenwerth, Günther, Busse, Dennis, Nikkho, Sylvia, Wells, Athol U, Nathan, Steven D, Cottin, Vincent, Behr, Juergen, Hoeper, Marius M, Martinez, Fernando J, Corte, Tamera J, Keogh, Anne M, Leuchte, Hanno, Mogulkoc, Nesrin, Ulrich, Silvia, Wuyts, Wim A, Yao, Zhen, Ley-Zaporozhan, Julia, Müller-Lisse, Ullrich G, Scholle, Frank-Detlef, Brüggenwerth, Günther, Busse, Dennis, Nikkho, Sylvia, and Wells, Athol U

Abstract

Background Riociguat in Patients with Symptomatic Pulmonary Hypertension associated with Idiopathic Interstitial Pneumonias (RISE-IIP), a randomized, controlled, phase 2b trial of riociguat for pulmonary hypertension associated with idiopathic interstitial pneumonia, was terminated early due to increased mortality in riociguat-treated patients. Baseline characteristics of enrolled patients demonstrated a low diffusing capacity of the lung for carbon monoxide (DLCO) with preserved lung volumes at baseline, suggesting the presence of combined pulmonary fibrosis and emphysema (CPFE) in some patients. This post hoc analysis of RISE-IIP was undertaken to explore lung morphology, assessed by high-resolution computed tomography, and associated clinical outcomes. Methods Available baseline/pre-baseline high-resolution computed tomography scans were reviewed centrally by 2 radiologists. The extent of emphysema and fibrosis was retrospectively scored and combined to provide the total CPFE score. Results Data were available for 65/147 patients (44%), including 15/27 fatal cases (56%). Of these, 41/65 patients (63%) had CPFE. Mortality was higher in patients with CPFE (12/41; 29%) than those without (3/24; 13%). Fourteen patients with CPFE had emphysema > fibrosis (4 died). No relationship was observed between CPFE score, survival status, and treatment assignment. A low DLCO, short 6-min walking distance, and high forced vital capacity:DLCO ratio at baseline also appeared to be risk factors for mortality. Conclusions High parenchymal lung disease burden and the presence of more emphysema than fibrosis might have predisposed patients with pulmonary hypertension associated with idiopathic interstitial pneumonia to poor outcomes in RISE-IIP. Future studies of therapy for group 3 pulmonary hypertension should include centrally adjudicated imaging for morphologic phenotyping and disease burden evaluation during screening.

Published
2021

49. Aortic dimensions on cardiovascular magnetic resonance imaging relate to pregnancy outcomes in women with coarctation of the aorta: a multicenter study

Author

Juan Laura, Krieger Eric, Valente Anne, Ley-Zaporozhan Julia, Moshonov Hadas, Wintersperger Bernd J, Silversides Candice, Siu Sam, Crean Andrew M, Ley Sebastian, Colman Jack M, Nguyen Elsie T, Paul Narinder S, Sermer Mathew, and Wald Rachel M

Subjects
Diseases of the circulatory (Cardiovascular) system, RC666-701
Published
2012
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50. Heterozygous OAS1 gain-of-function variants cause an autoinflammatory immunodeficiency

Author

Magg, Thomas, primary, Okano, Tsubasa, additional, Koenig, Lars M., additional, Boehmer, Daniel F.R., additional, Schwartz, Samantha L., additional, Inoue, Kento, additional, Heimall, Jennifer, additional, Licciardi, Francesco, additional, Ley-Zaporozhan, Julia, additional, Ferdman, Ronald M., additional, Caballero-Oteyza, Andrés, additional, Park, Esther N., additional, Calderon, Brenda M., additional, Dey, Debayan, additional, Kanegane, Hirokazu, additional, Cho, Kazutoshi, additional, Montin, Davide, additional, Reiter, Karl, additional, Griese, Matthias, additional, Albert, Michael H., additional, Rohlfs, Meino, additional, Gray, Paul, additional, Walz, Christoph, additional, Conn, Graeme L., additional, Sullivan, Kathleen E., additional, Klein, Christoph, additional, Morio, Tomohiro, additional, and Hauck, Fabian, additional

Published
2021
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