Pulmonary interstitial glycogenosis - A systematic analysis of new cases
| العنوان: | Pulmonary interstitial glycogenosis - A systematic analysis of new cases |
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| المؤلفون: | C. Lex, Julia Ley-Zaporozhan, Sune Rubak, Matthias Griese, Elias Seidl, D. Borensztajn, Nicolaus Schwerk, Julia Carlens, E. Haarmann, Frank Brasch, Traudl Wesselak, Luise A. Schuch, Petra Schelstraete, B. Hinrichs, Matthias Kappler, M. van de Loo, Martin Wetzke, Daniela Rauch, M. Wolf, Andrea Schams, Karl Reiter, F. Stehling, Simone Reu |
| المساهمون: | VU University medical center |
| المصدر: | Seidl, E, Carlens, J, Reu, S, Wetzke, M, Ley-Zaporozhan, J, Brasch, F, Wesselak, T, Schams, A, Rauch, D, Schuch, L, Kappler, M, Schelstraete, P, Wolf, M, Stehling, F, Haarmann, E, Borensztajn, D, van de Loo, M, Rubak, S, Lex, C, Hinrichs, B, Reiter, K, Schwerk, N & Griese, M 2018, ' Pulmonary interstitial glycogenosis-A systematic analysis of new cases ', Respiratory Medicine, vol. 140, pp. 11-20 . https://doi.org/10.1016/j.rmed.2018.05.009 Seidl, E, Carlens, J, Reu, S, Wetzke, M, Ley-Zaporozhan, J, Brasch, F, Wesselak, T, Schams, A, Rauch, D, Schuch, L, Kappler, M, Schelstraete, P, Wolf, M, Stehling, F, Haarmann, E, Borensztajn, D, van de Loo, M, Rubak, S, Lex, C, Hinrichs, B, Reiter, K, Schwerk, N & Griese, M 2018, ' Pulmonary interstitial glycogenosis – A systematic analysis of new cases ', Respiratory Medicine, vol. 140, pp. 11-20 . https://doi.org/10.1016/j.rmed.2018.05.009 Respiratory Medicine, 140, 11-20. W.B. Saunders Ltd |
| سنة النشر: | 2018 |
| مصطلحات موضوعية: | Lung Diseases, Male, Biopsy, Medizin, Gastroenterology, FAVORABLE RESPONSE, 0302 clinical medicine, Registries, Child, Glycogen Storage Disease/diagnosis, Tomography, Lung, Respiratory distress, children's interstitial lung disease, Interstitial lung disease, Glycogen Storage Disease, X-Ray Computed, Interstitial thickening, Child, Preschool, Diffuse parenchymal lung disease, Breathing, Female, Infants, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Lung/pathology, Gestational Age, Lung biopsy, Drug Administration Schedule, Interstitial/diagnosis, 03 medical and health sciences, Rare Diseases, Lung Diseases, Interstitial/diagnosis, 030225 pediatrics, Internal medicine, medicine, Humans, Preschool, Glucocorticoids, Retrospective Studies, Pulmonary interstitial glycogenosis, business.industry, Glucocorticoids/administration & dosage, Infant, Rare Diseases/diagnosis, medicine.disease, 030228 respiratory system, Lung Diseases, Interstitial, business, Tomography, X-Ray Computed |
| الوصف: | BACKGROUND: Pulmonary interstitial glycogenosis (PIG) is a rare paediatric interstitial lung disease of unknown cause. The diagnosis can only be made by lung biopsy. Less than 100 cases have been reported. Clinical features, treatment and outcomes have rarely been assessed systematically in decent cohorts of patients.METHODS: In this retrospective multicentre study, the clinical presentation, radiologic findings, pattern of lung biopsy, extrapulmonary comorbidities, treatment and outcome of eleven children with PIG were collected systematically.RESULTS: 10/11 children presented with respiratory distress immediatly after birth and 8/11 needed invasive ventilation. In 8/11 children extrapulmonary comorbidities were present, congenital heart defects being the most common. 7/11 children received systemic glucocorticoids and of these four showed a clear favorable response. During a median follow-up of 3.0 years (range 0.42-12.0) one child died, while 10 patients improved. Chest CT-scans showed ground-glass opacities (7/10), consolidations (6/10), linear opacities (5/10) and mosaic attenuation (4/10) without uniform pattern. Besides interstitial thickening related to undifferentiated glycogen positive mesenchymal cells all tissue samples showed growth abnormalities with reduced alveolarization.CONCLUSIONS: PIG is associated with alveolar growth abnormalities and has to be considered in all newborns with unexplained respiratory distress. Apparent treatment benefit of glucocorticosteroids needs to be evaluated systematically. |
| اللغة: | English |
| تدمد: | 0954-6111 |
| DOI: | 10.1016/j.rmed.2018.05.009 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::32e20e176e51e994ead3f5ee1e2d5aa7 https://pure.au.dk/portal/da/publications/pulmonary-interstitial-glycogenosis--a-systematic-analysis-of-new-cases(dbe90159-15b3-4bf6-97ca-9733d428f815).html |
| Rights: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....32e20e176e51e994ead3f5ee1e2d5aa7 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 09546111 |
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| DOI: | 10.1016/j.rmed.2018.05.009 |