作者
Elias Seidl, Julia Carlens, Simone Reu, Martin Wetzke, Julia Ley-Zaporozhan, Frank Brasch, T Wesselak, Andrea Schams, Daniela Rauch, Luise Schuch, Matthias Kappler, Petra Schelstraete, Monika Wolf, Floria Stehling, Eric Haarmann, Dorine Borensztajn, Moniek Van de Loo, Sune Rubak, C Lex, B Hinrichs, K Reiter, N Schwerk, M Griese
发表日期
2018/7/1
来源
Respiratory Medicine
卷号
140
页码范围
11-20
出版商
WB Saunders
简介
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 …
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E Seidl, J Carlens, S Reu, M Wetzke… - Respiratory Medicine, 2018