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. 2009 Dec 12:4:26.
doi: 10.1186/1750-1172-4-26.

Incidence and classification of pediatric diffuse parenchymal lung diseases in Germany

Matthias Griese  1 , Melanie Haug, Frank Brasch, Achim Freihorst, Peter Lohse, Rüdiger von Kries, Theodor Zimmermann, Dominik Hartl
Affiliations

Incidence and classification of pediatric diffuse parenchymal lung diseases in Germany

Matthias Griese et al. Orphanet J Rare Dis. .

Abstract

Background: Diffuse parenchymal lung diseases (DPLD) in children represent a rare and heterogeneous group of chronic pulmonary disorders. Despite substantial advances in enetics and pathomechanisms, these often lethal diseases are still under-diagnosed. This is due to the fact that (i) the incidence is low, and (ii) clinical presentation, (iii) disease classification and (iv) specific treatment options are largely unknown.

Methods: Here we systematically assessed the incidence, the presentation, the diagnostic yield and treatments of pediatric DPLD in Germany, using the Surveillance Unit for Rare Paediatric Disorders (ESPED).

Results: The incidence of DPLD was 1.32 new cases per 1 million of children per year. The majority of these children were diagnosed within the first year of life. Overall survival was 87%. Using centralized data entry and stratification tools, the patients were categorized into an advanced classification system based on diagnostic algorithms, including clinical presentations, genetics and/or histology. Combining molecular and clinical information, this survey provides an etiological overview and specific diagnostic recommendations for children with DPLD.

Conclusions: Standardized surveys and systematic classifications are valuable tools for the clinical handling of children with DPLD and aim to improve the disease understanding and the prognosis of these rare detrimental lung diseases.

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Figures

Figure 1
Figure 1
Children with diffuse parenchymal lung diseases incident during 2005 and 2006 in Germany divided into different age groups. Given are absolute numbers and the percentages are indicated on top of the columns.
Figure 2
Figure 2
The pie-chart illustrates the distribution of the 38 diagnoses classified according to a recent categorization system [6] expressed as percentages.
See this image and copyright information in PMC

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