Incidence and classification of pediatric diffuse parenchymal lung diseases in Germany.
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...
| Publicado en: | Orphanet Journal of Rare Diseases Vol. 4; pp. 26 - 27 |
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| Autores principales: | , , , , , , , , , , , , , , , |
| Formato: | Journal Article |
| Publicado: |
BioMed Central
2009
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105275922&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 105275922 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 17501172 38NP jtl: Orphanet Journal of Rare Diseases issn: 17501172 maglogo: N pubinfo: dt: 2009 vid: 4 pid: 24147 pub: BioMed Central artinfo: ui: 105275922 NLM20003372 2010516798 10.1186/1750-1172-4-26 NLM20003372 PMC2800105 105275922 ppf: 26 ppct: 1 formats: tig: atl: Incidence and classification of pediatric diffuse parenchymal lung diseases in Germany. aug: au: Griese M Haug M Brasch F Freihorst A Lohse P von Kries R Zimmermann T Hartl D Griese, Matthias Haug, Melanie Brasch, Frank Freihorst, Achim Lohse, Peter von Kries, Rüdiger Zimmermann, Theodor Hartl, Dominik affil: Dr von Haunersches Kinderspital, University of Munich, Lindwurmstr 4a, D-80337 Munich, Germany sug: subj: Lung Diseases, Interstitial Classification Lung Diseases, Interstitial Epidemiology Population Surveillance Methods Adolescence Child Child, Preschool Female Germany Incidence Infant Infant, Newborn Lung Pathology Lung Diseases, Interstitial Diagnosis Lung Diseases, Interstitial Physiopathology Male Adolescent: 13-18 years Child: 6-12 years Child, Preschool: 2-5 years Infant: 1-23 months Infant, Newborn: birth-1 month Female Male ab: 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. pubtype: Academic Journal doctype: Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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