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Table 3 Categorization and diagnosis - Entities occurring at all ages (B), continues tables 1 and 2

From: Incidence and classification of pediatric diffuse parenchymal lung diseases in Germany

Patient No. Interstitial lung disease Final pulmonary diagnosis Classifi-cation of pediatric DPLD* Additional diagnosis Center type Coop-Diagnosis*** Lung biopsy, result Genetics SFTPB, SFTPC, ABCA3
22 Related to lung vessels/heart Idiopathic pulmonary hemosiderosis B1 Anemia U no Pulmonary hemosiderosis no
23 Related to lung vessels/heart Idiopathic pulmonary hemosiderosis B1 Celiac disease U no no n.k.
24 Related to systemic disease Sarcoidosis B1 Familial small stature, anemia R no Sarcoidosis n.k.
25 Immune intact host HSP B2   U no HSP n.k.
26 Immune intact host HSP B2   U yes HSP no
27 Immune intact host HSP B2 Celiac disease R no HSP n.k.
28 Immune intact host HSP B2   U no no n.k.
29 Immune intact host HSP B2   U no no n.k.
30 Immune intact host HSP B2 Insufficiency of tricuspidal valve R no no n.k.
31 Immune intact host HSP B2   U yes no n.k.
32 Immune intact host HSP B2   R no no n.k.
33 Immune intact host HSP B2   R no no n.k.
34 Immune intact host HSP B2   U no no n.k.
35 Immune intact host HSP B2 Diabetes mellitus type I, hyperthyreosis, adipositas R yes no n.k.
36 No further categorisation n.d. Bx Status following Clamydia pneumoniae pneumonia R no no n.k.
37 No further categorisation n.d. Bx   R no no n.k.
38 No further categorisation n.d. Bx Adenoids, hypertrophy of tonsils, episodes of upper airway obstruction R no no n.k.
  1. *Classification of pediatric DPLD according to Deutsch et al 2007; entities mainly prevalent in infants (A; i.e. diffuse developmental disorders (A1), alveolarization abnormalities (A2), specific conditions of undefined etiology (A3) and surfactant dysfunction disorders (A4)). Entities occurring at all ages (B; i.e. related to systemic diseases (B1), in immune-intact hosts (B2), in immunocompromised hosts (B3) and disorders masquerading as DPLD (B4)). A4, possible; Ax and Bx denote children with the clear clinical diagnosis of pediatric DPLD, in whom however the diagnostic process was stopped prematurely.
  2. **Two mutations means compound heterozygous or homozygous mutations in the ABCA3 gene
  3. ***Diagnosis in co-operation with German consultation network for rare lung diseases
  4. Abbreviations: HSP, Hypersensitivity pneumonitis, NEHI, Neuroendocrine cell hyperplasia of infancy; PAP, pulmonary alveolar proteinosis; NSIP, non-specific interstitial pneumonitis; DIP, desquamative interstitial pneumonitis; R, regional hospital; U, University hospital; n.d., not determined; SFTPB or SFTPC, gene encoding surfactant protein B or C.