Incidence and classification of pediatric diffuse parenchymal lung diseases in Germany

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

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.

*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.
**Two mutations means compound heterozygous or homozygous mutations in the ABCA3 gene
***Diagnosis in co-operation with German consultation network for rare lung diseases
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.

ISSN: 1750-1172