All children with DPLD (= ILD) and those who have got a lung biopsy

1. All diffus parenchymatous lung diseases (DPLD), also called interstitial lung diseases, are rare and should be collected and followed long term in the register. The entities covered are listed in tab. 1.

The current classification table is here.

2. Additionally, all children with a diagnostic lung biopsy should be included.


Tab. 1 List of the diagnosis to be included in the register and biobank. Entities not yet covered in the list are also welcome.

A1 DPLD-Diffuse developmental disorders
 Acinar dysplasia
 Alveolar capillary dysplasia with misalignment pulmonary veins
 Alveloar capillary dysplasia, no misalignment of pulm.veins (+ anophthalmia, cong. heart disease, diaph. hernia, lung hypoplasia, ment. retard., others)
 Congenital alveolar dysplasia
A2 DPLD-Growth abnormalities reflecting deficient alveolarisation
 Intrauterine growth retardation (alcohol)
 Pulmonary hypoplasia
 Pulmonary hypoplasia associated with diagphragmatic hernia
 Related to chromosomal disorders
Related to congenital heart disease
Related to preterm birth (BDP-cLDI)
Related to preterm birth (Wilson Mikity, new BPD)
A3 DPLD-Infant chronic tachypnoe and firm morphology
Chronic tachypnoe of infancy (CTI)
Neuroendocrine cell hyperplasia of infancy (NEHI)
Pulmonary interstitial glycogenosis (PIG)
A4 DPLD–related to alveolar surfactant region
ABCA3 mutations 1
ABCA3 mutations 2
Alveolar microlithiasis
Chronic pneumonitis of infancy (CPI)
Desquamative interstitial pneumonitis (DIP)
Lipoidpneumonitis, Cholesterol pneumonia
Nkx21 gene defect
Nonspecific interstitial pneumonia (NSIP)
Pulmonary alveolar proteinosis (PAP), adult NO GMCSF autoantibodies
PAP, adult with GMCSF autoantibodies
PAP, juvenile
PAP, neonatal
PAP, secondary to associated disease
PAP+DIP+cholesterin granulomas
Surfactant protein B mutations
Surfactant protein C mutations
Usual interstitial pneumonitis
Cryptogenic Organizing Pneumonia (Bronchiolitis Obliterans Organizing Pneumonia)  
Diffuse Alveolar Damage and Acute Interstitial Pneumonia 
Acute Fibrinous and Organizing Pneumonia
Respiratory Bronchiolitis-Interstitial Lung Disease 
Ax DPLD-unclear RDS in the mature neonate
to be determined
No or very low SP-C biochemically
No SP-B biochemically
Pulmonary hypertension
Ay DPLD-unclear RDS in the almost (30-36 wks) mature neonate
No or very low SP-C biochemically
No SP-B biochemically
Pulmonary hypertension
B1 DPLD-related to systemic disease processes
Achondroplasie,  zB Cartilage-Hair Hypoplasia
Familial dysautonomia (Chromosome 9q31 encoding ICAP)
Familial ILD, growth defic., hepatopathy, normal psychomotor development, triventri.hydrocephalus
Hermansky-Pudlak Syndrome
Hoyeral Hreidasson Syndrom (Dyskeratosis congenita)
Immune-mediated/collagen vascular disorders
Diffuse alveolar hemorrhage due to vasculitic disorders
Idiopathic pulmonary hemosiderosis
Antibasement Membrane Antibody Disease (Good pasture’s Syndrome) 
Lymphangioleiomyomatosis (LAM)
Erdheim-Chester Disease
Langerhans cell histiocytosis
Sinus Histiocytosis with Massive Lymphadenopathy
Churg-Strauss Syndrome
Microscopic Polyangiitis
Necrotizing Sarcoid Granulomatosis
Other Rare Causes of Granulomatous Arteritis
Giant Cell Arteritis
Disseminated Visceral Giant Cell Angiitis
Polyarteritis Nodosa
Takayasu's Arteritis
Behcet's Syndrome
Blau Syndrome (polyarthritis, uveitis, papuloerythematous rash) + rarely lung.
Storage diseases
Wegener Granulomatosis
B2 DPLD-in the presumed  immune intact host, related to exposures (infectious/non-infectious)
Aspiration syndromes
Central hypoventilation syndrome (Ondine)
Congenital muscle disease
Eosinophilic pneumonitis
Exogen allergic alveolitis/hypersensitivity pneumonitis
Infectious/post-infectious processes
Drug Reactions
Occupational Lung Diseases and Pneumoconioses
Radiation Lung Injury 
Toxic inhalation
B3 DPLD-in the immunocompromised host or transplanted
Diffuse lung damage of unknown etiology
Infections–Antibody deficiencies
Infections–Phagocyte defects
Infections–T cell deficiencies
Related to therapeutic intervention
Related to transplantation and rejection
B4 DPLD-related to lung vessels structural processes
Arterial hypertensive vasculopathy (Primary pulmonary hypertension Chr 2q33)
Congestive changes related to cardiac dysfunction
Lymphatic disorders
M. Osler
Pulmonary capillary hemangiomatosis
Pulmonary hemorrhage
Pulmonary hemorrhage due to infection
Pulmonary hypertension
Veno-occlusive disease
B5 DPLD-related to reactive lypmphoid lesions
Follicular bronchitis/bronchiolits
Giant lymphnode hyperplasia (Castleman´s disease)
Intrapulmonary lymph nodes
Lymphocytic interstitial pneumonia (LIP)
Nodular lymphoid hyperplasia of the lung
Bx DPLD-unclear RDS in the NON-neonate
to be determined