Alle Kinder mit DPLD (= ILD)
1. Alle diffus parenchymatösen Lungenerkrankungen (DPLD), auch interstitielle Lungenerkrankungen genannt, sollten wegen ihrer Seltenheit im Register gesammelt und im Langzeitverlauf beobachtet werden.
Die einzelnen Erkrankungen sind in Tab. 1 gelistet.
Die aktuelle Klassifikation findet sich hier.
2. Ferner sollten alle Kinder, die eine diagnostische Lungenbiopsie bekommen, eingeschlossen werden.
Tab. 1 Listung der ins Register einzuschließenden Diagnosen. Selbstverständlich können gerne Kinder mit Diagnosen, die nocht nicht genannt sind eingeschlossen werden.
| A1 DPLD-Diffuse developmental disorders |
| Acinar dysplasia |
| Alveolar capillary dysplasia (ACD) with misalignment pulmonary vein |
| Alveloar capillary dysplasia, no misalignment of pulm.veins (event. + anoph-thalmia, cong. heart disease, diaph. hernia, lung hypoplasia, ment. retard. |
| 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) |
| NSIP+PAP+Microvasulopathie |
| 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 |
| Familial |
| 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 |
| Familial |
| 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. |
| Sarcoidosis |
| 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 |
| Mac-Leod-Swyer-James-Syndrom |
| 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–Miscellaneous |
| 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 |
| Familial |