title: Diffuse lung disease in young children: application of a novel classification scheme.
abstract: BACKGROUND
  Considerable confusion exists regarding nomenclature, classification, and management of pediatric diffuse lung diseases due to the relative rarity and differences in the spectrum of disease between adults and young children.
  
  
  OBJECTIVE
  A multidisciplinary working group was formed to: (1) apply consensus terminology and diagnostic criteria for disorders presenting with diffuse lung disease in infancy; and (2) describe the distribution of disease entities, clinical features, and outcome in young children who currently undergo lung biopsy in North America.
  
  
  METHODS
  Eleven centers provided pathologic material, clinical data, and imaging from all children less than 2 years of age who underwent lung biopsy for diffuse lung disease from 1999 to 2004.
  
  
  RESULTS
  Multidisciplinary review categorized 88% of 187 cases. Disorders more prevalent in infancy, including primary developmental and lung growth abnormalities, neuroendocrine cell hyperplasia of infancy, and surfactant-dysfunction disorders, constituted the majority of cases (60%). Lung growth disorders were often unsuspected clinically and under-recognized histologically. Cases with known surfactant mutations had characteristic pathologic features. Age at biopsy and clinical presentation varied among categories. Pulmonary hypertension, presence of a primary developmental abnormality, or ABCA3 mutation was associated with high mortality, while no deaths occurred in cases of pulmonary interstitial glycogenosis, or neuroendocrine cell hyperplasia of infancy.
  
  
  CONCLUSIONS
  This retrospective cohort study identifies a diverse spectrum of lung disorders, largely unique to young children. Application of a classification scheme grouped clinically distinct patients with variable age of biopsy and mortality. Standardized terminology and classification will enhance accurate description and diagnosis of these disorders.
authors: Divisions of Pathology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors: Gail H
authors: GH
authors: Deutsch
authors: Lisa R
authors: LR
authors: Young
authors: Robin R
authors: RR
authors: Deterding
authors: Leland L
authors: LL
authors: Fan
authors: Sharon D
authors: SD
authors: Dell
authors: Judy A
authors: JA
authors: Bean
authors: Alan S
authors: AS
authors: Brody
authors: Lawrence M
authors: LM
authors: Nogee
authors: Bruce C
authors: BC
authors: Trapnell
authors: Claire
authors: C
authors: Langston
authors: Eric A
authors: EA
authors: Albright
authors: Frederic B
authors: FB
authors: Askin
authors: Peter
authors: P
authors: Baker
authors: Pauline M
authors: PM
authors: Chou
authors: Carlyne M
authors: CM
authors: Cool
authors: Susan C
authors: SC
authors: Coventry
authors: Ernest
authors: E
authors: Cutz
authors: Mary M
authors: MM
authors: Davis
authors: Megan K
authors: MK
authors: Dishop
authors: Csaba
authors: C
authors: Galambos
authors: Kathleen
authors: K
authors: Patterson
authors: William D
authors: WD
authors: Travis
authors: Susan E
authors: SE
authors: Wert
authors: Frances V
authors: FV
authors: White
mesh_terms: D018528:ATP-Binding Cassette Transporters / Q000235:genetics; D015331:Cohort Studies; D004700:Endocrine System Diseases / Q000145:classification; D006130:Growth Disorders / Q000145:classification; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000254:growth & development / Q000473:pathology; D008171:Lung Diseases / Q000145:classification* / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology; D009154:Mutation; D009422:Nervous System Diseases / Q000145:classification; D011663:Pulmonary Surfactants; D012189:Retrospective Studies; D012720:Severity of Illness Index; D009626:Terminology as Topic
chemical_list: C441524:ABCA3 protein, human; D018528:ATP-Binding Cassette Transporters; D011663:Pulmonary Surfactants