Warning: Undefined variable $zfal in C:\Inetpub\vhosts\kidney.de\httpdocs\mlpefetch.php on line 525
Deprecated: str_replace(): Passing null to parameter #3 ($subject) of type array|string is deprecated in C:\Inetpub\vhosts\kidney.de\httpdocs\mlpefetch.php on line 525
Warning: Undefined variable $sterm in C:\Inetpub\vhosts\kidney.de\httpdocs\mlpefetch.php on line 530
free
Warning: Undefined variable $sterm in C:\Inetpub\vhosts\kidney.de\httpdocs\mlpefetch.php on line 531
free free
English Wikipedia
Nephropedia Template TP (
Twit Text
DeepDyve Pubget Overpricing |
lüll Diffuse lung disease in young children: application of a novel classification scheme Deutsch GH; Young LR; Deterding RR; Fan LL; Dell SD; Bean JA; Brody AS; Nogee LM; Trapnell BC; Langston C; Albright EA; Askin FB; Baker P; Chou PM; Cool CM; Coventry SC; Cutz E; Davis MM; Dishop MK; Galambos C; Patterson K; Travis WD; Wert SE; White FVAm J Respir Crit Care Med 2007[Dec]; 176 (11): 1120-8RATIONALE: 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. OBJECTIVES: 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. MEASUREMENTS AND MAIN 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.|ATP-Binding Cassette Transporters/genetics[MESH]|Cohort Studies[MESH]|Endocrine System Diseases/classification[MESH]|Growth Disorders/classification[MESH]|Humans[MESH]|Hypertension, Pulmonary/classification[MESH]|Infant[MESH]|Infant, Newborn[MESH]|Lung Diseases/*classification/diagnosis/mortality/physiopathology[MESH]|Lung/growth & development/pathology[MESH]|Mutation[MESH]|Nervous System Diseases/classification[MESH]|Pulmonary Surfactants[MESH]|Retrospective Studies[MESH]|Severity of Illness Index[MESH]|Terminology as Topic[MESH] |