[Classification of pulmonary alveolar proteinosis in newborns, infants, and children]. / Klassifikation der Alveolarproteinosen im Neugeborenen-, Säuglings- und Kindesalter.
Pathologe
; 25(4): 299-309, 2004 Jul.
Article
en De
| MEDLINE
| ID: mdl-15241624
ABSTRACT
Pulmonary alveolar proteinoses are rare pulmonary diseases characterised by an intraalveolar accumulation of surfactant protein A. Subtyping of alveolar proteinoses Type I alveolar proteinoses severe respiratory insufficiency in newborns, which will take a lethal course without lung transplant; hereditary SP-B deficiency and an intraalveolar accumulation of N-terminal incompletely processed SP-C. Type II alveolar proteinoses occur in newborns and infants; often take a lethal course; show intraalveolar accumulation of precursors of SP-B and mature SP-B as well as an accompanying interstitial lung disease of variable severity. Type III alveolar proteinoses in infants and children; do not generally take a lethal course; they are characterised by an intraalveolar accumulation of precursors of SP-B and mature SP-B without accompanying interstitial lung disease. "Cryptogenic" congenital, acquired (idiopathic), and secondary type III alveolar proteinoses can be distinguished. In newborns, infants, and children with pulmonary alveolar proteinosis, a detailed pathological-anatomical examination including immunohistochemical and molecular genetic analyses, should be performed in order to optimise the therapeutical management.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Proteinosis Alveolar Pulmonar
Tipo de estudio:
Diagnostic_studies
Límite:
Child
/
Humans
/
Infant
/
Newborn
Idioma:
De
Revista:
Pathologe
Año:
2004
Tipo del documento:
Article