منابع مشابه
Pulmonary surfactant metabolism in infants lacking surfactant protein B.
Infants with inherited deficiency of pulmonary surfactant protein (SP) B develop respiratory failure at birth and die without lung transplantation. We examined aspects of surfactant metabolism in lung tissue and lavage fluid acquired at transplantation or postmortem from ten infants born at term with inherited deficiency of SP-B; comparison groups were infants with other forms of chronic lung d...
متن کاملFatal familial surfactant protein B deficiency.
Fatal familial surfactant protein B deficiency EDrroR,-In 1993 a sibship of three infants who died of hyaline membrane disease had had an absence of surfactant protein B (SP-B) and its mRNA in lung tissue.' The gene coding for SP-B has been located and a frameshift mutation in this gene has been identified in the reported sibship and in two other families .2 A further six cases of SP-B deficien...
متن کاملSurfactant protein B and C analogues.
Mammalian lung surfactant is a mixture of phospholipids and four surfactant-associated proteins (SP-A, SP-B, SP-C, and SP-D). Its major function is to reduce surface tension at the air-water interface in the terminal airways by the formation of a surface-active film highly enriched in dipalmitoyl phosphatidylcholine (DPPC), thereby preventing alveolar collapse during expiration. SP-A and SP-D a...
متن کاملAllelic heterogeneity in hereditary surfactant protein B (SP-B) deficiency.
Inability to produce surfactant protein B (SP-B) causes fatal neonatal respiratory disease. A frame-shift mutation (121ins2) is the predominant but not exclusive cause of disease. To determine the range of mechanisms responsible for SP-B deficiency, both alleles from 32 affected infants were characterized. Sixteen infants were homozygous for the 121ins2 mutation, 10 infants were heterozygous fo...
متن کاملPlasma pro-surfactant protein B and lung function decline in smokers.
Plasma pro-surfactant protein B (pro-SFTPB) levels have recently been shown to predict the development of lung cancer in current and ex-smokers, but the ability of pro-SFTPB to predict measures of chronic obstructive pulmonary disease (COPD) severity is unknown. We evaluated the performance characteristics of pro-SFTPB as a biomarker of lung function decline in a population of current and ex-sm...
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ژورنال
عنوان ژورنال: Circulation
سال: 2004
ISSN: 0009-7322,1524-4539
DOI: 10.1161/01.cir.0000140260.73611.fa