Prenatal diagnosis of congenital alveolar proteinosis (surfactant protein B deficiency)

M Stuhrmann, B Bohnhorst, U Peters… - … in Affiliation With the …, 1998 - Wiley Online Library
We report on the DNA‐based prenatal diagnosis of congenital pulmonary alveolar
proteinosis in a family in which alveolar proteinosis was associated with surfactant protein B …

Surfactant protein B: unambiguously necessary for adult pulmonary function

FS Cole - American Journal of Physiology-Lung Cellular …, 2003 - journals.physiology.org
IN THE REPORT FROM MELTON ET AL., one of the current articles in focus (Ref. 21, see p.
L543 in this issue), they use genetically engineered mice to demonstrate that at least 25% of …

Primary alveolar capillary dysplasia (acinar dysplasia) and surfactant protein B deficiency: a clinical, radiological and pathological study

CO Hugosson, HM Salama, F Al-Dayel, N Khoumais… - Pediatric …, 2005 - Springer
Background: Full-term infants with severe and prolonged respiratory distress represent a
diagnostic challenge. Plain radiographic findings may be nonspecific or similar to classic …

Congenital alveolar proteinosis in the Netherlands: a report of five cases with immunohistochemical and genetic studies on surfactant apoproteins

AA de la Fuente, WF Voorhout… - Pediatric Pathology & …, 1997 - Taylor & Francis
Congenital alveolar proteinosis is a recently described cause of lung dysfunction and
respiratory distress in term neonates. In several cases a deficiency or insufficiency of …

Inherited surfactant protein-B deficiency

A Hamvas - Advances in pediatrics, 1997 - Elsevier
Pulmonary surfactant is a complex mixture of phospholipid and protein that is synthesized by
alveolar type II pneumocytes and is critical for maintaining alveolar expansion at end …

Surfactant apoprotein B deficiency.

PA Chetcuti, RJ Ball - Archives of Disease in Childhood. Fetal and …, 1995 - ncbi.nlm.nih.gov
Fatal respiratory distress in term infants is rare and possible causes include persistent fetal
circulation, meconium aspiration syndrome, pneumonia or congen-ital heart and lung …

Use of transgenic mice to study lung morphogenesis and function

JP Bridges, TE Weaver - ILAR journal, 2006 - academic.oup.com
Successful transition to air breathing at birth depends on perinatal maturation of the gas
exchange surface, resorption of fluid from the air spaces, and synthesis and secretion of …

Surfactant replacement therapy in lung injury

PR Chess, JN Finkelstein, BA Holm - Lung Injury, 2005 - api.taylorfrancis.com
This chapter examines exogenous surfactant therapy and its utility in miti-gating clinical
acute lung injury (ALI) and the acute respiratory distress syndrome (ARDS). Extensive …

Respiratory distress syndrome

HL Halliday - Neonatal respiratory disorders, 2003 - api.taylorfrancis.com
Figure 19.1 Incidence of RDS at different gestations.(Adapted from Greenough, A., Morley,
CJ, Roberton, NRC (1992) Acute respiratory disease in the newborn. In Roberton, NRC …

Classification of pulmonary alveolar proteinosis in newborns, infants, and children

F Brasch, KM Müller - Der Pathologe, 2004 - Springer
Alveolarproteinosen sind seltene Lungenerkrankungen, die durch eine intraalveoläre
Akkumulation des Surfactantprotein (SP-) A charakterisiert sind. Folgende Subtypen können …