Absence of lamellar bodies accumulation of dense bodies characterizes a novel form of congenital surfactant defect

Pediatric and Developmental Pathology

Volumn 3, Issue 4, 2000, Pages 335-345

  Tryka, A Francine ^a   Wert, Susan E ^b   Mazursky, Jon E ^a   Arrington, Robert W ^a   Nogee, Lawrence M ^c  

^a ARKANSAS CHILDREN S HOSPITAL   (United States)

^b CINCINNATI CHILDREN'S HOSPITAL MEDICAL CENTER   (United States)

^c JOHNS HOPKINS UNIVERSITY   (United States)

Author keywords

Deficiency; Neonatal respiratory failure; Surfactant

Indexed keywords

LUNG SURFACTANT; SURFACTANT PROTEIN A; SURFACTANT PROTEIN B; SURFACTANT PROTEIN C;

ARTICLE; CASE REPORT; CELL ULTRASTRUCTURE; CONGENITAL LUNG DISEASE; DENSE BODY; FEMALE; GENETIC ANALYSIS; HISTOPATHOLOGY; HUMAN; HUMAN TISSUE; IMMUNOHISTOCHEMISTRY; LAMELLAR BODY; LUNG BIOPSY; NEWBORN; PRIORITY JOURNAL;

BRONCHOALVEOLAR LAVAGE FLUID; DNA, COMPLEMENTARY; FEMALE; HUMANS; IMMUNOHISTOCHEMISTRY; INFANT, NEWBORN; LUNG; MACROPHAGES, ALVEOLAR; MUTATION; NUCLEAR FAMILY; PULMONARY SURFACTANTS; RESPIRATORY DISTRESS SYNDROME, NEWBORN;

EID: 0033946086     PISSN: 10935266     EISSN: None     Source Type: Journal    
DOI: 10.1007/s100249910048     Document Type: Article

References (30)

1. Nogee LM, deMello DE, Dehner LP, Colten HR. Brief report: deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis. N Engl J Med 1993;328: 406-410.
2. Nogee LM, Garnier G, Dietz HC, Singer L, Murphy AM, deMello DE, Colten HR. A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds. J Clin Invest 1994;93:1860-1863.
3. Stahlman MT, Gray ME, Ross GF, et al. Human surfactant protein-A contains blood group A antigenic determinants. Pediatr Res 1992;31:364-371.
4. Vorbroker DK, Profitt SA, Nogee LM, Whitsett JA. Aberrant processing of surfactant protein C in hereditary SP-B deficiency. Am J Physiol 1995;268:L647-L656.
5. Lin S, Akinibi HT, Breslin JS, Weaver TE. Structural requirements for targeting of surfactant protein B (SP-B) to secretory granules in vitro and in vivo. J Biol Chem 1996; 271:19689-19695.
6. Floros J, Veletza SV, Kotikalapudi P, et al. Dinucleotide repeats in the human surfactant protein-B gene and respiratory-distress syndrome. Biochem J 1995;305:583-590.
7. Ballard PL, Nogee LM, Beers MF, et al. Partial deficiency of surfactant protein B in an infant with chronic lung disease. Pediatrics 1995;96:1046-1052.
8. Murphy JD, Rabinovitch M, Goldstein JD, Reid LM. The structural basis of persistent pulmonary hypertension of the newborn infant. J Pediatr 1981;98:962-967.
9. Sorokin SP. A morphologic and cytochemical study on the great alveolar cell. J Histochem Cytochem 1966;14:884-897.
10. 9a. Pilot-Matias TJ, Kister SE, Fox JL, Kropp K, Glasser SW, Whitsett JA. Structure and organization of the gene encoding human surfactant phospholipid SP-B. DNA 1989;8:75-86.
11. 9b. Glasser SW, Korshagen TR, Perme CM, Pilot-Matias TJ, Kister SE, Whitsett JA. Two SP-C genes encoding human pulmonary surfactant proteolipid. J Biol Chem 1988;263: 10326-10331.
12. Hamvas A, Nogee LM, Mallory GB, et al. Lung transplantation for treatment of infants with surfactant protein B deficiency. J Pediatr 1997;130:231-239.
13. Whitsett JA, Nogee LM, Weaver TE, Horowitz AD. Human surfactant protein B: structure, function, regulation and genetic disease. Physiol Rev 1995;75:749-757.
14. Johansson J, Curstedt T, Robertson B. The proteins of the surfactant system. Eur Respir J 1994;7:372-391.
15. Hawgood S, Clements JA. Pulmonary surfactant and its apoproteins. J Clin Invest 1990;86:1-6.
16. Crouch E, Rust K, Marienchek, Parghi D, Chang D, Persson A. Developmental expression of pulmonary surfactant protein D (SP-D). Am J Respir Cell Mol Biol 1991;5:13-18.
17. Haagsman HP, van Golde LM. Synthesis and assembly of lung surfactant. Annu Rev Physiol 1991;53:441-464.
18. Hallman M, Enhorning G, Possmayer F. Composition and surface activity of normal and phosphatidylglycerol-deficient lung surfactant. Pediatr Res 1985;19:286-292.
19. Vorbroker DK, Voorhout WF, Weaver TE, Whitsett JA. Posttranslational processing of surfactant protein C in rat type II cells. Am J Physiol 1995;269:L727-L733.
20. Voorhout WF, Weaver TE, Haagsman HP, Geuze HJ, van Golde LMG. Biosynthetic routing of pulmonary surfactant proteins in alveolar type II cells. Microsc Res Tech 1993; 26:366-373.
21. Chevalier G, Collet AJ. In vivo incorporation of choline-3H, leucine-3H and galactose-3H in alveolar type II pneumocytes in relation of surfactant synthesis. A quantitative radioautographic study in mouse by electron microscopy. Anat Rec 1972;174:289-310.
22. Williams MC, Hawgood S, Hamilton RL. Changes in lipid structure produced by surfactant proteins SP-A, SP-B, and SP-C. Am J Respir Cell Mol Biol 1991;5:41-50.
23. Suzuki Y, Fujita Y, Kogishi K. Reconstitution of tubular myelin from synthetic lipids and proteins associated with pig pulmonary surfactant. Am Rev Respir Dis 1989;140: 75-81.
24. Korfhagen TR, Bruno MD, Ross GF, et al. Altered surfactant function and structure in SP-A gene targeted mice. Proc Natl Acad Sci USA 1996;93:9594-9599.
25. deMello DE, Heyman S, Phelps DS, et al. Ultrastructure of lung in surfactant protein B deficiency. Am J Respir Cell Mol Biol 1994;11:230-239.
26. Clark JC, Wert SE, Bachurski CJ, et al. Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice. Proc Natl Acad Sci USA 1995;92:7794-7798.
27. Akinbi HT, Breslin JS, Ikegami M, et al. Rescue of SP-B knockout mice with a truncated SP-B proprotein. J Biol Chem 1997;272:9640-9647.
28. Tryka AF, Witschi H, Gosslee DC, McArthur AH, Clapp NK. Patterns of cell proliferation during recovery from oxygen injury: species differences. Am Rev Respir Dis 1986;133:1055-1059.
29. Lindenschmidt RC, Tryka AF, Godfrey GA, Frome EL, Witschi HP. Intratracheal versus intravenous administration of bleomycin in mice: acute effects. Toxicol Appl Pharmacol 1986;85:69-77.
30. Ghadially FN. Lysosomes. Ultrastructural Pathology of the Cell and Matrix. London: Butterworths, 1988;606-607.