Original Article
Brief Report
Deficiency of Pulmonary Surfactant Protein B in Congenital Alveolar Proteinosis
N Engl J Med 1993; 328:406-410February 11, 1993
- Article
Congenital pulmonary alveolar proteinosis is an uncommon cause of respiratory failure in full-term newborns1-4. Although its histopathological appearance is similar to that of the alveolar proteinosis observed in older children and adults,5 the congenital form of the illness follows a different clinical course. All reported infants with congenital alveolar proteinosis have died within the first year of life despite maximal medical therapy. The incidence and cause of congenital alveolar proteinosis are unknown. Familial cases have been reported, and it has been speculated that the cause is an inborn error of surfactant metabolism4.
In this report we describe two siblings with the typical course and histopathologic features of congenital alveolar proteinosis. Analysis of their lung tissue by immunologic and molecular biologic methods revealed an absence of one of the surfactant specific proteins, surfactant protein B (SP-B), and its messenger RNA (mRNA). SP-B is important for the proper biophysical function of surfactant, suggesting that the cause of respiratory failure in these infants with congenital alveolar proteinosis was an inherited deficiency of SP-B caused by a pretranslational mechanism (implied by the absence of mRNA).
Case Report
The case patient was a 3600-g boy, the product of a nonconsanguineous marriage, who was delivered by cesarean section at 40 weeks of gestation because of breech presentation, maternal fever, and maternal hypertension. Shortly after birth, respiratory distress developed, and he required intubation and mechanical ventilation with 100 percent oxygen. Chest radiographs demonstrated diffuse granularity and air bronchograms. At 60 hours of life, extracorporeal membrane oxygenation was instituted because of refractory hypoxemia; it was maintained for 14 days without notable improvement in pulmonary function. The patient continued to require mechanical ventilation with 70 to 100 percent oxygen. He was treated with parenteral antibiotics; all bacterial and viral cultures were negative. He received corticosteroids (initially dexamethasone and subsequently methylprednisolone) throughout his course. At two months of age he received two doses of an exogenous surfactant (Survanta, 4 ml per kilogram of body weight) intratracheally. His condition improved slightly after the first dose, but not after the second, and this therapy was not continued. An open-lung biopsy performed when the patient was three months old demonstrated the characteristic findings of pulmonary alveolar proteinosis: eosinophilic, diastase-resistant granular material that was positive on periodic acid-Schiff staining filled the air spaces. In addition, foamy alveolar macrophages and desquamated alveolar epithelial cells were noted within the proteinaceous material, and there was extensive interstitial fibrosis and hyperplasia of alveolar epithelial cells. The patient died of progressive respiratory failure at five months of age. An autopsy was not permitted.
Neither the parents nor the three living siblings had a history of pulmonary disease. Nineteen years earlier, a 3750-g sister born at term had died of respiratory disease at one month of age. After the results of the case patient's biopsy were known, the clinical course and autopsy slides of his sister were reexamined. Signs of respiratory distress had developed shortly after birth and led to progressive respiratory failure. Treatment included 100 percent oxygen; she did not receive mechanical ventilation or corticosteroids. The autopsy revealed alveolar proteinosis, as described above, and changes consistent with bronchopulmonary dysplasia.
Methods
Patients
Control lung tissue was obtained from patients who were undergoing lung transplantation for end-stage pulmonary disease. These included a 2-year-old girl with bronchopulmonary dysplasia who was receiving mechanical ventilation with 100 percent oxygen; a 15-year-old boy and an 11-year-old boy with pulmonary hypertension, neither of whom was receiving supplemental oxygen; a 13-year-old girl with the adult respiratory distress syndrome who was receiving mechanical ventilation with 100 percent oxygen; and a 17-year-old girl with cystic fibrosis who was receiving supplemental oxygen. Only the girl with the adult respiratory distress syndrome was treated with corticosteroids. An unused donor lung specimen from an 18-year-old man without pulmonary disease was also obtained. Specimens were obtained at the time of surgery and immediately frozen in liquid nitrogen. A lung obtained at autopsy from a 19-day-old full-term girl with meconium aspiration syndrome who had received mechanical ventilation with 100 percent oxygen and extracorporeal membrane oxygenation was used as a positive control for immunohistochemical analysis.
Antiserum and Immunohistochemical Studies
Antiserum directed against surfactant protein A (SP-A), SP-B, and surfactant proprotein C (proSP-C) was prepared as described elsewhere6-11. Immunostaining was performed on paraffin sections as previously described,12 with antiserum against SP-A, SP-B, and proSP-C, diluted 1:500 in phosphate-buffered saline. Sections were incubated with nonimmune rabbit serum for negative staining controls.
Protein Analysis
From 15 to 50 mg of lung tissue, frozen at the time of biopsy or removal for transplantation, was homogenized while on ice in TRIS buffer containing proteinase inhibitors, and boiled in loading buffer containing sodium dodecyl sulfate and β-mercaptoethanol. Aliquots containing 75 or 150 μg of total protein, as determined by a modified Lowry assay,13 were loaded directly onto polyacrylamide gels and underwent electrophoresis with tricine in the cathode buffer for resolution of low-molecular-weight proteins14. The surfactant proteins were detected by immunoblotting with the antiserum against SP-A, SP-B, and proSP-C, as previously described15.
RNA Analysis
Frozen lung tissue was pulverized in liquid nitrogen, and RNA was isolated by an acid-phenol extraction method16. In each sample, 10 μg of total cellular RNA was separated on a 1 percent agarose formaldehyde gel and transferred to a nylon membrane17. Human complementary DNA (cDNA) probes specific for SP-A,18 SP-B,19 and SP-C20 were radiolabeled with [32P]deoxycytidine triphosphate (ICN Pharmaceuticals) with use of a commercially available random-priming kit (Boehringer-Mannheim Biochemicals). The blots were hybridized, washed in solutions as previously described,15 and exposed to x-ray film (Kodak). The blots were sequentially probed with SP-C, SP-B, and SP-A cDNAs after stripping off the previously hybridized probe.
Results
Immunoblotting of Surfactant Proteins
SP-B was readily detected in the lung tissue of the control patients with various pulmonary disorders but was absent in the case patient's lung tissue (Figure 1Figure 1
Protein Blotting for SP-A, SP-B, and SP-C.). SP-A was readily detected in all lung samples in roughly comparable amounts. We detected proSP-C in all the patients, but in larger amounts in the case patient. A 6-kd protein identified with the anti-proSP-C antiserum was detected only in the case patient's lung tissue. This molecular weight corresponds to the size of a dimer of SP-C, which is known to form oligomers21. Alternatively, this protein could be a partial cleavage product of the proprotein.Immunohistochemical Studies
In the control patient, abundant immunostaining for SP-B was detected in epithelial cells lining alveolar spaces and lying within alveoli (Figure 2AFigure 2
Immunohistochemical Analysis for SP-B.). In both siblings with congenital alveolar proteinosis immunostaining for SP-B was absent in alveolar epithelial cells and in the intraalveolar proteinaceous material (Figure 2B and Figure 2C). However, the proteinaceous material stained intensely for both SP-C (Figure 3BFigure 3
Immunohistochemical Analysis for SP-C. and Figure 3C) and SP-A (data not shown). In both siblings extensive, intense staining for SP-C was present in the alveolar epithelium, whereas staining was focal and faint in the control patient (Figure 3A). Epithelial-cell SP-A staining was sparse in both siblings and abundant in the control patient (data not shown). The distribution and intensity of immunostaining for SP-A and SP-B in the control patient, including staining in alveolar macrophages and Clara cells, were similar to those observed in other infants12. No staining was observed with nonimmune serum.RNA Analysis
To investigate the mechanisms underlying the absence of immunoreactive SP-B protein in the case patient's lung tissue, RNA blotting was performed (Figure 4Figure 4
RNA Blotting for SP-A, SP-B, and SP-C.). SP-A and SP-C mRNAs were found in all lung tissues examined. SP-B mRNA was present in all lung samples except that of the case patient, in which no signal was detected even after prolonged exposure. No signal was detected at higher or lower molecular weights to suggest an alternatively processed or degraded message. This result suggests that the lack of SP-B protein in the case patient's lung was due to a pretranslational mechanism -- either a decrease in transcription of the SP-B gene or the production of an unstable SP-B mRNA.Discussion
Pulmonary surfactant is a complex mixture of lipids and proteins that reduces surface tension at the interface between air and liquid. A lack of pulmonary surfactant is recognized as the principal cause of the respiratory distress syndrome in premature infants22. Specific proteins have been identified that appear to be essential for surfactant function21,23,24. These include SP-A, a glycoprotein that is likely to be important in surfactant metabolism and that may also play a part in host defense, and SP-B and SP-C, which are hydrophobic proteins of low molecular weight that increase the rate at which surfactant phospholipids are adsorbed to the air-liquid interface25,26.
Given their importance in the function and metabolism of surfactant, inherited deficiencies of specific surfactant proteins could potentially cause respiratory disease in newborn infants. The two siblings described in this report had the characteristic clinical and histopathologic features of congenital pulmonary alveolar proteinosis and a specific deficiency of SP-B. These observations, and previous studies of SP-B function,25-29 support the hypothesis that an inherited deficiency of SP-B was the cause of the respiratory disease in these infants.
Multiple lines of evidence demonstrate the importance of SP-B in surfactant functions. The expression of SP-B is lung-specific and is developmentally regulated30-32. Combinations of surfactant phospholipids and synthetic SP-B peptides exhibited biophysical properties similar to those of native surfactant27. Respiratory failure developed in rabbits treated intratracheally with a monoclonal antibody to SP-B, but not to SP-A33,34. Synthetic SP-B peptides stimulated the uptake of phosphatidylcholine into alveolar type II cells in primary culture, suggesting that SP-B may be important in surfactant metabolism28. The exact mechanisms by which SP-B deficiency could lead to the histopathological appearance of alveolar proteinosis are unclear, but our observations indicate an essential role for SP-B in the function and metabolism of surfactant.
The absence of SP-B mRNA in the case patient's lungs accounts for the lack of SP-B protein, and it is unlikely to be due to therapy or immaturity of the lungs. The biopsy specimen was obtained at an age when SP-B mRNA should be abundant,31 and the deficiency was specific for SP-B, without corresponding decreases in SP-A and SP-C. Glucocorticoid treatment30,31,35 and exposure to hyperoxia15,36-38 have been associated with increased expression of SP-B. Finally, the immunohistochemical findings in his sibling's lung tissue indicate that she also had SP-B deficiency, which suggests a genetic mechanism.
The respective roles of SP-B and SP-C in surfactant function are unclear. In the infants with SP-B deficiency described in this report, respiratory failure developed despite increased amounts of SP-C, suggesting that SP-B is essential for normal respiratory function. The mechanisms underlying the increased amounts and cellular distribution of SP-C observed in these infants are unknown. The similar patterns of immunostaining in both siblings, and minor variations in SP-C content among the control patients, some of whom were also exposed to oxygen or corticosteroids (or both), suggest that these changes were not secondary to another disease process or therapy.
It is unknown whether infants with congenital alveolar proteinosis from other families are deficient in SP-B. SP-B is necessary to the formation of tubular myelin39,40. An absence of tubular myelin was noted on ultrastructural examination of the alveolar material in several previously reported cases,3,4 a finding consistent with the hypothesis that those infants were deficient in SP-B. However, SP-B has been purified from, and tubular myelin has been observed in, the lungs of adults with alveolar proteinosis,11,41 indicating that the histopathological appearance of alveolar proteinosis can result from conditions other than SP-B deficiency. Whether other infants with congenital alveolar proteinosis have abnormalities of SP-B quantity or function, or possibly deficiencies of other surfactant proteins, remains to be determined.
Finally, the prognosis for infants with congenital alveolar proteinosis has been uniformly poor, even with the use of extracorporeal membrane oxygenation. Therapeutic whole-lung lavage has also been considered4. If SP-B deficiency is the basis for congenital alveolar proteinosis in other infants, then replacement therapy (the administration of aerosolized protein, gene therapy, or lung transplantation) may be needed to treat this disorder.
Addendum
Another apparently affected sibling has recently been born to the case family. Severe lung disease has developed in the newborn period; protein-blot analysis of amniotic and lung-lavage fluid showed no detectable SP-B and markedly increased amounts of SP-C.
Supported in part by grants to Dr. Colten (HL-37591) and Dr. deMello (HL-34748) from the National Institutes of Health, and by a grant from the American Lung Association to Dr. deMello.
We are indebted to Dr. Jeffrey A. Whitsett for providing antibodies and cDNA probes to SP-A, SP-B, and SP-C; to Dr. David Phelps for providing antibodies to SP-A and SP-B; to Sarah Heyman for technical assistance in the immunohistochemical studies; to Drs. Thomas Spray, Charles Huddleston, and Michael Crossman and Roberta Mackay, R.N., for assistance in obtaining tissue specimens; and to Drs. Anne Murphy and Arnold Strauss for helpful suggestions.
Source Information
From the Department of Pediatrics, Division of Allergy and Pulmonary Medicine (L.M.N., H.R.C.), and the Department of Pathology (L.P.D.), Washington University School of Medicine; and the Department of Pathology, Cardinal Glennon Children's Hospital (D.E.D.) -- both in St. Louis.
Address reprint requests to Dr. Nogee at the Division of Neonatology, CMSC 210, Johns Hopkins Children's Center, 600 N. Wolfe St., Baltimore, MD 21287-3200.
- References
References
1
Coleman M ,Dehner LP ,Sibley RK ,Burke BA ,L'Heureux PR ,Thompson TR . Pulmonary alveolar proteinosis: an uncommon cause of chronic neonatal respiratory distress. Am Rev Respir Dis 1980;121:583-586
Web of Science | Medline2
Knight DP ,Knight JA . Pulmonary alveolar proteinosis in the newborn. Arch Pathol Lab Med 1985;109:529-531
Web of Science | Medline3
Schumacher RE ,Marrogi AJ ,Heidelberger KP . Pulmonary alveolar proteinosis in a newborn. Pediatr Pulmonol 1989;7:178-182
CrossRef | Web of Science | Medline4
Moulton SL ,Krous HF ,Merritt TA ,Odell RM ,Gangitano E ,Cornish JD . Congenital pulmonary alveolar proteinosis: failure of treatment with extracorporeal life support. J Pediatr 1992;120:297-302
CrossRef | Web of Science | Medline5
Rosen SH ,Castleman B ,Liebow AA . Pulmonary alveolar proteinosis. N Engl J Med 1958;258:1123-1142
Full Text | Web of Science | Medline6
Whitsett JA ,Hull WM ,Ohning B ,Ross G ,Weaver TE . Immunologic identification of a pulmonary surfactant-associated protein of molecular weight = 6000 daltons. Pediatr Res 1986;20:744-749
CrossRef | Web of Science | Medline7
Weaver TE ,Sarin VK ,Sawtell N ,Hull WM ,Whitsett JA . Identification of surfactant proteolipid SP-B in human surfactant and fetal lung. J Appl Physiol 1988;65:982-987
Web of Science | Medline8
Bolling TJ ,Mandecki W . An Escherichia coli expression vector for high-level production of heterologous proteins in fusion with CMP-KDO synthetase. Biotechniques 1990;8:488-492
CrossRef | Web of Science | Medline9
Vorbroker DK ,Dey C ,Weaver TE ,Whitsett JA . Surfactant protein C precursor is palmitoylated and associates with subcellular membranes. Biochim Biophys Acta 1992;1105:161-169
CrossRef | Web of Science | Medline10
Phelps DS ,Floros J ,Taeusch HW Jr . Post-translational modification of the major human surfactant-associated proteins. Biochem J 1986;237:373-377
Web of Science | Medline11
Phelps DS ,Floros J . Localization of pulmonary surfactant proteins using immunohistochemistry and tissue in situ hybridization. Exp Lung Res 1991;17:985-995
CrossRef | Web of Science | Medline12
deMello DE ,Phelps DS ,Patel G ,Floros J ,Lagunoff D . Expression of the 35kDa and low molecular weight surfactant-associated proteins in the lungs of infants dying with respiratory distress syndrome. Am J Pathol 1989;134:1285-1293
Web of Science | Medline13
Lees MB ,Paxman S . Modification of the Lowry procedure for the analysis of proteolipid protein. Anal Biochem 1972;47:184-192
CrossRef | Web of Science | Medline14
Schagger H ,von Jagow G . Tricine-sodium dodecyl sulfate-polyacrylamide gel electrophoresis for the separation of proteins in the range from 1 to 100 kDa. Anal Biochem 1987;166:368-379
CrossRef | Web of Science | Medline15
Nogee LM ,Wispe JR ,Clark JC ,Weaver TE ,Whitsett JA . Increased expression of pulmonary surfactant proteins in oxygen-exposed rats. Am J Respir Cell Mol Biol 1991;4:102-107
Web of Science | Medline16
Chomczynski P ,Sacchi N . Single-step method of RNA isolation by acid guanidinium thiocyanate-phenol-chloroform extraction. Anal Biochem 1987;162:156-159
CrossRef | Web of Science | Medline17
Sambrook J, Fritsch EF, Maniatis T. Molecular cloning: a laboratory manual. Cold Spring Harbor, N.Y.: Cold Spring Harbor Laboratory Press, 1989:7.43-7.50.
18
Korfhagen TR ,Glasser SW ,Bruno MD ,McMahan MJ ,Whitsett JA . A portion of the human surfactant protein A (SP-A) gene locus consists of a pseudogene. Am J Respir Cell Mol Biol 1991;4:463-469
Web of Science | Medline19
Glasser SW ,Korfhagen TR ,Weaver T ,Pilot-Matias T ,Fox JL ,Whitsett JA . cDNA and deduced amino acid sequence of human pulmonary surfactant-associated proteolipid SPL(Phe). Proc Natl Acad Sci U S A 1987;84:4007-4011
CrossRef | Web of Science | Medline20
Glasser SW ,Korfhagen TR ,Weaver TE , et al. cDNA, deduced polypeptide structure and chromosomal assignment of human pulmonary surfactant proteolipid, SPL (pVAL). J Biol Chem 1988;263:9-12
Web of Science | Medline21
Weaver TE ,Whitsett JA . Structure and function of pulmonary surfactant proteins. Semin Perinatol 1988;12:213-220
Web of Science | Medline22
Farrell PM ,Avery ME . Hyaline membrane disease. Am Rev Respir Dis 1975;111:657-688
Web of Science | Medline23
Hawgood S ,Clements JA . Pulmonary surfactant and its apoproteins. J Clin Invest 1990;86:1-6
CrossRef | Web of Science | Medline24
Weaver TE ,Whitsett JA . Function and regulation of expression of pulmonary surfactant-associated proteins. Biochem J 1991;273:249-264
Web of Science | Medline25
Whitsett JA ,Ohning BL ,Ross G , et al. Hydrophobic surfactant-associated protein in whole lung surfactant and its importance for biophysical activity in lung surfactant extracts used for replacement therapy. Pediatr Res 1986;20:460-467
CrossRef | Web of Science | Medline26
Hawgood S ,Benson BJ ,Schilling J ,Damm D ,Clements JA ,White RT . Nucleotide and amino acid sequences of pulmonary surfactant protein SP 18 and evidence for cooperation between SP 18 and SP 28-36 in surfactant lipid adsorption. Proc Natl Acad Sci U S A 1987;84:66-70
CrossRef | Web of Science | Medline27
Sarin VK ,Gupta S ,Leung TK , et al. Biophysical and biological activity of a synthetic 8.7-kDA hydrophobic pulmonary surfactant protein SP-B. Proc Natl Acad Sci U S A 1990;87:2633-2637
CrossRef | Web of Science | Medline28
Rice WR ,Sarin VK ,Fox JL ,Baatz J ,Wert S ,Whitsett JA . Surfactant peptides stimulate uptake of phosphatidylcholine by isolated cells. Biochim Biophys Acta 1989;1006:237-245
Web of Science | Medline29
Yu SH ,Possmayer F . Comparative studies on the biophysical activities of the low-molecular-weight hydrophobic proteins purified from bovine pulmonary surfactant. Biochim Biophys Acta 1988;961:337-350
Web of Science | Medline30
Whitsett JA ,Weaver TE ,Clark JC , et al. Glucocortocoid enhances surfactant proteolipid Phe and pVal synthesis and RNA in fetal lung. J Biol Chem 1987;262:15618-15623
Web of Science | Medline31
Liley HG ,White RT ,Warr RG ,Benson BJ ,Hawgood S ,Ballard PL . Regulation of messenger RNAs for the hydrophobic surfactant proteins in human lung. J Clin Invest 1989;83:1191-1197
CrossRef | Web of Science | Medline32
Pryhuber GS ,Hull WM ,Fink I ,McMahan MJ ,Whitsett JA . Ontogeny of surfactant proteins A and B in human amniotic fluid as indices of fetal lung maturity. Pediatr Res 1991;30:597-605
CrossRef | Web of Science | Medline33
Kobayashi T ,Nitta K ,Takahashi R ,Kurashima K ,Robertson B ,Suzuki Y . Activity of pulmonary surfactant after blocking the associated proteins SP-A and SP-B. J Appl Physiol 1991;71:530-536
Web of Science | Medline34
Robertson B ,Kobayashi T ,Ganzuka M ,Grossmann G ,Li WZ ,Suzuki Y . Experimental neonatal respiratory failure induced by a monoclonal antibody to the hydrophobic surfactant-associated protein SP-B. Pediatr Res 1991;30:239-243
CrossRef | Web of Science | Medline35
Phelps DS ,Floros J . Dexamethasone in vivo raises surfactant protein B mRNA in alveolar and bronchiolar epithelium. Am J Physiol 1991;260:L146-L152
Web of Science | Medline36
Minoo P ,Segura L ,Coalson JJ ,King RJ ,DeLemos RA . Alterations in surfactant protein gene expression associated with premature birth and exposure to hyperoxia. Am J Physiol 1991;261:L386-L392
Web of Science | Medline37
Wikenheiser KA ,Wert SE ,Wispe JR , et al. Distinct effects of oxygen on surfactant protein B expression in bronchiolar and alveolar epithelium. Am J Physiol 1992;262:L32-L39
Web of Science | Medline38
Horowitz S ,Watkins RH ,Auten RL Jr ,Mercier CE ,Cheng ER . Differential accumulation of surfactant protein A, B, and C mRNAs in two epithelial cell types of hyperoxic lung. Am J Respir Cell Mol Biol 1991;5:511-515
Web of Science | Medline39
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
CrossRef | Web of Science | Medline40
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
Web of Science | Medline41
Hook GER ,Gilmore LB ,Talley FA . Dissolution and reassembly of tubular myelin-like multilamellated structures from the lungs of patients with pulmonary alveolar proteinosis. Lab Invest 1986;55:194-208
Web of Science | Medline
- Citing Articles (162)
Citing Articles
1
Megan K. Dishop. (2011) Paediatric Interstitial Lung Disease: Classification and Definitions. Paediatric Respiratory Reviews 12:4, 230-237
CrossRef2
Megan Stuebner Devine, Christine Kim Garcia. (2011) Genetic Interstitial Lung Disease. Clinics in Chest Medicine
CrossRef3
S. Guttentag, C. D. Foster. (2011) Update in Surfactant Therapy. NeoReviews 12:11, e625-e634
CrossRef4
J D Merrill, P L Ballard, S E Courtney, D J Durand, A Hamvas, A M Hibbs, K W Lu, R M Ryan, A M Reynolds, K Spence, R H Steinhorn, W E Truog, E C Eichenwald, R A Ballard. (2011) Pilot trial of late booster doses of surfactant for ventilated premature infants. Journal of Perinatology 31:9, 599-606
CrossRef5
Alessandro Borghesi, Paolo Manzoni, Roberta Maragliano, Margherita Massa, Mauro Stronati. (2011) From the lab to the bedside: The present of research, i.e. the future of neonatology. Early Human Development 87, S23-S25
CrossRef6
Michael S D Kormann, Günther Hasenpusch, Manish K Aneja, Gabriela Nica, Andreas W Flemmer, Susanne Herber-Jonat, Marceline Huppmann, Lauren E Mays, Marta Illenyi, Andrea Schams, Matthias Griese, Iris Bittmann, Rupert Handgretinger, Dominik Hartl, Joseph Rosenecker, Carsten Rudolph. (2011) Expression of therapeutic proteins after delivery of chemically modified mRNA in mice. Nature Biotechnology 29:2, 154-157
CrossRef7
Matthias Griese, Jan Ripper, Anke Sibbersen, Pia Lohse, Peter Lohse, Frank Brasch, Andrea Schams, Asli Pamir, Bianca Schaub, Oliver J Muensterer, Carola Schön, Judith Glöckner-Pagel, Thomas Nicolai, Karl Reiter, Andreas Hector. (2011) Long-term follow-up and treatment of congenital alveolar proteinosis. BMC Pediatrics 11:1, 72
CrossRef8
Waleed Maamoun, Amber E. Fort, James J. Cummings. 2011. Neonatal Respiratory Disease. , 590-608.
CrossRef9
Bryan Corrin, Andrew G. Nicholson. 2011. Diffuse parenchymal disease of the lung. , 263-326.
CrossRef10
Bryan Corrin, Andrew G. Nicholson. 2011. Development of the lungs; perinatal and developmental lung disease. , 39-90.
CrossRef11
Van Leung-Pineda, Ann M Gronowski. (2010) Biomarker tests for fetal lung maturity. Biomarkers in Medicine 4:6, 849-857
CrossRef12
Margarita Martinez-Moczygemba, David P. Huston. (2010) Immune Dysregulation in the Pathogenesis of Pulmonary Alveolar Proteinosis. Current Allergy and Asthma Reports 10:5, 320-325
CrossRef13
Megan K. Dishop. (2010) New Entities and Diagnostic Challenges in Pediatric Lung Disease. Surgical Pathology Clinics 3:3, 495-513
CrossRef14
W. Adam Gower, Susan E. Wert, Jennifer S. Ginsberg, Agneta Golan, Jeffrey A. Whitsett, Lawrence M. Nogee. (2010) Fatal Familial Lung Disease Caused by ABCA3 Deficiency without Identified ABCA3 Mutations. The Journal of Pediatrics 157:1, 62-68
CrossRef15
Megan K. Dishop. (2010) Diagnostic Pathology of Diffuse Lung Disease in Children. Pediatric Allergy, Immunology, and Pulmonology 23:1, 69-85
CrossRef16
Lawrence M. Nogee. (2010) Genetic Basis of Children's Interstitial Lung Disease. Pediatric Allergy, Immunology, and Pulmonology 23:1, 15-24
CrossRef17
Stephan W. Glasser, William D. Hardie, James S. Hagood. (2010) Pathogenesis of Interstitial Lung Disease in Children and Adults. Pediatric Allergy, Immunology, and Pulmonology 23:1, 9-14
CrossRef18
Robert H. Pfister, Roger Soll, Thomas E Wiswell. (2010) Cochrane review: Protein containing synthetic surfactant versus animal derived surfactant extract for the prevention and treatment of respiratory distress syndrome. Evidence-Based Child Health: A Cochrane Review Journal 5:1, 17-51
CrossRef19
B S Poterjoy, Y Vibert, M Sola-Visner, J McGowan, G Visner, L M Nogee. (2010) Neonatal respiratory failure due to a novel mutation in the surfactant protein C gene. Journal of Perinatology 30:2, 151-153
CrossRef20
Joanna R. Long, Frank D. Mills, Omjoy K. Ganesh, Vijay C. Antharam, R. Suzanne Farver. (2010) Partitioning, dynamics, and orientation of lung surfactant peptide KL4 in phospholipid bilayers. Biochimica et Biophysica Acta (BBA) - Biomembranes 1798:2, 216-222
CrossRef21
Lawrence M. Nogee. (2010) Genetic Basis of Children’s Interstitial Lung Disease. Pediatric Allergy, Immunology, and Pulmonology110525163459060
CrossRef22
Toshimichi Nakamura, Ryohei Fujiwara, Naoki Ishiguro, Masanobu Oyabu, Takeo Nakanishi, Yoshiyuki Shirasaka, Tomoji Maeda, Ikumi Tamai. (2010) Involvement of Choline Transporter-Like Proteins, CTL1 and CTL2, in Glucocorticoid-Induced Acceleration of Phosphatidylcholine Synthesis via Increased Choline Uptake. Biological & Pharmaceutical Bulletin 33:4, 691-696
CrossRef23
Min Kwang Byun, Dong Soon Kim, Young Whan Kim, Man Pyo Chung, Jae Jeong Shim, Seung Ick Cha, Soo-Taek Uh, Choon Sik Park, Sung Hwan Jeong, Yong Bum Park, Hong Lyeol Lee, Moo Suk Park. (2010) Clinical Features and Outcomes of Idiopathic Pulmonary Alveolar Proteinosis in Korean Population. Journal of Korean Medical Science 25:3, 393
CrossRef24
Claire Langston, Megan K. Dishop. (2009) Diffuse Lung Disease in Infancy: A Proposed Classification Applied to 259 Diagnostic Biopsies. Pediatric and Developmental Pathology 12:6, 421-437
CrossRef25
Susan E. Wert, Jeffrey A. Whitsett, Lawrence M. Nogee. (2009) Genetic Disorders of Surfactant Dysfunction. Pediatric and Developmental Pathology 12:4, 253-274
CrossRef26
Paul O. Nkadi, T. Allen Merritt, De-Ann M. Pillers. (2009) An overview of pulmonary surfactant in the neonate: Genetics, metabolism, and the role of surfactant in health and disease. Molecular Genetics and Metabolism 97:2, 95-101
CrossRef27
Vijay C. Antharam, Douglas W. Elliott, Frank D. Mills, R. Suzanne Farver, Edward Sternin, Joanna R. Long. (2009) Penetration Depth of Surfactant Peptide KL4 into Membranes Is Determined by Fatty Acid Saturation. Biophysical Journal 96:10, 4085-4098
CrossRef28
V P Carnielli, L J I Zimmermann, A Hamvas, P E Cogo. (2009) Pulmonary surfactant kinetics of the newborn infant: novel insights from studies with stable isotopes. Journal of Perinatology 29, S29-S37
CrossRef29
Karen Maresso, Ulrich Broeckel. (2009) The Role of Genomics in the Neonatal ICU. Clinics in Perinatology 36:1, 189-204
CrossRef30
Raul C. Ribeiro, Carlos Rodriguez-Galindo, Guillermo Chantada. 2009. Pulmonary Manifestations of Hematologic and Oncologic Diseases. , 135-169.
CrossRef31
T. Suzuki, T. Sakagami, B. K. Rubin, L. M. Nogee, R. E. Wood, S. L. Zimmerman, T. Smolarek, M. K. Dishop, S. E. Wert, J. A. Whitsett, G. Grabowski, B. C. Carey, C. Stevens, J. C.M. van der Loo, B. C. Trapnell. (2008) Familial pulmonary alveolar proteinosis caused by mutations in CSF2RA. Journal of Experimental Medicine 205:12, 2703-2710
CrossRef32
M. Martinez-Moczygemba, M. L. Doan, O. Elidemir, L. L. Fan, S. W. Cheung, J. T. Lei, J. P. Moore, G. Tavana, L. R. Lewis, Y. Zhu, D. M. Muzny, R. A. Gibbs, D. P. Huston. (2008) Pulmonary alveolar proteinosis caused by deletion of the GM-CSFR gene in the X chromosome pseudoautosomal region 1. Journal of Experimental Medicine 205:12, 2711-2716
CrossRef33
Sandra Rugonyi, Samares C. Biswas, Stephen B. Hall. (2008) The biophysical function of pulmonary surfactant. Respiratory Physiology & Neurobiology 163:1-3, 244-255
CrossRef34
A. Faro, A. Hamvas. (2008) Lung Transplantation for Inherited Disorders of Surfactant Metabolism. NeoReviews 9:10, e468-e476
CrossRef35
W. A. Gower, S. E. Wert, L. M. Nogee. (2008) Inherited Surfactant Disorders. NeoReviews 9:10, e458-e467
CrossRef36
Türkan Patiroglu, Başak Akyıldız, Tahir E. Patiroglu, Inci Y. Gulmez. (2008) Recurrent pulmonary alveolar proteinosis secondary to agammaglobulinemia. Pediatric Pulmonology 43:7, 710-713
CrossRef37
Hiroto Inaba, Jesse J. Jenkins, M. Beth McCarville, R. Ray Morrison, Scott C. Howard, Ching-Hon Pui, Raul C. Ribeiro. (2008) Pulmonary alveolar proteinosis in pediatric leukemia. Pediatric Blood & Cancer 51:1, 66-70
CrossRef38
Amy D. McBee, Daniel J. Wegner, Christopher S. Carlson, Jennifer A. Wambach, Ping Yang, Hillary B. Heins, Ola D. Saugstad, Michelle A. Trusgnich, Julie Watkins-Torry, Lawrence M. Nogee, Howard Henderson, F. Sessions Cole, Aaron Hamvas. (2008) Recombination as a mechanism for sporadic mutation in the surfactant protein-C gene. Pediatric Pulmonology 43:5, 443-450
CrossRef39
Robert H. Pfister, Roger Soll, Thomas E Wiswell, Robert H. Pfister. 2007. Protein containing synthetic surfactant versus animal derived surfactant extract for the prevention and treatment of respiratory distress syndrome. .
CrossRef40
Elisabeth Bruder, Jörg Hofmeister, Charalampos Aslanidis, Jürg Hammer, Lukas Bubendorf, Gerd Schmitz, Alex Rufle, Christoph Bührer. (2007) Ultrastructural and molecular analysis in fatal neonatal interstitial pneumonia caused by a novel ABCA3 mutation. Modern Pathology 20:10, 1009-1018
CrossRef41
Rolf Lindemann, Thomas Rajka, Thore Henrichsen, Ole Georg Vinorum, Charlotte de Lange, Aage Erichsen, Drude Fugelseth. (2007) Bronchioalveolar lavage with perfluorochemical liquid during conventional ventilation. Pediatric Critical Care Medicine 8:5, 486-488
CrossRef42
N Mimura, H Hamada, M Kashio, H Jin, Y Toyama, K Kimura, M Iida, S Goto, H Saisho, K Toshimori, H Koseki, T Aoe. (2007) Aberrant quality control in the endoplasmic reticulum impairs the biosynthesis of pulmonary surfactant in mice expressing mutant BiP. Cell Death and Differentiation 14:8, 1475-1485
CrossRef43
Jasper V. Been, Luc J. I. Zimmermann. (2007) What’s new in surfactant?. European Journal of Pediatrics 166:9, 889-899
CrossRef44
RH Pfister, RF Soll, T Wiswell, Robert Pfister. 2007. Protein containing synthetic surfactant versus animal derived surfactant extract for the prevention and treatment of respiratory distress syndrome. .
CrossRef45
Marco Somaschini, Lawrence M. Nogee, Isabella Sassi, Olivier Danhaive, Silvia Presi, Renata Boldrini, Cristina Montrasio, Maurizio Ferrari, Susan E. Wert, Paola Carrera. (2007) Unexplained Neonatal Respiratory Distress Due to Congenital Surfactant Deficiency. The Journal of Pediatrics 150:6, 649-653.e1
CrossRef46
Nilanjana Biswas, Alan J. Waring, Frans J. Walther, Richard A. Dluhy. (2007) Structure and conformation of the disulfide bond in dimeric lung surfactant peptides SP-B1–25 and SP-B8–25. Biochimica et Biophysica Acta (BBA) - Biomembranes 1768:5, 1070-1082
CrossRef47
Daniel J Wegner, Torbjörn Hertzberg, Hillary B Heins, Göran Elmberger, Michael J MacCoss, Christopher S Carlson, Lawrence M Nogee, F Sessions Cole, Aaron Hamvas. (2007) A major deletion in the surfactant protein-B gene causing lethal respiratory distress. Acta Paediatrica 96:4, 516-520
CrossRef48
D. Wang, D. L. Haviland, A. R. Burns, E. Zsigmond, R. A. Wetsel. (2007) A pure population of lung alveolar epithelial type II cells derived from human embryonic stem cells. Proceedings of the National Academy of Sciences 104:11, 4449-4454
CrossRef49
Ola D. Saugstad, Thor Willy Ruud Hansen, Arild Rønnestad, Britt Nakstad, Per Arne Tølløfsrud, Finn Reinholt, Aaron Hamvas, F. Sessions Cole, Michael Dean, Susan E. Wert, Jeffrey A. Whitsett, Lawrence M. Nogee. (2007) Novel mutations in the gene encoding ATP binding cassette protein member A3 (ABCA3) resulting in fatal neonatal lung disease. Acta Paediatrica 96:2, 185-190
CrossRef50
Andreas Almlén, Guido Stichtenoth, Bengt Robertson, Jan Johansson, Tore Curstedt. (2007) Concentration Dependence of a Poly-Leucine Surfactant Protein C Analogue on in vitro and in vivo Surfactant Activity. Neonatology 92:3, 194-200
CrossRef51
Harutaka Yamada, Naoto Miura, Wataru Kitagawa, Yukari Kashima, Seiko Matsui, Norio Ozeki, Kazuhiro Nishikawa, Hirokazu Imai. (2007) Membranous Nephropathy and Pulmonary Alveolar Proteinosis. Internal Medicine 46:17, 1441-1446
CrossRef52
Janine E. Bullard, Susan E. Wert, Lawrence M. Nogee. (2006) ABCA3 Deficiency: Neonatal Respiratory Failure and Interstitial Lung Disease. Seminars in Perinatology 30:6, 327-334
CrossRef53
Aaron Hamvas. (2006) Inherited Surfactant Protein-B Deficiency and Surfactant Protein-C Associated Disease: Clinical Features and Evaluation. Seminars in Perinatology 30:6, 316-326
CrossRef54
Robert H. Pfister, Roger Soll, Thomas E Wiswell, Robert H. Pfister. 2006. Protein-containing synthetic surfactant versus protein-free synthetic surfactant for the prevention and treatment of respiratory distress syndrome. .
CrossRef55
Paul Kinniry, Jeremy Pick, Scott Stephens, Deepika Jain, Charalambos C. Solomides, Ralph Niven, Robert Segal, Melpo Christofidou-Solomidou. (2006) KL4-surfactant prevents hyperoxic and LPS-induced lung injury in mice. Pediatric Pulmonology 41:10, 916-928
CrossRef56
JULIO PEREZ FONTAN, S BRUCE DOWTON, DAVID PERLMUTTER, JONATHAN GITLIN, F SESSIONS COLE. (2006) Plasma Proteins in Health and Disease: A Scientific Symposium to Celebrate the Career and Accomplishments of Harvey R. Colten, M.D.. Pediatric Research 60:2, 231-232
CrossRef57
Natalie J. Miller, Anthony D. Postle, Sandra Orgeig, Grielof Koster, Christopher B. Daniels. (2006) The composition of pulmonary surfactant from diving mammals. Respiratory Physiology & Neurobiology 152:2, 152-168
CrossRef58
Lawrence M. Nogee. (2006) Genetics of pediatric interstitial lung disease. Current Opinion in Pediatrics 18:3, 287-292
CrossRef59
April Price, David Manson, Ernest Cutz, Sharon Dell. (2006) Pulmonary alveolar proteinosis associated with anti-GM-CSF antibodies in a child: Successful treatment with inhaled GM-CSF. Pediatric Pulmonology 41:4, 367-370
CrossRef60
F. Brasch. (2006) Interstitielle Lungenerkrankungen. Der Pathologe 27:2, 116-132
CrossRef61
David G. Grenache, Ann M. Gronowski. (2006) Fetal lung maturity. Clinical Biochemistry 39:1, 1-10
CrossRef62
RAFAEL CARTAGENA, ANTHONY N. PASSANNANTE, PETER ROCK. 2006. Respiratory Diseases. , 127-149.
CrossRef63
Mohammed Tredano, David N. Cooper, Manfred Stuhrmann, John Christodoulou, Nadia A. Chuzhanova, Françoise Roudot-Thoraval, Pierre-Yves Boëlle, Jacques Elion, Marc Jeanpierre, Josué Feingold, Rémy Couderc, Michel Bahuau. (2006) Origin of the prevalent SFTPB indel g.1549C > GAA (121ins2) mutation causing surfactant protein B (SP-B) deficiency. American Journal of Medical Genetics Part A 140A:1, 62-69
CrossRef64
A.J. Waring, F.J. Walther, L.M. Gordon, J.M. Hernandez-Juviel, T. Hong, M.A. Sherman, C. Alonso, T. Alig, A. Braun, D. Bacon, J.A. Zasadzinski. (2005) The role of charged amphipathic helices in the structure and function of surfactant protein B. The Journal of Peptide Research 66:6, 364-374
CrossRef65
C Seifart, H-M Lin, U Seifart, A Plagens, S DiAngelo, P Von Wichert, J Floros. (2005) Rare SP-A alleles and the SP-A1-6A4 allele associate with risk for lung carcinoma. Clinical Genetics 68:2, 128-136
CrossRef66
Howard Clark, Lucy Side Clark. (2005) The genetics of neonatal respiratory disease. Seminars in Fetal and Neonatal Medicine 10:3, 271-282
CrossRef67
PAOLA COGO, ALDO BARITUSSIO, FEDERICA ROSSO, ANTONINA GUCCIARDI, VINCENZO MORETTI, TAMARA BADON, ELENA DUNER, LUC ZIMMERNANN, VIRGILIO P. CARNIELLI. (2005) Surfactant-Associated Protein B Kinetics In Vivo in Newborn Infants by Stable Isotopes. Pediatric Research 57:4, 519-522
CrossRef68
Michael F. Beers, Surafel Mulugeta. (2005) SURFACTANT PROTEIN C BIOSYNTHESIS AND ITS EMERGING ROLE IN CONFORMATIONAL LUNG DISEASE. Annual Review of Physiology 67:1, 663-696
CrossRef69
Claes O. Hugosson, Husam M. Salama, Fouad Al-Dayel, Nuha Khoumais, Abdul H. Kattan. (2005) Primary alveolar capillary dysplasia (acinar dysplasia) and surfactant protein B deficiency: a clinical, radiological and pathological study. Pediatric Radiology 35:3, 311-316
CrossRef70
Alan S. Brody. (2005) Imaging Considerations: Interstitial Lung Disease in Children. Radiologic Clinics of North America 43:2, 391-403
CrossRef71
Steven M Donn. (2005) Lucinactant: a novel synthetic surfactant for the treatment of respiratory distress syndrome. Expert Opinion on Investigational Drugs 14:3, 329-334
CrossRef72
L.J.I. Zimmermann, D.J.M.T. Janssen, D. Tibboel, A. Hamvas, V.P. Carnielli. (2005) Surfactant Metabolism in the Neonate. Biology of the Neonate 87:4, 296-307
CrossRef73
Jacques de Blic. (2004) Pulmonary alveolar proteinosis in children. Paediatric Respiratory Reviews 5:4, 316-322
CrossRef74
JEFFREY D. MERRILL, ROBERTA A. BALLARD, AVITAL CNAAN, ANNA MARIA HIBBS, RODOLFO I. GODINEZ, MARYE H. GODINEZ, WILLIAM E. TRUOG, PHILIP L. BALLARD. (2004) Dysfunction of Pulmonary Surfactant in Chronically Ventilated Premature Infants. Pediatric Research 56:6, 918-926
CrossRef75
Leland L. Fan, Robin R. Deterding, Claire Langston. (2004) Pediatric interstitial lung disease revisited. Pediatric Pulmonology 38:5, 369-378
CrossRef76
Ichiro Yoshida, Nobuhiro Ban, Nobuya Inagaki. (2004) Expression of ABCA3, a causative gene for fatal surfactant deficiency, is up-regulated by glucocorticoids in lung alveolar type II cells. Biochemical and Biophysical Research Communications 323:2, 547-555
CrossRef77
Christine Kim Garcia, Ganesh Raghu. (2004) Inherited interstitial lung disease. Clinics in Chest Medicine 25:3, 421-433
CrossRef78
R Dinwiddie. (2004) Treatment of interstitial lung disease in children. Paediatric Respiratory Reviews 5:2, 108-115
CrossRef79
Bettina C Hilman, Rodolfo Amaro-Galvez. (2004) Diagnosis of interstitial lung disease in children. Paediatric Respiratory Reviews 5:2, 101-107
CrossRef80
Brigitte Fauroux, Ralph Epaud, Annick Clément. (2004) Clinical presentation of interstitial lung disease in children. Paediatric Respiratory Reviews 5:2, 98-100
CrossRef81
Lawrence M. Nogee. (2004) Alterations in SP-B and SP-C Expression in Neonatal Lung Disease. Annual Review of Physiology 66:1, 601-623
CrossRef82
George B. Mallory. (2004) Surfactant protein B deficiency as a prototype. Pediatric Pulmonology 37:S26, 180-182
CrossRef83
David G Grier, Henry L Halliday. (2004) Effects of Glucocorticoids on Fetal and Neonatal Lung Development. Treatments in Respiratory Medicine 3:5, 295-306
CrossRef84
Saiprakash B Venkateshiah, Mary Jane Thomassen, Mani S Kavuru. (2004) Pulmonary Alveolar Proteinosis. Treatments in Respiratory Medicine 3:4, 217-227
CrossRef85
Trapnell, Bruce C., Whitsett, Jeffrey A., Nakata, Koh, . (2003) Pulmonary Alveolar Proteinosis. New England Journal of Medicine 349:26, 2527-2539
Full Text86
S SWEET. (2003) Pediatric lung transplantation: update 2003. Pediatric Clinics of North America 50:6, 1393-1417
CrossRef87
S POYNTER, A LEVINE. (2003) Surfactant biology and clinical application. Critical Care Clinics 19:3, 459-472
CrossRef88
Mohammed Tredano, Matthias Griese, Jacques de Blic, Tifenn Lorant, Claude Houdayer, Silja Schumacher, Fran
ois Cartault, Fr
CrossRef89
S. W. J. Terheggen-Lagro, J. A. Schipper, J. A. A. M. Diemen-Steenvoorde, F. B. Plötz. (2003) Respiratoire distress bij de à terme neonaat. Tijdschrift voor kindergeneeskunde 71:6, 29-34
CrossRef90
R.K. WILSON, T.J. LEY, F.S. COLE, J.D. MILBRANDT, S. CLIFTON, L. FULTON, G. FEWELL, P. MINX, H. SUN, M. MCLELLAN, C. POHL, E.R. MARDIS. (2003) Mutational Profiling in the Human Genome. Cold Spring Harbor Symposia on Quantitative Biology 68:1, 23-30
CrossRef91
Whitsett, Jeffrey A., Weaver, Timothy E., . (2002) Hydrophobic Surfactant Proteins in Lung Function and Disease. New England Journal of Medicine 347:26, 2141-2148
Full Text92
Mikko Hallman, Ritva Haataja, Riitta Marttila. (2002) Surfactant proteins and genetic predisposition to respiratory distress syndrome. Seminars in Perinatology 26:6, 450-460
CrossRef93
Cindy Wu, Annelise Barron. 2002. Biomimetic Lung Surfactant Replacements. .
CrossRef94
Timothy E Weaver, Cheng-Lun Na, Mildred Stahlman. (2002) Biogenesis of lamellar bodies, lysosome-related organelles involved in storage and secretion of pulmonary surfactant. Seminars in Cell & Developmental Biology 13:4, 263-270
CrossRef95
Aaron Hamvas, Bruce W Patterson. (2002) Pulmonary dysfunction and the integrity of the pulmonary surfactant system. Nutrition 18:7-8, 680-681
CrossRef96
M.Nurul Alam, Kiflu Berhane, Vijayakumar Boggaram. (2002) Lung surfactant protein B promoter function is dependent on the helical phasing, orientation and combinatorial actions of cis-DNA elements. Gene 282:1-2, 103-111
CrossRef97
Jerald P Kuhn, Alan S Brody. (2002) High-resolution CT of pediatric lung disease. Radiologic Clinics of North America 40:1, 89-110
CrossRef98
Robert H Notter, Z Wang, E.A Egan, B.A Holm. (2002) Component-specific surface and physiological activity in bovine-derived lung surfactants. Chemistry and Physics of Lipids 114:1, 21-34
CrossRef99
AARON HAMVAS, MICHELLE TRUSGNICH, HEATHER BRICE, JAMES BAUMGARTNER, YULING HONG, LAWRENCE M. NOGEE, AND, F. SESSIONS COLE. (2001) Population-Based Screening for Rare Mutations: High-Throughput DNA Extraction and Molecular Amplification from Guthrie Cards. Pediatric Research 50:5, 666-668
CrossRef100
Doug Willson. (2001) Calfactant. Expert Opinion on Pharmacotherapy 2:9, 1479-1493
CrossRef101
F. SESSIONS COLE, AARON HAMVAS, AND, LAWRENCE M. NOGEE. (2001) Genetic Disorders of Neonatal Respiratory Function. Pediatric Research 50:2, 157-162
CrossRef102
G.B.Mallory Jr.. (2001) Surfactant proteins: role in lung physiology and disease in early life. Paediatric Respiratory Reviews 2:2, 151-158
CrossRef103
Jacques R Bourbon, Bernadette Chailley-Heu. (2001) Surfactant proteins in the digestive tract, mesentery, and other organs: evolutionary significance. Comparative Biochemistry and Physiology - Part A: Molecular & Integrative Physiology 129:1, 151-161
CrossRef104
Mohammed Tredano, Jacques de Blic, Matthias Griese, Jean-Christophe Fournet, Jacques Elion, Michel Bahuau. (2001) Clinical Biological and Genetic Heterogeneity of the Inborn Errors of Pulmonary Surfactant Metabolism. Clinical Chemistry and Laboratory Medicine 39:2, 90-108
CrossRef105
Timothy E Weaver, Juliana Johnson Conkright. (2001) F
UNCTION OF SURFACTANT PROTEINS BAND C. Annual Review of Physiology 63:1, 555-578
CrossRef106
Samuel Hawgood, Francis R Poulain. (2001) T
HE PULMONARY COLLECTINS AND SURFACTANT METABOLISM . Annual Review of Physiology 63:1, 495-519
CrossRef107
Bettina C. Hilman. (2001) Evaluation of Interstitial Lung Disease in Children. Clinical Pulmonary Medicine 8:2, 70-77
CrossRef108
Nogee, Lawrence M., Dunbar, Alston E., Wert, Susan E., Askin, Frederic, Hamvas, Aaron, Whitsett, Jeffrey A., . (2001) A Mutation in the Surfactant Protein C Gene Associated with Familial Interstitial Lung Disease. New England Journal of Medicine 344:8, 573-579
Full Text109
Marie Palmblad, Magnus Gustafsson, Tore Curstedt, Jan Johansson, Samuel Schürch. (2001) Surface activity and film formation from the surface associated material of artificial surfactant preparations. Biochimica et Biophysica Acta (BBA) - Biomembranes 1510:1-2, 106-117
CrossRef110
Cabot, Richard C.Scully, Robert E., Mark, Eugene J., McNeely, William F., Shepard, Jo-Anne O., Ebeling, Sally H.Ellender, Stacey M.Peters, Christine C., Sherter, Carl B.Mark, Eugene J.. (2001) Case 2-2001. New England Journal of Medicine 344:3, 212-220
Full Text111
Claire Langston, Leland L. Fan. (2001) Diffuse interstitial lung disease in infants. Pediatric Pulmonology 32:S23, 74-76
CrossRef112
Mark W. Thompson. (2001) Surfactant Protein B Deficiency:. The American Journal of the Medical Sciences 321:1, 26-32
CrossRef113
Claire Langston, Leland L. Fan. (2001) Diffuse interstitial lung disease in infants. Pediatric Pulmonology 26:S23, 74-76
CrossRef114
A. G. Kaspers, R. M. vanElburg, W. P. F. Fetter. (2000) Een voldragen pasgeborene met surfactansproteïne B-deficiëntie. Tijdschrift voor kindergeneeskunde 68:5, 71-74
CrossRef115
Frans J. Walther, Larry M. Gordon, Joseph A. Zasadzinski, Mark A. Sherman, Alan J. Waring. (2000) Surfactant Protein B and C Analogues. Molecular Genetics and Metabolism 71:1-2, 342-351
CrossRef116
Deborah Silverstein, Craig Greene, Christopher Gregory, Stephen Lucas, Jane Quandt. (2000) Pulmonary Alveolar Proteinosis in a Dog. Journal of Veterinary Internal Medicine 14:5, 546-551
CrossRef117
Alston E Dunbar, Susan E Wert, Machiko Ikegami, Jeffrey A Whitsett, Aaron Hamvas, Frances V White, Bruno Piedboeuf, Catherine Jobin, Susan Guttentag, Lawrence M Nogee. (2000) Prolonged Survival in Hereditary Surfactant Protein B (SP-B) Deficiency Associated with a Novel Splicing Mutation. Pediatric Research 48:3, 275-282
CrossRef118
David S. Strayer, Laxminarayana Korutla. (2000) Activation of surfactant protein-B transcription: Signaling through the SP-A receptor utilizing the PI3 kinase pathway. Journal of Cellular Physiology 184:2, 229-238
CrossRef119
SB Ainsworth, MW Beresford, DWA Milligan, NJ Shaw, JNS Matthews, AC Fenton, MP Ward Platt. (2000) Pumactant and poractant alfa for treatment of respiratory distress syndrome in neonates born at 25–29 weeks' gestation: a randomised trial. The Lancet 355:9213, 1387-1392
CrossRef120
L.M. Gordon, K. Y. C. Lee, M.M. Lipp, J.A. Zasadzinski, F.J. Walther, M.A. Sherman, A.J. Waring. (2000) Conformational mapping of the N-terminal segment of surfactant protein B in lipid using 13C-enhanced Fourier transform infrared spectroscopy. Journal of Peptide Research 55:4, 330-347
CrossRef121
Mildred T Stahlman, Mary Phillips Gray, Mary W Falconieri, Jeffrey A Whitsett, Timothy E Weaver. (2000) Lamellar Body Formation in Normal and Surfactant Protein B-Deficient Fetal Mice. Laboratory Investigation 80:3, 395-403
CrossRef122
Carole R. Mendelson. (2000) Role of Transcription Factors in Fetal Lung Development and Surfactant Protein Gene Expression. Annual Review of Physiology 62:1, 875-915
CrossRef123
DAPHNE E. deMELLO, SOHIR MAHMOUD, PHILIP J. PADFIELD, JOSEPH W. HOFFMANN. (2000) GENERATION OF AN IMMORTAL DIFFERENTIATED LUNG TYPE-II EPITHELIAL CELL LINE FROM THE ADULT H-2KbtsA58 TRANSGENIC MOUSE. In Vitro Cellular & Developmental Biology - Animal 36:6, 374
CrossRef124
KEISUKE TOKIEDA, MACHIKO IKEGAMI, SUSAN E. WERT, JOHN E. BAATZ, YONG ZOU, JEFFREY A. WHITSETT. (1999) Surfactant Protein B Corrects Oxygen-Induced Pulmonary Dysfunction in Heterozygous Surfactant Protein B???Deficient Mice. Pediatric Research 46:6, 708
CrossRef125
DIANN M. McCOY, RONALD G. SALOME, DAVID J. KUSNER, SHANKAR S. IYAR, RAMA K. MALLAMPALLI. (1999) Identification of Sex-Specific Differences in Surfactant Synthesis in Rat Lung. Pediatric Research 46:6, 722
CrossRef126
Charles B. Huddleston, Stuart C. Sweet, George B. Mallory, Aaron Hamvas, Eric N. Mendeloff. (1999) Lung transplantation in very young infants. The Journal of Thoracic and Cardiovascular Surgery 118:5, 796-804
CrossRef127
Ian R Doyle, Terence E Nicholas, Andrew D Bersten. (1999) PARTITIONING LUNG AND PLASMA PROTEINS: CIRCULATING SURFACTANT PROTEINS AS BIOMARKERS OF ALVEOLOCAPILLARY PERMEABILITY. Clinical and Experimental Pharmacology and Physiology 26:3, 185-197
CrossRef128
L NOGEE. (1998) Genetics of the hydrophobic surfactant proteins. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1408:2-3, 323-333
CrossRef129
B ROBERTSON, H HALLIDAY. (1998) Principles of surfactant replacement. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1408:2-3, 346-361
CrossRef130
Wan Chong Tsai, Dorothy Lewis, Samya Z. Nasr, Ronald B. Hirschl. (1998) Liquid ventilation in an infant with pulmonary alveolar proteinosis. Pediatric Pulmonology 26:4, 283-286
CrossRef131
Joanna Floros, Padma Kala. (1998) SURFACTANT PROTEINS: Molecular Genetics of Neonatal Pulmonary Diseases. Annual Review of Physiology 60:1, 365-384
CrossRef132
A G Nicholson, H Kim, B Corrin, A Bush, R M du Bois, M Rosenthal, M N Sheppard. (1998) The value of classifying interstitial pneumonitis in childhood according to defined histological patterns. Histopathology 33:3, 203-211
CrossRef133
Gloria S. Pryhuber. (1998) Regulation and Function of Pulmonary Surfactant Protein B. Molecular Genetics and Metabolism 64:4, 217-228
CrossRef134
Rupert Chang, Shlomo Nir, Francis R Poulain. (1998) Analysis of binding and membrane destabilization of phospholipid membranes by surfactant apoprotein B. Biochimica et Biophysica Acta (BBA) - Biomembranes 1371:2, 254-264
CrossRef135
FRANS J. WALTHER, JOS?? HERN??NDEZ-JUVIEL, ROBERTA BRUNI, ALAN J. WARING. (1998) Protein Composition of Synthetic Surfactant Affects Gas Exchange in Surfactant-Deficient Rats. Pediatric Research 43:5, 666-673
CrossRef136
PADMA KALA, THOMAS TEN HAVE, HEBER NIELSEN, MICHAEL DUNN, JOANNA FLOROS. (1998) Association of Pulmonary Surfactant Protein A (SP-A) Gene and Respiratory Distress Syndrome: Interaction with SP-B. Pediatric Research 43:2, 169-177
CrossRef137
Robert H. Notter,, Zhengdong Wang,. (1997) PULMONARY SURFACTANT: PHYSICAL CHEMISTRY, PHYSIOLOGY, AND REPLACEMENT. Reviews in Chemical Engineering 13:4, 1-118
CrossRef138
John A. Clements. (1997) LUNG SURFACTANT: A Personal Perspective. Annual Review of Physiology 59:1, 1-21
CrossRef139
KOLA O. SOLARIN, PHILIP L. BALLARD, SUSAN H. GUTTENTAG, CATHERINE A. LOMAX, MICHAEL F. BEERS. (1997) Expression and Glucocorticoid Regulation of Surfactant Protein C in Human Fetal Lung. Pediatric Research 42:3, 356-364
CrossRef140
Emile M. Scarpelli, Alan J. Mautone, Mala R. Chinoy, David O. Defouw, Bella C. Clutario. (1997) Intraalveolar bubbles and bubble films: III. Vulnerability and preservation in the laboratory. The Anatomical Record 248:4, 498-520
CrossRef141
Zsuzsanna K. Zsengellér, Susan E. Wert, Cindy J. Bachurski, Kevin L. Kirwin, Bruce C. Trapnell, Jeffrey A. Whitsett. (1997) Recombinant Adenoviral Vector Disrupts Surfactant Homeostasis in Mouse Lung. Human Gene Therapy 8:11, 1331-1344
CrossRef142
E Sleight, RC Coombs, AT Gibson, RA Primhak. (1997) Neonatal respiratory distress in near-term infants—consider surfactant protein B deficiency. Acta Paediatrica 86:4, 428-430
CrossRef143
Jan Johansson, Tore Curstedt. (1997) Molecular Structures and Interactions of Pulmonary Surfactant Components. European Journal of Biochemistry 244:3, 675-693
CrossRef144
Matthew R Kaser, George G Skouteris. (1997) Inhibition of Bacterial Growth by Synthetic SP-B1-78 Peptides. Peptides 18:9, 1441-1444
CrossRef145
Lambert A.J.M. Creuwels, Lambert M.G. van Golde, Henk P. Haagsman. (1996) Surfactant protein B: effects on lipid domain formation and intermembrane lipid flow. Biochimica et Biophysica Acta (BBA) - Biomembranes 1285:1, 1-8
CrossRef146
Ulrich Pison, Ralf Herold, Samuel Schürch. (1996) The pulmonary surfactant system: biological functions, components, physicochemical properties and alterations during lung disease. Colloids and Surfaces A: Physicochemical and Engineering Aspects 114, 165-184
CrossRef147
D Liau. (1996) Effects of human polymorphonuclear leukocyte elastase upon surfactant proteins in vitro. Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism 1302:2, 117-128
CrossRef148
CHARLENE DUNCAN McWHINNEY. (1996) A Downstream AP-1 Element Regulates In Vitro Lung Transcription from the Human Pulmonary Surfactant Protein B Promoter. DNA and Cell Biology 15:7, 519-529
CrossRef149
J Johansson, T Curstedt, B Robertson. (1996) Synthetic protein analogues in artificial surfactants. Acta Paediatrica 85:6, 642-646
CrossRef150
H. Hamm, C. Kroegel, J. Hohlfeld. (1996) Surfactant: a review of its functions and relevance in adult respiratory disorders. Respiratory Medicine 90:5, 251-270
CrossRef151
SUSAN D. REVAK, T. ALLEN MERRITT, CHARLES G. COCHRANE, GREGORY P. HELDT, MONICA S. ALBERTS, DAVID W. ANDERSON, AHMED KHEITER. (1996) Efficacy of Synthetic Peptide-Containing Surfactant in the Treatment of Respiratory Distress Syndrome in Preterm Infant Rhesus Monkeys. Pediatric Research 39:4, 715-724
CrossRef152
Francis R Poulain, Shlomo Nir, Samuel Hawgood. (1996) Kinetics of phospholipid membrane fusion induced by surfactant apoproteins A and B. Biochimica et Biophysica Acta (BBA) - Biomembranes 1278:2, 169-175
CrossRef153
A. D. Postle. (1995) Lung surfactants and asthma. Clinical <html_ent glyph="@amp;" ascii="&"/> Experimental Allergy 25:11, 1030-1033
CrossRef154
Martin C. Vincent, Bruce C. Trapnell, Robert P. Baughman, Susan E. Wert, Jeffrey A. Whitsett, Harriet S. Iwamoto. (1995) Adenovirus-Mediated Gene Transfer to the Respiratory Tract of Fetal Sheep In Utero. Human Gene Therapy 6:8, 1019-1028
CrossRef155
Robert J. Korst, Burkhard Bewig, Ronald G. Crystal. (1995) In Vitro and In Vivo Transfer and Expression of Human Surfactant SP-A- and SP-B-Associated Protein cDNAs Mediated by Replication-Deficient, Recombinant Adenoviral Vectors. Human Gene Therapy 6:3, 277-287
CrossRef156
L.A.J.M. Creuwels, R.A. Demel, L.M.G. van Golde, H.P. Haagsman. (1995) Characterization of a dimeric canine form of surfactant protein C (SP-C). Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism 1254:3, 326-332
CrossRef157
Samuel Hawgood, Francis R. Poulain. (1995) Functions of the surfactant proteins: A perspective. Pediatric Pulmonology 19:2, 99-104
CrossRef158
Martin C. Vincent, Bruce C. Trapnell, Robert P. Baughman, Susan E. Wert, Jeffrey A. Whitsett, Harriet S. Iwamoto. (1995) Adenovirus-Mediated Gene Transfer to the Respiratory Tract of Fetal SheepIn Utero. Human Gene Therapy 6:8, 1019
CrossRef159
Daphne E. deMello, Lawrence M. Nogee, Sarah Heyman, Henry F. Krous, Mukarram Hussain, T.Allen Merritt, Wei Hsueh, Joel E. Haas, Kathleen Heidelberger, Robert Schumacher, Harvey R. Colten. (1994) Molecular and phenotypic variability in the congenital alveolar proteinosis syndrome associated with inherited surfactant protein B deficiency. The Journal of Pediatrics 125:1, 43-50
CrossRef160
N. Sharief, O. F. Crawford, R. Dinwiddie. (1994) Fibrosing alveolitis and desquamative interstitial pneumonitis. Pediatric Pulmonology 17:6, 359-365
CrossRef161
Cabot, Richard C.Scully, Robert E., Mark, Eugene J., McNeely, William F., McNeely, Betty U., Colin, Andrew A.Mark, Eugene J.. (1993) Case 49-1993. New England Journal of Medicine 329:24, 1797-1805
Full Text162
Leland L. Fan, Claire Langston. (1993) Chronic interstitial lung disease in children. Pediatric Pulmonology 16:3, 184-196
CrossRef
