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Surfactant Protein A Deficiency

MedGen UID:
476155
•Concept ID:
C3274522
•
Disease or Syndrome

Definition

Deficiency of surfactant protein A. When present in normal amounts, this protein protects the lungs against a variety of bacteria, viruses, and fungi. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSurfactant Protein A Deficiency

Professional guidelines

PubMed

Nitric oxide treatment and acute pulmonary inflammatory response in very premature infants with intractable respiratory failure shortly after birth.
Aikio O, Saarela T, Pokela ML, Hallman M
Acta Paediatr 2003;92(1):65-9. doi: 10.1111/j.1651-2227.2003.tb00471.x. PMID: 12650302

Recent clinical studies

Etiology

Alpha-1 antitrypsin deficiency impairs lung antibacterial immunity in mice.
Ostermann L, Maus R, Stolper J, Schütte L, Katsarou K, Tumpara S, Pich A, Mueller C, Janciauskiene S, Welte T, Maus UA
JCI Insight 2021 Feb 8;6(3) doi: 10.1172/jci.insight.140816. PMID: 33554955Free PMC Article
SP-A and SP-D: Dual Functioning Immune Molecules With Antiviral and Immunomodulatory Properties.
Watson A, Madsen J, Clark HW
Front Immunol 2020;11:622598. Epub 2021 Jan 19 doi: 10.3389/fimmu.2020.622598. PMID: 33542724Free PMC Article
Surfactant protein A deficiency exacerbates renal interstitial fibrosis following obstructive injury in mice.
Tian S, Li C, Ran R, Chen SY
Biochim Biophys Acta Mol Basis Dis 2017 Feb;1863(2):509-517. Epub 2016 Dec 1 doi: 10.1016/j.bbadis.2016.11.032. PMID: 27916681Free PMC Article
The importance of surfactant on the development of neonatal pulmonary diseases.
Lyra PP, Diniz EM
Clinics (Sao Paulo) 2007 Apr;62(2):181-90. doi: 10.1590/s1807-59322007000200014. PMID: 17505704
Pulmonary collectins and innate host defense of the lung.
LeVine AM, Whitsett JA
Microbes Infect 2001 Feb;3(2):161-6. doi: 10.1016/s1286-4579(00)01363-0. PMID: 11251302

Diagnosis

Surfactant protein expression in human skin: evidence and implications.
Mo YK, Kankavi O, Masci PP, Mellick GD, Whitehouse MW, Boyle GM, Parsons PG, Roberts MS, Cross SE
J Invest Dermatol 2007 Feb;127(2):381-6. Epub 2006 Sep 28 doi: 10.1038/sj.jid.5700561. PMID: 17008883
Pulmonary pathology.
deMello DE
Semin Neonatol 2004 Aug;9(4):311-29. doi: 10.1016/j.siny.2003.12.001. PMID: 15251148
Pulmonary surfactant protein A, B, and C mRNA and protein expression in the nitrofen-induced congenital diaphragmatic hernia rat model.
Van Tuyl M, Blommaart Pj, Keijzer R, Wert SE, Ruijter J, Lamers WH, Tibboel D
Pediatr Res 2003 Nov;54(5):641-52. Epub 2003 Aug 6 doi: 10.1203/01.PDR.0000086906.19683.42. PMID: 12904592
Surfactant protein deficiency in familial interstitial lung disease.
Amin RS, Wert SE, Baughman RP, Tomashefski JF Jr, Nogee LM, Brody AS, Hull WM, Whitsett JA
J Pediatr 2001 Jul;139(1):85-92. doi: 10.1067/mpd.2001.114545. PMID: 11445799
Deficient hydrophilic lung surfactant proteins A and D with normal surfactant phospholipid molecular species in cystic fibrosis.
Postle AD, Mander A, Reid KB, Wang JY, Wright SM, Moustaki M, Warner JO
Am J Respir Cell Mol Biol 1999 Jan;20(1):90-8. doi: 10.1165/ajrcmb.20.1.3253. PMID: 9870921

Therapy

SP-A and SP-D: Dual Functioning Immune Molecules With Antiviral and Immunomodulatory Properties.
Watson A, Madsen J, Clark HW
Front Immunol 2020;11:622598. Epub 2021 Jan 19 doi: 10.3389/fimmu.2020.622598. PMID: 33542724Free PMC Article
New Directions in Pulmonary Gene Therapy.
Vu A, McCray PB , Jr
Hum Gene Ther 2020 Sep;31(17-18):921-939. doi: 10.1089/hum.2020.166. PMID: 32814451Free PMC Article
Surfactant-associated proteins: structure, function and clinical implications.
Ketko AK, Donn SM
Curr Pediatr Rev 2014;10(2):162-7. doi: 10.2174/157339631130900006. PMID: 25088270
Surfactant protein (SP)-A and SP-D as antimicrobial and immunotherapeutic agents.
Awasthi S
Recent Pat Antiinfect Drug Discov 2010 Jun;5(2):115-23. doi: 10.2174/157489110791233559. PMID: 20230362
Neonatal chronic lung disease and exogenous surfactant therapy.
Greenough A
Eur J Pediatr 1998 Jan;157 Suppl 1:S16-8. doi: 10.1007/pl00014283. PMID: 9462901

Prognosis

A Newfoundland cohort of familial and sporadic idiopathic pulmonary fibrosis patients: clinical and genetic features.
Fernandez BA, Fox G, Bhatia R, Sala E, Noble B, Denic N, Fernandez D, Duguid N, Dohey A, Kamel F, Edwards L, Mahoney K, Stuckless S, Parfrey PS, Woods MO
Respir Res 2012 Aug 1;13(1):64. doi: 10.1186/1465-9921-13-64. PMID: 22853774Free PMC Article
Lung surfactant alterations in pulmonary thromboembolism.
Calkovska A, Mokra D, Calkovsky V
Eur J Med Res 2009 Dec 7;14 Suppl 4(Suppl 4):38-41. doi: 10.1186/2047-783x-14-s4-38. PMID: 20156722Free PMC Article
Pulmonary surfactant protein A, B, and C mRNA and protein expression in the nitrofen-induced congenital diaphragmatic hernia rat model.
Van Tuyl M, Blommaart Pj, Keijzer R, Wert SE, Ruijter J, Lamers WH, Tibboel D
Pediatr Res 2003 Nov;54(5):641-52. Epub 2003 Aug 6 doi: 10.1203/01.PDR.0000086906.19683.42. PMID: 12904592
Bronchoalveolar lavage fluid surfactant protein-A and surfactant protein-D are inversely related to inflammation in early cystic fibrosis.
Noah TL, Murphy PC, Alink JJ, Leigh MW, Hull WM, Stahlman MT, Whitsett JA
Am J Respir Crit Care Med 2003 Sep 15;168(6):685-91. Epub 2003 Jun 26 doi: 10.1164/rccm.200301-005OC. PMID: 12829455
Surfactant protein A decreases lung injury and mortality after murine marrow transplantation.
Yang S, Milla C, Panoskaltsis-Mortari A, Hawgood S, Blazar BR, Haddad IY
Am J Respir Cell Mol Biol 2002 Sep;27(3):297-305. doi: 10.1165/rcmb.2002-0035OC. PMID: 12204891

Clinical prediction guides

Alpha-1 antitrypsin deficiency impairs lung antibacterial immunity in mice.
Ostermann L, Maus R, Stolper J, Schütte L, Katsarou K, Tumpara S, Pich A, Mueller C, Janciauskiene S, Welte T, Maus UA
JCI Insight 2021 Feb 8;6(3) doi: 10.1172/jci.insight.140816. PMID: 33554955Free PMC Article
Surfactant Protein-A Protects against IL-13-Induced Inflammation in Asthma.
Francisco D, Wang Y, Conway M, Hurbon AN, Dy ABC, Addison KJ, Chu HW, Voelker DR, Ledford JG, Kraft M
J Immunol 2020 May 15;204(10):2829-2839. Epub 2020 Apr 3 doi: 10.4049/jimmunol.1901227. PMID: 32245819Free PMC Article
Surfactant protein A binds flagellin enhancing phagocytosis and IL-1β production.
Ketko AK, Lin C, Moore BB, LeVine AM
PLoS One 2013;8(12):e82680. Epub 2013 Dec 2 doi: 10.1371/journal.pone.0082680. PMID: 24312669Free PMC Article
Surfactant protein (SP)-A and SP-D as antimicrobial and immunotherapeutic agents.
Awasthi S
Recent Pat Antiinfect Drug Discov 2010 Jun;5(2):115-23. doi: 10.2174/157489110791233559. PMID: 20230362
Polymorphisms of surfactant protein A genes and the risk of bronchopulmonary dysplasia in preterm infants.
Weber B, Borkhardt A, Stoll-Becker S, Reiss I, Gortner L
Turk J Pediatr 2000 Jul-Sep;42(3):181-5. PMID: 11105614

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