Human surfactant proteins A2 (SP-A2) and B (SP-B) genes as determinants of respiratory distress syndrome

2015 ◽  
Vol 52 (5) ◽  
pp. 391-394
Author(s):  
Walaa A. Abuelhamed ◽  
Nancy Zeidan ◽  
Walaa A. Shahin ◽  
Hoda I. Rizk ◽  
Walaa A. Rabie
Keyword(s):  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Distress Syndrome ◽  
Surfactant Proteins

Surfactant Proteins and Respiratory Distress Syndrome

2015 ◽  
pp. 231-236
Author(s):  
Joanna Floros ◽  
David S. Phelps ◽  
Daphne E. deMello ◽  
Jeff Longmate ◽  
Heather Harding
Keyword(s):  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Distress Syndrome ◽  
Surfactant Proteins

Pulmonary glycogen deficiency as a new potential cause of respiratory distress syndrome

2020 ◽  
Author(s):  
Giorgia Testoni ◽  
Bárbara Olmeda ◽  
Jordi Duran ◽  
Elena López-Rodríguez ◽  
Mònica Aguilera ◽  
...  
Keyword(s):  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Perinatal Mortality ◽  
Distress Syndrome ◽  
Glycogen Synthase ◽  
Glycogen Storage Disease Type ◽  
Glycogen Storage ◽  
Surfactant Proteins ◽  
Lung Expansion ◽  
Key Factor

Abstract The glycogenin knockout mouse is a model of Glycogen Storage Disease type XV. These animals show high perinatal mortality (90%) due to respiratory failure. The lungs of glycogenin-deficient embryos and P0 mice have a lower glycogen content than that of wild-type counterparts. Embryonic lungs were found to have decreased levels of mature surfactant proteins SP-B and SP-C, together with incomplete processing of precursors. Furthermore, non-surviving pups showed collapsed sacculi, which may be linked to a significantly reduced amount of surfactant proteins. A similar pattern was observed in glycogen synthase1-deficient mice, which are devoid of glycogen in the lungs and are also affected by high perinatal mortality due to atelectasis. These results indicate that glycogen availability is a key factor for the burst of surfactant production required to ensure correct lung expansion at the establishment of air breathing. Our findings confirm that glycogen deficiency in lungs can cause respiratory distress syndrome and suggest that mutations in glycogenin and glycogen synthase 1 genes may underlie cases of idiopathic neonatal death.

Surfactant Proteins and Anti-Surfactant Antibodies in Sera from Infants With Respiratory Distress Syndrome With and Without Surfactant Treatment

PEDIATRICS ◽  
1991 ◽  
Vol 88 (1) ◽  
pp. 84-89
Author(s):  
Shoichi Chida ◽  
David S. Phelps ◽  
Roger F. Soll ◽  
H. William Taeusch
Keyword(s):  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Distress Syndrome ◽  
Surfactant Protein ◽  
Surfactant Proteins ◽  
Lung Damage ◽  
Control Group ◽  
Severe Respiratory Distress ◽  
Surfactant Treatment ◽  
And Control

The presence of surfactant protein antigenemia and of surfactant protein antibodies was determined in serum from surfactant-treated and control infants with respiratory distress syndrome who were enrolled in a prospective randomized clinical trial. The surfactant used for treatment (surfactant TA) contained surfactant proteins (SPs) B and C and no SP-A. Enzymelinked immunosorbent assays (ELISAs) that identify surfactant-associated proteins and ELISAs that identify IgG or IgM directed against surfactant proteins were used to investigate sera from these infants obtained prior to treatment, at 1 week of age, and at 2 months of age. There were no significant differences between average values in the surfactant-treated and control groups at each time period. However, in the control group, averaged results from ELISAs that identify SP-A and that identify IgM antibodies to SP-A or to SP-B,C showed significant differences between pretreatment sera and sera obtained at 1 week of age. No significant differences were noted in averaged results for IgG. Positive ELISA values were more frequently found in the control group than in the surfactant-treated group with regard to SP-A, and IgM against SP-A and SP-B,C in sera from neonates at 1 week of age. No positive ELISA values were found in sera from infants at 2 months of age. It is concluded that some patients with severe respiratory distress syndrome presumably leak surfactant proteins into the circulation and that this induces transient low titers of IgM antibody. This occurrence is decreased with surfactant treatment. Surfactant treatment may reduce leak of surfactant proteins into the vascular space by reducing lung damage.

scholarly journals Hericium Erinaceum (Yamabushitake) Extract-induced Acute Respiratory Distress Syndrome Monitored by Serum Surfactant Proteins

2003 ◽  
Vol 42 (12) ◽  
pp. 1219-1222 ◽  
Author(s):  
Munehide NAKATSUGAWA ◽  
Hiroki TAKAHASHI ◽  
Chikako TAKEZAWA ◽  
Kazutaka NAKAJIMA ◽  
Kazutoki HARADA ◽  
...  
Keyword(s):  
Acute Respiratory Distress Syndrome ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Distress Syndrome ◽  
Surfactant Proteins ◽  
Hericium Erinaceum

scholarly journals Expression of acute-phase cytokines, surfactant proteins, and epithelial apoptosis in small airways of human acute respiratory distress syndrome

2013 ◽  
Vol 28 (1) ◽  
pp. 111.e9-111.e15 ◽  
Author(s):  
Ruy Camargo Pires-Neto ◽  
Maina Maria Barbosa Morales ◽  
Tatiana Lancas ◽  
Nicole Inforsato ◽  
Maria Irma Seixas Duarte ◽  
...  
Keyword(s):  
Acute Respiratory Distress Syndrome ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Acute Phase ◽  
Distress Syndrome ◽  
Surfactant Proteins ◽  
Small Airways

scholarly journals Review: The intersection of surfactant homeostasis and innate host defense of the lung: lessons from newborn infants

2010 ◽  
Vol 16 (3) ◽  
pp. 138-142 ◽  
Author(s):  
Jeffrey A. Whitsett
Keyword(s):  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Host Defense ◽  
Distress Syndrome ◽  
Newborn Infants ◽  
Structure And Function ◽  
Surfactant Proteins ◽  
Inherited Disorders ◽  
Expression Of Genes ◽  
And Function

The study of pulmonary surfactant, directed towards prevention and treatment of respiratory distress syndrome in preterm infants, led to the identification of novel proteins/genes that determine the synthesis, packaging, secretion, function, and catabolism of alveolar surfactant. The surfactant proteins, SP-A, SP-B, SP-C, and SP-D, and the surfactant lipid associated transporter, ABCA3, play critical roles in surfactant homeostasis. The study of their structure and function provided insight into a system that integrates the biophysical need to reduce surface tension in the alveoli and the innate host defenses required to maintain pulmonary structure and function after birth. Alveolar homeostasis depends on the intrinsic, multifunctional structures of the surfactant-associated proteins and the shared transcriptional regulatory modules that determine both the expression of genes involved in surfactant production as well as those critical for host defense. Identification of the surfactant proteins and the elucidation of the genetic networks regulating alveolar homeostasis have provided the basis for understanding and diagnosing rare and common pulmonary disorders, including respiratory distress syndrome, inherited disorders of surfactant homeostasis, and pulmonary alveolar proteinosis.

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