Surfactant protein B deficiency and gene mutations for neonatal respiratory distress syndrome in China Han ethnic population

Int J Clin Exp Pathol. 2013;6(2):267-72. Epub 2013 Jan 15.

Abstract

Objective: To determine whether the SP-B deficiency and gene mutations in exon 4 is associated with neonatal RDS in China Han ethnic population.

Methods: The study population consisted of 40 neonates with RDS and 40 neonates with other diseases as control in China Han ethnic population. We Compared SP-B expression in lung tissue and bronchoalveolar lavage fluid with immunoblotting, and analyzed mutations in the SP-B gene with polymerase chain reaction (PCR) and gene sequencing.

Results: In RDS group, low mature Surfactant protein B was found in both lung tissue and bronchoalveolar lavage fluid in 8 neonates. In control group, only 4 neonates with low mature Surfactant protein B in both lung tissue and bronchoalveolar lavage fluid. In RDS group, 20 neonates were found to have mutations in exon 4, 12 homozygous mutations with C/C genotype and 8 heterozygous mutations with C/T genotype in surfactant protein B gene+1580 polymorphism. There were 8 cases mutations in control group, 1 in C/C and 7 in C/T genotype. The frequency of homozygotes with C/C genotype was 0.3 and frequency of heterozygotes with C/T genotype was 0.02 in RDS group. In control group, frequency of homozygotes with C/C genotype was 0.025 and frequency of heterozygote with C/T genotype was 0.175.

Conclusion: Low mature Surfactant protein B is associated with the pathogenesis of neonatal respiratory distress syndrome (RDS) in China Han ethnic population. Mutations in exon 4 of the surfactant protein B gene demonstrate an association between homozygous mutations with C/C genotype in SP-B gene and neonatal RDS.

Keywords: Polymorphisms; neonates; respiratory distress syndrome; surfactant protein B.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Asian People / genetics*
  • Asian People / statistics & numerical data
  • Bronchoalveolar Lavage Fluid / chemistry
  • Exons / genetics
  • Female
  • Genetic Predisposition to Disease / ethnology
  • Genotype
  • Heterozygote
  • Homozygote
  • Humans
  • Infant, Newborn
  • Infant, Premature
  • Lung / metabolism
  • Male
  • Polymorphism, Single Nucleotide
  • Pulmonary Alveolar Proteinosis / congenital*
  • Pulmonary Alveolar Proteinosis / ethnology
  • Pulmonary Alveolar Proteinosis / genetics
  • Pulmonary Surfactant-Associated Protein B / deficiency
  • Pulmonary Surfactant-Associated Protein B / genetics*
  • Pulmonary Surfactant-Associated Protein B / metabolism
  • Respiratory Distress Syndrome, Newborn / ethnology
  • Respiratory Distress Syndrome, Newborn / genetics*

Substances

  • Pulmonary Surfactant-Associated Protein B

Supplementary concepts

  • Congenital Deficiency of Pulmonary Surfactant Protein B