Surfactant Protein B Deficency

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Abstract

Surfactant Protein B is critically responsible for the functioning of healthy lungs. In the absence of Surfactant Protein B adverse lung condition such as acute respiratory distress syndrome arise in infants victims. Due to this, SP-B’s condition has drawn the attention of medical researches. Physiological ailments such as lung failure, molecular defects and cellular deficiencies in infants are associated with opportunistic to Surfactant Protein B syndrome. This relation and linkage stirs up the development of various treatment strategies of neonatal respiratory diseases. Surfactant Protein B syndrome was first identified as the main cause of congenital alveolar proteinosis, a condition where two infants from the same mother exhibit distinct histopathologic physical appearance, since their alveoli contain lipid rich acid Schiff-positive and granular proteinaceous material. Infants suffering from Surfactant Protein B syndrome exhibit foamy alveolar macrophages and desquamated alveolar epithelial cells. Acquired Sporadic and congenital alveolar proteinosis are the main clinical forms of alveolar proteinosis associated with infants. Since SP- B syndrome has become rampant and established in infants with respiratory distress, this paper evaluates the causative agents and implications of SP-B giving various recommendations for the use of Surfactant therapy in diverse clinical situations. This includes subjecting newborns to prophylactic natural surfactant therapy immediately after incubation. By so doing, paper primarily relates Surfactant Protein B syndrome to the clinical setting while exploring the implications for the neonatal nurse practitioner.

Definition of the Disease, Incidence

SP- B Deficiency is an inherited illness by newborn babies leading to severe respiratory failure in the early stages of life and is resistant to surfactant therapy, mechanical ventilation and extracorporeal membrane oxygenation. Genetic examination of infants suffering from Surfactant Protein B Deficiency shows identical mutation in surfactant protein gene gotten in many unrelated kindred. This interrupts the functioning and composition of pulmonary surfactant. Reported incidence of approximately 0.8 infants out of 1000 obtained from Missouri Department of Health Newborn Screening Program indicates that Surfactant Protein B syndrome is a major factor in causing pathophysiology of respiratory distress syndrome (Refere and Wilmott, 2012). The inability of young infants to produce surfactant and the structural immaturity of their lungs increases with the increase in gestation period. This indicates that out of 1000 babies, about 25% of them are caught with SP-B syndrome at 34th week adding up to 80% babies affected in a period of less than 54th week (Kendig and Wilmott, 2012). The 121ins2 mutation never showed in South African or Korean cohorts instead, there was similar correlation and frequency in Norwegian and Missouri cohorts. This indicated 173 incidences of infants suffering from Surfactant Protein B syndrome out of every 4000 samples of infants from Missouri cohort that were screened (Refere and Wilmott, 2012).

In the incidence describing two siblings with the condition of histopathologic due to congenital alveolar proteinases, examined lung tissues reveals the absence in one of the making blocks of surfactant protein called SP-B protein . This suggests that the incident of Surfactant Protein B syndrome causes respiratory failure in children possessing congenital alveolar proteinosis,

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