Bile Acid Pneumonia: A “New” Form of Neonatal Respiratory Distress Syndrome?

Abstract

We describe 3 cases of neonatal respiratory distress syndrome (RDS) in near-term infants, born from mothers with severe intrahepatic cholestasis of pregnancy. Common pictures of the cases were: good indices of lung maturity in the amniotic fluid; severe RDS requiring mechanical ventilation; high serum bile acid (BA) levels in the early days of life; no meconium aspiration; negative cultures; and absence of indirect laboratory signs of infection. After the first case, we hypothesized that abnormally high BA levels could have reversed the action of phospholipase A2 in the lungs, causing a degradation of phosphatidylcholines to lysophosphatidylcholines and the consequent lack of surfactant activity, leading to the severe respiratory distress. Consequently, in cases 2 and 3, we gave intratracheal surfactant to the infants, which, although administered around the first 24 hours of life, showed to be helpful. Our experience suggests that a high level of attention in the management of newborn infants (even near-term infants) born from women with intrahepatic cholestasis of pregnancy is necessary to detect as soon as possible signs and symptoms of this "unexpected" RDS, which can assume a very severe clinical picture. In such instances, we recommend that the diagnosis of BA pneumonia be kept in mind and that exogenous surfactant be given as soon as possible, even in the presence of indices of normal lung maturity in the amniotic fluid. Finding high levels of BA and lysophosphatidylcholines in the bronchoalveolar lavage of affected infants would aid in support of the diagnosis.

Anesthesia Fatality due to Bile Aspiration: Lessons to be Learnt

“…Bile acid and bile salts have been incriminated in causing mucosal injury to esophagus, larynx [4], epiglottis and lung parenchyma. They have been found to be associated with neonatal respiratory distress syndrome [5], bile pneumonia [5], alveolitis, bronchioloitis obliterans [6,7], and exaggerated injury in ventilator associated pneumonias [8,9]. There is understandably a paucity of studies done on humans but experimental studies conducted on rats [10], rabbits [11] and pigs [12], have demonstrated that bile acids cause inflammation and cytotoxic effects on histologic examinations.…”

“…Bile acid has repeatedly been shown to cause direct pulmonary damage. The mechanisms have been oxidative stress, damage to endothelial cells, Type 1 and 2 pneumocytes, depletion of surfactant and damage to phospholipids [4][5][6]. This aspiration leads to chemical pneumonitis that turns into a fulminant ARDS carrying a much higher mortality.…”

“…Anesthesia management needs to be improvised because patient is high risk for aspiration. Rapid sequence intubation should be the standard practice [1][2][3][4][5][6][7].…”

Dissecting the regulation of bile-induced biofilm formation in Staphylococcus aureus

“…El-Serag & Sonnenberg showed that patients with erosive oesophagitis, a sign of significant GERD, had increased incidence of pulmonary fibrosis, chronic bronchitis or chronic obstructive pulmonary disease in a case-control study of more than 200 000 patients (el-Serag & Sonnenberg, 1997). A strong correlation between GERD-derived reflux, pulmonary aspiration and increased lung damage also extends to several other respiratory diseases (Navarro et al, 2001), including idiopathic pulmonary disease and advanced lung damage arising from lung transplantation (Sweet et al, 2006(Sweet et al, , 2007(Sweet et al, , 2009, ventilator-associated pneumonia (Wu et al, 2009), Barrett's oesophagus and oesophageal adenocarcinoma (Nassr et al, 2011), and bile acid pneumonia in neonates (Zecca et al, 2004(Zecca et al, , 2008. Therefore, the implications of elucidating the link between bile aspiration and chronic pathogen behaviour has consequences for a range of clinical conditions.…”

Toxic effects of different meconium fractions on lung function: new therapeutic strategies for meconium aspiration syndrome?

“…Furthermore, the lipid fraction has a stronger inhibitory effect on surfactant function than the water-soluble subfraction. [4][5][6][7] It is well known that bile salts may cause inflammation 8,9 and that FFA, such as oleic acid, induce severe lung failure when administered intravenously. 10 We have tested the effects of different meconium subfractions on pulmonary function in an animal model of newborn piglets.…”