Carlos Botas

Surfactant protein D (SP-D) is one of two collectins found in the pulmonary alveolus. On the basis of homology with other collectins, potential functions for SP-D include roles in innate immunity and surfactant metabolism. The SP-D gene was disrupted in embryonic stem cells by homologous recombination to generate mice deficient in SP-D. Mice heterozygous for the mutant SP-D allele had SP-D concentrations that were approximately 50% wild type but no other obvious phenotypic abnormality. Mice totally deficient in SP-D were healthy to 7 months but had a progressive accumulation of surfactant lipids, SP-A, and SP-B in the alveolar space. By 8 weeks the alveolar phospholipid pool was 8-fold higher than wild-type littermates. There was also a 10-fold accumulation of alveolar macrophages in the null mice, and many macrophages were both multinucleated and foamy in appearance. Type II cells in the null mice were hyperplastic and contained giant lamellar bodies. These alterations in surfactant homeostasis were not associated with detectable changes in surfactant surface activity, postnatal respiratory function, or survival. The findings in the SP-D-deficient mice suggest a role for SP-D in surfactant homeostasis.

BACKGROUND: Intravenous (i.v.) hydrocortisone (HC) has been used recently in selected preterm infants for hypotension soon after birth. During the same time period that HC was used, there was a marked increase in the incidence of disseminated candidal infections (DCIs). OBJECTIVE: To determine whether there is an association between DCI in the first 35 days of life and i.v. HC in preterm infants. RESEARCH DESIGN: A hospital case-control study comparing the exposure of HC between preterm infants with DCI and matched infants without DCI. SETTING: A tertiary level intensive care nursery in a major teaching hospital in San Francisco, CA. PATIENTS: Seventeen preterm infants with DCI and 25 infants without DCI, with gestational age younger than 28 weeks and birth weight less than 1000 g, inborn and outborn admitted to the intensive care nursery between January 1992 and September 1993. METHODS: All preterm infants diagnosed with DCI at younger than 35 days of age were identified using a perinatal and neonatal database. DCI was defined as a blood, cerebrospinal fluid, or two urine cultures positive for Candida requiring antifungal therapy. A control group of uninfected infants matched for the major risk factors for DCI (gestational age, birth weight, duration of intubation, broad-spectrum antibiotics, and i.v. alimentation, including lipids and central venous catheters) admitted during the same period was identified using the same database. Postmatching comparison was performed for several other factors to detect any other differences between the groups. RESULTS: The infants with DCI (n = 17) and control infants (n = 25) had no statistical difference in exposure to the major risk factors for DCI or in postmatching comparison. Ten (59%) of the infants with DCI were receiving HC at the time of infection, whereas four (16%) of the control infants received HC during the first 35 days of life. Infants with DCI were 7.5 times as likely as control infants (95% confidence interval, 5 to 11) to have received IV HC before the onset of fungal infection. CONCLUSION: We conclude that the administration of i.v. HC significantly increases the risk of DCI in susceptible preterm infants younger than 35 days of age. The potentially serious risks of DCI should be considered particularly in the patient selection process for administration of i.v. HC.

Neonatal lupus (NLE) is a rare acquired autoimmune disorder caused by transplacental passage of maternal autoantibodies to Sjogren's Syndrome A or B (SSA-SSB) autoantigens (Vanoni et al. in Clin Rev Allerg Immunol 53:469-476, 2017) which target fetal and neonatal tissues for immune destruction. The cardiac trademark of NLE is autoimmune heart block, which accounts for more than 80% of cases of complete atrioventricular heart block (AVB) in newborns with a structurally normal heart (Martin in Cardiol Young 24: 41-46, 2014). NLE presenting with cardiac alterations not involving rhythm disturbances are described in the literature, but they are rare. Here, we report a case of a neonate with high anti-SSA antibodies who developed severe ventricular dysfunction in the absence of rhythm abnormalities, endocardial fibroelastosis, and dilated cardiomyopathy (Trucco et al. in J Am Coll Cardiol 57:715-723, https://doi.org/10.1016/j.jacc.2010.09.044 , 2011), the most common cardiac presentations of NLE. The patient developed severe multiorgan dysfunction syndrome that required prolonged critical care support but fully recovered and was discharged home. We highlight the unusual clinical features of this NLE case and the importance of timely treatment of NLE allowing complete recovery of a critically ill neonate.