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OBJECTIVE: Prior studies have evaluated maternal outcomes in patients with hereditary hemorrhagic telangiectasia (HHT), yet pregnancy- and delivery-specific data remain limited. This study aims to evaluate pregnancy and delivery outcomes in patients with HHT. STUDY DESIGN: This retrospective cohort study used the Nationwide Readmissions Database to identify patients with HHT diagnosis on delivery between 2010 and 2021. The primary outcome was severe maternal morbidity (SMM). Secondary outcomes included nontransfusion SMM, preterm birth, stillbirth, prelabor rupture of membranes or preterm prelabor rupture of membranes, cesarean delivery, respiratory bleeding, cerebrovascular complications, patient disposition, and length of stay. Trends in the prevalence of HHT at delivery were assessed with logistic regression. Logistic regression analyses, adjusting for age, payer, zip code income, hospital size, and teaching status, were also used to produce adjusted relationships between HHT status and outcomes. RESULTS: The cohort of 21,698,861 delivered pregnancies corresponded to a national estimate of 44,325,599. Of those, 612 (national estimate: 1,265; 2.8 per 100,000) had a diagnosis of HHT. A steady rise in the HHT diagnosis rate during pregnancy from 2010 to 2021 (1.7 per 100,000 in 2010, 3.8 per 100,000 in 2021, p < 0.001 for trend) was seen. Patients with HHT were significantly more likely to experience SMM compared with patients without HHT (7.8 vs. 1.7%, adjusted relative risk [aRR]: 4.49 [95% confidence interval, CI: 3.06, 6.58]). Rates of preterm birth (14.2 vs. 8.5%, aRR: 1.57 [95% CI: 1.22, 2.03]), cesarean delivery (41.0 vs. 32.9%, aRR: 1.23 [95% CI: 1.07, 1.41]), respiratory bleeding (2.1 vs. <0.1%, aRR: 94.44 [56.64, 157.46]), and cerebrovascular complications (0.9 vs. <0.1%, aRR: 22.89 [9.89, 52.96]) were higher in patients with HHT than non-HHT patients. There was no difference in stillbirth rates between groups. CONCLUSION: Patients with HHT have higher rates of SMM and adverse delivery outcomes when compared with the baseline population. KEY POINTS: · There was a steady rise in the rates of HHT during pregnancy from 2010 to 2021.. · Patients with HHT are more likely to experience SMM.. · Patients with HHT are more likely to have a preterm delivery and cesarean delivery..
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Lung disease causes most of the morbidity and mortality in cystic fibrosis (CF). Understanding the pathogenesis of this disease has been hindered, however, by the lack of an animal model with characteristic features of CF. To overcome this problem, we recently generated pigs with mutated CFTR genes. We now report that, within months of birth, CF pigs spontaneously developed hallmark features of CF lung disease, including airway inflammation, remodeling, mucus accumulation, and infection. Their lungs contained multiple bacterial species, suggesting that the lungs of CF pigs have a host defense defect against a wide spectrum of bacteria. In humans, the temporal and causal relations between inflammation and infection have remained uncertain. To investigate these processes, we studied newborn pigs. Their lungs showed no inflammation but were less often sterile than controls. Moreover, after introduction of bacteria into their lungs, pigs with CF failed to eradicate bacteria as effectively as wild-type pigs. These results suggest that impaired bacterial elimination is the pathogenic event that initiates a cascade of inflammation and pathology in CF lungs. Our finding that pigs with CF have a host defense defect against bacteria within hours of birth provides an opportunity to further investigate CF pathogenesis and to test therapeutic and preventive strategies that could be deployed before secondary consequences develop.