The Annals of thoracic surgery
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Case Reports
Birt-Hogg-Dubé syndrome in a patient presenting with familial spontaneous pneumothorax.
Birt-Hogg-Dubé (BHD) syndrome is a recently discovered autosomal-dominant disease caused by a mutation in the folliculin gene. We report a patient with familial spontaneous pneumothorax who was found to have BHD syndrome. Patients with a personal and family history of pneumothoraces and computed tomographic (CT) findings of multiple pulmonary cysts should alert the thoracic surgeon to this syndrome; additional evaluation and testing may be warranted.
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The prevalence for thoracic aortic aneurysms (TAAs) is significantly increased in patients with a bicuspid aortic valve (BAV) compared with patients who have a normal tricuspid aortic valve (TAV). TAA rupture is a life-threatening event, and biomechanics-based simulations of the aorta may help to disentangle the molecular mechanism behind its development and progression. The present study used polarized microscopy and macroscopic in vitro tensile testing to explore collagen organization and mechanical properties of TAA wall specimens from BAV and TAV patients. ⋯ The aneurysmal aortas of BAV patients show a higher macroscopic strength, mainly due to an increased collagen-related stiffness, compared with TAV patients. The increased wall stiffness in BAV patients may contribute to the higher prevalence for TAAs in this group.
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The use of an epicardial pacing lead may be essential in pediatric patients with rhythm disturbances. In patients with single-ventricle physiology who need scheduled multistage operations, the lead could be an obstacle because of the induction of tight adhesions and the need for dissection in the next operation. We propose a novel reproducible technique for the placement of an epicardial pacing lead followed by easy dissection of the entire length of the lead in the next operation in neonates or young infants who need multistage operations.
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Multicenter Study Clinical Trial
Serum brain natriuretic peptide and risk of acute kidney injury after cardiac operations in children.
Acute kidney injury (AKI) after pediatric cardiac operations is associated with poor outcomes and is difficult to predict. We conducted a prospective study to evaluate whether preoperative brain natriuretic peptide (BNP) levels predict postoperative AKI among children undergoing cardiac operations. ⋯ Preoperative BNP levels did not predict postoperative AKI in this cohort of children undergoing cardiac operations. Both preoperative and postoperative BNP levels are associated with postoperative outcomes. Clinical Trial Registration at Clinicaltrials.gov as NCT00774137.