Targeting Molecular Chaperones for the Treatment of Cystic Fibrosis: Is It a Viable Approach?

Cystic Fibrosis (CF) is largely caused by protein misfolding and the loss of function of a plasma membrane anion channel known as the cystic fibrosis transmembrane conductance regulator (CFTR). The most common CF-causing mutation, F508del, leads to severe conformational defect in CFTR. The cellular chaperone machinery plays an important role in CFTR biogenesis and quality control.

Evolution of surgical techniques for aneurysms of the descending thoracic aorta: twenty-nine years experience with 659 patients.

Between 1953 and 1993, 659 patients underwent descending thoracic aneurysm resection. The most common etiology was atherosclerosis. Pain was the main presenting symptom.

Five thousand seven hundred sixty cardiovascular injuries in 4459 patients. Epidemiologic evolution 1958 to 1987.

Large epidemiologic analyses of cardiovascular injuries have been limited to studies of military campaigns compiled from many surgeons working in many hospitals with variable protocols. A detailed civilian vascular trauma registry provides a unique opportunity for an epidemiologic evolutionary profile. During the last 30 years in a single civilian trauma center directed by a consistent evaluation and treatment philosophy, 4459 patients were treated for 5760 cardiovascular injuries.

Civilian trauma in the 1980s. A 1-year experience with 456 vascular and cardiac injuries.

During 1982, 312 patients with 408 vascular injuries and 48 cardiac injuries were seen. Two or more vascular or cardiac injuries were present in 34% of patients. Over 87% of injuries were secondary to gunshot wounds, stab wounds, or shotgun wounds.

Congenital partial pericardial defect: surgical correction by partial pericardiectomy through a median sternotomy.

A case of symptomatic congenital pericardial defect with herniation of the left atrial appendage is reported. The patient had experienced increasing fatigue, chest pain, shortness of breath, and slight cyanosis. A partial pericardiectomy through a median sternotomy was performed and the patient made an uneventful recovery.

Double prosthetic aortic valve. Case report.

A 55-year-old man underwent insection of a Hufnagel valve into his descending thoracic aorta for aortic valve insufficiency 18 years ago. He remained asymptomatic for 16 years. Because of progressive left ventricular failure his incompetent aortic valve was replaced with a De Bakey carbon ball aortic valve prosthesis 2 years later.

Complete occlusion of the infrarenal abdominal aorta: management and results in 64 patients.

Sixty-four patients with complete occlusion of the infrarenal abdominal aorta were reviewed. The clinical findings, diagnostic procedures, and surgical management are described. Sixty-one patients underwent thromboendarterectomy and bypass grafts from the infrarenal abdominal aorta to the iliac or common femoral arteries.

Inadvertent femoral artery "stripping": surgical management.

Following "vein stripping" for varicosities, two patients were referred to our service for evaluation of arterial insufficiency of the lower extremities. Both patients had had surgical interruption of the femoral arterial system which required reconstruction. This paper emphasizes the importance of understanding surgical anatomy and presents the techniques of successful surgical management of both cases.