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Thoracic Outlook Syndrome

Alain Li-Wan-Po, Bart Loeys, Peter Farndon, David Latham, Caroline Bradley
The translational path from pharmacological insight to effective therapy can be a long one. We aim to describe the management of Marfan syndrome as a case-example of how pharmacological and genomic insights can contribute to improved therapy. We undertook a literature search for studies of Marfan syndrome, to identify milestones in description, understanding and therapy of the syndrome. From the studies retrieved we then weaved an evidence-based description of progress. Marfan syndrome shows considerable heterogeneity in clinical presentation...
July 2011: British Journal of Clinical Pharmacology
A Galindo, O Nieto, M T Nieto, M O Rodríguez-Martín, I Herraiz, D Escribano, M A Granados
OBJECTIVES: To analyze the characteristics and clinical implications of right aortic arch (RAA) detected in fetal life. METHODS: Retrospective study of all cases of RAA diagnosed prenatally in high-risk patients who underwent fetal echocardiography between 2000 and 2007. RESULTS: There were 48 RAA; 18 had vascular ring (Group 1), including 15 RAA with aberrant left subclavian artery and 3 double aortic arch, and 30 had not vascular ring (Group 2), all RAA with mirror-image branching...
October 2009: Prenatal Diagnosis
Anthony J Fillmore, R James Valentine
BACKGROUND: Mortality in patients with infected aortic aneurysms remains high. A number of patient- and infection-specific risk factors for death have been proposed, but none is consistently predictive of poor outcomes. The purpose of this study was to examine the possible contribution of infection-related risk factors and the systemic inflammatory response syndrome (SIRS) to outcomes of patients with infected aortic aneurysms. STUDY DESIGN: Ten patients with infected aortic aneurysms presenting to our institution over a recent 6-year period were studied...
March 2003: Journal of the American College of Surgeons
Juan P Umaña, D Craig Miller, R Scott Mitchell
BACKGROUND: Controversy continues regarding treatment for patients with acute type B aortic dissection. METHODS: One hundred eighty-nine patients with acute type B aortic dissection managed over a 36-year period were analyzed retrospectively for three outcome endpoints: survival; freedom from reoperation, and freedom from late aortic-related complications or late death. Risk factors for death were identified using a multivariable Cox proportional hazards model. Then to account for patient selection bias, heterogeneity of the population, and continuous evolution in techniques, propensity score analysis was used to identify risk-matched cohorts (quintiles I and II) in which the results of medical (n = 111) or surgical (n = 31) therapy were compared more comprehensively...
November 2002: Annals of Thoracic Surgery
Shelagh Joss, John C S Dean
We report two 12-year-old monozygotic twins followed from birth. Their features include midface hypoplasia, a prominent forehead, coarse features, sensorineural deafness, short stature with thoracic kyphosis and lumbar lordosis and intellectual delay. As they have developed, their features have been reminiscent of a storage disorder but mucopolysaccharidoses, mucolipidoses and gangliosidoses have been excluded by biochemical testing. We discuss the phenotypic overlap with the Schinzel-Giedion syndrome but highlight the important differences...
October 2002: Clinical Dysmorphology
N C Poirier, J J Drummond-Webb, K Hisamochi, M Imamura, A M Harrison, R B Mee
OBJECTIVE: The results of our modification of the stage I Norwood procedure, in which we use only autologous tissue to reconstruct the aortic arch, were reviewed. A high-flow, low-pressure cardiopulmonary bypass protocol (with phenoxybenzamine), before and after a period of deep hypothermic circulatory arrest, was used. METHODS: Between 1993 and 1999, 59 patients, aged 1 to 353 days (median 4 days) and weighing 1.7 to 6.8 kg (median 3.2 kg), underwent a modified Norwood procedure...
November 2000: Journal of Thoracic and Cardiovascular Surgery
Y Ducic, A Crepeau, L Ducic, A Lamothe, M Corsten
BACKGROUND: Traditionally, Pancoast tumors have been associated with an extremely poor outlook. Recently, Dartevelle and colleagues have noted a significant survival advantage in patients treated by wide en bloc excision. METHODS: Utilizing an illustrative case example, step by step exposure of the lung apex and first rib is provided with the Dartevelle approach. RESULTS: Safe exposure was provided by this combined transcervical and transthoracic approach...
December 1999: Head & Neck
A Wilhelm
The striking similarity of certain pain symptoms and neurologic changes in RSD and the findings of severe thoracic outlet and inlet syndrome were the reason why clinical diagnostic studies of this dystrophy were also completed by radiologic examinations of the vessel system. Surprisingly, more or less striking and differently configurated stenoses in the area of the subclavian vein, with a resulting impairment of the venous run-off, were found. Those findings have been documented in 20 of 21 patients with RSD since 1984 by functional venography and, in the meantime, they were confirmed intraoperatively in nine resistant cases...
August 1997: Hand Clinics
I Simpson, P E Campbell
From 1970 to 1989, 121 children with mediastinal masses of various sorts were seen in the Department of Pathology, Royal Children's Hospital, Melbourne. The series is considered representative of the true incidence of these conditions in the state of Victoria, which had an average paediatric population during the time of this series of 900,000 children. The commonest cause of a mediastinal mass was NHL (36 cases). This was followed by HD (24 cases), then neuroblastoma and ganglioneuroma (16 and 9 cases respectively), duplication cysts (10 cases), teratomas (7 cases), neurofibroma (4 cases) and lymphangioma (3 cases)...
1991: Progress in Pediatric Surgery
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