Aneurysms.

Congenital contractural arachnodactyly (CCA) was described by Beals and Hecht as an autosomal dominant disorder distinct from Marfan syndrome and comprising joint contractures, arachnodactyly, scoliosis, and a distinct "crumpled ear" deformity. While the disorder is similar to Marfan syndrome, it was split from it due to the distinct physical appearance of the patients and, more importantly, the lack of heart and eye findings. Since the original report, several CCA patients have been found to have mitral valve prolapse, structural cardiac anomalies, and occasionally aortic root dilatations similar to those seen in Marfan syndrome. We report on a patient with CCA with bilateral ectopia lentis and aortic root dilatation. Our review of the literature of CCA showed that serial echocardiography and careful eye examinations have not become a standard of medical practice in this condition. Partly this may be due to a lack of documented cases of CCA having severe ectopia lentis and cardiac complications. This patient underscores the need for periodic eye and echocardiographic evaluations of all CCA patients.

Aortic Aneurysm↗

PubMed1992

A retrospective mortality study within operating segments of a petroleum company.

This retrospective mortality study was conducted among 34,597 oil industry workers in diverse operating segments. Employees were traced through Statistics Canada, and overall mortality (SMR = 0.85) was lower than general population rates and similar to other petrochemical cohorts. The most notable finding was a significant excess of malignant melanoma [observed deaths (N) = 16, SMR = 1.87, 95% CI = 1.07, 3.04], which concentrated among upstream workers (N = 6, SMR = 6.00, 95% CI = 2.19, 13.06), and was directly related to employment duration and latency. Specific substances or hydrocarbon (HC) streams could not be implicated, although possible explanations include dermal HC exposure, ultraviolet light exposure, or a synergistic effect between these two factors. Marketing/transportation workers showed a non-significant excess of multiple myeloma (SMR = 1.81), which was also related to employment duration, latency, and commencement of employment before 1950. Lymphatic cancer, skin cancer, and kidney cancer mortality was not elevated in refinery workers, a finding at odds with some previous refinery worker studies. Although the malignant melanoma and possibly the multiple myeloma mortality patterns are consistent with an occupational link, further studies are needed to investigate the relationship of these diseases with particular exposures.

Aortic Aneurysm↗