Fragile X Syndrome and Attentional Deficits

This study examined whether boys with Fragile X syndrome have a characteristic behavioural profile associated with the underlying genetic abnormality. Parents of 49 boys with Fragile X syndrome, 45 boys with Down's syndrome and 42 boys with intellectual disabilities of unknown aetiology were interviewed. Measures included the Childhood Behaviour Checklist, parent and teacher versions (CBCL), Parental Account of Childhood Symptoms (PACS) and Vineland social age and social quotient derived from the MRC Schedule of Handicaps, Behaviour and Skills (HBS).     

Syphilitic caries of the skull--the changing face of medicine.

This paper describes the specimens of caries sicca of calvaria due to syphilis in the collections of the Museums of the Royal College of Surgeons of England. The condition appears not to have been uncommon in the nineteenth century. The mechanism of bone destruction is discussed as is also the role of secondary infection and treatment with mercury.     

Composite resection of posterior pelvic malignancy.

Advanced pelvic cancer is a formidable challenge to surgical resection. These tumors commonly invade the bony pelvis, may involve other viscera, and usually have been irradiated previously. The authors are presenting experience with 76 patients who had composite resection of posterior or lateral pelvic malignancy. Fifty-eight patients had secondary cancers involving the musculoskeletal pelvis. This included 47 patients with advanced carcinoma of the rectum (41 curative, 6 palliative), 10 epidermoid cancers of the anorectum (8) or cervix (2), and 1 bladder cancer. Among the 18 patients with primary pelvic tumors were three patients with chordomas, six with bone tumors (osteosarcoma chondrosarcoma, grade III giant cell tumor), and nine with soft tissue tumors. All required major resection of the sacrum or pelvic side walls, and one half had an additional exenterative procedure. The overall mortality rate was 7.9%. Long-term estimated survival was 24% in patients having curative resection of recurrent rectal cancer, and 22.5% in 10 patients with advanced epidermoid cancer. Fifty per cent of patients with primary bone or soft tissue tumors survived from 13 to 88 months. Most patients had reasonable return of function, and were able to return to work or resume their normal previous lifestyle.     

Benzazepinone calcium channel blockers. 2. Structure-activity and drug metabolism studies leading to potent antihypertensive agents. Comparison with benzothiazepinones.

As part of a program to discover potent antihypertensive analogues of diltiazem (3a), we prepared 1-benzazepin-2-ones (4). Benzazepinones competitively displace radiolabeled diltiazem, and show the same absolute stereochemical preferences at the calcium channel receptor protein. Derivatives of 4 containing a trifluoromethyl substituent in the fused aromatic ring show potent and long-acting antihypertensive activity. Studies of the metabolism of 4 lead to the metabolically stable antihypertensive calcium channel blockers 5a and 5c. Benzazepinone 5a is a longer acting and more potent antihypertensive agent than the second generation diltiazem analogue TA-3090 (3e).     

An internet survey of 2,596 people with fibromyalgia

Background  

This study explored the feasibility of using an Internet survey of people with fibromyalgia (FM), with a view to providing information on demographics, sources of information, symptoms, functionality, perceived aggravating factors, perceived triggering events, health care utilization, management strategies, and medication use.     

Arterial endothelial function in a porcine model of early stage atherosclerotic vascular disease

Cardiovascular disease (CVD) is the leading cause of morbidity and mortality in the United States and is projected to become the leading cause of mortality in the world. Atherosclerosis is the most important single factor contributing to this disease burden. In this study, we characterize relationships between endothelial dysfunction and vascular disease in an animal model of diet-induced, early-stage atherosclerotic vascular disease. We tested the hypothesis that hypercholesterolaemia induces vascular disease and impairs endothelium-dependent relaxation (EDR) in conduit arteries of adult male Yucatan pigs. Pigs were fed a normal fat (NF) or high fat cholesterol (HFC) diet for 20-24 weeks. Results indicate that, while the HFC diet did not alter EDR in femoral or brachial arteries, EDR was significantly decreased in both carotid and coronary arteries. Sudanophilic fatty streaks were significantly present in the abdominal aorta and common carotid artery. Histopathology revealed increased intima-media thickness (IMT) and foam cell accumulation in Stary Stage I-III lesions in the abdominal aorta, common carotid artery and femoral arteries. In the coronary arteries, the accumulation of foam cells in Stary Stage I and II lesions resulted in a trend for increased IMT. There was no evidence of vascular disease in the brachial arteries. These results indicate that early stages of CVD (Stary Stage I-III) precede decreases in EDR induced by HFC diet, because femoral arteries exhibited foam cell accumulation and an increased IMT but no change in endothelial function.