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).     

Retention of "safe" blood donors. The Retrovirus Epidemiology Donor Study

BACKGROUND: There are obvious advantages to increasing donor retention. However, for reasons of blood safety, certain donors may, in fact, be more desirable to retain than others. “Safe” donors are defined as those who provided a blood donation that was negative on all laboratory screening tests and who subsequently reported no behavioral risks in response to an anonymous survey. This study identifies the most important factors affecting the intention of “safe” donors to provide another donation. STUDY DESIGN AND METHODS: An anonymous survey asking about donation history, sexual history, injecting drug use, and recent donation experience was mailed to 50,162 randomly selected allogeneic donors (including directed donors) who gave blood from April through July or from October through December 1993 at one of the five United States blood centers participating in the Retrovirus Epidemiology Donor Study. Before mailing, questionnaires were coded to designate donors with nonreactive laboratory screening tests at their most recent donation. RESULTS: A total of 34,726 donors (69%) responded, with substantially higher response among repeat donors. According to reported intentions only, the vast majority of “safe” donors indicated a high likelihood of donating again within the next 12 months. Only 3.4 percent reported a low likelihood of donating again. A comparison of those likely to return and those unlikely to return reveals significant differences in demographics and in ratings of the donation experience. A higher proportion of those unlikely to return were first-time donors, minority-group donors, and donors with less education. The highest projected loss among “safe” donors was seen for those who gave a fair to poor assessment of their treatment by blood center staff or of their physical well-being during or after donating. CONCLUSION: These data suggest that efforts to improve donors' perceptions of their donation experience, as well as attention to the physical effects of blood donation, may aid in the retention of both repeat and first-time donors.     

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).