The syndrome of multisynostotic osteodysgenesis with long-bone fractures

Described here are two patients with a newly recognized syndrome of bone and cartilage maldevelopment which, we believe, results from a single embryonic defect, probably of genetic origin. The cardinal manifestations of this association are craniosynostosis, radiohumeral synostosis (RHS), and femoral bowing. Specific secondary defects include midface hypoplasia with characteristic facial appearance and ears, neonatal femoral fractures, and multiple minor anomalies of the limbs. Though the differential diagnosis includes such disorders as the campomelic syndrome, osteogenesis imperfecta (OI) and certain of acrocephalosyndactyly syndromes, the unique combination of clinical and radiographic abnormalities allows ready differentiation. The cause cannot be determined from these two cases.     

Needle biopsy of renal allografts: comparison of two techniques

Two techniques for renal allograft biopsy were retrospectively evaluated to compare relative safety and efficacy. After ultrasound (US) localization of the kidney and biopsy with a hand-held 14-gauge cutting needle, an adequate specimen was obtained in 74 of 77 cases (96%). Major complications occurred in six of these 77 cases (8%). One hundred four biopsies were performed by using a smaller 18-gauge cutting needle with a spring-loaded biopsy "gun" and real-time US guidance. With this newer technique, specimens adequate for diagnosis were obtained in 99 biopsies (95%). There was a single major complication with this technique (1%). The 18-gauge needle with real-time US guidance yields comparably adequate specimens with a lower frequency of complications.     

Electrochemotherapy: an emerging drug delivery method for the treatment of cancer

The combined treatment consisting of a chemotherapeutic agent and pulsed electric fields has been termed electrochemotherapy. This relatively new treatment modality relies on the physical effects of locally applied electric fields to destabilize cell membranes in the presence of a drug. Membrane destabilization, electroporation, allows increased movement of molecules into the cytosol. Thus, the pulses are used to locally deliver drugs to the interior of cells. This type of treatment has principally been used to deliver bleomycin to tumor cells in vitro and in vivo. Marked antitumor effects have been reported in preclinical studies. In addition, electrochemotherapy clinical trials have been conducted for the treatment of head and neck squamous cell carcinoma, melanoma, and basal cell carcinoma. Objective response rates ranging from 72 to 100% have been reported from these trials. A review of the preclinical and clinical data for this novel drug delivery method is presented.     

Effectiveness of interactive videodisc instruction for the continuing education of paramedics

OBJECTIVES. To evaluate the effectiveness of interactive videodisc (IVD) instruction of paramedics through the use of computer analysis of trip sheets. DESIGN/SETTING. Prospective, controlled, in an urban 9-1-1, paramedic, emergency medical services (EMS) system with total call volume of 62,000/year; 15,000 advanced life support (ALS). INTERVENTIONS. All 150 paramedics in the system received eight hours of IVD instruction covering five subject areas: 1) airway; 2) head/cervical trauma; 3) chest; 4) shock; and 5) cardiac arrest. Trip sheets from 9,943 runs in the pre-IVD period were subjected to computer analysis, and a compliance score was generated using previously developed algorithms that assigned a weight to each omission and commission. After a nine-month IVD training period, 4,303 cases were collected and analyzed in the post-IVD period. Statistical analyses were made using "Student's" t-test and Chi-square with alpha set at 0.05. EXCLUSIONS. Only those records of adult patients who fit one of the five protocols were eligible for computer analysis. Of the 9,943 cases in the pre-IVD group, 480 (4.8%) were excluded, all due to inadequate data recording by the paramedics. A statistically similar portion, 233 (5.4%) of the 4,303 post-IVD instruction cases were excluded (p = .15). RESULTS. Overall the mean compliance score of the pre-IVD group was 0.65 +/-0.19 (+/-SD). The post-IVD group score was 0.65 +/-0.19 (p = 0.99). Analysis of scores for each algorithm also showed no significant differences. This study had an observed power of .94 to detect a difference in compliance as small as 0.030. CONCLUSION.(ABSTRACT TRUNCATED AT 250 WORDS)     

Osteocalcin levels in chronic osteomyelitis

Osteocalcin is a vitamin K-dependent bone protein synthesized by osteoblasts. In generalized bone disorders serum osteocalcin correlates with osteoblast activity. Bone resorption and new bone formation occur in chronic osteomyelitis, dependent on the level of inflammatory activity. In 17 patients with active chronic osteomyelitis undergoing surgery, the serum levels of osteocalcin, alkaline phosphatase and C-reactive protein were measured before and after treatment. The osteocalcin levels were within the normal range preoperatively (10.8 ±11.0 g/l), in the early postoperative period, and at discharge. It is therefore not a helpful marker in the clinical management of this condition.     

Pharmacokinetics of Nisoldipine Coat--Core Formulation in Subjects with Liver Cirrhosis

The pharmacokinetics of a controlled-release formulation (coat--core) of the calcium channel blocker nisoldipine was investigated in eight subjects with biopsy-proved liver cirrhosis and eight healthy subjects. In Stage I, subjects received a single 10-mg dose to determine if this dose would be safely tolerated in the subjects with cirrhosis. Because all subjects in both groups tolerated the dose without difficulty, all were continued to Stage II. In Stage II, subjects received a once-daily dose of 10-mg coat-core tablets for 7 days. Serial plasma samples were assayed for nisoldipine in both stages. The C(max) and AUC of nisoldipine were approximately fourfold to fivefold higher (p < 0.01) in subjects with cirrhosis as compared to healthy subjects; however, there was overlap in the range of pharmacokinetic parameters between the two groups. The accumulation factor following multiple dosing was similar in both groups. Results suggest that nisoldipine dose should be optimized by monitoring of a pharmacodynamic end point, such as effect on blood pressure. It is likely that dose requirements for patients with liver disease will be lower.     

The cross cultural context of obesity: an INCLEN multicentre collaborative study

Associations between obesity and cardiovascular disease have been documented in the Developed World and have been linked with levels of 'development' in Developing World countries. This study aimed to explore the sociocultural influences on attitudes towards obesity, physical activity and dietary change using a four level conceptual framework (from personal to societal influences). Focus group discussions were conducted in: India, Indonesia, Cameroon, Egypt and Australia. The results of these discussions were analysed to reveal emergent themes. The interpretation of the qualitative data emphasised the need to incorporate influences beyond the individual in efforts to reverse or prevent obesity related disease.     

Autonomy in residential facilities and community functioning of adults with mental retardation

Over a 3-year period, we examined whether the autonomy of 58 adults living in residences for people with developmental disabilities was associated with their adaptive behavior and community integration. Degree of resident autonomy included measures of opportunities for choice-making and level of involvement in policymaking. All of the participants lived in nursing homes at baseline and in residential settings for people with developmental disabilities at follow-up. Results indicated that opportunities for autonomy in residential settings were related to residents' adaptive behavior and community integration. More opportunities for choice-making in residences was associated with greater adaptive behavior, whereas smaller residence size and more resident involvement in decision-making were associated with greater community integration.