Frontotemporal dementia. Results from "Frontotemporal Demential and Pick's Disease Conference"

At the Frontotemporal Dementia and Pick's Disease Conference, recent data concerning the epidemiology, diagnosis, and therapy of frontotemporal dementia were presented. Topics included clinical manifestations, differential diagnosis, pathologic and genetic bases of the disease, therapy, and specific problems facing patients and care givers. A consensus in the terminology of this condition was sought.     

The natural history of prenatally diagnosed conjoined twins

Background/Purpose: Accurate prenatal diagnosis of complex anatomic connections and associated anomalies has only been possible recently with the use of ultrasonography, echocardiography, and fetal magnetic resonance imaging (MRI). To assess the impact of improved antenatal diagnosis in the management and outcome of conjoined twins, the authors reviewed their experience with 14 cases. Methods: A retrospective review of prenatally diagnosed conjoined twins referred to our institution from 1996 to present was conducted. Results: In 14 sets of conjoined twins, there were 10 thoracoomphalopagus, 2 dicephalus tribrachius dipus, 1 ischiopagus, and 1 ischioomphalopagus. The earliest age at diagnosis was 9 weeks' gestation (range, 9 to 29; mean, 20). Prenatal imaging with ultrasonography, echocardiography, and ultrafast fetal MRI accurately defined the shared anatomy in all cases. Associated anomalies included cardiac malformations (11 of 14), congenital diaphragmatic hernia (4 of 14), abdominal wall defects (2 of 14), and imperforate anus (2 of 14). Three sets of twins underwent therapeutic abortion, 1 set of twins died in utero, and 10 were delivered via cesarean section at a mean gestational age of 34 weeks. There were 5 individual survivors in the series after separation (18%). In one case, in which a twin with a normal heart perfused the cotwin with a rudimentary heart, the ex utero intrapartum treatment procedure (EXIT) was utilized because of concern that the normal twin would suffer immediate cardiac decompensation at birth. This EXIT-to-separation strategy allowed prompt control of the airway and circulation before clamping the umbilical cord and optimized control over a potentially emergent situation, leading to survival of the normal cotwin. In 2 sets of twins in which each twin had a normal heart, tissue expanders were inserted before separation. Conclusions: Advances in prenatal diagnosis allow detailed, accurate evaluations of conjoined twins. Careful prenatal studies may uncover cases in which emergent separation at birth is lifesaving.     

Comparison of cyclizine and ondansetron for the prevention of postoperative nausea and vomiting in laparoscopic day-case gynaecological surgery

Seventy-four patients undergoing laparoscopic gynaecological surgery were randomly allocated to two groups receiving cyclizine 50 mg or ondansetron 4 mg at induction of anaesthesia. Anaesthetic and postoperative analgesia regimens were standardised. Approximately half of the patients in each group experienced some degree of postoperative nausea and vomiting (cyclizine, 56%; ondansetron, 54%). There was no difference between groups in respect of pre- and postdischarge incidence. Mean (SD) time to eye opening was significantly prolonged in the cyclizine group [10 (4) min vs. 8 (2) min; p < 0.001], but this had no influence on discharge times. Cyclizine and ondansetron appear equally effective in preventing postoperative nausea and vomiting but the 10-fold price differential favours cyclizine.     

Revascularization in severe ventricular dysfunction (15% < OR = LVEF < OR = 30%): a comparison of bypass grafting and percutaneous intervention

BACKGROUND: We sought to determine the optimal approach to revascularization of patients with severe left ventricular (LV) dysfunction. METHODS: We conducted a single-center observational study of 117 consecutive patients who had severe LV dysfunction (15% < OR = LV ejection fraction < OR = 30%) and underwent either coronary artery bypass grafting (CABG, n = 69) or percutaneous revascularization (n = 48) between 1992 and 1997. RESULTS: The CABG group was younger (62 versus 67 years, p = 0.026), and fewer previous bypasses (7% versus 40%, p < 0.0001) and fewer prior percutaneous revascularizations (16% versus 42%, p = 0.0019) were noted. More vessels were revascularized (3 +/- 0.8 versus 1.5 +/- 0.7, p < 0.0001), and revascularization was more complete by CABG (84% versus 48%, p < 0.0001). Morbidity and mortality at 30 days were similar, and there was no significant difference in 3-year survival (73% versus 67%), although 3-year cardiac event-free survival (52% versus 25%, p = 0.0011) and 3-year target vessel revascularization-free survival (71% versus 41%, p < 0.0001) were significantly better in the CABG group, and LV ejection fraction was significantly improved after CABG. In the subgroup of patients 65 years of age or older and those without proximal left anterior descending coronary artery lesions, significant benefit of CABG in cardiac event-free and target vessel revascularization-free survival disappeared. CONCLUSIONS: We found that in clinically selected patients with severe ventricular dysfunction, CABG compared with percutaneous revascularization achieves more complete revascularization, improved LV function, fewer cardiac events, and fewer target vessel revascularizations, but does not affect mid-term survival. A prospective controlled trial with defined criteria for treatment assignment is warranted to confirm our results regarding the two revascularization strategies in patients with severe LV dysfunction.     

Revascularization in severe ventricular dysfunction (15% ≤ LVEF ≤ 30%): a comparison of bypass grafting and percutaneous intervention

Background

We sought to determine the optimal approach to revascularization of patients with severe left ventricular (LV) dysfunction.

Methods

We conducted a single-center observational study of 117 consecutive patients who had severe LV dysfunction (15% ≤ LV ejection fraction ≤ 30%) and underwent either coronary artery bypass grafting (CABG, n = 69) or percutaneous revascularization (n = 48) between 1992 and 1997.

Results

The CABG group was younger (62 versus 67 years, p = 0.026), and fewer previous bypasses (7% versus 40%, p < 0.0001) and fewer prior percutaneous revascularizations (16% versus 42%, p = 0.0019) were noted. More vessels were revascularized (3 ± 0.8 versus 1.5 ± 0.7, p < 0.0001), and revascularization was more complete by CABG (84% versus 48%, p < 0.0001). Morbidity and mortality at 30 days were similar, and there was no significant difference in 3-year survival (73% versus 67%), although 3-year cardiac event-free survival (52% versus 25%, p = 0.0011) and 3-year target vessel revascularization-free survival (71% versus 41%, p < 0.0001) were significantly better in the CABG group, and LV ejection fraction was significantly improved after CABG. In the subgroup of patients 65 years of age or older and those without proximal left anterior descending coronary artery lesions, significant benefit of CABG in cardiac event-free and target vessel revascularization-free survival disappeared.

Conclusions

We found that in clinically selected patients with severe ventricular dysfunction, CABG compared with percutaneous revascularization achieves more complete revascularization, improved LV function, fewer cardiac events, and fewer target vessel revascularizations, but does not affect mid-term survival. A prospective controlled trial with defined criteria for treatment assignment is warranted to confirm our results regarding the two revascularization strategies in patients with severe LV dysfunction.     

Engraftment of bone marrow and fetal liver cells after in utero transplantation in MDX mice

Background/Purpose: In utero hematopoietic stem cell transplantation (IUHSCTx) has been experimentally or clinically effective only in circumstances in which there is a survival advantage for donor cells. A survival advantage exists for normal muscle cells in muscular dystrophy. Because hematopoietic and mesenchymal stem cells may have the capacity to differentiate into muscle cells, the authors hypothesized that in utero bone marrow (BM) or fetal liver (FL) stem cell transplantation may be used to treat muscular dystrophy. Methods: Time-dated 14-day-gestation fetal muscular dystrophy mice (mdx) were injected intraperitoneally with 1 to 5 [times ] 10⁶ BM or FL cells per fetus from Rosa26 donor mice (transgenic for lacZ). Four weeks after birth, peripheral blood from the pups was analyzed for hematopoietic chimerism by using fluorescence-activated cell sorting (FACS) for the Ly-9.1 marker. Chimeric mice (6 BM and 2 FL recipients) were sacrificed at 12 to 14 months of age, muscles were stained with X-gal, and analyzed by 1- to 2-[mu ]m plastic sections. Polymerase chain reaction (PCR) for lacZ was performed in other organs to determine systemic engraftment. Results: At the time of death, all animals that were chimeric at 4 weeks continued to show hematopoietic chimerism of 0.2% to 9% by FACS. Engrafted donor cells were found in multiple sections from hindlimb skeletal muscles, diaphragms, and hearts from both BM and FL recipients. These cells had incorporated into the host muscles, and their morphology was consistent with myogenic differentiation. PCR of BM, liver, spleen, thymus, kidney, and lung for lacZ was positive in multiple animals. Conclusions: IUHSCTx leads to widespread engraftment of donor cells in multiple muscle compartments of hematopoietic chimeras. The advantage for normal myocytes offered in the mdx model allows engraftment and myogenic differentiation of transplanted BM or FL cells by morphology at a relatively higher frequency in muscle relative to other tissues, without the need for host conditioning. Because muscular dystrophy now can be detected early in gestation, such a strategy may offer a future alternative in the clinical treatment of this disease. J Pediatr Surg 37:1058-1064.     

Effects of chronic dietary exposure to trace elements on banded water snakes (Nerodia fasciata)

Little currently is known about the accumulation or effects of contaminants on reptiles. To date, most studies examining reptile exposure to trace elements report tissue burdens of field-captured animals, but seldom provide insight into the dose, duration, or mode of exposure involved. For two years, we fed juvenile banded water snakes (Nerodia fasciata) prey items collected from a coal ash-contaminated site that contained elevated levels of As, Cd, Cu, Se, Sr, and V. With the exception of Cu, snakes accumulated significant concentrations of elements, usually in a dose-dependent manner. Accumulation varied significantly among liver, kidney, and gonads, and in most cases between sexes. Selenium accumulation was most notable, greatly exceeding established toxicity thresholds for other vertebrates. Despite the high concentrations of pollutants accumulated, snakes exposed to the contaminated diet survived through the study and exhibited normal food consumption, growth, condition factor, overwinter survival and mass loss, metabolic rate, and gonadosomatic index. The results of this study confirm that diet can be a significant route of exposure to trace elements in snakes and indicate that further studies on snakes are warranted to better understand their responses to contaminants.     

Retrospective study of management of uterine sarcomas at Oxford 1990-1998: role of adjuvant treatment

We report a retrospective study of 47 consecutive patients with uterine sarcoma treated at the Churchill Hospital in Oxford between 1990-1998. The mainstay of treatment was surgery with adjuvant chemotherapy and radiotherapy reserved for selected patients with early stage disease. Overall 1 and 2 year survival was 49% and 30% respectively compared with 73% and 55% in the group who received adjuvant chemotherapy/radiotherapy. Median survival was 11 months for the group as a whole compared to 32.9 months in the adjuvant therapy group. This is a retrospective review with small numbers and considerable selection bias, however, given the poor survival of patients with this disease, adjuvant treatment should be considered in future trials of patients with uterine sarcoma.     

Emergency Medical Services Outcomes Project (EMSOP) IV: pain measurement in out-of-hospital outcomes research

The purpose of the Emergency Medical Services Outcomes Project (EMSOP) is to develop a foundation and framework for out-of-hospital outcomes research. In prior work (EMSOP I), discomfort had the highest weighted score among outcome categories for the top 3 adult conditions (ie, minor trauma, respiratory distress, chest pain) and the first and third highest rankings for children's conditions (ie, minor trauma, respiratory distress). In this fourth article in the EMSOP series, we discuss issues relevant to the measurement of pain in the out-of-hospital setting, recommended pain measures that require evaluation, and implications for outcomes research focusing on pain. For adults, adolescents, and older children, 2 verbal pain-rating scales are recommended for out-of-hospital evaluation: (1) the Adjective Response Scale, which includes the responses "none," "slight," "moderate," "severe," and "agonizing," and (2) the Numeric Response Scale, which includes responses from 0 (no pain) to 100 (worst pain imaginable). The Oucher Scale, combining a visual analog scale with pictures, seems most promising for out-of-hospital use among younger children. Future research in out-of-hospital care should be conducted to determine the utility and feasibility of these measures, as well as the effectiveness of interventions for pain relief.