Cardiac involvement in neuromuscular diseases

Many neuromuscular disorders involve the heart, occasionally with overt clinical disease. Muscular dystrophies (dystrophinopathies, limb girdle muscular dystrophy, Emery-Dreifuss muscular dystrophy, Steinert's myotonic dystrophy), congenital myopathies, inflammatory myopathies and metabolic diseases (glycogenosis, periodic paralysis, mitochondrial diseases) may produce dilated or hypertrophic cardiomyopathy and heart rhythm or conduction disturbances. Furthermore the heart is commonly involved in some hereditary and degenerative diseases (Friedreich's ataxia and Kugelberg-Welander syndrome) and acquired (Guillain-Barré syndrome) or inherited (Refsum's disease and Charcot-Marie-Tooth syndrome) polyneuropathies. A cardiologist's high clinical suspicion and a simple but systematic skeletal muscle and peripheral nerve investigation, including muscle enzymes quantification, neurophysiological study and muscle biopsy, are necessary for an accurate diagnosis. In selected patients, more sophisticated biochemical and genetic analysis will be necessary. In most cases, endomyocardial biopsy is not essential for the diagnosis.     

Fast heuristic principle for spare capacity placement inmesh-restorable SONET/SDH transport networks

The authors propose a useful new insight into the problem of spare capacity design for mesh-restorable networks. A straightforward heuristic of average case complexity O(S) based on the new principle is within 21% (13% on average) of the minimum capacity and runs 8.5-80 times faster than the optimal integer program (IF). Application to evaluate benefits from six new span addition candidates in a 53 node, 81 span network yielded the correct rank-order of merit 650 times faster than optimal methods. This contributes to a practical assault on the problem of finding the globally best single or multiple new span additions in the evolution of large transport networks     

Dependence of aortic pulse wave assessments on behavioural state in normal term fetus

To investigate any influence of behavioural states on the pulse waves in the descending aorta, 21 human fetuses were studied in utero in uncomplicated gestation at the age of 36-41 weeks. The fetal behavioural states were identified using two real-time scanners and one cardiotocograph. The aortic waveform data and pulse wave velocity (PWV) were recorded by means of two double phase-locking echo-trackers. The PWV of the fetal aorta was significantly lower in fetal behavioural state (FBS) 2F than in FBS 1F, when the fetus was apnoeic, and was also reduced in both states during fetal breathing. The calculated pulse pressure showed the same trend as the PWV. The fetal aortic end diastolic diameter and the pulse amplitude did not alter, when the two states changed. Our data suggest that the central haemodynamics in term fetuses are independently influenced by their behavioural state as well as by fetal breathing. The study of pulse waves in the fetal aorta should preferably be performed during apnoea in state 1F, when neither gross body movements nor breathing movements disturb the recording.     

The safety and efficacy of transcatheter arterial chemoembolization in the treatment of patients with hepatocellular carcinoma and main portal vein obstruction. A prospective controlled study

BACKGROUND: Transcatheter arterial chemoembolization (TACE) has been contra-indicated for the treatment of patients with hepatocellular carcinoma (HCC) and main portal vein (MPV) obstruction because of the potential risk of hepatic insufficiency resulting from ischemia after TACE. The current controlled study was undertaken to assess the safety, efficacy, and predictive factors of favorable response to TACE in patients with HCC and MPV obstruction with good hepatic function and adequate collateral circulation around the MPV. METHODS: Of a total of 47 patients, 31 were treated with TACE, and 16 who declined the procedures were untreated controls. Thirty-six patients (77%) had diffuse-type HCC and 11 (23%) had nodular-type HCC. During the first week after TACE immediate postprocedural complications were evaluated, and the development of hepatic insufficiency as a late complication was assessed at the end of the fourth week. The cumulative survival rate was estimated by the Kaplan-Meier method, and predictors of better prognosis were obtained by univariate and multivariate analyses. RESULTS: Although no patients showed clinical evidence of hepatic insufficiency as an immediate complication, transient fever and abdominal pain were common. Progressive hepatic insufficiency developed at the fourth week; however, there was no difference between the treated and untreated groups. The survival time of treated patients was statistically no longer than that of untreated patients. In the univariate analysis, tumor type and size, the pattern of iodized oil uptake in the tumor, and the presence of iodized oil uptake in the tumor thrombi at the MPV significantly influenced the prognosis. Tumor type, whether treated or not, was the most important prognostic factor patients with nodular-type HCC had significantly longer survival time (median, 11 months) than those with diffuse-type HCC (median, 4 months). Regarding the efficacy of TACE, there was no statistical difference in survival between treated and untreated diffuse-type HCC patients. In comparison, with nodular-type HCC it seemed that survival time was longer for TACE-treated patients (median, 30 months) than for untreated patients (median, 7 months). CONCLUSIONS: TACE may be a safe modality for the treatment of patients with HCC and MPV obstruction, provided that the patients have good hepatic function and collateral circulation around the MPV. However, TACE was not efficacious in the treatment of diffuse-type HCC. The authors recommend TACE for treating nodular-type HCC because of the potential benefit of prolonged survival.     

STARCH DEGRADATION IN ENDOSPERMS OF DEVELOPING BARLEY KERNELS*

Shortly after anthesis, empty endosperm cells adjacent to the embryo were crushed between the developing embryo and endosperm tissues to form the crushed cell layer. Starch granules in cells adjacent to this layer were hydrolysed and the empty cells were added to the crushed layer. In this way, the crushed layer increased in thickness throughout kernel development. α-Amylase 2 was detected in the crushed cell layer region of barley endosperms during the period that starch granule hydrolysis was occurring.     

Expression and function of a recombinant endothelial nitric oxide synthase gene in porcine coronary arteries

PURPOSE: To evaluate the influence of prognostic factors in postoperative radiotherapy of NSCLC with special emphasis on the time interval between surgery and start of radiotherapy. METHODS AND MATERIALS: Between January 1976 and December 1993, 340 cases were treated and retrospectively analyzed meeting the following criteria: complete follow-up; complete staging information including pathological confirmation of resection status; maximum interval between surgery (SX) and radiotherapy (RT) of 12 weeks (median 36 days, range 18 to 84 days); minimum dose of 50 Gy (R0), and maximum dose of 70 Gy (R2). Two hundred thirty patients (68%) had N2 disease; 228 patients were completely resected (R0). One hundred six (31%) had adenocarcinoma, 172 (51%) squamous cell carcinoma. RESULTS: In univariate analysis, Karnofsky performance status (90+ >60-80%; p = 0.019 log rank), resection status stratified for nodal disease (R+ 相似文献   

Random shuttle mutagenesis: gonococcal mutants deficient in pilin antigenic variation

Shuttle mutagenesis has been adapted to randomly mutate the genome of Neisseria gonorrhoeae (gonococcus; Gc). A size-restricted plasmid library of Gc strain FA1090 was mutated with the mini-transposon mTnEGNS. Radomness was tested by checking for transposon insertion bias between vector and insert DNA, Gc transformation efficiency of individual mutated clones, and representation of unique clones before and after Gc transformation with a mutated pool of DNA. Mutants created by random shuttle mutagenesis were screened, using a colony-based polymerase chain reaction assay, for the ability to undergo pilin antigenic variation. Out of 8,064 mutants screened, 22 unique transposon insertion mutants were found to be antigenic variation deficient (Avd). The Avd mutants were separated into five types according to recombination defect-associated phenotypes, including colony growth, natural DNA transformation competence, and repair of DNA damage caused by ultraviolet radiation.     

Extracorporeal photochemotherapy (photopheresis) induces apoptosis in lymphocytes: a possible mechanism of action of PUVA therapy

The mechanism of action of psoralen plus UVA (PUVA) and photopheresis is not entirely understood. These therapies are assumed to be immunomodulating partly by gradually decreasing leukocyte viability. We investigated whether this delayed form of cell death was due to apoptosis. Untreated and treated (PUVA exposed) leukocytes obtained from six patients with systemic sclerosis and (untreated) leukocytes from healthy control individuals were studied. Qualitative gel electrophoresis and quantitative in situ nick translation analysis of DNA fragmentation was performed. Apoptosis of the treated cells did occur (gel electrophoresis) after 24 h. At t = 0 h, immediately after exposure to PUVA, there was no evidence of DNA fragmentation in the treated cells. The percentage of treated cells undergoing apoptosis was 20-55% at t = 24 h (in situ nick translation). The untreated leukocytes of the patients and the healthy individuals showed no distinctive rise in apoptotic cells. Apoptosis of the leukocytes after PUVA or photopheresis treatment might be a mechanism of action and might explain the therapeutic response.     

Spatial and temporal expression of angiogenic molecules during tumor growth and progression

The growth and metastasis of cancer directly correlates with tumor angiogenesis. A better understanding of the expression of regulatory factors controlling angiogenesis is important in exploiting this process therapeutically. Our present study demonstrates that small tumors (3-4 mm in diameter) express more basic fibroblast growth factor (bFGF) and interleukin 8 (IL-8) than large tumors (> 10 mm in diameter), whereas more vascular endothelial growth factor (VEGF) is expressed in large tumors. Immunostaining showed a heterogeneous distribution of angiogenic factors within the tumor; expression of bFGF and IL-8 was highest on the periphery of a large tumor, where cell division is maximum. VEGF expression was higher in the center of the tumor. In vitro studies demonstrated that sparse cultures of tumor cells expressed higher levels of bFGF and IL-8 than confluent cultures. In contrast, the expression of bFGF and IL-8 was not diminished in tumor cells growing on confluent monolayers of normal cells. VEGF expression was upregulated by cell density irrespective of contact with tumor cells or normal cells. These results demonstrate that the expression of different angiogenic factors in tumor cells can be regulated by their proximity to other tumor cells or host cells.