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991.
992.
KV Shah TD Kessis F Shah JW Gupta D Shibata RW Jones 《Canadian Metallurgical Quarterly》1996,15(2):127-130
Unstable expansion of the CTG repeats in the 3' untranslated region encoding a member of the protein kinase family in the q13.3 band on chromosome 19 is a mutation specific for myotonic dystrophy. To examine the correlation between clinical expression and CTG trinucleotide repeat length, we carried out Southern blot analysis in a family with myotonic dystrophy. In this pedigree, the expanded CTG repeats were transmitted maternally. The mother had three female children. The mother had about 200 CTG repeats, and the number of repeats for each child was about 800, 1500 and 1600 in birth order. The mother and the patient with 800 repeats were unaware of muscle weakness or myotonia. Symptoms were present from age 3 years in the patient with 1500 repeats and from birth in the one with 1600 repeats. Although the mother menstruated regularly, the patients with 800 and 1500 repeats both menstruated irregularly, and the one with 1600 repeats has never menstruated. The age of onset and severity of the disease were correlated with the size of the expanded repeats. Endocrinological studies revealed that the basal levels of the gonadotropins, PRL and E2 were within normal range, and a pituitary response to LHRH was observed. These data suggest that the amenorrhea and menstrual irregularities were caused by a suprahypophyseal dysfunction. When expanded CTG repeats are transmitted maternally, abnormal products resulting from the metabolic disturbance in the affected mother may harm the fetus in utero. A heterozygous fetus, who has more CTG repeats, may be unable to metabolize the pathologic products sufficiently and therefore may become more severely affected. This may explain the exclusive maternal transmission of congenital myotonic dystrophy. 相似文献
993.
M Schietroma A Risetti F Carlei MV Santirocco F Muzi G Maggi C De Santis CL Recchia D Iannucci R Rampa M Simi 《Canadian Metallurgical Quarterly》1996,48(4):43-46
Adhesions have been suggested as a possible cause of chronic abdominal pain, but the reports of their etiological role conflict. Lysis of adhesions has been proposed as the therapeutic modality of choice, although the reports of success are controversial. The aim our prospective study was to determine whether laparoscopic adhesiolysis ameliorates chronic abdominal pain in patients with abdominal adhesions. Forty-one patients with chronic abdominal pain lasting for more than 6 months, but with no abnormal findings other than adhesions found at laparoscopy, underwent laparoscopic adhesiolysis. 37 patients (90.2%) were available for follow-up after a median time interval of 18 months (range: 12-41 months). Twenty-two patients (59.4%) were free from abdominal pain and 9 (24.3%) patients reported significant amelioration of their pain. Six (16.2%) patients had no amelioration. In conclusion the laparoscopy is an effective tool for the evaluation of patients with chronic abdominal pain, and laparoscopic adhesiolysis cures of ameliorates chronic abdominal pain in more than 80% of patients. 相似文献
994.
M Tanji F Iwaya T Igari T Ogawa K Takahashi S Hoshino 《Canadian Metallurgical Quarterly》1996,44(12):2146-2150
This report describes a 5-year-old girl with congenital tricuspid regurgitation associated with an atrial septal defect and peripheral pulmonary stenosis. The girl was diagnosed with the heart murmur at birth and recently developed the cardiomegaly. Cardiac echocardiography and catheterization showed severe tricuspid regurgitation, an atrial septal defect of the secundum type and peripheral pulmonary stenosis. In the operative findings, the tricuspid annulus was dilated to 33 mm in diameter, and leaflets were attached normally to the antomic annulus. There was a large cleft of the anterior leaflet of the tricuspid valve. Suture of the cleft and annuloplasty of the tricuspid valve, suture closure of the atrial septal defect and patch dilatation of peripheral pulmonary stenosis were successfully performed. Including this case, 19 other cases with congenital tricuspid regurgitation undergoing surgery were reported to date. 相似文献
995.
996.
Mason G.M. Hamilton D.C. Walpole P.H. Heuerman K.F. James T.L. Lennard M.H. Mazur J.E. 《Geoscience and Remote Sensing, IEEE Transactions on》1993,31(3):549-556
The SAMPEX (Solar, Anomalous, and Magnetospheric Particle Explorer) LEICA instrument is designed to measure ~0.5-5-MeV/nucleon solar and magnetospheric ions over the range from He-Ni. The instrument is a time-of-flight (TOF) mass spectrometer, which measures particle TOF over an ~0.5-m path and the residual energy deposited in an array of Si solid state detectors. Large-area microchannel plates are used, resulting in a large geometrical factor for the instrument (0.6 cm2 sr), which is essential for accurate compositional measurements in small solar flares and in studies of precipitating magnetospheric heavy ions 相似文献
997.
EA Scodeller SG Tisminetzky F Porro M Schiappacassi A De Rossi L Chiecco-Bianchi FE Baralle 《Canadian Metallurgical Quarterly》1995,13(13):1233-1239
The principal neutralizing domain, IGPGRAF sequence, from the V3-loop of HIV-1 was inserted in two positions on the surface of the protein that makes up the capside shell of the insect Flock House Virus. The hybrid proteins were expressed in insect cells via recombinant baculoviruses. Three different hybrids were used as immunogens: two with a single copy of the insert in different positions of the carrier protein and a third with two copies of the insert at the same positions as before. All hybrid proteins induced strong and broad specific immune response in guinea pigs against different V3-loop sequences. However, only one of the hybrid proteins was able to induce a strong neutralizing response against MN and IIIB HIV-1 isolates. Our results demonstrate that a very short peptide sequence of HIV-1 can constitute a valuable immunogen able to induce a neutralizing response if presented to the immune system in the context of the FHV capsomer structure. 相似文献
998.
Sabate J.A. Jovanovic M.M. Lee F.C. Gean R.T. 《Industrial Electronics, IEEE Transactions on》1995,42(1):63-71
The analysis and design of an LCC resonant inverter for a 20 kHz AC distributed power system are presented. Several resonant converter topologies are assessed to determine their suitability for high efficiency power conversion, under resistive and reactive loads. Two LCC-resonant inverter designs were implemented. One with all switches operating with zero voltage switching (ZVS), and another with two switches operating with ZVS and two switches with zero current switching (ZCS). The experimental results are presented along with a performance comparison of the two versions 相似文献
999.
1000.