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The present study documents the prevalence of deficits in the ability to carry out a variety of activities of daily living in early Huntington's disease (HD), along with the associated neuropsychological and motor deficits. Eighty patients with HD were assessed with the Huntington's Disease-Activities of Daily Living Questionnaire (HD-ADL). Sixty-seven patients also completed a comprehensive assessment of cognitive and voluntary motor functioning and chorea. The latter measures were correlated with HD-ADL total score and with most HD-ADL items, but not with those items dealing with marital and family relationship adjustment. Findings suggest that psychomotor speed and the ability to regulate attention may be particularly important determinants of everyday functioning in mild HD. Consistent with previously reported observations, this appears to be true even after accounting for individual differences in the severity of chorea and voluntary motor impairment.  相似文献   

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Cholelithiasis is a common disease in women and can lead to serious complications. At Wilford Hall USAF Medical Center, we have performed 21 cholecystectomies at the time of abdominal hysterectomy. All patients had been asymptomatic with regard to gallbladder disease, but showed either preoperative or intraoperative evidence of gallbladder disease. The mean surgical time was 3.3 hours, mean blood loss was 474 mL, and only one patient had febrile morbidity (> 38.3 degrees C). Because of the low morbidity, we recommend that this combined surgical approach be considered by both the gynecologist and general surgeon.  相似文献   

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We report a study of 41 radical hysterectomies performed through laparoscopic approach for carcinoma of the cervix uteri. According to the FIGO staging, the tumors were classified as 12 stages Ia2, 24 stages Ib, 4 stages IIa and 1 stage IIb. 17 patients were treated by exclusive surgical procedure. 24 patients received a combination of radiation therapy and surgery 2 patients had a tumor of the cervical stump after subtotal hysterectomy. The mean duration of the procedure was 270 minutes. The post-op stay was 6.5 days. There was no major operative and postoperative complication. Only one patient required a blood transfusion. The intravenous pyelogram control was correct in all cases. At this time, with a 4 to 76 months follow-up, no recurrence was observed. This experience suggests the faisability of the laparoscopic radical hysterectomy. This approach seems to be convenient in young and non obese women with a tumor less than 4 cm in the greatest diameter.  相似文献   

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Percutaneous nephrolithotomy (PCNL) has radically changed the treatment of renal stones. The indications for this technique have been modified by the development and refinement of extracorporeal lithotripsy (ECL). The authors present their experience of 390 PCNL performed between 1984 and 1991, for solitary stones in 75% of cases. There were only 11 cases of failed puncture (3.8%). The overall morbidity was 18% with only 4.4% of major complications, i.e. life threatening or requiring reoperation. There were two deaths (0.5%). Stones were completely eliminated in 80.25% of patients, after a second operation (PCNL or other technique) in 32 cases (11%). 45 staghorn calculi were treated with 11% of complications and a 51% complete cure rate. The mean hospital stay was 6 days (2 to 30). PCNL is a safe and reliable technique. Its indications have decreased (6.5% of our patients treated for renal stones), but it still retains a place in the therapeutic approach to patients with renal stones.  相似文献   

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From 1978 to 1992, 121 cases of postinfarction left ventricular aneurysm (99 males, 22 females, mean age 60 years) were operated on. The authors insist on a high rate of clinical arhythmogenicity (31.4%) and associated mechanical complications (21%). 76% of patients were in functional NYHA class III or IV. Resection was performed in 90% of patients, plication in 10%. 58% underwent coronary artery bypass grafting (1.7 graft/patient), 16% encircling ventriculotomy, 8% mitral valve replacement and 13% closure of ventricular septal defect. Operative mortality was 14.9% (10% when other mechanical complications where excluded). 5-year survival is 67.9%. Late cardiac deaths are as follow: left ventricular failure (1.8% A/P), Sudden death (1.4% A/P), Myocardial infarction (0.6% A/P). 82% of survivals are in functional NYHA class I or II. Only functional class NYHA III or IV is predictive of late death. We conclude that postinfarction left ventricular aneurysm remains a high risk complication especially when associated with other mechanical complications. When arhythmogenicity is present we suggest rhythmologic surgery and in all cases, complete revascularization.  相似文献   

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The Miles assay for vascular permeability has high intra- and interassay coefficients of variation (CVs). Quantification, usually by dye extraction and spectrophotometry, is time consuming. In this study, quantification by this means was compared with image analysis using the Olympus CUE-2 Image Analyzer (version 4). The test substance was recombinant human vascular permeability factor (rhVPF). The quantification process took approximately 10 min with image analysis. Formamide extraction and spectrophotometry required 1 hr of preparation, 4-6 days of incubation, and 1 hr for filtration and spectrophotometry. Between assay CVs ranged from 0 to 30% for spectrophotometry, but were all < 10% for image analysis. The sensitivity (2SD above the negative control mean) of the image analysis approach was 64 +/- 25 ng/mL, whereas for spectrophotometry it was 65 +/- 29 ng/mL. Interanimal CVs for rhVPF at 200 and 1000 ng/mL were 15% and 26% when assessed by spectrophotometry and 7% and 22%, respectively, by image analysis. The R2 value for the correlation of image analysis with spectrophotometry was 81.4%. Test substances injected close to the spine evoked a greater permeability response than those injected laterally: at 200 ng/mL p = 0.005, at 1000 ng/mL p = 0.1 (unpaired t tests).  相似文献   

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The authors report a series of 40 cases of genitourinary tuberculosis diagnosed and treated in the department of urology "B" of Avicenne hospital over a 7-year period. The objective of this study is to define the various diagnostic and therapeutic aspects of this disease. The patients were predominantly males (62.5%) with a mean age of 40 years. 25% of cases reported a history of extra-urinary tuberculosis. The very polymorphous clinical presentation is dominated by signs of cystitis (45%). Intravenous urography is frequently suggestive of the diagnosis based on the appearance and multiplicity of the lesions. The radiological lesions most frequently encountered were silent kidney (19 cases) and small tuberculous bladder (11 cases). The definitive diagnosis was established by pathological examination in 38 cases (biopsies, operative specimens, prostatic resection chips) and/or by demonstration of AFB in 2 cases (urine, pus). Tuberculostatic treatment was administered to all patients, either alone (5 cases) or, more usually, in combination with surgical and/or endo-urological treatment (35 cases), reflecting the magnitude and severity of the destructive and scar lesions.  相似文献   

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Natural infections with the lancet fluke (Dicrocoelium dendriticum) were detected in a group of seven diseased or dead/euthanized South American Camelids (five Llamas, two Alpacas) from Switzerland and Southern Germany. Clinical findings in almost all the animals revealed an acute decline of general condition followed by recumbency, decreased body temperature and a varying degree of anaemia. Concurrently, all animals showed an average to poor nutritional status. All liver enzyme activities analysed in serum biochemistry conformed to the reference values and therefore offered no diagnostic clues for this disease. Necropsy however, disclosed major alterations in the liver in the form of cirrhosis, abscesses, granulomas, and a massive infestation with D. dendriticum. The coprological investigations performed at the outset of the examinations revealed eggs of the lancet fluke in only two animals. This suggests that clinical findings alone permit at best only a provisional diagnosis. Repeated coprologic follow-ups showed that the presence of eggs of D. dendriticum can be diagnosed accurately and that clinical signs appear with an excretion rate above 1000 eggs per gram faeces (EpG). In these cases, praziquantel in a single dose of 50 mg/kg per os was given. This treatment was well tolerated and achieved a quite acceptable 90% reduction of eggs in the faeces.  相似文献   

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OBJECTIVE: To outline the most appropriate treatment of descending necrotizing mediastinitis. MATERIALS AND METHODS: Three adult patients had mediastinitis occurring from a descending odontogenic infection in one case and oropharynx infection in two cases. All patients underwent extensive surgical debridement and a cervicomediastinal drainage through a cervical incision. All patients survived. DISCUSSION: Soft-tissue infections of the neck with mediastinitis demand early diagnosis aided by CT scan in order to decrease their threat to life. Extensive surgical debridement and a cervico mediastinal drainage through a cervical incision is adequate when mediastinitis is limited to the upper mediastinum. Thoracotomy has to be performed when the process spread below the carina. Patients must be treated initially aggressively with the aid of multidisciplinary support team (intensive care physicians, thoracic and head and neck surgeons). Early surgery must treat both neck and mediastin. While usually associated with greater than 40% mortality, all the patients in this series survived.  相似文献   

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OBJECTIVE: The authors report an exceptional site of tuberculous osteo-articular infection which must be diagnosed before the destructive stage. OBSERVATIONS: Case 1 : a 21 years old woman presented an inflammatory pubic pain after a trauma with weight loss of 4 kgs in 3 weeks. She presented also 2 satellite inguinal nodes. Erythrocyte sedimentation rate (ESR) was elevated, X-rays showed an important osteolysis of the left ischio-pubic rami, tuberculous skin test (TST) was positive, mycobacterium tuberculosis (MT) could not be found neither in sputtum nor in urine but the node biopsy showed the specific features of tuberculosis. Evolution under a 6 months antibiotic treatment was good. Case 2 : a 19 years old woman, with history of tuberculous contagion, presented in April 1996 cervical nodes and a month later inflammatory pubic and knee pain with weight loss and vesperal sudation. ESR was elevated, TST was phlyctenular, MT searching and HIV serology were negative. X rays showed irregular osteolysis of the pubic symphysis. Scintigraphy showed an increased fixation of pubis and left knee. Cervical nodes biopsy diagnosed tuberculosis. Evolution was good under a 6 months antibiotic treatment. DISCUSSION: Many factors can favorize the development of a pubic tuberculosis and are similar for all forms of tuberculous osteo-articular infection (trauma and contagion in our cases). Radiological features, characterized by a slow evolution, are note specific. Diagnostic confirmation must be bacteriologic or pathologic, and if possible far from the pubic foci. Any traumatic medical procedure has to be avoided because of painful outcome and local risk. Evolution under specific treatment, even of short course (6 months), is sufficient for a good outcome. CONCLUSION: One must think to pubic tuberculous osteo-arthritis in any pubic pain even if it is post-traumatic especially, in endemic countries of tuberculosis.  相似文献   

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It has been considered that thromboxane A2 (TXA2) is involved in the development of bronchial hyperresponsiveness (BHR), a characteristic feature of asthma. To ensure the involvement of TXA2 in BHR of asthma, effects of a 1-week treatment with two orally active TXA2 antagonists, BAY u 3405 and S-1452, on BHR were examined in 10 and 13 patients with stable asthma, respectively, in two consecutive double-blinded, randomized, placebo-controlled, two-phase crossover studies. Provocative concentration of methacholine causing a 20% fall in FEV1 (PC20-FEV1) with BAY u 3405 (0.78 (GSEM, 1.50) mg/ml) was significantly greater than the value with placebo (0.65 (GSEM, 1.46) mg/ml) (ratio 1.23 times, 95% CI 1.01 to 1.46: P = 0.0401). PC20-FEV1 was also significantly increased with S-1452 (0.43 (GSEM, 1.39) mg/ml) compared with placebo (0.29 (GSEM, 1.27) mg/ml) (ratio 1.75 times, 95% CI 1.05 to 2.45: P = 0.0189). Baseline pulmonary function was not altered by these treatments. These results may ensure that TXA2 is significantly involved in the BHR of asthma while the degree of contribution may be small.  相似文献   

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Spontaneous coronary dissection is a rare and still poorly understood pathological disease entity; the authors report two cases in which the diagnosis was reached in the context of incipient necrosis in young patients. A review of the literature reveals the main characteristics of this problem: the clinical aspect is generally that of acute coronary failure with the constitution of an infarction. The subjects are young with no risk factor but some cases of combination with a disease affecting the elastic tissue have been described. Female gender and the post-partum phase are promoting factors. Diagnosis was long established by means of autopsy but can now be based on coronary angiography. Histologically, the lesion is a hematoma which dissects the arterial wall between the internal two thirds and external third of the media. The main etiological hypothesis is acquired or congenital fragility of the media, which promotes the formation of a dissecting hematoma, however the triggering phenomenon remains poorly understood.  相似文献   

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The authors report 22 observations of patients showing an intra-hepatic cholestasis following a traumatism. The frequency of this complication amounted to about 1.5 p. 100. Among the mechanisms of this cholestasis some factors were kept in mind: 1. the severity of the traumatism; 2. the surgical act; 3. the use of massive transfusions, the polyvisceral complications of the traumatic shock, the infectious complications. The pathophysiology of this cholestasis seems to be in relation to an increase of the production of the conjugated bilirubin. This production is associated with a decrease of the biliary excretion depending essentially on hepatic intracellular injury, secondary to: the hemorragic shock, the anoxia, the surgical intervention, the infection. From these different factors, they draw practical conclusions as much in the diagnosis of cholestasis as in the therapeutics.  相似文献   

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