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1.
Transcultural understanding of a hereditary disorder. Mucopolysaccharidosis VI in a Vietnamese family 总被引:1,自引:0,他引:1
A case study of a family referred for clarification of cultural issues illustrates how a transcultural psychiatric service developed in the pediatric hospital setting can be used to advantage. A Vietnamese family with an inherited disorder. Mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome, MPS VI), resisted genetic counseling and contraception. Three out of their six children were affected, one with a fatal outcome. The transcultural consultation offered an understanding of their behavior and facilitated subsequent management. 相似文献
2.
Andrea Berger Michelle Sadeh Gabriel Tzur I Avinoam Shuper Liora Kornreich Dov Inbar Ian J Cohen Shalom Michowiz Isaac Yaniv Shlomi Constantini Eli Vakil 《Journal of the International Neuropsychological Society》2005,11(4):482-487
Cerebellar involvement in motor and non-motor sequence learning was examined with serial reaction time tasks (SRT). Our sample consisted of 8 children and adolescents who had undergone surgical removal of a benign posterior fossa tumor (PFT) during childhood. None of them had undergone chemotherapy or cranial radiation therapy (CRT). Ages ranged from 1-11 years at surgery and 9-17 years at testing. The children were tested not earlier than 2.5 years after surgery (M = 5.9 years), enabling brain plasticity and recovery of functions. Their performance was compared with a matched control sample. The PFT group was not impaired in the implicit learning of sequences, as reflected in their performance in blocks with a repeated sequence, both before and after a random block. However, in the perceptual task, their performance deteriorated more than that of the control group when a random block was introduced, suggesting that it was more difficult for the patients to respond flexibly or change their response set when encountering changing task demands. These results are in line with another study by our group on task switching with the same patients. 相似文献
3.
Tammar Kushnir Tatyana Knubovets Yacov Itzchak Uzi Eliav Menachem Sadeh Lubov Rapoport Edna Kott Gil Navon 《Magnetic resonance in medicine》1997,37(2):192-196
Myotonic dystrophy is an inherited multi-system disease. Its pathophysiology leading to muscle malfunction and damage is not well understood. 23Na NMR spectroscopy was applied here for an in vivo comparative study of the calf muscles of 7 myotonic dystrophy patients at various stages of the disease and 11 healthy volunteers. Both the total sodium content, expressed as the ratio of the 23Na and 1H water signals, and the fast transverse relaxation time, T21, determined from the triple quantum-filtered spectra, increased in correlation with the severity of the disease. The results demonstrate that 23Na NMR enables the quantitation of myotonic dystrophy progression. 相似文献
4.
Menahem Neuman Boris Friedman Avi Stein A. Ami Sidi Alexander Tsivian 《Gynecological surgery》2007,4(3):175-178
The objective of the study was to compare the clinical outcomes at the short-term follow-ups of two novel transobturator mid-urethral
sling procedures – the transobturator tape (TOT) procedure and the tension-free vaginal tape (TVT)-obturator procedure. The
study cohort consisted two groups of 40 women with urodynamically proven stress urinary incontinence (SUI). The patients in
one group underwent the TOT procedure, performed according to Delorme (Prog Urol 11:1306–1313, 2001); those in the second
group underwent the TVT-obturator operation, performed according to de Leval (Eur Urol 44:724–730, 2003). Intra-operative
diagnostic cystoscopy was not performed with either the TVT-obturator or the TOT procedures. The average follow-up was 12 months.
The two patient groups were similar in terms of demographic and therapeutic criteria, except for patient age, which was significantly
younger in the TVT-obturator group. Previously reported TVT-related operative complications, such as bladder penetration,
intra-operative bleeding, field infection and post-operative pelvic floor relaxation, were not observed in patients of either
group. Bowel and urethral injuries were also not recorded. The therapeutic failure rates were 10% for the TOT procedure and
5% for the TVT-obturator procedure. Urinary frequency and urgency post-operatively were reported in 25% of the TOT patients
and 19% of the TVT-obturator patients, pelvic or vaginal pain affected 10% of the TOT and 5% of the TVT-obturator patients,
while post-operative voiding difficulty was experienced by 12.5% of the TOT and 7.5% of the TVT-obturator patients. None of
the above-mentioned differences between the two patient groups were of statistical significance. The TVT-obturator and TOT
procedures, both minimally invasive, novel, mid-urethral sling procedures, seem to be safe, easy-to-perform and effective
in treating female SUI. The patients of both study groups suffered less intra- and post-operative surgical complications than
previously been reported in connection with the TVT operation. The TVT-obturator patients had fewer therapeutic failures,
less post-operative urinary frequency and urgency, less pelvic pain and less voiding difficulty. All of these findings, however,
had no statistical significance; consequently, long-term comparative data collection will be required before solid conclusions
can be drawn on the superiority of either of these two operative techniques. 相似文献
5.
Malcolm E. Richardson Samuel Menahem James L. Wilkinson 《International journal of cardiology》1991,30(3):351-353
In this report, we give details of two families in which fixed subaortic stenosis was found in more than one member. It is rare for this entity to show familial incidence. 相似文献
6.
123I-FP-CIT SPECT imaging of dopamine transporters in patients with cerebrovascular disease and clinical diagnosis of vascular parkinsonism. 总被引:2,自引:0,他引:2
Mordechai Lorberboym Ruth Djaldetti Eldad Melamed Menahem Sadeh Yair Lampl 《Journal of nuclear medicine》2004,45(10):1688-1693
The purpose of our study was to prospectively evaluate the striatal uptake of 123I-labeled N-(3-fluoropropyl)-2beta-carbomethoxy-3beta-(4-iodophenyl)nortropane (FP-CIT) and the response to l-dopa therapy in patients with cerebrovascular disease (CVD) who develop clinical symptoms of vascular parkinsonism (VP). METHODS: Twenty consecutive patients who developed VP in the course of CVD were prospectively enrolled in the study. All patients had CT evidence of CVD (17 patients had lacunar infarcts, 3 patients had territorial strokes). The clinical stage of the patients was assessed using the Hoehn and Yahr scale, and the severity of the symptoms was measured using the Unified Parkinson's Disease Rating Scale score. Ten age-matched subjects were used as controls. SPECT was performed 180 min after injection of 185 MBq 123I-FP-CIT using a dual-head gamma-camera. The ratio of the mean specific-to-nonspecific striatal binding for the entire striatum, caudate, and putamen was calculated in all patients and compared with that of controls. Putamen-to-caudate binding ratios were compared as well. The response to therapy was compared between patients with normal and abnormal 123I-FP-CIT binding. RESULTS: No correlation was found between any of the clinical variables and response to therapy in patients with VP. Nine patients had normal striatal 123I-FP-CIT binding with no significant differences in striatal or subregional binding ratios compared with those of the controls. In contrast, 11 patients had significantly diminished striatal binding compared with that of controls (P < 0.001). Subanalyses showed significantly decreased binding in the caudate (P < 0.04 and P < 0.01 for the right and left caudate, respectively), diminished binding in the putamen (P < 0.04 and P < 0.01 for the right and left putamen, respectively), and a decreased putamen-to-caudate ratio on the right side (P < 0.001). The latter ratio was not significant on the left. Two of the 3 patients with territorial strokes had significantly diminished striatal 123I-FP-CIT binding in the hemisphere contralateral to the CT lesion. All 9 patients with normal scan findings had a poor response to L-dopa. Six of 11 patients with abnormal studies had no response to L-dopa, whereas 5 patients had a good response (P < 0.03). CONCLUSION: The diagnosis of VP cannot be accurately confirmed on the basis of clinical features alone because CVD may alter the typical presentation of PD. Functional imaging with 123I-FP-CIT is highly recommended in patients with CVD who develop symptoms of VP to confirm or exclude the existence of nigrostriatal dopaminergic degeneration. Identifying a subset of patients with reduced 123I-FP-CIT binding in the striatum is important for better treatment selection. 相似文献
7.
Cardiac conduction abnormalities and rhythm changes after neonatal anatomical correction of transposition of the great arteries.
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S Menahem M S Ranjit C Stewart W J Brawn R B Mee J L Wilkinson 《Heart (British Cardiac Society)》1992,67(3):246-249
Seventy three infants who underwent neonatal anatomical correction for transposition of the great arteries with or without a ventricular septal defect were reviewed for evidence of conduction and rhythm abnormalities on preoperative and postoperative 12 lead electrocardiograms and during 24 hour Holter monitoring. There was a partial right bundle branch block pattern in 47% (29/62) of all patients and in 60% (24/40) of those with simple transposition. Complete right bundle branch block was noted in 21% including 5% with simple transposition. Holter monitoring showed sinus rhythm in all patients except three: one had episodes of supraventricular tachycardia, another an intermittent second degree heart block, and a third a complete heart block. Atrial extrasystoles were noted in 47% (29/62) of patients but were frequent in only three patients. Occasional unifocal ventricular extrasystoles were encountered in 37% (23/62) of patients and were frequent in a further 3% (2/62). Only one patient (2%) developed multifocal ventricular extrasystoles. The frequency of important cardiac arrhythmias after neonatal anatomical correction of transposition of the great arteries was 5%, significantly less than that reported after atrial inflow diversion for the same malformation. 相似文献
8.
Nir Lubezky Ur Metser Ravit Geva Richard Nakache Einat Shmueli Joseph M. Klausner Einat Even-Sapir Arie Figer Menahem Ben-Haim 《Journal of gastrointestinal surgery》2007,11(4):472-478
Background Recent data confirmed the importance of 18-fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET) in the selection of patients with colorectal hepatic metastases for surgery.
Neoadjuvant chemotherapy before hepatic resection in selected cases may improve outcome. The influence of chemotherapy on
the sensitivity of FDG-PET and CT in detecting liver metastases is not known.
Methods Patients were assigned to either neoadjuvant treatment or immediate hepatic resection according to resectability, risk of
recurrence, extrahepatic disease, and patient preference. Two-thirds of them underwent FDG-PET/CT before chemotherapy; all
underwent preoperative contrast-enhanced CT and FDG-PET/CT. Those without extensive extrahepatic disease underwent open exploration
and resection of all the metastases according to original imaging findings. Operative and pathological findings were compared
to imaging results.
Results Twenty-seven patients (33 lesions) underwent immediate hepatic resection (group 1), and 48 patients (122 lesions) received
preoperative neoadjuvant chemotherapy (group 2). Sensitivity of FDG-PET and CT in detecting colorectal (CR) metastases was
significantly higher in group 1 than in group 2 (FDG-PET: 93.3 vs 49%, P < 0.0001; CT: 87.5 vs 65.3, P = 0.038). CT had a higher sensitivity than FDG-PET in detecting CR metastases following neoadjuvant therapy (65.3 vs 49%,
P < 0.0001). Sensitivity of FDG-PET, but not of CT, was lower in group 2 patients whose chemotherapy included bevacizumab compared
to patients who did not receive bevacizumab (39 vs 59%, P = 0.068).
Conclusions FDG-PET/CT sensitivity is lowered by neoadjuvant chemotherapy. CT is more sensitive than FDG-PET in detecting CR metastases
following neoadjuvant therapy. Surgical decision-making requires information from multiple imaging modalities and pretreatment
findings. Baseline FDG-PET and CT before neoadjuvant therapy are mandatory.
The abstract was presented before the 58th Cancer Symposium of the Society of Surgical Oncology, Atlanta, GA, USA, 2005, and
before the 2005 Congress of the American Hepato-Pancreato-Biliary Association, Fort-Lauderdale, FL, USA. 相似文献
9.
Monoamine oxidase activity and triiodothyronine biosynthesis in human cultured thyroid cells.
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1. The proposal that monoamine oxidase (MAO) is a source of peroxide in thyroid hormone biosynthesis has been examined by use of isolated cultured human thyroid cells which retain the ability to secrete triiodothyronine (T3) in response to thyroid stimulating hormone (TSH). 2. The results demonstrated the presence of MAO A and B in human thyroid cells which oxidized 5-hydroxytryptamine and 2-phenylethylamine, respectively, and were selectively inhibited by the MAO inhibitors clorgyline and (-)-deprenyl. 3. Addition of propylthiouracil to the culture system induced a 61% reduction in TSH-stimulated T3 secretion, indicating that the bulk of such secretion apparently derives from de novo iodothyronine synthesis. 4. The MAO A and B substrate, tyramine, was ineffective in stimulating T3 secretion. 5. The selective MAO inhibitors, clorgyline and (-)-deprenyl, alone and in combination, and in the presence and absence of tyramine, failed to inhibit basal as well as TSH-stimulated T3 secretion in cultured human thyrocytes. 6. It is therefore apparent that even though thyroid MAO A and B enzyme reactions result in the generation of H2O2, this H2O2 does not seem to play a significant role in T3 biosynthesis. 相似文献
10.
Therapies that neutralize the function of TNF-alpha suppress rheumatoid arthritis (RA) but not osteoarthritis (OA). We show that patients suffering from RA but not OA have significant levels of autoantibodies directed to TNF-alpha. Thus, the immune system can selectively generate autoimmunity to proinflammatory mediators when such a response is beneficial for the host. A well-defined model of RA was used to elaborate the contribution of beneficial autoimmunity to the regulation of disease. We show that during the disease autoantibody production is elicited against few inflammatory, but not regulatory, mediators. Selective amplification of these beneficial antibodies by targeted DNA vaccines provided protective immunity. Epitope mapping revealed that anti-TNF-alpha immunity is highly restricted and excretes no crossreactivity to other known gene products. Its selective exclusion substantially exacerbated the disease. Administration of anti-TNF-alpha antibodies could then override this aggravation. This substantiates the significance of beneficial autoimmunity in restraining self-destructive immunity. 相似文献