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1.
Chiari III malformations are extremely rare hindbrain malformations that are associated with a high early mortality rate, or severe neurologic deficits in the survivors. The preferred treatment is early operative closure and CSF shunting. We report a case of a newborn infant with a Chiari III malformation with displacement of the brainstem and cerebellum into the cervical encephalocele which precluded immediate operative closure of the defect. Instead, a ventriculoperitoneal shunt was placed and the patient was followed with serial imaging studies. The child survived. The shunt allowed the brainstem and cerebellum to regress into the cervical spinal canal as the dilated cerebral aqueduct and fourth ventricle decompressed. A delayed closure of the cervical encephalocele was performed at 30 months of age. Cerebrospinal fluid diversion with delayed closure may be an option for large lesions.  相似文献   

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To assess the action of neurotrophin in human dopaminergic neurons, we studied the immunolocalization of neurotrophins or trks in human substantia nigra pars compacta (SNc). The neuromelanin-containing neurons in the SNc showed immunoreactivities for neurotrophins or trks, suggesting an autocrine/paracrine regulation. Quantitative analysis revealed that the percentage of those expressing NGF-like immunoreactivity (NGF-LI), BDNF-LI, NT3-LI, trkA-LI, trkB-LI, or trkC-LI was 66%, 74%, 85%, 66%, 71% or 86%, respectively. The percentage of cells expressing neurotrophins or trks was higher in the medial part than in the lateral part of the SNc. The preferential expression of neurotrophin-trk systems in the medial neurons may, at least partially, explain the differential susceptibility in Parkinson's disease.  相似文献   

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CONTEXT: There is urgent need to strengthen the area of pediatric HIV/AIDS care in developing countries. Clinical research in this area is also scarce. METHODOLOGY: A literature review and a postal survey were used to obtain updated information on mortality, morbidity and current standards of care of children born to HIV-infected mothers in developing countries. A 2-day workshop was organized to review the available data and to identify the key areas where clinical research should be conducted. MAIN FINDINGS: Rates of mortality and morbidity were very different from one study to another but generally higher than in industrialized countries. Prognostic studies for HIV-1-infected children in developing countries were not available. Based on the report of 14 teams from 11 countries, specific protocols for HIV-infected children with persistent diarrhea or severe malnutrition were documented in fewer than one-half of the cases. Secondary antimicrobial prophylaxis after interstitial pneumonia or recurrent infections was still infrequent, as primary prophylaxis of opportunistic infections. The following list of clinical research priorities was identified by the workshop participants: primary prophylaxis of opportunistic and bacterial infections; case management of persistent diarrhea; reassessment of the performance of p24 antigen for diagnostic and prognosis use; studies on the etiology of pulmonary infections; long term observational pediatric cohorts; current weaning practices and duration of breast-feeding; counseling and HIV testing of children and families; prevention of HIV sexual transmission in children and adolescents.  相似文献   

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A female infant was found to have a congenital, firm, pea-sized tumour nasally in the lower left eyelid. A dermoid cyst was suspected, but microscopy showed structures simulating a lens. The tumour has been considered to have an origin in surface ectoderm induced to dip down into the mesoderm of the lid, there forming lenticular anlage structures. This so-called phakomatous choristoma has only been reported five times previously, all cases outside Europe.  相似文献   

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BACKGROUND: Two types of antineutrophil cytoplasmic antibodies (ANCA), antiproteinase 3 antibodies (anti-PR3) and antimyeloperoxidase antibodies (anti-MPO), are useful in the diagnosis of such types of vasculitis as Wegener granulomatosis and microscopic polyangiitis. Connective tissue diseases frequently appear in the differential diagnosis of this spectrum of vasculitis. OBJECTIVE: To determine the prevalence of ANCA in patients with connective tissue disease. DESIGN: Blinded, controlled study of a 5-year inception cohort. SETTING: Tertiary-care university teaching hospitals. PATIENTS: 70 patients with rheumatoid arthritis, 70 patients with systemic lupus erythematosus, 45 patients with scleroderma, 36 patients with inflammatory myositis, 44 patients with the sj?gren syndrome, 33 patients with the antiphospholipid syndrome, and 165 patients with early undifferentiated connective tissue disease (EUCTD). Serum was taken from 200 blood donors and 52 patients who had known vasculitis and positive results on tests for anti-PR3 or anti-MPO; these patients served as controls. MEASUREMENTS: The presence of anti-PR3 and anti-MPO was determined by combining the results of indirect immunofluorescence tests for cytoplasmic (C-ANCA) and perinuclear (P-ANCA) patterns with the results of enzymelinked immunosorbent assays (ELISAs) directed to measure antigen. RESULTS: Cytoplasmic ANCA was not detected in any study or control patient. Perinuclear ANCA was commonly detected among patients with lupus (31%) but was uncommon among patients in other groups (0% to 5%). In all cases, P-ANCA was associated with the presence of antinuclear antibodies. Atypical ANCA immunofluorescence patterns were fairly common in all groups (11% to 39%). Antiproteinase 3 was detected by ELISA in study patients (1 patient with rheumatoid arthritis, 1 with lupus, 1 with polymyositis, and 6 with EUCTD). Antimyeloperoxidase was detected by ELISA in 2 study patients (1 with rheumatoid arthritis and 1 with lupus). None of the patients with positive ELISA results had evidence of renal vasculitis during follow-up. When an ANCA scoring system that combines immunofluorescence and ELISA was used, the test specificity for vasculitis was 99.5% among patients with connective tissue disease. CONCLUSIONS: Patients with connective tissue disease are known to develop multiple autoantibodies; positivity for anti-PR3 and anti-MPO ANCA in such patients is highly specific for anti-PR3. However, P-ANCA immunofluorescence, which may have positive results because of the presence of antinuclear antibodies, is not a specific marker of anti-MPO. A rigorous ANCA testing system that combines the results of immunofluorescence with those of ELISA is highly specific for Wegener granulomatosis and related vasculitides even in patients with connective tissue disease.  相似文献   

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BACKGROUND: Various cryopreservation techniques have been investigated to elongate preservation time, however, most have failed to be clinically induced because of damage due to ice crystal formation. Subzero nonfreezing conditions could theoretically reduce organ metabolism without damage due to ice crystal formation. We evaluated the superiority of subzero nonfreezing storage compared with conventional hypothermic storage using isolated rat hepatocytes stored in University of Wisconsin (UW) solution without cryoprotectants. METHODS: Hepatocytes of Wistar rats isolated by collagenase digestion were suspended in UW solution and divided into the following three groups: subzero nonfreezing group (-4 degrees C), zero nonfreezing group (0 degrees C), and control group (4 degrees C). They were stored for 48 hr at the temperatures indicated. After 24 and 48 hr of storage, we carried out a trypan blue exclusion test and a 3-[4,5-dimethylthiazol-2-yl]-2,5-diphenyltetrazolium bromide (MTT) assay, and measured lactate dehydrogenase release, lactic acid, ATP content, and the ability of hepatocytes to synthesize urea. After 48 hr of storage, morphological differences between the control group and the subzero nonfreezing group were investigated by scanning and transmission electron microscopy. RESULTS: Significant improvements of the trypan blue exclusion test and ATP contents in the subzero nonfreezing group were observed. Lactic acid production was also significantly suppressed in the subzero nonfreezing group compared with that in the control group. The MTT assay value was significantly better at -4 degrees C than at 4 degrees C. The rate of urea synthesis at -4 degrees C was higher than that at 4 degrees C. Electron microscopy revealed that subzero nonfreezing delayed the lethal bleb-forming process of stored hepatocytes, which was followed by mitochondrial swelling, compared with the control group. CONCLUSIONS: Subzero nonfreezing storage (-4 degrees C) in UW solution could provide better preservability for isolated rat hepatocytes with protection against hypoxic cell injury compared with conventional hypothermic storage (4 degrees C).  相似文献   

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铜转炉优化操作智能决策支持系统的研究及应用   总被引:5,自引:1,他引:4  
为了提高炼铜转炉利用系数,挖掘转炉潜力,采用基于人工智能和解析方法相结合的集成建模方法研制了一套优化操作智能决策支持系统,该系统已成功地应用于贵溪冶炼厂的4^#炼铜转炉中。实际应用表明,粗铜产量、冷料处理量、平均炉寿等各项生产指标显著提高,为炼铜转炉实现在线控制提供了新思路。  相似文献   

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Provision of long-term support and rehabilitation after stroke varies in the UK. Patients and their carers are not always aware of services available. Family support service organisers can help increase awareness of risk factors and minimise recurrence of stroke.  相似文献   

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Lower eyelid malposition is the most common long-term complication following transcutaneous lower eyelid blepharoplasty. The malposition may include rounding of the lateral canthal angle, lower eyelid retraction with inferior scleral show, or frank ectropion. The result is cosmetically unacceptable and may be associated with tearing, irritation, and other exposure keratitis symptoms. Multiple factors, including lower eyelid laxity, shortage of skin, and scarring of the middle lamella, may be responsible for this malposition. A systematic examination of the lower eyelid, as presented, helps to assess the degree to which each of these factors is responsible for the malposition. Patients with the most severe degree of lower eyelid malposition generally have middle lamella scarring. If this abnormality is not addressed, lower eyelid procedures aimed at correcting the malposition are doomed to failure. In the presence of significant middle lamella scarring, a spacer is required to provide vertical height and stiffness to support the lower eyelid following release of the cicatrix. A systematic approach aimed at addressing the underlying abnormalities was developed. In patients with significant middle lamella scarring, hard palate mucosa grafts were used as spacers in 29 eyelids (17 patients). A lateral canthotomy and transconjunctival incision allow access to the scarring in the lower eyelid retractors and septum. After careful release of all cicatrix, a hard palate mucosa graft is inserted between the lower border of the tarsal plate and the recessed conjunctiva, lower eyelid retractors, and septum. Horizontal lower eyelid laxity, when present, is corrected by performing a lateral tarsal strip. Most patients do not have a true deficiency of the anterior lamella (skin and orbicularis oculi muscle). When a moderate amount of anterior lamella deficiency is present with significant scarring of the middle lamella, the technique we describe allows correction of the lower eyelid malposition without a skin graft. After a follow-up interval of 6 to 30 months (mean 14 months), excellent results were obtained in all eyelids. Complications included corneal abrasions in two eyes before routine use of bandage cornea contact lenses at the end of surgery and a secondary bleed from the roof of the mouth in one patient. Palate mucosa closely resembles tarsus and provides excellent vertical support to the eyelid. It is stiff enough to maintain eyelid contour without causing a cosmetically unacceptable bump. Tissue can be obtained with ease. The technique, as described, addresses the underlying causes of lower eyelid malposition and gives excellent functional and cosmetic results.  相似文献   

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BACKGROUND: Published reports indicate that eyelid involvement occurs in approximately 25% of patients with systemic blastomycosis and that mortality exceeds 60% in untreated patients. The purpose of this study is to investigate this purportedly frequent association. METHODS: The Medical Diagnostic index of the Mayo Clinic was used to identify patients who were treated for blastomycosis from 1976 through 1993. The medical records of affected patients were reviewed. RESULTS: Of 79 patients with blastomycosis, only 1 (1.27%; 95% confidence interval, 0.03%-6.85%) had eyelid involvement. The diagnosis of systemic disease was established in this patient after biopsy of the eyelid lesion, and the infection responded satisfactorily to treatment with itraconazole. CONCLUSION: Contrary to previous reports, eyelid involvement with systemic blastomycosis is uncommon.  相似文献   

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In acknowledging that 'counselling is generally recognized as beneficial', the Human Fertilization and Embryology Authority (HFEA) Code of Practice requires that all infertility units provide counselling facilities to be available for patients. In this study, we intended to evaluate the support and counselling services made available by the licensed units in the UK. A questionnaire consisting of 30 questions was designed and sent to every licensed treatment unit in the UK. The data were coded on a nominal scale and, using a data entry program, loaded onto a computer. Using the Statistical Package for the Social Sciences program, a non-parametric frequency analysis was performed. Associations were examined with cross-tabulations and chi 2 analysis. A total of 62 units (61.4%) responded to the questionnaire, from both the private and National Health Service sectors. Of these, 95% have their own counsellor, most of whom (84%) practised on the premises. One-third of these counsellors had a dual role, mainly as nurses, social workers or in administration; 98.6% were trained in counselling, with only 28% having either the Certificate or Diploma in Counselling. One-third (32.2%) of centres charged for counselling, with only 13 units indicating their charges. The majority of centres (78.8%) do not actively follow-up patients after counselling and one-quarter (25.5%) did not have a specific counselling room. Over two-thirds (68.4%) of centres described their support network as adequate. The results of this survey suggest that, although the requirements of the HFEA Code of Practice are being adhered to reasonably well, overall patient uptake of counselling is low.(ABSTRACT TRUNCATED AT 250 WORDS)  相似文献   

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Previous studies identify a positive association between social support and favorable posttreatment functioning. The next step for this line of inquiry is to identify the mechanisms through which social relationships affect alcohol treatment outcome by discrimination of the types and sources of social support. This article presents versions of the Perceived Social Support instrument (M. Procidano & K. Heller, 1983), which measures support from both family and friends. The item sets are reduced from 20 to 7 and ask identical questions from these two support sources. The internal consistency for the Family scale (α?=?.84) and for the Friends scale (α?=?.81) is acceptable. Test-retest reliability is also good for both the Family (r?=?.94) and the Friends (r?=?.88) scales. Correlations with social functioning and alcohol involvement are similar between the short scales and the original 20 items. Normative levels of family and friends support by gender are presented. These scales expand the ability to include a multifaceted social support construct in comprehensive assessments of alcohol treatment populations. (PsycINFO Database Record (c) 2010 APA, all rights reserved)  相似文献   

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We treated patients with Kaposi's sarcoma of the lid or conjunctiva, molluscum contagiosum and papilloma of the lid with dye laser photocoagulation. This therapeutic device is extremely efficient as the chosen wavelength of 577 nm is absorbed selectively by haemoglobin. Since Kaposi's sarcoma is a haemoglobin-containing tumour composed of endothelial cells and papilloma is a benign tumour composed of connective tissue and branching vessels, dye laser photocoagulation causes selective damage to abnormal vessels and surrounding connective tissue. After local anaesthesia a small incision was made at the margin of the lesion five patients each with papilloma and with molluscum contagiosum. In six patients with Kaposi's sarcoma no incision had to be done. Starting from the incision or the margin of the Kaposi's sarcoma 50-100 shots of 0.3-0.5 mm diameter and 1 s duration were applied until the lesion was completely gone. We used a dye laser with 577 nm wavelength and 1 W energy. In patients with papilloma, the tumour basis was coagulated and the tumour itself underwent histological examination. There was a marked decrease of the size of the lesion or even complete disappearance. Since Kaposi's sarcoma in AIDS patients can be relatively fast growing, resulting in cosmetically irritating manifestations and, rarely, haemorrhages, treatment may become necessary. AIDS patients with molluscum contagiosum may also benefit from treatment, as spreading of DNA virus particles may result in viral follicular conjunctivitis. Dye laser photocoagulation, however, cannot protect against relapse.  相似文献   

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PURPOSE: To present a case of focal mucinosis of the eyelid. PATIENT: A male patient of Turkish origin born in 1982 desired therapy in 1995 for an asymptomatic tumour of the left medial upper lid which had been present probably since birth. The tumour was reduced with argon laser photocoagulation. Fourteen months later, in 1997, the tumour had enlarged so that a surgical excision was performed which histologically was interpreted as an unspecific inflammation. In 1998, the tumour had grown again at the original site, and there were additional three pedunculated tumours nearby at the lid margin. All tumours were surgically excised. Histology showed empty spaces in the upper dermis which contained some connective fibers, benign appearing fibroblasts and some leukocytes. The PAS-reaction and S100-immunohistology were negative while the lesion stained positive for alcianblue. The epidermis was intact. A diagnosis of a focal mucinosis of the eyelid was established. CONCLUSIONS: Focal mucinosis of the eyelid seems to be exceedingly rare. It is probably caused by a local overproduction of hyaluronic acid of unknown etiology. Our patient demonstrates that focal mucinosis might perhaps be congenital. Moreover it may recur if excision is not complete. Focal mucinosis should be included in the differential diagnosis of long-lasting, asymptomatic lid tumours.  相似文献   

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Stereotactic medial posteroventral pallidotomy for treatment of Parkinson's disease attracts increasing attention. We report on the preliminary results of 12 patients at 1 year after microelectrode-guided unilateral pallidotomy. The primary indications were severe bradykinesia and levodopa-induced dyskinesias. After radiofrequency lesioning all patients had immediate improvement of contralateral parkinsonian signs. Postoperative magnetic resonance imaging confirmed the localization of the lesions. At the 1-year follow-up, all patients had sustained benefit. The global improvement was rated as moderate in six cases, and as marked in six other cases. The mean values of various subscores of the Unified Parkinson's Disease Rating Scale (UPDRS) showed highly significant changes in the "off" state (pre/postoperatively): UPDRS Motor score (60.3/31). UPDRS Activities of Daily Living (ADL) score (33.2/18.3), gait/postural stability score (13.8/7.0), and subscores for contralateral rigidity (4.9/2.1), tremor (7.1/1.4) and bradykinesia (11.6/5.3). There was also significant improvement of ipsilateral bradykinesia and rigidity. Furthermore, we found significant changes of the mean values of the UPDRS ADL and motor "on" scores, an increase of the percentage of "on" time with reduced on-off fluctuations, and a decrease of the percentage of levodopa-induced dyskinesias, with marked improvement or complete abolition of contralateral dyskinesias in particular. The preoperative levodopa regimen was maintained, in general, or only slightly modified, if necessary. Two patients had transient complications: one patient suffered postoperative pneumonia and altered mental status; another patient displayed mild Broca's aphasia secondary to a small stroke involving the dorsal thalamus and the adjacent white matter. There were no persistent side effects at the 1-year follow-up. Contemporary unilateral pallidotomy is an effective and promising therapeutical option for surgical treatment of advanced Parkinson's disease.  相似文献   

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BACKGROUND: To investigate the role and impact of multiplane transesophageal echocardiography during thrombectomy in the inferior vena cava or the right atrium. EXPERIMENTAL DESIGN: Retrospective. SETTING: A university hospital. PARTICIPANTS: Four patients who underwent removal of tumor thrombus in the inferior vena cava (IVC) or the right atrium. INTERVENTIONS: The medical records of 4 patients and videotapes of these intraoperative transesophageal echocardiography examinations were reviewed. RESULTS: Before thrombectomy, multiplane transesophageal echocardiography (MTEE) provided excellent IVC long axis view, which offered precise recognition of the cephalic extent of tumor, extent of caval occlusion, characterization of the tumor head. During surgery, MTEE could provide continuous monitoring of cardiac function, cardiac volume, and pulmonary embolism. Moreover, MTEE could provide the useful images of a cannula or the caval occlusion balloon catheter, which facilitated removal of neoplasm extending into the IVC. CONCLUSIONS: We presented four surgical cases, in which the removal of the tumor extended into the inferior vena cava or the right atrium using MTEE. MTEE could provide valuable information such as excellent images of the tumor, cardiac function, the position of a cannula or the caval occlusion balloon catheter. These findings could improve the anesthetic management of the patients, as well as the surgical approach and technical maneuvers, and facilitate removal of neoplasm into the IVC.  相似文献   

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