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1.
Friedreich ataxia (FRDA) is a multisystem neurodegenerative disorder and the most common hereditary ataxia. Dysphagia (swallowing impairment) is present in 98% of individuals with FRDA and is characterized by lingual and pharyngeal dysfunction (manifesting in impaired bolus preparation and transfer, and post-swallow residue in the mouth and pharynx), delayed swallow initiation, and entry of material into the airway (penetration/aspiration). Dysphagia severity correlates with disease severity and duration however no longitudinal studies describe changes in function in FRDA. The aim of this study was to investigate the progression of dysphagia in FRDA over one year. Fifty-nine individuals with FRDA and confirmed dysphagia were recruited and 23 of them underwent a second assessment 12 months later. Assessments of swallowing related quality of life, oral motor function (Frenchay Dysarthria Assessment 2nd Ed [FDA-2]) and functional swallowing via videofluoroscopy were conducted. Trials of thin liquid, puree and biscuit were interpreted using the Bethlehem Assessment Scale and the Penetration-Aspiration Scale by two blinded raters. Data from the videofluoroscopy revealed a decline in tongue function, pharyngeal clearance and cricopharyngeal function on solid food. However, severity of penetration/aspiration did not increase. Swallowing-related quality of life and oral-motor function remained stable. A decline in function was observed at three anatomical sites considered important for safe and effective swallowing (tongue, pharyngeal, and cricopharyngeal). However, these deficits did not translate into any meaningful functional decline in swallowing related health over 12 months for individuals with FRDA.  相似文献   

2.
Screening assessments for dysphagia are essential in neurodegenerative disease. Yet there are no purpose-built tools to quantify swallowing deficits at bedside or in clinical trials. A quantifiable, brief, easy to administer assessment that measures the impact of dysphagia and predicts the presence or absence of aspiration is needed. The Clinical Assessment of Dysphagia in Neurodegeneration (CADN) was designed by a multidisciplinary team (neurology, neuropsychology, speech pathology) validated against strict methodological criteria in two neurodegenerative diseases, Parkinson’s disease (PD) and degenerative ataxia (DA). CADN comprises two parts, an anamnesis (part one) and consumption (part two). Two-thirds of patients were assessed using reference tests, the SWAL-QOL symptoms subscale (part one) and videofluoroscopic assessment of swallowing (part two). CADN has 11 items and can be administered and scored in an average of 7 min. Test–retest reliability was established using correlation and Bland–Altman plots. 125 patients with a neurodegenerative disease were recruited; 60 PD and 65 DA. Validity was established using ROC graphs and correlations. CADN has sensitivity of 79 and 84% and specificity 71 and 69% for parts one and two, respectively. Significant correlations with disease severity were also observed (p < 0.001) for PD with small to large associations between disease severity and CADN scores for DA. Cutoff scores were identified that signal the presence of clinically meaningful dysphagia symptomatology and risk of aspiration. The CADN is a reliable, valid, brief, quantifiable, and easily deployed assessment of swallowing in neurodegenerative disease. It is thus ideally suited for both clinical bedside assessment and future multicentre clinical trials in neurodegenerative disease.  相似文献   

3.
Dysphagia is a frequent and early symptom in progressive supranuclear palsy (PSP) predisposing patients to aspiration pneumonia. Fiberoptic endoscopic evaluation of swallowing (FEES®) has emerged as a valuable apparative tool for objective evaluation of neurogenic dysphagia. This is the first study using FEES® to investigate the nature of swallowing impairment in PSP. Eighteen consecutive PSP patients (mean age 69.7 ± 9.0 years) were included. The salient findings of FEES® in PSP patients were compared with those of 15 patients with Parkinson's disease (PD). In 7 PSP patients, a standardized FEES® protocol was performed to explore levodopa (L ‐dopa) responsiveness of dysphagia. Most frequent abnormalities detected by FEES® were bolus leakage, delayed swallowing reflex, and residues in valleculae and piriformes. Aspiration events with at least one food consistency occurred in nearly 30% of PSP patients. Significant pharyngeal saliva pooling was observed in 4 PSP patients. We found no difference of salient endoscopic findings between PSP and PD patients. Endoscopic dysphagia severity in PSP correlated positively with disease duration, clinical disability, and cognitive impairment. No correlation was found with dysarthria severity. In early PSP patients, swallowing dysfunction was solely characterized by liquid leakage with the risk of predeglutitive aspiration during the oral phase of swallowing. Two PSP patients showed relevant improvement of swallowing function after L ‐dopa challenge. Chin tuck—maneuver, hard swallow, and modification of food consistency were identified as the most effective therapeutic interventions. In conclusion, FEES® assessment can deliver important findings for the diagnosis and refined therapy of dysphagia in PSP patients. © 2010 Movement Disorder Society  相似文献   

4.
ObjectiveA large proportion of patients with Parkinson's disease develop dysphagia during the course of the disease. Dysphagia in Parkinson's disease affects different phases of deglutition, has a strong impact on quality of life and may cause severe complications, i.e. aspirational pneumonia. So far, little is known on how deep-brain-stimulation of the subthalamic nucleus influences deglutition in PD.MethodsVideofluoroscopic swallowing studies on 18 patients with Parkinson's disease, which had been performed preoperatively, and postoperatively with deep-brain-stimulation-on and deep-brain-stimulation-off, were analyzed retrospectively. The patients were examined in each condition with three consistencies (viscous, fluid and solid). The ‘New Zealand Index for Multidisciplinary Evaluation of Swallowing (NZIMES) Subscale One’ for qualitative and ‘Logemann-MBS-Parameters’ for quantitative evaluation were assessed.ResultsPreoperatively, none of the patients presented with clinically relevant signs of dysphagia. While postoperatively, the mean daily levodopa equivalent dosage was reduced by 50% and deep-brain-stimulation led to a 50% improvement in motor symptoms measured by the UPDRS III, no clinically relevant influence of deep-brain-stimulation-on swallowing was observed using qualitative parameters (NZIMES). However quantitative parameters (Logemann scale) found significant changes of pharyngeal parameters with deep-brain-stimulation-on as compared to preoperative condition and deep-brain-stimulation-off mostly with fluid consistency.ConclusionIn Parkinson patients without dysphagia deep-brain-stimulation of the subthalamic nucleus modulates the pharyngeal deglutition phase but has no clinically relevant influence on deglutition. Further studies are needed to test if deep-brain-stimulation is a therapeutic option for patients with swallowing disorders.  相似文献   

5.
This study assessed the clinical indicators and severity of dysphagia in a representative sample of children with severe generalized cerebral palsy and intellectual disability. A total of 166 children (85 males, 81 females) with Gross Motor Function Classification System Level IV or V and IQ<55 were recruited from 54 daycare centres. Mean age was 9 years 4 months (range 2 y 1 mo-19 y 1 mo). Clinically apparent presence and severity of dysphagia were assessed with a standardized mealtime observation, the Dysphagia Disorders Survey (DDS), and a dysphagia severity scale. Additional measures were parental report on feeding problems and mealtime duration. Of all 166 participating children, 1% had no dysphagia, 8% mild dysphagia, 76% moderate to severe dysphagia, and 15% profound dysphagia (receiving nil by mouth), resulting in a prevalence of dysphagia of 99%. Dysphagia was positively related to severity of motor impairment, and, surprisingly, to a higher weight for height. Low frequency of parent-reported feeding problems indicated that actual severity of dysphagia tended to be underestimated by parents. Proactive identification of dysphagia is warranted in this population, and feasible using a structured mealtime observation. Children with problems in the pharyngeal and esophageal phases, apparent on the DDS, should be referred for appropriate clinical evaluation of swallowing function.  相似文献   

6.
Few studies exist in the literature investigating the impact of idiopathic Parkinson's Disease (IPD) on swallow‐related quality of life. We therefore aimed in this project to: (1) evaluate swallow‐specific quality of life in IPD; (2) delineate potential relationships between IPD duration and severity with swallow‐specific quality of life; (3) investigate relationships between swallow‐specific quality of life and general health‐related quality of life; and (4) investigate relationships between swallow‐specific quality of life and depression. Thirty‐six patients diagnosed with IPD with and without dysphagia filled out self‐report assessments of the SWAL‐QOL, Parkinson's Disease Questionnaire‐39 (PDQ‐39), and Beck Depression Inventory (BDI). A series of Mann Whitney U tests were performed between non‐dysphagic and dysphagic groups for the total SWAL‐QOL score and the 10 SWAL‐QOL domains. Spearman's Rho correlation analyses were performed between the SWAL‐QOL and (1) PDQ‐39; (2) Hoehn and Yahr stage; (3) PD disease duration; (4) UPDRS “on” score; and (5) the BDI. The dysphagia swallowing group reported significant reductions compared to the non‐dysphagic group for the total SWAL‐QOL score (P = 0.02), mental health domain score (P = 0.002) and social domain score (P = 0.002). No relationships existed between swallow‐specific quality of life and disease duration or severity. Significant relationships existed between swallow‐specific quality of life and general health‐related quality of life (rs =?0.56, P = 0.000) and depression (rs = ?0.48, P = 0.003). These exploratory data highlight the psychosocial sequelae that swallowing impairment can have in those with IPD and suggest a possible association between swallowing, social function, and depression. © 2009 Movement Disorder Society  相似文献   

7.
To use optical coherence tomography (OCT) and contrast letter acuity to characterize vision loss in Friedreich ataxia (FRDA). High- and low-contrast letter acuity and neurological measures were assessed in 507 patients with FRDA. In addition, OCT was performed on 63 FRDA patients to evaluate retinal nerve fiber layer (RNFL) and macular thickness. Both OCT and acuity measures were analyzed in relation to genetic severity, neurologic function, and other disease features. High- and low-contrast letter acuity was significantly predicted by age and GAA repeat length, and highly correlated with neurological outcomes. When tested by OCT, 52.7 % of eyes (n = 110) had RNFL thickness values below the fifth percentile for age-matched controls. RNFL thickness was significantly lowest for those with worse scores on the Friedreich ataxia rating scale (FARS), worse performance measure composite Z 2 scores, and lower scores for high- and low-contrast acuity. In linear regression analysis, GAA repeat length and age independently predicted RNFL thickness. In a subcohort of participants, 21 % of eyes from adult subjects (n = 29 eyes) had macular thickness values below the first percentile for age-matched controls, suggesting that macular abnormalities can also be present in FRDA. Low-contrast acuity and RNFL thickness capture visual and neurologic function in FRDA, and reflect genetic severity and disease progression independently. This suggests that such measures are useful markers of neurologic progression in FRDA.  相似文献   

8.
Objective: A detailed knowledge of dysphagia outcomes in lateral medullary infarct (LMI) patients would enable proper establishment of swallowing therapy goals and strategies. However, little is known about the impact of infarct location on dysphagia outcomes in patients with LMI. Methods: Twenty patients with rostral LMI (rostral group) and 20 patients with caudal LMI (caudal group) participated in the study. All patients underwent swallowing therapy, which included compensatory treatments and strengthening exercises, for >3 months. Dysphagia evaluation was performed twice (during the subacute stage and six months after stroke onset) using videofluoroscopic swallowing studies. Dysphagia degree was assessed using the functional dysphagia scale (FDS), the penetration–aspiration scale (PAS) and the American Speech-Language-Hearing Association (ASHA) National Outcome Measurement System (NOMS) swallowing scale. Results: In the subacute stage, the rostral group had significantly higher FDS and PAS scores and a significantly lower ASHA NOMS score than the caudal group. Patients from both groups showed significant improvement from the initial evaluation to the six-month evaluation. There were no significant differences in these scale scores between the two groups at the six-month evaluation. Conclusion: In the subacute stage, patients in the rostral group had more severe dysphagia than those in the caudal group. Dysphagia improved in both groups after 3–6 months of swallowing therapy. At six months after onset, there were no significant differences in dysphagia severity between the two groups. Recovery from dysphagia after LMI was observed regardless of the infarct location.  相似文献   

9.
The aim of the study was to analyse swallowing function and to identify reliable prognostic factors associated with dysphagia in a consecutive series of patients with multiple sclerosis (MS). Swallowing examination was performed by means of indirect and direct methods (fiberendoscopic evaluation) in 143 consecutive patients with primary and secondary progressive MS. Dysphagia was found in 49 patients (34.3%). A close relationship with dysphagia was found in the patients with severe brainstem impairment (OR=3.24; 95% CI 1.44-7.31) as compared to the patients without. There was also a significant correlation with pronounced severity of illness (OR=2.99; CI 1.36-6.59). Compensatory strategies were sufficient to resolve the dysphagia in 46 cases (93.8%). The potential risk of aspiration and malnutrition and the high efficacy of swallowing rehabilitation suggests that all MS patients should have a careful evaluation of deglutition functionality, especially those with brainstem impairment and a high grade of disability level.  相似文献   

10.
Dysphagia in X-linked bulbospinal muscular atrophy (Kennedy disease) has never been characterized in detail by objective swallowing studies. We assessed the nature of swallowing impairment in Kennedy disease by undertaking fiberoptic endoscopic evaluation of swallowing examinations of 10 genetically confirmed patients with Kennedy disease who were scored according to an ordinal rating scale including 25 different items. The results were compared to an age-matched control group of 10 healthy volunteers. Swallowing dysfunction was found in 80% of patients with Kennedy disease. The main pattern of dysphagia was an incomplete food bolus clearance through the pharynx with residues left in the valleculae overflowing into the laryngeal vestibule after the swallow. Total duration of the pharyngeal swallow was significantly shorter in patients with Kennedy disease compared to the control group. These findings suggest that dysphagia in Kennedy disease is predominantly characterized by an impairment of the pharyngeal phase of swallowing resulting from reduced base-of-tongue movement and bilateral paresis of pharyngeal and laryngeal muscles.  相似文献   

11.
Dysphagia in Huntington's disease   总被引:2,自引:0,他引:2  
Dysphagia is a common complication of Huntington's disease (HD) that is frequently responsible for the potentially lethal respiratory events of aspiration or asphyxiation. Twelve patients who had HD and a history of dysphagia underwent extensive multidisciplinary clinical examinations. All of the patients, regardless of the clinical severity of their disease, demonstrated impaired control of many voluntary aspects of food intake that affected swallowing efficiency. Abnormalities of the rate of food consumption, mastication, bolus transfer, respiration, and swallow initiation seem to be responsible for most dysphagic symptoms in HD. Less prominent abnormalities of the pharyngoesophageal phases of ingestion were also noted. Dysphagia therapy was initiated in 11 of 12 patients. All of the patients' conditions improved; a majority (8/11) of the patients returned to an unrestricted diet. This improvement persisted for as long as three years, while other clinical features of HD intensified.  相似文献   

12.
Swallowing disorders in Parkinson's disease   总被引:4,自引:0,他引:4  
The aim of this study was to assess the reflex and oral, pharyngeal, esophageal phase of swallowing in patients with Parkinson's disease (PD). Eighteen patients with PD and 22 healthy control subjects were investigated using electromyography (EMG) and esophageal scintigraphy. This study demonstrated delayed triggering of the swallowing reflex (443±84 ms in patients with PD vs. 230±96 ms in controls, p<0.05) and prolongation of laryngeal movement (980±140 vs. 649±145 ms, p<0.05). We found prolongation of the esophageal phase of swallowing (14.46±5.30 vs. 7.45±1.64 s, p<0.001) in PD patients. The dysphagia limit i.e. the maximum amount of water swallowed at once was smaller in PD patients than in controls (6.23±3.67 vs. >20 ml). Dysphagia was observed in all patients studied although only 13 of them complained about it. In the remaining five cases swallowing impairment was subclinical and it consisted of decreased dysphagia limit and prolongation of the esophageal phase. Dysphagia at the subclinical level may be one of the early symptoms of PD.  相似文献   

13.
We evaluated swallowing function in patients with myasthenia gravis (MG) with or without dysphagia symptoms using different evaluation parameters and compared the results with those of healthy subjects. A total of 36 patients with MG and 25 healthy volunteers were included in the study. The subjects were classified into three groups; patients without dysphagia (group 1), patients with dysphagia (group 2), and healthy participants (group 3). The presence and severity of dysphagia, the oropharyngeal, pharyngeal, pharyngoesophageal, and esophageal phases were assessed using a screening test, manometric test, electrophysiologic studies [electroneuromyography (EMG)], fiberoptic endoscopic evaluation of swallowing (FEES), and barium swallow pharyngeal esophagography (BSPE), respectively. There was a significant difference between group 1 and group 3 in terms of BSPE (p = 0.001) and manometry tests (p = 0.001). A significant difference was found in all methods between group 2 and group 3 (p = 0.001, for all). In the comparison of the patient groups, although the number of patients with dysphagia in group 2 was significantly higher in the clinical tests (p = 0.007), FEES (p = 0.001), and EMG (p = 0.043) than in group 1, no difference was detected for BSPE (p = 0.119) and manometry (p = 0.644). Swallowing functions in patients with MG may be affected even without symptoms. This condition should be considered in their follow-up.  相似文献   

14.
Dysphagia is found in up to 80% of acute stroke patients. To date most studies have focused on ischemic stroke only. Little is known about the incidence and pattern of dysphagia in hemorrhagic stroke. Here we describe the characteristics of dysphagia in patients with striatocapsular hemorrhage. Fiberoptic Endoscopic Evaluation of Swallowing (FEES) was carried out in 30 patients with acute striatocapsular hemorrhage. Dysphagia was classified according to the six-point Fiberoptic Endoscopic Dysphagia Severity Scale (FEDSS) within 72 h after admission. Lesion volume, hemisphere and occurrence of ventricular rupture were determined from computer tomography scans. Data on initial NIH-SS, clinical symptoms, need for endotracheal intubation, diagnosis of pneumonia and feeding status on discharge were recorded. Swallowing impairment was observed in 76.7% of patients (n = 23). Mean FEDSS score was 3.1 ± 1.5. Main findings were penetration or aspiration of liquids as well as leakage to valleculae and piriform sinus. Incidence of pneumonia was 30.0% (n = 9). Age, NIH-SS and hematoma volume did not correlate with dysphagia severity. None of the clinical characteristics was predictive for dysphagia. On discharge after 12.9 ± 5.3 days, a two-point improvement on the FEDSS was seen in seven patients, (30.4%) and five patients (21.7%) had gained at least one point. In striatocapsular hemorrhage, dysphagia is a common and so far underrecognized symptom. FEES results indicate predominant impairment of oral motor control. Swallowing impairment is not related to other clinical deficits, stroke severity or lesion characteristics. Thus, detailed dysphagia assessment is indicated in all cases.  相似文献   

15.
ObjectiveWe performed an electrophysiological study of swallowing (EPSS) in multiple sclerosis (MS) to describe oropharyngeal swallowing abnormalities and to analyze their correlations with dysphagia and with overall neurological impairment.MethodsNeurological examinations were quantified using the Kurtzke Functional Systems and the Expanded Disability Status Scale (EDSS). Dysphagia was evaluated using the Dysphagia in Multiple Sclerosis (DYMUS) questionnaire, while fiberoptic endoscopic evaluation of swallowing (FEES) was used to establish the degree of aspiration and penetration, graded using the penetration–aspiration scale (PAS). The EPSS measured the duration of suprahyoid/submental muscle EMG activity (SHEMG-D), the duration of the laryngeal–pharyngeal mechanogram (LPM-D), and the duration of the pause in cricopharyngeal muscle EMG activity (CPEMG-PD); it also measured the interval between onset of the suprahyoid/submental muscle EMG activity (SHEMG) and onset of the laryngeal–pharyngeal mechanogram (I-SHEMG-LPM).Results92% of patients showed at least one electrophysiological abnormality. I-SHEMG-LPM correlated positively with the DYMUS questionnaire. I-SHEMG-LPM, SHEMG-D, and DYMUS correlated positively with the PAS. Moderate to severe bladder sphincter dysfunction was associated with a significant reduction, or absence, of CPEMG-PD.ConclusionEPSS improves our understanding of the pathophysiology of dysphagia in MS.SignificanceThis investigation could be useful in MS patients with swallowing abnormalities.  相似文献   

16.
Swallowing and feeding disorder (dysphagia) have high incidence and prevalence in children and adults with developmental disability. Standardized screening and clinical assessments are needed to identify and describe the disorder. The aim of this study was to describe the psychometric properties of the Dysphagia Disorder Survey (DDS), a screening and clinical assessment of swallowing and feeding function for eating and drinking developed specifically for this population. The statistical analysis was performed on a sample of 654 individuals (age range 8–82) with intellectual and developmental disability living in two residential settings in the United States that served somewhat different populations. The two samples had similar factor structures. Internal consistency of the DDS and subscales was confirmed using Chronbach's coefficient alpha. The DDS demonstrated convergent validity when compared to judgments of swallowing and feeding disorder severity made by clinical swallowing specialists. Discriminative validity for severity of disorder was tested by comparing the two samples. The results of the study suggest that the DDS is a reliable and valid test for identifying and describing swallowing and feeding disorder in children and adults with developmental disability.  相似文献   

17.
Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by a combination of autonomic failure plus cerebellar syndrome and/or parkinsonism. Dysphagia is a frequent and disabling symptom in MSA and its occurrence within 5 years of motor onset is an additional diagnostic feature. Dysphagia can lead to aspiration pneumonia, a recognized cause of death in MSA. Guidelines for diagnosis and management of dysphagia in MSA are lacking. An International Consensus Conference among experts with methodological support was convened in Bologna to reach consensus statements for the diagnosis, prognosis, and treatment of dysphagia in MSA. Abnormalities of the oral and pharyngeal phases of swallowing, esophageal dysfunction and aspiration occur in MSA and worsen as the disease progresses. According to the consensus, dysphagia should be investigated through available screening questionnaires and clinical and instrumental assessment (videofluoroscopic study or fiberoptic endoscopic evaluation of swallowing and manometry) at the time of MSA diagnosis and periodically thereafter. There is evidence that dysphagia is associated with poor survival in MSA, however effective treatments for dysphagia are lacking. Compensatory strategies like diet modification, swallowing maneuvers and head postures should be applied and botulinum toxin injection may be effective in specific conditions. Percutaneous endoscopic gastrostomy may be performed when there is a severe risk of malnutrition and pulmonary complications, but its impact on survival is undetermined. Several research gaps and unmet needs for research involving diagnosis, prognosis, and treatment were identified.  相似文献   

18.
BACKGROUND: Vocal fold motion impairment (VFMI), especially vocal fold abductor paralysis, is frequently seen in multiple system atrophy (MSA). Since the regulation system of laryngeal function is closely related to swallowing function, swallowing function is considered to be more involved in MSA patients with VFMI than in patients that do not have VFMI. However, the relationship between dysphagia and VFMI in MSA patients has not been systematically explored. OBJECTIVE: To elucidate the relationship between VFMI and dysphagia in MSA. METHODS: We evaluated swallowing function of 36 MSA patients with and without VFMI, by videofluoroscopy, and investigated the relationship between VFMI and pharyngeal swallowing function. RESULTS: VFMI was found in 17 patients (47.2%). Patients with VFMI had advanced severity of the disease. Although there was a tendency for bolus stasis at the pyriform sinus and the upper oesophageal sphincter opening to be more involved in patients with VFMI, statistical analysis did not show significant differences in swallowing function of MSA patients between with and without VFMI. In contrast, patients who underwent a tracheotomy ultimately required tube feeding or a laryngectomy. CONCLUSIONS: Appearance of VFMI is a sign of disease progression but does not necessary mean patients should change their way of taking nutrition. However, MSA patients who need a tracheotomy might have advanced to a high-risk group for dysphagia. Appropriate evaluation and treatment for VFMI and dysphagia are required to maintain patients' quality of life in MSA.  相似文献   

19.
The phenotype of myotonic dystrophy type 2 (DM2) shows similarities as well as differences to that of myotonic dystrophy type 1 (DM1). Dysphagia, a predominant feature in DM1, has not yet been examined in DM2. In a recent nationwide questionnaire survey of gastrointestinal symptoms in DM2, 12 out of 29 DM2 patients reported to have difficulty in swallowing for solid food. The aim of the study was to investigate the presence of dysphagia in patients with genetically proven DM2 who reported difficulty in swallowing for solid food at the questionnaire survey. Swallowing function and fiberoptic endoscopic evaluation of swallowing (FEES) were examined by a speech therapist and otorhinolaryngologist, respectively. In DM2 patients who reported difficulty in swallowing the presence of dysphagia could be confirmed (clinically in 100%, by FEES in 88%). A correlation exists between Dysphagia Outcome and Severity Score (DOSS) and age (p = 0.05). None of the patients was underweight, and none of the patients had suffered aspiration pneumonia in the past. Dysphagia is present among DM2 patients and is more severe in older patients. However, dysphagia is generally mild, and do not lead to weight loss, or aspiration pneumonia.  相似文献   

20.
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