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1.
Little is known about the behavioral characteristics of adults with Down syndrome (DS) without dementia. The main purpose of this study was to investigate the psychopathology and social behavior among adults with DS compared to adults with nonspecific intellectual disability (NSID). Thirty-four adults with DS were individually matched with 34 adults with NSID for raw scores on a task that measured nonverbal reasoning ability. Participants' caregivers completed 3 questionnaires assessing mental illness, behavior problems, and social behavior. The group with DS presented fewer difficulties associated with mental illness and behavior problems, as well as better adapted social behavior, than the group with NSID despite a similar level of nonverbal reasoning ability. Aging in the group with DS was found to be positively correlated with behavioral signs of depression and autistic symptoms. These findings highlight the specificity of the behavioral profile of adults with DS without dementia.  相似文献   

2.
There is a scarcity of follow‐up studies into adult life of psychiatric disorder in young people with intellectual disability. The key aims of the present study were: (1) to determine the outcome of psychopathology present in childhood in individuals with Down's syndrome (DS); and (2) to look at childhood predictors of adult psychiatric disorder. Fifty‐two young people with DS were identified from a sample of 193 subjects examined in childhood and adolescence for psychiatric and behaviour disorder. These young adults were interviewed for the presence of psychiatric disorder. No significant relationship was found between childhood mental disorder and psychiatric disorder in adult life for those individuals with DS. Early childhood factors of psychiatric disorder, challenging behaviour and family environment, except social background, did not predict adult psychopathology in young people with DS. Childhood disorder in individuals with DS has a good early prognosis with little evidence of continuity of the disorder into adult life.  相似文献   

3.
Background : 22q11.2 Deletion Syndrome (22q11.2 DS) is a common micro‐deletion syndrome associated with intellectual disability, brain abnormalities and a complex spectrum of psychiatric disorders in both adults and children. Many previous studies have shown that adults with 22q11.2 DS have approximately thirty times greater risk for the developing schizophrenia compared to the general population. Furthermore, studies of children and adolescents with 22q11.2 DS have found high rates of psychotic symptoms, ADHD and anxiety disorders. In this study we initially aim to characterize the psychiatric and neuropsychological phenotype of all individuals in Ireland with 22q11.2 DS and then correlate our findings with genetic association studies and brain imaging. We then intend to undertake a longitudinal study to assess for risk factors of future psychotic illness. Methods : Forty‐four individuals with 22q11.2 DS (Mean age = 14 years, SD = 9) were compared to 25 non‐affected sibling controls (Mean age = 12 years, SD = 4). Psychiatric phenotype assessment was performed using a range of standardized clinical assessments including, the Diagnostic Interview Schedule for Children (DISC), Psychotic Supplement of K‐SADS, Comprehensive Assessment of At Risk Mental State (CAARMS), the Schedule for Clinical Assessment in Neuropsychiatry (SCAN), Child Behaviour Checklist (CBCL) and the Social Communication Questionnaire (SCQ). Results : Preliminary data indicates that individuals in the 22q11.2 DS group have a higher prevalence of psychiatric disorders including; ADHD (41%), psychotic symptoms (19%), schizophrenia (2.7%), specific phobias (38%), depressive episodes (11%), and anxiety disorders (32%). Conclusion : This is the first stage in a longitudinal follow‐up study of individuals with 22q11.2DS in Ireland. Our preliminary results are consistent with previous findings of high rates of psychiatric disorders in people with 22q11.2DS. The challenge remains to identify precursor symptoms in childhood that predicts the later development of psychiatric disorders, particularly schizophrenia in this vulnerable group.  相似文献   

4.
The literature on the association between behavioural and emotional problems and ageing in adults with Down syndrome (DS) without dementia is limited and has generally not reported on a wide range of behavioural and emotional problems. This research aimed to extend the field by examining the associations between age and a wide spectrum of behavioural and emotional problems in adults with DS without dementia. A preliminary analysis of the association between potential covariates and behavioural and emotional problems was also undertaken.Parents and caregivers completed a questionnaire on behavioural and emotional problems for 53 adults with DS aged between 16 and 56 years. Twenty-eight adults with DS and their caregivers were part of a longitudinal sample, which provided two time points of data approximately four years apart. Additionally, 25 participants with DS and their caregivers were from a cross sectional sample, which provided one time point of data. Random effects regression analyses were used to examine the patterns in item scores for behavioural and emotional problems associated with age.No significant associations between age and the range or severity of any behavioural and emotional items were found. This suggested a more positive pattern for ageing adults with DS than has been previously described. Given that behavioural and emotional problems were not associated with age, investigation into other factors that may be associated with the behavioural and emotional difficulties for adults with DS is discussed.  相似文献   

5.
Adults with Down syndrome (DS) are at risk for developing Alzheimer's disease (AD). While plasma amyloid-β (Aβ) is known to be elevated in DS, its relationship to cognitive functioning is unknown. To assess this relationship, samples from two groups of subjects were used. In the first group, nondemented adults with DS were compared to: 1) a group of young and old individuals without DS and 2) to a group of patients with AD. Compared to these controls, there were significantly higher levels of plasma Aβ in nondemented adults with DS while AD patients showed lower levels of plasma Aβ. A larger second group included demented and nondemented adults with DS, in order to test the hypothesis that plasma Aβ may vary as a function of dementia and Apolipoprotein E (ApoE) genotype. Plasma Aβ levels alone did not dissociate DS adults with and without dementia. However, in demented adults with DS, ApoE4 was associated with higher Aβ40 but not Aβ42. After controlling for level of intellectual disability (mild, moderate, severe) and the presence or absence of dementia, there was an improved prediction of neuropsychological scores by plasma Aβ. In summary, plasma Aβ can help predict cognitive function in adults with DS independently of the presence or absence of dementia.  相似文献   

6.
22q11.2 deletion syndrome (22q11DS) has a complex phenotype with more than 180 characteristics, including cardiac anomalies, cleft palate, intellectual disabilities, a typical facial morphology, and mental health problems. However, the variable phenotype makes it difficult to predict clinical outcome, such as the high prevalence of psychosis among adults with 22q11DS (~25-30% vs. ~1% in the general population). The purpose of this study was to investigate whether subtypes exist among people with 22q11DS, with a similar phenotype and an increased risk of developing mental health problems. Physical, cognitive and behavioural data from 50 children and adolescents with 22q11DS were included in a k-means cluster analysis. Two distinct phenotypes were identified: Type-1 presented with a more severe phenotype including significantly impaired verbal memory, lower intellectual and academic ability, as well as statistically significant reduced total brain volume. In addition, we identified a trend effect for reduced temporal grey matter. Type-1 also presented with autism-spectrum traits, whereas Type-2 could be described as having more 22q11DS-typical face morphology, being predominately affected by executive function deficits, but otherwise being relatively high functioning with regard to cognition and behaviour. The confirmation of well-defined subtypes in 22q11DS can lead to better prognostic information enabling early identification of people with 22q11DS at high risk of psychiatric disorders. The identification of subtypes in a group of people with a relatively homogenous genetic deletion such as 22q11DS is also valuable to understand clinical outcomes.  相似文献   

7.
22q11.2 deletion syndrome (22q11DS) has a complex phenotype with more than 180 characteristics, including cardiac anomalies, cleft palate, intellectual disabilities, a typical facial morphology, and mental health problems. However, the variable phenotype makes it difficult to predict clinical outcome, such as the high prevalence of psychosis among adults with 22q11DS (~25–30% vs. ~1% in the general population). The purpose of this study was to investigate whether subtypes exist among people with 22q11DS, with a similar phenotype and an increased risk of developing mental health problems. Physical, cognitive and behavioural data from 50 children and adolescents with 22q11DS were included in a k-means cluster analysis. Two distinct phenotypes were identified: Type-1 presented with a more severe phenotype including significantly impaired verbal memory, lower intellectual and academic ability, as well as statistically significant reduced total brain volume. In addition, we identified a trend effect for reduced temporal grey matter. Type-1 also presented with autism-spectrum traits, whereas Type-2 could be described as having more 22q11DS-typical face morphology, being predominately affected by executive function deficits, but otherwise being relatively high functioning with regard to cognition and behaviour. The confirmation of well-defined subtypes in 22q11DS can lead to better prognostic information enabling early identification of people with 22q11DS at high risk of psychiatric disorders. The identification of subtypes in a group of people with a relatively homogenous genetic deletion such as 22q11DS is also valuable to understand clinical outcomes.  相似文献   

8.
9.
BackgroundThe Strengths and Difficulties Questionnaire (SDQ) has been widely used to screen typically developing children for mental health problems; in recent years it has also been used with children with intellectual disabilities. The present study investigated the possible use of the SDQ to screen adults with Down syndrome (DS).MethodOnly four items on the SDQ were changed slightly to remove references to children. Parents or carers completed the SDQ and all 125 adults with DS (aged 18–43 years) were assessed for mental health problems by a psychiatrist.ResultsTwenty-eight adults were diagnosed with a psychiatric condition. Parents and carers found the SDQ easy to complete and liked the inclusion of positive behaviours. The SDQ did discriminate significantly between those with and without a diagnosis, however the sensitivity and specificity were insufficient. Exploratory and Confirmatory Factor Analyses showed a four factor model (pro-social behaviour, emotional difficulties, hyperactivity and conduct disorders) to be the best solution. This structure was similar to previous research findings of three factors of pro-social behaviour, internalising disorders and externalising disorders.ConclusionConstruct validity and reliability suggest that the SDQ has potential for use with adults with DS and possibly those with other intellectual disabilities. The SDQ is user friendly for parents and carers, and did highlight behavioural and mental health needs, suggesting that it would be worthwhile to develop the SDQ specifically for adults with intellectual disability.  相似文献   

10.
Background Aging adults with Down's syndrome (DS) experience more relocations and other life events than adults with intellectual disabilities aged 50 and older without DS. Age‐related functional decline and the higher incidence of dementia were implicated as the contributing factors that led to relocation and nursing home placement. Method A retrospective study of adults with intellectual disabilities who were born prior to the year 1946 was conducted to analyse the number of relocations experienced over a 5‐ and 10‐year period. The cohort consisted of 140 individuals (61 with DS between ages 50–71 years, and 79 without DS between ages 57–89 years) who had been referred to a diagnostic and research clinic. Results Analyses revealed the number of relocations over a 5‐ and 10‐year period were significantly greater in the DS group. Placement in a nursing home for end of life care was significantly higher in the DS group whereas the majority (90%) in the non‐DS group remained in a group home setting. Mortality was significantly earlier in the DS group with the mean age at death to be 61.4 years compared with 73.2 years in the non‐DS group. Conclusions The present results suggest that aging adults with DS encounter more relocations, and are more likely to have their final placement for end of life care in a nursing home. In contrast, the adults without DS were subjected to less relocation and remained in the same group home setting.  相似文献   

11.
The present study sought to examine the longitudinal psychoeducational, neurocognitive, and psychiatric outcomes of children and adolescents with chromosome 22q11.2 deletion syndrome (22q11DS), a population with a high incidence of major psychiatric illnesses appearing in late adolescence/early adulthood. Little is known of the developmental changes that occur in the early teen years, prior to the age of highest psychosis risk. Data were collected from 71 participants (42 subjects with 22q11DS and 29 control subjects) at Time 1 (T1) and Time 2 (T2), approximately 3.5 years later. The 22q11DS group was significantly lower functioning than controls on IQ, neurocognition, and academic achievement at both T1 and T2. Children with 22q11DS also showed significantly greater social-behavioral difficulties and psychiatric symptoms, and were more likely to meet criteria for psychiatric disorders at both time points. In evaluating change over time from T1 to T2, the 22q11DS group did not show significant changes in psychoeducational or psychiatric outcomes relative to the controls, however, lack of expected age-related gains in attention regulation were noted. Within the 22q11DS group, an increase in the Attenuated Prodrome for Schizophrenia (number of psychiatric symptoms) was noted from T1 to T2 and four children with 22q11DS met criteria for Psychosis for the first time. Predictors at T1 that uncovered psychopathology symptoms at T2 included full-scale IQ, externalizing symptoms, and problem social behaviors. Overall, younger adolescent and preadolescent children with 22q11DS in this study exhibited slowed growth in attention regulation, with an increase in subclinical symptoms of schizophrenia, suggestive of increasing impairments in domains that are relevant to the high risk of Schizophrenia. Early predictors of later psychopathology included both cognitive and behavioral abnormalities. These findings begin to elucidate the trajectory of changes in psychopathology in children with 22q11DS in the years leading up to the onset of major psychiatric illnesses.  相似文献   

12.
ABSTRACT. This article reports on age-specific findings of mental health problems among residents with Down's syndrome (DS) (n=307) and without (non-DS) (n=1274 in dutch group homes and institutes for people with mental retardation. Whereas a proportional increase of psychological problems was found for elderly DS persons with severe mental retardation, non-DS residents did not show such age-specific differences. High ratings of psychological problems for the elderly DS residents corresponded very well with the diagnosis of 'dementia' made by the physicians. Only for non-DS persons with severe mental retardation was a proportional decrease of challenging behaviour found with advanced age. Whereas psychological problems in elderly DS persons could be explained for the greater part of the diagnosis 'dementia', challenging behaviour-although also common in elderly DS-was shown to be a more independent phenomenon. With regard to psychiatric diagnosis, non-DS residents with mild retardation had six times as much a mental disorder, and non-DS residents with severe or profound mental retardation had up to 15 times as much, compared with their DS peers.  相似文献   

13.
OBJECTIVE: This study examined psychiatric symptom exacerbation (or deterioration) among patients treated for substance use disorders. The study examined several questions. What is the prevalence of deterioration during residential treatment? Compared with patients who show improvement in psychiatric symptoms, do those with deterioration of symptoms report poorer functioning during treatment and one year after treatment entry? Do patients who experience deterioration of symptoms one year after treatment entry enter treatment with more problems and poorer coping skills? Is the prevalence of deterioration associated with treatment orientation, and do patients whose psychiatric symptoms deteriorate view the treatment environment more negatively than patients whose symptoms improve? METHODS: A total of 3,322 male patients were recruited at 15 residential substance abuse treatment programs in the Department of Veterans Affairs health care system between 1992 and 1995. On the basis of changes in psychiatric symptoms during treatment, these patients were categorized as having either deteriorated or improved psychiatric symptoms. Patients whose symptoms deteriorated were compared with a matched group of patients with improved symptoms. RESULTS: A total of 426 patients (13 percent) reported a worsening of psychiatric symptoms during treatment. Compared with patients in the improved group (N=426), patients in the deteriorated group reported more psychiatric problems and substance use one year after treatment. Patients in the deteriorated group were more likely to have a psychotic diagnosis, lower self-efficacy, and more reliance on coping by expression of emotions and to view the treatment experience more negatively. CONCLUSIONS: Thirteen percent of the patients experienced an exacerbation of psychiatric symptoms during residential substance use disorder treatment. Improved monitoring procedures, such as regular assessments for changes in psychiatric symptoms, are needed to routinely obtain information about declines in psychiatric conditions during treatment.  相似文献   

14.
To ascertain the influence of juvenile-onset chronic physical diseases and associating factors of social environment on delayed social maturation and mental health disorders in young adults, we analysed a group of 407 (184 female, 223 male) subjects with these conditions and compared the results with those of 123 (63 female, 60 male) healthy controls studied at the age of 19-25 years. The social maturation index was formed on the basis of a demographic interview, which also reviewed the state of social development and the family situation during childhood. Mental health disorders were assessed with a Present State Examination (PSE) interview analysed with the CATEGO program. With regard to social maturation at least half of the patients and controls were doing well, whereas for 29% (CI(95), 25%-33%) of the patients and 17% (CI(95), 10%-24%) of the controls the index showed delayed maturation. Subjects with poor social maturation were found most often among the disabled patients but also among the patients without severe diseases. The prevalence of PSE-CATEGO-identified psychiatric syndromes was equal in the patients and the controls (22% versus 20%). However, the patients with severe or disabling diseases had more severe psychiatric syndromes. The prevalences of depressive syndromes were also equal, but the depression of the patients was more often a profound affective disorder. Male sex, poor scholastic and vocational success, and social problems in the family during childhood were significantly associated with poor social maturation. On the other hand, the most significant predictors of mental health problems in young adults were female sex, family distress during childhood, and a severe disease. Juvenile-onset physical disease was considered to delay social maturation in some subjects and to deepen or modulate the clinical picture of mental health disorders. It is concluded that juvenile-onset physical diseases combined with family-related factors affect in different ways the social growth and psychiatric well-being. The results suggest that the subjects with chronic diseases during childhood should be thoroughly assessed by a child psychiatrist to evaluate the orientation of psychological development and the impact of the child's disease on the family and to ensure balanced psychological and social growth.  相似文献   

15.
OBJECTIVES: Although nystagmus has been reported in Down syndrome (DS), it has been poorly characterized, because most investigators have relied on clinical observations rather than on eye movement recordings. This study was conducted to investigate nystagmus in DS, using quantitative measurements of eye movements. METHODS: Ocular motility and visual functions were examined in 26 unselected adults with DS and compared with those in an age-matched group of 35 subjects with other causes of mental retardation. The eye movements of those with clinically evident nystagmus were recorded with the infrared technique. We also recorded the eye movements of a child with DS and nystagmus. RESULTS: Nystagmus was identified in six (23%) adults with DS and in none in the control group. All six patients showed latent/manifest latent nystagmus (LMLN), prominent with the covering of one eye, and esodeviations of 10 to 30 prism diopters. Eye movement recordings confirmed LMLN with its exponentially decaying waveform. Frequencies ranged from 2 to 5 Hz and amplitudes from 5 degrees to 20 degrees. While attempting to fixate straight ahead in the absence of visual cues, three subjects exhibited shifts in the mean eye position. In contrast with the findings in adults, the only child with DS examined had both congenital nystagmus and LMLN waveforms. CONCLUSIONS: The predominant type of nystagmus in the study subjects with DS is LMLN. The high prevalence of LMLN may reflect abnormal integration of visuospatial information that is typical of DS. The concurrent presence of congenital nystagmus in a child but only LMLN in the adults with DS raises the possibility of age-related waveform changes or could reflect sample variation.  相似文献   

16.
Background Although the prevalence of mental illness and behaviour problems is lower in adults with Down syndrome (DS) than in other populations with intellectual disabilities, they do present emotional and relational problems, as well as social integration difficulties. However, studies reporting on specific competences known to be central in developing appropriate social relationships (e.g. social reasoning, emotion processing, theory of mind) remain rare in the adult DS population and the mechanisms underlying these people's emotional and relational difficulties are unclear. Method The present study investigated the ability to understand the appropriateness of others' social behaviour in 34 adults with DS, using the Social Resolution Task (SRT). Their results were compared with those of 34 typically developing (TD) children matched for gender and receptive vocabulary. The relationships among the SRT experimental task, cognitive competences (receptive and productive vocabulary, non‐verbal reasoning, inhibition, selective attention) and a caregiver‐rated measure of socio‐emotional behaviour were examined in the DS group. Results The DS participants' global SRT scores did not differ from those of the controls. However, analyses of the SRT subscores revealed that the DS group identified significantly fewer inappropriate situations than the control group. Nevertheless, when they correctly identified the behaviour as inappropriate, they were as well as the controls to explain the rules underlying their responses. Regression analyses showed that receptive vocabulary and selective attention and a specific dimension of the socio‐emotional profile (social relating skills) constituted the best predictors of the DS adults' performance on the SRT. Conclusions The main findings show that the DS participants demonstrate relatively good social reasoning skills in comparison with TD children matched for verbal age. However, the two groups present distinctions in their response patterns, and the influence of cognitive variables on success on the SRT also appears different. While selective attention skills are found to be significant predictors for both groups, the influence of receptive vocabulary level is much stronger in the DS group. The implications of particular cognitive and socio‐emotional factors for success on the SRT in this group are considered in more detail.  相似文献   

17.
Background Although distinctive neuropsychological impairments have been delineated in children with chromosome 22q11 deletion syndrome (22q11DS), social skills and social cognition remain less well‐characterised. Objective To examine social skills and social cognition and their relationship with neuropsychological function/behaviour and psychiatric diagnoses in children with 22q11DS. Methods Sixty‐six children with 22q11DS and 54 control participants underwent neuropsychological testing and were administered the Diagnostic Analysis of Non‐Verbal Accuracy (DANVA) for face and auditory emotion recognition, a measure of social cognition: their parents/guardians were administered the Social Skills Rating System (SSRS) – parent version, Child Behavior Checklist (CBCL) – parent version and the Computerised Diagnostic Interview Schedule for Children (C‐DISC). Results The 22q11DS group exhibited significantly lower social skills total score and more problem social behaviours, lower neurocognitive functioning, higher rates of anxiety disorders and more internalising symptoms than the control group. Participants with 22q11DS also exhibited significant deficits in their ability to read facial expressions compared with the control group, but performed no differently than the control participants in the processing of emotions by tone of voice. Within the 22q11DS group, higher social competency was correlated with higher global assessment of functioning and parental socio‐economic status. Social competency was worse in those with anxiety disorders, attention deficit hyperactivity disorder, more than two psychiatric diagnoses on the C‐DISC and higher internalising symptoms. No significant correlations of SSRS scores were seen with IQ, executive functions, attention, or verbal learning and memory. No correlations were found between social cognition and social skill scores. Conclusion Our results indicate that social skills in children with 22q11DS are associated with behaviour/emotional functioning and not with neurocognition. Thus, treating the behaviour or emotional problems such as attention deficit hyperactivity disorder and anxiety disorders may provide a pathway for improving social skills in these children.  相似文献   

18.
Background This preliminary study served as a pilot for an ongoing analysis of spectral power in adults with Down syndrome (DS) using a 151 channel whole head magnetoencephalography (MEG). The present study is the first step for examining and comparing cortical responses during spontaneous and task related activity in DS. Method Cortical responses were recorded with a 151 channel whole head MEG system in three adults with DS and three age‐matched adults without DS. MEG data were obtained at rest with eyes open and during observation of point‐light displays of human motion and object motion. Data from both groups were evaluated by spectral analysis. Results The preliminary results showed greater alpha (8–14 Hz) power particularly in the occipital and parietal areas during the eyes open condition in the adults with DS in relation to a normal comparison group. The visual task had little effect on alpha power in the comparison group. Engaging in the visual task reduced power in alpha across all regions in the DS group to the level observed in comparisons. In the gamma band (30–50 Hz), power values were similar across both groups for the eyes open condition. In the comparison group, large reductions in gamma were observed in the occipital and bilateral temporal areas during the visual task. This change was not observed in the DS group. Conclusions The results from this pilot study suggest that MEG may be useful in characterizing task‐specific changes in cortical activity in individuals with DS. Future studies with a larger group of individuals will further contribute to our understanding of the neurophysiology of Down syndrome.  相似文献   

19.
IntroductionAutism and behavioral characteristics in adults with Dravet syndrome (DS) have rarely been systematically studied.MethodThree scales were used to assess the outcomes of DS in adulthood in terms of autism and behavior. All the adult patients with DS, nine male and four female, aged between 18 and 60 years, living at the Epilepsy Center Kempenhaeghe in The Netherlands were included in the study. In addition, the past medical history of each patient was systematically screened for diagnoses like autism, Pervasive Development Disorder-Not Otherwise Specified (PDD-NOS), autism spectrum disorder (ASD), hyperactivity, Attention Deficit Hyperactivity Disorder (ADHD), and self-mutilation. Information concerning past and current use of psychoactive drugs was also evaluated.ResultsEight patients (61.5%) were classified as having autism spectrum disorder (ASD) according to the AVZ-R or according to the medical record. Self-mutilation was seen in four patients (30.8%), hyperactivity in none. Three patients (23.1%) currently used psychoactive drugs.ConclusionAutism spectrum disorders persist in adult patients with DS, while certain characteristics associated with behavioral problems, such as hyperactivity or use of psychoactive medication, seem to be less prominent than in childhood.  相似文献   

20.
ABSTRACT. Down's syndrome (DS) is, associated with respiratory infections, lymphoblastic leukemia and a mortality rate that is elevated particularly during the first 5 years of life. Unfortunately, most studies on immune function in DS have been performed on adults or older children, the results of which may be not relevant to a primary immune defect in DS since maturation of the immune system and compensatory mechanisms may mask to a certain degree primary immune defects which are associated with this syndrome. In the present study, several immune and hematologic parameters wert studied in DS children under 6 years of age and in age-matched controls. The children with DS exhibited a reduced number of lymphocytes while the total number of white blood cells was normal. In addition, the children with DS had reduced numbers of circulating OKT4+ cells and a significantly depressed ratio of OKT4+ to OKT8+ cells, but their overall proportion of T-cells was normal. Normal NK activity was observed and Cells from DS children were no more sensitive to the augmenting effects of alpha interferon than cells from control children. Hematocrit levels were slightly elevated in the DS group while plasma zinc levels appeared to be normal in this group.  相似文献   

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