ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1988.tb01413.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1988.tb01414.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

ABSTRACT.The present paper is a reply to the comments of Odor (1988) and Aitken (1988) on the present authors’ study concerning a computer‐aided programme for low‐functioning persons (Lancioni&Oliva, 1988). The research problem and the objectives of the study will be restated first. Subsequently, a series of issues on which Odor and Aitken have commented will be addressed. These issues include the activities presented to the subjects, the characteristics of the computer system, the differences between computer and card systems, intrinsic motivation and external reinforcement, the kind of subjects who can benefit from the computer system, and ethical as

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1988.tb01415.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

ABSTRACT.Screening of 1 042 000 Greek newborns for PKU using the Guthrie test, revealed a mean incidence 1: 10 420 for Hyperphenylalaninaemia (1:24 233 classic PKU and 1:18 280 benign variants). The mean Phe concentration of the first sample (3–15 days) was 14.1 mg/dl (6–40) for PKU while, for variants, it was 7.7 mg/dl (4.16). Using 4 mg/dl as the limit under which no repeat test was requested, no case of PKU has been missed. On the whole, 100 newborns with persistent Hphe have been detected: 43 proved to be classical PKU and 57 benign variants. After excluding BH4deficiency, Phe blood level has been kept below 10 mg/dl in all Hphe babies during the first 2 years of life, irrespective of the type of Hphe. All treated Hphe babies have developed normally. Discrimination between PKU and variants was achieved in the 6–18 month of life with protein loading tests. Treated PKU children had the lowest Phe concentrations during the first year of life (mean 5.1 mg/dl). From then on, a steady yearly increase was observed. Their mean IQ in the Stanford‐Binet L‐M dropped from 107.2 in the second year to 95.8 in the sixth year. A high negative correlation is not clearly shown, probably due to the small number of eases, PKU clinic (supportive and counselling group therapy) along with individual consultations have proved to be helpful, as has the promotion of diet compliance. The need to individualize each of Hphe before deciding upon ways of treatment is stressed and a protocol to be followed for each Hphe is

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1988.tb01416.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

ABSTRACT.Extinction responding of children with severe retardation was compared when one component of a multiple schedule (VR‐20/VR‐20) was interpolated with one session of additional reinforcement. Either variable ratio 10 (VR‐10), variable ratio 5 (VR‐5) or continuous reinforcement (CRF) were interpolated in one component prior to extinction in both. Interpolated reinforcement resulted in an extinction process at least as efficient as traditional methods. Total responding in the VR‐10 and VR‐5 components was less than in the comparable unchanged components. Results support and extend research with animals and humans. Results also suggest the feasibility of using such a procedure to gain control of behaviours that are controlled by intermittent schedules of reinforcement, are under multiple or inconsistent stimulus control or occur i

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1988.tb01417.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1988.tb01418.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

ABSTRACT.The administration of several compounds thai can increase plasma phcnylalanine levels and or inhibit phcnylalanine hydroxylase in rats was studied in order to determine iheir usefulness in inducing a phenylketirtiuria‐like state. The results of this investigation revealed that 4′5 um/10 g p‐chlorophenylalanine is more el'lective lhan L‐phcnylalaninc, a‐methylphenylalanine, trimelhoprim, Hactrim and Scptra, since the lormer auii Mukl julidiiced both adequate hyperphenylalaninemia and marked inhibition of hepatic phenylalanine hydroxylase activity. In addition, a 24‐h study provided important insights into the changing diurnal patterns of specified biochemical

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1988.tb01419.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

ABSTRACT.Socializing with peers and opposite‐sex relationships were examined in two subsets of a mildly mentally retarded (MMR) young adult population living in the community: those who were no longer receiving MR services and those who were attending day centres for MR adults. The MMR young people not receiving services had fewer best friends and socialized significantly less often than a nonretarded comparison population. The MMR males not receiving services also had fewer opposite‐sex relationships than comparisons but this difference was not significant for the females. At age 22, these MMR young people continued to see friends from school significantly less often than comparisons, indicating a possible detrimental effect of special schooling on later socializing with peers. The MMR young people at the day centres socialized more often but this was done almost wholly with others who were MR. Within the MMR study population, the young women not receiving services socialized with peers the least, less than the young women at the day centres and less than the young men not receiving services, but they were married most of

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1988.tb01420.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

ABSTRACT.This case‐control study was designed to determine whether aortic regurgitation (AR) and mitral valve prolapse (MVP) are specifically associated with Down's syndrome, or occur in a comparably retarded population without Down's syndrome. The 92 control subjects resided at the same institution as the 131 cases, and had mental retardation attributable to low birth weight or perinatal asphyxia. Mean (±SD) ages of the cases and the control group were 41 × 11 years compared with 40 × 13 years, respectively. The relative risk of AR among the cases (those with Down's syndrome) was 2.9 (95% confidence interval, 0.65–13.2; P=0.16) and that of MVP was 3.5 (95% confidence interval, 1.2–10.2;P=0.02). The results showed that these two valvular abnormalities are specifically associated with Down's syndrome i

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1988.tb01421.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

ABSTRACT.In this paper, the authors present a description of the clinical and psychological data of three girls with ring chromosome 15, These children were carefully observed and tested on their mental level and school skills, and the results are compared with the findings in the literature. In general, patients with ring 15 present a mild to moderate mental retardation, wish some language difficulties at early age, Observation of behaviour and social skills, present these patients as cordial, willing and with good social integration, despite their severe growth failure.

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1988.tb01422.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY