Brief report: a case of schizophrenia in a population with Down syndrome.
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Biomedical subjects
Publications and source records attributed to B A Myers.
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This study was designed to investigate the family environment and the temperament of children with Down's syndrome (DS). Parents of 40 children with DS completed the Family Environmental Scale (FES). They also were asked to fill out the Temperament Assessment Battery for Children (TABC) for both the child with DS and the nearest same-sex sibling. A similar questionnaire of the TABC (teacher form) was sent to the children's teachers. The results of these investigations revealed that there were high scores in categories cohesion, expressiveness, achievement, moral/religious emphasis, organization and control on the FES, indicating that family members in the study cohort are relationship oriented, provide support for one another, emphasize ethical values and are able to express their feelings. Comparing TABC results between children with DS with their siblings, significant differences were observed in such categories as adaptability, approach/withdrawal and persistence. Control children were scoring higher in the adaptability and persistence categories, whereas children with DS achieved significantly higher scores than the control children in the approach/withdrawal category.
Complete assessment of biventricular function from planar ECG-gated cardiac blood pool studies has been limited because of the overlap of adjacent activity-containing structures. Theoretically, single-photon emission tomography (SPET) can be used to comprehensively evaluate both ventricles by isolating them from surrounding anatomy. However, an enormous amount of parametric data is generated from gated SPET studies, and much of it is diagnostically irrelevant for ventricular wall motion analysis. To compress this information to a more easily interpretable format, a two-dimensional parametric display has been developed. Fourier analysis of short-axis tomograms from a gated cardiac blood pool SPET study generates three-dimensional, first-harmonic phase data. Circumferential profile data from the parametric tomograms of the right and left ventricle are mapped onto a two-dimensional polar display. This method is demonstrated in a normal patient and in three patients with abnormal ventricular contraction patterns and appears to have potential application for the analysis and characterization of biventricular wall motion.
Two studies evaluated the effects of soya bean trypsin inhibitor concentrate (STIC) on early stages of pancreatic carcinogenesis in Wistar rats. In experiment 1, the effects of a 3-month administration of diets containing 3.7% STIC were compared with the effects of administration of diets containing 20% corn oil, in rats pretreated with a single azaserine injection sufficient to initiate putative preneoplastic atypical acinar cell foci. Experiment 2 investigated the capacity of STIC to initiate pancreatic carcinogenesis. Diets containing 3.7% STIC were fed for 4 wk, then diets containing either 5 or 20% corn oil were fed for 3 months. Pancreases were quantitatively evaluated for foci. All groups of azaserine-initiated rats had large numbers of atypical acinar cell foci per cm3 of pancreas. Of these, the group fed 3.7% STIC had pancreatic foci that occupied a significantly greater (P less than 0.01) percentage volume of pancreas than did groups fed 20% corn oil or control diets, which contained 5% corn oil and no added trypsin inhibitor. Very few or no foci were observed in all other groups of either experiment 1 or 2. STIC had a much greater effect on the growth of azaserine-induced lesions than did corn oil. STIC alone did not appear to initiate pancreatic lesions.
This study was designed to provide information on the prevalence and nature of psychiatric disorders in 497 individuals with Down syndrome. There were 261 patients under 20 years of age and 164 patients 20 years and older, with both groups followed as outpatients, and 72 residents from a state school. The overall frequency of psychiatric disorders in our study population was 22.1%. Patients under 20 years of age often displayed disruptive behaviors, anxiety disorders, and repetitive behaviors. Individuals with Down syndrome 20 years and older who were followed as outpatients more often exhibited major depressive disorders and state school residents were found to have an increased prevalence of dementia.
Sexual offenses, particularly paraphilias, although infrequent, are committed by people with mental retardation. Their management poses clinical and ethical dilemmas. Available treatments were reviewed, especially the literature on the most effective agent, medroxyprogesterone acetate (MPA). A case history was presented of a young man with mild mental retardation who was successfully treated with MPA. The role of antiandrogen treatments of paraphilias among the mentally retarded population includes a consideration of informed consent and ethical aspects of treatment.
Fasted Bolivian squirrel monkeys (BoSM) exhibit a marked hyperbilirubinemia when compared to fed BoSM. This fasting hyperbilirubinemia (FH) is similar to that in human patients with Gilbert's syndrome. Endogenous bilirubin (BR) excretion (production) into bile was elevated two-fold in BoSM upon fasting. The fraction of injected dose of 3 H-amino-levulinic acid (ALA) incorporated into biliary BR in fasted monkeys was of less magnitude than in fed monkeys and was associated with lower specific activities of 3 H-BR. Both the lower incorporation of ALA and lower specific activities of 3H-BR in fasted BoSM suggest that increased BR excreted may have arisen from pre-existing non-labeled pools of either heme or BR.
1. Bolivian squirrel monkeys (BoSMs), which are animal models for Gilbert's syndrome, have 40% less hepatic bilirubin UDP-glucuronyltransferase (BR-UPPG-T) activity than Brazilian squirrel monkeys (BrSMs). 2. Although fasting results in similar decreases in hepatic UDP-glucose and UDP-glucuronate levels in both simian subspecies, increased activities (55%) of BR-UDPG-T are induced only in the fasted control BrSMs, which do not exhibit the marked fasting hyperbilirubinemia (FH). 3. Total hepatic bilirubin (BR) concentrations were 50% greater in both fed and fasted BoSMs when compared to BrSMs. 4. Hepatic unconjugated BR levels increase upon fasting only in Gilbert-like BoSMs, reaching concentrations twice that observed in BrSMs. 5. Elevated hepatic BR levels in fasted BoSMs may reflect BR overproduction or inadequate glucuronidation. 6. The increased BR-UDPG-T activity induced in BrSMs during fasting could compensate in-part for the UDPGA depletion and prevent the marked FH as observed in BoSMs.
The Comprehensive Health Effects Testing Program for the Denver Water Department's Potable Water Reuse Demonstration Project is designed to evaluate the relative health effects of highly treated reclaimed water derived from secondary wastewater compared to Denver's present high-quality drinking water. The 1 million gallon per day (1 mgd) demonstration plant provides water to be evaluated in the studies treating unchlorinated secondary treated wastewater with the following additional processes: high pH lime clarification, recarbonation, filtration, ultraviolet irradiation, activated carbon adsorption, reverse osmosis, air stripping, ozonation, and chloramination. An additional sample is obtained from the identical treatment process substituting ultrafiltration for reverse osmosis. The toxicology tests to evaluate the possible long-term health effects are chronic toxicity and oncogenicity studies in Fischer 344 rats and B6C3F1 mice and reproductive/teratology in Sprague-Dawley rats. The results of these evaluations will be correlated with microbiological, chemical, and physical test results to establish the relative quality of reclaimed water compared to all established health standards as well as Denver's pristine drinking water.
Fasted Brazilian squirrel monkeys (BrSMs) exhibited slightly higher serum bilirubin levels (0.30 +/- 0.05 mg/dl) than others in the fed state (0.13 +/- 0.01). The mean liver weight was 50% lower following a 22 h fast. The rate of bile flow was unaffected by fasting and averaged 13.8 microliters/min/kg and 47.5 microliters/min/100g liver in six BrSMs. No significant difference in mean bilirubin excretion/min was observed on a body weight basis following fasting. When the mean rate of bilirubin excretion was calculated as a function of liver weight, a two-fold higher rate was present in fasted monkeys, but only at the p = 0.06 level of statistical significance. From data collected in this and earlier studies, it would appear that BrSMs represent the best animals studied to date to serve as experimental controls in comparative studies with Bolivian squirrel monkeys which exhibit a Gilbert-like syndrome.
The diagnosis of behavioral and cognitive disorders in preschool children is difficult. Infantile austism is more likely to be diagnosed than is mental retardation. Yet most children with infantile autism are also mentally retarded, and many of those with severe and profound mental retardation show autistic behavior. Factors misleading professionals into overlooking mental retardation when assessing preschool children were discussed, and essential components in the management of preschool children with behavioral, developmental, or cognitive deviations were examined.
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Bolivian (Bo) and Brazilian (Br) squirrel monkeys (SMs) were captured and restrained to determine patterns of glucagon and glucose response. In both subspecies, plasma glucagon and glucose levels rose rapidly in response to restraint procedures; glucagon levels in BrSMs and glucose levels in both subspecies returned to basal values in 15 minutes. The magnitude of the glucagon and glucose responses differed significantly between BoSMs and BrSMs.
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The Mini Mental State Examination is a useful screen for the detection of cognitive disorders among psychiatric patients. In the absence of a developmental disability, the inability to perform satisfactorily on the Mini Mental State (MMS) (score less than 24) is likely to be related to delirium, dementia, or affective disorders. The mildly retarded or learning disabled should perform satisfactorily (greater than 24) on the MMS but may show impairment with delirium, dementia, or affective disorders. In the absence of a psychiatric disorder, it takes moderate mental retardation (IQ less than 55) or severe reading disability with IQ less than 70 to produce an MMS score of less than 24. The MMS should not be considered a screen for mental retardation, because retarded individuals can function adequately (greater than 24) on the MMS.
Intellectual and behavioral deterioration in an individual who is mentally retarded presents a diagnostic challenge to the clinician, since the deterioration may be the result of the primary disorder or a new process. The mentally retarded individual's limitations in communication make the diagnostic assessment more difficult. The authors report the case of pseudodementia in a mentally retarded teenager presumed to have been caused by impacted otic cerumen and corrected by removal of the cerumen. The discussion includes a review of medical, neurologic, psychiatric, and environmental causes of intellectual and behavioral deterioration.