摘要:

Adipocyte plasma membranes were isolated from four patients with type la pseudohypoparathyroidism, a disease in which deficiency of the stimulatory guanine nucleotide binding protein Gshas been reported, and from controls. Stimulation of adenylate cyclase by isoproterenol was defective, whereas inhibition of forskolin-stimulated cyclase activity by N6-(phenylisopropyl)adenosine was normal. The patients had low serum FFA concentrations and developed obesity in childhood. These results suggest that pseudohypoparathyroidism 1a is connected with a blunted stimulatory response of adenylate cyclase, possibly because of low Gsactivity, and that this blunted response may lead to decreased lipolysis and to obesity.

ISSN:0031-3998    

出版商:OVID    

年代:1994

数据来源: OVID

摘要:

To explain the pathogenesis of excessive copper accumulation in Long-Evans Cinnamon (LEC) rats, regarded as one of the animal models for hepatic-type Wilson's disease, we measured copper contents in liver tissue and bile, serum total copper concentration, and ceruloplamin oxidase activity in LEC rats before and after the onset of spontaneous hepatitis. The copper contents in liver tissue of both 11-wk-old and 18-mo-old LEC rats were about 60 times the amounts in age-matched Wistar and Long-Evans Agouti rats. The biliary copper excretion in 11-wk-old LEC rats was significantly lower than that of the Long-Evans Agouti and Wistar rats that were the same age (27.9 and 41.4%, respectively). In 18-mo-old LEC rats, biliary copper excretion was lower than that in the Long-Evans Agouti rats that were the same age, but the finding was statistically not significant. Serum copper and cerulo-plasmin levels were markedly reduced in LEC rats of both ages. These findings suggest that LEC rats have similar defects of biliary copper excretion as observed in patients with Wilson's disease.

ISSN:0031-3998    

出版商:OVID    

年代:1994

数据来源: OVID

摘要:

To assess relationships of total plasma cholesterol (TC) and triglyceride (TG) values to suicide, suicide ideation, and hospitalization for psychiatric disease, we studied 220 children, ages 5 to 18 y, hospitalized with affective, adjustment, disruptive, anxiety, schizophrenic, other, and organic psychiatric disorders. The 135 male and 85 female patients had higher TG values (p = 0.0001 and 0.0003, respectively) and higher Quetelet Indices (p = 0.0001 and 0.003, respectively) than the 732 male and 316 female schoolchild controls; male patients had higher TC values than male controls (p = 0.014). Substance abuse in patients was an independent inverse determinant of TC value (p = 0.05); TG value correlated positively with alcohol use (p ≤ 0.1) and sustance abuse (p < 0.05). After covariance adjustment for age, race, sex, and Quetelet, children having adjustment disorders with depression had much lower covariance-adjusted TC value than control schoolchildren (3.91versus4.29 mmol/L, p = 0.003), whereas those with disruptive behavior with oppositional defiant disorder had much higher adjusted TC value (5.09 mmol/L, p = 0.0001). After covariance adjusting for age, race, sex, Quetelet, cigarette smoking, alcohol use, and substance abuse, children having adjustment disorders with concomitant depression had the highest group suicide tendencies (attempts and ideation) and the lowest covariance-adjusted TC value (4.03 mmol/L). Conversely, children having disruptive behavior with attention deficit hyperactivity disorder or disruptive behavior with oppositional defiant disorder had 50% lower suicide index than those with adjustment disorders with concomitant depression and higher adjusted TC levels (4.45 and 5.12 mmol/L, p = 0.0003). Children with psychiatric hospitalisations have high TG values, and those with adjustment disorders with concomitant depression have low TC values, which is positively associated with suicide tendencies. We speculate that the associations between low TC level, high TG level, suicide tendency, and hospitalization for psychiatric disease do not reflect cause and effect but may be epiphenomena.

ISSN:0031-3998    

出版商:OVID    

年代:1994

数据来源: OVID

摘要:

In the course of the German Collaborative PKU Study, the results of the Personality Questionnaire for Children (PFK 9–14) of 58 10-y-old patients treated early for phenylkctonuria were analyzed. The main message of our data is that patients with phenylketonuria who were treated early and strictly did not show a higher risk for severe emotional and behavioral maladjustment compared with healthy controls at the age of 10 y. The patients were characterized only by “less masculinity of attitudes.” Because no significant correlations were observed between the quality of dietary control and the results of the questionnaire, the noticed personality characteristic of the patients could be interpreted as a psychosocial consequence of the chronic disease.

ISSN:0031-3998    

出版商:OVID    

年代:1994

数据来源: OVID

摘要:

Morphologic appearance and metabolic disturbances of the brain of a patient with L-2-hydroxyglutaric acidemia were investigated with use of magnetic resonance imaging and localized proton magnetic resonance spectros-copyin vivo. Whereas magnetic resonance imaging revealed increased internal and external ccrebrospinal fluid spaces as well as patchy white matter lesions, metabolic deviations included a 50% decrease of N-acetylaspartate (neuronal marker), a 75% increase ofmyo-inositol (glial marker), and a 40% decrease of choline-containing compounds in white matter relative to age-matched controls. A clinical deterioration of the patient was clearly reflected in a follow-up examination 22 mo later, resulting in a further reduction of N-acetylaspartate and a more pronounced enhancement ofmyo-inositol. No elevation of lactate was observed. The magnetic resonance spectroscopy findings are in line with a generalized neurodegcnerative process in L-2-hydroxyglutaric acidemia but also suggest a defect in phosphatidyl inositol metabolism of glial cells.

ISSN:0031-3998    

出版商:OVID    

年代:1994

数据来源: OVID

摘要:

Despite the increasing use of tetrapolar whole-body bioelectric impedance (BI) analysis in the assessment of body composition, its usefulness in estimating fat-free mass (FFM) has not been evaluated in comparison with conventional skinfold anthropometry in children. We therefore compared 1) the intraobserver and interobserver reproducibility of BI and skinfold measurements and the derived FFM estimates, and 2) the predictability of FFM as calculated from measurements of total body potassium (TBK) using40K spectrometry by equations based on either BI or skinfold measurements in 112 healthy children, adolescents, and young adults aged 3.9 to 19.3 y. A best-fitting equation to predict TBK-derived FFM from BI and other potential independent predictors was developed and cross validated in two randomly selected subgroups of the study population by stepwise multiple regression analysis. Although the technical error associated with BI measurements was much smaller than that of skinfold measurements, the reproducibility of BI-derived FFM estimates (intraobserver coefficient of variation [CV], 0.39%; inter-observer CV, 1.23%) was only slightly better than that of FFM estimates obtained by use of weight and two skinfold measurements (0.62% and 1.39%, respectively). The cross validation procedure yielded the following best-fitting prediction equation: FFM = 0.65. (height2/impedance) + 0.68-age + 0.15 (R2= 0.975, root mean square error = 1.98 kg, CV = 5.8%, 95% limits of agreement = −11.1% to + 12.4%). Conventional anthropometry, using published equations to estimate FFM from skinfolds, slightly overestimated TBK-derived FFM, but predicted FFM with precision similar to the best-fitting equation involving BI. Previously published FFM equations incorporating BI predicted TBK-derived FFM with variable predictive precision and accuracy. We conclude that BI analysis provides an alternative technique to assess FFM in children. At least within the range of normal body composition, its predictive power is similar to that of established skinfold techniques.

ISSN:0031-3998    

出版商:OVID    

年代:1994

数据来源: OVID

摘要:

Human parotid glands from 55 forensic autopsy subjects, 1–12 mo of age, were examined by immunohistochemistry without knowledge about the cause of death. Various combinations of monoclonal or polyclonal antibody reagents of the following specificities were applied in two-color immunofluorescence analyses: HLA class I or II (DR, DP, or DQ); pan-T cell (CD3); leukocyte common antigen (CD45); and secretory component (poly-Ig receptor). Sudden infant death syndrome victims (n = 17) were shown to have significantly increased numbers of CD45+stromal leukocytes and intensified epithelial expression of HLA-DR and secretory component as well as increased endothelial expression of both HLA class I and II (DR, DP, and DQ) determinants compared with controls (n = 31) who had died from noninfectious causes. Seven overtly infectious subjects (bronchopneumonia) showed still more up-regulated expression. This result suggested that enhanced stimulation of the local immune system exists in sudden infant death syndrome, with release of certain cytokines that are known to up-regulate epithelial expression of HLA-DR and secretory component.

ISSN:0031-3998    

出版商:OVID    

年代:1994

数据来源: OVID