Identification of a common mutation (R245H) in Sanfilippo A patients from The Netherlands

Two groups of male rats were tested to determine whether pre-exposure to d-amphetamine would enhance the motivation to self-administer the drug under a progressive ratio schedule of reinforcement. In the first phase of the experiment, one group of rats received d-amphetamine (2 mg/kg IP), while a second group received saline on alternate days for a total of ten injections. Following a 21-day drug withdrawal period, behavioral sensitization was confirmed by a significant increase in amphetamine-induced stereotypy in the d-amphetamine-pretreated group, relative to the saline-pretreated group. In the second phase of the study, all rats were implanted with chronic jugular catheters and trained to self-administer d-amphetamine (0.2 mg/kg per infusion) under a fixed-ratio schedule of reinforcement. The progressive ratio paradigm was then imposed for 7 consecutive days; d-amphetamine-pretreated rats attained significantly higher break points than saline-pretreated animals. These data suggest that pre-exposure to d-amphetamine may enhance the motivation to self-administer this drug.     

An analog CMOS front-end for a D2-MAC TV decoder

This paper describes the analog front-end of a fully integrated CMOS TV decoder, suitable for the reception of terrestrial as well as satellite signals, based on the D2-MAC transmission system. While the video reconstruction is undertaken using DSP, the front-end subsystem incorporates many linear and non-linear analog functions, including amplitude measuring, AGC, clamping, data slicing, clock recovery and of course, A/D conversion for the MAC signal processing. The chip is fabricated in 1-μ CMOS, and operates from a single 5-V supply     

Characterization of sulfated oligosaccharides in mucopolysaccharidosis type IIIA by electrospray ionization mass spectrometry

Heparan sulfate is a linear glycosaminoglycan with considerable structural diversity that binds a myriad of growth factors and proteins that play pivotal roles in a variety of biological processes. We have investigated the structural complexity of partially degraded fragments of heparan sulfate in mucopolysaccharidosis type IIIA in which there is a defect in heparan sulfate catabolism. Mono- to hexadecasaccharides were isolated from the urine of a mucopolysaccharidosis IIIA patient and shown to have non-reducing end glucosamine N-sulfate residues, reflecting the catabolic deficiency in heparan N-sulfatase (sulfamidase) activity. The use of nitrous acid digestion (pH 1.5) combined with separation by reverse-phase high-performance liquid chromatography and analysis by electrospray ionization-mass spectrometry identified multiple forms of these oligosaccharides with some N-acetylated glucosamine residues and one to three sulfates per disaccharide. Furthermore, we demonstrated that each oligosaccharide existed in multiple sulfated forms. Many structural isomers were present, suggesting a complex mixture of oligosaccharides present in the urine as a consequence of a defect in heparan sulfate degradation.     

Functioning thyroid masses in childhood and adolescence. Clinical, surgical, and pathologic correlations

Six girls, aged 5 to 15 years, presented with thyroid masses in otherwise nonpalpable thyroid glands and with normal serum thyroxine levels. Scintiscanning before and after TSH stimulation confirmed the presence of autonomous nodules in the four adolescents, of whom two had elevated T3 levels. Surgical exploration revealed adenomatous thyroid hyperplasia in three of the girls and papillary adenocarcinoma in the fourth. Scans in the other two girls revealed absence of the left lobe. One of them proved to have agenesis of the left lobe with enlargement of the right lobe because of lymphocytic thyroiditis. The other girl had an ectopic thyroid with chronic inflammation. A thorough diagnostic evaluation of single or multiple functioning thyroid masses in children and adolescents is essential in establishing the correct diagnosis. The possibility that carcinoma can occur in autonomous nodules as well as in hemiagenesis and ectopic thyroid tissue is discussed. An approach to the management of functioning thyroid masses in the pediatric age group is proposed.     

Effect of methotrexate therapy in psoriatics on the Ito cells in liver biopsies, assessed by point-counting

To evaluate the relationship, both quantitative and qualitative, between the Ito cells and methotrexate (MTX) therapy Ito cells were studied by light microscopy in 1 mum toluidine blue stained sections and by electron microscopy in 24 pairs of Menghini needle biopsies before and after MTX therapy of 24 consenting patients with severe psoriasis. Light microscopy showed a statistically significant increase in pathological findings (P less than 0-05) and in the number of Ito cells and their size (P less than 0-0001) after MTX therapy. It was not possible to show a statistically significant correlation between the increase in the number of Ito cells and the cumulative dose of MTX. Ultrastructural analysis of Ito cells showed no marked difference from pre to post-MTX specimens. The fibrosis and cirrhosis seen after MTX therapy in some liver biopsies from psoriatics and the post-MTX increase in the number of Ito cells direct attention to the possible role of Ito cells as fibroblast precursors.     

Psychometric characteristics and clinical correlates of NEO-PI-R fearless dominance and impulsive antisociality in the Collaborative Longitudinal Personality Disorders Study.

[Correction Notice: An erratum for this article was reported online in Psychological Assessment on September 26 2011 (see record 2011-21951-001). The Appendix should have read: Fearless Dominance: 7*, 11, 16*, 37, 46, 71, 72, 102*, 127*, 132, 144, 152, 162*, 166, 192, 227, 232 Impulsive Antisociality: 14, 30, 44*, 45, 50*, 60*, 65*, 66, 95, 99, 159, 169, 189, 201, 205, 229, 240* * Indicates a reverse-scored item (based on original item content). NEO-PI-R = NEO Personality Inventory–Revised (Costa & McCrae, 1992).] This study evaluates the validity of derived measures of the psychopathic personality traits of Fearless Dominance and Impulsive Antisociality from the NEO Personality Inventory–Revised (NEO-PI-R; Costa & McCrae, 1992) using data from the Collaborative Longitudinal Personality Disorders Study (baseline N = 733). These 3 issues were examined: (a) the stability of the measures over a 10-year interval, (b) their criterion-related validity, and (c) their incremental validity relative to an alternative NEO-PI-R profile-rating approach for assessing psychopathy. NEO-PI-R Fearless Dominance and Impulsive Antisociality scales were relatively stable across 10 years and demonstrated differential associations with measures of personality pathology and psychopathology generally consistent with past research and theoretical considerations. Moreover, these measures demonstrated an appreciable degree of incremental validity over the NEO-PI-R profile-rating approach. (PsycINFO Database Record (c) 2011 APA, all rights reserved)