Development and results of the Spanish registry of patients with alpha-1-antitrypsin deficiency

The Spanish registry of alpha-1 antitrypsin deficiency was founded in 1993 and became a member of the International Registry (AIR) in 1999. We describe the updating process following its incorporation into AIR and compare the data collected in the first period (1993–1999) and the second period (1999–2005), during which time patients were included exclusively by internet.The registry included 301 patients during period 1, 69% males and 46% had a history of smoking. Their mean age was 46 years (SD = 13) and 284 (94%) had the ZZ phenotype, 49% received augmentation therapy. During period 2, 161 new cases were included, 63% of whom were males with a mean age of 44 years (SD = 16). A total of 126 (78%) had the ZZ phenotype. Only 12% received augmentation therapy. A total of 462 different patients were included in both periods. Significant differences were observed in the number of cases with the SZ phenotype and the severity of FEV₁ impairment between the two periods.Implementation of an internet-based collection of data did not result in a lower rate of reporting to the registry. However, data from a significant number of patient included in period 1 could not be actualized in the new data base.     

Engineering angiogenesis following spinal cord injury: a coculture of neural progenitor and endothelial cells in a degradable polymer implant leads to an increase in vessel density and formation of the blood–spinal cord barrier

Angiogenesis precedes recovery following spinal cord injury and its extent correlates with neural regeneration, suggesting that angiogenesis may play a role in repair. An important precondition for studying the role of angiogenesis is the ability to induce it in a controlled manner. Previously, we showed that a coculture of endothelial cells (ECs) and neural progenitor cells (NPCs) promoted the formation of stable tubes in vitro and stable, functional vascular networks in vivo in a subcutaneous model. We sought to test whether a similar coculture would lead to the formation of stable functional vessels in the spinal cord following injury. We created microvascular networks in a biodegradable two-component implant system and tested the ability of the coculture or controls (lesion control, implant alone, implant + ECs or implant + NPCs) to promote angiogenesis in a rat hemisection model of spinal cord injury. The coculture implant led to a fourfold increase in functional vessels compared with the lesion control, implant alone or implant + NPCs groups and a twofold increase in functional vessels over the implant + ECs group. Furthermore, half of the vessels in the coculture implant exhibited positive staining for the endothelial barrier antigen, a marker for the formation of the blood–spinal cord barrier. No other groups have shown positive staining for the blood–spinal cord barrier in the injury epicenter. This work provides a novel method to induce angiogenesis following spinal cord injury and a foundation for studying its role in repair.     

Maturation of executive function in autism.

BACKGROUND: Executive dysfunction has been reported at different ages in autism. It is not clear however, when this impairment emerges or how its expression is affected by development. METHODS: 61 non-mentally retarded autism participants (AUT) and 61 age, gender, and IQ matched typically developing participants (CON) were assessed with two oculomotor executive function tasks, the oculomotor delayed response task (ODR) and the antisaccade task (AS), as well as a visually-guided saccade sensorimotor task (VGS). RESULTS: The AUT group demonstrated impairments in response inhibition and spatial working memory at all ages tested. Developmental improvements in speed of sensorimotor processing and voluntary response inhibition were similar in both groups indicating sparing of some attentional control of behavior. Developmental progression in the speed of initiating a cognitive plan and maintaining information on line over time, however, was impaired in the AUT group indicating abnormal development of working memory. CONCLUSIONS: These results indicate that while executive dysfunction is present throughout development, there is evidence for both typical and atypical developmental progression of executive functions in autism. The plasticity suggested by the developmental improvements may have implications regarding appropriate developmental epochs and types of interventions aimed at enhancing cognitive capacities in individuals with autism.     

Adverse drug reactions in internal medicine units and associated risk factors

Objectives  

This study was designed to assess the prevalence of adverse drug reactions (ADRs) in the internal medicine wards of two teaching Hospitals, identify the most common ADRs, the principal medications involved, and determine the risk factors implicated in the occurrence of such ADRs.     

Mercury Concentrations in Quagga Mussels, Dreissena bugensis, from Lakes Mead, Mohave and Havasu

The recent invasion of the Dressenid species, the quagga mussel, Dreissena bugensis, into Lakes Mead, Mohave and Havasu has raised questions about their ability to alter contaminant cycling. Mussels were collected from 25 locations in the three lakes. The overall average was 0.036 ± 0.016 μg g⁻¹ Hg dry wt. The range of the three lakes was from 0.014–0.093 μg g⁻¹ Hg dry wt. There were no significant differences in mercury concentrations among the three lakes (F = 0.07; p = 0.794). From this baseline data of contaminants in quagga mussels from the lower Colorado River, this species may be used to biomonitor lake health.     

Accumulation of toxic metals (Pb and Cd) in the sea urchin Diadema aff. antillarum Philippi, 1845, in an oceanic island (Tenerife,Canary Islands)

This document shows the results obtained from a study on the concentration of toxic heavy metals in the internal tissue and exoskeleton of sea urchins, collected from their natural habitat. The levels of lead and cadmium were measured by Graphite Furnace Atomic Absorption Spectrometry. The mean concentrations of lead and cadmium in the internal tissue were 304.04 and 260.54 μg/kg respectively, whereas in the shell they were 185.02 and 142.48 μg/kg. We also performed a statistical analysis of the differences in the distribution of metals between their exoskeleton and their internal content, a correlation study of the metal content in internal tissue and shell and sampling areas, and a correlation study between the metal content and sample size. Since the sea urchin Diadema antillarum presents a wide range of variation in metal content, this study suggests that this species is an excellent bioindicator of heavy metal contamination. © 2009 Wiley Periodicals, Inc. Environ Toxicol, 2010.     

A case-controlled study of altered visual art production in Alzheimer's and FTLD.

OBJECTIVE: To characterize dementia-induced changes in visual art production. BACKGROUND: Although case studies show altered visual artistic production in some patients with neurodegenerative disease, no case-controlled studies have quantified this phenomenon across groups of patients. METHOD: Forty-nine subjects [18 Alzheimer disease, 9 frontotemporal dementia (FTD), 9 semantic dementia (SD), 15 healthy older controls (NC)] underwent formal neuropsychologic testing of visuospatial, perceptual, and creative functioning, and produced 4 drawings. Subjective elements of drawings were rated by an expert panel that was blind to diagnosis. RESULTS: Despite equal performance on standard visuospatial tests, dementia groups produced distinct patterns of artistic features that were significantly different from NCs. FTDs used more disordered composition and less active mark-making (P<0.05). Both FTDs and SDs drawings were rated as more bizarre and demonstrated more facial distortion than NCs (P<0.05). Also, SDs drastically failed a standardized test of divergent creativity. Alzheimer disease artwork was more similar to controls than to FTDs or SDs, but showed a more muted color palette (P<0.05) and trends toward including fewer details, less ordered compositions, and occasional facial distortion. CONCLUSIONS: These group differences in artistic style likely resulted from disease-specific focal neurodegeneration, and elucidate the contributions of particular brain regions to the production of visual art.     

Inadequate oral intake in surgical patients: a rational indication for nutritional support

Seven days or more of inadequate oral intake (IOI) inevitably results in a deterioration in nutritional status. Despite this well-known fact, little information is available as to the frequency with which such periods of IOI occur in clinical practice. This study results from an audit of IOI in a gastroenterological unit over a 6-month period. The results demonstrate that 17% of patients sustained significant periods of IOI of 7 days or longer. This has important implications with regard to the provision of adjuvant nutritional support.