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PubMed2001

In situ high resolution electron microscopy/electron energy loss spectroscopy observation of wetting of a Si surface by molten Al.

Electron energy loss spectroscopy was used to observe the segregation of Al on a Si surface above the melting point of Al. A mixture of Al and Si particles was heated above the melting point of Al in a vacuum of 1 x 10(-5) Pa. The Si surface, which initially had been covered with an amorphous oxide layer before heating, became clean and atomically facetted when the Al melted. It was shown that the Si surface was segregated with Al.

Journal Article↗
PubMed2003

High-dose intravenous melphalan with autologous stem cell transplantation in AL amyloidosis-associated end-stage renal disease.

BACKGROUND: The development of end-stage renal disease (ESRD) is common among patients with amyloid light-chain AL amyloidosis-associated renal disease and survival of these patients is poor. High-dose intravenous melphalan and autologous stem cell transplantation induce remission of the plasma cell dyscrasia in a significant proportion of patients with AL amyloidosis. The efficacy and tolerability of such treatment for patients with AL amyloidosis-associated ESRD are unknown. METHODS: Between June 1994 and June 2000, 15 patients with AL amyloidosis-associated ESRD were treated with intravenous melphalan (70 to 200 mg/m2) and autologous peripheral blood stem cell transplantation. Clinical and laboratory data were prospectively collected prior to treatment, during the peritransplant period, and at 3 months, 12 months, and annually thereafter. Treatment outcomes and toxicities were compared with 180 non-ESRD patients treated during the study period. RESULTS: Eight of 15 patients (53%) had a hematologic complete response following treatment. Two patients (13%) died during the peritransplant period. Transfusion requirements were greater and there was a trend toward increased severity of mucositis in the ESRD patients compared with the non-ESRD patients. Median survival for the ESRD patients with a hematologic complete response was 4.5 years. Five patients with hematologic complete response have either undergone or are awaiting renal transplantation. CONCLUSION: High-dose intravenous melphalan with stem cell transplantation is an effective treatment in selected patients with AL amyloidosis-associated ESRD. Although the toxicity profile is greater in ESRD patients, the treatment offers the possibility of successful renal transplantation if hematologic remission is achieved. This treatment should be considered for patients with AL amyloidosis-associated ESRD.

Adult↗
PubMed2005

Polarizabilities and hyperpolarizabilities for the atoms Al, Si, P, S, Cl, and Ar: Coupled cluster calculations.

Accurate static dipole polarizabilities and hyperpolarizabilities are calculated for the ground states of the Al, Si, P, S, Cl, and Ar atoms. The finite-field computations use energies obtained with various ab initio methods including Moller-Plesset perturbation theory and the coupled cluster approach. Excellent agreement with experiment is found for argon. The experimental alpha for Al is likely to be in error. Only limited comparisons are possible for the other atoms because hyperpolarizabilities have not been reported previously for most of these atoms. Our recommended values of the mean dipole polarizability (in the order Al-Ar) are alpha/e(2)a(0) (2)E(h) (-1)=57.74, 37.17, 24.93, 19.37, 14.57, and 11.085 with an error estimate of +/-0.5%. The recommended values of the mean second dipole hyperpolarizability (in the order Al-Ar) are gamma/e(4)a(0) (4)E(h) (-3)=2.02 x 10(5), 4.31 x 10(4), 1.14 x 10(4), 6.51 x 10(3), 2.73 x 10(3), and 1.18 x 10(3) with an error estimate of +/-2%. Our recommended polarizability anisotropy values are Deltaalpha/e(2)a(0) (2)E(h) (-1)=-25.60, 8.41, -3.63, and 1.71 for Al, Si, S, and Cl respectively, with an error estimate of +/-1%. The recommended hyperpolarizability anisotropies are Deltagamma/e(4)a(0) (4)E(h) (-3)=-3.88 x 10(5), 4.16 x 10(4), -7.00 x 10(3), and 1.65 x 10(3) for Al, Si, S, and Cl, respectively, with an error estimate of +/-4%.

Journal Article↗
PubMed2006

Pregnancy-induced changes in insulin-like growth factor I (IGF-I), insulin-like growth factor binding protein 3 (IGFBP-3), and acid-labile subunit (ALS) in patients with growth hormone (GH) deficiency and excess.

BACKGROUND: Under most circumstances with altered growth hormone (GH) secretion, the changes of insulin-like growth factor I (IGF-I), insulin-like growth factor binding protein 3 (IGFBP-3), and acid-labile subunit (ALS) are in parallel. The aim of the present study was to compare the effects of pregnancy in a hypopituitary patient with those of pregnancy in an acromegalic patient on IGF-I, IGFBP-3, and ALS. METHODS AND RESULTS: IGF-I and ALS were low before pregnancy in the hypopituitary patient under glucocorticoid and thyroxine treatment. Gonadotropin treatment allowed her to become pregnant; IGF-I and ALS levels rose in the second half of pregnancy and fell again after delivery. IGF-I concentrations were elevated in the patient with persistent acromegaly before and dropped into the normal range during the first half of pregnancy. In the second half of pregnancy and following delivery, IGF-I levels increased again. IGFBP-3 levels (as assessed by immunoblot analysis as well as by 125I-IGF II ligand blotting) decreased markedly during pregnancy in both patients, suggesting that the placenta rather than pituitary GH regulates IGFBP-3 proteolysis in human pregnancy. The increase of IGF-I (and ALS) during the second half of pregnancy in the individual with pituitary GH deficiency may be attributed to placental GH. The fall of IGF-I (and ALS) into the normal range in the acromegalic patient during the first trimester of pregnancy may be related to decreased production or decreased half-life of these proteins. CONCLUSION: Our data suggest that measures to continuously replace GH or to suppress GH secretion during pregnancy in patients with GH deficiency or excess, respectively, may not be warranted.

Acromegaly↗
PubMed2000

Improving the quality of life for people with ALS: the challenge ahead.

In tandem with recent progress in basic and clinical research in amyotrophic lateral sclerosis (ALS), clinical care and management has improved substantially. Key processes and practices have been established and are specified in current ALS Practice Parameters; the international ALS databases identify the current status of care and management given by physicians. A number of prospective clinical management research studies are now well underway to establish the effective utility of enteral feeding, assistive ventilatory device, and exercise in patients with ALS. One of the main research objectives in the clinical management of ALS is to improve patients' quality of life. For patients with ALS, only comprehensive multidisciplinary care can effectively improve their overall quality of life from diagnosis to death. This paper will briefly review a number of factors that must be considered if we are to improve the quality of life for these patients, including comprehensive medical management, patient education, the importance of hope, the role of the health care professional, and the patients themselves.

Attitude to Health↗
PubMed2002

Subjective experience and coping in ALS.

OBJECTIVE: Amyotrophic lateral sclerosis is a rapidly progressive and fatal disease which has no known cure and limited symptomatic treatment. While coping strategies in more common diseases are widely assessed, coping is poorly understood in ALS. METHODS: We examined 41 ALS patients using a standardised interview, a validated coping self-rating questionnaire and a self-rating depression scale. The evaluation was repeated after six months. RESULTS: "Loss of speech", "loss of mobility" and "the poor prognosis" were the most frequent answers in the standardised interview to questions regarding the worst aspect of the disease. Pain was seldom mentioned. "Family members" were most helpful in coping with the disease, followed by "unspecific mechanisms" and "technical aids". None of our patients expressed a wish for assisted suicide. In comparison with other fatal diseases, patients with ALS had similar rankings in the coping mechanism of "rumination", but lower rankings in "search for social integration", "defence of fear", "search for information and communication". In contrast,* "search for hold in the religion" was of high importance for our ALS patients. In the follow-up examination the importance of "search for information and communication" increased. CONCLUSION: The results emphasise the importance of "loss of speech" and the importance of the caring family as well as the availability of technical aids in ALS. Coping in ALS seems to be based mainly on "rumination" and *"hold in the religion", but the increasing importance of "search for information" indicates that the sustained offer of information is essential.

Adaptation, Psychological↗
PubMed2004

Communication strategies of people with ALS and their partners.

There are wider issues relating to the communication difficulties experienced by people with amyotrophic lateral sclerosis (ALS) than simply the physical problems caused by diminished oral control. In addition, existing literature on ALS rarely considers communication to be a joint interaction which depends on the strategies adopted by both communication partners, nor does it present communication in real life settings. This paper presents some of the findings from a 3-year research project which investigated the communication of people with ALS and their partners in their own homes. It discusses the purpose of human communication, and through examination of conversations in people's own homes has identified a range of strategies and techniques that families with ALS employ. For some people with ALS, although speech may deteriorate, they are still able to communicate closely and in a way that is more focused on topics that are particularly important to them. The findings from this study will be of interest to those who work with people with ALS.

Aged↗
PubMed2006

Premorbid personality characteristics of patients with ALS.

Experienced neurologists caring for patients with amyotrophic lateral sclerosis (ALS) have commented that these patients commonly exhibit similar personality features. Previous research studies on the premorbid personality characteristics of ALS patients have however not clearly substantiated this observation. In the present study, caregivers of newly diagnosed ALS patients provided ratings of patients' premorbid personality traits, and these ratings were compared with those of caregivers of patients newly diagnosed with other chronic, progressive diseases. Results indicated that ALS patients were rated as significantly lower than the other medical patients in the Openness trait on the NEO-Personality Inventory. This finding is discussed in relation to past research findings, prevailing clinical characterizations of ALS patients, and current research on cognitive and behavioral changes in ALS.

Adult↗
PubMed2003

Distribution of GABAA receptor mRNA in the motor cortex of ALS patients.

The pathomechanism of amyotrophic lateral sclerosis (ALS) remains unclear. There is some evidence that excitotoxic cell death is involved in the degeneration of the motor nervous system, and that ligand-gated receptor channels play a role in the pathogenesis of the disease. Several electrophysiological and anatomical studies support the pathophysiological concept of an impaired inhibitory, namely GABAergic, control of the motoneurons in the cerebral cortex of ALS patients. The aim of our study was to investigate the expression of GABAA-receptor subunit mRNAs and the GABA synthesizing enzyme glutamic acid decarboxylase (GAD) in the motor cortex of ALS patients compared to tissue of control persons. We performed in situ hybridization histochemistry (ISH) on human postmortem motor cortex sections of ALS patients (n = 5) and age matched controls with no history of neurological disease (n = 5). The most intriguing finding was a significantly reduced mRNA expression of the alpha1-subunit in ALS patients while the level of beta1-subunit mRNA was elevated in the patients group. This may indicate specific alterations of the GABAA receptor subunit composition and result in distinct physiological and pharmacological properties of these receptors in ALS patients.

Aged↗
PubMed2003

Comparison of Al-induced gene expression in sensitive and tolerant soybean cultivars.

In order to identify genes involved in soybean resistance to aluminium (Al) stress differential gene expression patterns of Al-stressed and non-stressed tolerant and sensitive soybean cultivars were compared. Out of eight described genes, potentially related to mechanisms of aluminium stress, only phosphoenolpyruvate carboxylase (PEPC) revealed enhanced expression in roots of tolerant as compared to sensitive soybean cultivars under stress conditions. Additionally, two novel full-length cDNA sequences, homologous to translationally controlled tumour proteins (TCTP, clone 58, GenBank accession number AF421558) and inosine-5'-monophosphate dehydrogenases (IMPDH, clone 633, GenBank accession number AF421559) with enhanced expression of the corresponding genes only in roots of Al-tolerant soybean cultivar under stress conditions were isolated and characterized. For functional analysis full-length cDNA 633 was transferred in Arabidopsis thaliana. Only 6% of the seedlings from the wild type survived Al stress, whereas 86% of transgenics were vital demonstrating superiority in stress protection. Compared with the wild type, transgenic plants showed diminished Al penetration into the roots after the stress treatment especially in the division and elongation zones of the roots. Formation of numerous lateral roots in transgenic plants with low elicited callose accumulation under stress conditions indicated ability of the IMPDH homologue to mediate aluminium tolerance in transgenic plants. Possible functional activities of Al up-regulated genes in resistance mechanisms are discussed.

Aluminum↗
PubMed2005

Amyloidosis of pancreatic islets in primary amyloidosis (AL type).

Seven cases of primary amyloidosis (AL-type) were studied immunocytochemically for the possible involvement of pancreatic islets. The two cases with extensive organ involvement by AL-amyloidosis revealed amyloid deposits in pancreatic islets by routine HE and Congo red staining, which were positive for amyloid p and amyloid a, but were only focally positive for light chains kappa and lambda. Positive staining for amyloid p and amyloid a was also noted in the scattered pancreatic acinar tissues, and this positive staining was not specifically located in pancreatic islets as seen in type 2 diabetes mellitus. It is concluded that amyloid deposits in pancreatic islets occur in systemic AL-amyloidosis by a different mechanism from type 2 diabetes. Islet amyloidosis in AL-amyloidosis appears to deposit via circulation, depositing in both pancreatic islets and acinar tissue through blood vessels. In type 2 diabetes, beta islet cells die by cytotoxic effects of smaller amylin (islet amyloid polypeptide, IAPP) aggregates, and the interstitial space created by the necrotic beta cells is replaced by larger IAPP aggregates, to form complex, polymerized islet amyloid. In AL-amyloidosis, the amount of amyloid and light chain deposits in pancreatic islets is much less than that of the other organs and appears to have no connection to type 2 diabetes because the patients did not present diabetes or hyperglycemia. However, considerable islet amyloidosis can be seen in severe AL-type amyloidosis.

Aged↗
PubMed2004

Benefit of valproic acid in suppressing disease progression of ALS model mice.

Valproic acid (VPA) has long been used as an antiepileptic drug and recently as a mood stabilizer, and evidence is increasing that VPA exerts neuroprotective effects through changes in a variety of intracellular signalling pathways including upregulation of Bcl-2 protein with an antiapoptotic property and inhibiting glycogen synthase kinase 3-beta, which is considered to promote cell survival. Although the neuroprotective effects of VPA have been demonstrated in a murine model of human immunodeficiency virus-1 encephalitis, there have been no reports on the effect of VPA in chronic progressing neurodegenerative disease models including amyotrophic lateral sclerosis (ALS). ALS is a devastating disease selectively affecting motoneurons, and its disease model mice bear a close resemblance to ALS symptomatically and pathologically. First, we used an organotypic slice culture using mouse spinal cord, and showed that VPA protected spinal motoneurons against death from glutamate toxicity in vitro. Then, we treated ALS model mice with VPA at the dose effective level for epileptic model mice after 45 days of age (pre-onset treatment) or the day of the disease onset (post-onset treatment). We found a significant prolongation of the disease duration in ALS model mice in both methods of treatment. Considering the long usage of VPA for epileptic patients with good tolerance and safety, these data strongly support the clinical application of VPA for ALS treatment.

Age of Onset↗
PubMed1996

Physical activity, trauma, and ALS: a case-control study.

AIMS: The association of trauma and physical activity with ALS is controversial. We explored the relation in a pilot case-control study. MATERIAL AND METHODS: ALS patients were selected from a university muscle disease clinic and paired with two matched controls: one from the clinic, but having different diseases, and one from the community. RESULTS: We found several strong and statistically significant differences between ALS cases and the matched controls. These included severe head, neck and back injury (OR = 5.3), the frequency of sweating in work (OR = 1.6) or leisure activity (also OR = 1.6), and earning a school letter (OR = 3.1). Other measures of trauma and activity, while not achieving statistical significance (p < 0.05), were in accord with these findings. DISCUSSION: Possible explanations include trauma and vigorous exercise precipitating ALS; trauma as an early sign of disease; or a third factor associated with ALS predisposing to injury. CONCLUSIONS: Severe head, neck, and back injury and frequency of sweating both in work and leisure activity showed a strong association with ALS. Further study could test narrower and less common exposures with greater statistical power.

Amyotrophic Lateral Sclerosis↗
PubMed2004

Quantification of Al-equivalent thickness of just visible microcalcifications in full field digital mammograms.

Characterization of digital mammography systems is often performed by means of contrast-detail curves using a homogeneous phantom with inserts of different sizes and thicknesses. In this article, a more direct measure of the threshold contrast-detail characteristics of microcalcifications in clinical mammograms is proposed, which also takes into account routine processing and display. The proposed method scores the detectability of simulated microcalcifications with known size and aluminum-equivalent thickness. Thickness estimates, based on x-ray transmission coefficients, were first validated for Al particles. The same approach was then applied to associate Al-equivalent thickness with simulated microcalcifications. Thirty-five mammograms of patients were acquired using a full field digital mammography (FFDM) system operating under standard exposure conditions. Different microcalcifications were simulated using templates of real microcalcifications as described in Med. Phys. 30, 2234-2240 (2003). These templates were first modified such that they simulated a template of the same microcalcification for an ideally sharp detector. They were then adjusted for the imaging characteristics of the FFDM, beam quality, and breast thickness. Microcalcification sizes in the image plane ranged from 200 to 800 microm. Their peak Al-equivalent thickness varied between 70 and 1000 microm. Software phantoms were created. They consisted of 0-10 simulated microcalcifications randomly distributed in 2 cm by 2 cm frames embedded within digital mammograms. Routine processing and printing followed. Three experienced radiologists recorded the locations of the microcalcifications, and confidence ratings were given. Free response receiver operating characteristics (FROC) analysis was performed. Using a binary score, the fractions of detected microcalcifications were plotted as a function of equivalent diameter for the different Al-equivalent thicknesses. Pair-wise agreement of the detected microcalcifications was calculated for the different Al-equivalent thickness groups. The FROC curves of each radiologist indicated similar true positive fractions for a given number of false positives per image. One radiologist applied a more conservative scoring. Detected fractions for the different sizes of the microcalcifications showed the same trend for all observers. In addition, the observer with the least FP also detected less microcalcifications. The pair-wise agreement of the detected microcalcifications was good. The average detected fractions were >0.5 for microcalcifications with equivalent diameter >400 microm and Al-equivalent thickness >400 microm. An average detected fraction >0.5 was also seen for microcalcifications with equivalent diameter <400 microm and equivalent thickness >800 microm. The detected fractions of smaller microcalcifications were <0.5. The results obtained with this method indicate that it may be possible to quantify the performance of a digital mammography detector including processing and viewing for the detection of microcalcifications. We hypothesize that the FROC curves and detected fractions of simulated microcalcifications of different sizes reflect the clinical reality.

Algorithms↗
PubMed2000

Structure of the hydrated alpha-Al(2)O(3) (0001) surface

The physical and chemical properties of the hydrated alpha-Al(2)O(3) (0001) surface are important for understanding the reactivity of natural and synthetic aluminum-containing oxides. The structure of this surface was determined in the presence of water vapor at 300 kelvin by crystal truncation rod diffraction at a third-generation synchrotron x-ray source. The fully hydrated surface is oxygen terminated, with a 53% contracted double Al layer directly below. The structure is an intermediate between alpha-Al(2)O(3) and gamma-Al(OH)(3), a fully hydroxylated form of alumina. A semiordered oxygen layer about 2.3 angstroms above the terminal oxygen layer is interpreted as adsorbed water. The clean alpha-Al(2)O(3) (0001) surface, in contrast, is Al terminated and significantly relaxed relative to the bulk structure. These differences explain the different reactivities of the clean and hydroxylated surfaces.

Journal Article↗
PubMed2001

The effect of non-invasive positive pressure ventilation (NIPPV) on cognitive function in amyotrophic lateral sclerosis (ALS): a prospective study.

OBJECTIVES: Neuropsychological investigations have shown a degree of cognitive dysfunction in a proportion of non-demented patients with ALS. Respiratory muscle weakness in ALS can lead to nocturnal hypoventilation, resulting in sleep disturbance and daytime somnolence. Sleep deprivation of this type may cause impairments in cognitive function, but this has not been formally evaluated in ALS. METHODS: Cognitive functioning was evaluated in nine patients with ALS with sleep disturbance caused by nocturnal hypoventilation (NIPPV group), and in a comparison group of 10 similar patients without ventilation problems (control group). The NIPPV group then started non-invasive positive pressure ventilation (NIPPV) at night. After about 6 weeks, change in cognitive function was evaluated. RESULTS: Statistically significant improvement in scores on two of the seven cognitive tests was demonstrated in the NIPPV group postventilation, and a trend towards significant improvement was found for two further tests. Scores in the control group did not improve significantly for these four tests, although an improvement was found on one other test. CONCLUSIONS: Nocturnal hypoventilation and sleep disturbance may cause cognitive dysfunction in ALS. These deficits may be partially improved by NIPPV over a 6 week period. This has important implications for investigations of both cognitive dysfunction in non-demented patients with ALS, and the effect of ventilation on quality of life.

Aged↗
PubMed1976

Effect of antilymphocyte serum (ALS) on shock in rats.

Effects of treatment with rabbit antirat anti-lymphocyte serum and globulin (ALS and ALG) on shock survival were studied in Sprague-Dawley derived male rats. Because of their known cytotoxic capability, it was postulated that lymphocytes might play a role in the pathogenesis of shock and that suppression of lymphocyte function by ALS/ALG treatment should then protect against shock. Shock models used were tourniquet, endotoxin, and hemorrhagic shock. Protection against tourniquet shock was found for ALS made against thymocytes but not for ALS against spleen cells or lymph node cells. The shock-protective factor was found in the ALG-containing serum fraction but not in the primarily albumin fraction. No significant protection was found for ALS treatment against either endotoxin or hemorrhagic shock. ALS effects on blood cell counts, reticulo endothelial system clearance, and inflammation were studied to help identify effects of ALS on shock survival. It was concluded from these studies that thymic or thymus-processed lymphocytes could play a role in the pathogenesis of shock but that multiple effects of ALS/ALG treatment necessitate further studies to elucidate any role for lymphocytes in shock.

Animals↗
PubMed2005

Anti-ALS activity of alsin, the product of the ALS2 gene, and activity-dependent neurotrophic factor.

Amyotrophic lateral sclerosis (ALS) is an incurable degenerative motoneuronal disease. The complete suppression of motoneuronal death is the ultimate goal of ALS therapy. Two new prosurvival pathways have been recently demonstrated to antagonize neurotoxicity by familial ALS-linked mutant Cu/Zn-superoxide dismutase (FSOD1). Alsin, the product of the recently cloned ALS-causative gene, the ALS2 gene, is linked to a Rac1/phosphatidylinositol-3 kinase/Akt3 pathway that specifically suppresses motoneuronal death induced by FSOD1. Activity-dependent neurotrophic factor, originally identified as an anti-Alzheimer neurotrophic factor, has been shown to suppress motoneuronal death by FSOD1 through a prosurvival pathway mediated by Ca(2+)/calmodulin-dependent protein kinase IV. Activation of these novel anti-ALS pathways may serve as a promising way to suppress ALS-related motoneuronal cell death.

Amyotrophic Lateral Sclerosis↗