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PubMed2003

Correlation of very long chain fatty acid accumulation and inflammatory disease progression in childhood X-ALD: implications for potential therapies.

This study was designed to understand the role of inflammatory mediators involved in the neurobiology of childhood adrenoleukodystrophy (cALD) by comparing the differential expression of the inflammatory mediators with metabolite very long chain fatty acids that accumulate in this disease. Histopathological examinations indicated extensive demyelination and accumulation of infiltrates in perivascular cuffs in plaque area (PA) and inflammatory area (IA) compared to normal looking area (NLA) of the cALD brain and controls. The PA had excessive accumulation of cholesterol ester (25-30-fold), VLC fatty acids (8-12-fold), and exhaustive depletion of cholesterol (60-70%) and sphingomyelin (50-55%) in comparison to controls. The mRNA expression of cytokines (IL-1alpha, IL-2, IL-3, IL-6, TNF-alpha, and GM-CSF), chemokines (CCL2, -4, -7, -11, -16, -21, -22, CXCL1, CX3CL1, and SDF-2) and iNOS in IA was significantly increased compared to NLA of the cALD and controls determined by gene array, semiquantitative RT-PCR, and immunohistochemistry. These results indicate that accumulation of VLC fatty acid contents in membrane domains associated with signal transduction pathways may trigger the inflammatory process through activation of resident glial cells (microglia and astrocytes) resulting in loss of myelin and oligodendrocytes.

Adrenoleukodystrophy↗
PubMed2005

The Academic Medical Center Linear Disability Score (ALDS) item bank: item response theory analysis in a mixed patient population.

BACKGROUND: Currently, there is a lot of interest in the flexible framework offered by item banks for measuring patient relevant outcomes. However, there are few item banks, which have been developed to quantify functional status, as expressed by the ability to perform activities of daily life. This paper examines the measurement properties of the Academic Medical Center linear disability score item bank in a mixed population. METHODS: This paper uses item response theory to analyse data on 115 of 170 items from a total of 1002 respondents. These were: 551 (55%) residents of supported housing, residential care or nursing homes; 235 (23%) patients with chronic pain; 127 (13%) inpatients on a neurology ward following a stroke; and 89 (9%) patients suffering from Parkinson's disease. RESULTS: Of the 170 items, 115 were judged to be clinically relevant. Of these 115 items, 77 were retained in the item bank following the item response theory analysis. Of the 38 items that were excluded from the item bank, 24 had either been presented to fewer than 200 respondents or had fewer than 10% or more than 90% of responses in the category 'can carry out'. A further 11 items had different measurement properties for younger and older or for male and female respondents. Finally, 3 items were excluded because the item response theory model did not fit the data. CONCLUSION: The Academic Medical Center linear disability score item bank has promising measurement characteristics for the mixed patient population described in this paper. Further studies will be needed to examine the measurement properties of the item bank in other populations.

Academic Medical Centers↗
PubMed1999

Synthesis of mevalonate pathway lipids in fibroblasts from Zellweger and X-linked ALD patients.

Fibroblasts were cultured to determine the involvement of peroxisomes in cholesterol and dolichol synthesis. For this purpose, the behavior of cells from patients with Zellweger syndrome, with X-linked adrenoleukodystrophy, and from nondiseased control subjects was studied. Cells both after pretreatment with mevinolin and without pretreatment were incubated in a medium containing [3H]-mevalonate. In fibroblasts from patients with peroxisomal defects, the cholesterol content and mevalonate incorporation into cholesterol were decreased by 10-20% in comparison with control cells. Mevinolin pretreatment decreased the incorporation rate of [3H]-mevalonate into cholesterol but increased the labeling of ubiquinone and dolichol both in diseased and control cells. Squalene synthase activity was unchanged, whereas the activity of farnesyl-pyrophosphate synthase was increased in the diseased states. The results show that in patients with peroxisomal deficiency neither the amount nor the rate of synthesis of cholesterol and dolichol is reduced to any greater extent.

Adrenoleukodystrophy↗
PubMed1998

Desmoplastic variant of ameloblastoma in an 83-year-ald Asian female: report of a case with literature review.

The desmoplastic ameloblastoma is a rare histologic variant of ameloblastoma. This presentation outlines the successful treatment of an 83-year-old Asian female with this type of tumour by surgical resection and peripheral ostectomy with preservation of the interior border of the mandible. Clinical, radiographic, histopathologic and surgical aspects of this tumour were reviewed.

Aged↗
PubMedDate not supplied

Specificity of antinuclear and antiphospholipid antibodies in sera of patients with autoimmune lymphoproliferative disease (ALD).

OBJECTIVE: A human lymphoproliferative syndrome characterized by a defect of the Fas-mediated apoptosis pathway in the absence of a fas gene mutation (Autoimmune Lymphoproliferative Disease) has recently been described and characterized by autoimmune phenomena. The aim of this study was to investigate the presence of antinuclear and antiphospholipid antibodies and to define their specificity in 5 pediatric patients with this syndrome. METHODS: Antinuclear antibodies were investigated by Western Blot and IIF performed under standard as well as apoptotic conditions. The fine specificity of antiphospholipid antibodies was dissected by an ELISA for anti-beta 2-glycoprotein I, anti-prothrombin, anti-annexin V and anti-protein S antibodies, and by immunostaining on thin layer chromatography plates for antiphospholipid molecule antibodies. RESULTS: This study showed that the autoantibodies found in these patients targeted a broad spectrum of nuclear antigens which undergo redistribution from the nucleus to the cytoplasm and plasma membrane during the course of the apoptotic process. This reactivity does not comprise known specificities such as anti-extractable nuclear antigens or anti-dsDNA. Antiphospholipid antibodies were also found in these sera. A further characterization of the antiphospholipid antibodies showed the presence of a heterogeneous response with antibodies directed to negatively-charged phospholipids and antibodies targeting coagulation-related proteins (beta 2-GPI, prothrombin, annexin V) which are considered relevant antigens in the antiphospholipid syndrome. CONCLUSIONS: These results suggest that lack of tolerance due to a defect of Fas-mediated apoptosis allows the survival of B and T clones involved in the antinuclear and antiphospholipid immune responses.

Adolescent↗
PubMed2003

Six hour ambulation after elective coronary angioplasty and stenting with 7F guiding catheters ald low dose heparin.

BACKGROUND: Early ambulation after coronary angioplasty may reduce in-hospital stay and add to the patient's comfort. This approach, however, may increase the risk of insertion site related complications, such as arterial bleeding, haematoma, pseudoaneurysm, and the need for surgical repair. AIM: To evaluate the feasibility and safety of ambulation of patients six hours after elective coronary angioplasty or stenting, or both. METHODS: Coronary angioplasty and stenting were performed using 7F guiding catheters via the femoral or brachial approach. The first dose of heparin 5000 IU was given immediately after insertion of the arterial sheath and the second dose heparin 2500 IU was given 90 minutes later. There were no angiographic exclusion criteria. The arterial sheath was removed immediately after the procedure. Haemostasis was achieved by manual compression and maintained with a compression bandage. Early ambulation was attempted after six hours of supine bed rest following removal of the bandage. The incidence of bleeding at ambulation requiring compression and additional bed rest, and insertion site complications documented 48 hours after the procedure, were analysed. RESULTS: 326 patients (290 femoral, 36 brachial route) were included. Stent implantation was performed in 267 patients (82%). The mean+/-SD time to haemostasis was 14+/-4 minutes. Bleeding at ambulation occurred in 7 (2.14%) patients, and major haematomas were seen in 8 (2.45%) patients during 48-hour follow up. All were seen in patients in whom the femoral route was used and who were treated conservatively. There were no late bleeding or vascular complications. CONCLUSIONS: Ambulation six hours after elective balloon angioplasty or stent implantation with 7F guiding catheters using femoral or brachial route and low dose heparin is feasible and safe, with a low incidence of insertion site complications. This early ambulation protocol shortens hospital stay.

Adult↗