Home About us Contact
|
Home About us Contact | ||
|
|
||
Disease Subjects (disease + subject)
Selected AbstractsHaemorheology in Gaucher diseaseEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 3 2005Bridget E. Bax Abstract:, In Gaucher disease, a deficiency of glucocerebrosidase results in the accumulation of glucocerebroside within the lysosomes of the monocyte,macrophage system. Prior to the availability of enzyme replacement therapy (ERT), splenectomy was often indicated for hypersplenism. Haemorheological abnormalities could be expected in view of the anaemia and abnormal lipid metabolism in these patients and the role of the spleen in controlling erythrocyte quality. Objectives: To investigate the effect of Gaucher disease on blood and plasma viscosity, erythrocyte aggregation and erythrocyte deformability, and to determine whether observed rheological differences could be attributed to splenectomy. Methods: Haematological and haemorheological measurements were made on blood collected from 26 spleen-intact patients with Gaucher disease, 16 splenectomised patients with Gaucher disease, 6 otherwise healthy asplenic non-Gaucher disease subjects and 15 healthy controls. Results: No haemorheological differences could be demonstrated between spleen-intact patients with Gaucher disease and the control group. Compared to controls, both asplenic Gaucher disease and asplenic non-Gaucher disease study groups had a reduced MCHC (P = 0.003 and 0.005, respectively) and increased whole blood viscosity at 45% haematocrit (Hct), relative viscosity and red cell aggregation index , all measured at low shear (P < 0.05 for all). Additionally, asplenic patients with Gaucher disease alone showed an increased MCV (P = 0.006), an increased whole blood viscosity at 45% Hct measured at high shear (P = 0.019), and a reduced relative filtration rate (P = 0.0001), compared to controls. Conclusion: These observations demonstrate a direct and measurable haemorheological abnormality in Gaucher disease only revealed when there is no functioning spleen to control erythrocyte quality. [source] Gingival crevicular fluid levels of RANKL and OPG in periodontal diseases: implications of their relative ratioJOURNAL OF CLINICAL PERIODONTOLOGY, Issue 5 2007Nagihan Bostanci Abstract Aim: Receptor activator of NF-,B ligand (RANKL) and osteoprotegerin (OPG) are a system of molecules that regulate bone resorption. This study aims to compare the levels of RANKL, OPG and their relative ratio in gingival crevicular fluid (GCF) of healthy and periodontal disease subjects. Material and Methods: GCF was obtained from healthy (n=21), gingivitis (n=22), chronic periodontitis (n=28), generalized aggressive periodontitis (n=25) and chronic periodontitis subjects under immunosuppressant therapy (n=11). RANKL and OPG concentrations in GCF were measured by enzyme-linked immunosorbent assays. Results: RANKL levels were low in health and gingivitis groups, but increased in all three forms of periodontitis. OPG levels were higher in health than all three periodontitis, or gingivitis groups. There were no differences in RANKL and OPG levels between chronic and generalized aggressive periodontitis groups, whereas these were lower in the immunosuppressed chronic periodontitis group. The RANKL/OPG ratio was significantly elevated in all three periodontitis forms, compared with health or gingivitis, and positively correlated to probing pocket depth and clinical attachment level. Conclusion: GCF RANKL and OPG levels were oppositely regulated in periodontitis, but not gingivitis, resulting in an enhanced RANKL/OPG ratio. This ratio was similar in all three periodontitis groups and may therefore predict disease occurrence. [source] Adult coeliac disease: Prevalence and clinical significanceJOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY, Issue 9 2000H Bramwell Cook Abstract Background and Aims: Although coeliac disease is a common condition, the role of population screening is not clear. The aim of this study was to determine the prevalence and clinical significance of coeliac disease in the adult population of Christchurch, New Zealand. Methods: A total of 1064 adults randomly selected from the 1996 Christchurch electoral rolls were enlisted. The subjects were screened for coeliac disease using the anti-endomysial antibody test (EMA), and all those with positive tests were reviewed and underwent a small bowel biopsy. Results: Twelve of the 1064 persons tested (1.1%) were EMA positive and all had small bowel biopsy histology consistent with coeliac disease. Two of the 12 subjects were previously known to be EMA positive although neither had a small bowel biopsy. One additional subject with known and treated coeliac disease was also enrolled but was EMA negative. Thus, the overall prevalence of coeliac disease was 13 of 1064 subjects (1.2%, or 1 : 82), 10 of whom were newly diagnosed (0.9%, or 1 : 106) and three were previously known or suspected to have coeliac disease (0.3%, or 1 : 355). The prevalence in both sexes was similar. Nine of the 12 EMA-positive coeliac disease subjects identified by the use of screening reported symptoms, of which tiredness and lethargy were the most common. The subjects were of normal stature, although females tended to be lean. None of the subjects were anaemic, but four were iron deficient and four folate deficient. Five of the 12 had sustained bone fractures. Bone mineral density was reduced in males but not in females. Conclusions: The prevalence of coeliac disease in the adult population of Christchurch, New Zealand, is 1.2%. Unrecognized coeliac disease which was detected by population screening was three-fold more common than proven or suspected coeliac disease. Population screening may identify subjects who could benefit from treatment. [source] Oesophageal pH has a power-law distribution in control and gastro-oesophageal reflux disease subjectsALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 11-12 2004J. D. Gardner Summary Background :,We are unaware of any solid theoretical or pathophysiological basis for selecting pH 4 or any other pH value to assess oesophageal acid exposure or to define oesophageal reflux episodes. Aim :,To examine the frequency of different oesophageal pH values in control and GERD subjects. Methods :,Oesophageal pH was measured for 24 h in 57 gastro-oesophageal reflux disease subjects and 26 control subjects. Histograms were constructed using the 21 600 values from each recording and bins of 0.25 pH units. Results :,Compared with controls, gastro-oesophageal reflux disease subjects had significantly more low pH values and significantly fewer high pH values. In both gastro-oesophageal reflux disease and control subjects, the frequency of oesophageal pH values was characterized by a power-law distribution indicating that the same relationship that describes low pH values also describes high pH values, as well as all values in between. Conclusions :,The distribution of oesophageal pH values indicates that a variety of different pH values can be used to assess oesophageal acid exposure, but raises important questions regarding how oesophageal reflux episodes are defined. [source] Frequency analyses of gastric pH in control and gastro-oesophageal reflux disease subjects treated with a proton-pump inhibitorALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 11-12 2004J. D. Gardner Summary Background :,We are unaware of any solid theoretical or pathophysiological basis for selecting pH 4 or any other pH value to assess gastric acidity. Aim :,To examine the frequency of different gastric pH values in control and GERD subjects. Methods :,Gastric pH was measured for 24 h in 26 control subjects, 26 gastro-oesophageal reflux disease subjects at baseline and the same 26 gastro-oesophageal reflux disease subjects during treatment with a proton-pump inhibitor. Histograms were constructed using the 21 600 values generated from each recording and bins of 0.25 pH units. Results :,The distribution of gastric pH values in gastro-oesophageal reflux disease subjects was significantly different from that in controls and in some instances the distributions detected significant differences that were not detected by integrated acidity. Proton-pump inhibitor treatment significantly altered the distribution of gastric pH values and the nature of this alteration during the postprandial period was different from that during the nocturnal period. Using time pH,4 can significantly underestimate the magnitude of inhibition of gastric acidity caused by a proton-pump inhibitor. Conclusions :,The distribution of gastric pH values provides a rationale for selecting a particular pH value to assess gastric acidity. In some instances, the distribution of gastric pH values detects significant differences between gastro-oesophageal reflux disease and normal subjects that are not detected by integrated acidity. [source] Amyloid , protein toxicity mediated by the formation of amyloid-, protein precursor complexesANNALS OF NEUROLOGY, Issue 6 2003Daniel C. Lu MD The amyloid-, protein precursor, a type 1 transmembrane protein, gives rise to the amyloid ,-protein, a neurotoxic peptide postulated to be involved in the pathogenesis of Alzheimer's disease. Here, we show that soluble amyloid , protein accelerates amyloid precursor protein complex formation, a process that contributes to neuronal cell death. The mechanism of cell death involves the recruitment of caspase-8 to the complex, followed by intracytoplasmic caspase cleavage of amyloid precursor protein. In vivo, the levels of soluble amyloid , protein correlated with caspase-cleaved fragments of the amyloid precursor protein in brains of Alzheimer's disease subjects. These findings suggest that soluble amyloid , protein,induced multimerization of the amyloid precursor protein may be another mechanism by which amyloid , protein contributes to synapse loss and neuronal cell death seen in Alzheimer's disease. Ann Neurol 2003;54:781,789 [source] | ||||||||||