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PubMed2004

Frequency of non-vascular accompanying diseases in patients with peripheral arterial disease.

BACKGROUND: Since there are so far no relevant figures on the frequency of non-vascular associated diseases in patients with peripheral arterial disease (PAD), an analysis was carried out to determine how frequently co-morbidity is found in such patients. PATIENTS AND METHODS: In the course of a retrospective analysis, 1693 patients (55.4% men, 44.6% women/average 68.6years) with peripheral arterial diseases (PAD) of Fontaine stage II to IV were examined to find out how often cardiopulmonary, gastrointestinal, haematological, endocrinological, neurological, orthopaedic, urological-nephrological, psychiatric diseases and tumours were seen. RESULT: Coronary ischaemic heart disease was present in 63.1% of the cases, heart failure existed in 20.7%, hypertension in 57.9%, endocrine and metabolic diseases in 60.4%, gastrointestinal diseases in 14.8%, pulmonary diseases in 14.5%, psychological and behavioural deficiencies in 16.2%, orthopaedic diseases in 21.6%, haematological diseases in 9.0%, urological diseases in 16.9%, chronic renal insufficiency in 7.3% and malignant tumours in 5.8%. CONCLUSIONS: The frequency of non-vascular comorbidity in patients with PAD necessitates systematic interdisciplinary diagnosis prior to any therapy decision to take into account the patient's associated diseases, life expectancy and current quality of life.

Aged↗

PubMed1976

Chest pain as a predictor of coronary artery disease in patients with obstructive aortic valve disease.

To clarify the association between chest pain and significant coronary artery disease in patients who have aortic valve disease, 76 consecutive candidates for aortic valve replacement were evaluated prospectively with use of a historical questionnaire and coronary arteriography. Of the 76 patients, 19 (25 percent) had no chest pain, 21 (28 percent) had chest pain that was not typical of angina pectoris and 36 (47 percent) had chest pain typical of anigina pectoris. In 18 of 19 patients the absence of chest pain correlated with the absence of coronary artery disease. The single patient without chest pain who had coronary artery disease had evidence of an inferior myocardial infarction in the electrocardiogram. Thus, absence of chest pain and the absence of electrocardiographic evidence of infarction predicted the absence of coronary disease in all cases. The presence of chest pain did not predict the presence of coronary artery disease, but the more typical the pain of angina pectoris the more likely were patients to have significant coronary artery disease. Of the 21 patients with atypical chest pain, 6 (29 percent) had coronary artery disease, but of the 36 patients with typical angina pectoris 23 (64 percent) had significant coronary artery disease. In addition, when patients with chest pain not typical of angina pectoris also had coronary artery disease, the diseased vessels usually supplied smaller areas of the left ventricle than when the pain was typical of angina pectoris. In 21 of 23 patients (91 percent) with typical angina pectoris and significant coronary artery disease, lesions were present in the left coronary artery. There was no systolic pressure gradient across the aortic valve that excluded the presence of coronary artery disease, although all patients with a calculated aortic valve area of less than 0.4 cm2 were free of coronary artery disease. Patients with severe left ventricular dysfunction were more likely to have normal coronary arteries.

Adult↗

PubMed2006

CARD15 in inflammatory bowel disease and Crohn's disease phenotypes: an association study and pooled analysis.

BACKGROUND: Three major polymorphisms of the Caspase-Activation Recruitment Domain containing protein 15 gene have been described to be associated with Crohn's disease. Genotype-phenotype studies reported in literature provide conflicting data on disease localisation and behaviour. We investigated the relation of Caspase-Activation Recruitment Domain containing protein 15 with inflammatory bowel disease and Crohn's disease phenotypic characteristics in a large Dutch cohort and performed a pooled analysis on inflammatory bowel disease patients and Crohn's disease phenotypic characteristics reported in association studies. METHODS: We genotyped 781 cases and 315 controls for the R702W, G908R and 1007fsinsC variants and for six microsatellite markers in and close to Caspase-Activation Recruitment Domain containing protein 15. In the pooled analysis data of 7201 inflammatory bowel disease patients and 3720 controls from 20 studies were included. RESULTS: Association was found for Crohn's disease with R702W and 1007fsinsC, including several disease characteristics, and not for ulcerative colitis. In the pooled analysis all three common Caspase-Activation Recruitment Domain containing protein 15 variants showed strong association with Crohn's disease (p<0.00001; odds ratio varying from 3.0 for single heterozygotes to 14.7 for compound heterozygotes) and not with ulcerative colitis. Phenotype analysis showed association with small bowel involvement, stricturing and penetrating disease. CONCLUSION: Caspase-Activation Recruitment Domain containing protein 15 is associated with Crohn's disease and not with ulcerative colitis. All three common Crohn's disease-associated variants are associated with small bowel involvement, the G908R and 1007fsinsC alleles also being associated with a complicated disease course.

Adolescent↗

PubMed2001

Coronary artery disease among diabetic and non-diabetic patients with end stage renal disease.

Cardiovascular disease is the major cause of death among patients with end stage renal disease and accounts for about half the deaths among the dialysis population. Several researchers have reported a high prevalence of coronary artery disease among diabetic patients with renal failure and coronary arteriography is often considered an integral part of the pre-transplant evaluation of diabetic patients with end stage renal disease. However, very few reports have addressed the question of coronary disease in non-diabetic patients, and the pattern and prevalence of coronary artery disease in non-diabetic patients with end stage renal disease are not well defined. We evaluated the clinical and coronary angiographic findings in 158 consecutive patients (84 diabetic and 74 non-diabetic) with end stage renal disease. The coronary arteries were divided into 16 segments and each segment was analyzed for the presence of coronary disease, which was defined as the presence of > or = 50% luminal diameter stenosis. Diabetic patients had more adverse risk factors for coronary artery disease, yet there was no significant difference in the prevalence of coronary artery disease between the diabetic and non-diabetic patients (67% vs. 55%, p = 0.15), or in the number of affected coronary artery segments (2.0 vs. 1.4, p = 0.05). Triple vessel coronary artery disease was however, significantly more common among the diabetic subjects (27% vs. 12%, p = 0.005). Non-diabetic patients with end stage renal disease also have a high prevalence of coronary artery disease and may merit as careful investigation of their coronary status as their diabetic counterparts.

Adult↗

PubMed2004

The law of mass action applied to neurodegenerative disease: a hypothesis concerning the etiology and pathogenesis of complex diseases.

Loci underlying autosomal dominant forms of most neurodegenerative disease have been identified: prion mutations cause Gerstmann Straussler syndrome and hereditary Creuzfeldt-Jakob disease, tau mutations cause autosomal dominant frontal temporal dementia, and alpha-synuclein mutations cause autosomal dominant Parkinson's disease. In all these cases, the pathogenic mutation is in the protein that is deposited in the diseased tissue and in these cases the whole protein is deposited. In Alzheimer's disease, mutations in APP or presenilin 1 or 2 cause autosomal dominant disease and these are the substrate and proteases, respectively, which are responsible for the production of the deposited peptide, Abeta. Thus, in all cases, the mutations lead to the disease by a mechanism that involves the deposition process. We briefly review this remarkably predictable biology, but also point out that it seems sporadic forms of all these diseases are predisposed to by genetic variability at the same loci, strongly suggesting that the quantity of the normal protein produced influences risk for the sporadic forms of the disease. The evidence for this assertion is strongest in Parkinson's disease (PD), where genetic variability in alpha-synuclein expression affects risk of developing disease, although the oldest evidence for the notion that increased expression of normal sequence protein can lead to disease comes from the observation of Alzheimer's disease in trisomy 21 cases. From these observations, we make predictions concerning the etiology and pathogenesis of neurodegenerative diseases in general.

Haplotypes↗

PubMed1993

Predictors of disease severity and survival in patients with coronary artery disease.

BACKGROUND: The objective of this study was to determine whether coronary angiographic findings and survival could be predicted using standard clinical and exercise-test data. METHODS: Five hundred and ninety-five men who had undergone both exercise treadmill testing and cardiac catheterization were followed for up to 5 years. Left main (LM) disease (> or = 50% stenosis) was present in 42 patients, whereas 30 patients had LM equivalent disease (> or = 70% stenosis of both the proximal left anterior descending and circumflex coronary artery disease (n = 152), one-vessel disease (n = 186), two-vessel disease (n = 118), three-vessel disease (n = 67), LM or LM equivalent disease without significant (> or = 70%) right coronary artery involvement (n = 26), and LM or LM equivalent disease with right coronary artery involvement (n = 46). RESULTS: ST-segment depression was more marked, whereas ejection fraction, maximal heart rate, maximal systolic blood pressure, and exercise capacity were lower in each group as disease severity worsened. Using Kaplan-Meier analysis, the subgroup with the poorest survival was found to be those with LM or LM equivalent disease with right coronary artery disease, and the next worse was the three-vessel disease group, in which survival was poorer than in all other subgroups (P < 0.01). Stepwise regression analysis revealed that the most powerful predictor for coronary artery disease severity was exercise-induced ST depression (P < 0.001), but it predicted survival poorly. History of congestive heart failure, low ejection fraction (50% or lower), and poor exercise capacity (5 metabolic equivalents or less) emerged as strong predictors of survival using stepwise Cox regression analysis (P < 0.001). CONCLUSION: Exercise-induced ST depression predicted the severity of angiographic disease but not survival, whereas the strongest predictors of survival were history of congestive heart failure, low ejection fraction, and poor exercise capacity.

Adult↗

PubMed2003

Tobacco consumption and disease duration are associated with fistulizing and stricturing behaviors in the first 8 years of Crohn's disease.

OBJECTIVES: Crohn's disease (CD) can be classified by site of involvement and by clinical behavior. Claims for stability of behavior are based on patients who have had surgery, usually within 8 yr of diagnosis. Disease during this period may begin as inflammatory and may evolve into stricturing or fistulizing behavior. Our aim was to determine the influence of genetic and environmental factors on the prevalence of inflammatory behavior among patients who have had CD for <8 yr. METHODS: Disease type (inflammatory, stricturing, or fistulizing), site, and duration were determined in 311 consecutive patients with CD. The analysis was then restricted to those with a disease duration of <8 yr, and influences on the prevalence of inflammatory type disease were compared with those of the complicated type (fistulizing and stricturing), including disease site, family history, Jewish ethnicity, and pack-years of cigarette smoking after diagnosis were determined through univariate and multivariate analyses. RESULTS: The prevalence of inflammatory type disease was 0.63 and 0.3 (p < 0.0001) in patients with a disease duration of <8 yr and > or =8 yr, respectively. Multivariate analysis revealed a 91% decrease in the odds of inflammatory disease among those with > or =1 pack-year of smoking after diagnosis among individuals with disease <8 yr. This was not influenced by disease location, family history of inflammatory bowel disease (IBD), or Jewish ethnicity. CONCLUSIONS: The prevalence of inflammatory CD decreased with time. Tobacco consumption was associated with this decline during the early phase of disease, suggesting that tobacco may influence the progression of inflammatory to stricturing or fistulizing type disease.

Adult↗

PubMed1997

Do disease specific characteristics add to the explanation of mobility limitations in patients with different chronic diseases? A study in The Netherlands.

STUDY OBJECTIVES: To determine whether disease specific characteristics, reflecting clinical disease severity, add to the explanation of mobility limitations in patients with specific chronic diseases. DESIGN AND SETTING: Cross sectional study of survey data from community dwelling elderly people, aged 55-85 years, in the Netherlands. PARTICIPANTS AND METHODS: The additional explanation of mobility limitations by disease specific characteristics was examined by logistic regression analyses on data from 2830 community dwelling elderly people. MAIN RESULTS: In the total sample, chronic non-specific lung disease, cardiac disease, peripheral atherosclerosis, diabetes mellitus, stroke, arthritis and cancer (the index diseases), were all independently associated with mobility limitations. Adjusted for age, sex, comorbidity, and medical treatment disease specific characteristics that explain the association between disease and mobility mostly reflect decreased endurance capacity (shortness of breath and disturbed night rest in chronic non-specific lung disease, angina pectoris and congestive heart failure in cardiac disease), or are directly related to mobility function (stiffness and lower body complaints in arthritis). For atherosclerosis and diabetes mellitus, disease specific characteristics did not add to the explanation of mobility limitations. CONCLUSIONS: The results provide evidence that, to obtain more detailed information about the differential impact of chronic diseases on mobility, disease specific characteristics are important to take into account.

Activities of Daily Living↗

PubMed2006

Clinical value of MDCT in the diagnosis of coronary artery disease in patients with a low pretest likelihood of significant disease.

OBJECTIVE: The aim of this study was to evaluate the clinical value of MDCT in the diagnosis of coronary artery disease in a population having a low pretest likelihood of significant disease. SUBJECTS AND METHODS: Sixty-four patients with suspected coronary artery disease and a low pretest likelihood of significant disease according to the criteria of the American Heart Association underwent both MDCT of the heart and quantitative conventional coronary angiography (QCA). MDCT examinations were performed on a 16-MDCT scanner. CT data sets were evaluated on a per-patient basis and a per-segment basis and were classified as indicating no disease, nonsignificant disease (stenoses </= 50%), or significant disease (stenoses > 50%). Sensitivity, specificity, negative predictive value (NPV), and positive predictive value (PPV) of 16-MDCT in the detection or exclusion of significant and nonsignificant coronary artery disease were evaluated on both per-patient and per-segment bases. RESULTS: Regarding the success rate of 16-MDCT, 94% (60/64) of patients and 92% (388/420) of vessel segments were of sufficient quality for diagnosis. In the remaining 60 patients evaluated, QCA revealed significant coronary artery disease, nonsignificant disease, and no disease in 8.3% (5/60), 75.0% (45/60), and 16.7% (10/60) of cases, respectively, on a per-patient basis, and in 1.3% (5/388), 23.2% (90/388), and 75.5% (293/388) of cases, respectively, on a per-segment basis. The sensitivity, specificity, NPV, and PPV of 16-MDCT for the detection of significant coronary artery disease were 80.0%, 94.5%, 98.1%, and 57.1%, respectively, on a per-patient basis, and 80.0%, 99.2%, 99.7%, and 57.1% on a per-segment basis. CONCLUSION: In a population having a low pretest likelihood of significant coronary artery disease, 16-MDCT shows a moderate to high sensitivity and high NPV for the detection or exclusion of significant disease, but has a somewhat reduced PPV compared with QCA.

Adult↗

PubMed2005

[Risk factors for cystic fibrosis related liver disease]

OBJECTIVE: To identify the risk factors for cystic fibrosis related liver disease. METHODS: Ten patients out of a total of 106 patients regularly followed-up during 1999 met the clinical, biochemical and/or ultrasound criteria for liver disease (9.4%). Using information from the admissions records at the service, we collected data on demography, genotype, age and manifestations at diagnosis of cystic fibrosis, nutritional status and laboratory findings. Variables associated with liver disease were initially identified by the Kaplan-Meier method. Those factors that were significant in the univariate analysis were included in the multivariate analysis by means of a Cox regression model. RESULTS: Under univariate analysis the following factors were associated with liver disease: male sex, age at diagnosis of cystic fibrosis, pancreatic insufficiency, z score for weight at admission, Shwachman score and biochemistry at admission. After adjustment by Cox model, two variables were independently associated with liver disease: Shwachman score (p = 0.0057) and age at diagnosis of cystic fibrosis (p = 0.014). CONCLUSIONS: The risk of developing liver disease is higher among patients diagnosed at an early age and those with worse clinical status as assessed by the Shwachman score, indicating that liver involvement might be part of a more severe form of the condition. These patients merit greater attention in terms of screening for liver disease and should be given treatment with ursodeoxycholic acid earlier in the event of abnormal findings.

Journal Article↗

PubMed1999

"Crohn's disease activity index" is inaccurate to detect the post-operative recurrence in Crohn's disease. A GISC study. Gruppo Italiano per lo Studio del Colon e del Retto.

BACKGROUND: Even though endoscopy is considered to be the gold standard for the detection of post-operative recurrence in Crohn's disease, the Crohn's disease activity index continues to be used for this purpose. AIM: The aim of this study was, therefore, to assess the accuracy of Crohn's disease activity index to detect post-operative recurrence in Crohn's disease in comparison to endoscopy. PATIENTS AND METHODS: The study was performed in 110 patients submitted to intestinal resection for Crohn's disease of the terminal ileum. Crohn's disease activity index score was assessed at 6, 12, 24 and 36 months after surgery. At the same intervals colon-ileoscopy was performed in all patients. The Receiver Operating Characteristic analysis was used to assess the accuracy of the Crohn's disease activity index score. Some routine laboratory variables were also analyzed. RESULTS: Crohn's disease activity index was able to discriminate patients with and without recurrence in 65% of the cases. Considering 150 the standard cut-off value of Crohn's disease activity index, this figure corresponds to a specificity of 89% and a sensitivity of 30%. Out of the laboratory variables erythrocyte sedimentation rate, chloraemia and serum albumin, globally considered in a discriminant function, showed a predictive ability significantly better than Crohn's disease activity index (z = 1.97; two tailed p-value = 0.04). These variables were able to discriminate patients with and without recurrence in 88% of the cases, and at the same value of specificity for the Crohn's disease activity index reached double value of sensitivity (65%). Positive and negative predictive values of laboratory variables (80% and 78%, respectively) were better than the corresponding values of the Crohn's disease activity index score (71% and 56%, respectively). CONCLUSIONS: These results indicate that the Crohn's disease activity index alone is inadequate for detection of post-operative recurrence in Crohn's disease.

Blood Cell Count↗

PubMed1991

Frequent detection of Epstein-Barr viral deoxyribonucleic acid and absence of cytomegalovirus deoxyribonucleic acid in Hodgkin's disease and acquired immunodeficiency syndrome-related Hodgkin's disease.

Epstein-Barr viral DNA (EBV DNA) has been detected in 20 to 58% of Hodgkin's disease tumors analyzed by Southern blotting or polymerase chain reaction (PCR). Because patients with Hodgkin's disease are generally immunodepressed, it is possible that the EBV is not directly involved in the pathogenesis of Hodgkin's disease but is merely detectable by molecular techniques because of reactivation of a latent infection. The purpose of this study was to determine if EBV DNA could be detected in an even higher percentage of cases of Hodgkin's disease, including acquired immunodeficiency syndrome (AIDS)-related Hodgkin's disease, by using newly designed, PCR amplification primers, and to compare the incidence of EBV DNA with the incidence of another common, latent virus (cytomegalovirus) in Hodgkin's disease tissue. The PCR was performed on DNA extracted from cells from 15 benign hyperplastic lymph nodes and from 15 cryopreserved cases of Hodgkin's disease, including 2 cases of AIDS-related Hodgkin's disease. For negative controls, PCR was also performed without template DNA and on genomic DNA from E. coli, calf thymus, a murine myeloma, and from a human cell line. After 32 cycles of amplification, a 225 base-pair amplification product comigrating with an EBV-positive control was detected in none of the negative controls but was present in 14 out of 15 cases (93%) of Hodgkin's disease, including both cases of AIDS-related Hodgkin's disease, and in 2 out of 15 cases of benign lymphoid hyperplasia. By contrast, cytomegalovirus DNA was undetectable by PCR in any of our specimens. We conclude that in our study set, the PCR procedure detected EBV-DNA but not cytomegalovirus DNA in a high percentage of cases of Hodgkin's disease, including two cases of AIDS-related Hodgkin's disease. These findings strengthen the hypothesis that EBV may be involved in the pathogenesis of Hodgkin's disease and AIDS-related Hodgkin's disease.

Acquired Immunodeficiency Syndrome↗

PubMed1995

Quality of life in inflammatory bowel disease: the interaction of disease activity with psychosocial function.

OBJECTIVES: To evaluate the disease-specific and psychological factors that influence quality of life (QOL) in patients with inflammatory bowel disease (IBD) as measured by the recently developed IBD disease-specific QOL measure the Inflammatory Bowel Disease Questionnaire (IBDQ). METHODS: Twenty-two patients (eight males and 14 females) were studied, 16 with Crohn's disease and six with ulcerative colitis (mean age 32 yr). Patients' disease activity was measured with the Dutch Crohn's activity index and the St. Mark's colitis activity index, and QOL was measured by the IBDQ. All patients completed self-report questionnaires for psychological function, which included the Sickness Impact Profile (SIP), the Symptom Checklist-90-R (SCL), and the Self-Control Schedule (SCS). RESULTS: By combining the psychological measures and the disease activity into a single variable, this composite variable significantly predicted QOL (r = 0.53, p < 0.02), but disease activity alone did not predict overall QOL. Improved psychosocial functioning (Sickness Impact Profile) predicted greater overall QOL (IBDQ total: r = -0.49, p < 0.05) and better social functioning (r = -0.65, p < 0.01). Greater psychological distress (SCL) and disease activity predicted more systemic symptoms [SCL-Positive Symptom Total (PST): r = -0.48, p < 0.05; disease activity: r = -0.65, p < 0.03) and poorer emotional functioning (SCL-PST: r = minus] 0.75, p < 0.001; disease activity: r = -0.82, p < .03). Disease type, gender, age of onset, and psychological coping (SCS) were not predictive of disease-specific QOL, but coping (SCS) was significantly correlated with psychological distress (r = -0.58, p < 0.05 for the SCL-90-PST). CONCLUSION: This study confirms the importance of psychological functioning in determining QOL of IBD patients and must be considered jointly with disease activity.

Adaptation, Psychological↗

PubMed1995

Disease-related worries and concerns: a study on out-patients with inflammatory bowel disease.

OBJECTIVE: To investigate the relationship between inflammatory bowel disease (IBD) patients' disease-related worries and concerns and their disease-related data, sociodemographic variables and perceived information level with respect to IBD. STUDY DESIGN: Prospective evaluation of disease-related concerns. SETTING: Out-patient IBD clinic of a university hospital. STUDY POPULATION: The study included 105 patients with IBD (72 with Crohn's disease and 33 with ulcerative colitis). MEASUREMENTS: Worries and concerns were measured using the standardized 25-item rating form of IBD patient concerns; actual disease activity was assessed by physicians using the Crohn's disease and clinical activity indices. Sociodemographic and other disease-related data were collected using a structured questionnaire. Patients' perceived information level was measured using a visual analogue scale. RESULTS: The issues of greatest concern to our patients were, in descending order of importance, having an ostomy bag (mean score +/- SD 63.6 +/- 38), the effects of medication (53.1 +/- 34), having surgery (51.6 +/- 36), the uncertain nature of the disease (46.5 +/- 32) and energy level (41.5 +/- 34). Patients with ulcerative colitis scored higher with respect to concern about loss of bowel control (P < 0.03). Disease-related worries and concerns correlated poorly with disease-related data (actual disease activity, severity of the course of IBD, diagnosis, disease duration or location, medication) but showed a significant negative correlation with patients' perceived information level about IBD (t = 0.2, P < 0.004). Lower information-level scores were associated with greater concerns. CONCLUSION: We conclude that the patients' information level about IBD and disease-related concerns have to be considered in clinical practice. Better information about IBD and psychosomatic counselling for patients who show high levels of concern may improve their quality of life and clinical care.

Adolescent↗

PubMed2006

CSF amyloid-beta-peptides in Alzheimer's disease, dementia with Lewy bodies and Parkinson's disease dementia.

As the differential diagnosis of dementias based on established clinical criteria is often difficult, biomarkers for applicable diagnostic testing are currently under intensive investigation. Amyloid plaques deposited in the brain of patients suffering from Alzheimer's disease, dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD) mainly consist of carboxy-terminally elongated forms of amyloid-beta (Abeta) peptides, such as Abeta1-42. Absolute Abeta1-42 levels in CSF have shown diagnostic value for the diagnosis of Alzheimer's disease, but the discrimination among Alzheimer's disease, DLB and PDD was poor. A recently established quantitative urea-based Abeta-sodium-dodecylsulphate-polyacrylamide-gel-electrophoresis with Western immunoblot (Abeta-SDS-PAGE/immunoblot) revealed a highly conserved Abeta peptide pattern of the carboxy-terminally truncated Abeta peptides 1-37, 1-38, 1-39 in addition to 1-40 and 1-42 in human CSF. We used the Abeta-SDS-PAGE/immunoblot to investigate the CSF of 23 patients with Alzheimer's disease, 21 with DLB, 21 with PDD and 23 non-demented disease controls (NDC) for disease-specific alterations of the Abeta peptide patterns in its absolute and relative quantities. The diagnostic groups were matched for age and severity of dementia. The present study is the first attempt to evaluate the meaning of Abeta peptide patterns in CSF for differential diagnosis of the three neurodegenerative diseases--Alzheimer's disease, DLB and PDD. The Abeta peptide patterns displayed disease-specific variations and the ratio of the differentially altered Abeta1-42 to the Abeta1-37 levels subsequently discriminated all diagnostic groups from each other at a highly significant level, except DLB from PDD. Additionally, a novel peptide with Abeta-like immunoreactivity was observed constantly in the CSF of all 88 investigated patients. The pronounced percentage increase of this peptide in DLB allowed a highly significant discrimination from PDD. Using a cut-off point of 0.954%, this marker yielded a diagnostic sensitivity and specificity of 81 and 71%, respectively. From several lines of indication, we consider this peptide to represent an oxidized alpha-helical form of Abeta1-40 (Abeta1-40*). The increased abundance of Abeta1-40* probably reflects a disease-specific alteration of the Abeta1-40 metabolism in DLB. We conclude that Abeta peptide patterns reflect disease-specific pathophysiological pathways of different dementia syndromes as distinct neurochemical phenotypes. Although Abeta peptide patterns failed to fulfil the requirements for a sole biomarker, their combined evaluation with other biomarkers is promising in neurochemical dementia diagnosis. It is noteworthy that DLB and PDD exhibit distinct clinical temporal courses, despite their similar neuropathological appearance. Their distinct molecular phenotypes support the view of different pathophysiological pathways for each of these neurodegenerative diseases.

Aged↗

PubMed2006

CT features of hepatic venoocclusive disease and hepatic graft-versus-host disease in patients after hematopoietic stem cell transplantation.

OBJECTIVE: We conducted this study to evaluate whether CT scans could be used to differentiate hepatic venoocclusive disease from hepatic graft-versus-host disease in patients treated with hematopoietic stem cell transplantation. SUBJECTS AND METHODS: We retrospectively evaluated 18 patients (eight women, 10 men; mean age, 42.4 years) after hematopoietic stem cell transplantation with biopsy-proven hepatic venoocclusive disease (n = 5), hepatic graft-versus-host disease (n = 6), or both (n = 7). Two radiologists reviewed abdominal and pelvic CT scans for hepatomegaly (> 18 cm), splenomegaly (> 13 cm), size of main portal and right hepatic veins, presence of periportal edema, gallbladder wall edema, hydropic gallbladder, ascites, and small-bowel wall thickening. CT and histopathology findings were correlated using analysis of variance and Fisher-Free-man-Holton tests. RESULTS: Ascites and periportal edema were present in all five patients with venoocclusive disease, but of six patients with graft-versus-host disease, ascites was seen in two (p < 0.05) and periportal edema in only one (p < 0.05). Small-bowel wall thickening was encountered in five patients with graft-versus-host disease and in none with venoocclusive disease (p < 0.05). The right hepatic vein diameter in patients with venoocclusive disease (mean, 0.27 cm) was significantly smaller than the right hepatic vein diameter in patients with graft-versus-host disease (mean, 0.87 cm; p < 0.05). CONCLUSION: In patients treated with hematopoietic stem cell transplantation, CT findings of periportal edema, ascites, and a narrow right hepatic vein suggest venoocclusive disease rather than graft-versus-host disease. Small-bowel wall thickening suggests graft-versus-host disease.

Adult↗

PubMed2004

Systemic connective tissue disease complicated by Castleman's disease: report of a case and review of the literature.

Castleman's disease is an atypical lymphoproliferative disorder characterized by the prevalence of B CD5-positive cells in the marginal zone. Autoimmune manifestations have often been reported, but the association of Castleman's disease with systemic autoimmune syndromes has been rarely described. However, many authors stress the difficulties in distinguishing between connective tissue disease and Castleman's disease in most cases. To clarify this issue, we describe a patient and review the literature reports of all cases of Castleman's disease associated with a connective tissue disease. A 19-year old woman presented with autoimmune thyroiditis and polymyositis. Seven years after the onset she developed a systemic inflammatory flare and a burst of autoimmunity, followed by generalized lymphoadenopathy. A mediastinal lymph node biopsy led to the diagnosis of Castleman disease of mixed type. Chemotherapy was given, with rapid response of the lymphoproliferative disorder but persistence of the underlying autoimmune disorder. The plasma concentration of B-lymphocyte stimulator (BLyS) was high (13.3 ng/mL) at the diagnosis of Castleman's disease. It fell dramatically after chemotherapy (4.97 ng/mL), even though it remained just above the mean BLyS value found in healthy blood donors (3.37+/-0.78 ng/mL).Castleman's disease can present autoimmune traits. In our patient, Castleman's disease complicated the course of a connective tissue disorder several years after the onset. We hypothesize that chronic stimulation of B-cell clones, particularly CD5+, by BLyS could favor the development of both autoimmune diseases and a broad range of lymphoproliferative disorders (such as Castleman's disease). This is the first report of increased BLyS levels in a patient with Castleman's disease, supporting a possible pathogenetic role of BLyS in the development of an autoimmune disorder and of a B lymphoproliferative disorder years later.

Adult↗

PubMed2006

Pathophysiological characteristics of the various forms of gastro-oesophageal reflux disease. Spectrum disease or distinct phenotypic presentations?

BACKGROUND: The traditional approach to gastro-oesophageal reflux disease as a spectrum disease has recently been criticised and the distinct phenotypic presentations model has been proposed. AIM: To evaluate the main pathophysiological characteristics of various gastro-oesophageal reflux disease presentations. METHODS: Oesophageal manometry and 24-h pH-monitoring were performed in a gastro-oesophageal reflux disease series collected in a 7-year period. RESULTS: Four hundred and twenty-one subjects were studied. Mean total percentage acid reflux time was significantly higher in long-segment Barrett's oesophagus and in ulcerative oesophagitis than in all the other gastro-oesophageal reflux disease groups, whilst in short-segment Barrett's oesophagus results were quite similar to those found in non-erosive reflux disease and in erosive reflux disease. Patients with ulcerative oesophagitis and long-segment Barrett's oesophagus were older than all the other gastro-oesophageal reflux disease groups. The mean lower oesophageal sphincter pressure was significantly reduced in non-erosive reflux disease, erosive reflux disease, ulcerative oesophagitis, short-segment Barrett's oesophagus and long-segment Barrett's oesophagus as compared with functional heartburn and hypersensitive oesophagus and with controls. CONCLUSIONS: In keeping with the spectrum model of gastro-oesophageal reflux disease, severity of acid reflux increases from non-erosive reflux disease through erosive reflux disease up to ulcerative oesophagitis and long-segment Barrett's oesophagus. Ulcerative oesophagitis and long-segment Barrett's oesophagus could represent an advanced step in the natural history of gastro-oesophageal reflux disease. Our results do not confirm the distinct phenotypic presentations hypothesis.

Adult↗