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At least 73 records · Page 4Linked to original sources

The relationship between Alzheimer's disease, Parkinson's disease and motor neuron disease.

We argue against the dominant status assigned to conventional microscopy in the categorization of disorders such as Alzheimer's disease, Parkinson's disease and ALS. As an example we criticize the emphasis that has been placed on correlating the presence of Lewy bodies with the diagnosis of Parkinson's disease. In essence, we submit that Parkinson's disease can exist without Lewy bodies, and Lewy bodies can exist without Parkinson's disease. Nevertheless, we consider that the newer techniques available to histology have led to an important concept that constitutes a shared feature for Alzheimer's disease. Parkinson's disease and ALS; they are all characterized by the deposition of cytoskeletal debris in tissue, so they may perhaps be collectively termed the "Cytoskeletal Disorders".

Aged↗

Clinical presentation, course, and prognostic factors in lymphocyte-predominant Hodgkin's disease and lymphocyte-rich classical Hodgkin's disease: report from the European Task Force on Lymphoma Project on Lymphocyte-Predominant Hodgkin's Disease.

PURPOSE: Recent studies have suggested that lymphocyte-predominant Hodgkin's disease (LPHD) is both clinically and pathologically distinct from other forms of Hodgkin's disease, including classical Hodgkin's disease (CHD). However, large-scale clinical studies were lacking. This multicenter, retrospective study investigated the clinical characteristics and course of LPHD patients and lymphocyte-rich classical Hodgkin's disease (LRCHD) patients classified according to morphologic and immunophenotypic criteria. MATERIALS AND METHODS: Clinical data and biopsy material of all available cases initially submitted as LPHD were collected from 17 European and American centers, stained, and reclassified by expert pathologists. RESULTS: The 426 assessable cases were reclassified as LPHD (51%), LRCHD (27%), CHD (5%), non-Hodgkin's lymphoma (3%), and reactive lesion (3%); 11% of cases were not assessable. Patients with LPHD and LRCHD were predominantly male, with early-stage disease and few risk factors. Patients with LRCHD were significantly older. Survival and failure-free survival rates with adequate therapy were similar for patients with LPHD and LRCHD, and were stage-dependent and not significantly better than stage-comparable results for CHD (German trial data). Twenty-seven percent of relapsing LPHD patients had multiple relapses, which is significantly more than the 5% of relapsing LRCHD patients who had multiple relapses. Lymphocyte-predominant Hodgkin's disease patients had significantly superior survival after relapse compared with LRCHD or CHD patients; however, this was partly due to the younger average age of LPHD patients. CONCLUSION: The two subgroups of LPHD and LRCHD bore a close clinical resemblance that was distinct from CHD; the course was similar to that of comparable nodular sclerosis and mixed cellularity patients. Thorough staging is necessary to detect advanced disease in LPHD and LRCHD patients. The question of how to treat such patients, either by reducing treatment intensity or following a "watch and wait" approach, remains unanswered.

Adult↗

Quality of care of children with chronic diseases in Alexandria, Egypt: the models of asthma, type I diabetes, epilepsy, and rheumatic heart disease. Egyptian-Italian Collaborative Group on Pediatric Chronic Diseases.

OBJECTIVES: To evaluate the quality of care delivered to children suffering from index chronic diseases using specific indicators of health care delivery and to study the predictors of suboptimal quality of care (SQC) and its outcome on children. DESIGN: Over a 9-month period, guidelines for optimal care were formulated. A specific questionnaire for every studied chronic disease was prepared in collaboration with the clinicians in charge of the diseased children (66% pediatricians and pediatric specialists and 34% adult specialists). The clinicians were asked to write the details of daily practice, ie, how these children were managed on a routine basis as well as in an emergency situation. A cross-sectional study was conducted over a 4-month period and included 953 children suffering from bronchial asthma (BA), childhood epilepsy (CE), type I diabetes mellitus (IDDM), and rheumatic heart disease (RHD). A systematic random sample of children was selected from children visiting the ambulatory settings of all children's hospitals. Every fourth child was selected on 2 randomly chosen days each week, while all diseased children admitted in the hospital settings of the children's hospitals during the study were included. A general form describing the impact of the diseases on the child was prepared. A network of clinicians was created in all children's hospitals; seminars were held during which the content validity of the questionnaire was tested. Items were evaluated for their internal consistency using the Cronbach alpha. According to the degree of adherence to the recent therapeutic guidelines concerning selected indicators of the quality of care specific to every disease, children were categorized as receiving optimal quality of care or SQC. These indicators were: the use of inhaled bronchodilators in acute asthmatic attacks in mild asthma and the use of the prophylactic drugs (inhaled sodium cromoglycate or inhaled beclomethasone) in moderate to severe chronic BA in between acute asthmatic attacks; compliance with antiepileptic drugs in epileptic children; regular performance of self-monitoring of blood glucose and/or urine testing in diabetic children; and compliance with prophylactic antibiotics in children suffering from RHD. The records of the outpatient clinics for ambulatory and hospitalized cases were reviewed to assess the degree of compliance with the prescribed management before the index visit. Sociodemographic characteristics and health care system-related predictors of SQC were analyzed via stepwise logistic regression analysis. The impact of illness on the child was assessed by 7 items which were: dependence on parents in domestic activities, level of activity compared with peers, mood compared with peers, level of socializing, degree of discomfort attributable to illness, level of physical disadvantage, and urinary incontinence. Factor analysis with Varimax rotation was performed on items related to the impact of illness. Parental satisfaction with care was rated as excellent, very good, fair, or poor. Information on school outcome was obtained by asking the caretakers whether the child was able to attend school regularly despite his sickness. Scholastic achievement was also rated as excellent, very good, good, and acceptable. Parents were asked whether the child had ever repeated a grade because of his sickness. SETTING: Ambulatory and hospital settings of all children's hospitals in Alexandria, Egypt. RESULTS: Only 52% of mild asthmatics were given inhaled bronchodilators during acute attacks and 6.84% of moderate to severe asthmatics were taking prophylactic drugs (inhaled sodium cromoglycate and/or inhaled beclomethasone) between acute attacks. Similarly, only 53 of 134 (39.6%) of diabetic children were regularly performing self-monitoring of blood glucose and/or urine testing. In contrast, in epileptic children, 121 of 173 (69.9%) were judged as being compliant by their managing clinicians and more than two

Adolescent↗

[An autopsy case of Alzheimer's disease associated with Parkinson's disease, compared to 2 autopsy cases of diffuse Lewy body disease].

A 68 year old male, diagnosed as Alzheimer's disease clinically, pathologically showed both findings of Alzheimer's disease and Parkinson's disease. The brain weight was 940 g. Macroscopically, severe cortical brain atrophy and depigmentation of the substantia nigra was noted. Microscopic examination showed marked appearance of senile plaque and a large number of neurofibrillary tangle with sever neuronal loss of the cerebral cortex. Additionally, the loss of neuron with many Lewy bodies was found in the substantia nigra. Lewy bodies were also found in the locus ceruleus and the dorsal vagal nucleus, but few in the cerebral cortical neurons. We compared this case neuropathologically with two autopsy cases of diffuse Lewy body disease (DLBD). There was no distinction concerning the lesions of the brain stem between this case and the cases of DLBD. In all three cases, the nucleus of basalis of Meynert showed marked neuronal loss. However, the brain was lighter than those of the cases of DLBD. Senile changes such as senile plaque and neurofibrillary tangles were more marked in this cases than in the cases of DLBD. Furthermore a large number of cortical Lewy bodies were found in the cases of DLBD, but few in this case. The distribution and number of Lewy bodies did not correspond with those of senile changes in the cases of DLBD. Also the cerebral cortical structure was better preserved in the cases of DLBD than in this case. In conclusion, from the clinicopathological findings, we considered that this case is Alzheimer's disease associated with Parkinson's disease. According to Kosaka's study, this case seemed to correspond with a transitional type of the Lewy body disease.

Aged↗

Left main coronary artery disease. Clinical and angiographic features. A retrospective study regarding two groups of patients with left main coronary artery disease and three-vessel disease.

BACKGROUND: The aim of this study was to assess the ability of clinical and instrumental features to identify patients with left main coronary artery disease (LMCD) compared with a three-vessel coronary artery disease group. METHODS: A cohort of 70 patients with LMCD was matched with another one of 66 patients with three-vessel disease. A history of angina before angiography was similar in both groups; the higher degrees of stable angina and the forms of unstable angina were moderately prevalent in the group with LMCD. RESULTS: In the last subgroup a significantly reduced incidence of previous acute myocardial infarction (AMI) was observed (p < 0.05). The resting electrocardiogram (ECG) showed higher incidence of atrial fibrillation (fa) and left bundle branch block (BBS) in the subjects with LMCD, with a statistic value (p < 0.05). The exercise test performed by a lot of patients appeared equally positive for inducible ischemia in the 2 groups. Significantly higher exercise peak load was achieved by the patients with three-vessel disease (p < 0.05). The coronary angiography showed a prevalence of right dominant circulation in the 2 groups; significantly the collateral circulation was more represented in the subjects with three-vessel disease (p < 0.05). Most patients with LMCD underwent a bypass coronary artery graft surgery (CABG surgery) more frequently than the ones with three-vessel disease (p < 0.01). In the former group the cardiovascular mortality within an average 2-year follow-up proved higher as to the latter group even if without statistic significance. CONCLUSIONS: Nevertheless this retrospective study showed some limitations. Particularly the incidence of clinical and instrumental variables and their capacity to differentiate LMCD patients from those with three-vessel disease were not demonstrated.

Acute Disease↗

The quantitative measurement of autoantibodies to thyroglobulin and thyroid peroxidase by automated microparticle based immunoassays in Hashimoto's disease, Graves' disease and a follow-up study on postpartum thyroid disease.

The aim of this study was to investigate the association between various autoimmune thyroid diseases and the presence of anti-TPO and anti-Tg antibodies using two novel automated microparticle based immunoassays developed for the AxSYM analyzer. Serum samples from 65 individuals with Hashimoto's Disease, 38 with Graves' Disease and 80 UK blood donors were assayed. In addition, samples were taken from 50 women known to be positive for TPO antibodies, for up to 24 weeks following delivery. Precision for both assays ranges from 5.7-9.1% CV, while analytical sensitivity was determined to be 1.0 IU/ml for Anti-Tg and 0.3 IU/ml for Anti-TPO. The Anti-TPO test showed positive results in 86% of Hashimoto's Disease and 87% of Graves' Disease. The figures obtained for Anti-Tg were 58% and 73% respectively. Specificity was 94% with Anti-TPO and 99% with Anti-Tg. The postpartum women were divided into 2 groups, group A remained symptomless while group B developed thyroid dysfunction. Within the 2 groups, medians calculated at each time point were compared between and within groups using the Mann-Whitney Rank Sum Test or the Kruskal-Wallis One Way ANOVA on Ranks. Anti-TPO baseline levels (week 6) were statistically different between both groups (median 36 vs. 167 IU/ml, p = 0.002). In group A, the median values increased from 36 to 87 IU/ml within the observation period, although the difference was not statistically significant. In group B, antibody titres showed a statistically significant increase from 168 IU/ml (week 6) up to 676 IU/ml after 20 weeks (p < 0.001). Anti-Tg baseline levels were not statistically different between the two groups. In group A, the median values did not change significantly over time (range: 47-86 IU/ml) whereas antibody titres in group B showed a statistically significant increase from 79 IU/ml (week 6) to 276 IU/ml after 24 weeks (p = 0.002). Results obtained indicate that these assays provide useful tools for the quantitative determination of autoantibodies in both primary diagnosis as shown with the Hashimoto's disease and Graves' disease samples and patient follow-up as demonstrated with the postpartum samples. The automation and high precision of the assays make them perfectly suited to routine diagnostic use.

Autoanalysis↗

Association of celiac disease with connective tissue diseases and autoimmune diseases of the digestive tract.

Celiac disease (CD) is an autoimmune enteropathy triggered by the ingestion of gluten in susceptible individuals, and is one of the most frequent genetically based diseases, with a prevalence of 1:200 in the general population. The association between CD and connective tissue diseases (CTD) and autoimmune diseases of the digestive tract (DT) has been described in several case reports but in few extensive studies, with varying prevalence. A high rate of false positive results were observed when low specific tests, such as the anti-gliadin and the guinea pig tissue transglutaminase (tTG) antibody assays were used. In a study of 400 patients with CTD and 218 with autoimmune DT disease, tested for IgA and IgG anti-tTG using the more specific human recombinant antigen, 12 cases (1.9%) of anti-tTG antibody positivity were found, but only 2 (0.3%) were confirmed as affected by CD following small bowel biopsy. Most of the patients testing false positive had primary biliary cirrhosis. In this short review we describe the association between CD and CTD, inflammatory bowel disease and primary biliary cirrhosis, with special emphasis on the diagnostic accuracy of CD antibody assays.

Animals↗

Early undifferentiated connective tissue disease. IV.Musculoskeletal manifestations in a large cohort of patients with undifferentiated connective tissue diseases compared with cohorts of patients with well-established connective tissue diseases: followup analyses in patients with unexplained polyarthritis and patients with rheumatoid arthritis at baseline.

OBJECTIVES: To examine the musculoskeletal manifestations in a large cohort of patients (n = 410) diagnosed with either a well-established connective tissue disease (CTD) (n = 197) or an early undifferentiated CTD (n = 213) with a symptom duration of <1 year. This study was aimed at determining the predictive value of demographic, clinical, and laboratory features on outcome in patients with unexplained polyarthritis (UPA) (from the early undifferentiated CTD cohort; n = 67) or rheumatoid arthritis (RA) (from the well-established CTD cohort; n = 57), over a 5-year followup period. METHODS: Patients from both cohorts were assessed at years 1, 3, and 5. At the study visits, clinical data were collected in a standardized manner, and sera were obtained and stored. A priori criteria were established for patient ascertainment and diagnosis over the duration of the study. Standard statistics were used for comparisons of baseline characteristics in patients diagnosed as having systemic lupus erythematosus, RA, undifferentiated CTD, and UPA at entry into the cohorts. Baseline features in patients with UPA were examined according to the different subsequent outcomes (RA, CTD, or undifferentiated CTD, remission [nonpersistent], or persistent or active UPA). Baseline features in patients with RA whose disease remained active versus those in whom remission was attained were also examined. Two multivariable analyses, classification trees and polychotomous logistic regression, were performed to predict disease outcomes over time. RESULTS: The overall rate of ascertainment for the 410 patients ranged from 90 % at year 1 to 71 % at year 5. Patients with established CTDs showed a tendency for more stable diagnoses than those with early undifferentiated CTDs (90-100% versus 45-70%). Consistent baseline predictors of persistent active disease among patients with RA, in both univariate and multivariable analyses, were higher joint counts for pain and tenderness and higher erythrocyte sedimentation rate (ESR). In approximately 20% of patients who were classified as having RA when they originally entered the cohort, the disease was in remission at 5 years. Twenty percent of the patients originally classified as having UPA developed RA over the duration of the study. These patients tended to be older and to have swelling of small joints at baseline. However, a consistent pattern of predictive variables could not be identified in the multivariable analyses, other than at year 1 (higher small joint counts for swelling and higher ESR). CONCLUSION: Baseline features (joint counts, and ESR) among RA patients were variously predictive of persistently active disease at years 1-5. Consistent baseline predictors of outcome among patients with UPA only emerged at year 1. Remission occurred in approximately 20% of RA patients, whereas a similar percentage of patients with UPA developed RA. These findings have implications with regard to treatment decisions in patients with early RA and/or UPA.

Adult↗

Frequent occurrence of non-specific gliadin antibodies in chronic liver disease. Endomysial but not gliadin antibodies predict coeliac disease in patients with chronic liver disease.

BACKGROUND: The frequency of gliadin antibody (GA) positivity has been found to be increased among patients with chronic liver disease, as has that of coeliac disease (CD). CD has also been found to be increased among patients with primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). METHODS: To investigate these relationships further, a micro-enzyme-linked immunosorbent assay and immunofluorescence tests for GAs and endomysial antibodies (EMAs) were performed in large subgroups of patients representing various chronic liver diseases and in healthy blood donors. RESULTS: As compared with blood donors (among whom it was 5%) the frequency of IgA GA positivity was higher in all patient subgroups: alcoholic liver disease, 20% (22 of 110, P < 0.001); PBC, 16% (16 of 101, P < 0.001); PSC, 24% (19 of 80, P < 0.001); chronic hepatitis, 19% (13 of 70, P < 0.001); and hepatitis C virus infection, 11% (11 of 104, P < 0.01). Two patients with autoimmune chronic hepatitis were EMA-positive, and in both cases the presence of CD was verified by small-bowel biopsy. CONCLUSIONS: IgA GA positivity generally occurs at increased frequency among patients with chronic liver disease and may represent non-specific immune activation. In liver disease GA testing is not useful in screening for CD, whereas the EMA test seems to be highly specific. CD is more prevalent than expected among patients with autoimmune chronic hepatitis but not among those with PBC or PSC.

Adult↗

Raised serum activity of phospholipase A2 immunochemically related to group II enzyme in inflammatory bowel disease: its correlation with disease activity of Crohn's disease and ulcerative colitis.

Calcium dependent phospholipase A2 activity in the mixed micelles of 1-palmitoyl-2-oleoyl-phosphatidylglycerol and cholate was measured in sera of 39 patients with Crohn's disease, 40 patients with ulcerative colitis, and 40 healthy controls. The phospholipase A2 activity was significantly raised in those sera of the patients with active Crohn's disease and those with moderate and severe ulcerative colitis. The major phospholipase A2 activity derived from the sera was separated into two peaks by reverse phase high performance liquid chromatography. The phospholipase A2 active fractions were immunochemically characterised using specific antibody directed against human group II phospholipase A2 purified from rheumatoid synovial fluid. The results suggest that raised serum phospholipase A2 activity in patients with Crohn's disease and ulcerative colitis was mainly attributed to the two forms of phospholipase A2 immunochemically related to group II enzyme. In patients with Crohn's disease, serum phospholipase A2 activity decreased in parallel with clinical improvement, and correlated with serum C-reactive protein and erythrocyte sedimentation rate. The results suggest that serum phospholipase A2 activity may serve as an additional indicator of disease activity. Serum phospholipase A2 activity in patients with ulcerative colitis tends to increase in relation with endoscopic severity, and may be a more sensitive laboratory index than serum C-reactive protein and erythrocyte sedimentation rate to evaluate disease activity.

Acute Disease↗

Breathing pattern during and after maximal exercise in patients with chronic obstructive lung disease, interstitial lung disease, and cardiac disease, and in normal subjects.

Inspiratory muscle fatigue and pulmonary edema are both known to cause rapid shallow breathing. It has been suggested that exercise tolerance in patients with pulmonary disease and cardiac disease may be limited by the development of inspiratory muscle fatigue and pulmonary edema, respectively, at maximal exercise. If these hypotheses are correct, breathing pattern during recovery from maximal exercise in these patients should be rapid and shallow compared with that during exercise. This study was performed to test these hypotheses. Seven patients with chronic obstructive pulmonary disease (COPD), 8 patients with interstitial lung disease (ILD), 7 patients with cardiac disease (CD) (mitral valve disease or left ventricular dysfunction) and 8 normal (NR) subjects each performed maximal incremental exercise on a cycle ergometer. Exercise breathing pattern was compared with that during recovery by calculating the mean difference in tidal volume (at the same levels of minute ventilation) between exercise and recovery for each subject. Recovery breathing pattern was similar to that during exercise for the COPD, ILD, and NR subjects. In contrast, breathing pattern during recovery was rapid and shallow compared with that during exercise for the CD patients; recovery tidal volume was less than that during exercise for the same level of minute ventilation. The fact that rapid shallow breathing does not develop during recovery from maximal exercise in patients with COPD or ILD suggests that inspiratory muscle fatigue does not limit their exercise tolerance. The relative rapid shallow breathing during recovery from maximal exercise in patients with CD is probably due to the development of pulmonary edema at maximal exercise, but further studies are needed to confirm this.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Tubulointerstitial renal diseases of children: pathologic features and pathogenetic mechanisms in Fanconi's familial nephronophthisis, antitubular basement membrane antibody disease, and medullary cyst disease.

The renal lesions of 15 children with tubulointerstitial diseases (TID), originally diagnosed as Fanconi's familial nephronophthisis (FFN), medullary cyst disease (MCD), or antitubular basement membrane (TBM) antibody disease were studied by light, electron, and immunofluorescence microscopy and by peroxidase-antiperoxidase stain for IgG. Kidneys originally considered in all three categories showed similar pathologic findings, with periglomerular fibrosis, interstitial fibrosis and infiltration, TBM thickening, and glomerulopathy. The data suggest that MCD and FFN are not synonyms, but that FFN can lead to medullary cystic disease, as can other tubulointerstitial diseases. Anti-TBM disease appears to be a more frequent cause of TID than is usually recognized. Glomerular involvement in TID may be due to deposition of anti-TBM antibody/TBM antigen complexes.

Autoimmune Diseases↗

Control of communicable diseases; apprehension and detention of persons with specific diseases; transfer of regulations. Food and Drug Administration and Centers for Disease Control and Prevention, HHS. Final rule.

The Secretary of Health and Human Service (the Secretary) is transferring a portion of the Food and Drug Administration (FDA) "Control of Communicable Diseases" regulations to the Centers for Disease Control and Prevention (CDC). In general, these regulations provide the Secretary with the authority to apprehend, detain, or conditionally release individuals to prevent the spread of specified communicable diseases. The regulations implement the provisions of the Public Health Service Act (PHS Act) to prevent the introduction, transmission, or spread of communicable diseases from one State or possession into any other State or possession. CDC will have authority for interstate quarantine over persons, while FDA will retain regulatory authority over animals and other products that may transmit or spread communicable diseases. The Secretary is taking this action to consolidate regulations designed to control the spread of communicable diseases, thereby increasing the agencies' efficiency and effectiveness.

Centers for Disease Control and Prevention, U.S.↗

Chronic disease management: treating the patient with disease(s) vs treating disease(s) in the patient.

The treatment of chronic disease is often complicated by the coexistence of multiple medical conditions and by the presence of social and psychological impediments. The needs posed by patients with chronic disease are overwhelming the capacity of the American health care system. Alternative disease management systems that rely on specially trained nurse case managers to implement detailed clinical protocols, including drug algorithms, have shown efficacy in managing chronic medical conditions, singly and in combination. By fostering integration of care across subspecialty and medical-social boundaries, such systems enable treatment of the patient with disease(s), not simply treatment of disease(s) in the patient. Working closely with primary care physicians, often by telephone-mediated interaction with patients, nurse case managers may take an expanded role in meeting the challenges posed by chronic disease.

Case Management↗