An autopsy case of idiopathic enlargement of the right atrium, and a review of the literature

A 69-year-old man in whom idiopathic enlargement of the right atrium was revealed at autopsy is described. The patient had had cardiomegaly of at least 19 years' duration prior to his death, even though cardiac symptoms were absent. Cause of death was pancreatic carcinoma. Postmortem examination revealed marked and diffuse dilatation of the right atrium and moderate dilatation of the left atrium. Measurement of the cardiac chambers showed that the right and left atria were 7.6 and 4.7 times as large as those of normal hearts, respectively. The volume of either ventricle was about twice the normal value. Histologically, widespread cardiac muscular degeneration and necrosis, diffuse fibrosis, and focal lymphocytic infiltration were found in the right atrium and also, to a lesser degree, in the left atrium. Such pathologic changes were not found in either of the ventricles. The etiology of these muscular changes, which might have been related to atrial enlargement, was unclear. The present case was thought to be consistent with idiopathic enlargement of the right atrium, and a brief review of the literature is given.     

HLA class I genotyping including HLA-B*51 allele typing in the Iranian patients with Behçet's disease

It is well known that Beh?et's disease (BD) is strongly associated with human leukocyte antigen (HLA) B51 in many ethnic groups. However, there has been no published report as yet with respect to this association among the Iranian people. Furthermore, since it is now known that the B51 antigen can be encoded by 21 alleles, B*5101-B*5121, we performed HLA-B*51 allele typing as well as HLA class I genotyping of 48 Iranian patients with this disease. As a result, the frequency of the B*51 allele was significantly higher (62.1%) in the patient group as compared with the ethnically matched control group (31.8%) (Pc=0.067, R.R.=3.51). In the genotyping of B*51 alleles, 33 out of the 36 B*51-positive patients possessed B*5101 and the remaining 3 carried B*5108. This study revealed that Iranian patients with BD also had a strong association with HLA-B51. In addition, this significantly high incidence of HLA-B*51 was found to be caused by an increase in both the HLA-B*5101 and HLA-B*5108 alleles. However, there was no significant difference in the HLA-B*51 allelic distribution between the patient and control groups.     

Significant associations of HLA-B*5101 and B*5108, and lack of association of class II alleles with Behçet's disease in Italian patients

Beh?et's disease has been known to be strongly associated with human leukocyte antigen (HLA) B51, one of the split antigens of HLA-B5. An increased incidence of HLA-B51 in the patient group has also been reported in an Italian population. Since the B51 antigen has been recently identified to comprise nine alleles, B*5101-B*5109, we performed HLA-B51 allele genotyping by the polymerase chain reaction-sequencing based typing (PCR-SBT) method as well as serological HLA-A and -B typing among 21 Italian patients with Beh?et's disease in order to investigate whether there is any correlation of one particular B51-associated allele with Behcet's disease. In addition, HLA class II genotyping was performed by the PCR-restriction fragment length polymorphism (RFLP) method. As a result, only the phenotype frequency of the B51 antigen was found to be significantly increased in the patient group as compared to the ethnically matched control group by the corrected P-value analysis (71.4% in patients vs. 17.9% in controls; chi2 = 14.26, Pc = 0.0042, R.R. = 11.5). In the B51 allele genotyping, 11 out of 15 B51-positive patients were B*5101 and the remaining four were B*5108, whereas all of 5 normal controls were B*5101, showing significant association of each allele with Beh?et's disease. No significant difference was observed between the patient and control groups in the HLA class II allelic distribution. This study revealed a strong association of Beh?et's disease in Italian with B*5108 as well as B*5101, providing important insight into the molecular mechanism underlying an HLA association with Beh?et's disease.     

Association of gastric epithelial apoptosis with the ability of Helicobacter pylori to induce a neutrophil oxidative burst

Both polymorphonuclear cell infiltration and increased epithelial apoptosis are seen in gastric mucosa in the presence of Helicobacter pylori infection. This study examined the association between bacterial ability to stimulate an oxidative burst in neutrophils and epithelial apoptosis. Biopsy specimens were obtained from 15 patients to detect apoptotic cells by the TUNEL method. H. pylori strains isolated from corresponding stomach biopsy samples were tested for the ability to stimulate an oxidative burst in human neutrophils. Neutrophils were isolated from healthy subjects without H. pylori infection and the oxidative burst was measured by flow cytometry with dichlorofluorescein diacetate. Stimulation with H. pylori increased both the percentage of activated cells and fluorescence intensity. There was a significant positive correlation between the number of epithelial apoptotic cells and fluorescence intensity. Increased neutrophil oxidative burst stimulated by H. pylori may play a role in enhanced gastric mucosal DNA damage and consequent atrophic gastritis and gastric cancer.     

Transition from Film to Electronic Media in the First-Year Medical School Gross Anatomy Lab

For the benefit of the first-year gross anatomy students, we digitized and published on a Web site images that had been collected over a 30-year period. We provided a CD-ROM (compact disk, read-only media) containing the image set in higher quality format to students and faculty. We supplemented basic images with hot topics such as CT angiography, virtual colonography, computer-aided diagnosis, and 3D post-processing. Full motion video and moving JPEG (Joint Photo Expert Group) animations were integrated into the atlas. On the post course questionnaire medical students reported that the images on CD-ROM were helpful during the course and for review prior to examinations. Faculty and medical students used the CD-ROM for problem-based learning sections and facilitator training. The images were clear and easily projected during review sessions and were useful for the small group sessions, where they served as examples of normal anatomy.     

Responses of lateral hypothalamic glucose-sensitive and glucose-insensitive neurons to chemical stimuli in behaving rhesus monkeys.

1. Extracellular single neuron activity was recorded in the lateral hypothalamic area (LHA) of awake, behaving monkeys, with particular regard to the feeding-related functional characteristics of glucose-sensitive (GS) versus glucose-insensitive (GIS) neurons. Firing rate changes were recorded by means of carbon fiber, multibarreled glass microelectrodes during 1) microelectrophoretic application of various chemicals, 2) gustatory and olfactory stimulation, and 3) a high fixed-ratio schedule (FR) bar press feeding task. 2. In 336 neurons examined, 91 (27%) were suppressed by electrophoretically administered glucose, and so they were designated as GS cells. The 245 neurons (73%) in which the firing rates did not change during glucose applications were pronounced GIS. The 179 GS and GIS cells tested exhibited different responses to the catecholamines (CAs), noradrenaline (NA) and dopamine (DA), both of which are intimately involved in the control of feeding. More GS neurons responded to NA than did GIS cells; the predominant effect of both CAs on GS neurons was inhibition. 3. The taste responsiveness of 111 LHA neurons was examined. Fifty-seven cells (52%) showed responses to gustatory stimulation. Of 50 GS neurons tested, 33 (66%) exhibited firing rate changes to tastes. On the contrary, only 24 (39%) of the 61 GIS neurons examined responded to gustatory stimuli. Activity changes of GS neurons commonly occurred to two or more tastants, in distinction to the relative gustatory specificity shown by GIS cells. 4. Two hundred fifty-six (84%) of the 303 neurons tested responded during one or more phases of the bar press feeding task. Most activity changes occurred during the bar press (BP) and reward (RW) periods, however numerous phasic responses to cue light (CL) and cue tone (CT) were also observed. A higher proportion of the GS neurons showed task-related activity changes than did the GIS cells (77, 95% and 179, 81%, respectively). GS neurons responded more during the BP phase and to the food reward; GIS cells were more responsive during the CL that enabled acquisition and the CT that signaled reward. Thus GS neurons were responsive during the acquisition and consumption of reward, whereas GIS cells responded to external cues signaling both of these events. The gustatory neurons displayed specific task-related activity changes only in the CL (GIS cells) and BP phases (GS neurons), that is, in phases most intimately involved in sensory-motor integration. 5. Two-thirds of the 30 GS neurons tested were responsive to both gustatory and olfactory stimulation as opposed to only one-third of GIS cells.(ABSTRACT TRUNCATED AT 400 WORDS)     

HLA and hypocretin studies in Korean patients with narcolepsy

STUDY OBJECTIVES: Very few studies have evaluated narcolepsy in Asian countries, outside of Japan. Our goal was to study narcolepsy at the genetic, clinical and pathophysiological level in Korea. DESIGN: Prospective study of consecutive patients and age matched controls. Clinical data ascertained from the Stanford Sleep Inventory, Polysomnography and MSLT data, as well as clinical notes. High resolution DRB1 and DQB1 typing in all subjects and studies of CSF hypocretin-1 was also evaluated in a subset of patients. PARTICIPANTS AND SETTING: 20 patients diagnosed at St. Vincent and Korea University Hospitals (Seoul, Korea). 21 Korean control subjects. INTERVENTIONS: N/A. MEASUREMENTS AND RESULTS: For narcoleptic subjects, mean age was 28.2 years old and 45% were female. Mean BMI was 23.9+/-3.4 kg/m2, a significantly higher value than that expected in an age- and sex-matched sample (p<0.01). All patients had sleepiness and cataplexy while the prevalence of other symptoms ranged from 60-75%. All but 2 subjects were HLA-DR15 (DR2), DQB1*0602 positive (90%). This high DQB1*0602 percentage compared with 24% DQB1*0602 positivity in 21 control Koreans. Protective effects were observed for the DQB1*0601 and DRB1*0406 alleles, Hypocretin (orexin) CSF studies were also performed in 6 cataplectic subjects, all of which had undetectable CSF hypocretin levels. Two of these subjects had started narcolepsy less than 1 year before analysis yet had undetectable hypocretin levels. CONCLUSION: These results illustrate the similarity of narcolepsy-cataplexy in Korea in comparisons with other more studied populations. We also identified a new potential HLA protective subtype, HLA-DRB1*0406.