Large gangliocytic paraganglioma of the duodenum: A rare entity

Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.     

Ruvalcaba-Myhre-Smith Syndrome

In 1980 a syndrome was first described in two adult males, consisting of macrocephaly, pigmented macules on the glans and shaft of the penis, and hamartomatous intestinal polyps. Since then, 10 additional cases have been identified. Herein, we present two new cases and review the cutaneous manifestations as well as additional features in patients with the Ruvalcaba-Myhre-Smith syndrome.     

Development of DNA vaccines against hemolytic-uremic syndrome in a murine model

Shiga toxin type 2 (Stx2) produced by Escherichia coli O:157H7 can cause hemolytic-uremic syndrome in children, a disease for which there is neither a vaccine nor an effective treatment. This toxin consists of an enzymatically active A subunit and a pentameric B subunit responsible for the toxin binding to host cells, and also found to be immunogenic in rabbits. In this study we developed eukaryotic plasmids expressing the B subunit gene of Stx2 (pStx2B) and the B subunit plus the gene coding for the A subunit with an active-site deletion (pStx2 Delta A). Transfection of eukaryotic cells with these plasmids produced proteins of the expected molecular weight which reacted with specific monoclonal antibodies. Newborn and adult BALB/c mice immunized with two intramuscular injections of each plasmid, either alone or together with the same vector expressing the granulocyte and monocyte colony-stimulating factor (pGM-CSF), elicited a specific Th1-biased humoral response. The effect of pGM-CSF as an adjuvant plasmid was particularly notable in newborn mice and in pStx2B-vaccinated adult mice. Stx2-neutralizing activity, evaluated in vitro on VERO cell monolayers, correlated with in vivo protection. This is the first report using plasmids to induce a neutralizing humoral immune response against the Stx2.     

Histological study of the proximal and distal segments of the embryonic outflow tract and great arteries

The normal development of the ventricular outlets and proximal region of the great arteries is a controversial subject. It is known that the conus, truncus arteriosus (truncus), and aortic sac participate; however, there are some doubts as to the actual prospective fate of the truncus. Some authors propose that it gives origin to the proximal region of the great arteries and that the myocardial cells of its wall become smooth muscle. Nevertheless, others think that the truncus only forms the arterial valve apparatus and that therefore the myocardial cells transform into fibroblasts. As a first approach to beginning to elucidate which process occurs, the aim of this article was to study the histological changes in the wall of these components of the developing heart in chick embryos whose hearts had been labeled at the truncoconal boundary at stage 22HH, tracing the changes up to stage 36HH. Also, the histological constitution of the wall of the pulmonary arterial trunk and its valve apparatus were studied in the posthatching and adult hearts of chickens and rats. The conus and truncus walls were always encircled by a myocardial sleeve from the outset of their development. Between stages 26HH to 28HH, the truncal myocardial cells adjacent to the mesenchymal tissue of the ridges began to lose cell-to-cell contacts and invaded the extracellular matrix. At stage 24HH, the aortic sac began to project into the pericardial cavity and became divided into two channels by the aortic-pulmonary septum at stage 26HH. The wall of the aortic sac is mostly constituted by a compact mesenchymal tissue. Initially, it does not have smooth muscle but this starts to appear at stage 30HH. The insertion ring of the valves, a broad structure, was formed by mesenchymal tissue. Both structures were always covered by a myocardial sleeve. The leaflets developed from the truncal ridges, the segment immediately proximal to the aortic sac. Our results indicate that the proximal region of the pulmonary and aortic arteries do not originate from the truncus arteriosus; rather, we found that they take origin from the aortic sac. Thus, our findings agree with the proposal that the myocardial cells of the external sleeve of the truncus become fibroblastic and suggest that the insertion ring of the arterial valves has a dual origin: fibroblasts produced by truncal myocardial transdiferentiation and the mesenchymal tissue of the proximal region of the truncal ridges, while the leaflets have their origin from the truncal ridges. We discuss the fact that, because the truncus arteriosus does not give origin to the trunks of the aortic and pulmonary arteries, it may be necessary to modify terminology. Based on our results, together with the new findings obtained by in vivo labeling, immunostaining, a chimeric approach, and ultrastructural studies, we propose a developmental model that correlates the fate of the conus, truncus, and aortic sac with the normal morphogenesis of the ventricular outlet tracts and the trunks of the great arteries. (c) 2005 Wiley-Liss, Inc.     

The influence of the causes and contexts of medical errors on emergency medicine residents' responses to their errors: an exploration.

PURPOSE: To determine emergency medicine residents' emotional and behavioral responses to their medical errors and examine associations between residents' responses to medical error and perceptions of their training. METHOD: In 2003, 55 residents at two U.S. residency programs were asked to complete questionnaires about their errors and responses to their errors in three domains: emotional response, learning behavior, and disclosure. The questions were a mixture of free text, yes/no responses, and some were rated using a five-point Likert scale. Based on a conceptual framework, the authors constructed scales to describe the various domains and associations between the residents' responses to medical error and perceptions of their training were examined using Somers' D. RESULTS: A total of 43 residents returned questionnaires (80%); 40 of these residents described errors. Thirty-three (83%) residents discussed the error with someone; 27 (71%) with the attending and 10 (28%) with the patient/family. Negative emotions were common: 27 (68%) felt remorse, 21 (53%) guilt, 23 (58%) inadequacy, and 22 (55%) frustration. Residents' negative emotional responses were associated with their personal characteristics [26% (95% CI, 5-47%) association with lack of experience] and residents' perceptions of their training environment: 15% association with job overload (95% CI, -8-38%) and 23% association with lack of institutional support (95% CI, 5-41%). While 32 (81%) residents increased attention to detail, only 2 (5%) increased their use of evidence-based medicine. CONCLUSIONS: Errors committed by emergency medicine residents often resulted in negative emotions, limited constructive system-based improvements, and inadequate disclosure. Negative perceptions of the training environment are associated with negative emotional responses.     

Disseminated Klebsiella rhinoscleromatis infection

A 35-year-old obese black American woman presented with nausea, vomiting, diarrhea, fever, cough, and chest pain of 2 weeks duration. She was pancytopenic and acidotic, with respiratory failure and hypotension. A diagnosis of septic shock was made, and the patient died 48 hours after admission. Blood cultures were positive for organisms that were reported to be Klebsiella rhinoscleromatis. At autopsy she had massive hepatic necrosis with numerous Mikulicz's cells. The lungs, spleen, and bone marrow were also involved. To our knowledge, this is the first report of a case of systemic infection with K rhinoscleromatis.     

Myofibroblastic differentiation in simple peritoneal sclerosis

OBJECTIVE: To analyze the presence of myofibroblasts in a series of peritoneal dialysis (PD) patients with simple sclerosis and non-PD, uremic patients. Since there is a close correlation between active fibrosis and myofibroblastic differentiation we wanted to test if myofibroblasts are present in uremic, non-PD peritoneal samples. To determine if there are correlations between myofibroblastic presence and other functional and morphologic peritoneal parameters. METHODS: Biopsies were collected from three patient groups: 1) Normal control samples (n = 15) of parietal and visceral peritoneum 2) non-PD uremic patients (n = 16); and 3) uremic patients on PD (n = 32). Peritoneal morphologic and functional parameters and immunohistochemical expression of alfa-smooth muscle actin was analyzed in each case. Vascular endothelial growth factor (VEGF), bcl-2 anti-apoptotic protein, and progesterone receptor was evaluated in a subset of cases. RESULTS: Myofibroblasts were present in 56.3% of the patients with PD-related simple sclerosis. In most cases they were distributed in the upper submesothelial area. None of the biopsies from normal controls and uremic, non-PD patients showed myofibroblasts. Within the group of PD patients, myofibroblasts showed no correlation with time on dialysis, urea/creatinine MTAC, episodes of peritonitis, submesothelial thickening, hyalinizing vasculopathy or mesothelial status. In a subset of PD patients VEGF expression was observed in submesothelial fibroblastic cells. No expression of progesterone receptor or bcl-2 was observed. CONCLUSIONS: Myofibroblasts are a reliable and simple indicator of fibrosis since they appear in early stages of PD treatment and in patients with minor morphologic anomalies. They are not exclusive of patients with sclerosing peritonitis, ultrafiltration loss or long standing treatment. Their absence in non-PD, uremic patients suggest that uremia-related fibrosis takes place without a significant participation of myofibroblasts.     

Bone-specific antibodies in sera from patients with celiac disease: characterization and implications in osteoporosis

Osteopenia and osteoporosis are well-known complications detected in celiac disease patients with still obscure pathogenesis. In the present study we investigated the presence of circulating anti-bone autoantibodies in patients with celiac disease and explored their role in the associated bone disease. We evaluated serum samples from 33 patients at the time of diagnosis and from 20 of them after treatment. Sera from patients with inflammatory bowel disease (n = 9), nonceliac osteoporotic (n = 18), and healthy individuals (n = 10) were used as controls. The presence of IgA specific anti-bone antibodies was first investigated using indirect immunofluorescence on cryosections of fetal rat tibia (20-day pregnancy). Furthermore, samples were homogenized and total tissue extracts were subjected to Western blot analysis to confirm immunoreactivity. At diagnosis, sera from 51.5% (17/33) of celiac patients had antibodies that recognized antigenic structures in chondrocytes and the extracellular matrix along mature cartilage, bone interface, and perichondrium of fetal rat bone. Among controls, only two osteoporotic patients showed very low titles of anti-bone autoantibodies. The immunostaining was localized in areas where an active mineralization process occurred and was similar to the distribution of the native bone tissue transglutaminase. The frequency of patients with positive baseline titers of anti-bone antibodies diminished significantly after treatment (P = 0.048). Western blot assays confirmed the presence of autoantibodies in sera from patients with a positive immunofluorescence staining. Autoantibodies recognized a major protein band on tissue extracts with a molecular weight of 77–80 kDa, which could be displaced when sera were preadsorbed with human recombinant tissue transglutaminase. We provide original evidence that patients with celiac disease have IgA-type circulating autoantibodies against intra- and extracellular structures of fetal rat tibia. Our findings suggest that these antibodies recognize bone tissue transglutaminase as the autoantigen, and based on the localization of the immunoreactivity we speculate that they might have an active role in the pathophysiology of celiac disease-associated bone complications.     

Association of a Mediterranean Diet and Fruit and Vegetable Consumption with Subjective Well-Being among Adults with Overweight and Obesity

Recent evidence suggests that among behavioral-lifestyle factors, adherence to a healthy dietary pattern such as the Mediterranean Diet (MedDiet) is linked not only to better psychological health and mental positive status but also to increased subjective well-being (SWB). Nevertheless, this association has been unexplored among individuals with excessive weight. This study explored whether adherence to the MedDiet and the intake of healthy foods such as fruits and vegetables (FV) are associated with increased happiness and life satisfaction among Spanish adults with overweight or obesity when weight, body image, and body satisfaction are also considered. A convenience sample of adult individuals with excessive weight completed self-reports on the study variables, and weight and BMI were measured by bioimpedance. No evidence of a relationship with SWB indicators was obtained for MedDiet global indicators, probably due to the low adherence to a healthy diet by these individuals. In contrast, FV intake, as a powerful indicator of healthy eating, was associated with life satisfaction when BMI and body image dimensions were considered, among which body satisfaction had a key role. Moreover, life satisfaction fully mediated the relationship between FV consumption and happiness. Our findings are expected to make a relevant contribution to knowledge on the positive correlates or protective factors for overall well-being in obesity, including dietary habits and body appreciation. Our results may inform obesity management actions focused on inclusive, positive aesthetic models and promoting a healthy lifestyle for happiness in obesity.