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.     

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.     

Association of Polyphenols from Oranges and Apples with Specific Intestinal Microorganisms in Systemic Lupus Erythematosus Patients

Our group has recently shown the existence of a gut microbial dysbiosis in systemic lupus erythematosus (SLE), supporting previous evidence involving intestinal bacteria in the initiation and amplification of autoimmune diseases. While several studies have addressed the use of dietary fibres to modify intestinal microbiota, information about other correlated components, such as polyphenols, is scarce. The aim of this work was to identify dietary components able to influence this altered microbiota in 20 SLE women and 20 age-matched controls. Food intake was recorded by means of a food frequency questionnaire. The intake of fibres was calculated from Marlett tables, and Phenol-Explorer was used for polyphenol consumption. Results showed positive associations between flavone intake and Blautia, flavanones and Lactobacillus, and dihydrochalcones and Bifidobacterium in the SLE group. Regarding the controls, dihydroflavonols were directly associated with Faecalibacterium, whereas flavonol intake was inversely associated with Bifidobacterium. From the food sources of these polyphenols related to microbiota, orange intake was directly associated with Lactobacillus and apple with Bifidobacterium in SLE, whilst red wine was the best contributor to Faecalibacterium variation. The association between common foods and particular microbial genera, reported to be decreased in SLE, could be of great importance for these patients.     

Use of icodextrin for diurnal exchange in patients undergoing automatic peritoneal dialysis. Comparison with glucose solutions

Icodextrin (IC) is an osmotic agent that produces sustained ultrafiltration (UF) during long dwell time periods in peritoneal dialysis patients. The aim of this study was to evaluate the effects of 7.5% IC for the diurnal exchange in automated peritoneal dialysis (APD) patients and to compare them with that of 2.27% glucose solutions. Seventeen patients treated on APD during 13.9 +/- 12.7 months were included. The study was divided into three eight weeks phases. During the baseline period patients used 2.27% glucose for the daytime, second, IC 7.5% was prescribed for the day-exchange, and finally 2.27% glucose solution was used for the last eight weeks. Daytime UF increased in all patients during IC use (-53 +/- 22 to 270 +/- 304 ml/day, p < 0.01). Patients with higher peritoneal permeability capacity obtained more benefits. Daytime urea KT/V and weekly creatinine clearance (WCC) augmented significantly during IC use, but the increase of weekly urea KT/V and WCC was not significant (2.18 +/- 0.45 to 2.26 +/- 0.41 and 62.7 +/- 18 to 66.6 +/- 15 l/week/1.73 m2; respectively). On IC, nightly glucose load significantly decreased (289 +/- 82 to 266 +/- 94 g, p < 0.05), returning to previous value after withdrawal. Plasma osmolality did not change, although plasma sodium levels decreased during IC use (140 +/- 3 to 136 +/- 2, p < 0.001). Serum amylase levels significantly declined during IC use (279 +/- 151 to 29 +/- 9 U/l), returning to previous values after transfer to glucose. Peritoneal function transport parameters and peritoneal protein losses did not change. IC metabolite plasma levels increased during the use of this solution, returning to previous values after withdrawal. In conclusion, IC dialysate is an excellent alternative to glucose dialysate for the day-exchange in APD patients. Daytime UF increased in all patients, but those with higher peritoneal permeability capacity obtained more benefits. The decrease of the glucose peritoneal load overnight and the reduction for more than 50% of exposure time of the peritoneal membrane to glucose solutions, probably make IC solution a more biocompatible fluid.     

First report and molecular characterization of hepatitis E virus infection in renal transplant recipients in Brazil

Hepatitis E virus (HEV) causes acute and chronic hepatitis in organ transplant recipients. Serological evidence for HEV infection has been discovered in various population groups in Brazil, and a single acute case has been confirmed. To date, however, no cases of HEV infection in immunocompromised patients have been reported in Brazil. This study aimed to identify and characterize hepatitis E cases in renal transplant recipients in Brazil. A retrospective study was performed on 96 serum samples from renal transplant recipients with unexplained liver enzymes elevation. Three confirmed cases of HEV infection were identified that lacked seroconversion to HEV IgG antibodies. The prevalence of HEV in these patients was 3.1%. Using a sequence analysis of a 304‐nucleotide fragment within ORF2, the strains were classified as genotype 3 with a low percent identity to previously characterized strains. This is the first report of hepatitis E infection in renal transplant recipients in Brazil, and the data indicate that a novel genotype 3 subvariant may be present and that further investigation is necessary to characterize the circulating HEV strains. In this setting, HEV infection should be considered as a potential cause of abnormal liver tests of unknown origin. J. Med. Virol. 85:615–619, 2013. © 2013 Wiley Periodicals, Inc.     

Blood flow velocity in monocular retinoblastoma assessed by color doppler

OBJECTIVE:

To analyze the flow of retrobulbar vessels in retinoblastoma by color Doppler imaging.

METHODS:

A prospective study of monocular retinoblastoma treated by enucleation between 2010 and 2014. The examination comprised fundoscopy, magnetic resonance imaging, ultrasonography and color Doppler imaging. The peak blood velocities in the central retinal artery and central retinal vein of tumor-containing eyes (tuCRAv and tuCRVv, respectively) were assessed. The velocities were compared with those for normal eyes (nlCRAv and nlCRVv) and correlated with clinical and pathological findings. Tumor dimensions in the pathological sections were compared with those in magnetic resonance imaging and ultrasonography and were correlated with tuCRAv and tuCRVv. In tumor-containing eyes, the resistivity index in the central retinal artery and the pulse index in the central retinal vein were studied in relation to all variables.

RESULTS:

Eighteen patients were included. Comparisons between tuCRAv and nlCRAv and between tuCRVv and nlCRVv revealed higher velocities in tumor-containing eyes (p<0.001 for both), with a greater effect in the central retinal artery than in the central retinal vein (p=0.024). Magnetic resonance imaging and ultrasonography measurements were as reliable as pathology assessments (p=0.675 and p=0.375, respectively). A positive relationship was found between tuCRAv and the tumor volume (p=0.027). The pulse index in the central retinal vein was lower in male patients (p=0.017) and in eyes with optic nerve invasion (p=0.0088).

CONCLUSIONS:

TuCRAv and tuCRVv are higher in tumor-containing eyes than in normal eyes. Magnetic resonance imaging and ultrasonography measurements are reliable. The tumor volume is correlated with a higher tuCRAv and a reduced pulse in the central retinal vein is correlated with male sex and optic nerve invasion.     

Myocardial Revascularization in Dyalitic Patients: In-Hospital Period Evaluation

Background

Coronary artery bypass grafting currently is the best treatment for dialytic patients with multivessel coronary disease, but hospital morbidity and mortality related to procedure is still high.

Objective

Evaluate results and in-hospital outcomes of coronary artery bypass grafting in dialytic patients.

Methods

Retrospective unicentric study including 50 consecutive and not selected dialytic patients, who underwent coronary artery bypass grafting in a tertiary university hospital from 2007 to 2012.

Results

High prevalence of cardiovascular risk factors was observed (100% hypertensive, 68% diabetic and 40% dyslipidemic). There was no intra-operative death and 60% of the procedures were performed off-pump. There were seven (14%) in-hospital deaths. Postoperative infection, previous heart failure, cardiopulmonary bypass, abnormal ventricular function and surgical re-exploration were associated with increased mortality.

Conclusion

Coronary artery bypass grafting is feasible to dialytic patients although high in-hospital morbidity and mortality. It is necessary better understanding about metabolic aspects to plan adequate interventions.     

Microscopic evaluation of induced tooth movement in traumatized teeth: an experimental study in rats

Abstract – The clinical management of orthodontic patients with dental trauma before or during the treatment is mainly founded on clinical experience, expert opinions, and individual case reports. It is proposed in the literature that teeth sustaining mild trauma with minor damage to the periodontium (e.g. subluxation) should be followed for a period of time before being subjected to orthodontic forces. A minimum period of 3 months has been proposed. In this study, we used an animal model to investigate whether shorter observation periods could be established in case of mild trauma. The periradicular region of rat molars was examined microscopically to determine the biological events of tooth movement started 15 and 30 days after intentional subluxation using an experimental method to induce dentoalveolar trauma. Thirty adult male Wistar rats were assigned to 6 groups (n = 5): Group 1 (control – no trauma/orthodontic movement); Group 2: the animals received an orthodontic device and were sacrificed after 7 days; Groups 3 and 4: dentoalveolar trauma (subluxation) was experimentally induced by the application of an axial force of 900 cN on the occlusal surface of the maxillary right first molar, and the animals were sacrificed after 22 and 37 days, respectively; and Groups 5 and 6: 15 and 30 days, respectively, after force application, an orthodontic device was installed and the rats were sacrificed 7 days later. In G5 and G6, the periodontal ligament and pulp tissue were rich in cellular elements and blood vessels, the alveolar bone was preserved, and the root surface presented only very small areas of surface resorption (cementum), maintaining the characteristics of normality. In conclusion, the microscopic alterations in the gingival and periodontal tissues in response to an experimentally induced mild dentoalveolar trauma simulating subluxation were not sufficient to contraindicate starting the orthodontic movement 15 and 30 days after trauma.