Successful Outcome in Patients with Fanconi Anemia Undergoing T Cell-Replete Mismatched Related Donor Hematopoietic Cell Transplantation Using Reduced-Dose Cyclophosphamide Post-Transplantation

Allogeneic hematopoietic cell transplantation (HCT) has been shown to restore normal hematopoiesis in patients with Fanconi anemia (FA), with excellent results in matched related donor HCT. Outcomes of alternative donor HCT are less favorable, however. In patients without FA, several reports have documented stable engraftment and/or a low risk of graft-versus-host disease (GVHD) using unmanipulated HLA-mismatched related donors and post-HCT cyclophosphamide (PT-CY) for GVHD prophylaxis. Data on the use of this approach in patients with FA are scarce, and thus we launched a study of HLA-mismatched related donor HCT in these patient. Here we report our findings in 19 patients. The conditioning was fludarabine 30 mg/m²/day for 5 days, antithymocyte globulin 5 mg/kg/day for 4 days, and total body irradiation (total dose, 200 cGy). GVHD prophylaxis was cyclosporine and mycophenolate and reduced doses of PT-CY, 25 mg/kg, on days +3 and +5. All patients exhibited absolute neutrophil count recovery. Grade III-IV acute GVHD occurred in 3 patients, and chronic GVHD occurred in 1 patient. At a mean follow-up of 38.3 ± 5.8 months, the 5-year probability of overall survival for our patients was 89.2% ± 7.2%. The regimen was well tolerated; hemorrhagic cystitis occurred in 7 patients, and severe mucositis occurred in 5 patients. There were 2 deaths; the primary cause of death was severe GVHD in 1 patient and leukemia recurrence in the other. We conclude that in patients with FA lacking a matched related donor, the use of mismatched related HCT with low-dose PT-CY is a viable option; it is well tolerated, with a high rate of engraftment and an acceptable incidence of GVHD.     

Coronary sinus dilatation is associated with left ventricular systolic dysfunction and poor functional status ın subjects with chronic heart failure

Background and aims: Reliable echocardiographic markers additional to plasma biomarkers that would establish prognosis of chronic heart failure and guide therapeutic approach would be beneficial. In our hypothesis, echocardiographic assesment of coronary sinus anatomic alteration, which has been ignored, may be part of remodeling process in heart failure. We also aimed to evaluate relationship between coronary sinus anatomic alteration and left ventricular systolic dysfunction. We echocardiographically analysed 112 patients with heart failure and 61 normal subjects. Left/right ventricular volumes, left atrial area and mean coronary sinus were measured. Coronary sinus diameter was significantly higher in patients than in control group. Statisticallly positive correlation were present between coronary sinus measurements and left/right ventricular volumes (R = 0.5, P < 0.001; R = 0.4, P < 001, respectively), left atrial area (R = 0.6, P < 0.001), NYHA class (R = 0.3, P < 0.001), mitral regurgitation (R = 0.329, P < 0.001), tricuspid regurgitation (R = 0.215, P < 0.02) and left ventricular mass (R = 0.482, P < 0.001). Statistically negative correlation were present between coronary sinus measurements and left ventricle ejection fraction (R = ?0.4, P < 0.001). However, coronary sinus diameter was not correlated with body surface area and pulmonary artery pressure. Tricuspid and mitral regurgitation, left ventricular mass, ejection fraction and functional classs were included in multivariate analysis and only ejection fraction was independent predictor of coronary sinus diameter (P = 0.012). We demonstrated that, dilated coronary sinus is possibly a part of entire process of cardiac remodeling and echocardiographic assessment of dilated coronary sinus may provide useful additional information, predicting the severity of chronic heart failure and poor functional class.     

Study of cystic echinococcosis in slaughtered animals in Al Baha region, Saudi Arabia: Interaction between some biotic and abiotic factors

The variation in cystic echinococcosis (CE) prevalence and mean intensity was studied in relation to site, season and host age and sex. A total of 12,911 slaughtered animals, 140 camels, 2668 cattle, 6525 sheep and 3578 goats were inspected for hydatid cysts in Al Baha region, Saudi Arabia, in three study areas during four seasons from June 2008 to May 2009. The prevalence of infection was 32.85%, 8.28%, 12.61% and 6.56% in camels, cattle, sheep and goats respectively. The prevalence of the parasite varied significantly in relation to site, season and host age classes and sex in most host species. Spring showed the highest prevalence in camels, cattle and sheep. A significant association was found among host age classes and likelihood of infection in all examined hosts and the oldest age class was significantly more likely to be infected. The main effects in parasite intensity were host sex and age in most examined host species. A positive correlation was found between intensity of CE and host age class in all animal species examined. The most commonly infected organs were liver and lungs which constituted 48.75% and 32.83% respectively, of the total infected organs. There was a significant difference among host species in fertile cysts (P < 0.0001). The higher percentages of fertile cysts were in sheep (47.67%) and goats (23.99%) indicating that sheep and goats are the most important intermediate hosts for Echinococcus granulosus. Examined hydatid cysts of the liver had a higher fertility rate (38.79%) than those of the lungs (25.13%). Cysts size ranged from 1 to 8 cm in diameter. The mean cyst diameter was found in the lungs higher than that in the liver in all hosts. The range in the number of cysts was 1-33 in infected animals. The mean number of cysts was higher in lungs than that in liver in all examined animals. The viability rate of protoscoleces of liver fertile cysts (62.20%) was significantly higher than that of lung cysts (52.73%). In conclusion, these findings of infection, mean abundance and fertility rates of CE in slaughtered animals, prompt plans for further epidemiological studies and control programmes.     

Description and evaluation of an innovative course on learning and study skills for the first year medical students

The purpose of this study is to share educational structure and evaluation results of an innovative course on effective learning and study skills for the first year medical students. In Turkey, undergraduate medical education takes six years and each year nearly 5,000 high school graduates start medical schools. However, many students experience frustration and failure because of their lack in the learning and studying strategies. At the Ege University Faculty of Medicine, preclinical curriculum consists of the body function systems-based teaching blocks. Year one has three blocks. We implemented an effective learning and study skills course at the first and third blocks of the 2003-2004 curriculum. We evaluated the course by students' feedbacks derived from block questionnaires and students' homework performance analysis. At the first block questionnaire, out of 297, 163 students (54.8%) clearly stated that the course positively influenced their learning process. Structured analysis of the first block's students' homework showed that an average of 206 students (69.3%) can sufficiently describe their learning and studying approach, while 218 (73.4%) can identify the strengths and weaknesses of the curriculum. The average student scores were 74 +/- 10 and 68 +/- 11 out of 100 for first and third blocks, respectively. We interpreted these results as students enjoyed the course and learned the content. In conclusion, a course on effective learning and study skills is likely to assist first year medical students in improving their learning and adaptation to the school.     

An intelligent system for diagnosis of the heart valve diseases with wavelet packet neural networks

In this paper, an intelligent system is presented for interpretation of the Doppler signals of the heart valve diseases based on the pattern recognition. This paper especially deals with combination of the feature extraction and classification from measured Doppler signal waveforms at the heart valve using the Doppler Ultrasound. Because of this, a wavelet packet neural network model developed by us is used. The model consists of two layers: wavelet and multi-layer perceptron. The wavelet layer is used for adaptive feature extraction in the time-frequency domain and is composed of wavelet packet decomposition and wavelet packet entropy. The multi-layer perceptron used for classification is a feed-forward neural network. The performance of the developed system has been evaluated in 215 samples. The test results showed that this system was effective in detecting Doppler heart sounds. The correct classification rate was about 94% for abnormal and normal subjects.     

Brown recluse spider envenomation

Brown recluse spider bite is a common diagnosis in almost every state in America. In fact, cases have been reported in areas where the spider has never been seen. A review of medical literature reveals that most current concepts regarding brown recluse spider envenomation are based on supposition. In this article, we attempt to review critically our present understanding of brown recluse bites with a focus on the published evidence.     

Multivariate Statistical Model for 3D Image Segmentation with Application to Medical Images

In this article we describe a statistical model that was developed to segment brain magnetic resonance images. The statistical segmentation algorithm was applied after a pre-processing stage involving the use of a 3D anisotropic filter along with histogram equalization techniques. The segmentation algorithm makes use of prior knowledge and a probability-based multivariate model designed to semi-automate the process of segmentation. The algorithm was applied to images obtained from the Center for Morphometric Analysis at Massachusetts General Hospital as part of the Internet Brain Segmentation Repository (IBSR). The developed algorithm showed improved accuracy over the k-means, adaptive Maximum Apriori Probability (MAP), biased MAP, and other algorithms. Experimental results showing the segmentation and the results of comparisons with other algorithms are provided. Results are based on an overlap criterion against expertly segmented images from the IBSR. The algorithm produced average results of approximately 80% overlap with the expertly segmented images (compared with 85% for manual segmentation and 55% for other algorithms).     

Can Bridge to Recovery Help to Reveal the Secrets of the Failing Heart?

Despite several decades of research and clinical experience, the basic mechanisms of the failing heart remain largely a secret. While pharmacological therapy can induce limited reverse remodeling, left ventricular assist device (LVAD) therapy offers the opportunity to induce significant improvements to the structure and function of the heart, with major clinical implications. LVAD therapy also provides significant insight into which changes have an impact on function and which do not, and could therefore reveal some of the secrets of the failing heart. In addition, LVAD-induced mechanical unloading may unlock further myocardial properties hitherto unknown such as the proliferation of the stem cell compartment. It may also serve as an important platform for emerging therapies such as gene and cell therapies. In this review, we highlight the most recent novel discoveries related to LVAD therapy and bridge to recovery (BTR). Discovering the integrated network of events that underlies BTR could unravel the secrets of the failing heart.     

The prevalance of respiratory viruses among healthcare workers serving pilgrims in Makkah during the 2009 influenza A (H1N1) pandemic

Despite the high risk of acquiring respiratory infections, healthcare workers who treat pilgrims at Hajj have not been studied in previous research on respiratory diseases during Hajj. The objective of this study was to determine the prevalence of different respiratory viruses among healthcare workers who treated pilgrims during Hajj 2009, the year of the influenza A H1N1 pandemic. A cross-sectional study was performed just before and after Hajj (25-29 November, 2009). Nasal and throat swabs were tested for 18 respiratory virus types and subtypes. A total of 184 healthcare workers were examined. Most were men (85%) with an average age of 41 years. Before the Hajj, rates of seasonal influenza vaccination were higher (51%) than rates of pandemic influenza A H1N1 vaccination (22%). After the Hajj, participants reported high rates of maintaining hand hygiene (98%), cough etiquette (89%), and wearing a face mask (90%). Among all the viruses tested, only two were detected: rhinovirus was detected in 12.6% and Coronavirus 229E in 0.6%. Rhinovirus was detected in 21% of those who had respiratory symptoms during Hajj. Influenza A (including H1N1), influenza B. respiratory syncytial virus, other coronaviruses, parainfluenza viruses, human metapneumovirus, adenovirus, and human bocavirus were not detected. The finding of high rates of rhinovirus infection corresponds to their frequent occurrence in adults. None of the participants had influenza A H1N1 2009, possibly because it was also infrequent among the 2009 pilgrims.