RESUMEN
Birt-Hogg-Dube syndrome is a rare autosomal dominant disorder characterized by pulmonary cysts, renal tumors, and dermal lesions. This syndrome results from a mutation in the gene folliculin, located on chromosome 17p11.2. Herein, a case is described in which the presence of bilateral renal oncocytomas led to the diagnosis of Birt-Hogg-Dube syndrome via an interdisciplinary effort by radiology, pathology, and primary care medicine. No radiographic features alone are sufficient to confirm the diagnosis of Birt-Hogg-Dube. A high index of suspicion must be maintained by both the pathologist and radiologist in the appropriate clinical setting.
RESUMEN
A 53-year-old male with no significant past medical history presented with an acute traumatic fracture of his thumb. Preoperative chest radiograph before K-wire fixation demonstrated an incidental 9 cm opacity of the left lung. Chest computed tomography revealed a 6.3 cm aggressive appearing pleural-based mass with erosion and destruction of the underlying rib. The patient underwent percutaneous biopsy with interventional radiology, and pathology revealed a small round blue cell tumor with positive CD99 staining and a FUS-ERG chromosomal translocation. The patient was diagnosed with Askin tumor, a peripheral primitive neuroectodermal tumor of the thoracopulmonary region belonging to the Ewing sarcoma tumor family. Computed tomography and magnetic resonance imaging of Askin tumors may show features such as a heterogeneous soft tissue mass, pleural effusion, rib destruction, hemorrhage, necrosis, and cystic degeneration. Askin tumors typically exhibit the EWS-FLI1 fusion mutation, although FUS-ERG chromosomal translocation has been described. Both rarity and variability of Askin tumors present a diagnostic challenge for clinicians. Collaborative effort amongst radiologists and pathologists is essential for diagnosis.
RESUMEN
INTRODUCTION: Clostridial toxic shock is a rare syndrome traditionally described in case studies and reviews as a uniformly fatal condition associated with post-partum and post-abortion. Clostridium sordellii was first mentioned in scientific literature as a human pathogen in 1922 when Argentinian microbiologist Alfredo Sordellii identified the organism in gangrenous wounds. Over the past century, there has been infrequent discussion and little awareness surrounding this serious condition. Information about patients who survived C. sordellii toxic shock is essentially nonexistent. PRESENTATION OF CASE: A 31-year old post-partum female who underwent caesarian section 17 days prior, presented with abdominal pain and septic features. She underwent six operations/procedures over the next 17 days and was hospitalized for 25 days before discharge to her home with a successful outcome. DISCUSSION: This case provides insight on the process of identifying and managing complications associated with clostridial toxic shock syndrome. Out of now, 16 reported cases of C sordellii gynecologic infections in the world literature, our patient is the second patient to survive infection by this organism. More research is needed to provide clinicians with evidence-based approaches for managing these unique cases. CONCLUSION: We present a case report of a rare toxic shock syndrome in a post-partum female caused by Clostridium sordellii. Her survival is notable among 16 patients with whom this infection now has a documented 87.5% mortality rate. Aggressive surgical measures and optimized intensive care management may have contributed to this patient's survival.
RESUMEN
Concern over the adequacy of biosafety training and incident-reporting practices within biological laboratories in the United States has risen in recent years due to the increase in research on infectious diseases and the concomitant rise in the number of biocontainment laboratories. Reports of laboratory-acquired infections and delays in reporting such incidents have also contributed to the concern. Consequently, biosafety training and incident-reporting practices are being given considerable attention by both the executive branch and Congress. We conducted a 51-question survey of biosafety professionals in June 2008 to capture information on methods used to train new laboratory workers within biosafety level 2 (BSL-2) laboratories, animal biosafety level 2 (ABSL-2) laboratories, biosafety level 3 (BSL-3) laboratories, and animal biosafety level 3 (ABSL-3) laboratories. The survey results suggest nearly all senior scientists, faculty, staff, and students working in these biocontainment laboratories are required to have biosafety training, and three-quarters of respondents indicated a biosafety or environmental health and safety professional provides explicit instructions on reporting incidents to each new lab worker. Only half of the respondents with BSL-2/ABSL-2 laboratories at their institution and 59% of respondents from institutions with BSL-3/ABSL-3 laboratories indicated custodial or maintenance workers are required to receive biosafety training at the BSL-2/ABSL-2 and BSL-3/ABSL-3 levels, respectively. Opportunities for targeted improvement such as providing training to non-traditional laboratory workers (e.g., custodians, maintenance workers) and posting laboratory incident-reporting protocols on institutional environmental health and safety websites may exist. Variations in biosafety training requirements, incident-reporting practices, and attitudes towards laboratory safety revealed through this survey of biosafety professionals also support the development of core competencies in biosafety practice that could lead to more uniform practices and robust safety cultures.
RESUMEN
The anthrax attack in 2001 created new challenges to health educators working on the response effort in New Jersey. Never before had there been a need for educating a group of people who had been exposed to a biological weapon. Coming on the heels of the catastrophic World Trade Center collapse on September 11, 2001, the New Jersey Department of Health and Senior Services was entrenched in the response to, and management of, the anthrax attack that placed a heavy emphasis on educating the postal workers of the United States Postal Service Trenton Processing and Distribution Center. This article provides an account of the preparation and delivery of educational materials and activities in the midst of a biological emergency, emphasizes the role health educators play in responding to bioterrorism events, and encourages health educators to become involved in bioterrorism preparedness efforts.
