Oral Dis. 2005 Nov;11(6):338-49.
Number V Oral lichen planus: clinical features and management.
Dermatology Research Associates, Cincinnati, OH 45230, USA. firstname.lastname@example.org
Oral lichen planus (OLP) is a relatively common chronic inflammatory disorder affecting stratified squamous epithelia. Whereas in the majority of instances, cutaneous lesions of lichen planus (LP) are self-limiting and cause itching, oral lesions in OLP are chronic, rarely undergo spontaneous remission, are potentially premalignant and are often a source of morbidity. Current data suggest that OLP is a T cell-mediated autoimmune disease in which auto-cytotoxic CD8+ T cells trigger apoptosis of oral epithelial cells. The characteristic clinical aspects of OLP may be sufficient to make a correct diagnosis if there are classic skin lesions present. An oral biopsy with histopathologic study is recommended to confirm the clinical diagnosis and mainly to exclude dysplasia and malignancy. The most commonly employed and useful agents for the treatment of lichen planus (LP) are topical corticosteroids but other newer agents are available.
About two years ago, Dr. Philippe Gros, a McGill University professor in the Department of Biochemistry and a Principal Investigator in thd McGill Life Sciences Complex, described a mouse mutant that was immunodeficient and hypersensitive to the Bacille Calmette-Gu-rin (BCG) vaccine and to tuberculosis (TB). In this model, Gros’s team had found that the immunodeficiency was caused by a mutation in a regulatory protein of the immune system named IRF8.
A year later, a physician in Newcastle who had heard about Gros’s work, contacted him about a three-month-old patient who was gravely ill and dying. The infant was suffering from an infection following a perinatal BCG vaccination. She had been treated aggressively with antibiotics but relapsed with additional infections. In addition, she showed a complete absence of circulating monocytes and dendritic cells in her blood – two critically important types of immune cells. She was admitted into an ICU and it seemed nothing could be done to save her.
The clinical aspects of the infant’s immuno-deficiency were so strikingly similar to those of Gros’s earlier mouse model findings, that his research team investigated the human IRF8 gene for the presence of mutations in this infant. Dr. Gros group also examined IRF8 in a number of additional clinical cases of disseminated BCG infection following vaccination.
What they found were two distinct disease-causing mutations – one that causes the severe reaction seen in the infant (autosomal recessive) and requires stem cell transplantation, and the other that causes a milder form of disease (autosomal dominant).
These findings, recently published in the New England Journal of Medicine, point to a critical role for IRF8 in the development and function of monocytes and dendritic cells and in protecting against mycobacterial infections like TB in humans.
According to Gros, the best part of this story is that the infant received the much needed stem cell transplant that ultimately cured her. Based on the team’s research, her doctors were going to transplant her with one of the parents’ cells as they were found to be a perfect match. However, when the team learned that the father was carrying one copy of the dysfunctional gene, and knowing that such a situation is deleterious in mice, the physicians opted instead to graft her with an unrelated donor.
“I think this is a great example of the ‘discovery pipeline’ we have tried to set up at the Complex Traits Group lab,” said Gros. “This began as basic research. It evolved from genetic discoveries in mouse models through to validation in humans and knowledge translation to a positive clinical outcome.”
“This is archetypal translational research,” said Dr. Richard I. Levin, Vice-Principal of Health Affairs and Dean of Medicine at McGill. “When results from lab work conducted in the Life Sciences Complex can be shared across the ocean in context and in time to save a child’s life, we know our objectives are being fulfilled.”
Source: McGill University...Read On
Clinical Review: Asbestos-related Disease.
Intern Med J. 2011 Feb 10;
Authors: Jamrozik E, de Klerk N, Musk A
Inhalation of airborne asbestos fibres causes several diseases. These include asbestosis, lung cancer, malignant mesothelioma as well as pleural effusion, discrete (plaques) or diffuse benign pleural fibrosis and rolled atelectasis. The lag time between exposure and the development of disease may be many decades, thus the health risks of asbestos continue to be relevant despite bans on the use of asbestos and improvements in safety regulations for those who are still exposed. Asbestos was mined and used extensively in Australia for over 100 years and Australia is now experiencing part of a worldwide epidemic of asbestos related disease. This review provides insight into the history and epidemiology of asbestos related disease in Australia and discusses relevant clinical aspects in their diagnosis and management. The past and current medico legal aspects of asbestos as well as currently evolving areas of research and future projections are summarised.
PMID: 21309996 [PubMed - as supplied by publisher]www.ncbi.nlm.nih.gov
Malignant Mesothelioma: A Clinical Study of 238 Cases.
Ind Health. 2010 Dec 16;
Authors: Haber SE, Haber JM
Malignant mesothelioma is a diffuse tumor arising in the pleura, peritoneum, or other serosal surface and is closely associated with asbestos exposure. An estimated 2,500 to 3,000 cases are diagnosed each year in the United States. Although there are individual case reports and small series detailing the clinical aspects of mesothelioma, few studies examine a large series of patients with malignant mesothelioma from the clinical perspective. This study reports on the findings of 238 cases of malignant mesothelioma from a private consultative medical practice. Most cases had a history of occupational asbestos exposure. The mean latency was 48.5 yr, with women having a longer latency than men. The mean age at diagnosis was 70. Survival overall was poor (mean 8.8 months), but treatment was beneficial (mean 11.3 versus 6.4 months). Epithelioid histology conferred a survival advantage over sarcomatoid and responded better to treatment. Our data support an inverse relationship between asbestos dose and latency.
PMID: 21173534 [PubMed - as supplied by publisher]www.ncbi.nlm.nih.gov
You can link to this article on your web site using following code:
We're looking for comments that are interesting, substantial or highly amusing. If your comments are excessively self-promotional (use your real name, no keywords please), obnoxious, or even worse, boring, you will be banned from commenting. Your comment must be related to the post. Please do not comment on how great or wonderful the post is. All comments are moderated and, if approved, will display in less than 24 hours.
blog comments powered by Disqus