Management of an unusual extreme extension contracture of the wrist: role of a custom-designed exercise program in achieving a good range of movement and prevention of recontraction

An extreme extension contracture of wrist with dorsal contracture of fingers 15 years after burn injury is described. Shortening of extensor tendons, secondary lengthening of flexor tendons, contracted wrist joint capsule, unusual dislocation of carpals, dorsal dislocation of metacarpophalangeal joints of fingers, and provision of sufficient amount of good-quality skin were some of the issues that had to be addressed in treatment. The contracture was released, the carpals and metacarpophalangeal joint dislocations were corrected and fixed with K wires, and the resulting defect was covered with a sheet split-thickness skin graft. An exercise program was designed that consisted of isotonic, isokinetic, and isometric resistance exercises and passive, active, and active-assistive range of motion exercises. These exercises were pursued with the intention of increasing dynamic strength, endurance, and overall functional recovery of the flexor muscles by exploiting the immature nature of early scar tissue. The resultant enhanced flexor muscle power from exercises along with the dynamic splint helped in lengthening of extensor tendons, wrist joint capsule, and split-thickness skin graft. It also helped in resisting the recontracting tendency, with further recovery of good range of wrist and fingers movements, obviating the need of tendon-lengthening surgery and flap coverage. One and half years of follow up didn't show any sign of recontracture, and the patient was able to perform his routine activities. Postburn wrist contractures of such magnitude have been seldom described. Emphasis is put on simple contracture release and a postoperative exercise program.     

Bolivian health providers' attitudes toward alternative technologies for cervical cancer prevention: a focus on visual inspection with acetic Acid and cryotherapy

Abstract Background: Little is known about health providers' attitudes toward visual inspection with acetic acid (VIA) and cryotherapy in the prevention of cervical cancer, as most research in Latin America and the Caribbean (LAC) has examined attitudes of the general population. This study describes attitudes of Bolivian health professionals toward new technologies for cervical cancer prevention, focusing on VIA and cryotherapy. Methods: Between February 2011 and March 2012, we surveyed 7 nurses and 35 physicians who participated in 5-day workshops on VIA and cryotherapy conducted in Bolivia. Multiple choice and open-ended questions were used to assess participants' acceptability of these procedures and the feasibility of their implementation in the context of perceived barriers for the early detection of cervical cancer in this country. Results: Most believed that cultural factors represent the main barrier for the early detection of cervical cancer (70%), although all stated that VIA and cryotherapy would be accepted by women, citing the advantages of VIA over cytology for this belief. Most also believed their colleagues would accept VIA and cryotherapy (71%) and that VIA should replace Pap testing (61%), reiterating the advantages of VIA for these beliefs. Those who believed the contrary expressed a general resistance to change associated with an already existing cytology program and national norms prioritizing Pap testing. Conclusions: Most participants had favorable attitudes toward VIA and cryotherapy; however, a sizable minority cited challenges to their adoption by colleagues and believed VIA should not replace cytology. This report can inform the development of strategies to expand the use of alternative cervical cancer screening methods in LAC and Bolivia.     

The connection between human papillomavirus and oropharyngeal squamous cell carcinomas in the United States: implications for dentistry

BackgroundResults from studies conducted in the past several years suggest that some oropharyngeal cancers, those of the base of the tongue and the tonsils, are associated with high-risk types of human papillomavirus (HPV). In this article, the authors summarize the availabel evidence regarding the epidemiology of HPV-associated oropharyngeal cancers in the United States, the availabel HPV vaccines and the implications of these for dentistry. They also examine the differences in HPV prevalence between cancers of the oral cavity and those of the oropharynx.Types of Studies ReviewedThe authors searched PubMed, Web of Science, The Cochrane Library and the National Guideline Clearinghouse to identify English-language systematic reviews and meta-analyses focused on HPV-associated oropharyngeal squamous cell cancers published from January 2005 through May 2011.ResultsMolecular and epidemiologic evidence suggest a strong etiologic association of HPV with oropharyngeal cancers. The incidence of oropharyngeal cancers in the United States has increased between 1973 and 2007, whereas that of cancers at other head and neck sites has decreased steadily. Compared with HPV-negative cancers, HPV-positive oropharyngeal cancers are associated with certain sexual behaviors, occur more often among white men and people who do not use tobacco or alcohol, and may occur in a population younger by about four years (median ages, 52–56 years). Despite often having a later stage of diagnosis, people with HPV-positive oropharyngeal cancers have a lower risk of dying or recurrence than do those with HPV-negative cancers. The effectiveness of the HPV vaccine in preventing oropharyngeal cancers is unknown.Clinical ImplicationsDental health care personnel (DHCP) should be knowledgeable about the role of HPV in carcinogenesis, the association of HPV with oropharyngeal cancers and HPV vaccines, and they should be prompt in referring patients with suggestive symptoms for evaluation. DHCP can play an important role in increasing patients’ knowledge about HPV and oropharyngeal cancers.     

The Multiple Faces of Valosin-Containing Protein-Associated Diseases: Inclusion Body Myopathy with Paget’s Disease of Bone, Frontotemporal Dementia, and Amyotrophic Lateral Sclerosis

Inclusion body myopathy associated with Paget's disease of bone and frontotemporal dementia (IBMPFD) is a progressive, fatal genetic disorder with variable penetrance, predominantly affecting three main tissue types: muscle (IBM), bone (PDB), and brain (FTD). IBMPFD is caused by mutations in the ubiquitously expressed valosin-containing protein (VCP) gene, a member of the AAA-ATPase superfamily. The majority of individuals who develop IBM have progressive proximal muscle weakness. Muscle biopsies reveal rimmed vacuoles and inclusions that are ubiquitin- and TAR DNA binding protein-43 (TDP-43)-positive using immunohistochemistry. PDB, seen in half the individuals, is caused by overactive osteoclasts and is associated clinically with pain, elevated serum alkaline phosphatase, and X-ray findings of coarse trabeculation and sclerotic lesions. FTD diagnosed at a mean age of 55 years in a third of individuals is characterized clinically by comprehension deficits, dysnomia, dyscalculia, and social unawareness. Ubiquitin- and TDP-43-positive neuronal inclusions are also found in the brain. Genotype-phenotype correlations are difficult with marked intra-familial and inter-familial variations being seen. Varied phenotypes within families include frontotemporal dementia, amyotrophic lateral sclerosis, Parkinsonism, myotonia, cataracts, and anal incompetence, among others. Cellular and animal models indicate pathogenetic disturbances in IBMPFD tissues including altered protein degradation, autophagy pathway alterations, apoptosis, and mitochondrial dysfunction. Currently, mouse and drosophila models carrying VCP mutations provide insights into the human IBMPFD pathology and are useful as tools for preclinical studies and testing of therapeutic strategies. In this review, we will explore the pathogenesis and clinical phenotype of IBMPFD caused by VCP mutations.     

Rare presentation of post-transplant lymphoproliferative disorder isolated to gastroesophageal junction

Post transplant lymphoproliferative disorder (PTLD) represents a life threatening disorder occurring after transplantation, ranging from a polyclonal mononucleosis like illness to a monomorphic high grade neoplasm with cytologic and histopathologic evidence indicative of transformation to lymphoma. PTLD of diffuse large B-cell lymphoma (DLBCL) subtype, isolated to the esophagus is a rare diagnosis. We describe the first case of an immunocompromised adult patient diagnosed with DLBCL-PTLD limited to his esophagus without an associated mass or locoregional lymphadenopathy on imaging since the institution of the revised Cheson criteria, which includes positron emission tomography-computed tomography as the standard staging modality. Even more unique to our case was the suggestion of underlying cytomegalovirus (CMV) gastritis leading to a hypothesis about a less well understood relationship between CMV and Epstein Barr virus (EBV). In the post transplant setting, immunocompromised state, or EBV positive state, upper gastrointestinal symptoms should prompt investigation with an upper endoscopy (EGD). Additionally, specific to our case, the fact that the patients’ presentation was suspicious for CMV gastritis raises the possibility that the CMV infection predated his PTLD increasing his risk of acquiring PTLD. This reemphasizes the importance and diagnostic utility of early screening with EGD in patients after transplantation.     

Comparison of attitudes regarding preimplantation genetic diagnosis among patients with hereditary cancer syndromes

Preimplantation genetic diagnosis (PGD) allows couples to avoid having a child with an inherited condition, potentially reducing cancer burden in families with a hereditary cancer predisposition. This study investigated and compared awareness and acceptance of PGD among patients with different hereditary cancer syndromes. Questionnaires were mailed to 984 adults with hereditary breast and ovarian cancer, Lynch syndrome, familial adenomatous polyposis, or multiple endocrine neoplasia type 1 or 2. Associations between clinical, demographic, and psychosocial factors and awareness and acceptance of PGD were examined. Of 370 respondents (38 % return rate), 28 % felt their syndrome impacted family planning, 24 % were aware of PGD, 72 % felt that PGD should be offered, 43 % would consider using PGD, and 29 % were uncertain. Family experience and syndrome-specific characteristics, such as disease severity, quality of life and availability of medical interventions as well as gender, family planning stage, and religiosity impact perceptions of the acceptability of PGD, though a high level of uncertainty exists. Hereditary cancer patients lack awareness of PGD despite feeling that PGD should be offered, highlighting the need for education on this topic. While we found attitudes about the acceptability of PGD to be generally similar to those reported in the literature and of genetics and ethics experts, we observed similarities and differences between syndromes that provide insight into why some hereditary cancer patients may find PGD more acceptable than others.     

Herpes simplex virus entry receptor nectin-1 is widely expressed in the murine eye

PURPOSE: Nectin-1 belongs to the immunoglobulin superfamily, mediates cell-cell adhesion in cadherin-based adherens junctions, and acts as a receptor for herpes simplex virus (HSV). The goals of this study were (1) to determine whether nectin-1 is expressed in ocular tissue that is an important target of HSV infections and (2) to determine whether HSV type 1 (HSV-1) infection affects nectin-1 expression in the eye. METHODS: Expression of nectin-1 and HSV-1 protein was determined by immunohistochemical analysis of ocular tissues of untreated BALB/c mice and mice that were euthanized either 7 days or 7 months after corneal inoculation of HSV-1 or sterile tissue-culture medium (mock). RESULTS: In ocular tissues derived from untreated and mock-infected mice, widespread nectin-1 expression was detected among cells of the corneal epithelium and endothelium, conjunctiva, lens epithelium, ciliary body, iris, choroid, and retina. However, fibroblasts in the corneal stroma and the sclera did not express detectable levels of nectin-1. Ocular tissues from mice euthanized 7 days after corneal inoculation of HSV-1 frequently demonstrated corneal ulceration and inflammation and HSV-1 protein expression in the corneal epithelium, stroma, endothelium, conjunctiva, iris, and ciliary body but rarely in the retina. Ocular tissues from mice euthanized 7 months after HSV-1 inoculation demonstrated corneal epithelial and stromal inflammation, but HSV-1 protein expression was not detected. HSV-1 infection did not lead to a loss of nectin-1 expression in any of the tissues examined. In contrast to uninfected corneas, the inflamed and vascularized stroma of infected corneas contained mononuclear inflammatory cells, vascular cells, and fibroblasts that stained positive for nectin-1. CONCLUSIONS: Findings report that nectin-1 is widely expressed in murine ocular tissues. Only fibroblasts in the corneal stroma and sclera of uninfected tissues were devoid of nectin-1 expression. HSV-1-infected inflamed corneas contained some stromal fibroblasts with detectable nectin-1 expression, which potentially could be targeted by the virus. Widespread nectin-1 expression in the eye suggests that this receptor may play a role in the pathogenesis of ocular HSV infections.     

Correlates of sunburn experiences among U.S. adults: results of the 2000 National Health Interview Survey

OBJECTIVE: The purpose of this study was to determine the rate of sunburns in the U.S. adult population and the correlates of sunburns. METHODS: Data from the 2000 National Health Interview Survey Cancer Control Module were used to calculate the number of sunburns (0, 1, 2, or > or = 3) experienced during the past year by age, sex, race/ethnicity, and skin sensitivity to sun exposure. The relationship between no sunburns vs. one or more sunburns and additional demographic, health, and behavioral factors for adults who self-identify as white Hispanic or white non-Hispanic was assessed using general linear contrasts. Multivariate logistic regression modeling was conducted to determine the most important covariates associated with sunburns. All analyses were weighted for the complex sampling design. RESULTS: The study data suggest that overall, 18.5% (95% confidence interval [CI] 17.9, 19.1) of U.S. adults experience one sunburn a year, 9.7% (95% CI 9.3, 10.1) experience two, and 8.0% (95% CI 7.6, 8.4) experience > or = 3 sunburns. The data also indicate that adults who self-identify as white non-Hispanic experience sunburns more frequently than (in order of prevalence) those who identify as American Indian/Alaska Native, white Hispanic, Asian/Pacific Islander, or black. Sunburns were found to be more common among men than among women, more common among younger age groups than among older age groups, and more common among those with skin more prone to sunburn than among those with skin less prone to sunburn. Among individuals who self-identify as white Hispanic or white non-Hispanic, protective behaviors associated with lower rates of one or more sunburns in multivariate analyses are staying in the shade (odds ratio [OR] 0.73, 95% CI 0.66, 0.80) and wearing long-sleeved shirts (OR 0.86, 95% CI 0.75, 0.99). CONCLUSIONS: Many American adults have one or more sunburns per year. Methods to protect from sun exposure may not be used as needed to prevent sunburn.