Oesophageal causes of dysphagia localised only to the pharynx: Implications for the suspected head and neck cancer pathway.

OBJECTIVES: Dysphagia is a presenting symptom of both pharyngeal and oesophageal cancers. The referral pathway choice is determined by whether it is thought to be oropharyngeal or oesophageal, and this is in turn influenced by whether dysphagia is perceived to be above or below the suprasternal notch. We studied the concordance between the presence of pharynx-localised dysphagia (PLD) and the location of the underlying disease processes. DESIGN: A subset analysis of the Dysphagia Hotline Cohort, collected between 2004 and 2015, of patients with PLD and a structural diagnosis. MAIN OUTCOME MEASURES: Information about patient demography and presenting symptoms were recorded. The incisor-to-pathology distance, and the nature of the pathology, were recorded. Logistic regression analysis was used to identify independent predictors of malignancy. RESULTS: The study included 177 patients. There were 92 males, and mean age at presentation was 74 years. The commonest benign pathologies were cricopharyngeal dysfunction with or without pharyngeal pouch (n = 67), peptic stricture (n = 44) and Schatzki's ring (n = 11). There were 49 cases of cancer, including one hypopharyngeal cancer, one cervical oesophageal cancer, 28 cancers of the upper/mid-thoracic oesophagus, 15 cancers of the lower thoracic oesophagus and 4 cardio-oesophageal cancers. In 105 (59%) patients, PLD was caused by oesophageal disease. Independent predictors of malignancy were weight-change (loss >2.7 kg), a short history (<12 weeks) and presence of odynophagia. Nineteen (39%) of oesophageal cancers that presented with dysphagia that was localised only to the pharynx would have been beyond the reach of rigid oesophagoscopy. CONCLUSIONS: Pharynx-localised dysphagia is more likely to be a referred symptom of structural oesophageal disease, including cancer, than a primary symptom of structural pharyngeal disease. Absence of additional alarm symptoms such as a short history, weight-loss, and odynophagia, do not adequately exclude the possibility of oesophageal cancer. When the differential diagnosis of PLD includes malignancy, cancer should be presumed to be arising from the oesophagus or the cardio-oesophageal region until proven otherwise. This requires direct visualisation of the mucosal surfaces of the oesophagus and the cardio-oesophageal region, using either transoral or transnasal flexible endoscopy, irrespective of whether the initial assessment occurs within head and neck or upper gastrointestinal suspected cancer pathways.

Skin disease in sheep farmers.

BACKGROUND: An unusual inflammation of the pinna has been reported to occur in some sheep farmers at the time of lambing. AIMS: To explore the prevalence of this disorder and its possible causal associations. METHODS: While on attachment to sheep farms during lambing, veterinary students used a standardized questionnaire to interview a sample of farmers about their work and about symptoms of skin inflammation in their hands, face and ears. RESULTS: Interviews were completed by 76 (67%) of the farmers approached. Among 74 farmers who had carried out lambing, 3 (4%, 95% CI 1-11%) had experienced temporally related ear symptoms, all on multiple occasions. No farmers with ear symptoms had ever been involved in calving or farrowing, and no ear symptoms were reported in relation to shearing or dipping sheep. There was also an excess of hand symptoms related to lambing outdoors (24% of those who had done such work) and indoors (also 24%) compared with other farming activities. CONCLUSIONS: Our findings suggest that temporally related ear inflammation occurs in at least 1% of farmers who carry out lambing but not in association with the other farming activities investigated. Lambing appears to be associated also with hand inflammation, but the pathology may differ from that in the pinna.

The impact of modern techniques on the recurrence rate of inverted papilloma treated by endonasal surgery.

Sinonasal inverted papilloma (IP) is a benign epithelial tumour which displays aggressive local behaviour, has a high local recurrence rate and the potential for malignant transformation. It is treated by surgical excision which must be thorough to avoid recurrence. Traditionally this was done by an open approach, but since the early 1990's endoscopic techniques have been increasingly employed and are now widely accepted for the treatment of IP. This has led to debate as to whether the access afforded endoscopically is adequate to treat IP without a higher recurrence rate. Studies comparing the recurrence rates of open to endoscopic approaches have shown similar rates but open approaches are usually considered the gold standard for advanced disease, despite the higher morbidity. Reviewing the literature we found that the recurrence rates with endoscopic surgery have improved significantly since the technique was first introduced and conclude that to accurately compare open and endoscopic techniques historical data, from the early days of endoscopic surgery, should be excluded as it does not truly represent the outcome with modern techniques. In doing this it is apparent that endoscopic surgery is the gold standard for the treatment of the vast majority of IP.

'Lambing ears': a blistering disorder affecting farmers at lambing time.

BACKGROUND: At lambing time some farmers experience blistering and crusting of the pinnae. This occupational disease, termed 'lambing ears', does not feature in the medical literature. OBJECTIVES: To define the condition and explore its pathogenesis. METHODS: We obtained five biopsies from affected individuals and sent questionnaires to 69 farmers in the U.K. Farming communities abroad were also contacted. RESULTS: The eruption lasts for the duration of the lambing practice. The histological features are dominated by a pandermal perivascular and diffuse, predominantly T-cell lymphocytic infiltrate. Only the pinnae are affected and its incidence is related to the degree of involvement a farmer has with the animals around parturition. The condition also occurs, but less frequently, in farmers who are calving. CONCLUSIONS: This occupational disease occurs with close contact to lambing ewes or calving cows. The histology and distribution are comparable with the juvenile spring eruption variant of polymorphic light eruption, but its demographics are unique.

A connexin 26 mutation causes a syndrome of sensorineural hearing loss and palmoplantar hyperkeratosis (MIM 148350).

We report a missense mutation in the connexin 26 gene (GJB2) in a family with an autosomal dominant syndrome of hearing loss and hyperkeratosis. The affected family members have high frequency, slowly progressive, bilateral, sensorineural hearing loss and palmoplantar hyperkeratosis. The mutation causes an amino acid substitution (G59A), which may disrupt a reverse turn in the first extracellular loop of connexin 26. Connexin 26 mutations have been reported in syndromes of deafness and palmoplantar keratoderma. These data provide additional evidence for the role of connexin 26 in syndromes of this type.

Genetic control of the circulating concentration of transforming growth factor type beta1.

The concentration of transforming growth factor beta (TGF-beta) in plasma has been correlated with the development of several diseases, including atherosclerosis and certain forms of cancer. However, the mechanisms that control the concentration of TGF-beta in plasma are poorly understood. In a study of 170 pairs of female twins (average age 57.7 years) we show that the concentration of active plus acid-activatable latent TGF-beta1 [(a+l) TGF-beta therefore is predominantly under genetic control (heritability estimate 0.54). Single strand conformation polymorphism (SSCP) mapping of the TGF-beta1 gene promoter has identified two single base substitution polymorphisms. The two polymorphisms (G-->A at position -800 bp and C-->T at position -509 bp) are in linkage disequilibrium (correlation coefficient Delta = 0.215, P < 0.01). The C-509T polymorphism is significantly associated with the plasma concentration of (a+l) TGF-beta1, explaining 8.2% of the additive genetic variance of (a+l) TGF-beta1 concentration. It is therefore possible that predisposition to atherosclerosis, bone diseases or various forms of cancer may be correlated with the presence of particular alleles at the TGFB1 locus.

Transforming growth factor-beta1 gene polymorphisms and coronary artery disease.

1. Transforming growth factor-beta1 is a cytokine with a very wide spectrum of biological activities. Previous studies have shown that it is involved in a number of physiological and pathological processes including heart disease. In our study we aimed to scan the transforming growth factor-beta1 locus for polymorphisms and to identify haplotypes significantly associated with a predisposition to coronary atherosclerosis.2. Two patient groups comprising 244 angiographically normal individuals and 655 patients with coronary artery disease were recruited from London and Sheffield. DNA samples from these subjects were screened for mutations in the transforming growth factor-beta1 locus and all subjects were genotyped by a coupled polymerase chain reaction-restriction enzyme digestion method.3. Five polymorphisms have been identified in the transforming growth factor-beta1 gene at positions G-800A, C-509T in the promoter region, Leu10-->Pro, Arg25-->Pro in exon 1 and Thr263-->Ile in exon 5. No significant difference in frequencies for any of the five polymorphisms was found between controls and patients with coronary artery disease. Similarly, there was no correlation between these polymorphisms and hypertension.4. The genotypes of all the individuals participating in the study were assigned to seven main haplotypes of the transforming growth factor-beta1 locus. Based on species comparison data we propose that GCCGC is the ancestral haplotype in humans.5. Our data suggest that these transforming growth factor-beta1 polymorphisms are not associated with coronary artery disease and therefore their presence alone would not be a genetic risk factor for predisposition to coronary artery disease.

Measuring the quality of life of older persons: a model with implications for community and public health nursing.

Measuring the quality of life (QOL) of older persons can assist health professionals in achieving a number of important objectives. These include assessing the effects of illness and treatment, identifying need for support services, and developing health enhancing environments. Most QOL models focus unduly on illness and disability, define QOL too narrowly, and do not consider aspects of personal control and potential opportunities for change. A new model of QOL with associated instrumentation, the Quality of Life Profile: Seniors Version (QOLPSV), is described. Administration of the QOLPSV to 205 older persons in Ontario, Canada found it to be reliable and valid. Limitations of the instrument are presented and potential uses explored.

Frailty: a public health perspective.

Traditionally, frailty has been conceptualized as reflecting an inevitable decline in abilities usually associated with physical aspects of aging. More recent thinking now sees frailty as reflecting an interaction among individual factors and a range of environmental elements. These emerging models, however, continue to consider frailty as a condition that resides within the individual rather than a situation that exists for the individual. We present a new model that defines frailty as occurring when there is diminished ability to carry out the important practical and social activities of daily living. Factors related to the occurrence of frailty are considered, as are the theoretical and practice implications of viewing frailty as a social construction.

The quality of life of seniors living in the community: a conceptualization with implications for public health practice.

Maintaining and improving the quality of life (QOL) of seniors in the community is an increasingly important goal of public health planning and programming. To provide authentic assessments of seniors' quality of life, a partnership of public health workers and university researchers, working closely with seniors in the community, developed the Quality of Life Profile: Seniors Version (QOLPSV). Administration of the QOLPSV to a wide range of North York, Ontario seniors (n = 205) participating in community-based healthful living programs provided evidence of the instrument's psychometric integrity. Additionally, results indicated specific areas of need among seniors, suggesting shifts in emphasis in program delivery. Additional public health applications of a quality of life perspective are outlined.