Laryngeal manifestations of pachyonychia congenita: a clinical case and discussion on management for the otolaryngologist.

BACKGROUND: Pachyonychia congenita is a rare keratinising disorder, which typically presents during the first three years of life and usually affects the nails and palmoplantar surfaces. It can involve the larynx and potentially result in life-threatening airway obstruction. METHODS: A case report is presented and the findings of a literature review are reported. The review involved a PubMed search using the keywords 'pachyonychia congenita' together with 'larynx', 'laryngeal involvement', 'laryngeal obstruction', 'airway obstruction', 'hoarseness' and/or 'stridor'. RESULTS: A five-year-old boy, with confirmed pachyonychia congenita, presented with complications of laryngeal involvement over a four-year period. He required three intubations and a tracheostomy for acute airway obstruction. Treatment with potassium titanyl phosphate laser laryngoscopy stabilised the progression of laryngeal disease. CONCLUSION: Patients with pachyonychia congenita and laryngeal involvement can have a varied presentation, ranging from hoarseness to acute airway obstruction. Management can be a challenge, requiring early evaluation, regular surveillance and aggressive treatment. This paper reports our experience in managing and treating the laryngeal complications of a child with pachyonychia congenita.

Monitoring of radon gas in caves of the Yorkshire Dales, United Kingdom.

A number of vocational training courses are held in caves in the Yorkshire Dales region of the United Kingdom. The instructors and students involved in these courses have the potential to be exposed to enhanced levels of radon ((222)Rn) and its progeny as a result of their occupations. A prior radiological risk assessment for the training courses recommended that an environmental monitoring programme be carried out to establish the radon concentrations in the caves, and that the caving instructors wear personal radon dosemeters. Radon gas concentrations varied seasonally, being at their highest in summer and their lowest in winter. The lowest result was 40  Bq m(-3) recorded in Lower Longchurn cave during winter, whilst the highest result was 4440  Bq m(-3) recorded in Crackpot cave during the summer. As the individuals involved in the caving are entering atmospheres with radon gas concentrations in excess of 400  Bq m(-3), the Ionising Radiation Regulations 1999 (GB Parliament 2000 Ionising Radiations Regulations 1999 (London: Stationary Office) SI 1999/3232) apply. A system of work is therefore in place to control exposure to radon. This system of work stipulates an initial dose investigation level of 1 mSv, a second dose investigation level of 2 mSv and an annual dose limit of 6 mSv. The highest annual dose recorded to date is 2.2 mSv, although the average (median) annual dose is only 0.5 mSv.

Having a future: sexual decision making in early adolescence.

There is meager information about how adolescents decide to become sexually active and use protection against pregnancy and disease. The purpose of this project was to explore the thinking of adolescents, ages 12 to 14, about becoming sexually active and using protection during sexual encounters. In a health class, 45 adolescents responded to questions about influences on having sex and using protection. Short-answer, written responses were analyzed using Heideggerian hermeneutic interpretation. The reflections of youth faced with sexual decision-making focused on self-protection against pregnancy and disease. Postponing sexuality or using protection meant having a future, one that could hold the promise of love, marriage, and desired children.

How to start a back school.

Back schools have been shown to be a care efficient and cost effective approach to the management of patients with low back pain, decreasing back symptoms, restricted working capacity, and additional medical care pursuits. This paper presents the indications, goals, and contents of a back school and instructs the reader in how to implement such a program in a physical therapy setting.J Orthop Sports Phys Ther 1988;10(1):1-7.

The prediction of habitual alcohol use from alcohol related expectancies and personality.

Two studies testing social learning theory predictions of habitual alcohol use in college students are reported. Study 1 (N = 96) investigated alcohol related expectancies and social desirability as predictors of habitual alcohol use. Higher habitual alcohol use was correlated with higher expected dominance, but this correlation was not modified by social desirability or subject sex. Study 2 (N = 88) tested effects of personality traits on habitual alcohol use. Habitual alcohol use was positively correlated with EPI extraversion. Examination of extraversion sub-factors suggests that impulsivity and sociability independently predict drinking. The data indicate the strengths and limitations of alcohol related expectancy as an explanatory construct.

Is there a role for exercise in the treatment of patients with low back pain?

A paucity of information exists concerning the exact role of exercise in the management of low back pain. Few controlled clinical studies exist, and the selection of an exercise program is often relegated to traditional or empiric regimens. Aerobic conditioning exercises appear to offer the greatest benefit for all types of patients with back problems, although the value of these exercises is often overstated. Indirect evidence can be found for strengthening exercises that treat physical incapacity. No information is available to substantiate effects on pain relief. Comprehensive electromyographic studies, biomechanical analyses, and controlled clinical studies are needed to validate the role of exercises in the treatment of patients with back pain.

Analysis of current approaches and a practical guide to prescription of exercise.

Based on current information, aerobic exercises offer the greatest benefit to patients with back pain. Evidence supports the use of abdominal and extension exercises in a selected group of patients, but indications are limited, as are the useful types of exercises in both of these approaches. No support exists for the use of preprinted handouts, which assume that every patient with back pain, regardless of clinical presentation, requires the same six or eight exercises. Rather, selectivity of exercises based on a thorough clinical evaluation is recommended. Use of a specific exercise should be governed by the sound scientific principles presented in this paper. Many other types of therapeutic exercises not mentioned in the present paper are currently utilized in the care of patients with back pain.