Multicentric Reticulohistiocytosis-A rare and disabling disease.

Multicentric reticulohistiocytosis is a rare multisystemic condition associated with papulonodular skin lesions, severe arthritis and malignancy. Histopathology shows histiocytes containing abundant eosinophilic ground glass cytoplasm and multinucleated giant cells. Early recognition, age-appropriate malignancy work-up and treatment is important to prevent impairment of daily life activity.

Implementation of a Children's Safe Asthma Discharge Care Pathway Reduces the Risk of Future Asthma Attacks in Children-A Retrospective Quality Improvement Report.

Background: Many children attend Emergency Departments (ED) and Out of Hours (OoH) frequently for acute asthma. Follow up care is often suboptimal leaving these children at risk of a future attacks. We report on the development, implementation and evaluation of a safe asthma discharge care pathway (SADCP). Methods: This is a retrospective report on the development, implementation and evaluation of outcomes of a SADCP. The pathway was based on the Teach-to-goal educational methodology that supported the mastery correct inhaler technique and ability to action the personalized asthma action plan (PAAP). Children with frequent asthma attacks were entered as they were discharged from the Emergency Department or ward. The first training session occurred within 1-3 weeks of the index asthma attack with 2 further sessions in the following 8 weeks. Children exiting the pathway were discharged either back to primary care or to a hospital clinic. Results: 81 children entered the pathway (median age 5 years) with 72 discharged from the ED and 9 from the medical wards of the Royal Belfast Hospital for Sick Children. At pathway entry 13% had correct inhaler technique, 10% had a Personalized Asthma Action Plan (PAAP), and 5% had >80% (45% >50%) repeat refill evidence of adherence to inhaled corticosteroid over the previous 12 months. On pathway exit all children demonstrated correct inhaler technique and were able to action their PAAP. One year later 51% and 95% had refill evidence of >80% and >50% adherence. Comparisons of the 12 months before and 12 months after exit from the pathway the median number of emergency ED or OoH asthma attendances and courses of oral corticosteroids reduced to zero with >75% having no attacks requiring this level of attention. Similar findings resulted when the SADCP was implemented in a district general hospital pediatric unit. Conclusion: Implementing an asthma care pathway, using Teach-to-Goal skill training methods and frequent early reviews after an index asthma attack can reduce the future risk of asthma attacks in the next 6 to 12 months.

Cutaneous venous malformations presenting in an adolescent boy: Cerebral cavernous malformation syndrome.

Cerebral cavernous malformations (CCM) are collections of enlarged capillaries with irregular structure, located in the brain or spinal cord, that can be life-threatening. We describe a neurologically asymptomatic 15-year-old boy who presented with cutaneous venous malformations (CVM) that developed in adolescence. Owing to his family history of CCM, genetic testing was obtained and confirmed a new mutation in the CCM3 (PDCD10) gene. Brain imaging showed multiple CCM. Although currently asymptomatic, he will continue to be monitored annually by neurology to assess for symptoms of intracranial hemorrhage (ICH) that would warrant investigation with MRI.

Successful topical rapamycin treatment for facial angiofibromata in two children.

Topical sirolimus, or rapamycin, is known to inhibit tumorigenesis in tuberous sclerosis. We report two cases of successful treatment of children with facial angiofibromata and summarize the encouraging evidence of the effectiveness of this therapy in the literature.

Comparative repeatability of two handheld fractional exhaled nitric oxide monitors.

BACKGROUND: The use of portable fractional exhaled nitric oxide (FENO) devices is increasingly common in the diagnosis and management of allergic airways inflammation. METHODS: We tested two handheld FENO devices, to determine (a) if there was adequate intradevice repeatability to allow the use of single breath testing, and (b) if the devices could be used interchangeably. In a mixed pediatric population, including normal, asthmatic, and children with peanut allergies, 858 paired values were collected from the NIOX-MINO® and/or the NObreath® devices. RESULTS: The NIOX-MINO® showed excellent repeatability (mean difference of 0.1 with 95% limits of agreement between -7.93 to 7.72 ppb), while the NObreath® showed good repeatability (mean difference of -1.61 with 95% limits of agreement between -14.1 and 10.8 ppb). Intradevice repeatability was good but not adequate and the NIOX-MINO® systematically produced higher results than the NObreath® [mean difference of 7.8 ppb with 95% limits of agreement from -11.55 to 27.52 ppb (-33% to 290%)]. CONCLUSIONS: Our results support the manufacturer's advice that single breath testing is appropriate for the NIOX-MINO®. NObreath® results indicate that the mean of more than one breath should be utilized. The devices cannot be used interchangeably.

The 'unified airway': the RCPCH care pathway for children with asthma and/or rhinitis.

AIMS: The Royal College of Paediatrics and Child Health (RCPCH) Science and Research Department was commissioned by the Department of Health to develop national care pathways for children with allergies: the asthma/rhinitis care pathway is the third such pathway. Asthma and rhinitis have been considered together. These conditions co-exist commonly, have remarkably similar immuno-pathology and an integrated management approach benefits symptom control. METHOD: The asthma/rhinitis pathway was developed by a multidisciplinary working group and was based on a comprehensive review of evidence. The pathway was reviewed by a broad group of stakeholders including the public and was approved by the Allergy Care Pathways Project Board and the RCPCH Clinical Standards Committee. RESULTS: The pathway entry points are defined by symptom type and severity at presentation. Acute severe rhinitis and life-threatening asthma are presented as distinct entry routes to the pathway, recognising that initial care of these conditions requires presentation-specific treatments. However, the pathway emphasises that ideal long term care should take account of both conditions in order to achieve maximal improvements in disease control and quality of life. CONCLUSIONS: The pathway recommends that acute presentations of asthma and/or rhinitis should be treated separately. Where both conditions exist, ongoing management should address the upper and lower airways. The authors recommend that this pathway is implemented locally by a multidisciplinary team (MDT) with a focus on creating networks. The MDT within these networks should work with patients to develop and agree on care plans that are age and culturally appropriate.

Does one size fit all? Investigating heterogeneity in men's preferences for benign prostatic hyperplasia treatment using mixed logit analysis.

In this study, the authors demonstrate how mixed logit analysis of discrete choice experiment (DCE) data can provide information about unobserved preference heterogeneity. Their application investigates unobserved heterogeneity in men's preferences for benign prostatic hyperplasia (BPH) treatment. They use a DCE to elicit preferences for seven characteristics of BPH treatment: time to symptom improvement, sexual and nonsexual treatment side effects, risks of acute urinary retention and surgery, cost of treatment, and reduction in prostate size. They investigate the importance of these characteristics and the trade-offs men are willing to make between them. Preferences are elicited from a sample of 100 men attending an outpatient clinic in Ireland. The authors find all treatment characteristics are significant determinants of treatment choice. There is significant preference heterogeneity in the population for four treatment characteristics: time to symptom improvement, treatment reducing prostate size, risk of surgery, and sexual side effects. The importance of preference heterogeneity at the policy level within the context of shared decision making is discussed.

Building health impact assessment capacity as a lever for healthy public policy in urban planning.

Building capacity to improve health through applying health impact assessment (HIA) increases the range of people, organisations and communities who are able to address health problems and, in particular, the problems that arise out of social inequity and social exclusion. To achieve this, a range of strategies is required across the areas of organisational development, workforce development, resource allocation, leadership and partnerships. A conceptual framework to guide understanding of capacity building evolved during a three-year capacity building project that supported the implementation of HIA. This is also applicable to the broader agenda of healthy public policy.

Use of IGIV in the treatment of atopic dermatitis, urticaria, scleromyxedema, pyoderma gangrenosum, psoriasis, and pretibial myxedema.

There has been a rapid expansion in the use of IGIV for an ever-growing number of conditions. It is a product with an excellent safety record without the side effects of steroids or other immunosuppressive agents. There have been numerous recent advances in our understanding of the mechanisms of action of IGIV in many of the conditions for which it is being used, but there is still much to be learned. IGIV has had a major impact in neurology, haematology, immunology, rheumatology and dermatology. The limitations for IGIV are cost of the preparation itself and the logistical problems associated with its administration. Here we describe the published evidence for the use of high-dose IGIV in the dermatological conditions atopic dermatitis, urticaria, scleromyxedema, pyoderma gangrenosum, psoriasis and pretibial myxedema. These conditions have an emerging evidence base for hdIGIV which is relatively small consisting mainly of case reports and small case series. The outcomes in a number of these conditions appear encouraging, but as the reports are likely to reflect a bias for positive results, one must be cautious about drawing firm conclusions.

Postinflammatory hyperpigmentation following torture.

Hyperpigmentation after torture in darker skinned patients has regularly been noted, although its pathophysiology, and thus its forensic importance, has not previously been documented. Hyperpigmentation is not well described in the dermatological literature. It is the result of inflammation. Any inflammation can cause hyperpigmentation, and the shape of the resulting lesion can closely follow the contours of the site of original inflammatory response. This can be important in correlating the lesion with the alleged cause. It also helps to establish the differential diagnosis of the lesion, which also assists in assessing the degree of consistency between the lesion and the alleged cause. Patterns of hyperpigmentation can therefore, be helpful in assessing allegations of torture months or years after the event.

Sexually transmitted infections: a medical anthropological study from the Tari research unit 1990-1991.

This paper describes medical anthropological research conducted while I was based at the Tari Research Unit for six months in 1990-1991. The research aimed to gain a deeper understanding of the social factors surrounding the transmission of sexually transmitted infections, which had escalated following a local gold rush in 1989. Although HIV/AIDS was a very minor health issue in Papua New Guinea at that time, medical staff were aware of the likelihood of the disease becoming prevalent in the highlands in the near future. The research indicated that many people regarded sexually transmitted infections (STIs) as a nuisance, rather than a serious health risk. Discussions with chronic sufferers revealed that they were more concerned about the dangers of infertility than the immediate effects of the infections. The paper considers the risk-taking that the people of Tari, the Huli, were prepared to accept and suggests ways in which these risks might be minimized.