Hyper-IgE syndrome in an 11 year old female presenting with acneiform rash.

Hyper-IgE (HIES) is a rare, primary immunodeficiency characterised by eczema, recurrent staphylococcal infections, pneumonia, increased serum IgE and eosinophilia. We present the case of an 11-year-old girl presenting to dermatology with an acneiform facial rash and associated bacterial lymphadenitis. History was significant for otitis media, primary tooth retention, low impact wrist fracture, infantile acne and an absence of eczema or pneumonia. Investigations demonstrated mildly elevated IgE, normal eosinophils but positivity for a STAT3 gene mutation-thus representing a case of HIES presenting as an acneiform facial rash with absence of other primary immunological features.

The impact of providing flexible meals on patients' nutritional intake, fasting times and cost when admitted to a trauma unit.

BACKGROUND: Malnutrition is associated with increased morbidity, mortality, decreased quality of life, increased length of hospital stay and higher treatment costs. Prevalence rates in Australian hospitals is reported between 30% and 50%. Trauma patients experience significant periods of restricted oral intake resulting from pre-operative fasting for complex and frequent surgical procedures. In addition, they have elevated nutritional requirements for recovery resulting in significant accrued nutritional deficits. The present study hypothesised that not having hot meals available outside of the hospital food service system was contributing to increased nutritional deficits. The study aimed to investigate the impact of providing flexible frozen meals and snacks in a trauma ward, on nutritional intake, cost and duration of perioperative fasting. METHODS: This was a pre- and post-interventional study examining 40 fasting experiences of hospitalised patients in a trauma ward. Frozen meals and snack bags were readily accessible to nursing staff to provide to patients out of kitchen service hours. Nutritional intake and fasting times were measured from patient records and interviews. RESULTS: Implementing flexible food items increased patient nutritional intake by 28% (15%-43%) on the day of fasting. Fasting duration was not significantly reduced (1.73 h); however, when patient fasting was ceased, food was provided more responsively. Nursing staff showed improved satisfaction with project implementation. CONCLUSIONS: The present study has demonstrated that provision of food items outside of regular meal service hours is a low cost intervention that improves nutritional intake, provides nutrition more responsively and is well received by nursing staff.

Pain, swelling and irritability in the sun: what is the diagnosis?

A 7-year-old boy presented with a 24-hour history of severe burning pain affecting both hands that had started within minutes of playing outdoors. His mother reported that he had been running his hands under cold water and shaking his hands and head to try and relieve the pain. On examination, there was swelling of his hands, eyelids and cheeks. His parents mentioned that his hands and face had 'swollen in the sun' every summer from the age of 2 years.A 3-year-old girl presented with a 2-day history of left upper limb swelling following a day of prolonged sun exposure. Examination revealed non-pitting oedema extending from her left shoulder to hand with no associated tenderness, erythema or rash. Her mother reported six previous episodes of irritability following sun exposure during which she would cry and flap her hands 'for hours'.Oral steroids and antihistamines were prescribed in both cases with little effect. Findings of routine baseline investigations were normal in both cases. A radiograph of the upper limb in the second patient disclosed nothing abnormal.

Sialorrhoea Management Practices in Residential Older Adults Care Settings: A Qualitative Study.

AIM: The aim of this research was to explore both older adults' and health care professionals' experience and views of sialorrhoea management practices in older adult residential care settings. BACKGROUND: Sialorrhoea is quite a bothersome symptom among patients with certain neurological conditions. The complexity of sialorrhoea and its complications can be quite challenging for health care professionals. In the management of sialorrhoea, a multidisciplinary approach is proposed as an effective way of sialorrhoea management. METHODS: Thematic analysis of collected data via semi-structured qualitative interviews with five focus groups involving 28 multidisciplinary health care members and 1 patient. RESULTS: The older adult and multidisciplinary health care professionals' view of the management of sialorrhoea in residential care settings were established under three main themes: 1) 'Sialorrhoea compromising patient's dignity', 2) 'Ad hoc local management' and 3) 'Further integration of care required'. CONCLUSION: At present, there have been no comprehensive multidisciplinary sialorrhoea management strategies to meet the various needs of older adults with sialorrhoea. IMPLICATION FOR NURSING MANAGEMENT: It is important to minimize the negative impact of sialorrhoea on the patients. The recognition of issues associated with sialorrhoea provides constructive scope for the health care professionals to further investigate and develop more effective integrated sialorrhoea care protocols.

Cross-sectional study of the association between skin tags and vascular risk factors in a bariatric clinic-based cohort of Irish adults with morbid obesity.

OBJECTIVE: Skin tags are associated with an insulin resistant phenotype but studies in White Europeans with morbid obesity are lacking. We sought to determine whether the presence of cervical or axillary skin tags was associated with increased cardiovascular risk in Irish adults with morbid obesity. We conducted a cross-sectional study of patients attending our Irish regional bariatric centre with a BMI ≥ 40 kg m-2 (or ≥ 35 kg m-2 with co-morbidities). We compared anthropometric and metabolic characteristics in those with versus without skin tags. RESULTS: Of 164 patients, 100 (31 male, 37 with type 2 diabetes, 36 on lipid lowering therapy, 41 on antihypertensive therapy) participated. Mean age was 53.7 ± 11.3 (range 31.1-80) years. Cervical or axillary tags were present in 85 patients. Those with tags had higher systolic blood pressure 138.0 ± 16.0 versus 125.1 ± 8.3 mmHg, p = 0.003) and HbA1c (46.5 ± 13.2 versus 36.8 ± 3.5 mmol/mol, p = 0.017). Tags were present in 94.6% of patients with diabetes, compared to 79.4% of those without diabetes (p = 0.039). Antihypertensive therapy was used by 45.8% of patients with skin tags compared to 13.3% without tags (p = 0.018). In bariatric clinic attenders skin tags were associated with higher SBP and HbA1c and a higher prevalence of diabetes and hypertension, consistent with increased vascular risk, but lipid profiles were similar.

A systematic review of noninvasive and invasive sialorrhoea management.

AIMS AND OBJECTIVES: To review published literature pertaining to the management of sialorrhoea while also highlighting the significance of the multidisciplinary approach. BACKGROUND: Sialorrhoea is a common and troublesome problem among certain neurological patients. It is distressing for patients and caregivers, and can be challenging for healthcare professionals. Various sialorrhoea management approaches have been documented. However, there is no clear consensus on best management practices. Therefore, it is necessary to systematically review and synthesise various approaches so as to provide an understanding of the efficacy of management approaches. DESIGN: Systematic literature review using PRISMA checklist (see Appendix S1). METHOD: Five databases (ScienceDirect, Wiley Online Library, CINAHL, Cochrane Library and PubMed) were searched (years 2001-2018) following inclusion criteria. Out of 1,294 identified records, 29 studies met the inclusion criteria. RESULTS: Various management approaches identified, ranging from noninvasive, such as speech therapy aiming to enhance swallowing behaviour, to invasive treatment including anticholinergic medication, botulinum toxin injection and surgical techniques. However, in the majority of cases, there is no scientific evidence-based management protocol leading to favourable results, and the evidence base for intervention effectiveness remains weak. CONCLUSIONS: The multifactor nature of sialorrhoea and its associated complications presents challenges for the medical care team. None of the management strategies stand alone as the best modality; therefore, it is proposed that management strategies follow a multidisciplinary approach to meet the diverse needs of patients. RELEVANCE TO CLINICAL PRACTICE: A comprehensive understanding of different sialorrhoea management approaches will enable healthcare professionals to identify the signs and symptoms regarding sialorrhoea, and to assist in effective management implementation. This will help to improve the management of sialorrhoea, hence, to improve quality of life of patients and provide formative scope to the development of an integrated care pathway.

Case Report of Calciphylaxis Secondary to Calcium and Vitamin D3 Supplementation.

BACKGROUND: Calciphylaxis is a rare disorder that is very unusual outside the setting of end-stage kidney disease. CASE SUMMARY: A 64-year-old woman with normal renal function presented with painful leg ulcers. She had previously received 300 000 IU of vitamin D3 followed by daily calcium and vitamin D3 supplementation. A skin biopsy was consistent with calciphylaxis, and she was treated with sodium thiosulphate infusions and wound debridement. CONCLUSION: Calcium and vitamin D3 supplements are widely prescribed. We report a case of calciphylaxis triggered by calcium and vitamin D3 supplementation in a patient with none of the typical risk factors. Our patient had an excellent response to treatment with sodium thiosulphate.

Cutaneous vasculitis overlap with acute generalised exanthematous pustulosis (AGEP).

A man in his 40s developed a severe cutaneous adverse reaction following treatment of septic arthritis with flucloxacillin. The eruption had overlap features of cutaneous vasculitis and acute generalised exanthematous pustulosis which was complicated by renal and liver impairment. This case heightens the variation in presentation of a severe drug eruption.

Panton-Valentine leukocidin-producing Staphylococcal aureus: report of four siblings.

Panton-Valentine leukocidin (PVL)-producing Staphylococcus aureus results in leukocyte destruction and tissue necrosis (Pediatric Dermatology 2007;24:401). It can be associated with a spectrum of clinical manifestations that range from localized staphylococcal skin infections to sometimes severe necrotizing pneumonia (Clin Infect Dis 1999;29:1128). We report a case of four siblings, three brothers whose atopic dermatitis was complicated by cutaneous lesions and furunculosis, while their 21-month-old sister had a fatal PVL positive staphylococcal pneumonia.