Real World Management of Anaphylaxis Versus the National Institute for Health and Clinical Excellence (NICE) Guidelines.

Objectives  Anaphylaxis is an acute, life-threatening immediate allergic reaction caused by the sudden systemic release of mediators from mast cells. This study aims to assess the current practice of emergency management of children and adults diagnosed with anaphylaxis at the Royal Hospital, Muscat, Oman, in line with the National Institute for Health and Clinical Excellence (NICE) guidelines.  Methods This is an observational retrospective study of all anaphylaxis cases seen at the emergency department (ED) from January 2013 to January 2018 and compared with the management of anaphylaxis in the ED as per the NICE guidelines. Inclusion criteria were all patients, children (age 16 and below), and adults diagnosed with anaphylaxis based on the World Allergy Organization (WAO) criteria. Exclusion criteria are all cases labeled as anaphylaxis that did not match the WAO criteria for anaphylaxis. Results Of 100 patients with a preliminary diagnosis of anaphylaxis, 49 patients (49%) were true-anaphylaxis cases based on the WAO definition 16 were children (age 16 years and below), and 33 were adults ( age 16 years and above). The other 51 patients (51%) with misdiagnosed anaphylaxis were later diagnosed with spontaneous urticaria, septic shock, vocal cord dysfunction, severe asthma, and anxiety attack. All 49 patients with true-anaphylaxis appropriately received adrenaline intramuscularly at the ED. All 16 children were admitted, seen by an allergist, and received an adrenaline auto-injector when indicated. Only 5 of the 33 adults were admitted and seen by an allergist, and 4 of those required an adrenaline auto-injector upon discharge. The remaining 28 adults were discharged from the ED, and only 3 of these were referred to the allergist. None received an adrenaline auto-injector upon discharge from the ED, and no mention in the ED notes on patient education regarding allergen avoidance. Conclusion Third of the patients who presented to ED were children (<16 years), and two third were adults. Insect venom was the main reason for anaphylaxis in both age groups. There was an underutilization of adrenaline auto-injector prescriptions for adult patients. This could be very well improved by disseminating policies and guidelines to adult physicians.

Approach to patients with refractory coeliac disease.

Refractory coeliac disease (RCD) is a recognised complication, albeit very rare, of coeliac disease (CD). This condition is described when individuals with CD continue to experience enteropathy and subsequent or ongoing malabsorption despite strict adherence to a diet devoid of gluten for at least 12 months and when all other causes mimicking this condition are excluded. Depending on the T-cell morphology and T-cell receptor (TCR) clonality at the ß/γ loci, RCD can be subdivided into type 1 (normal intra-epithelial lymphocyte morphology, polyclonal TCR population) and type 2 (aberrant IELs with clonal TCR). It is important to differentiate between the two types as type 1 has an 80% survival rate and is managed with strict nutritional and pharmacological management. RCD type 2 on the other hand has a 5-year mortality of 50% and can be complicated by ulcerative jejunitis or enteropathy-associated T-cell lymphoma (EATL). Management of RCD type 2 has challenged many experts, and different treatment approaches have been adopted with variable results. Some of these treatments include immunomodulation with azathioprine and steroids, methotrexate, cyclosporine, alemtuzumab (an anti CD-52 monoclonal antibody), and cladribine or fludarabine sometimes with autologous stem cell transplantation. In this article, we summarise the management approach to patients with RCD type 2.

Recognising and Managing Refractory Coeliac Disease: A Tertiary Centre Experience.

Refractory coeliac disease (RCD) is a rare complication of coeliac disease (CD) and involves malabsorption and villous atrophy despite adherence to a strict gluten-free diet (GFD) for at least 12 months in the absence of another cause. RCD is classified based on the T-cells in the intra-epithelial lymphocyte (IEL) morphology into type 1 with normal IEL and type 2 with aberrant IEL (clonal) by PCR (polymerase chain reaction) for T cell receptors (TCR) at the ß/γ loci. RCD type 1 is managed with strict nutritional and pharmacological management. RCD type 2 can be complicated by ulcerative jejunitis or enteropathy associated lymphoma (EATL), the latter having a five-year mortality of 50%. Management options for RCD type 2 and response to treatment differs across centres and there have been debates over the best treatment option. Treatment options that have been used include azathioprine and steroids, methotrexate, cyclosporine, campath (an anti CD-52 monoclonal antibody), and cladribine or fluadribine with or without autologous stem cell transplantation. We present a tertiary centre's experience in the treatment of RCD type 2 where treatment with prednisolone and azathioprine was used, and our results show good response with histological recovery in 56.6% of treated individuals.

Support for patients with celiac disease: A literature review.

BACKGROUND: Celiac disease (CD) is a lifelong disorder. Patients are at increased risk of complications and comorbidity. OBJECTIVES: We conducted a review of the literature on patient support and information in CD and aim to issue recommendations about patient information with regards to CD. DATA SOURCE: We searched PubMed for English-language articles published between 1900 and June 2014, containing terms related to costs, economics of CD, or education and CD. STUDY SELECTION: Papers deemed relevant by any of the participating authors were included in the study. DATA SYNTHESIS: No quantitative synthesis of data was performed. Instead we formulated a consensus view of the information that should be offered to all patients with CD. RESULTS: There are few randomized clinical trials examining the effect of patient support in CD. Patients and their families receive information from many sources. It is important that health care personnel guide the patient through the plethora of facts and comments on the Internet. An understanding of CD is likely to improve dietary adherence. Patients should be educated about current knowledge about risk factors for CD, as well as the increased risk of complications. Patients should also be advised to avoid other health hazards, such as smoking. Many patients are eager to learn about future non-dietary treatments of CD. This review also comments on novel therapies but it is important to stress that no such treatment is available at present. CONCLUSION: Based on mostly observational data, we suggest that patient support and information should be an integral part of the management of CD, and is likely to affect the outcome of CD.

Management of celiac disease.

Celiac disease (CD) is an enteropathy-induced immune response that occurs on exposure to toxic gluten in the diet and is reversible once gluten is withdrawn. A gluten-free diet is the preferred treatment for CD and leads to reversal of villous atrophy. Counseling, nutritional support, and follow-up are vital aspects in CD management. The pickup rate of CD has improved with the availability of serologic tests, and this has led to a reduction in morbidity in treated CD cases. Managing CD can potentially prevent or cure some of the associated conditions, such as neurologic complications, nutritional deficiencies, and osteoporosis.