Cold-Induced Urticaria in a Paediatric Patient: A Case Report and Literature Review.

Acquired cold-induced urticaria is a rare form of physical urticaria, especially in children. The variety of clinical presentations and the low estimated prevalence contribute to its underdiagnosis. Given the associated risk of anaphylaxis, it is crucial to alert clinicians to the different forms of presentation, diagnosis, and treatment. Starting with a case report of acquired cold-induced urticaria in a previously healthy nine-year-old boy, the authors then review the literature about acquired cold-induced urticaria and discuss the diagnostic exams and disease management.

Desensitization in children allergic to drugs: Indications, protocols, and limits.

Any drug can potentially induce a hypersensitivity reaction. If after the allergological work-up the drug hypersensitivity reaction is confirmed, in most cases, the simple avoidance of the culprit drug and a suggestion of an unrelated alternative is enough. However, there are circumstances where the choice to stop the treatment affects the survival, the safety and/or the quality of life of the patient and the global outcome of the disease in question. When this occurs, drug desensitization can be the answer and should not be viewed as an extravagance, nor the pediatric age should be considered a contraindication. Drug desensitization in children can be safely and successfully performed, having a positive impact on the survival and overall prognosis. In general, the indications for DDS are the same in adults as in children. However, in this age group there are specificities that this paper aimed to describe, reviewing the mechanisms behind drug hypersensitivity and rapid drug desensitization, types of protocols, indications, and contraindications, as well as several technical aspects that are specific to the pediatric age.

Idiopathic mast cell activation syndrome is more often suspected than diagnosed-A prospective real-life study.

BACKGROUND: Idiopathic mast cell activation syndrome (MCAS) is characterized by three diagnostic criteria: (1) episodic mast cell (MC)-driven signs/symptoms of at least two organ systems in the absence of clonal MC expansion and definite triggers, (2) episodic increase in tryptase, and (3) response to MC-targeted treatment. Many patients believe they have MCAS, but how often this is the case remains unknown. METHODS: We prospectively investigated patients with suspected MCAS (n = 100) for the diagnostic criteria including baseline tryptase, KIT D816V mutation, and patient-reported outcome measures (PROMs) over the course of 12 weeks. Comorbid depression and anxiety were explored with the Hospital Anxiety and Depression Scale (HADS). RESULTS: In 53% of our patients (80% females), suspicion of MCAS was based on self-evaluation. In total, patients reported 87 different symptoms, mostly fatigue (n = 57), musculoskeletal pain/weakness (n = 49), and abdominal pain (n = 43), with overall high disease activity and impact. Two of 79 patients had increased tryptase (by >20% +2 ng/ml) following an episode. Only 5%, with any of the PROMs used, showed complete response to MC-targeted treatment. Depression and anxiety disorders were frequent comorbidities (n = 23 each), and 65 patients had pathological HADS values, which were linked to high disease impact and poor symptom control. CONCLUSION: Mast cell activation syndrome was confirmed in only 2% of patients, which implies that it is not MC activation that drives signs and symptoms in most patients with suspected MCAS. There is a high need for comprehensive research efforts aimed at the identification of the true underlying pathomechanism(s) in patients with suspected MCAS.

Anaphylaxis in children and adolescents: The Portuguese Anaphylaxis Registry.

BACKGROUND: Anaphylaxis is increasing at pediatric age; however, its characterization is hampered by underdiagnosis and underreporting. The aim of this study was to identify the causes of anaphylaxis in children and adolescents in Portugal, thus contributing to a better knowledge of its etiology, clinical manifestations, and management. METHODS: During a 10-year period, a nationwide notification system for anaphylaxis was implemented, with voluntary reporting by allergists. Data on 533 patients under 18 years of age with anaphylaxis were included. RESULTS: Mean age was 8.5 ± 4.9 years, 61% were male; 45% had asthma. Mean age at the first anaphylaxis episode was 5.3 ± 4.7 years (ranging from 1 month to 17 years of age), 63% at pre-school age. Most reactions occurred at home (57%). Food-induced anaphylaxis was the leading cause (77%). The main culprit foods were cow's milk (32%), tree nuts (16%), shellfish (13%), egg (12%), fresh fruits (11%), fish (8%), and peanut (8%). Other causes included drugs (11%), insect sting (5%), cold-induced anaphylaxis (4%), exercise-induced anaphylaxis (2%), latex (1%), and idiopathic anaphylaxis (1%). Most patients (83%) were admitted to the emergency department; only 46% received adrenaline treatment. Recurrence of anaphylaxis occurred in 41% of the patients (3 or more episodes in 21%). An adrenaline autoinjector was used in 9% of the patients. CONCLUSIONS: In the Portuguese pediatric population, food is the leading cause of anaphylaxis. Undertreatment with adrenaline and high recurrence of anaphylaxis highlight the need to improve both the diagnosis and the therapeutic management of this life-threatening entity.

How to Treat Patients with Chronic Spontaneous Urticaria with Omalizumab: Questions and Answers.

Omalizumab is an effective treatment for patients with chronic spontaneous urticaria (CSU). In routine clinical practice, physicians often face complex cases of CSU and need to decide whether patients are suitable for omalizumab treatment and how to manage this therapy. Here, we provide evidence and experience-based answers to the most common and important questions on omalizumab treatment of CSU. At 4 large urticaria centers, questions on the use of omalizumab in the treatment of patients with CSU were collected and then ranked by frequency and importance and grouped into top 5 domains using an interactive consensus approach. We suggest that omalizumab can be used to treat patients with CSU with any of the 3 CSU phenotypes (wheals only, angioedema only, wheals and angioedema), with comorbid chronic inducible urticaria, with cancer, who receive other biologics or cyclosporine, or who are pregnant or want to become pregnant, or are breast-feeding. Omalizumab treatment should be started with 300 mg every 4 weeks, monitored with validated patient-reported outcome measures, and maintained, in responders, until remission of CSU. Finally, partial responders or non responders can benefit from omalizumab updosing or adding or switching to cyclosporine. We believe our suggestions on the use of omalizumab in CSU will help to inform clinical decision making. Follow-up efforts should increase, systematically review, and profile the data available and provide evidence-based recommendations on how to best use omalizumab in CSU.

Multiple drug hypersensitivity syndrome to antituberculosis drugs: a case report

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The effect of inspiratory muscle training on swimming performance, inspiratory muscle strength, lung function, and perceived breathlessness in elite swimmers: a randomized controlled trial.

BACKGROUND: According to studies performed on terrestrial sports athletes, inspiratory muscle training (IMT) may improve athletes' performance. However, evidence of its effects in elite swimmers is lacking. Therefore, we aimed to assess the effect of 12-week IMT on swimming performance, inspiratory muscle strength, lung function, and perceived breathlessness in elite swimmers. METHODS: Elite swimmers from the main FC Porto swimming team (in competitive training for a minimum period of 3 years) were invited to participate and were randomly allocated into intervention or control groups. The intervention group performed 30 inspiratory efforts, twice a day, 5 times a week, against a pressure threshold load equivalent to 50% of maximal inspiratory pressure, whereas the control group performed inspiratory efforts at the same frequency but against a 15% load. Swimming performance was assessed through time trials, converted into points according to International Swimming Federation Points Table. Outcomes were evaluated before and following the 12-week study period. RESULTS: A total of 32 participants (22 girls) were included. The median age was 15 and 14 years old for the intervention (n = 17) and control (n = 12) groups, respectively. No differences were found in swimming performance (P = .271), inspiratory muscle strength (P = .914), forced vital capacity (P = .262), forced expiratory volume in 1st second (P = .265), peak expiratory flow (P = .270), and perceived breathlessness (P = .568) between groups after 12 weeks of intervention. CONCLUSION: Twelve weeks of IMT had no effect on swimming performance, lung function, and perceived breathlessness in elite swimmers. These results may be related to swimming-specific factors and/or an applied load insufficient to achieve training overload that could induce further improvements.

Reasons for Declining Venom Immunotherapy.

INTRODUCTION: Hymenoptera venom allergy is associated with significant morbidity and deterioration in health-related quality of life, and risk of fatal systemic reactions. Although venom immunotherapy is safe and the only effective treatment in allergic individuals, some patients prefer not to pursue this treatment. Since 2011, when the 50% reimbursement was stopped, patients must fully support the cost of immunotherapy. This study aimed to ascertain the reasons why patients decline immunotherapy. MATERIAL AND METHODS: A medical records review of all patients proposed to receive venom immunotherapy at an Allergy and Clinical Immunology Department in Porto, Portugal, between 2006 and 2015, followed by a phone interview to patients refusing treatment. RESULTS: A total of 83 subjects were enrolled, with a mean (± SD) age of 44.4 (14.7) years and 55 (66%) males; 27 refused venom immunotherapy between 2006 and 2015. Nineteen were interviewed and 14 of those stated price as the main reason for declining treatment. The only identified risk factor associated with immunotherapy refusal was being proposed after 2011 (OR: 3.29; 95% CI: 1.12 - 9.68; p = 0.03). DISCUSSION: The number of patients refusing venom immunotherapy doubled since reimbursement was withdrawn. Price was identified as the major obstacle to treatment completion. Immunotherapy proposal after reimbursement was stopped was associated with a 3-fold increase in the risk of refusing treatment. CONCLUSION: These findings show how economic decisions may have a detrimental effect on patient care, as immunotherapy refusal left them exposed to an avoidable life-threatening risk.

Introdução: A alergia a veneno de himenópteros está associada a uma significativa morbilidade e diminuição da qualidade de vida, bem como a risco de reações alérgicas fatais. Apesar da imunoterapia com veneno de himenópteros ser um tratamento seguro e o único eficaz nesta patologia, alguns doentes decidem não o realizar. Desde 2011, quando a comparticipação de 50% terminou, o custo da imunoterapia é totalmente suportado pelos doentes. Este trabalho pretendeu identificar os motivos da recusa desta terapêutica. Material e Métodos: Revisão dos registos clínicos de todos os doentes propostos para imunoterapia com veneno de himenópteros num serviço de Imunoalergologia, no período 2006 - 2015, seguida de entrevista telefónica aos que a recusaram. Resultados: Foram incluídos 83 doentes, com uma idade média (± DP) de 44,4 (14,7) anos. Cinquenta e cinco (66%) eram homens; 27 recusaram imunoterapia entre 2006 e 2015. Dezanove foram entrevistados e 14 identificaram o preço como principal motivo de recusa. O único fator de risco identificado para a recusa de imunoterapia foi ser proposta depois de 2011 (OR: 3,29; 95% CI: 1,12 ­ 9,68; p = 0,03). Discussão: O número de doentes a recusar imunoterapia duplicou desde que a comparticipação foi retirada. O preço foi o principal obstáculo à realização do tratamento. Ser proposto após o término da comparticipação do tratamento aumentou em três vezes o risco de recusa. Conclusão: Estes achados revelam o impacto negativo de uma decisão económica na saúde e segurança destes doentes, já que a recusa da imunoterapia os manteve expostos a um risco de vida evitável.