Alpha-gal syndrome initially misdiagnosed as chronic spontaneous urticaria in a pediatric patient: a case report and review of the literature.

INTRODUCTION: Delayed allergy to red meat, also termed alpha-gal syndrome, is increasingly reported in adults and African communities, while pediatric cases remain rare. CASE PRESENTATION: Here, we report on a 7-year-old Caucasian boy presenting with recurrent wheals since the age of 5 years old. Episodes with hives occurred around every 3 weeks, mainly in the evening. One of these episodes was also associated with angioedema. No clear trigger was identified. At the first visit, after excluding an infection and autoimmune thyroiditis, chronic spontaneous urticaria was suspected and symptomatic treatment with antihistamines was prescribed. Six months later, the boy presented at the emergency room with generalized urticaria, dyspnoea, and emesis. Symptoms resolved after administration of epinephrine and antihistamines. A detailed medical history after this event revealed that he had eaten three sausages as well as jelly beans containing gelatine several hours prior to this episode. More precisely, after eating the sausages and jelly beans during the day, he had shown some hives before going to bed, and later developed the other symptoms in the middle of the night, suggesting alpha-gal syndrome. In his history, several tick bites are reported. Immunoglobulin E levels for alpha-gal were clearly elevated, confirming the diagnosis of a delayed-appearing immunoglobulin E-mediated allergic reaction to alpha-gal. Emergency medication was prescribed and avoidance of red meat and gelatine-containing foods was recommended. Under this exclusion diet, the boy remained asymptomatic, with the exception of two accidents in the follow up of 3 years, one developing during a barbecue and the second after exceptionally eating marshmallows. CONCLUSION: A detailed clinical history led to the diagnosis of alpha-gal syndrome. Although alpha-gal syndrome is typically seen in adults, our case illustrates that children can also present with this potentially life-threatening allergy. Since alpha-gal syndrome is rare in Europe, the disease is not well known and often overlooked for several years, especially in children.

Chronic pain concepts of pediatricians: a qualitative survey.

Introduction: Chronic pain is a prevalent, yet underrecognized, condition in children and adolescents. A biopsychosocial framework has been widely adopted over the past decades and resulted in a new pain classification in the International Classification of Diseases, 11th revision (ICD-11). Nevertheless, little is known about pediatricians' pain concepts. Objectives: We explored pain concepts of Swiss pediatricians by means of a qualitative analysis. Method: A cross-sectional online survey was sent to clinically active Swiss pediatricians registered with the Swiss Society for Pediatrics. A case vignette of a girl with chronic musculoskeletal pain was presented and pediatricians were asked (1) what they think caused the pain, and (2) how they would explain the pain to the patient and their family. Structuring content analysis was applied to describe major themes within the answers. Results: The following main categories emerged: psychological factors, biological factors, unclear etiology, social context, disorder specific, and multifactorial. Most pediatricians reported the belief that psychological factors explained the pain. However, when explaining the pain to the patient, biological factors were reported most often. Conclusion: There is a discrepancy between pediatricians' conviction that chronic pain is mostly explained by psychological factors and their exploratory model towards patients that focuses on biological factors. Promoting the biopsychosocial framework of chronic pain is key to ensure timely and effective treatment. The new pain classification in the ICD-11 has the potential to increase the use of the biopsychosocial model.

[Pediatric chronic pain : interprofessional and integrative care]. / Douleurs chroniques en pédiatrie : une approche interprofessionnelle et intégrative.

Pediatric chronic pain is an increasingly recognized condition in children and adolescents. Current treatment is based on the bio-psycho-social model of chronic pain: an interprofessional and integrative team will help the young patient and his environment, in order to find a balance between physical, psychological and social impacts. This article focuses on different treatment approaches for pediatric chronic pain in the outpatient and inpatient setting.

Les douleurs chroniques sont de plus en plus reconnues chez les enfants et adolescents. L'approche thérapeutique se base sur la compréhension du modèle biopsychosocial de la douleur chronique : une collaboration interprofessionnelle et intégrative est alors nécessaire avec le jeune patient et son environnement, afin de trouver ensemble un équilibre avec les répercussions physiques, psychologiques et sociales liées à la douleur. Cet article vise à montrer comment intégrer avec succès différentes approches.

Serious adverse events in children with juvenile idiopathic arthritis and other rheumatic diseases on tocilizumab - a real-world experience.

OBJECTIVES: To assess the incidence rate and type of serious adverse events (SAE) in children with rheumatic inflammatory diseases treated with the interleukin 6 blocker tocilizumab (TCZ). METHODS: A retrospective review of all consecutive patients diagnosed with an inflammatory rheumatic disease and receiving at least one dose of TCZ was performed in two French tertiary pediatric rheumatology centers between 01/2007 and 06/2019. SAE were defined as a life-threatening event and/or an event requiring hospital admission, leading to permanent disability or treatment discontinuation. RESULTS: One hundred four children (64 female) were included. Most children suffered from systemic (n = 43) or polyarticular-course juvenile idiopathic arthritis (n = 43). Median age at TCZ start was 8.9 years (IQR 4.7 - 12.1), most children had received prednisone (81%), and/or a biologic agent (84%) prior to TCZ. Median TCZ treatment duration was 1.6 years (IQR 0.5 - 2.7), total TCZ exposure 215 patient years. Thirty-three SAE were observed in 26 (25%) children (SAE 15.3/100 patient years), mostly infections and infusion reactions. Children with SAE were significantly younger at disease onset (p = 0.034) and TCZ initiation (p = 0.016). Children experiencing infusion reactions were more likely to have systemic JIA or another autoinflammatory disease (p = 0.021), they all had active disease. At last follow up, 61 (59%) children remained on TCZ. CONCLUSION: In this cohort, SAE and most commonly serious infections were observed in a quarter of children. Severe infusion reactions were associated with persistently active autoinflammatory disease. Ongoing careful monitoring of TCZ-treated patients, especially young children with marked systemic inflammation is required.

Characteristics of children and adolescents at the Switzerland-wide first ambulatory interdisciplinary pain clinic at the University Children's Hospital Basel - a retrospective study.

AIMS OF THE STUDY: Given the long history of underestimating chronic pain in children and adolescents, we lack valid data on its assessment and treatment. The psychological and economic burden for patients, their families and society is substantial. The aim of this study was to assess patient characteristics of the first ambulatory interdisciplinary clinic for children and adolescents with chronic pain in Switzerland and compare them with data from other international centres. METHODS: All patients of the ambulatory interdisciplinary pain clinic at the University Children’s Hospital in Basel during the period from 4 January 2012 to 4 July 2016 were included in this retrospective study. Data were collected from the patients’ medical records and from a questionnaire, which the patients and their parents received and completed in advance of their first visit. Demographic information, pain, referral, social environment, therapies and school absences of the patients were statistically analysed with means, percentages, 95% confidence intervals (CIs) and standard deviations (SDs). RESULTS: Of the 135 patients included in this study, 80% were female and the mean age of all patients was 13.95 years (95% CI 13.5–14.4). The commonest pain presentations were: musculoskeletal (38%, 95% CI 0.30–0.46), back (25%, 95% CI 0.18–0.33), multiple regions (21%, 95% CI 0.15–0.28) and headache (7%, 95% CI 0.03–0.12). Mean duration of pain until the patients came to the clinic was 24.5 months (95% CI 19.82–29.22). Physiotherapy (71%, 95% CI 0.63–0.79) and non-opioids (50%, 95% CI 0.42–0.59) were the most used therapies before the first meeting. Psychotherapy (52%, 95% CI 0.44–0.61), most often using a psychosomatic therapeutic approach (psychosomatic therapy) 34% (95% CI 0.26–0.42), physiotherapy (36%, 95% CI 0.27–0.44) and non-opioids (33%, 95% CI 0.25–0.42) afterwards. The mean number of school absences during the last month before the first visit was 5.1 days per month (95% CI 3.48–6.73). The parents of our study participants suffered more often from psychiatric diseases than the mean Swiss population. CONCLUSIONS: The average of more than 2 years of pain before referral to the clinic seems to be a long time. Assuming that specialised support is mandatory for young patients with complex pain syndromes, the referral time should be reduced. Furthermore, patients with headache were underrepresented in Basel compared with other centres. Interestingly, in our study, patients’ parents suffered more often from psychiatric diseases than the mean Swiss population.

[Treatment of psychosomatic disorders occurring in the vocational context - concept and first findings ]. / Behandlung psychosomatischer Beschwerden im Arbeitskontext - Konzept und erste Daten.

OBJECTIVES: Psychosomatic symptoms often present within the vocational context. The unmet need for easily accessible and early interventions led to the development of a variety of offers in this area. From a scientific point of view, the question of how such a "psychosomatic consultation in the workplace" (PCIW) is best conceptualized remains open. METHODS: We analysed treatment documentation for all patients of two recently established PCIW from January 2011 to January 2012 both descriptively and by qualitative content analysis. RESULTS: A total of 67 patients were seen, 75% of whom were male. For 75% of users PCIW constituted the first contact with psychosomatic-psychotherapeutic-psychiatric services. For about 80%of the patients a work-related aetiology could be considered. 40%of patients were recommended to outpatient psychotherapeutic care. CONCLUSIONS: PCIW represents an easily accessible therapeutic offer in the vocational context. There often is a mix of work-related and personal problems that suggests the cooperation of occupational and psychosomatic physicians.

Solid-state NMR and functional studies on proteorhodopsin.

Proteins of the proteorhodopsin (PR) family are found abundantly in many marine bacteria in the photic zone of the oceans. They are colour-tuned to their environment. The green absorbing species has been shown to act as a light-driven proton pump and thus could form a potential source of energy. The pK(a) of the primary proton acceptor is close to the pH of seawater which could also indicate a regulatory role. Here, we review and summarize our own recent findings in the context of known data and present some new results. Proton transfer in vitro by PR is shown by a fluorescence assay which confirms a pH dependent vectoriality. Previously reported low diffracting 2D crystal preparations of PR are assessed for their use for solid-state NMR by two dimensional (13)C-(13)C DARR spectra. (15)N-(1)H HETCOR MAS NMR experiments show bound water in the vicinity of the protonated Schiff base which could play a role in proton transfer. The effect of highly conserved H75 onto the properties of the chromophore has been investigated by single site mutations. They do show a pronounced effect onto the optical absorption maximum and the pK(a) of the proton acceptor but have only a small effect onto the (15)N chemical shifts of the protonated Schiff base.