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Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was given US Food and Drug Administration approval based on its therapeutic benefits to treat patients with cystic fibrosis (CF) who had at least 1 allele of the CF transmembrane conductance regulator (CFTR) with phenylalanine deleted at position 508 (F508del). The increase in genotyping studies has increased the frequency of use of CFTR modulators; however, severe allergic reactions to CFTR modulators have also been described. It is critical to avoid the offending medication and select alternative treatments while dealing with drug allergies. Drug desensitization may be taken into consideration in situations where there is no other option. This article describes home desensitization treatment for a patient with CF who developed a maculopapular rash following CFTR modulator medication. There are currently no alternative drugs for CFTR modulators, which are crucial for patients with CF, and limited experience is available with allergic reactions to these drugs. It is important to establish desensitization protocols in order to control drug reactions to CFTR modulators, which are vital for individuals with CF., Competing Interests: Disclosure. The authors declare that there are no conflict of interests. No funding. The authors had full access to all patient information in this report and take responsibility for the integrity and accuracy of the report., (Copyright. Pediatric Pharmacy Association. All rights reserved. For permissions, email: membership@pediatricpharmacy.org.)

Background: Quality of life, which is impaired in patients with chronic spontaneous urticaria (CSU), is influenced by comorbid mental disorders. Headaches could be another comorbid mental disorder that affects quality of life in children with CSU., Objectives: To investigate the effect of headaches on urticaria symptoms, disease activity and quality of life in children with CSU., Methods: A total of 83 patients with CSU were enrolled in the study and were separated into two groups as those with or without headache. Demographic and clinical characteristics were studied with the Urticaria Activity Score (UAS7), Urticaria Control test (UCT) and Chronic Urticaria Quality of Life Questionnaire (CU-Q2QoL). The headache questionnaire designed according to the Department of International Classification of Headache Disorders, second edition (ICHD-II) was used and VAS (Visual Analogue Scale) and NRS (Numerical Rating Scale) were used to assess the pain measurement. In patients diagnosed with migraine, the paediatric Migraine Disability Assessment Scale (PedMIDAS) was applied., Results: CU-QoL total scores were significantly higher in patients with CSU with headache than in those without headache (p=0.015). In the five domains of CU-QoL, impact of daily life activities domain and sleep problems domain had higher scores in CSU with headache (p=0.008, 0.028, respectively). There was no significant relationship between UCT, UAS and CU-QoL and headache severity (p<0.05). No differences were found between the groups in respect of duration of urticaria, UAS7 and UCT., Conclusion: Headache may be an important factor that affects and impairs quality of life in children with chronic urticaria., (Copyright © 2018 SEICAP. Published by Elsevier España, S.L.U. All rights reserved.)

We explored the clinical course of acute high-grade gastrointestinal graft-versus-host disease in children in a single center. This was a retrospective analysis of 28 pediatric patients who presented with a clinical diagnosis of stage III and IV acute graft-versus-host disease (aGVHD) of the gastrointestinal system (GIS). Generally, skin involvement was the initial manifestation of aGVHD that began in the first 3 weeks of hematopoietic stem cell transplantation (HSCT); on the other hand, GIS involvement predominated after the second week of HSCT. Reported adult data show a survival rate of only 25%; however, our study showed more favorable outcomes in children with a survival rate of 55%. We monitored levels of albumin and immunoglobulin G and observed low levels overall during treatment of unresponsive patients, although only albumin levels were shown to be significantly different. We observed a significant increase in mortality with the use of antithymocyte globulin in GIS aGVHD, although antithymocyte globulin used for graft-versus-host disease prophylaxis had no demonstrable effect on GIS aGVHD mortality. Whether the significantly lower GIS aGVHD mortality among the children recruited in our study than among their historical adult counterparts is a primary result of the specific attributes of the pediatric GIS, or whether it originated from HSCT kinetics remains to be determined by future studies.