Sputum induction is feasible and safe in a community setting. Sputum induction was useful for making a microbiological diagnosis, increasing the number of children diagnosed and treated for PTB.
Multiple myeloma rarely presents with a fever of unknown origin and diagnosis may be delayed. We describe a case of myeloma presenting in this way with raised serum-free light chains and TP53 deletion on cytogenetics. The patient developed thrombotic thrombocytopenia purpura (TTP) following bortezomib therapy but recovered spontaneously and was successfully re-challenged. We believe this is only the second case to describe this phenomenon post-bortezomib and the first to rechallenge the patient successfully without further recurrence of TTP. Possible mechanisms for this successful rechallenge are discussed.
Background and Aims:
The incidence and prevalence of eosinophilic esophagitis (EoE) and inflammatory bowel disease (IBD) are rising with similar patterns. Co-occurrence of both diseases in the same patient has been increasingly reported. We sought to examine the pediatric population with both EoE and IBD to better understand the epidemiology and clinical implications of this overlap.
Methods:
We conducted a retrospective case-control study at 2 tertiary care children's hospitals. Subjects with both EoE and IBD were identified and compared with randomly selected controls with EoE and IBD alone in terms of: demographics, atopic conditions, IBD classification, location and phenotype of Crohn disease (CD), IBD medications, endoscopic findings, and histopathology. Descriptive statistics summarized the data.
Results:
Sixty-seven subjects with dual-diagnosis were identified across both institutions. The prevalence of IBD in the EoE population was 2.2% and EoE in IBD was 1.5%. Subjects with both diseases were more likely to have IgE-mediated food allergy compared with IBD alone (36% vs 7%, P < 0.001). Subjects with CD-EoE were less likely to have perianal disease than CD alone (2% vs 20%, P = 0.004). There was no difference in fibrostenotic EoE between the dual-diagnosis group and EoE alone. Treatment with a TNF-alpha inhibitor (anti-TNF) for management of preexisting IBD was protective against development of EoE with a relative risk of 0.314 [95% confidence interval [CI] 0.159-0.619].
Conclusions:
This is a unique population in whom the underlying pathway leading to dual-diagnosis is unclear. Concomitant atopic conditions, especially IgE-mediated food allergy, and medication exposures, particularly anti-TNFs, may help predict likelihood of developing dual-diagnosis.
Objectives: Tofacitinib, a selective Janus kinase inhibitor, effectively induces and maintains remission in adults with inflammatory bowel disease (IBD), but data are limited in children. This study aimed to evaluate the efficacy and safety of tofacitinib for medically refractory pediatric-onset IBD. Methods: This single-center retrospective study included subjects ages 21 years and younger who started tofacitinib for medically refractory IBD. Clinical activity indices, clinical response, steroid-free remission, biochemical response, and adverse events (AEs) were evaluated over 52 weeks.Results: Twenty-one subjects, 18 with ulcerative colitis or indeterminate IBD, received tofacitinib. At the end of the 12-week induction period, 9 out of 21 (42.9%) subjects showed clinical response and 7 out of 21 (33.3%) were in steroid-free remission. Of evaluable subjects at 52 weeks, 7 out of 17 (41.2%) showed clinical response and were in steroid-free remission. Of those remaining on tofacitinib at 1 year, none required concomitant systemic corticosteroids. Tofacitinib was discontinued in 8 subjects because of refractory disease, including 8 who ultimately underwent colectomy, and in 1 subject who developed a sterile intra-abdominal abscess. There were no instances of thrombi, zoster reactivation, or clinically significant hyperlipidemia, all of which were AEs of interest. Conclusions: There is limited experience with tofacitinib in pediatric IBD. In this cohort, tofacitinib induced rapid clinical response with sustained efficacy in nearly half of subjects. This study provides encouraging evidence for the efficacy and safety of tofacitinib as part of the treatment paradigm for young individuals with moderate-to-severe IBD. Larger, well-powered, prospective studies are warranted.
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