Severe ileocecal inflammatory syndrome in adult patients with cystic fibrosis

2019 ◽  
Vol 57 (03) ◽  
pp. 312-316 ◽  
Author(s):  
Robert Henker ◽  
Annett Oltmanns ◽  
Alexandra Wald ◽  
Jan Tuennemann ◽  
Sabine Opitz ◽  
...  

AbstractThe relevance of gastrointestinal manifestations of cystic fibrosis (CF) is increasing due to an improved life expectancy. We report on 2 adult patients with prior lung transplantation who presented with a severe inflammatory disorder of the ileocecal region. One patient underwent ileocecal resection; the second patient died after emergency surgery for intestinal perforation. Both cases did not show typical signs of CF-related distal intestinal obstruction syndrome or extensive fibrosing colonopathy. However, the clinical and histopathological findings revealed CF-induced inflammatory alterations of the intestinal mucosa. Thus, these cases illustrate a further CF-related bowel disorder, which can be especially relevant in long-term CF survivors.

2021 ◽  
Vol 20 ◽  
pp. S68
Author(s):  
A.I. Yilmaz ◽  
G. Ünal ◽  
B.S. Kibar ◽  
P. Sevgi ◽  
O. Eĝil ◽  
...  

2013 ◽  
Vol 84 (10) ◽  
pp. 740-744 ◽  
Author(s):  
Rami Subhi ◽  
Rachel Ooi ◽  
Felicity Finlayson ◽  
Tom Kotsimbos ◽  
John Wilson ◽  
...  

2021 ◽  
Vol 57 (7) ◽  
pp. 501-503
Author(s):  
Antonio Álvarez ◽  
Karina Loor ◽  
Paula Fernández-Alvarez ◽  
Silvia Gartner ◽  
Eva Polverino ◽  
...  

Author(s):  
Mary Kathryn Bohn ◽  
Peter Yousef ◽  
Shannon Steele ◽  
Lusia Sepiashvili ◽  
Khosrow Adeli

Abstract Background Multi-inflammatory syndrome in children (MIS-C) is a novel and rare inflammatory disorder associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in school-age children. Reports in the past year have suggested a multi-system pathophysiology characterized by hyperinflammation, gastrointestinal distress, and cardiovascular complications. Clinical laboratory investigations, including routine blood testing for inflammatory (e.g., CRP, ferritin) and cardiac (e.g., troponin, brain natriuretic peptides) markers have provided insight into potential drivers of disease pathogenesis, highlighting the role of the laboratory in the differential diagnosis of patients presenting with similar conditions (e.g., Kawasaki Disease, Macrophage Activating Syndrome). Content While few studies have applied high-dimensional immune profiling to further characterize underlying MIS-C pathophysiology, much remains unknown regarding predisposing risk factors, etiology, and long-term impact of disease onset. The extent of autoimmune involvement is also unclear. In the current review, we summarize and critically evaluate available literature on potential pathogenic mechanisms underlying MIS-C onset and discuss the current and anticipated value of various laboratory testing paradigms in MIS-C diagnosis and monitoring. Summary From initial reports, it is clear that MIS-C has unique inflammatory signatures involving both adaptive and innate systems. Certain cytokines, inflammatory markers, and cardiac markers assist in the differentiation of MIS-C from other hyperinflammatory conditions. However, there are still major gaps in our understanding of MIS-C pathogenesis, including T cell, B cell, and innate response. It is essential that researchers not only continue to decipher initial pathogenesis but also monitor long-term health outcomes, particularly given observed presence of circulating autoantibodies with unknown impact.


2020 ◽  
Vol 96 (6) ◽  
pp. 732-740 ◽  
Author(s):  
Natascha S. Sandy ◽  
Lilian H.P. Massabki ◽  
Aline C. Gonçalves ◽  
Antonio F. Ribeiro ◽  
Jose D. Ribeiro ◽  
...  

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