scholarly journals Granulomatous Inflammation Causing Severe Supraglottic Edema and Airway Obstruction in a Pediatric Patient

2020 ◽  
pp. 014556132097377
Author(s):  
Sophia M. Colevas ◽  
Bradley T. Gietman ◽  
Shelly M. Cook ◽  
Tony L. Kille

A 12-year-old male with a family history of inflammatory bowel disease presented with sleep-disordered breathing and was found to have chronic, granulomatous swelling of the supraglottic larynx. His airway was managed with tracheostomy, regular interval laryngeal steroid injections, supraglottoplasty, and “pepper pot” CO2 laser resurfacing leading to eventual decannulation. Due to the non-necrotic nature of the granulomatous inflammation, as well as the patient’s family history of inflammatory bowel disease, the leading diagnosis was Crohn disease, but isolated laryngeal sarcoidosis could not be ruled out. There are only 13 reported cases of laryngeal manifestations of Crohn disease in the literature, with only 2 cases occurring in pediatric patients. This case report adds to this body of literature and discusses strategies for managing granulomatous supraglottic edema when definitive diagnosis is not fully clear.

2013 ◽  
Vol 144 (5) ◽  
pp. S-649-S-650
Author(s):  
Ryan E. Childers ◽  
Swathi Eluri ◽  
Christine Vazquez ◽  
Theodore M. Bayless ◽  
Susan Hutfless

Gut ◽  
1999 ◽  
Vol 44 (1) ◽  
pp. 91-95 ◽  
Author(s):  
F Carbonnel ◽  
G Macaigne ◽  
L Beaugerie ◽  
J P Gendre ◽  
J Cosnes

BackgroundHaving a relative with inflammatory bowel disease increases the risk for Crohn’s disease but may also increase its severity in affected patients.AimsTo evaluate the influence of a family history on Crohn’s disease course and severity.Methods1316 patients followed in the same unit were studied retrospectively. Age at onset, duration of illness, site, and extent of disease were determined in patients with and without a family history. Additionally, disease severity was estimated by the need for medical therapy (steroid and immunosuppressive requirement) and the frequency and extent of excisional surgery.Results152 (12%) patients had a family history of inflammatory bowel disease. Duration of follow up was longer in patients with a family history and there were more operations for perforating complications in familial cases. However, the importance of medical therapy, and the incidence and extent of excisional surgery were similar in familial and and sporadic cases. Kaplan-Meier estimated time to prescription of immunosuppressive drugs and first intestinal resection were similar in familial and sporadic cases. When the 152 patients with familial Crohn’s disease were paired for sex, location of disease at onset, date of birth, and date of diagnosis with 152 patients with sporadic Crohn’s disease, the disease severity remained similar in the two groups of paired patients.ConclusionPatients with Crohn’s disease and a family history of inflammatory bowel disease do not have a more severe course.


Author(s):  
Abigail Garrity ◽  
Trudy Lerer ◽  
Anthony Otley ◽  
James Markowitz ◽  
Anne Griffiths ◽  
...  

2014 ◽  
Vol 8 (11) ◽  
pp. 1480-1497 ◽  
Author(s):  
Ryan E. Childers ◽  
Swathi Eluri ◽  
Christine Vazquez ◽  
Rayna Matsuno Weise ◽  
Theodore M. Bayless ◽  
...  

2003 ◽  
Vol 124 (4) ◽  
pp. A212
Author(s):  
Suryakanth R. Gurudu ◽  
Layla Hejjafar ◽  
Pankaj Singh ◽  
Jeffry A. Katz

2021 ◽  
Author(s):  
Maya Ruban ◽  
Adam Slavick ◽  
Achiya Amir ◽  
Amir Ben-Tov ◽  
Hadar Moran-Lev ◽  
...  

Abstract The role of a positive family history in pediatric inflammatory bowel disease (IBD) in the era of biologic therapy has not been elucidated. We retrospectively reviewed the medical records of children with IBD and retrieved demographic and clinical characteristics, including the presence of a positive family history of IBD, IBD phenotype, disease course, and therapy. Overall, 325 children (age range at diagnosis 11-15 years) were included, of whom 82 (25.2%) had a positive family history. Children diagnosed during 2016-2020 had a higher frequency of positive family history compared to those diagnosed during 2010-2015 (31.8% versus 20.7%, respectively, p = 0.024). Children with a positive family history had a higher risk for a stricturing phenotype than those with a negative family history (11.3% versus 2.8%, respectively, p = 0.052). They more often received nutritional therapy (53.7% versus 36.6%, p = 0.007) and less often received corticosteroids (36.6% versus 52.7%, p = 0.012). More children with a negative family history needed intensification of biologic therapy (p = 0.041). Conclusion: The rate of a positive family history of IBD in the pediatric IBD population is increasing. A positive family history may have some impact upon IBD phenotype but none on IBD outcome.


2021 ◽  
Vol 15 (Supplement_1) ◽  
pp. S591-S591
Author(s):  
E A Torres ◽  
L Ramos ◽  
A Sanchez ◽  
C Amaya ◽  
A Perez-Gilbe

Abstract Background Inflammatory Bowel Disease (IBD) in Hispanics has increased, but characterization of this population is limited. We describe the demographic and clinical characteristics of a large Hispanic population with IBD and compare it among two periods, 1995- 2009 and 2010–2019. Methods The Registry of IBD has been recruiting patients with IBD continuously since 1995. Data is obtained from the subject and the medical record. This study includes 1365 Hispanics recruited between 1995 and 2019. Variables include age, gender, age at onset and diagnosis, IBD type, family history, smoking, extraintestinal manifestations (EIM), medications, and surgery for IBD. Descriptive statistics included frequency, median, mean, and standard deviation. SPSS software was used for comparison analysis utilizing Chi square and Fisher′s test. The protocol is approved by the IRB. Results 712 were males and 653 females. Crohn’s disease (CD) was more prevalent in males (479/836, 57.3%) and ulcerative colitis (UC) in females (288/517, 55.7%). The mean age at diagnosis was 34.1 + 15.4 for UC and 24.4 + 12 for CD (p<.001). History of smoking was infrequent (24.3%). Interval between onset of symptoms and diagnosis was 1.8 + 4.7 yrs. for UC and 2.5 + 5.3 yrs. for CD (p=.012). At recruitment, duration of disease was 7.4 + 8.4 yrs. for UC and 5.6 + 7.3 yrs. for CD (p<.001). Family history of IBD was present in 23% of CD and UC participants. The most frequent EIM was arthropathy in 37.9% and 25.9 % of UC and CD (p=.670), followed by skin manifestations in 13.2% and 18.9% respectively (p=.070). Aminosalicylates (94.9%) and corticosteroids (81.6%) were more frequent in UC, and immunomodulators (23%) and anti-TNF drugs (aTNF) (46.2%) in CD (p<.001). At the time of recruitment, 54.5% of CD and 23.7% of UC patients had previous surgery for IBD. Stratification of subjects into two groups by date of recruitment,1995–2009 and 2010–2019, showed similar ages at onset and diagnosis, but the time to diagnosis decreased for UC (2 vs 1.55 yrs.) and increased for CD (2.2 vs 2.6 yrs.) in the later interval. Medications varied between decades, with aTNF increasing markedly in CD and UC (p<.001) and aminosalicylates decreasing in CD (p<.001) in the later years. Surgery for UC decreased from 25.2% to 20.7%, whereas surgery for CD remained the same (52.2% vs 52.5%). Conclusion We describe a large cohort of Hispanics with IBD studied over two decades. Differences over time may reflect changes in disease phenotypes, environmental influences and the impact of physician awareness and new management guidelines and therapies. Further studies are needed to better characterize this population and explore outcomes.


Rheumatology ◽  
2021 ◽  
Author(s):  
Joeri W van Straalen ◽  
Roline M Krol ◽  
Gabriella Giancane ◽  
Violeta Panaviene ◽  
Laura Marinela Ailioaie ◽  
...  

Abstract Objectives To describe risk factors for inflammatory bowel disease (IBD) development in a cohort of children with juvenile idiopathic arthritis (JIA). Methods JIA patients who developed IBD were identified from the international Pharmachild register. Characteristics were compared between IBD and non-IBD patients and predictors of IBD were determined using multivariable logistic regression analysis. Incidence rates of IBD events on different disease-modifying anti-rheumatic drugs (DMARDs) were calculated, differences between therapies were expressed as relative risks (RR). Results Out of 8,942 patients, 48 (0.05%) developed IBD. These were more often male (47.9% vs 32.0%) and HLA-B27 positive (38.2% vs 21.0%) and older at JIA onset (median 8.94 vs 5.33 years) than patients without IBD development. They also had more often a family history of autoimmune disease (42.6% vs 24.4%) and enthesitis-related arthritis (ERA) (39.6% vs 10.8%). The strongest predictors of IBD on multivariable analysis were ERA (OR: 3.68, 95% CI: 1.41–9.40) and a family history of autoimmune disease (OR: 2.27, 95% CI: 1.12–4.54). Compared with methotrexate monotherapy, the incidence of IBD on etanercept monotherapy (RR: 7.69, 95% CI: 1.99–29.74), etanercept with methotrexate (RR: 5.70, 95% CI: 1.42–22.77) and infliximab (RR: 7.61, 95% CI: 1.27–45.57) therapy was significantly higher. Incidence on adalimumab was not significantly different (RR: 1.45, 95% CI: 0.15–13.89). Conclusion IBD in JIA was associated with ERA and a family history of autoimmune disease. An increased IBD incidence was observed for etanercept therapy regardless of concomitant methotrexate use.


Angiology ◽  
2017 ◽  
Vol 68 (10) ◽  
pp. 845-849 ◽  
Author(s):  
Grigorios Tsigkas ◽  
Periklis Davlouros ◽  
Stefanos Despotopoulos ◽  
Stelios F. Assimakopoulos ◽  
Georgios Theocharis ◽  
...  

Patients with inflammatory bowel disease (IBD) have a higher incidence of coronary artery disease (CAD) compared with the general population. Left main coronary artery (LMCA) thrombosis constitutes a very rare but catastrophic manifestation of acute coronary syndrome. Case reports describing young patients with IBD and LMCA thrombosis are scarce. Most importantly, patients with a positive family history of thrombotic events and those with significant genetic or acquired risk factors such as the antiphospholipid antibody syndrome, advanced age, immobilization, pregnancy, oral contraceptive use, obesity, diabetes, and cigarette smoking may have a higher risk of thrombosis among those with active IBD. We describe a 28-year-old man who was admitted for coronary angiography (CA) due to ST-segment elevation myocardial infarction. He had a recent exacerbation of ulcerative colitis. The patient was a smoker without a family history of CAD. Proximal total occlusion of the left anterior descending (LAD) artery and left circumflex (LCX) artery with massive thrombus was shown on CA, whereas a normal dominant right coronary artery delivered collaterals to the LAD artery.


Author(s):  
R. Mark Beattie ◽  
Anil Dhawan ◽  
John W.L. Puntis

Clinical presentations 310Investigation 311Clinical course 311Management 31225% of inflammatory bowel disease presents in childhood, 1/3 as ulcerative colitis. Presentation can occur at any age and ulcerative colitis is the commonest cause of inflammatory bowel disease in the younger child. Family history of Crohn's disease or ulcerative colitis is common in index cases....


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