scholarly journals Anticipation in an Indo-Canadian Family with Crohn's Disease

2001 ◽  
Vol 15 (10) ◽  
pp. 695-698 ◽  
Author(s):  
Hugh J Freeman ◽  
Noel B Hershfield
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Neurological Disorders ◽  
Molecular Basis ◽  
Present Report ◽  
Phenotypic Expression ◽  
Apparent Increase ◽  
The Family ◽  
Perianal Sepsis ◽  
Common Environmental Factor

Genetic anticipation, associated elsewhere with monogenic neurological disorders, has been hypothesized to be present in familial forms of Crohn's disease. Usually, with studies of parent-child pairs, the parent who is initially diagnosed is older at the onset of disease than the child. With each successive generation, an apparent increase in disease severity or behaviour occurs. This phenomenon is believed to have a molecular basis. In the present report, an Indo-Canadian family with Crohn's disease is described. In all members of the family, disease was diagnosed only after prolonged residence in Canada, supporting the view that Crohn's disease arises in individuals with a genetic predisposition following exposure to some, as yet unknown, common environmental factor. Three siblings with Crohn's disease, first diagnosed between ages 15 and 27 years, or six to 11 years after arrival in Canada, had phenotypically concordant disease localized in the ileum and colon, with fistulizing complications, including perianal sepsis. Crohn's disease was only diagnosed in the father at the age of 76 years, almost three decades after his arrival in Canada. His disease was localized to the ileum and had a fibrostenosing behaviour. This is the first reported instance of familial Crohn's disease in an immigrant population, illustrating potential biases in genetically based studies of Crohn's disease that rely solely on phenotypic expression.

Diving into inflammation: a pilot study exploring the dynamics of the immune-microbiota axis in ileal tissue layers of patients with Crohn’s disease

2021 ◽  
Author(s):  
Edda Russo ◽  
Francesco Giudici ◽  
Federica Ricci ◽  
Stefano Scaringi ◽  
Giulia Nannini ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Tissue Injury ◽  
Microbiota Composition ◽  
Response Distribution ◽  
Ileal Mucosa ◽  
The Family ◽  
New Biomarkers ◽  
Clinical Conditions ◽  
Ileal Tissue

Abstract Background and aims Crohn’s Disease (CD) pathogenesis is still unclear. Disorders in the mucosal immunoregulation and its crosstalk with the microbiota may represent an important component in tissue injury. We aimed to characterize the molecular immune response distribution within the ileal layers and to evaluate the correlated microbiota in pathological/healthy settings comparing first surgery/relapse clinical conditions. Methods We enrolled 12 CD patients. A comprehensive analysis of ileal mucosa, submucosa and serosa broad-spectrum cytokines’ panel was performed through a multiplex approach. In addition, ileal microbiota composition was assessed through Next Generation Sequencing. Results We observed a distinct profile (of IL1-α, IL-1β, IL-4, IL-8, ICAM-1, E-Selectin, P-Selectin, IP-10, IL 6, and IL 18) across the CD vs healthy ileal layers; and a different distribution of IFN-γ, P-Selectin, IL-27 and IL-21 in first surgery vs relapse patients. In addition, the phylum Tenericutes, the family of Ruminococcaceae, and the genus Mesoplasma and Mycoplasma were significantly enriched in pathological setting. Significant microbiota differences were observed between relapse vs first surgery patients regarding the class Bacteroidia, the genus of Prevotella, Flavobacterium, Tepidimonas and Escherichia/Shigella. Finally, the abundance of the genus Mycoplasma was positively correlated with IL-18. Conclusions We describe a dissimilarity of cytokines’ distribution and microbiota composition within the CD and the adjacent healthy ileal tissue layers and between first operation and surgical relapse. Our results give a potential insight into the dynamics of the gut microbiota-immune axis in CD patients, leading to new biomarkers’ detection.

Perianal Crohn's Disease in Children and Adolescents

2020 ◽  
Vol 30 (05) ◽  
pp. 395-400
Author(s):  
Annika Mutanen ◽  
Mikko P. Pakarinen
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Children And Adolescents ◽  
Perianal Disease ◽  
Ileocecal Region ◽  
Perianal Crohn’S Disease ◽  
Perianal Sepsis ◽  
Endoscopic Assessment ◽  
Perianal Crohn's Disease

AbstractThe incidence of Crohn's disease is increasing worldwide. The clinical course of childhood onset Crohn's disease is particularly aggressive with characteristic disease localization in the ileocecal region and colon, often associated with perianal disease. Severe complications of perianal disease include recurrent perianal sepsis, chronic fistulae, fecal incontinence, and rectal strictures that impair quality of life and may require fecal diversion. Care of patients with perianal Crohn's disease requires a multidisciplinary approach with systematic clinical evaluation, endoscopic assessment, and imaging studies followed by combined medical and surgical management. In this review, we provide an update of the epidemiology, pathophysiology, diagnostics, and management of perianal Crohn's disease in children and adolescents.

A risk haplotype in the Solute Carrier Family 22A4/22A5 gene cluster influences phenotypic expression of Crohn’s disease

2005 ◽  
Vol 128 (2) ◽  
pp. 260-269 ◽  
Author(s):  
Bill Newman ◽  
Xiangjun Gu ◽  
Richard Wintle ◽  
David Cescon ◽  
Mehrdad Yazdanpanah ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Gene Cluster ◽  
Phenotypic Expression ◽  
Risk Haplotype ◽  
Solute Carrier Family ◽  
Solute Carrier

scholarly journals Crohn's Disease Defined in Three Elderly Disters

2005 ◽  
Vol 19 (4) ◽  
pp. 251-252
Author(s):  
Hugh J Freeman
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Environmental Factor ◽  
Present Report ◽  
Granulomatous Inflammation ◽  
Disease Clustering ◽  
Endoscopic Biopsies ◽  
Unusual Occurrence ◽  
Shared Environmental Factor

Three elderly sisters presented with symptomatic Crohn's disease. All had ileocolic involvement, and granulomatous inflammation was documented in endoscopic biopsies or surgically resected intestinal specimens. The present report documents the unusual occurrence of very late phenotypical expression of familial ileocolic Crohn's disease. The observations presented here reflect a possible gene-based predisposition to Crohn's disease or, alternatively, disease clustering related to a commonly shared environmental factor.

scholarly journals P498 Loss of response of anti-TNF in Crohn’s disease patients single-centre experience

2020 ◽  
Vol 14 (Supplement_1) ◽  
pp. S434-S434
Author(s):  
A Atanassova ◽  
A C Georgieva ◽  
M Mirchev
Keyword(s):  
Risk Factors ◽  
Risk Factor ◽  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Clinical Response ◽  
Biological Treatment ◽  
Phenotypic Expression ◽  
Faecal Calprotectin ◽  
Loss Of Response

Abstract Background Despite the timely commencement of the biological treatment, only about 30% will respond to it, and about 1/3 will lose the initial anti-TNF (tumour necrosis factor) response. Methods We retrospectively studied the data of 69 Crohn’s disease (CD) patients who started biological treatment with anti-TNF-ADA/ IFX. We excluded patients who are primary nonresponders to IFX/ADA. In patients with induction of clinical response, we investigated and analysed the frequency of subsequent loss of response (LOR) to IFX/ADA. We analysed the possible risk factors that have led to LOR. Results Of the 69 patients undergoing biological treatment, 71.01% achieved a clinical response during the course of the follow-up. There is a correlation between the presence of a clinical response and the CD course- x2 = 10.78, p = 0.013, ρ = 0.241, (p = 0.046). Inflammatory phenotype (В1) manifestation among our patients is a factor for achieving a clinical response OR = 3.68 (1.116–11.73), p = 0.021, whereas the presence of a penetrating form is a risk factor for the lack of response OR = 6.13 (1.29–29.01), p = 0.019. The presence of intestinal complications is a risk factor for the lack of response- OR = 3.2 (1.61–6.37), p = 0.001. During the course of the follow-up in 30.61% of cases, we observed LOR (men/women - 86.66%/13.33%, p < 0.05 (p = 0.02), in 60.00 % this was between 1–2 years, on average 20.62 ± 13.07 months from the start of the biological treatment. A total of 46.66% of patients required treatment with another anti-TNF drug due to secondary loss of response. A total of 50% of patients needed a reduction of the dose interval. Over 50.00% of those with LOR have an extensive disease (L3). A total of 40% have В1 and 33% have stricturing (В2) and penetrating (В3) phenotypic expression. In 26.66%, we observed progression of the disease range, and in 46.66% of patients - intestinal complications, none of whom had subsequent surgery. In 21.42% of LOR patients there is a combination of intestinal complications and progression according to disease localisation. 93.33% of CD patients with LOR have a persistence of extraintestinal manifestations (EIMs); those with two or three EIMs predominate. We discovered that gender was a risk factor for loss of response, OR = 8.36 (1.16–60.26), p = 0.005, as is the combination of В2 and В3 form of the disease OR = 14.72 (2.47–87.79), p = 0.003. Patients who lost response during the course of treatment had higher mean faecal calprotectin, CDAI and CRP prior to the initiation of the anti-TNF therapy. Conclusion The high activity of the disease measured with CDAI, faecal calprotectin and CRP, male gender, the combination of penetrating and stricturing form during the course of the disease are all risk factors for the loss of response.

scholarly journals Osteonecrosis in a Patient with Crohn's Disease Unrelated to Corticosteroid Use

2001 ◽  
Vol 15 (11) ◽  
pp. 765-768 ◽  
Author(s):  
Ali Khan ◽  
Gerald Illiffe ◽  
Donald S Houston ◽  
Charles N Bernstein
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Present Report ◽  
Vascular Thrombosis ◽  
Inflammatory Disorders ◽  
Direct Association

Osteonecrosis is a feared complication of corticosteroid use. However, a direct association between corticosteroid use and osteonecrosis has never been proven. The present report examines the case of a patient with longstanding Crohn's disease who had never been treated with corticosteroids and who developed osteonecrosis of the talus. The association of systemic inflammatory disorders with osteonecrosis and the possible association with vascular thrombosis are discussed.

scholarly journals The molecular basis of NOD2 susceptibility mutations in Crohn's disease

2008 ◽  
Vol 1 (S1) ◽  
pp. S5-S9 ◽  
Author(s):  
W Strober ◽  
A Kitani ◽  
I Fuss ◽  
N Asano ◽  
T Watanabe
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Molecular Basis

scholarly journals Crohn’s Disease and Cheilitis

2003 ◽  
Vol 17 (7) ◽  
pp. 445-447 ◽  
Author(s):  
Abdulrahman A Al-Hussaini ◽  
Helen M Machida ◽  
J Decker Butzner
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Inflammatory Cell ◽  
Present Report ◽  
Inflammatory Cell Infiltrate ◽  
Chronic Inflammatory Cell ◽  
Colonoscopic Examination ◽  
One Year ◽  
Fissure Formation ◽  
Colonic Biopsies

A five-year-old boy presented to his family physician with painless swelling of both lips. One year later he developed abdominal pain, nonbloody diarrhea, weight loss and joint pains. Colonoscopic examination demonstrated patchy erythema, friability and multiple aph-thous ulcers consistent with the appearance of Crohn’s colitis, and treatment with prednisone was initiated. Colonic biopsies displayed a chronic inflammatory cell infiltrate, focal cryptitis and fissure formation. The patient’s lip swelling relapsed on multiple occasions when steroids were tapered, despite minimal intestinal symptoms of Crohn’s disease. The objective of the present report is to alert physicians to this unusual presentation of Crohn’s disease and that cheilitis may run a protracted course.

scholarly journals Dicrocoelium DendriticumInfection in a Patient with Crohn’s Disease

2008 ◽  
Vol 22 (6) ◽  
pp. 571-573 ◽  
Author(s):  
Franzjosef Schweiger ◽  
Magdalena Kuhn
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Medical Literature ◽  
Present Report ◽  
Land Snail ◽  
Human Infection ◽  
Complex Life Cycle ◽  
Intermediate Hosts ◽  
Animal Liver ◽  
Second Intermediate Host

Infection withDicrocoelium dendriticumin humans is rarely reported in the medical literature. This liver fluke, which commonly infects ruminants, has a complex life cycle with two intermediate hosts – the land snail and the ant. True human infection occurs by ingestion of the second intermediate host, but spurious infections have occurred after consumption of undercooked animal liver. The present report describes a patient with active Crohn’s disease whose stool containedD dendriticumeggs. A brief discussion of the medical literature is presented.

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