Inflammatory Bowel Disease 1

Alternative Names

  • Regional Enteritis
  • Crohn Disease
  • Ulcerative Colitis
  • Crohn Disease-Associated Growth Failure, Susceptibility to
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WHO-ICD-10 version:2010

Diseases of the digestive system

OMIM Number

266600

Mode of Inheritance

Multifactorial

Gene Map Locus

7p15.3,16q12.1

Description

Inflammatory bowel disease (IBD) is a chronic progressive inflammatory disorder that affects any area of the digestive tract, from the mouth to the anus. However, the lower part of the small intestine (the ileum) is the most commonly affected area. IBD is subdivided into Crohn disease and ulcerative colitis phenotypes.

Patients with Crohn's disease have abnormal chronic immune reaction which causes WBC accumulation in the lining portion of the digestive tract, and thereby chronic inflammation, leading to ulcerations and bowel injuries. The symptoms associated with Crohn's disease are very much similar to those of other intestinal disorders including abdominal pain, diarrhea, rectal bleeding, weight loss, arthritis, skin problems, and/or fever. However, in Crohn's disease all layers (mucosa, submucosa, and serosa) of the affected part of the digestive system may be involved. Also, ulceration can extend to the surrounding tissues such as the bladder, vagina, or skin and causes serious complications. Children with Crohn's disease may have delayed development and stunted growth. Crohn's disease affects both genders equally and it is diagnosed mostly in individuals of ages between 20 to 30 years. Moreover, it has been found that Jewish heritage will increase the risk to have Crohn's disease. Diagnosis can be made by blood test, X-ray in association with barium, and/or visual exam of the colon (sigmoidoscopy and colonoscopy). Treatment relies on taking certain drugs like anti-inflammation drugs and nutritional supplements, and/or performing a surgical operation.

In ulcerative colitis (UC), the most affected part is the large intestine; primarily affecting colonic mucosa; and the extent and severity of colon involvement are variable. In most patients, the distal rectum is initially affected. As the disease progresses, part or the entire colon is involved. UC is usually associated with recurrent attacks with complete remission of symptoms in the interim. The initial clinical presentation of UC is diarrhea with blood and mucus. The other symptoms are abdominal pain, fever and weight loss. In severe causes, patients may be at risk for certain serious complications such as perforation of the colon. In addition, UC is systematic disease; patients may present systemic symptoms such as certain inflammatory skin or eye conditions, arthritis and chronic inflammation of the liver (chronic active hepatitis). The exact cause of UC is unknown. However, several environmental factors may be involved in the pathogenesis and the progression of the disease such as cigarette smoking and psychological stress. The best test for diagnosis of UC is endoscopy. The treatment aims to induce remission and prevent a relapse of the disease by using aminosalicylates, corticosteroids and immunosuppressive drugs. Colectomy is necessary in the severe causes. UC is a rare disease. It is more common in Caucasians than in Blacks or Orientals with an increased incidence (three- to six-fold) in Jewish populations. Both sexes are equally affected. Although the disease has been reported in every decade of life, it has the highest incidence among adolescents and early adults, aged between 15-35 years.

In approximately 10% of cases confined to the rectum and colon, definitive classification of Crohn disease or ulcerative colitis cannot be made and are designated 'indeterminate colitis.' Crohn disease and ulcerative colitis are considered complex genetic traits as inheritance does not follow any simple Mendelian models.

Epidemiology in the Arab World

View Map
Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
266600.G.1.1TunisiaUnknown Crohn's diseaseNM_005214.4:c.49A>GHomozygousBen Alaya et al. 2009 Group of 33 Crohn's ...
266600.G.1.2TunisiaUnknown Crohn's diseaseNM_005214.4:c.49A>GHeterozygousBen Alaya et al. 2009 Group of 35 Crohn's ...
266600.G.2.1Lebanon Inflammation of the large intestineNM_001558.3:c.475A>GGhaith et al. 2010 Study of a group of ...
266600.G.2.3Lebanon Inflammation of the large intestineNM_001558.3:c.1051A>GGhaith et al. 2010 Study of a group of ...

Other Reports

Bahrain

Al-Hindi et al. (1999) described a 35-year-old Bahraini male patient with a rare form of Crohn's disease that involved the appendix only. The suthors reviewed the medical records in the medical center over an 11-year-period in order to identify the previous cases with Crohn's disease. There were 17 Bahraini patients who had the disease involving various parts of the intestine (commonly the ileocecal area), except in the case described above.

[Al-Hindi S, Hassan A, Haider F. Crohn's disease of the appendix. Bahrain Med Bull. 1999; 21(4):148-9.]

Ansari et al. (2006) carried out a prospective study on 58 patients with lower gastrointestinal symptoms at the Bahrain Specialist Hospital from July 2004 to April 2005 to compare RANTES expression between intestinal biopsy specimens of patients with Crohn's disease and those with ulcerative colitis. Of the patients, 40 had IBD (21 had Crohn's disease and 19 had ulcerative colitis) and 15 were controls and three had colonic inflammatory lesions other than IBD. The authors found that RANTES expression in lymphocytes or histiocytes was significantly higher (p = 0.04) in new patients with ulcerative colitis than in those with Crohn's disease analyzed by immunohistochemistry (IHC).

Egypt

[See: Saudi Arabia > Khan et al, 1996].

Kuwait

Khan et al. (2003) studied the role of Na+/H+ exchanger isoform-1 (NHE1) in 24 patients with IBD (13 with CD and 11 with UC) and 11 control cases that had all clinical symptoms of IBD, except for diarrhea. Colonic biopsies showed a significant increase in myeloperoxidase activity in the patients when compared to the controls; and significantly higher activity in CD patients when compared to UC patients. This increased inflammatory response was also evident in histology studies. UC patients had more ulcers, whereas CD patients had more granulomas and active edemas. There was a significant decrease in the level of NHE-1 protein in both UC and CD cases, which was contrary to the observation in animal models. RT-PCR studies identified a significant decrease in the NHE-1 mRNA levels in the colonic biopsies in these cases as well, indicating a possibility of transcriptional regulation. Khan et al. (2003) suggested that this suppression of NHE-1 expression possibly led to a decrease in intracellular Na+, and subsequently Ca+ levels. This could, in turn, contribute to the diarrhea and electrolyte disturbances seen in these conditions. This situation may also contribute to cellular acidosis, leading to tissue necrosis.

A 13 year old Kuwaiti female suffering from generalized vesicobullous eruption for a period of 2 weeks and demonstrating linear IgA bullous disease (LABD) of childhood was reported by Nanda et al. (2006) at the Autoimmune Bullous Diseases (ABD) clinic. The patient also experienced a history of intermittent diarrhea, abdominal pain and fever associated with weight loss throughout a year and a half, revealing a diagnosis of Crohn's disease and therefore she was treated with sulfasalazine and budesonide. Throughout examination, the patient demonstrated intact blisters, and crusted and eroded lesions on her face, scalp, neck, truck, and extremities involving 40% of her skin area. The patient was treated with dapsone (100 mg daily) which cleared the skin lesions within a period of 4 weeks; she was also kept on dapsone (50 mg daily) and underwent a 1 year follow-up demonstrating a skin free of lesions. Nanda et al. (2006) proposed improving the understanding of similar associations as the one reported (Crohn's disease correlated with LABD of childhood) to expand the present facts regarding these compound disorders.

Al-Qabandi et al. (2011) carried out an epidemiological study of IBD among children in Kuwait, by studying 130children diagnosed with the disease in a 10-year period (1998-2008). The majority of the patients (82%) were Kuwaitis. A family history of IBD was reported in 20% of the cases. Majority of the patients (71%) had Crohn's Disease. This was different from the adult population, which was reported to suffer predominantly from Ulcerlative Colitis. Based on this study, the annual incidence of IBD in Kuwait was calculated at 2.16 per 100,000 births. This was the highest reported incidence of this condition in the region. Incidence of UC and CD was calculated at 0.6 and 0.03 per 100,000 births, respectively. 

Lebanon

El-Hajj et al, 2007, described a 23 year old female Crohn's patient presenting with abdominal pain and fecaloid vomiting. She was found to have a rare case of benign duodenocolic fistula. 

Abdul-Baki et al, 2007, carried out a retrospective review of patients seen at the American University of Beirut Medical Centre between 2000 and 2004. Of 15,000 individuals, 8 were diagnosed with Crohn's disease (giving a prevalence of 53.1 per 100,000 people) and 16 had ulcerative colitis (giving a prevalence of 106.2 per 100,000 people). 

Mauritania

[See: Saudi Arabia > Khan et al, 1996].

Morocco

[See: Saudi Arabia > Khan et al, 1996].

Oman

Radhakrishnan et al. (1997) assessed the incidence and disease pattern of UC in Oman by applying a prospective study, over a period of 8 years (1987-1994), to study 108 patients with UC. The annual incidence of UC was 1.35/100,000 with a main occurrence in the middle and upper middle class group and the majority were non-smokers or ex-smokers. Total colitis was seen in 18%, and a significant number had disease extending up to the splenic flexure. Proctitis was seen in 8%. Although, the extent of the disease was similar to reports from the West, these patients had fewer hospital admissions, blood transfusions and none of them suffered local complications such as toxic dilatation, perforation or severe bleeding.

Palestine

In 2005, Butt et al. performed a retrospective study to assess the clinical characteristics of Crohn’s disease (CD) in Qatar which included at least one Palestinian patient.  Diagnosis of CD was based on the conventional endoscopic, radiologic, and histologic criteria.

[See also: Saudi Arabia > Khan et al, 1996; Qatar > Butt et al., 2005].

Qatar

Butt et al. (2005) assessed the clinical characteristics of Crohn's disease (CD) in Qatar by studying 31 Qatari patients retrospectively over a period of eight years. The majority of the studied subjects were under 40 years. Approximately half of the patients had ileocolonic disease, with a quarter each having ileal or colonic disease. Patients with ileocolonic disease were found to be affected with inflammations and fistulas, and presented with symptoms of weight loss, whereas those affected by the ileal form had added complications of obstruction, and presented with symptoms of abdominal pain. Patients with the colonic form of the disease presented with diarrhea. About one-third of the patients required surgery.

In a study by El-Menyar et al. (2006) analyzing data pertaining to all patients less than 50-years of age, who were hospitalized between 1996 and 2003 with cardiomyopathy in Qatar, a rare association was noticed in a 1-year old male with comorbidity of hypertrophic cardiomyopathy and Crohn's disease.

Saudi Arabia

The first report on Crohn’s disease in children from Saudi Arabia was made by Toonisi (1993).  This study covered a period of 10-years (1980 to 1990) with a follow up of 17 children (10 boys, 7 girls) with the condition from Western Saudi Arabia.  The age of presentation ranged from 5 to 15 years.  The most common presenting symptom was abdominal pain, followed by diarrhea, fever, and hematochezia.  Extra-intestinal manifestations included finger clubbing, and erythema nodosum.  Raised erythrocyte sedimentation rate (ESR) was the most common finding in the laboratory investigations, followed by anemia and hypoalbuminemia.  A quarter of the patients showed abnormal liver functions.  

In 1996, Khan et al. published the results of their retrospective analysis on the frequency of ulcerative colitis in among the residents of Al Madinah.  The study was based on the data generated by analysis of all lower gastrointestinal endoscopies performed at the referral hospital in the city between years 1990 and 1995.  Of the total of 1,182 endoscopies, 111 were diagnosed with ulcerative colitis.  Of these, 80 patients were Saudis, while another 15 were non-Saudi Arabs (four Egyptians, three Yemenis, two Mauritanians, one Moroccan, one Palestinian, one Sudanese, one Syrian, and one Tunisian).  Among the 80 Saudi patients (47 males, 33 females), the main presenting symptoms were rectal bleeding, diarrhea and abdominal pain.

Sudan

Khalifa et al. (2005) conducted a retrospective study at the National Center for Gastrointestinal and Liver disease in Khartoum, Sudan on patients diagnosed with inflammatory bowel disease (IBD) during the period between January 1990 and May 2001. All patients underwent a colonoscopy/sigmoidoscopy with histopathological diagnosis, or a barium study. Seventy three patients were diagnosed as Ulcerative colitis (UC), and twelve patients as Crohn's disease (CD). Both types tend to be more common in men.

[See also: Saudi Arabia > Khan et al, 1996].

Syria

[See: Saudi Arabia > Khan et al, 1996].

Tunisia

[See: Saudi Arabia > Khan et al, 1996].

Yemen

[See: Saudi Arabia > Khan et al, 1996].

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