The document summarizes key information about inflammatory bowel disease (IBD) in children, including: 1) IBD comprises ulcerative colitis and Crohn's disease, which have distinct but overlapping characteristics. The peak incidence is in adolescents and young adults between 15-30 years of age. 2) Diagnosis involves clinical suspicion based on symptoms, exclusion of other illnesses, differentiation of UC vs Crohn's based on endoscopy and imaging, and identification of extraintestinal manifestations. Laboratory tests like fecal calprotectin can help distinguish IBD from non-inflammatory diarrhea. 3) Treatment depends on disease severity and location, ranging from 5-ASA for mild disease to immunosuppressants, bi

1. Dr Joyce Mwatonoka
3rd Year Resident
Pediatric and Child Heath
2021

2. Introduction
 IBD is comprised of two major disorders: ulcerative
colitis (UC) and Crohn disease (CD)
 UC affects the colon and is characterized by
inflammation of the mucosal layer
 CD can involve any component of the gastrointestinal
tract from the oral cavity to the anus and is
characterized by transmural inflammation

3. Cont…
 These disorders have distinct pathologic and clinical
characteristics, but their pathogenesis remains poorly
understood
 The diseases may present at any age, but peak
incidence is in adolescents and young adults

4. Epidemiology
 Peak Incidence between 15 – 30yrs
 5 – 10% of pts develop IBD during childhood or
adolescence
 The incidence of pediatric IBD appears to be
increasing
 Rare under the age of 5 years
What is the peak age for onset of IBD?Johnston RD, Logan RF Inflamm Bowel Dis. 2008
Oct;14 Suppl 2:S4-5.

5. Cont…
 IBD that becomes symptomatic or is diagnosed before
6 years of age is termed "very early-onset IBD" (VEO-
IBD)
 Compared with children whose IBD develops later in
life, those with VEO-IBD, and particularly those with
onset before 2 yrs of age (sometimes termed "infantile
IBD") are more likely to have single gene defects that
alter immune function or disturb epithelial barrier
function, and often have a more severe disease course

6. Cont…
 Compared with adults, children with IBD;
 Are more likely to present with extensive intestinal
involvement
 Have rapid clinical progression
 Are more likely to have a family history of IBD
Definition of phenotypic characteristics of childhood-onset inflammatory bowel
disease. Van Limbergen J, et al Gastroenterology. 2008;135(4):1114. Epub 2008 Jul 3.

7. Cont…
 Data from a multicenter pediatric IBD registry; Family
hx (including both first-degree relatives and extended
family) was positive for IBD in 29% of pts overall, and
44% of children with UC who were < 3 years
Children with early-onset inflammatory bowel disease (IBD): analysis of a pediatric IBD
consortium registry. Heyman MB, Kirschner BS, et al, Pediatr. 2005;146(1):35.

8. Pathophysiology
 Full understanding of IBD pathogenesis is unclear. It
involves;
 Immune response dysregulation (Th17 – IL 17, TNF)
 Abnormal gut microbiota
 Environmental factors
 Genetic factors

9. Clinical manifestations
 GI sxs; loose stools or bloody diarrhea, abdominal
pain, or tenesmus
-UC; bloody diarrhoea, abdominal pain around bowel
movement, tenesmus
-CD; subtle; low gradeabdominal pain, weight loss,
lack of appetite, diarrhoea c/cout blood
 Systemic sxs; fever, fatigue and anorexia

10. O/E
 Abdominal tenderness and/or mass (especially in the
RLQ), perianal disease (fistulae, anal skin tags, or
fissures, or occult blood in stool)
 Extraintestinal manifestations; Oral ulcerations
(aphthous stomatitis), clubbing, rash (erythema
nodosum or pyoderma gangrenosum), eye
inflammation (uveitis), jaundice or hepatomegaly, or
arthritis
 Growth failure, delayed puberty

11. Clinical features that raise
suspicion for monogenic IBD
 Young age of onset
 Family history of IBD and/or immunodeficiency in
multiple family members
 Recurrent infections or unexplained fever
 Associated features of autoimmunity (eg, arthritis,
primary sclerosing cholangitis, anemia, or endocrine
dysfunction)
 Very severe IBD and/or resistance to conventional
therapies for IBD

12. Diagnosis
 The diagnostic evaluation of IBD involves five steps
 The first two typically are performed by the general
pediatrician, while the last three are performed by the
pediatric gastroenterologist

13. Cont…
 Clinical suspicion of the illness based upon clinical
sxs, examination, and screening laboratory data
 Exclusion of other illnesses that have a similar
presentation
 Differentiation between CD and UC
 Localization of the region of the disease
 Identification of extraintestinal manifestations

14. Suggestive laboratory features
 FBP; anemia, leukocytosis, and lymphocytosis;
Approx 70% of pts have anemia at dxs of IBD
 Elevated ESR (in 65-75% pts) or CRP (in 85%)
-somewhat more sensitive for detecting CD than UC
-no cut off point, an ESR >20 or >25 mm/hr and CRP
>5 or >10 mg/L have been used
 Hypoalbuminemia (in 40% of pts)
Laboratory values for children with newly diagnosed inflammatory bowel disease. Mack DR,
et al, Pediatric Inflammatory Bowel Disease Collaborative Research Group Pediatrics.
2007;119(6):1113.

15. Cont…
 Normal laboratory tests do not exclude the diagnosis
of IBD. In a study of more than 500 children who
ultimately were diagnosed with IBD, the ESR,
hemoglobin, platelet count, and albumin level were all
normal in 19% of children with UC and 9% of children
with CD
Laboratory values for children with newly diagnosed inflammatory bowel disease.
Mack DR, et al, Pediatric Inflammatory Bowel Disease Collaborative Research
Group Pediatrics. 2007;119(6):1113.

16. Cont; Stool tests
 Gross or occult blood; rectal bleeding is a presenting
feature in at least 80% of pts with UC and 40% of
those with CD
-If IBD is suspected but there is no history of gross
rectal bleeding, a stool guaiac (occult blood) test may
be useful
Epidemiologic and clinical characteristics of children with newly diagnosed
inflammatory bowel disease in Wisconsin: a statewide population-based study.
Kugathasan S, Judd RH, et al Wisconsin Pediatric Inflammatory Bowel Disease
Alliance J Pediatr. 2003;143(4):525

17. Cont…
 Fecal calprotectin; elevated in inflammatory
intestinal diseases and may be useful for
distinguishing inflammatory gastrointestinal disease
including IBD from non-inflammatory causes of
chronic diarrhea (such as functional abdominal pain)
Noninvasive Tests for Inflammatory Bowel Disease: A Meta-analysis. Holtman GA,
Lisman-van Leeuwen Y, Reitsma JB, Berger MY Pediatrics. 2016;137(1)

18. Cont…
 However, the test characteristics vary depending on
the prevalence of IBD in the study population
 High prevalence of IBD, eg a gastroenterology clinic,
elevated fecal calprotectin (eg, >200 mcg/g) is useful
for identifying pts with a high likelihood of having
IBD
 In studies from referral centers for pediatric
gastroenterology, fecal calprotectin had better
performance characteristics for this purpose than ESR,
CRP, or hypoalbuminemia

19. Cont…
 By contrast, in settings with a low prevalence of IBD,
eg a primary care setting, it is more useful to rule out
IBD (ie, if fecal calprotectin is normal [eg, <50 mcg/g],
then IBD is unlikely)
Safely ruling out inflammatory bowel disease in children and teenagers without referral for
endoscopy. Van de Vijver E, Schreuder AB, Cnossen WR, Muller Kobold AC, North
Netherlands Pediatric IBD Consortium Arch Dis Child. 2012;97(12):1014. Epub 2012 Sep 27.
The diagnostic accuracy of fecal calprotectin during the investigation of suspected pediatric
inflammatory bowel disease: a systematic review and meta-analysis. Henderson P,
Anderson NH, Wilson DC Am J Gastroenterol. 2014;109(5):637. Epub 2013 May 14

20. Cont…
 Elevated levels of fecal calprotectin are also found in
other causes of inflammatory diarrhea including
bacterial and viral enteritis, intestinal lymphoma,
celiac disease, food allergy, and immunodeficiency,
and also in the setting of juvenile polyps
Value of Fecal Calprotectin as a Biomarker for Juvenile Polyps in Children Investigated With
Colonoscopy. Olafsdottir I, Nemeth A, Lörinc E, Toth E, Agardh D J Pediatr Gastroenterol
Nutr. 2016 Jan;62(1):43-6

21. Cont…
 Fecal lactoferrin
 Fecal leukocytes; Compared with fecal calprotectin,
have considerably lower specificity and sensitivity for
detecting inflammatory diarrhea

22. Other tests;
 Stool bacterial culture, ova and parasite testing, C.
difficile testing
 Tuberculosis screening (interferon gamma testing or
tuberculin skin test)
 Titers for varicella and measles
 HBV serologies
 HIV
 Lactose/glucose hydrogen breath test for lactose
intolerance

23. Radiographic studies
For localization of small bowel disease, one of the
following:
 MRE (Magnetic resonance enterography)
-sensitivity of 83% and a specificity of 93%
 Abdominal CT with oral contrast (CTE – computed
enterography)
Diagnostic Performance of Magnetic Resonance Enterography for Detection of Active
Inflammation in Children and Adolescents With Inflammatory Bowel Disease: A
Systematic Review and Diagnostic Meta-analysis. Yoon HM, Suh CH, Kim JR, Lee JS,
Jung AY, Kim KM, Cho YA JAMA Pediatr. 2017;171(12):1208.

24. Endoscopic studies
 Colonoscopy (including ileoscopy) with biopsies;
-Colonoscopy should be performed in pts with
suspected IBD, even in the absence of clear lower GI
sxs such as bloody diarrhea
 Upper endoscopy with biopsies

25. Differentiation between CD and
UC
 Usually is accomplished with the combination of
endoscopy and imaging of the small bowel
 If the disease type remains uncertain after complete
evaluation, it is provisionally termed IBD-unclassified
(IBDU)

26. Cont…
 Antibody testing; commonest antibody tests are
perinuclear antineutrophil cytoplasmic antibodies (P-
ANCA, 60-80% of those with UC compared with 10-
27% of adults with CD) and anti-saccharomyces
cerevisiae antibodies (ASCA, 40-80% of pts with CD)
 Have reasonably high sensitivities for detecting IBD
(>90% in populations with sxs), but their ability to
distinguish between UC and CD is not well validated

28. Diagnosis (Dx)
 There are no specific diagnostic criteria for IBD
 The dx is usually established by the combination of
clinical features, c/cout laboratory abnormalities,
coupled with characteristic findings on imaging and
endoscopy, including histopathologic analysis
 Endoscopy and imaging also help to exclude some
other causes of the sxs, and usually can distinguish
between UC and CD

29. Differential diagnoses
Rectal bleeding Rectal beeding + other sxs
 Anal fissures or hemorrhoids
 Polyps
 Meckel's diverticulum
 Milk protein-induced
proctocolitis
 Enteric pathogens;
include Salmonella, Shigell, Y
ersinia, E coli, Amoeba, C.
difficile
 CMV; ass/c high rate of
steroid resistance
 Intussusception
 Immunoglobulin A
vasculitis (IgAV)
 Familial Mediterranean
fever

30. Management
 The treatment options depend on the disease location
and severity and response to initial therapy
 5- ASA (aminosalicylate; eg sulfasalazine) based
therapy – mild disease
 Glucocorticoids – acute attack
 Immunosupressants; azathioprine, 6-MP,
methotraxate
 Anti-TNF alpha
 Antibiotics