8. Definition
Chronic diarrhea is defined as diarrhea( 3 or
more stools /day ) of more than 2 weeks
duration
- Persistent : Acute in onset
- Chronic: Insidious in onset
- Malabsorption syndrome: Failure of
absorption of one or more nutrients
9. Causes : Age of Onset
Neonatal Congenital secretory diarrhea,
Microvillous Inclusion Disease
1- 6 months Persistent Diarrhea
CMPI
Autoimmune enteropathy
HIV
6months-2 years PD, CPMPI, HIV, malnutrition,
Celiac disease
2-5 years Celiac disease, Parasitic
Infections,
Malnutrition, HIV
>5 years IBD, Tuberculosis, HIV
10. Analysis of History / Physical
Examination
• Small bowel vs. large bowel diarrhea
• Chronic diarrhea with or without failure
to thrive /malnutrition
11. Small bowel/ large bowel diarrhea
Small Bowel Large Bowel
Volume large small
Frequency less more
Color light dark
Blood /Mucus No Yes
Undigested food Yes No
Smell Foul Rarely foul
Tenesmus No Yes
Urgency No Yes
Nutrient Def Common Uncommon
12. Clinical impression
8 yr child
• Chronic diarrhea
• Growth failure
• Pallor
• No recurrent
chest/skin infection
• No significant family
history
Did this child merit ATT ??
20. Celiac Disease in India
CD first reported in 1966 Walia et al
in children, Mishra et al in adults
• Lack of awareness
• Lack of widespread availability of
jejunal biopsies
• Stringent three-step European Society
of Pediatric Gastroenterology and
Nutrition (ESPGAN) diagnostic criteria.
22. Celiac disease in children
Yachha et al
Chronic diarrhea (> 3 wks) in Indian children
137 children < 2 y 45% , > 2y 55%
- Protracted diarrhea 45(33%)
- Celiac disease 35(26%)
- Parasitic infestations 13(9%)
- Milk protein intolerance 8(6%)
- Intestinal TB 7(5%)
- Idiopathic 18(13%)
Yachha et al IJG 1993;12(4):120-5
26. Case scenario 2
3 yr old boy with c/o
Inability to walk or
stand for 1 mo
Evaluated by adult
neurologist for AFP
Clinically no
neurological deficit,
NCV normal
Hb 12.5
No diarrhoea
Can this child have celiac?
27. Case scenario 2
Reevaluated
• On history : Twice BT in a
year, underwent BM
aspiration elsewhere-non
specific
Examination :
• Weight and height below 3
rd centile
• Rickets
So can now this child be celiac?
IgATTG 250
D2 bx STVA
29. • Short stature /Delayed puberty: Endocrinology
• Refractory anemia : Hematology
• Rickets (fracture or deformity) :Orthopedics
• Constipation with distension :Ped. Surgery
• Neuropathy, ataxia, seizure :Neurology
• Aphthous ulcers, dermatitis herpetiformis:
:Skin
• Amenorrhea, infertility, impotence: :Gynecology
Alert for atypical
presentation of CD
30. Conditions associated with an increased
prevalence of celiac disease
• Type I diabetes mellitus
• First degree relatives of celiac patients
• Selective IgA deficiency
• Autoimmune thyroiditis
• Down syndrome
• Turner syndrome
• William syndrome
31. Diagnostic principles
Confirm diagnosis before treating
- Mandates a strict GFD life
- Following the diet is not easy
- QOL implications
Failure to treat has long term adverse
health consequences
- increased morbidity and mortality
32. Revised ,ESPGHAN;1990
- Histological findings compatible with
celiac disease
– Unequivocal clinical response to GFD
– +/- Positive celiac serology
(ESPGHAN working group .Arch Dis Child 1990;65:909)
33. Serological tests
Identify symptomatic individuals
who need biopsy- classic and atypical
Screening of asymptomatic
“at risk” individuals
Supportive evidence for diagnosis
Monitoring dietary compliance
Epidemiological studies to find prevalence of CD
35. Best sensitivity and specificity of tTG antibody
a cut-off value of 10 U/mL
Pts mean tTG antibody titer 106+/-76 U/mL
while normal was 2+/-1.6 U/mL
Problems:
•Ig A deficient children (6-10%)
•Children less than 2 years
•Values between 10-100 U/ml
•Patient started on GFD
36. Comparison between EMA IgA
and TTG IgA
• The human recombinant based TTG has
similar sensitivity and specificity to the
EMA test
• The guinea pig protein– based tests are
less sensitive and specific compared
with the EMA test
37. Seronegative CD
IgA deficiency
1.7 – 3.0% of CD
False negative
Milder form of villous atrophy
Age < 2 yr ( EMA sensitivity-80%)
On immunosuppressive therapy
Already on GFD
(Arch dis child 1991;66:941-947)
(Dig dis and sci ,49,no 4,546)
(Cataldo F et al J Pediatr 1997;131:306-8)
(Cataldo F et al Gut 1998;42:362-65)
38. When to Screen
• 3 years and atleast 1 year of gluten based
diet.
41. Modified Marsh classification
Marsh 0 - Normal mucosa and villous architecture
Marsh 1 - infiltrative - Increased IEL
Marsh 2 - 1+ hyperplastic crypts
Marsh 3 - 2+ destructive – villous atrophy
3a - partial villous atrophy
3b - subtotal villous atrophy
3c - total villous atrophy
Marsh 4 - hypoplastic – flat atrophic mucosa - total
villous atrophy with crypt hypoplasia
Marsh et al Gastroenterology 1992;102:330-354
Rostami K et al Am J Gastroenterol 1999;94:888-94
Oberhuber G, Granditsch G, Vogelsang H. Histopathology of celiac disease: time for
standardized report scheme for pathologist. Eur J Gastroenterol 1999;11:1185-94.
42. HLA tests
HLA alleles associated with celiac disease
- DQ2 found in 95 %
- DQ8 found in rest
- DQ2 found in 30% general population
Value of HLA testing
High negative predictive value
Negative DQ2/8 excludes celiac with 99%confidence
Schuppan et al Gastroenterology 2000;119:234
Kaukinen et al Am J Gastroenterol 2002;97:695
43. Case 3
• 2 year old girl
• Chronic diarrhoea x 1 yr
• Mild anemia Hb 10.2
• IgA TTG 50
Next… Started on Gluten free diet
No improvement in symptoms in 3 mo
D2 bx – non specific
How to interpret this bx report?
44. Case 3
Further Options
• Gluten challenge
1. In which cases?
2. Can it be done in this case?
• HLA typing
1. When is it useful?
2. How to interpret ?
45. Case 3
HLA DQ 2, 8 negative
Revaluation
• Diet history
• Sigmoidoscopy
Don’t start GFD
based on serology
alone
46. Gluten Challenge
• Not be undertaken for at least two
years, and preferably not before the age
of 6 years - Can damage the dentition if
done earlier – enamel defects
• Control biopsy on GFD
• Repeat biopsy - Noticeable clinical
relapse or After three to six months.
47. Gluten Challenge
• If repeat biopsy is normal, look for late
relapse at 2 years with a biopsy.
• Further follow up and need for biopsy
guided by monitoring antibody levels
49. Management of Celiac Disease
Six key elements
•Consultation with a skilled dietitian
•Education about the disease
•Lifelong adherence to a gluten-free
diet
•Identification and treatment of
nutritional deficiencies
•Access to an advocacy group
•Continuous long-term follow-up by a
multidisciplinary team
50.
51. Treatment
Only treatment for celiac disease
is a gluten-free diet (GFD) < 20 ppm
– Strict, lifelong diet
– Avoid:
Wheat
Rye
Barley
52. 10 mg gliadin ~ 250 mg wheat flour
(less than an 1/8 teaspoon flour)
53. GFD limitations
• Impairs quality of life
• Psychological impact
• Increase risk of obesity- overfeeding/low
fibre
• Altered eating pattern
• Chronic inadequacy of micronutrients
(low iron,Zn,Ca, Mg, vit D)
54. Treatment challenges
• Dietary compliance
• To get gluten free diet
• No reliable test- detect gluten in food
products
• No identification mark for GFD products
• GFD-more costly
• Lack of varieties
55. Follow-up for dietary
adherance
• Pretreatment values taken
• IgA levels fall earlier than IgG
• Either IgA TTG or AGA can be used
• Serological normalization precedes histological
recovery
• Measurement of TTG after 6 months of GFD-
decrease in antibody titer indicates recovery
• Persistent or recurrent symptoms with GFD - Rise in
antibody levels indicates dietary nonadherence
• Asymptomatic patient- measurement of TTG at
intervals of 1 year or longer may serve as a monitor of
adherence to the GFD
56. Follow-up
• Growth and maturation assessment
• Assessment for nutritional deficiency
• Counselling and psychological support
57. RESPONSE TO A GLUTEN-FREE DIET
90% IMPROVE 10% FAIL TO IMPROVE
(within 2 weeks) Dietary indiscretion
Lactose or fructose Intolerance
Microscopic colitis
Wrong Diagnosis
Pancreatic Insufficiency
Bacterial overgrowth
Refractory sprue
58.
59. Clinical features in Indian
children
Poddar et al
300 celiac disease
Mean age - 6.7 +/- 3 years
Mean duration of symptoms - 3.5 +/- 2.5 years
Symptoms:
- Diarrhea 84 % - Failure to thrive 91%
- Anemia in 84% - Wasting in 87%
- Stunting in 60%
Majority – classic sprue , have delay in diagnosis
Prolonged breast-feeding and delayed
introduction of gluten in the diet could be
responsible for later onset
Poddar et al JPGN 2006;43(3):313-7
60. CD – Outcomes in India
• CD children (n=42)
• 60% were undernourished, 40% had
normal nutrition
• After 4 years, 84% cases had normal
nutrition
• Height for age was attained in 76%
cases
• None of the cases achieved normal
histology
Yachha SK,. J Gastroenterol Hepatol 2007;22:1300-5.
61. • Look for celiac disease in a
child with chronic diarrhoea
1
• Alert for atypical CD
and early diagnosis of CD
2
• Serology is supportive not
confirmatory
3
• Endoscopy & Duodenal biopsy
is a must for diagnosis.
4
