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Celiac common presentation of a uncommon disease saved with date
1.
2. A Common presentation of
an uncommon disease
By
Dr. Muhammad Arshad
Specialist Internist
Fellow College of Physicians and Surgeons,
Pakistan
KKH Tabuk, KSA
3. Does a Common presentation
of an uncommon disease
means ?
a) A common disease presenting in atypical way.
b) A rare disease presenting in atypical way.
c) A common disease presenting typically.
d) A rare disease presenting typically.
4. Personal Profile
38 years old lady, married last 10 years
,house wife, with out any issue,
admitted via E.R
with presenting complaints of
Abdominal distention - 6 months
B/L leg oedema - 6 months
Loose motions - 4 months
5. History of presenting
complaints
• Abdominal distention gradual onset,
preceded by pedal oedema, worse in
evening
• No history of cough, sputum ,fever, chest
pain, orthopnea, PND, and urinary
symptoms
• After 2 months of above symptoms diarrhea
started, watery, large volume, 5-6 episodes
in day and 5-6 episodes in night, no blood or
mucus with stools, no history of vomiting,
6. History Cont--
Personal History :
Illiterate ,non smoker
Socioeconomic History:
Residing with husband,
Bedouin, average living
Food and Drug History:
No history of any Food
or drug allergy
7. Past History
Three admissions in last 2 years
First admission
Symptoms at presentation were
Backache
Headache
Dizziness and
Fatigue
8. Investigations
Hb 5.8 G/dl
MCV 67.2
Albumin
T Protein
29 G/L
61G/l
TLC 6×103 Calcium
Corrected
2.02mmol
2.22
Plts 392 ×103 Iron 2
ESR 20 TIBC 80
Urea 6.4 AST
ALT
28
49
Creat 92 Alk
Phos
158
Uric Acid 3.2 T Bil 15
17. Patient visited OPD with fresh
Biochem
Albumin = 21 G/dl , Total Protein= 42 G/dl
Calcium = 1.71 mmol/L, Corrected = 2.06
Serum Ferritin= 26.30
RFTs and blood glucose = Normal
ALT =148 and AST = 197,
Alk Phos = 463 (136-145)
T. Bil= 10.8, D.Bil = 3.3
PT=16.3/16.27, aPTT = 45.3/45.5,
INR = 1.37
Repeat Hepatitis B and C serology =Negative
18. 3rd admission
Symptoms written on refferal were
Amenorrhea
B/L pedal pitting oedema
Diarrhea with weight loss, fatigue
Dyspepsia
H/o anemia and blood transfusion
19. Investigations
Hb 6.95 G/dl
MCV 57.2
Albumin
T Protein
19.3 G/L
38.8 G/l
TLC 7.8×103 PT 18.3/18.27
Plts 175 ×103 aPTT 40.6/40.57
Na 141 INR 1.3
K 2.93 AST
ALT
61
94.4
Creat 82 Alk
Phos
230
(136-145)
Urea 3.2 T Bil 15
22. Plan:
Referral to higher centre
for evaluation of cause of Chronic
liver disease but family refused
23. Patient discharged with
diagnosis
Liver cirrhosis , cause ?
Sub clinical hypothyroidism
Iron deficiency anemia
On
Iron, Spironolactone ,
Lactulose, Multivitamin
and frusemide
24. Summary
A 38 yrs old Saudi female, married
issueless , frequent admissions with
symptoms of anemia, wt loss, generalized
oedema, presently admitted with H/O
abdominal distention , B/L leg oedema ,
loose motions 4-6 months
33. Ultrasound Abdomen
Liver size 14cm with homogenous
echo texture (Fatty change)
Portal vein 1.4 cm
Splenomegaly 14 cm
Moderate Ascites
Normal size kidneys
Doppler for hepatic veins= Normal flow,
No sign of Budd Chiari syndrome
35. CT scan abdomen with contrast
Normal size liver, minimally dilated portal vein
and splenomegaly. Normal flow of blood in
hepatic veins and inferior vena cava.
Dilated small bowel loops with generalized
increased mucosal fold thickness
Impression= protein losing enteropathy
vs
Inflammatory bowel disease
50. Findings of malabsorption at barium
examination.
• (a) Image shows duodenitis with
nodularity in a fold-free duodenum.
• (b) Image shows flocculation, dilution,
and dilatation .
• (c) Image shows moulage, which is a
featureless bald appearance of the
jejunum caused by atrophy of folds
and wall edema.
• (d) Image shows reversal of the fold
pattern, with more prominent folds in
the ileum than in the jejunum.
51. • CT Bests Barium in Adult Celiac Disease Diagnosis
• By Todd Neff |July 22, 2011
• Improvements in computed tomography (CT)
resolution of the small bowel, colon, and
mesenteric lymph nodes have pushed CT scans
ahead of traditional barium examinations in the
diagnosis of celiac disease, according to a new
study in the journal RadioGraphics.
• The study, led by Francis Scholz, MD, a radiologist
in the Lahey Clinic in Burlington, Mass., reviewed
CT findings from more than 200 cases of celiac
disease from 1996 to 2009. The CT scans
highlighted abnormal structural changes known
to result from the disease, and in more detail
than possible with a traditional barium
examination, Scholz and colleagues said.
52. Ascitic Fluid
WBC 75/cmm
RBC 500/cmm
Lymphocytes 95%
No AFB seen
TP 5G/L
Albumin 3.8G/L
SAAG >1.2
Glucose 5.6
Cytology No malignant cells seen
53. Upper GIT Endoscopy
Esophagus = normal, no varices
Stomach = normal, except pale mucosa
Duodenum=Flattening of mucosal folds
and inflammatory exaudate was noted
D2 biopsy was taken
54. Histopathology report
Total villous atrophy ,with increased
intraepithelial lymphocytes,
crepts hyperplasia and
infiltration of lamina propria with
plasma cells and lymphocytes.
Consistent with Celiac disease
(Gluten sensitive enteropathy)
60. Marsh Classification
stage 0: preinfiltrative mucosa
stage 1: infiltration of the lamina propria
with lymphocytes
stage 2: Crypt hyperplasia
stage 3: villus atrophy with infiltration of
lamina propria with lymphocytes
,plasma cells
stage 4:
total mucosal atrophy, characterized by
complete loss of villi, enhanced
apoptosis,and crypt hyperplasia
61. Causes of Villous Atropy
Giardiasis
Zollinger-Ellison Syndrome
Crohn,disease
Tropical sprue
Small Intestinal Lymphoma
Graft-Verses host disease
Hypogammaglobulinemia
Eosinophilic gastroenteritis
Severe malnutrition
Radiation or cytotoxic chemotherapy
65. Celiac Disease
• T-cell mediated autoimmune inflammatory disorder of small bowel
• Intolerance to gluten protein (alcohol Soluble Prolamin ) found in
Wheat, Rye , Barley and Oats
• Prevalence variable world wide, in UK 1 in 200,
• 1 in 300-1500 in rest of world , more common in Irish
• 10% prevelance in 1st degree relatives
• 30% relative risk in siblings
• 50% individuals are asymptomatic
as Silent cases and latent celiac disease
69. Clinical Features
Can present at any age
Two peaks
In infancy,
In adults 3rd -5th decades of life
In infancy:
At weaning, infants and children present
with diarrhea, symptoms of malabsorption,
failure to thrive and short stature.
So affected child had both growth and
pubertal delay
70. Clinical features
• In adults peak onset 3rd - 5th decade,
• Females affected more than males
• Presentation highly variable, depending on
• severity and extent of small bowel involvement
• Female may land in Gynae floor with primary
amenorrhea and infertility
• Patient may land in surgical floor with fracture
71. Clinical features
Mostly adults present with diarrhea ,
steatorrhea , abdominal pain, tiredness,
weight loss, Anemia, bone pains,
osteomalacia
oral ulceration (Apthus), angular
stomatitis
Dyspepsia, and bloating - ( IBS ? )
Many patients present with iron deficiency
anemia alone.
72. Clinical presentation can be:
Gliadan Shock:
Massive watery diarrhea just like acute
cholera within hours of eating cereal
containing gluten by a patient who
was on treatment with gluten free
diet
73. Acute Celiac Crises
Manifested by Severe diarrhea,
dehydration, weight loss, acidosis,
hypocalcemia, and hypoproteinemia
74. Refractory Celiac
Symptomatic severe small intestinal
villus atrophy that does not respond
to at least 6 months of a strict
gluten-free diet and this villus
atrophy is not caused by any other
disease or overt intestinal lymphoma
75. Causes of Villous Atropy
Giardiasis
Zollinger-Ellison Syndrome
Crohn,s disease
Tropical sprue
Small Intestinal Lymphoma
Graft-Verses host disease
Hypogammaglobulinemia
Eosinophilic gastroenteritis
Severe malnutrition
Radiation or cytotoxic chemotherapy
76. Extra intestinal Manifestations
Anemia, any type
Hemorrhage
Thrombocytosis
Osteopenia, Pathologic fractures , Osteoarthropathy
Muscular Atrophy
Tetany
Weakness Generalized
Elevated liver enzymes, NASH,NAFLD
Peripheral neuropathy , Ataxia Cerebellar and
posterior column damage, Seizures
Secondary hyperparathyroidism, Amenorrhea, infertility,
impotence, hypothalamic-pituitary dysfunction
Follicular hyperkeratosis and dermatitis, Petechiae and
ecchymoses
Edema Hypoproteinemia
Dermatitis herpetiformis
77. Definite Association
Dermatitis herpetiformis
Type 1 diabetes mellitus 2-8 %
Hypothyroidism/hyperthyroidism 5%,
IgA deficiency 2 %
Epilepsy with cerebral calcification
Inflammatory bowel disease, and Microscopic colitis
Primary Billary Cirrhosis 3%
IgA mesangial nephropathy
Rheumatoid arthritis
Down syndrome
Fibrosing alveolitis, Bird-fancier's lung
Recurrent pericarditis
Idiopathic pulmonary hemosiderosis
Sjogren,s Syndrome 3 %
Sarcoidosis
78. Possible Association
Congenital heart disease, Cavitary lung disease
Systemic and cutaneous vasculitis
Systemic lupus erythematosus, Polymyositis
Myasthenia gravis, Iridocyclitis or choroiditis
Cystic fibrosis, Macroamylasemia
Addison's disease
Autoimmune thrombocytopenic purpura
Autoimmune hemolytic anemia
Schizophrenia, Autoimmune liver diseases
79. Diagnosis
History - Symptoms of Anemia and malabsorption
Examination
Investigation
CBC with peripheral film may show target
cells, spherocytes, and Howell-Jolly
bodies
Biochem, esp Serum Calcium, iron, folate,
B12, phosphate, magnesium, Vit D levels
ALT, AST, Alk Phos
DEXA scan for metabolic bone disease
80. Upper GIT endoscopy with D2 Biopsy
Anti-endomysial and Anti tTG antibodies
85-95 % sensitive and 99% specific
However need to check IgG antibodies in IgA
deficient individual
Become negative with successful treatment
82. Key Elements in the Management of
Celiac Sprue
• C onsultation with a skilled dietitian
• E ducation about the disease
• L ifelong adherence to a gluten-free diet
• I dentification and treatment of
nutritional deficiencies
• A ccess to an advocacy group
• C ontinuous long-term follow-up
83. Gluten free diet
No intake of products made of
Wheat
Barley
Rye
Oats
Can take foods as Rice , Maize , potato
meat, fish, chicken, vegetables, fruits
87. Complications
Increased risk of enteropathy associated
T-cell lymphoma, Small bowel carcinoma,
Squamous carcinoma of oesophagus, and
Metabolic bone disease
Ulcerative jejunoiletis
Collagenous collitis and Sprue
88. Prognosis
Excellent Prognosis: If early diagnosis
and treatment
Malnutrition and even death(complications)
if diagnosed late and no treatment
Early treatment: restore normal
absorptive functions
Advanced complications may not be completely
reversed such as neuropathy or ataxia
Its not always a life long condition
10-20 % of children become Tolerant to gluten
Other develop latent celiac sprue
89. Message
Celiac disease should be included in
differential diagnosis of unexplained Iron
deficiency anemia and malabsorption
