Congenital anomalies are very hard to detect by the lab investigations ,proper lab investigations and assessment is a challenge and this case is one of the challenges.
4. A four years old female child ,born with physiological
neonatal jaundice . At the age of six months she
presented with an attack of :
Bile stained vomitus
Epigastric and right hypochondrial pain
Pallor & fatigue
5. CASE PRESENTATION
• These attacks were repeated, twice at the age of two
years and 3 times at the age of three ,and twice at the
age of four .These last two attacks were associated with
fever????
• In between the attacks the girl was totally free except for
transient attacks of epigastric pain
• During these attacks the following was done:
7. IMAGING TECHNIQUES POSITIVE FINDINGS
Mild dilatation of the Gall Bladder
Dilatation and irregular caliber of the common
bile duct with (3-5 ) 5 mm stones , no intrahepatic
biliary dilatation
The spleen was slightly enlarged
Liver
and pancreas were totally free
In between the attacks there were no stones but the
G.B remained dilated and also was the CBD
9. AST & ALT AT THE AGE OF TWO YEARS
1200
AST
1000
Mean
800
600
400
200
0
ALT
Normal
10. AST & ALT AT THE AGE OF THREE YEARS
3000
AST
ALT
Normal
2500
Mean
2000
1500
1000
500
0
February
March
April
May
Months
Distribution of AST and ALT between the age of 3-4 years
July
November
11. AST & ALT AT THE AGE OF FOUR YEARS
1800
1600
AST
ALT
Normal
1400
1200
Mean
1000
800
600
400
200
0
January
February
Months
Distribution of AST and ALT at the age of four
April
12. 2007
Distribution of AST and ALT
Months
2008
2009
April
February
January
November
July
May
April
March
February
December
ALT
November
AST
July
May
April
March
February
Mean
3000
2500
Normal
2000
1500
1000
500
0
13. Total and direct bilirubin
1.4
Total bil
1.2
1.0
Mean
0.8
0.6
0.4
0.2
Direct bilerubin
Normal total bilerubin
Normal direct bilerubin
14. Total and direct bilirubin
1.4
Total bilerubin
1.2
1.0
Mean
0.8
0.6
0.4
0.2
Direct bilerubin
Normal total bilerubin
Normal direct bilerubin
15. Total and direct bilirubin
1.4
Tota l bilerubin
1.2
Direct bilerubin
Norm a l tota l bilerubin
Norm a l direct bilerubin
1.0
Mean
0.8
0.6
0.4
0.2
0.0
January
February
Months
Distribution of total bil and direct bil in 2009(age of 4)
April
16. 1.4
Total bil
1.2
Direct bilerubin
Normal total bilerubin
Normal direct bilerubin
0.8
0.6
0.4
0.2
2007
2008
Months
Distribution of total bil and direct bil
2009
April
February
January
November
July
May
April
March
February
December
November
July
May
April
March
0.0
February
Mean
1.0
18. Alkaline phosphatase
AT THE AGE OF THREE YEARS
800
Alk
700
600
Mean
500
400
300
200
100
Upper normal
Lowest normal
19. Alkaline phosphatase
AT THE AGE OF FOUR YEARS
800
700
Alk
Upper normal
Lowest normal
600
Mean
500
400
300
200
100
0
January
February
Months
Distribution of Alk in 2009 in between the age of (4-5)
April
20. Distribution of Alk
2007
Months
2008
February
Upper normal
January
November
July
Alk
May
April
March
February
December
November
July
May
April
March
February
Mean
800
700
Lowest normal
600
500
400
300
200
100
0
2009
21. GAMA GLUTMYLE TRANSFERASE
AT THE AGE OF TWO YEARS
350
CGT
Upper norm a l
Lowest norm a l
300
Mean
250
200
150
100
50
0
February
March
April
May
July
Months
Distribution of GGT in 2007(age of 2)
November
December
22. GAMA GLUTMYLE TRANSFERASE
AT THE AGE OF THREE YEARS
800
800
CGT
CGT
700
700
Upper norm a l
Upper norm
Lowest norm a l
Lowest norm a l
600
600
Mean
Mean
500
500
400
400
300
300
200
200
100
0
February
March
April
May
Months
July
Distribution of GGT in 2008(age of 3) the age of (3-4)
2008 between
Novembe
23. GAMA GLUTMYLE TRANSFERASE
IN THE AGE OF FOUR YEARS
400
350
CGT
Upper normal
Lowest normal
300
Mean
250
200
150
100
50
0
January
February
Months
Distribution of GGT in 2009 at the age of 4
April
25. CASE PRESENTATION
OTHER LAB. TESTS…….
(1) Normal PT & PTT
(2) Hb : 9.5 g/dl (Reference range 11.5-14.5)
(3) Bile salts : 3.0 µmol/l ( Reference up to 8.1)
(4) Cholesterol 220 mg/dl (140-200 mg/dl)
(5)ALL VIRAL INFECTIONS WERE EXCLUDED:
1. Hepatitis A virus IgM
2. Hepatitis B
3. Hepatitis c
4. EBV IgM
5. CMV IgM
27. • OTHER LAB. TESTS…….
•Plasma protein 6.7 mg/dl
•Albumin 3.5 mg/dl
•Globulins 3.2(2.0-3.8)
•Serum IgG 704.0 mg% ( 730.0-1350.0)
•Serum IgM 66.8 mg% (80.0-150.0)
• Serum IgA 62.3mg/dl (70.0-227.0md/dl)
28.
29.
30.
31.
32.
33. CASE PRESENTATION
SUMMARY
CLINICALLY
• Bile stained vomitus
• Epigastric and right hypochondrial pain
• Pallor & Fatigue
RADIOLOGICALLY
•Mild dilatation of the Gall bladder
•Dilatation and irregularity of the common bile duct with (3-5 ) 5 mm stones that
disappear in between the attacks
•The spleen is slightly enlarged
LABORATORY
•Marked elevation of AST & ALT
•Elevated ALK & GGT
•Mild anemia
•PositiveLKM1Abs
34. WHAT IS THE D.D OF THIS CASE?
WHAT IS THE MOST LIKELY
DIAGNOSIS?
35. WHAT IS THE DIFFERENTIAL DIAGNOSIS?
1.
Cholestatic liver diseases
2. Autoimmune Hepatitis
3. Hemolytic anemias??????
What findings are WITH?
What findings are AGANIST?
36. CASE PRESENTATION
Hemolytic anemia
WITH
•Age of incidence
•Mild spleenomegaly
•Hb 9.5 mg/dl
AGAINST
•Normal trace urobilinogen in urine
•CBC is normal with normochromic normocytic
anemia
•No reticulocytes
•Coomb`s test negative
•Osmotic fragility test negative
•Sickling test negative
•Normal Hb electrophoresis ( Hb A 97% & Hb A2 3%
37. Autoimmune Hepatitis
(International autoimmune hepatitis Group)
•
•
•
•
•
•
•
•
Normal level of alpha 1 antitrypsin
Seronegativity for IgM Antiviral hepatitis
Negative CMV
Negative EBV
Low ethanol ingestion
No recent use of hepatotoxic drugs
Serum gammaglobulins IgG> 1.5% of normal
Positive ANA,ASMA, LKM1
Liver biopsy to rule out other lesions
38. Autoimmune Hepatitis
Type I:
Type I: male
-female>
-Any age
-With other autoimmune disease
-Positive ANA, ASMA,Antiactin, increased gamma globulins in 97% of cases
Type II:
-Girls ages 2-14 years
--Signs of fatigue & abdominal pain
-LKM1 & increased gamma globulins
Type III:
-female> male
-Age between 20-40
-Positive SLA
39. CASE PRESENTATION
Autoimmune Hepatitis
WITH
•Age of incidence( 2-14)
•Female> Male
•Signs of fatigue&abd pain
•Mild spleenomegaly
•ElevatedAST & ALT
•Elevated ALK & GGT
•Increased ALKM1Abs
•Negative CMV,EBV
AGAINST
•Normal imaging of the liver
•Normal Bilirubin????
•No increase in gamma globulins
40. CASE PRESENTATION
CHOLESTATIC LIVER DISEASES
1. Mechanical Bile Duct Obstruction
( STONES)
2. Primary Biliary Cirrhosis
3. Primary Sclerosing Cholangitis
4.Autoimmune cholangitis
5.Congenital anomaly in the CBD
6. Drug Induced Cholestasis
41. CASE PRESENTATION
CHOLESTATIC LIVER DISEASES
2. Primary Biliary Cirrhosis
WITH
•Fatigue(70%)
•Spleenomegaly (15%)
•Gallstones (30%)
•Elevated Aminotransferases
•Elevated GGT & ALP
•Elevated cholesterol
AGAINST
•Age of the case (50 years)
•Imaging (no periportal halo sign)
•Usually associated with other autoimmune
disease
•Increased bilirubin
•Positive antimotochondrial Abs(sene
98%, spec 96%) & negative Antinuclear
Abs(35%)
42. CASE PRESENTATION
CHOLESTATIC LIVER DISEASES
3. Primary Schelerosing Cholangitis
WITH
•Symptoms(Fatigue 66%,Abd pain 50%,Fever/
cholangitis
13-45%)
•spleenomegaly
•Elevated Aminotransferases
(3x increase)
•Elevated ALP(3x higher)
•Normal bilirubin
AGAINST
•Age of the case (30 years)
•Predominates in males
•Imaging shows no beading of the bile
duct
•Positive Antineutrophil cytoplasmic Abs (
ANCA) in 80% of cases
43. CASE PRESENTATION
CHOLESTATIC LIVER DISEASES
3. Autoimmune cholangitis
Same picture of primary biliray cirrhosis with negative AMA
may overlap with autoimmune hepatitis
Liver biopsy is the gold standard
44. CASE PRESENTATION
CHOLESTATIC LIVER DISEASES
1. Mechanical Bile Duct Obstruction
( STONES)
•Bile stained vomitus
•Presence of stones by imaging techniques
•Increased plasma activities of canalicular enzymes ALP & GGT
• Increased cytosolic enzymes AST & ALT
•Bilirubin is not increased so this is
partial obstruction
45. A Question needs to be answered?
WHAT ARE THE CAUSES OF STONE
FORMATION?
46. There are three types of biliary
stones…….
•Cholesterol stone
•Pigmented stone
•Mixed stone
47. There are three types of biliary
stones…….
•Cholesterol stone
•Bile is supersaturated with cholesterol
Supported by increased cholesterol
( diet or genetic)
•Decreased bile acid secretion (terminal ileal
disease, cholestatic liver diseases)
48. There are three types of biliary
stones…….
Pigmented Stone
Hemolytic anemia
1.CBC
2. Reticulocytes
Deconjugation of bilirubin due to
cholestasis & infection
3. Coomb`s negative
4.Osmotic fragility negative
1.Stone formation
2.Sickling test
2.Congenital anomaly
49. Congenital anomalies
Anatomical deformities in the bile duct at the level
of the duodenum (Ampullary dysfunction)
This could be diagnosed and treated by ERCP
Due to the her age 4 years
and her size ERCP couldn't be done
8.3 - 10.3 years
SO if these attacks become life threatening so Cholecystectomy and
biliary diversion is the only solution
50. FINAL DIAGNOSIS
• Colestatic form of liver disease associated lately with
ascending cholangitis needs ERCP for Diagnosis
and treatment
• Autoimmune hepatitis (type II) and/or autoimmune
cholangitis
• need liver biopsy for further assessment
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