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A 17- Year- Old Boy with Biliary Obstruction
CC
HPI- 17 months prior to admission to MGH
Symptoms: Bloody diarrhea
admission to a hospital
what exams to do?
LAB
• Serum aspartate aminotransferase level: 75 U/l
• Test for Clostridium difficile: positive had not taken antibiotics before!
management?
MANAGEMENT
One month course of metronidazole
patient feels well
Several weeks prior to admission to MGH
Symptoms:
– constant crampy, nonradiatingpain in the epigastrium, right upper quadrant, periumbilical area,
– pain exacerbates by eating, accompanied by nausea
– intermittent loose stools without frequent or voluminous diarrhea
– temp. rises intermittently to 38.3 °C
Five days prior to admission
Symptoms: – leftsided pleuritic chest pain – dry cough
admission to a hospital
exams?
LAB
• Leucocytosis with a leftward shift
• BLOOD CHEMICAL AND ENZYME VALUES
601Alkaline phosphatase (U/l)
74Aspartate aminotransferase (U/l)
144Alanine aminotransferase (U/l)
1 Conjugated
2 Total
Bilirubin (mg/dl)
Globulin
3.1 Albumin
Total
Protein (g/dl)
5d. Before admission
Variable
CXR
Suggesting presence of pneumonia
of the right lower lobe
Abdominal US
No abnormalities
Progress of the patient
Various pain medications are
ineffective.
transfer to MGH
what to do?
PMH
No informations
Immunizations
His immunizations are up to date
and include viral hepatitis B
vaccination.
FH
No family history of inflammatory
bowel disease or rheumatic disorders
SH
• 17- year- old student
• No history of alcohol or illicit drugs
ROS• Constitutional:
lost 3kg in weight during the preceding two weeks
• GI:stools of normal color
• GU:urine darker than usual
Physical ExaminationVSVS:
Temp.: 37.8 °C
Pulse : 85
BP : 120/55 mm Hg
Resp. : 20
Physical Examination• EyesEyes: mild scleral icterus• ChestChest: supsternal pain• LungsLungs: clear• AbdomenAbdomen:
– soft with slight tenderness in the right upper quadrant– no hepatomegaly
admission testings
LAB (1) BLOOD CHEMICAL AND ENZYME VALUES Variable Five days
before admission
On admission
Protein (g/dl) Total 7.8 Albumin 3.1 2.3 Globulin 5.5 Bilirubin (mg/dl) Total 2 3.1 Conjugated 1 2.6 Cholesterol (mg/dl) 104 Alanine aminotransferase (U/l)
144 108
Aspartate aminotransferase (U/l)
74 119
Alkaline phosphatase (U/l)
601 629
Amylase Normal Lipase Normal
LAB (2)HEMATOLOGIC LABORATORY VALUES
35.3Partial-thromboplastin time (sec)
12.9Prothrombin time (sec)
504,000Platelet count (per mm3)
2 Eosinophils
5 Monocytes
7 Lymphocytes
86 Neutrophils
Differential count (%)
16,000White-cell count (per mm3)
36.8Hematocrit (%)
On admissionVariable
LAB (3)
Variable Result or Value
Screening of serum for illicit drugs Negative Alpha1-antitrypsin (mg/dl) 303 Test for hepatitis A Negative Test for hepatitis B surface antigen Negative Test for hepatitis B surface antibodies Positive Test for hepatitis C antibodies Negative
Assessment
The patient is a 17-year-old boy,who
suffers from epigastrical pain and
intermittently from diarrhea (even
bloody in the past).
Moreover there is evidence of biliary
obstruction.
CXRBilateral prominence of the interstitial
markings
Adominal US• Liver of normal texture• Inrahepatic ducts and the common bile duct of
normal diameter• Partially collapsed gallbladder• Normal pancreas
Stool• Stool specimen positive (+) for occult blood• Microscopical examination:
– excessive number of undigested muscle fibers and abundant yeasts
– no protozoa or helminthic ova
• No C. difficile toxin• No enteric pathogens
Urine• Positive (++) for bile• Minimally positive for urobilinogen• Normal sediment
management?
Management
Ranitidine, clarithromycin and
acetaminophen are given
Progress of the patient
Temp. rises to 39.7 °C
2nd hospital day
• Temp. does not exceed 39°C
• Abdominal pain ceases
exams?
Physical Examination
Unchanged
additional testings
LAB
Variable 2nd hospital day
Calcium (mg/dl) 8.1 Copper (μ g/dl) 1999 Ceroloplasmin (mg/dl) 63 Prothrombin time (sec) 13.5 Partial-thromboplastine time (sec)
36.8
Abdominal US
No abnormalities
CT
CT of the abdomen and pelvis after
oral and iv. administration of
contrast material
no abnormalities
Intestinal disease-differential diagnosis
• Infectious disease
• Celiac sprue
• Inflammatory bowel disease
Infectious disease
The patient´s clinical course and the result
of the limited testing that was performed
make it very improbable that the illness
has an infectious cause.
Celiac sprue
• Unlikely diagnosis in this case because the illness generally developes in adults or in children younger than this patient.
• An acute onset of marked upper gastrointestinal symptoms is atypical of celiac disease.
Inflammatory bowel disease
• The initial signs, symptoms and laboratory findings that suggest inflammatory bowel disease include diarrhea, fever, weight loss, leukocytosis, thrombocytosis and occult blood in the stool.
• Upper gastrointestinal involvement is more common in children with this disease than in adults.
Liver disease-differential diagnosis
• Primary sclerosing cholangitis
• Autoimmune hepatitis
Liver disease-differential diagnosis
• Primary sclerosing cholangitisPrimary sclerosing cholangitis: can involve the extrahepatic ducts, the intrahepatic or both
• Autoimmune hepatitisAutoimmune hepatitis: characteristically involves the hepatic parenchyma
=>both are common in inflammatory bowel disease
Exams
• Evaluation of autoimmune markers• Liver biopsy• Endoscopic retrograde cholangiopancreatography
Autoimmune markers
Variable
Test for antinuclear antibodies Positive (1:180, homogeneous pattern)
Test for anti-smooth – muscle antibodies Positive (1:80) Test for antimitochondrial antibodies Negative
Liver biopsy
The expanded portal tract (arrows) contains a duct surrounded by edema (arrowheads)
Liver biopsy
The pericuctal edema (arrow) results in an onionskin appearance.
There is no inflammation at the interfaces of the portal tracts and
hepatic lobules.
Pathological discussion
• Preservation of the hepatic architecture
• Expansion of the portal tracts, which are rounded and edematous
• Within the portal tracts almost all the interlobular bile ducts are acutely inflamed
• No inflammation at the interfaces of the portal tracts and hepatic lobules
• A singel so-called bile infarct
ERCP
Specimen of the Gastric Fundus. There is a granulomatous
reaction around a damaged gastric gland (arrows).
ERCP
Specimen of the Duodenum. The central duct is acutely inflamed
and ruptured and is surrounded by acute and chronic
inflammation.
Pathological discussion
• No evidence of extrahepatic bile-duct obstruction
• Severe inflammation and an epithelioid granuloma in the gastric wall
• Patchy, superficial inflammation and deep acute and chronic inflammation
Diagnosis
Primary sclerosing cholangitis
associated with Crohn`s disease.
Treatment
• Treatment with prednisone and ursodiol.Later on p. is replaced with mesalamine.
• Patient get`s introduced to the idea that he might be a candidate for liver transplantation (p.s.c.:risk for bile-duct-cancer).
Addendum
• 36 months later the aminotransferase levels are still slightly and the y-glutamyltransferase level is moderately elevated.
• A ERCP showes no change in the degree of narrowing of the intrahepatic ducts.