a 17 year old boy with biliary obstruction l.
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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

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hpi 17 months prior to admission to mgh
HPI- 17 months prior to admission to MGH

Symptoms: Bloody diarrhea admission to a hospital

 what exams to do?

slide3
LAB
  • Serum aspartate aminotransferase level: 75 U/l
  • Test for Clostridium difficile: positive

 had not taken antibiotics before!

 management?

management
MANAGEMENT

One month course of metronidazole

 patient feels well

several weeks prior to admission to mgh
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
Five days prior to admission

Symptoms:

  • leftsided pleuritic chest pain
  • dry cough

admission to a hospital

exams?

slide7

BLOOD CHEMICAL AND ENZYME VALUES

Variable

5d. Before admission

Protein (g/dl)

Total

Albumin

3.1

Globulin

Bilirubin (mg/dl)

Total

2

Conjugated

1

Alanine aminotransferase (U/l)

144

Aspartate aminotransferase (U/l)

74

Alkaline phosphatase (U/l)

601

LAB
  • Leucocytosis with a leftward shift
slide8
CXR

Suggesting presence of pneumonia

of the right lower lobe

abdominal us
Abdominal US

No abnormalities

progress of the patient
Progress of the patient

Various pain medications are

ineffective.

 transfer to MGH

 what to do?

slide11
PMH

No informations

immunizations
Immunizations

His immunizations are up to date

and include viral hepatitis B

vaccination.

slide13
FH

No family history of inflammatory

bowel disease or rheumatic disorders

slide14
SH
  • 17- year- old student
  • No history of alcohol or illicit drugs
slide15
ROS
  • Constitutional:

lost 3kg in weight during the preceding two weeks

  • GI:stools of normal color
  • GU:urine darker than usual
physical examination
Physical Examination

VS:

Temp.: 37.8 °C

Pulse : 85

BP : 120/55 mm Hg

Resp. : 20

physical examination17
Physical Examination
  • Eyes: mild scleral icterus
  • Chest: supsternal pain
  • Lungs: clear
  • Abdomen:
    • soft with slight tenderness in the right upper quadrant
    • no hepatomegaly

admission testings

lab 2

HEMATOLOGIC LABORATORY VALUES

Variable

On admission

Hematocrit (%)

36.8

White-cell count (per mm3)

16,000

Differential count (%)

Neutrophils

86

Lymphocytes

7

Monocytes

5

Eosinophils

2

Platelet count (per mm3)

504,000

Prothrombin time (sec)

12.9

Partial-thromboplastin time (sec)

35.3

LAB (2)
assessment
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.

slide22
CXR

Bilateral prominence of the interstitial

markings

adominal us
Adominal US
  • Liver of normal texture
  • Inrahepatic ducts and the common bile duct of normal diameter
  • Partially collapsed gallbladder
  • Normal pancreas
stool
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
Urine
  • Positive (++) for bile
  • Minimally positive for urobilinogen
  • Normal sediment

 management?

management26
Management

Ranitidine, clarithromycin and

acetaminophen are given

progress of the patient27
Progress of the patient

Temp. rises to 39.7 °C

2nd hospital day
2nd hospital day
  • Temp. does not exceed 39°C
  • Abdominal pain ceases

exams?

physical examination29
Physical Examination

Unchanged

 additional testings

abdominal us31
Abdominal US

No abnormalities

slide32
CT

CT of the abdomen and pelvis after

oral and iv. administration of

contrast material

 no abnormalities

intestinal disease differential diagnosis
Intestinal disease-differential diagnosis
  • Infectious disease
  • Celiac sprue
  • Inflammatory bowel disease
infectious 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
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
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
Liver disease-differential diagnosis
  • Primary sclerosing cholangitis
  • Autoimmune hepatitis
liver disease differential diagnosis38
Liver disease-differential diagnosis
  • Primary sclerosing cholangitis: can involve the extrahepatic ducts, the intrahepatic or both
  • Autoimmune hepatitis: characteristically involves the hepatic parenchyma

=>both are common in inflammatory bowel disease

exams
Exams
  • Evaluation of autoimmune markers
  • Liver biopsy
  • Endoscopic retrograde cholangiopancreatography
liver biopsy
Liver biopsy

The expanded portal tract (arrows) contains a duct surrounded by edema (arrowheads)

liver biopsy42
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
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
slide44
ERCP

Specimen of the Gastric Fundus. There is a granulomatous

reaction around a damaged gastric gland (arrows).

slide45
ERCP

Specimen of the Duodenum. The central duct is acutely inflamed

and ruptured and is surrounded by acute and chronic

inflammation.

pathological discussion46
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
Diagnosis

Primary sclerosing cholangitis

associated with Crohn`s disease.

treatment
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
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.