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.