A 17 year old boy with biliary obstruction
Download
1 / 55

A 17- Year- Old Boy with Biliary Obstruction - PowerPoint PPT Presentation


  • 501 Views
  • Uploaded on

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

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about 'A 17- Year- Old Boy with Biliary Obstruction' - mike_john


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript

Hpi 17 months prior to admission to mgh l.jpg
HPI- 17 months prior to admission to MGH

Symptoms: Bloody diarrhea admission to a hospital

 what exams to do?


Slide3 l.jpg
LAB

  • Serum aspartate aminotransferase level: 75 U/l

  • Test for Clostridium difficile: positive

     had not taken antibiotics before!

     management?


Management l.jpg
MANAGEMENT

One month course of metronidazole

 patient feels well


Several weeks prior to admission to mgh l.jpg
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 l.jpg
Five days prior to admission

Symptoms:

  • leftsided pleuritic chest pain

  • dry cough

    admission to a hospital

    exams?


Slide7 l.jpg

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 l.jpg
CXR

Suggesting presence of pneumonia

of the right lower lobe


Abdominal us l.jpg
Abdominal US

No abnormalities


Progress of the patient l.jpg
Progress of the patient

Various pain medications are

ineffective.

 transfer to MGH

 what to do?


Slide11 l.jpg
PMH

No informations


Immunizations l.jpg
Immunizations

His immunizations are up to date

and include viral hepatitis B

vaccination.


Slide13 l.jpg
FH

No family history of inflammatory

bowel disease or rheumatic disorders


Slide14 l.jpg
SH

  • 17- year- old student

  • No history of alcohol or illicit drugs


Slide15 l.jpg
ROS

  • Constitutional:

    lost 3kg in weight during the preceding two weeks

  • GI:stools of normal color

  • GU:urine darker than usual


Physical examination l.jpg
Physical Examination

VS:

Temp.: 37.8 °C

Pulse : 85

BP : 120/55 mm Hg

Resp. : 20


Physical examination17 l.jpg
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 l.jpg

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 l.jpg
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 l.jpg
CXR

Bilateral prominence of the interstitial

markings


Adominal us l.jpg
Adominal US

  • Liver of normal texture

  • Inrahepatic ducts and the common bile duct of normal diameter

  • Partially collapsed gallbladder

  • Normal pancreas


Stool l.jpg
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 l.jpg
Urine

  • Positive (++) for bile

  • Minimally positive for urobilinogen

  • Normal sediment

 management?


Management26 l.jpg
Management

Ranitidine, clarithromycin and

acetaminophen are given


Progress of the patient27 l.jpg
Progress of the patient

Temp. rises to 39.7 °C


2nd hospital day l.jpg
2nd hospital day

  • Temp. does not exceed 39°C

  • Abdominal pain ceases

    exams?


Physical examination29 l.jpg
Physical Examination

Unchanged

 additional testings



Abdominal us31 l.jpg
Abdominal US

No abnormalities


Slide32 l.jpg
CT

CT of the abdomen and pelvis after

oral and iv. administration of

contrast material

 no abnormalities


Intestinal disease differential diagnosis l.jpg
Intestinal disease-differential diagnosis

  • Infectious disease

  • Celiac sprue

  • Inflammatory bowel disease


Infectious disease l.jpg
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 l.jpg
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 l.jpg
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 l.jpg
Liver disease-differential diagnosis

  • Primary sclerosing cholangitis

  • Autoimmune hepatitis


Liver disease differential diagnosis38 l.jpg
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 l.jpg
Exams

  • Evaluation of autoimmune markers

  • Liver biopsy

  • Endoscopic retrograde cholangiopancreatography



Liver biopsy l.jpg
Liver biopsy

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


Liver biopsy42 l.jpg
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 l.jpg
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 l.jpg
ERCP

Specimen of the Gastric Fundus. There is a granulomatous

reaction around a damaged gastric gland (arrows).


Slide45 l.jpg
ERCP

Specimen of the Duodenum. The central duct is acutely inflamed

and ruptured and is surrounded by acute and chronic

inflammation.


Pathological discussion46 l.jpg
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 l.jpg
Diagnosis

Primary sclerosing cholangitis

associated with Crohn`s disease.


Treatment l.jpg
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 l.jpg
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.


ad