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year old woman with acute renal failure and Medizin

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A 17- Year- Old Boy with Biliary
          Obstruction
     HPI- 17 months prior to
      admission to MGH

Symptoms: Bloody diarrhea

  Þ admission to a hospital




Þ what exams to do?
                     LAB
• Serum aspartate aminotransferase level: 75
  U/l
• Test for Clostridium difficile: positive
   Þ had not taken antibiotics before!


Þ management?
          MANAGEMENT

One month course of metronidazole

Þ patient feels well
Several weeks prior to admission
           to MGH
Symptoms:
   – constant crampy, nonradiating
     pain 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
Þadmission to a hospital



Þexams?
                                     LAB
• Leucocytosis with a leftward shift
• 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
                  CXR
Suggesting presence of pneumonia
of the right lower lobe
           Abdominal US


No abnormalities
       Progress of the patient
Various pain medications are
ineffective.
Þ transfer to MGH



Þ 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 Examination
VS:
VS
  Temp.: 37.8 °C
   Pulse : 85
   BP : 120/55 mm Hg
   Resp. : 20
           Physical Examination
•   Eyes: mild scleral icterus
    Eyes
•   Chest: supsternal pain
    Chest
•   Lungs: clear
    Lungs
•   Abdomen:
    Abdomen
     – soft with slight tenderness in the right upper
       quadrant
     – no hepatomegaly


Þadmission testings
LAB (1)
                 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 (3)
               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.
                       CXR
Bilateral 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




 Þ 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




Þexams?
         Physical Examination

Unchanged




Þ additional testings
LAB
            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 cholangitis: can involve the
                      cholangitis
  extrahepatic ducts, the intrahepatic or both
• Autoimmune hepatitis: characteristically involves
                hepatitis
  the hepatic parenchyma

=>both are common in inflammatory
  bowel disease
                   Exams
• Evaluation of autoimmune markers
• Liver biopsy
• Endoscopic retrograde cholangiopancreatography
Autoimmune markers
                   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.

				
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