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, 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  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 Examination VS VS: 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)

LAB (2) HEMATOLOGIC LABORATORY VALUES 35.3Partial-thromboplastin time (sec) 12.9Prothrombin time (sec) 504,000Platelet count (per mm 3) 2 Eosinophils 5 Monocytes 7 Lymphocytes 86 Neutrophils Differential count (%) 16,000White-cell count (per mm 3) 36.8Hematocrit (%) On admissionVariable

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  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

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

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.