VIEWS: 8 PAGES: 55 POSTED ON: 7/22/2013
CC 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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