tively, a bile-stained, purulent subhepatic collection was found.The common bile duct was noted to be necrotic, and no bleedingwas noted when the duct was transected. Histopathological exam-ination later confirmed full thickness necrosis of the common bileduct. The duct was transected and the cut ends both showed fullthickness necrosis on pathological examination. The complete ex-trahepatic portion of the biliary tree was exposed and noted to begangrenous. Because of the patient’s hemodynamic instability andthe lack of viable extrahepatic bile ducts, continuity between theliver and bowel could not be restored. A drainage catheter wasplaced into the confluence of the hepatic ducts and directed into theliver. The catheter was brought out through a separate exit site onthe abdominal wall. A large sump drain was placed into thesubhepatic space and the abdomen closed.

Postoperatively, the patient remained stable. The intrahepaticdrain remained patent and continued to divert the flow of bilesuccessfully.

DISCUSSION

We conducted an extensive search using Medline and wereunable to find any reports of ischemic injury to the common bileduct under similar circumstances. Given its rich blood supply,ischemic necrosis of the common bile duct is not expected to be acommon occurrence in the absence of trauma or surgery. However,four reports of bile duct necrosis in other settings were found.

Makuuchi et al. described a case of extrahepatic bile ductnecrosis after transcatheter hepatic arterial embolization of a he-patic tumor. The mucosa of the common bile duct was describedas necrotic (1). However, complete full thickness necrosis of thecommon bile duct was not reported.

Gateleyet al. described a case of posttraumatic ischemic ne-crosis of the common bile duct in a 34-yr-old patient after blunttrauma. During abdominal exploration, the common bile duct andcommon hepatic ducts were found to be necrotic. The hepatic ductswere viable and a hepaticojejunostomy was performed to restorecontinuity between the liver and bowel (2).

Miyake et al. reported a case of common bile duct cancer withmassive necrosis in a 64-yr-old patient (3). This case representsnecrosis of a large malignant tumor of the common bile duct ratherthan primary necrosis of the common bile duct itself. Wiiget al.reported a case of necrosis of the common bile duct after radiationand chemotherapy for lymphoma of the skeleton. A 39.6-Gy doseof radiation was administered over 33 days as two opposinganterior/posterior fields including T11–L4 and the paravertebrallymph nodes. A necrotic gallbladder and common bile duct weredescribed (4). Of note, the common hepatic and hepatic ducts werespared.

In the case presented, the findings on initial surgical exploration,segmental necrosis of the small bowel and necrosis of the gall-bladder, were consistent with embolic phenomena. Given that thispatient had undergone cardiac catheterization 4 days before ad-mission, we postulate that embolic events secondary to cardiaccatheterization precipitated these intraabdominal injuries. We be-lieve that the necrosis of the extrahepatic biliary system was alsosecondary to arterial emboli that originated during the cardiaccatheterization. During the initial exploration no dissection of theporta hepatis or Kocher maneuver were performed that couldaccount for these findings. The blood supply to the extrahepaticbiliary system is described as axial and consists of the 3 o’clockand 9 o’clock arteries that run along the lateral borders of the duct.The 3 o’clock and 9 o’clock arteries are supplied mainly by the

retroduodenal, right hepatic, gastroduodenal, and retroportal arter-ies. An axial blood supply makes the extrahepatic biliary treesusceptible to ischemic injury (5). Emboli to the hepatic arterycould account for necrosis of the biliary tree seen in this case.

Reprint requests and correspondence: Daniel Char, M.D., 96 AspenDrive East, Woodbury, NY 11797.

REFERENCES

1. Makuuchi M, Sukigara M, Mori T, et al. Bile duct necrosis: Compli-cation of transcatheter hepatic arterial embolization. Radiology 1985;156:331–4.

2. Gately JF, Thomas EJ. Post-traumatic ischemic necrosis of the commonbile duct. Can J Surg 1985;28:32–3.

3. Miyake H, Matsumoto S, Ueda T, et al. Common bile duct cancer withmassive necrosis mimicking choledochal dilatation on CT. Acta Radiol1991;32:4–5.

4. Wiig JN, Telhaug R, Odegaard A, et al. Isolated necrosis of the commonbile duct subsequent to irradiation and chemotherapy. Acta Chir Scand1987;153:701–3.

5. Northover JMA, Terblanche J. A new look at the arterial supply of thebile duct in man and its surgical implications. Br J Surg 1979;66:379–84.

ASYMPTOMATIC ELEVATION OFAMINOTRANSFERASE LEVELS AND FATTY LIVER

SECONDARY TO HETEROZYGOUSHYPOBETALIPOPROTEINEMIA

Aijaz Ahmed, M.D., and Emmet B. Keeffe, M.D.

Division of Gastroenterology, Department of Medicine, StanfordUniversity School of Medicine, Stanford, California

INTRODUCTION

Heterozygous familial hypobetalipoproteinemia (HFBL) withapolipoprotein B (ApoB) deficiency can present as persistentlyelevated aminotransferase levels and/or fatty liver (1–5). Wepresent three asymptomatic patients with unexplained elevation ofaminotransferase levels and fatty liver detected by ultrasound whowere found to have HFBL. Hypocholesterolemia was the initialclue to the diagnosis. Laboratory workup was negative for meta-bolic, immunological, toxic, or drug-induced causes of liver dis-ease. Hepatitis B serological tests were consistent with previousexposure to hepatitis B virus infection in cases 2 and 3, and testsfor hepatitis C were negative in all three cases. These patients wereseen over a 1-yr period in a referral hepatology practice, suggestingthat this entity may be a more common explanation of elevatedaminotransferase levels than is generally appreciated.

CASE REPORT

Case 1

A 26-yr-old asymptomatic man from India was found to haveelevated aminotransferases. He denied use of medications, alcohol,or intravenous drugs. Past medical history was unremarkable.Physical examination was normal. Serum total bilirubin was 0.4mg/dl; alanine aminotransferase (ALT) 176 IU/L (normal, 0–49IU/L); aspartate aminotransferase (AST) 77 IU/L (normal, 0–41

Received Apr. 8, 1998; accepted July 16, 1998.

2598 BRIEF CASE REPORTS AJG – Vol. 93, No. 12, 1998

IU/L); g-glutamyltranspeptidase (GGT) 70 IU/L (normal, 2–65IU/L); and alkaline phosphatase (AP) 54 IU/L (normal, 20–115IU/L). The lipid profile revealed a total cholesterol of 93 mg/dl(normal, 100–199 mg/dl); serum triglycerides 36 mg/dl (normal,35–200 mg/dl); HDL cholesterol 53 mg/dl (normal, 35–95 mg/dl);LDL cholesterol 34 mg/dl (normal, 50–129 mg/dl); VLDL cho-lesterol 7 mg/dl (normal, 5–35 mg/dl); and ApoB,40 mg/dl(normal, 43–128 mg/dl). The patient’s mother had a normal lipidprofile. However, his father had normal liver enzymes but anabnormally low serum total cholesterol and ApoB levels compat-ible with HFBL.

Case 2

A 34-yr-old asymptomatic Chinese man was found to haveelevated aminotransferase levels. He denied drug use. He report-edly consumed a glass of beer once every few months. The phys-ical examination was within normal limits. His past medical his-tory was unremarkable. Serum total bilirubin was 1.2 mg/dl, ALT287 IU/L, AST 185 IU/L, AP 62 IU/L, and GGT 137 IU/L. Thelipid profile revealed a total cholesterol of 80 mg/dl, serum trig-lycerides 53 mg/dl, HDL cholesterol 40 mg/dl, LDL cholesterol 29mg/dl, VLDL cholesterol 11 mg/dl, and ApoB 29 mg/dl.

Case 3

A 48-yr-old asymptomatic Chinese man from Hong Kong wasfound to have elevated aminotransferases. The patient was takingallopurinol 300 mg/day intermittently for gout. He drank 1–2glasses of wine daily for 20 yr. He denied intravenous drug use.The patient had diet-controlled diabetes mellitus. The physicalexamination was within normal limits except for mild hepatomeg-aly. Cutaneous stigmata of chronic liver disease were absent.Fasting blood glucose was 143 mg/dl. Serum total bilirubin was 0.8mg/dl, ALT 58 IU/L, AST 23 IU/L, AP 80 IU/L, and GGT 102IU/L. Lipid profile revealed a total cholesterol of 99 mg/dl, serumtriglycerides 65 mg/dl, HDL cholesterol 57 mg/dl, LDL cholesterol29 mg/dl, and ApoB 34 mg/dl.

DISCUSSION

The constellation of clinical findings including mild elevation ofaminotransferase levels, reduced serum total cholesterol and LDLlevels, low ApoB levels, and presence of fatty infiltration onultrasound were compatible with the diagnosis of HFBL in all threepatients. The patients were followed expectantly and remainasymptomatic 3 yr after detection of elevated aminotransferaselevels. The ALT has ranged from 13 to 287 IU/L and AST from 15to 210 IU/L. The lipid profiles of the family members wereunknown in cases 2 and 3, but the father of case 1 had HFBL.Diabetes mellitus and alcohol use may have contributed to theabnormal liver enzymes and fatty liver in case 3.

HFBL is diagnosed by a characteristic lipid profile in the ab-sence of malabsorption or a disease known to cause secondaryhypobetalipoproteinemia (1–5). Cancer, virus infection, some gas-trointestinal disease, and drug use may result in secondary hypo-betalipoproteinemia. HFBL is an autosomal dominant disorderwith abnormalities of ApoB synthesis. ApoB is a major cholester-ol-carrying protein (6). The lack of ApoB results in decreasedVLDL formation. VLDL transports triglycerides from the liver tothe circulation. The trapping of the triglycerides in the hepatocytecytoplasm results in steatosis (7, 8). Tarugiet al. (3) reported aframe shift mutation in an ApoB gene causing a truncated ApoB to

be associated with fatty liver. Analysis at the gene and/or proteinlevels may help confirm heterozygosity and provide insight intothe pathogenetic mechanism of fatty liver in patients with HFBL.

The long term outcome of fatty liver associated with HFBLappears to be excellent, but needs to be studied further (5, 6). Anargument can be made to avoid liver biopsy in these patients, whenall other virological, toxic, and immunological causes are excludedby history and laboratory testing. We suggest that a complete lipidpanel, including ApoB levels, should be included in the investi-gative workup of persistently elevated aminotransferase levels ofunexplained etiology. An important clue to the diagnosis of HFBLis the finding of hypocholesterolemia on a routine chemistry panel.

Reprint requests and correspondence: Emmet B. Keeffe, M.D., StanfordUniversity Medical Center, 750 Welch Road, Suite 210, Palo Alto, CA94304-1509.

REFERENCES

1. Castellano G, Garfia C, Gomez-Coronado D, et al. Diffuse fatty liver infamilial heterozygous hypobetalipoproteinemia. J Clin Gastroenterol1997;25:379–82.

2. Ogata H, Akagi K, Baba M, et al. Fatty liver in a case with heterozygousfamilial hypobetalipoproteinemia. Am J Gastroenterol 1997;92:339–42.

3. Tarugi P, Lonardo A, Ballarini G, et al. Fatty liver in heterozygoushypobetalipoproteinemia caused by a novel truncated form of apoli-poprotein B. Gastroenterology 1996;111:1125–33.

4. Wishingrad M, Paaso B, Garcia G. Fatty liver due to heterozygoushypobetalipoproteinemia. Am J Gastroenterol 1994;89:1106–7.

5. Hagve TA, Myrseth LE, Schrumpf E, et al. Liver steatosis in hypobe-talipoproteinemia. J Hepatol 1991;13:104–11.

6. Kane JP, Havel RJ. Disorders of biogenesis and secretion of lipoproteinscontaining the B apolipoproteins. In: Scriver CR, Beaudet AL, Sly WS,et al., eds. The metabolic and molecular basis of inherited disease.Volume II. 7th ed. New York: McGraw-Hill, 1995:1853–85.

7. Isselbacher KJ, Scheig R, Plotkin GR, et al. Congenital B-lipoproteindeficiency: An hereditary disorder involving a defect in the absorptionand transport of lipids. Medicine 1964;43:347–61.

8. Avigan MI, Ishak KG, Gregg RE, et al. Morphologic features of liver inabetalipoproteinemia. Hepatology 1984;4:1223–6.

FAMILIAL CROHN’S DISEASE WITH SYSTEMICLUPUS ERYTHEMATOSUS

Yoshiyuki Nishida, M.D., Kunihiko Murase, M.D., Ph.D.,Ryuichi Ashida, M.D., Osamu Sasaki, M.D.,

Yoshiyuki Ozono, M.D., Ph.D., Yohei Mizuta, M.D., Ph.D.,Fumitoshi Takeshima, M.D., Ph.D.,

Kazuya Makiyama, M.D., Ph.D., andShigeru Kohno, M.D., Ph.D.

The Second Department of Internal Medicine and Department ofEndoscopy, Nagasaki University School of Medicine,

Nagasaki, Japan

We describe a young Japanese woman who was diagnosedwith Crohn’s disease affecting the ileum, transverse colon, andrectum, as confirmed by barium studies, colonoscopy, andhistopathological examination. Her father and sister also hadCrohn’s disease. After a 4-yr course of sulfasalazine and ele-mental diet therapy, she was readmitted for perianal abscessassociated with the presence of pancytopenia, microhematuria

Received Apr. 2, 1998; accepted July 22, 1998.

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