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Case of a young man with abdominal pain and jaundice
Accepted Manuscript Case of a young man with abdominal pain and jaundice Yuichiro Rikitake, MD, Shuhei Fukunaga, MD, Hirohiko Abe, MD
PII: DOI: Reference:
S0016-5085(17)35917-6 10.1053/j.gastro.2017.06.063 YGAST 61299
To appear in: Gastroenterology Accepted Date: 11 June 2017 Please cite this article as: Rikitake Y, Fukunaga S, Abe H, Case of a young man with abdominal pain and jaundice, Gastroenterology (2017), doi: 10.1053/j.gastro.2017.06.063. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Case of a young man with abdominal pain and jaundice Yuichiro Rikitake MD1, Shuhei Fukunaga MD2, and Hirohiko Abe MD1 Kumamoto Central Hospital. 955 Muro, Ozu-machi, Kikuchi-gun, Kumamoto
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1
869-1235, Japan
Division of Gastroenterology, Department of Medicine, Kurume University School of
SC
2
M AN U
Medicine. 67 Asahi-machi Kurume, Fukuoka 830-0011, Japan Conflict of interest statement: We have nothing to disclose. Patient consent: Obtained.
Correspondence to: Dr. Shuhei Fukunaga
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Contributors: YR wrote this paper. YR, SF and HA treated the patient.
EP
Division of Gastroenterology, Department of Medicine
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Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan Phone: +81-942-35-3311 Fax: +81-942-34-2623 E-mail: [email protected]
ACCEPTED MANUSCRIPT
Question: A 20-year-old man was admitted to our department with intermittent cramping pain in
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the right upper abdomen and yellow skin. He was a non-smoker and did not drink alcohol. Vital signs on initial examination were within the normal limits, except for his
SC
high fever. Laboratory data were as follows: total serum bilirubin level 6.5 mg/dL,
M AN U
direct serum bilirubin level 3.9 mg/dL, aspartate aminotransferase level 78 U/L, alanine aminotransferase level 120 U/L, gamma-glutamyltransferase level 207 U/L, and serum alkaline phosphatase level 296 U/L. Results for hepatitis B surface antigen and anti-hepatitis C virus were negative. The levels of tumor markers assessed, including
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carcinoembryonic antigen and cancer antigen 19-9, were within the normal ranges. Abdominal sonography and magnetic resonance cholangiopancreatography revealed
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cholecystolithiasis and choledocholithiasis. After extensive preparation, we decided to
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perform cholecystectomy and choledocholithotomy. We observed a black liver during the operation (Fig A). What is the diagnosis?
ACCEPTED MANUSCRIPT
Answer to the Image: Dubin–Johnson syndrome A liver biopsy was performed. Fig B shows the normal lobular architecture and coarse
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dark-brown pigmented granules scattered among hepatocytes and cytoplasm. Both the clinical and liver biopsy features were consistent with a diagnosis of Dubin–Johnson
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syndrome (DJS). This syndrome is also known as congenital non-hemolytic jaundice
M AN U
with hyperbilirubinemia, and is a rare disorder of bilirubin metabolism and autosomal recessive inheritance. It was first reported by Dubin and Johnson in 1954 [1], and correlates with multidrug resistance-associated protein 2 [2]. In this case, 65 gallstones and two bile duct stones were observed, and all were composed of cholesterol (Fig C).
TE D
Shaffer et al. reported that increased cholesterol secretion and reduced rates of bile salt and phospholipid secretion may cause cholesterol gallstones in DJS[3], but the role of
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DJS in cholecystolithiasis remains unclear. When a patient with DJS complains of
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abdominal pain, it is important to perform abdominal sonography to confirm cholecystolithiasis and choledocholithiasis.
ACCEPTED MANUSCRIPT
References 1. Dubin IN, Johnson FB. Chronic idiopathic jaundice with unidentified pigment in liver
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cells; a new clinicopathologic entity with a report of 12 cases. Medicine (Baltimore) 1954;33:155-197.
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2. Kartenbeck J, Leuschner U, Mayer R, et al. Absence of the canalicular isoform of the
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MRP gene-encoded conjugate export pump from the hepatocytes in Dubin-Johnson syndrome. Hepatology 1996;23:1061-1066.
3. Shaffer EA, Small DM. Biliary lipid secretion in cholesterol gallstone disease. The
AC C
EP
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effect of cholecystectomy and obesity. J Clin Invest 1977;59:828-840.
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SC
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ACCEPTED MANUSCRIPT
PII: DOI: Reference:
S0016-5085(17)35917-6 10.1053/j.gastro.2017.06.063 YGAST 61299
To appear in: Gastroenterology Accepted Date: 11 June 2017 Please cite this article as: Rikitake Y, Fukunaga S, Abe H, Case of a young man with abdominal pain and jaundice, Gastroenterology (2017), doi: 10.1053/j.gastro.2017.06.063. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Case of a young man with abdominal pain and jaundice Yuichiro Rikitake MD1, Shuhei Fukunaga MD2, and Hirohiko Abe MD1 Kumamoto Central Hospital. 955 Muro, Ozu-machi, Kikuchi-gun, Kumamoto
RI PT
1
869-1235, Japan
Division of Gastroenterology, Department of Medicine, Kurume University School of
SC
2
M AN U
Medicine. 67 Asahi-machi Kurume, Fukuoka 830-0011, Japan Conflict of interest statement: We have nothing to disclose. Patient consent: Obtained.
Correspondence to: Dr. Shuhei Fukunaga
TE D
Contributors: YR wrote this paper. YR, SF and HA treated the patient.
EP
Division of Gastroenterology, Department of Medicine
AC C
Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan Phone: +81-942-35-3311 Fax: +81-942-34-2623 E-mail: [email protected]
ACCEPTED MANUSCRIPT
Question: A 20-year-old man was admitted to our department with intermittent cramping pain in
RI PT
the right upper abdomen and yellow skin. He was a non-smoker and did not drink alcohol. Vital signs on initial examination were within the normal limits, except for his
SC
high fever. Laboratory data were as follows: total serum bilirubin level 6.5 mg/dL,
M AN U
direct serum bilirubin level 3.9 mg/dL, aspartate aminotransferase level 78 U/L, alanine aminotransferase level 120 U/L, gamma-glutamyltransferase level 207 U/L, and serum alkaline phosphatase level 296 U/L. Results for hepatitis B surface antigen and anti-hepatitis C virus were negative. The levels of tumor markers assessed, including
TE D
carcinoembryonic antigen and cancer antigen 19-9, were within the normal ranges. Abdominal sonography and magnetic resonance cholangiopancreatography revealed
EP
cholecystolithiasis and choledocholithiasis. After extensive preparation, we decided to
AC C
perform cholecystectomy and choledocholithotomy. We observed a black liver during the operation (Fig A). What is the diagnosis?
ACCEPTED MANUSCRIPT
Answer to the Image: Dubin–Johnson syndrome A liver biopsy was performed. Fig B shows the normal lobular architecture and coarse
RI PT
dark-brown pigmented granules scattered among hepatocytes and cytoplasm. Both the clinical and liver biopsy features were consistent with a diagnosis of Dubin–Johnson
SC
syndrome (DJS). This syndrome is also known as congenital non-hemolytic jaundice
M AN U
with hyperbilirubinemia, and is a rare disorder of bilirubin metabolism and autosomal recessive inheritance. It was first reported by Dubin and Johnson in 1954 [1], and correlates with multidrug resistance-associated protein 2 [2]. In this case, 65 gallstones and two bile duct stones were observed, and all were composed of cholesterol (Fig C).
TE D
Shaffer et al. reported that increased cholesterol secretion and reduced rates of bile salt and phospholipid secretion may cause cholesterol gallstones in DJS[3], but the role of
EP
DJS in cholecystolithiasis remains unclear. When a patient with DJS complains of
AC C
abdominal pain, it is important to perform abdominal sonography to confirm cholecystolithiasis and choledocholithiasis.
ACCEPTED MANUSCRIPT
References 1. Dubin IN, Johnson FB. Chronic idiopathic jaundice with unidentified pigment in liver
RI PT
cells; a new clinicopathologic entity with a report of 12 cases. Medicine (Baltimore) 1954;33:155-197.
SC
2. Kartenbeck J, Leuschner U, Mayer R, et al. Absence of the canalicular isoform of the
M AN U
MRP gene-encoded conjugate export pump from the hepatocytes in Dubin-Johnson syndrome. Hepatology 1996;23:1061-1066.
3. Shaffer EA, Small DM. Biliary lipid secretion in cholesterol gallstone disease. The
AC C
EP
TE D
effect of cholecystectomy and obesity. J Clin Invest 1977;59:828-840.
AC C
EP
TE D
M AN U
SC
RI PT
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EP
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