Journal of Hepatology, 1992; 14: 404-420
404
@ 1992 Elsevier
SciencePublishers B.V. All rights reserved. 0168-8278/92/$05.00
Letters to the Editor
HEPAT 00972
Gilbert’s syndrome - a possible cause of hyper orthotopic liver transplantation Hyperbilirubinemia is a common feature after liver transplantation. There are various possible causes, for example, during episodes of rejection, cholestasis, cholangitis or hemolysis. This is a report on another possible cause of hyperbilirubinemia: Gilbert’s syndrome, transferred by the donor liver. Case report: Liver transplantation was performed in a 39-year-old man for endstage cryptogenic cirrhosis. The post-operative course was uneventful and there were no episodes of rejection. The patient received a triple immunosuppressive regimen consisting of cyclosporine A, methylprednisolone and azathioprine. At discharge the patient had normal liver function tests, with bilirubin levels at 19 pmol/I (1.1 mg/dl). The patient was followed up in our out-patient clinic on a regular basis. Bilirubin levels fluctuated between 17-39 pmol/l (1.0-2.3 mg/dl). Other routine liver function tests were always within normal ranges. At this stage no further examinations were performed, since the patient felt clinically well. Due to an excessive rise in bilirubin levels to 73 pmol/l (4.3 mgldl) 11 months after transplantation further examinations were performed. The remaining routine liver function tests remained within normal ranges and hemolysis was excluded. Gilbert’s syndrome was suspected due to a high proportion of unconjugated bilirubin, 56 pmol/l (3.3 mgldl), after normal ultrasonography and ERCP, and since there were no signs of rejection and no apparent inflammation, cholestasis or infection in liver histology. The diagnosis was subsequently confirmed by a rise in References 1 Owens D, Sherlock S. Diagnosis of Gilbert’s syndrome:
role
reduced caloric intake. Br Med J 1973;3: 559-63. 2 Owens D, Evans J. Population studies on Gilbert’ssyndrome. J Med Genet 1975; 12: 152-7.
irubinemia after
bilirubin from 24 pmol/l (1.4 mg/dl) to 49 PmoUl (2.9 mgldl) while on reduced caloric intake (1). The proportion of conjugated bilirubin remained unchanged at 7 PmoUl (0.4 mgldl). Gilbert’s syndrome is the most common cause of nonhemolytic unconjugated hyperbilirubinemia, with a prevalence of 3-7% (2). Since this disorder often remains undiagnosed it is likely that a number of affected livers are transplanted. Impairment of glucoronide conjugation as a result of post-operative liver cell damage seems unlikely, since excretion is known to be first affected by harvesting injuries, leading to predominantly conjugated hyperbilirubinemia (3). After transplantation, hyperbilirubinemia in patients with Gilbert’s syndrome may be caused by banal infections, fasting or even seasonal fluctuations. Until this disorder is diagnosed, it may account for many distressing and costly examinations. Recognizing this disorder can be delayed by the reduction of unconjugated bilirubin concentrations due to steroid treatments, which are administered in high doses in the early post-transplant period (4). Gilbert’s syndrome should be considered an additional potential cause of hyperbilirubinemia after liver transplantation, and screening for this disorder might be useful. Joachim C. Arnold’, Gerd 0tto2: Thomas Kraus2, Burkhard Kommerell’ and Lorenz Theilmann’ ‘Medizinische Klinik, Abt. Gastr,.wnterologie and ZChirurgische Klinik, Universit& Heidelberg, Bergheimer Str. 58, O-6900 Heidelberg, Federal Republic of Germany
3 Koff RS. Postoperative jaundice. Med Clin N Am 1975; 59: 823-6. 4 Ohkubo H, Okuda K. The nicotinic acid test in constitutional conjugated hyperbilirubinemias and effects of corticosteroids. Hepdtology 1984; 4: 1206-S.
404
@ 1992 Elsevier
SciencePublishers B.V. All rights reserved. 0168-8278/92/$05.00
Letters to the Editor
HEPAT 00972
Gilbert’s syndrome - a possible cause of hyper orthotopic liver transplantation Hyperbilirubinemia is a common feature after liver transplantation. There are various possible causes, for example, during episodes of rejection, cholestasis, cholangitis or hemolysis. This is a report on another possible cause of hyperbilirubinemia: Gilbert’s syndrome, transferred by the donor liver. Case report: Liver transplantation was performed in a 39-year-old man for endstage cryptogenic cirrhosis. The post-operative course was uneventful and there were no episodes of rejection. The patient received a triple immunosuppressive regimen consisting of cyclosporine A, methylprednisolone and azathioprine. At discharge the patient had normal liver function tests, with bilirubin levels at 19 pmol/I (1.1 mg/dl). The patient was followed up in our out-patient clinic on a regular basis. Bilirubin levels fluctuated between 17-39 pmol/l (1.0-2.3 mg/dl). Other routine liver function tests were always within normal ranges. At this stage no further examinations were performed, since the patient felt clinically well. Due to an excessive rise in bilirubin levels to 73 pmol/l (4.3 mgldl) 11 months after transplantation further examinations were performed. The remaining routine liver function tests remained within normal ranges and hemolysis was excluded. Gilbert’s syndrome was suspected due to a high proportion of unconjugated bilirubin, 56 pmol/l (3.3 mgldl), after normal ultrasonography and ERCP, and since there were no signs of rejection and no apparent inflammation, cholestasis or infection in liver histology. The diagnosis was subsequently confirmed by a rise in References 1 Owens D, Sherlock S. Diagnosis of Gilbert’s syndrome:
role
reduced caloric intake. Br Med J 1973;3: 559-63. 2 Owens D, Evans J. Population studies on Gilbert’ssyndrome. J Med Genet 1975; 12: 152-7.
irubinemia after
bilirubin from 24 pmol/l (1.4 mg/dl) to 49 PmoUl (2.9 mgldl) while on reduced caloric intake (1). The proportion of conjugated bilirubin remained unchanged at 7 PmoUl (0.4 mgldl). Gilbert’s syndrome is the most common cause of nonhemolytic unconjugated hyperbilirubinemia, with a prevalence of 3-7% (2). Since this disorder often remains undiagnosed it is likely that a number of affected livers are transplanted. Impairment of glucoronide conjugation as a result of post-operative liver cell damage seems unlikely, since excretion is known to be first affected by harvesting injuries, leading to predominantly conjugated hyperbilirubinemia (3). After transplantation, hyperbilirubinemia in patients with Gilbert’s syndrome may be caused by banal infections, fasting or even seasonal fluctuations. Until this disorder is diagnosed, it may account for many distressing and costly examinations. Recognizing this disorder can be delayed by the reduction of unconjugated bilirubin concentrations due to steroid treatments, which are administered in high doses in the early post-transplant period (4). Gilbert’s syndrome should be considered an additional potential cause of hyperbilirubinemia after liver transplantation, and screening for this disorder might be useful. Joachim C. Arnold’, Gerd 0tto2: Thomas Kraus2, Burkhard Kommerell’ and Lorenz Theilmann’ ‘Medizinische Klinik, Abt. Gastr,.wnterologie and ZChirurgische Klinik, Universit& Heidelberg, Bergheimer Str. 58, O-6900 Heidelberg, Federal Republic of Germany
3 Koff RS. Postoperative jaundice. Med Clin N Am 1975; 59: 823-6. 4 Ohkubo H, Okuda K. The nicotinic acid test in constitutional conjugated hyperbilirubinemias and effects of corticosteroids. Hepdtology 1984; 4: 1206-S.
