Living donor liver transplantation using a graft from a donor with Dubin-Johnson syndrome.
DJS is an autosomal recessive disorder that causes an increase in conjugated bilirubin without elevation of liver enzymes. Most patients are asymptomatic and have normal life spans, but to the best of our knowledge, their livers have never been reported to be grafts in liver transplantation. Herein, we report an infant patient with MMA that received a partial liver graft from his mother, who had DJS. A biliary anastomosis stricture was noted five months after transplantation and was successfully treated with radiological interventions. Otherwise, the patient's liver functions were normal, and a liver biopsy showed a pathognomonic picture of DJS nine months after the transplantation. The patient was followed for one yr, and the results were satisfactory for an increase in oral intake and protein uptake, no recurrence of metabolic stroke and there was a gradual catch-up with regard to physical development despite having a persistently abnormal profile of amino acid analysis. From the experience of our case, we suggest that a liver from a donor with DJS can be used as a graft for liver transplantation, although long-term follow-up is mandatory to examine the grafted liver under the use of immunosuppressive medications.
Liu
Chinsu
C
Division of Pediatric Surgery, Taipei Veterans General Hospital, Taiwan. csliu@vghtpe.gov.tw
Niu
Dau-Ming
DM
Hsia
Cheng-Yuan
CY
Loong
Che-Chuan
CC
Lin
Niang-Cheng
NC
Tsai
Hsin-Lin
HL
Tsou
Mei-Yung
MY
Chin
Taiwai
T
D000328:Adult; D000469:Alkaline Phosphatase / Q000378:metabolism; D001663:Bilirubin / Q000378:metabolism; D001706:Biopsy; D003251:Constriction, Pathologic; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D007566:Jaundice, Chronic Idiopathic / Q000503:physiopathology*; D008099:Liver / Q000002:abnormalities; D017093:Liver Failure / Q000628:therapy; D016031:Liver Transplantation / Q000009:adverse effects / Q000379:methods*; D019520:Living Donors; D008297:Male; D016896:Treatment Outcome; D005723:gamma-Glutamyltransferase / Q000378:metabolism
D007166:Immunosuppressive Agents; D005723:gamma-Glutamyltransferase; D000469:Alkaline Phosphatase; D001663:Bilirubin