OBJECTIVE: To analyse the outcome of six @entity1 with @entity14015 ( @entity14015 ) and report the first three living-related liver transplants for this syndrome in Saudi Arabia and the Middle East. SETTINGS: To review the medical records of six @entity1 suffering from @entity14015 , three of whom underwent living-related liver transplantation (LRLT) between 22 November 1998 and January 2001. MAIN RESULTS: Living-related liver transplantation was performed in three @entity1 with a pre-transplant unconjugated @entity648 level of 362, 381 and 502 micromol/L, respectively, despite daily phototherapy of >or= 12 h. Two of the transplanted @entity1 developed acute hepatocellular rejection, which was successfully treated with @entity1667 pulse therapy. One tested cytomegalovirus positive (using the PP65 method), but showed no signs of @entity922 and was treated with @entity4540 . One @entity1 had a biliary leak at the cut surface of the graft which was surgically repaired. Post-operative @entity648 levels returned to normal in all three transplanted @entity1 and no further phototherapy was required. One @entity1 , who was not transplanted but received phototherapy, developed severe @entity16 prior to the start of our living-related liver transplant programme with a @entity648 level of 450 micromol/L, her sister is still awaiting transplantation. A 14-yr-old @entity1 with a @entity648 level of 420 micromol/L is presently undergoing phototherapy whilst awaiting orthotopic liver transplantation because of the lack of a suitable living-related donor. Six siblings of the six @entity1 in our series were reported dead by the families. CONCLUSION: @entity14015 is a relatively common disease in Saudi Arabia for which LRLT is a curative treatment when performed at an early age before the development of @entity16 . In countries where there is a severe shortage of cadaveric organs, as is the case in Saudi Arabia, LRLT is the optimum treatment modality for this syndrome.

Living-related liver transplantation for XXXX in Saudi Arabia.

@entity1 :: ('9606', 'Species') :: ['children', 'patient', 'child'] @entity1667 :: ('MESH:D008775', 'Chemical') :: ['methylprednisolone'] @entity648 :: ('MESH:D001663', 'Chemical') :: ['bilirubin'] @entity16 :: ('MESH:D009422', 'Disease') :: ['neurological damage', 'kernicterus and neurological deficiency'] @entity14015 :: ('MESH:D003414', 'Disease') :: ['CNS-I', 'Crigler-Najjar syndrome type I'] @entity4540 :: ('MESH:D015774', 'Chemical') :: ['ganciclovir'] @entity922 :: ('MESH:D013568', 'Disease') :: ['clinical infection']
@entity14015 :: (MESH:D003414,Disease) :: ['Crigler-Najjar syndrome']
