Patients with co-existing hereditary spherocytosis (HS) and UDP-glucuronosyltransferase 1A1 (UGT1A1) deficiency as Gilbert's syndrome (GS) have been reported, and previous studies have demonstrated an increased risk for developing gallstones in patients with co-inheritance of GS and HS. We experienced an interesting case of HS showing persistent jaundice after splenectomy, and upon further evaluation, the 25-year-old female patient was found to have HS combined with UGT1A1 deficiency. Sequence analysis of the UGT1A1 gene revealed that she was a compound heterozygote with p.[G71R; Y486D] + [Y486D] mutations, which suggests Crigler-Najjar syndrome type II rather than GS. Careful evaluation of inappropriately elevated bilirubin level compared with the degree of hemolysis is important, reflecting the therapeutic implication of splenectomy and cholecystectomy.
Adult ; Crigler-Najjar Syndrome/genetics ; Female ; Glucuronosyltransferase/*deficiency/genetics ; Heterozygote ; Homozygote ; Humans ; Jaundice/etiology/genetics ; Mutation, Missense/genetics ; Point Mutation/genetics ; Spherocytosis, Hereditary/complications/*genetics ; Splenectomy/adverse effects

Extra-anatomical aortic bypass through median sternotomy for aortic recoarctation allows concomitant repair of associated cardiac defects while avoiding potential complications of anatomic repair, including hemorrhage, bleeding from adhesions, nerve damage and spinal cord ischemia. We describe here the case of a 13-year-old boy who presented with aortic regurgitation and aortic recoarctation after two previous anatomic repairs with a prosthetic graft through thoracotomies. Ascending-abdominal aortic bypass and aortic valve replacement (AVR) were performed concomitantly through a median sternotomy. Extra-anatomical aortic bypass through a median sternotomy is useful for aortic recoarctation. Careful observation is required for younger patients.