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Washington University Experience | NEURODEGENERATION | Wilson Disease | 1A0 Case 1 History
Case 1 History ---- The patient was a 27yo male with 7 year history of Wilson's disease who presented to BH for an orthotopic liver transplant. He was diagnosed with Wilson's disease after a liver biopsy in 1984 when he presented with splenomegaly and thrombocytopenia. The patient was treated with various copper chelating agents which failed to control the progression of his CNS disease which included increasing dysphasia, aphasia and ataxia. He also had a history of spina bifida with autonomic dysfunction including chronic constipation/fecal impaction and a neurogenic bladder. On 10/15 he was admitted to BH and received an orthotopic liver transplant the same day. Postoperatively he was hemodynamical stable and T-tube bile drainage was normal. Immunosuppressive therapy initially included Immuran, Cyclosporin A, and Solumedrol. His postoperative liver function tests remained elevated and a liver biopsy on 10/22 showed mild rejection. On 10/22 the patient's temperature spiked to 38oC and wound and bile cultures grew enterococcal species and antibiotic therapy was initiated. Blood and urine cultures were negative and a chest x ray showed no change from previous studies. An abdominal/pelvic CT scan and sinus films were negative. On 10/27 OKT3 immunotherapy was begun secondary to increased graft rejection. Preoperative liver function tests showed an alkaline phosphatase of 68, gamma GT 39, SGPT 76 and SGOT 66. On 10/29 a wound culture grew Enterococcus and the patient was started on Vancomycin and Gentamicin. A liver biopsy performed on 11/4 showed mild rejection and on 11/5 FK506 immunotherapy was initiated. At this time the patient's liver function tests showed total bilirubin 2.6, SGOT 177, SGPT 151, gamma GT 63.5, alkaline phosphatase 38.1 and PT 14.1. On 11/13 the patient fell out of bed trying to go to the bathroom. On 11/15 a liver biopsy was performed to assess rejection and was interpreted as mild rejection. On 11/17 the patient was not feeling well and had episodes of vomiting. He was also noted to be in respiratory distress. At this time his abdomen was tense and very distended. His blood pressure by Doppler was 50-60 systolic. The patient was intubated and blood pressure was supported with Dopamine. On 11/18 the patient became clinically hypotensive with severe metabolic acidosis secondary to what was thought to represent an intra-abdominal hemorrhage. The patient was rushed to the OR where massive intra -abdominal hemorrhage as noted and he died.
