Case 41

Published on 18/02/2015 by admin

Filed under Allergy and Immunology

Last modified 18/02/2015

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CASE 41

Geoffrey is a 14-month-old child born with biliary atresia (absence of the biliary tree). His parents are advised that the only possibility of cure and a normal life is a liver transplant. Given the shortage of donors, the surgeon discusses the risk/benefit of living related transplants. Both parents are eager to consent, although the best match is deemed to be the father. Surgery involves transplantation of the left lobe of the father’s liver to his son. Both surgeries go well, and both patients make excellent recoveries, returning home within a week after surgery. There are no further complications of the father’s care. Some 4 weeks after the transplant, Geoffrey develops a desquamating, erythematous rash over his trunk, legs, and arms, a low-grade fever, and some diarrhea. His parents return him to the transplant center and are very concerned.

There has been no dramatic change in Geoffrey’s post-transplant immunosuppressive regimen. Blood work looks relatively normal, with some moderate elevation of liver function tests. The diarrhea is quite profuse, although stool cultures are negative for infectious organisms. In fact, blood cultures, urine, induced sputum, and even a lumbar puncture fail to identify an infectious cause of disease, despite evidence for a continuing low-grade fever. During the third hospitalization day he actually becomes quite tachypneic (short of breath), although again a chest radiograph fails to document any obvious pathologic process. He is holding reasonable oxygen saturation only when receiving high-flow 100% oxygen. Of particular concern, you have not been able to discern the cause of the rash, and he is only maintaining normal urine output with vigorous fluid resuscitation. The nurse in the intensive care unit comments that only in burn patients has she seen a rash this bad.

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