Patients with hereditary apolipoprotein AI (apoAI) amyloidosis often have extensive visceral amyloid deposits, and many develop end-stage renal failure as young adults. Solid organ transplantation to replace failing organ function in systemic amyloidosis is controversial due to the multisystem and progressive nature of the disease and the risk of recurrence of amyloid in the graft. We report the outcome of solid organ transplantation, including dual transplants in 4 cases, among 10 patients with apoAI amyloidosis who were followed for a median (range) of 16 (4-28) and 9 (0.2-27) years from diagnosis of amyloidosis and transplantation, respectively. Eight of 10 patients were alive, seven with a functioning graft at censor. Two patients died, one of disseminated cytomegalovirus infection 2 months after renal transplantation and the other of multisystem failure following severe trauma more than 13 years after renal transplantation. The renal transplant of one patient failed due to recurrence of amyloid after 25 years. Amyloid disease progression was very slow and the natural history of the condition was favorably altered in both cases in which the liver was transplanted. Failing organs in hereditary apoAI amyloidosis should be replaced since graft survival is excellent and confers substantial survival benefit.
We present two cases in which Aspergillus infection was identified at a late stage in the clinical evaluation as the cause for a painful, progressive and indolent orbital apex syndrome in the absence of any clinical or radiological sign of sinus involvement. Surgical investigation was undertaken with serious subsequent morbidity. Although treatment is often satisfactory, neurological outcome is without exception poor. A review of the literature has revealed that the risk of such investigations is high, and advice is provided to readers which may allow avoidance of such complications in the future.
A 66-year-old woman presented in October 2011 with bilateral visual failure of subacute onset. She has Rheumatoid Arthritis, treated with methotrexate (since 2002), etanercept (2003–2009). A right knee replacement was revised and underwent 6 washouts for persistent infection. Antibiotics included vancomycin, amoxicillin, and daptomycin. Chloramphenicol 4 g daily was started in June 2011 (14 weeks before visual loss). 10 days after onset of visual loss she had visual acuity of 3/24 (Snellen) bilaterally, hyperaemic optic discs and centrocaecal scotomas. The control Ishihara plate could not be read. Toxic optic neuropathy secondary to chloramphenicol was suspected; chloramphenicol had been stopped on Day 6 of visual symptoms. 4 weeks after stopping chloramphenicol acuity was 6/5 (right), 6/6 (left) and visual fields normal, but Ishihara colour plates impaired at 8/13 bilaterally. At the onset of visual symptoms she developed paraesthesiae of limbs which also improved. Examination showed L5 dermatomal loss to pinprick and abnormal proprioception at the toes. From 1950 to 1988 approximately 40 cases of chloramphenicol optic neuropathy were reported, but only two in the past 12 years. This case highlights the potential pitfalls of older generation antibiotics and unfamiliar adverse effects. This may become more pertinent as antibiotic resistance increase.
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