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• W1489096979 abstract "A patient with Anderson-Fabry disease presented for non-heart beating renal transplantation. A literature search revealed only one published case report on the anaesthetic implications [1]. Anderson-Fabry disease is a rare, panethnic, X-linked lysosomal storage disorder. It is the second commonest of the lysosomal storage disorders, following Gauchers, with an estimated incidence of between 1 in 117 000 in Australia [2] to 1 in 470 000 in the Netherlands [3]. The underlying defect is an inborn deficiency of α-galactosidase A. This leads to an accumulation of glycosphingolipids within the plasma and lysosomes of endothelial and smooth muscle cells. In particular, globotriasylceramide accumulates in the kidneys, myocardium, pancreas and lungs. The clinical features are numerous, but of particular clinical importance are impaired renal and respiratory function (which is significantly exacerbated in smokers), peripheral nervous system disease and cerebrovascular disease. Global concentric ventricular hypertrophy, valvular abnormalities and cardiac conduction disturbance are common in affected males, especially after the age of 30 years. Our patient had an estimated GFR < 15 ml.min−1. He had angiokeratomas, acroparaesthesia, ophthalmic signs and had a history of previous stroke. Pre-operative evaluation also revealed moderate concentric left ventricular hypertrophy. General anaesthesia was induced with fentanyl and propofol, and tracheal intubation facilitated with atracurium. Anaesthesia was maintained with sevoflurane in air and oxygen. Approximately 10 min after induction he developed bronchospasm. His peak airway pressures were elevated, tidal volumes reduced and on auscultation there was a widespread expiratory wheeze; there were no significant changes in his heart rate or blood pressure and oxygen saturations were maintained above 95%. Fibreoptic bronchoscopy revealed frothy sputum and oedematous airway mucosa. These respiratory signs settled spontaneously over the next 15–20 min without any specific intervention and the rest of the intra-operative course was uneventful. A blood sample taken 1 h later was sent for mast cell tryptase, which was normal. The bronchospasm was initially attributed to atracurium, but a further literature search revealed that many patients with Anderson-Fabry disease demonstrate significant airflow obstruction and have intrinsic airway disease. Disease of the airways appears to be significantly worse in smokers and older males [4]. This may account for the transient bronchospam or at least have contributed to it. Postoperatively, there were no thrombotic or respiratory complications, but graft function was delayed for 2 days and he was temporarily dialysed. He is now home and recovering well after a successful transplant. In summary, the pre-operative assessment of Anderson-Fabry disease should concentrate on end-organ damage to the heart, brain, lungs and kidneys. The older the patient, the more likely it is that they will have a significant degree of organ impairment that will require consideration before major surgery. Investigations should include urinalysis, 12-lead ECG, echocardiography, spirometry and a comprehensive renal assessment. Respiratory function should be carefully assessed in those who continue to smoke and pre-operative treatment with hydrocortisone should perhaps be considered. The need for bronchodilators must be anticipated and avoiding drugs and clinical interventions that are commonly associated with histamine release appears sensible. In elective cases we also advocate having a low threshold for noninvasive cardiac stress tests in those > 30 years of age and relevant symptoms." @default.
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• W1489096979 date "2007-12-07" @default.
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• W1489096979 title "Peri-operative considerations for Anderson-Fabry disease" @default.
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• W1489096979 doi "https://doi.org/10.1111/j.1365-2044.2007.05387.x" @default.
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