Pandey Ravindra, Kumar Anil, Garg Rakesh, Khanna Puneet, Darlong Vanlal
Department of Anaesthesiology and Intensive Care, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.
J Anaesthesiol Clin Pharmacol. 2011 Apr;27(2):259-61. doi: 10.4103/0970-9185.81839.
Alkaptonuria is a rare inherited genetic disorder of tyrosine metabolism characterized by a triad of homogentisic aciduria, ochronosis, and arthritis. The most common clinical manifestations of ochronosis involve the musculoskeletal, respiratory, airway, cardiovascular, genitourinary, cutaneous, and ocular systems. We report the perioperative anesthetic management of a 56-year-old alkaptonuric patient, with multiple comorbidities scheduled, for revision total hip replacement. A review of her medical history revealed alkaptonuria, hypothyroidism, rheumatoid arthritis, hypertension, diabetes mellitus, and Pott's spine with disc prolapse. We want to highlight the need of thorough preoperative evaluation in patients of alkaptonuria, as it is associated with multiple comorbidities. The systemic involvement should determine the anesthetic plan. Caution should be exercised during positioning to prevent injury to the joints and the spine.
黑尿症是一种罕见的遗传性酪氨酸代谢紊乱疾病，其特征为尿黑酸尿症、褐黄病和关节炎三联征。褐黄病最常见的临床表现累及肌肉骨骼、呼吸、气道、心血管、泌尿生殖、皮肤和眼部系统。我们报告了一名56岁黑尿症患者的围手术期麻醉管理情况，该患者有多种合并症，计划进行全髋关节翻修置换术。回顾其病史发现有黑尿症、甲状腺功能减退、类风湿性关节炎、高血压、糖尿病以及伴有椎间盘突出的波特氏脊柱病。我们想强调，由于黑尿症患者伴有多种合并症，因此对其进行全面的术前评估很有必要。全身受累情况应决定麻醉方案。在摆放体位时应谨慎操作，以防止关节和脊柱受伤。
