Perioperative management of patient with alkaptonuria and associated multiple comorbidities

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...

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Main Authors: Ravindra Pandey, Anil Kumar, Rakesh Garg, Puneet Khanna, Vanlal Darlong
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2011-01-01
Series:Journal of Anaesthesiology Clinical Pharmacology
Subjects:
Online Access:http://www.joacp.org/article.asp?issn=0970-9185;year=2011;volume=27;issue=2;spage=259;epage=261;aulast=Pandey
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author Ravindra Pandey
Anil Kumar
Rakesh Garg
Puneet Khanna
Vanlal Darlong
author_facet Ravindra Pandey
Anil Kumar
Rakesh Garg
Puneet Khanna
Vanlal Darlong
author_sort Ravindra Pandey
collection DOAJ
description 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.
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spelling doaj.art-46f97f0f081044b096c302853620b7912022-12-22T00:03:08ZengWolters Kluwer Medknow PublicationsJournal of Anaesthesiology Clinical Pharmacology0970-91852011-01-0127225926110.4103/0970-9185.81839Perioperative management of patient with alkaptonuria and associated multiple comorbiditiesRavindra PandeyAnil KumarRakesh GargPuneet KhannaVanlal DarlongAlkaptonuria 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.http://www.joacp.org/article.asp?issn=0970-9185;year=2011;volume=27;issue=2;spage=259;epage=261;aulast=PandeyAlkaptonuriaanesthesiacomorbidities
spellingShingle Ravindra Pandey
Anil Kumar
Rakesh Garg
Puneet Khanna
Vanlal Darlong
Perioperative management of patient with alkaptonuria and associated multiple comorbidities
Journal of Anaesthesiology Clinical Pharmacology
Alkaptonuria
anesthesia
comorbidities
title Perioperative management of patient with alkaptonuria and associated multiple comorbidities
title_full Perioperative management of patient with alkaptonuria and associated multiple comorbidities
title_fullStr Perioperative management of patient with alkaptonuria and associated multiple comorbidities
title_full_unstemmed Perioperative management of patient with alkaptonuria and associated multiple comorbidities
title_short Perioperative management of patient with alkaptonuria and associated multiple comorbidities
title_sort perioperative management of patient with alkaptonuria and associated multiple comorbidities
topic Alkaptonuria
anesthesia
comorbidities
url http://www.joacp.org/article.asp?issn=0970-9185;year=2011;volume=27;issue=2;spage=259;epage=261;aulast=Pandey
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AT rakeshgarg perioperativemanagementofpatientwithalkaptonuriaandassociatedmultiplecomorbidities
AT puneetkhanna perioperativemanagementofpatientwithalkaptonuriaandassociatedmultiplecomorbidities
AT vanlaldarlong perioperativemanagementofpatientwithalkaptonuriaandassociatedmultiplecomorbidities