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Current Anesthesiology Reports
Published in: Current Anesthesiology Reports 3/2019

01-09-2019 | Urea Cycle Disorder | Pediatric Anesthesia (J Lerman, Section Editor)

Metabolic Disorders and Anesthesia

Authors: Cindy Yeoh, Howard Teng, Jacob Jackson, Lee Hingula, Takeshi Irie, Aron Legler, Corrine Levine, Iris Chu, Casey Chai, Luis Tollinche

Published in: Current Anesthesiology Reports | Issue 3/2019

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Abstract

Purpose of Review

Metabolic disorders encompass a group of inherited inborn errors of metabolism that are uncommonly encountered but can pose challenges when encountered during the perioperative period. Hence, it is paramount that anesthesiologists are experienced and familiar with management of these conditions.

Recent Findings

Hundreds of inborn errors of metabolism have already been identified, yet new metabolic disorders continue to be discovered with advancements in genomic science.

Summary

In our general review, we define the more common metabolic disorders encountered in perioperative medicine and discuss the perioperative anesthetic considerations and challenges associated with each disorder. The following disorders are covered in our review: disorders of carbohydrate metabolism, disorders of amino acid metabolism, disorders of branched-chain amino acid metabolism, organic acidemias, mitochondrial disorders, lysosomal storage disorders, metal metabolism disorders, and urea cycle disorders.
Literature
1.
•• Kliegman R, Stanton B, Schor N, St. Geme J III, Behrman R. Nelson Textbook of Pediatrics. 19th ed: Elsevier Health Sciences; 2011. Published in 2011 this is a textbook review of common metabolic disorders
2.
Coelho AI, Rubio-Gozalbo ME, Vicente JB, Rivera I. Sweet and sour: an update on classic galactosemia. J Inherit Metab Dis. 2017;40:325–42.PubMedPubMedCentral
3.
Karadag N, Zenciroglu A, Eminoglu FT, Dilli D, Karagol BS, Kundak A, et al. Literature review and outcome of classic galactosemia diagnosed in the neonatal period. Clin Lab. 2013;59:1139–46.PubMed
4.
Dhillon A, Steadman RH. In: Fleisher LA, editor. Liver diseases, anesthesia and uncommon diseases. 6th ed. Philadelphia: Elsevier Saunders; 2012. p. 162–214.
5.
Choudhury A, Das S, Kiran U. Anaesthetic management of a newborn with galactosaemia for congenital heart surgery. Indian Journal of Anaesthesia. 2009;53:219–22.PubMedPubMedCentral
6.
Stuart G, Ahmad N. Perioperative care of children with inherited metabolic disorders. Continuing Education in Anaesthesia Critical Care & Pain. 2011;11:62–8.
7.
Hammond S, Krol A, Hampson-Evans D. Normoglycaemia in type 1b glycogen storage disease with difficult venous access. Anaesthesia. 2009;64:1150.PubMed
8.
Shenkman Z, Golub Y, Meretyk S, Shir Y, Landau D, Landau EH. Anaesthetic management of a patient with glycogen storage disease type 1b. Can J Anaesth. 1996;43:467–70.PubMed
9.
Bevan JC. Anaesthesia in Von Gierke’s disease. Current approach to management. Anaesthesia. 1980;35:699–702.PubMed
10.
Yu X, Huang Y, Du J. Bispectral index may not reflect the depth of anaesthesia in a patient with glycogen storage disease type I. Br J Anaesth. 2009;103:616.PubMed
11.
Wang LY, Ross AK, Li JS, Dearmey SM, Mackey JF, Worden M, et al. Cardiac arrhythmias following anesthesia induction in infantile-onset Pompe disease: a case series. Paediatr Anaesth. 2007;17:738–48.PubMed
12.
Ing RJ, Cook DR, Bengur RA, Williams EA, Eck J, Dear Gde L, et al. Anaesthetic management of infants with glycogen storage disease type II: a physiological approach. Paediatr Anaesth. 2004;14:514–9.PubMed
13.
Kim WS, Cho AR, Hong JM, Kim ES, Park SC, Yoon JY, et al. Combined general and epidural anesthesia for major abdominal surgery in a patient with Pompe disease. J Anesth. 2010;24:768–73.PubMed
14.
McFarlane HJ, Soni N. Pompe’s disease and anaesthesia. Anaesthesia. 1986;41:1219–24.PubMed
15.
Moses SW, Gadoth N, Bashan N, Ben-David E, Slonim A, Wanderman KL. Neuromuscular involvement in glycogen storage disease type III. Acta Paediatr Scand. 1986;75:289–96.PubMed
16.
Momoi T, Sano H, Yamanaka C, Sasaki H, Mikawa H. Glycogen storage disease type III with muscle involvement: reappraisal of phenotypic variability and prognosis. Am J Med Genet. 1992;42:696–9.PubMed
17.
Olson LJ, Reeder GS, Noller KL, Edwards WD, Howell RR, Michels VV. Cardiac involvement in glycogen storage disease III: morphologic and biochemical characterization with endomyocardial biopsy. Am J Cardiol. 1984;53:980–1.PubMed
18.
Miller CG, Alleyne GA, Brooks SE. Gross cardiac involvement in glycogen storage disease type 3. Br Heart J. 1972;34:862–4.PubMedPubMedCentral
19.
Tada H, Kurita T, Ohe T, Shimomura K, Ishihara T, Yamada Y, et al. Glycogen storage disease type III associated with ventricular tachycardia. Am Heart J. 1995;130:911–2.PubMed
20.
LaBarbera M, Milechman G, Dulbecco F. Premature coronary artery disease in a patient with glycogen storage disease III. J Invasive Cardiol. 2010;22:E156–8.PubMed
21.
Mohart D, Russo P, Tobias JD. Perioperative management of a child with glycogen storage disease type III undergoing cardiopulmonary bypass and repair of an atrial septal defect. Paediatr Anaesth. 2002;12:649–54.PubMed
22.
Bolton SD, Clark VA, Norman JE. Multidisciplinary management of an obstetric patient with glycogen storage disease type 3. Int J Obstet Anesth. 2012;21:86–9.PubMed
23.
Bollig G. McArdle’s disease (glycogen storage disease type V) and anesthesia--a case report and review of the literature. Paediatr Anaesth. 2013;23:817–23.PubMed
24.
Baum V, O’Flaherty J. Anesthesia for genetic, metabolic, and dysmorphic syndromes of childhood, 2nd ed edition. Philadelphia: Lippincott Williams & Wilkins; 2007.
25.
Coleman P. McArdle’s disease. Problems of anaesthetic management for Caesarean section. Anaesthesia. 1984;39:784–7.PubMed
26.
Bollig G, Mohr S, Raeder J. McArdle’s disease and anaesthesia: case reports. Review of potential problems and association with malignant hyperthermia. Acta Anaesthesiol Scand. 2005;49:1077–83.PubMed
27.
Luzardo GE, Karlnoski RA, Williams B, Mangar D, Camporesi EM. Anesthetic management of a parturient with hyperhomocysteinemia. Anesth Analg. 2008;106:1833–6.PubMed
28.
Slote MU, Khan KK, Khan AA, Butt MN. Is homocystinuria a real challenge for anesthetist? Are we making a difference? Saudi J Anaesth. 2018;12:172–3.PubMedPubMedCentral
29.
Chambers JC, McGregor A, Jean-Marie J, Kooner JS. Acute hyperhomocysteinaemia and endothelial dysfunction. Lancet. 1998;351:36–7.PubMed
30.
Al-Obaidi MK, Stubbs PJ, Collinson P, Conroy R, Graham I, Noble MI. Elevated homocysteine levels are associated with increased ischemic myocardial injury in acute coronary syndromes. J Am Coll Cardiol. 2000;36:1217–22.PubMed
31.
Kang SS, Wong PW, Malinow MR. Hyperhomocyst(e)inemia as a risk factor for occlusive vascular disease. Annu Rev Nutr. 1992;12:279–98.PubMed
32.
Rimm EB, Willett WC, Hu FB, Sampson L, Colditz GA, Manson JE, et al. Folate and vitamin B6 from diet and supplements in relation to risk of coronary heart disease among women. JAMA. 1998;279:359–64.PubMed
33.
Vermeulen EG, Stehouwer CD, Twisk JW, van den Berg M, de Jong SC, Mackaay AJ, et al. Effect of homocysteine-lowering treatment with folic acid plus vitamin B6 on progression of subclinical atherosclerosis: a randomised, placebo-controlled trial. Lancet. 2000;355:517–22.PubMed
34.
Dierkes J, Westphal S, Luley C. The effect of fibrates and other lipid-lowering drugs on plasma homocysteine levels. Expert Opin Drug Saf. 2004;3:101–11.PubMed
35.
Zhang Q, Li S, Li L, Li Q, Ren K, Sun X, et al. Metformin treatment and homocysteine: a systematic review and meta-analysis of randomized controlled trials. Nutrients. 2016;8.
36.
Ueland PM, Refsum H. Plasma homocysteine, a risk factor for vascular disease: plasma levels in health, disease, and drug therapy. J Lab Clin Med. 1989;114:473–501.PubMed
37.
Bazzano LA, Reynolds K, Holder KN, He J. Effect of folic acid supplementation on risk of cardiovascular diseases: a meta-analysis of randomized controlled trials. JAMA. 2006;296:2720–6.PubMed
38.
Miller ER 3rd, Juraschek S, Pastor-Barriuso R, Bazzano LA, Appel LJ, Guallar E. Meta-analysis of folic acid supplementation trials on risk of cardiovascular disease and risk interaction with baseline homocysteine levels. Am J Cardiol. 2010;106:517–27.PubMed
39.
Ray JG, Kearon C, Yi Q, Sheridan P, Lonn E. Heart Outcomes Prevention Evaluation I: homocysteine-lowering therapy and risk for venous thromboembolism: a randomized trial. Ann Intern Med. 2007;146:761–7.PubMed
40.
den Heijer M, Willems HP, Blom HJ, Gerrits WB, Cattaneo M, Eichinger S, et al. Homocysteine lowering by B vitamins and the secondary prevention of deep vein thrombosis and pulmonary embolism: a randomized, placebo-controlled, double-blind trial. Blood. 2007;109:139–44.
41.
Behman REKR, Jenson HB. Nelson textbook of paediatrics. 16th ed; 2000.
42.
Yap S, Boers GH, Wilcken B, Wilcken DE, Brenton DP, Lee PJ, et al. Vascular outcome in patients with homocystinuria due to cystathionine beta-synthase deficiency treated chronically: a multicenter observational study. Arterioscler Thromb Vasc Biol. 2001;21:2080–5.PubMed
43.
Teng YH, Sung CS, Liao WW, Kao SC, Huang YY, Tsou MY, et al. General anesthesia for patient with homocystinuria--a case report. Acta Anaesthesiol Sin. 2002;40:153–6.PubMed
44.
Badner NH, Beattie WS, Freeman D, Spence JD. Nitrous oxide-induced increased homocysteine concentrations are associated with increased postoperative myocardial ischemia in patients undergoing carotid endarterectomy. Anesth Analg. 2000;91:1073–9.PubMed
45.
Stoelting RKDS. Anaesthesia and coexisting diseases. 4th ed. Philadelphia: Churchill Livingstone; 2002.
46.
National Institutes of Health Consensus Development P. National Institutes of Health Consensus Development Conference Statement: phenylketonuria: screening and management, October 16-18, 2000. Pediatrics. 2001;108:972–82.
47.
Douglas TD, Jinnah HA, Bernhard D, Singh RH. The effects of sapropterin on urinary monoamine metabolites in phenylketonuria. Mol Genet Metab. 2013;109:243–50.PubMed
48.
Pietz J, Kreis R, Rupp A, Mayatepek E, Rating D, Boesch C, et al. Large neutral amino acids block phenylalanine transport into brain tissue in patients with phenylketonuria. J Clin Invest. 1999;103:1169–78.PubMedPubMedCentral
49.
Lee P, Smith I, Piesowicz A, Brenton D. Spastic paraparesis after anaesthesia. Lancet. 1999;353:554.PubMed
50.
Dal D, Celiker V. Anaesthetic management of a strabismus patient with phenylketonuria. Paediatr Anaesth. 2003;13:740–1.PubMed
51.
Rayadurg V, Uttarwar A, Surve R. Is propofol safe in patients with phenylketonuria? J Neurosurg Anesthesiol. 2018;30:85–6.PubMed
52.
Rivera-Cruz B. Mitochondrial diseases and anesthesia: a literature review of current opinions. AANA J. 2013;81:237–43.PubMed
53.
Rech VC, Feksa LR, Dutra-Filho CS, Wyse AT, Wajner M, Wannmacher CM. Inhibition of the mitochondrial respiratory chain by phenylalanine in rat cerebral cortex. Neurochem Res. 2002;27:353–7.PubMed
54.
Delaney A, Gal Thomas J. Hazards of anesthesia and operation in maple-syrup-urine disease. Anesthesiology. 1976;44:83–6.PubMed
55.
Kahraman S, Ercan M, Akkus O, Ercelen O, Erdem K, Coskun T. Anaesthetic management in maple syrup urine disease. Anaesthesia. 1996;51:575–8.PubMed
56.
Griffiths S, Stuart, G., Alp Karahan, M., & Frazier, D.: Anaesthesia recommendations for patients suffering from Maple syrup urine disease. . 2015
57.
Hamosh A, Kniffin, C., Krasikov, N., & McKusick, V. : Maple syrup urine disease; MSUD. . 2018
58.
Fuentes-Garcia D, Falcon-Arana L. Perioperative management of a patient with maple syrup urine disease. BJA: Br J Anaesth. 2009;102:144–5.PubMed
59.
Vaidyanathan K, Narayanan MP, Vasudevan DM. Organic acidurias: an updated review. Indian J Clin Biochem. 2011;26:319–25.PubMedPubMedCentral
60.
• Baumgartner MR, Horster F, Dionisi-Vici C, Haliloglu G, Karall D, Chapman KA, et al. Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia. Orphanet J Rare Dis. 2014;9:130. Guidelines developed using the SIGN methodology by having professionals on MMA/PA across twelve European countries and the USA gather all the existing evidence, score it according to the SIGN evidence level system, and make a series of conclusive statements supported by an associated level of evidence. PubMedPubMedCentral
61.
Aldubayan SH, Rodan LH, Berry GT, Levy HL. Acute illness protocol for organic acidemias: methylmalonic acidemia and propionic acidemia. Pediatr Emerg Care. 2017;33:142–6.PubMed
62.
Shaikh N, Hashmi MG, Shah C, Dhansura T. Anaesthetic considerations in a patient with methylmalonyl-coenzyme A mutase deficiency. Indian J Anaesth. 2017;61:1018–20.PubMedPubMedCentral
63.
Sharar SR, Haberkern CM, Jack R, Scott CR. Anesthetic management of a child with methylmalonyl-coenzyme A mutase deficiency. Anesth Analg. 1991;73:499–501.PubMed
64.
Arcas-Bellas JJ, Arevalo-Ludena J, Onate ML, Aranzubia M, Alvarez-Rementeria R, Munoz-Alameda L. General anesthesia in an adult female with propionic acidemia: anesthetic considerations. Minerva Anestesiol. 2013;79:313–5.PubMed
65.
Harker HE, Emhardt JD, Hainline BE. Propionic acidemia in a four-month-old male: a case study and anesthetic implications. Anesth Analg. 2000;91:309–11.PubMed
66.
Karagoz AH, Uzumcugil F, Celebi N, Canbay O, Ozgen S. Anesthetic management of a 2-year-old male with propionic acidemia. Paediatr Anaesth. 2006;16:1290–1.PubMed
67.
Rajakumar A, Kaliamoorthy I, Reddy MS, Rela M. Anaesthetic considerations for liver transplantation in propionic acidemia. Indian J Anaesth. 2016;60:50–4.PubMedPubMedCentral
68.
Ryu J, Shin YH, Ko JS, Gwak MS, Kim GS. Intractable metabolic acidosis in a child with propionic acidemia undergoing liver transplantation -a case report. Korean J Anesthesiol. 2013;65:257–61.PubMedPubMedCentral
69.
Baba C, Kasahara M, Kogure Y, Kasuya S, Ito S, Tamura T, et al. Perioperative management of living-donor liver transplantation for methylmalonic acidemia. Paediatr Anaesth. 2016;26:694–702.PubMed
70.
Ktena YP, Ramstad T, Baker EH, Sloan JL, Mannes AJ, Manoli I, et al. Propofol administration in patients with methylmalonic acidemia and intracellular cobalamin metabolism disorders: a review of theoretical concerns and clinical experiences in 28 patients. J Inherit Metab Dis. 2015;38:847–53.PubMedPubMedCentral
71.
Ruzkova K, Weingarten TN, Larson KJ, Friedhoff RJ, Gavrilov DK, Sprung J. Anesthesia and organic aciduria: is the use of lactated Ringer’s solution absolutely contraindicated? Paediatr Anaesth. 2015;25:807–17.PubMed
72.
Weinberg GL, Laurito CE, Geldner P, Pygon BH, Burton BK. Malignant ventricular dysrhythmias in a patient with isovaleric acidemia receiving general and local anesthesia for suction lipectomy. J Clin Anesth. 1997;9:668–70.PubMed
73.
Soberon JR, Elliott CE, Bland KS, Weinberg GL. Peripheral nerve block in a patient with propionic acidemia. Reg Anesth Pain Med. 2014;39:560–1.PubMed
74.
DiMauro S, Schon EA. Mitochondrial respiratory-chain diseases. N Engl J Med. 2003;348:2656–68.PubMed
75.
•• Hsieh VC, Krane EJ, Morgan PG. Mitochondrial Disease and Anesthesia. J Inborn Errors Metab Screen. 2017;5:2326409817707770. Published in 2017, this is a good review of mitochondrial disease and anesthesia.
76.
Muravchick S, Levy RJ. Clinical implications of mitochondrial dysfunction. Anesthesiology. 2006;105:819–37.PubMed
77.
• Niezgoda J, Morgan PG. Anesthetic considerations in patients with mitochondrial defects. Paediatr Anaesth. 2013;23:785–93. Published in 2013, this review provides detailed anesthetic considerations for patients with mitochondrial disorders.PubMedPubMedCentral
78.
Hanley PJ, Ray J, Brandt U, Daut J. Halothane, isoflurane and sevoflurane inhibit NADH:ubiquinone oxidoreductase (complex I) of cardiac mitochondria. J Physiol. 2002;544:687–93.PubMedPubMedCentral
79.
Wolf A, Weir P, Segar P, Stone J, Shield J. Impaired fatty acid oxidation in propofol infusion syndrome. Lancet. 2001;357:606–7.PubMed
80.
Weinberg GL, Palmer JW, VadeBoncouer TR, Zuechner MB, Edelman G, Hoppel CL. Bupivacaine inhibits acylcarnitine exchange in cardiac mitochondria. Anesthesiology. 2000;92:523–8.PubMed
81.
Parikh S, Saneto R, Falk MJ, Anselm I, Cohen BH, Haas R, et al. A modern approach to the treatment of mitochondrial disease. Curr Treat Options Neurol. 2009;11:414–30.PubMedPubMedCentral
82.
Richtsfeld M, Belani KG. Lysosomal storage diseases: past, present, and future. Anesth Analg. 2017;125:716–8.PubMed
83.
Platt FM, d’Azzo A, Davidson BL, Neufeld EF, Tifft CJ. Lysosomal storage diseases. Nat Rev Dis Primers. 2018;4:27.PubMed
84.
Yuasa T, Takenaka T, Higuchi K, Uchiyama N, Horizoe Y, Cyaen H, et al. Fabry disease. J Echocardiogr. 2017;15:151–7.PubMed
85.
Clark BM, Sprung J, Weingarten TN, Warner ME. Anesthesia for patients with mucopolysaccharidoses: comprehensive review of the literature with emphasis on airway management. Bosnian J Basic Med Sci. 18:1–7.
86.
Scarpa M, Lourenco CM, Amartino H. Epilepsy in mucopolysaccharidosis disorders. Mol Genet Metab. 2017;122(s):55–61.
87.
Mueller P, Attenhofer Jost CH, Rohrbach M, Valsangiacomo Buechel ER, Seifert B, Balmer C, et al. Cardiac disease in children and young adults with various lysosomal storage diseases: comparison of echocardiographic and ECG changes among clinical groups. Int J Cardiol Heart Vessel. 2013;2:1–7.PubMedPubMedCentral
88.
Linhart A, Elliott PM. The heart in Anderson-Fabry disease and other lysosomal storage disorders. Heart. 2007;93:528–35.PubMedPubMedCentral
89.
Megens JH, de Wit M, van Hasselt PM, Boelens JJ, van der Werff DB, de Graaff JC. Perioperative complications in patients diagnosed with mucopolysaccharidosis and the impact of enzyme replacement therapy followed by hematopoietic stem cell transplantation at early age. Paediatr Anaesth. 2014;24:521–7.PubMed
90.
Mattioli C, Gemma M, Baldoli C, Sessa M, Albertin A, Beretta L. Sedation for children with metachromatic leukodystrophy undergoing MRI. Paediatr Anaesth. 2007;17:64–9.PubMed
91.
Santamaria F, Montella S, Mirra V, De Stefano S, Andria G, Parenti G. Respiratory manifestations in patients with inherited metabolic diseases. 2013;22:437–53.
92.
Maria Fuller PJM, Hopwood JJ. Epidemiology of lysosomal storage diseases: an overview, Fabry disease: perspectives from 5 years of FOS. Oxford: Oxford PharmaGenesis; 2006.
93.
Gahl WA. Cystinosis, Pediatric Nephrology. Berlin: Springer; 2009.
94.
Schneider JA, Schulman JD. Cystinosis and the Fanconi syndrome. In: Andreoli TDHJF, Fanestil DD, Schultz SG, editors. Physiology of Membrane Disorders. Boston: Springer; 1986.
95.
Cherqui S, Courtoy PJ. The renal Fanconi syndrome in cystinosis: pathogenic insights and therapeutic perspectives. Nat Rev Nephrol. 2017;13:115–31.PubMed
96.
Ioscovich A, Elstein Y, Halpern S, Vatashsky E, Grisaru-Granovsky S, Elstein D. Anesthesia for obstetric patients with Gaucher disease: survey and review. Int J Obstet Anesth. 2004;13:244–50.PubMed
97.
Hernandez-Palazon J. Anaesthetic management in children with metachromatic leukodystrophy. Paediatr Anaesth. 2003;13:733–4.PubMed
98.
Huizing M, Helip-Wooley A, Westbroek W, Gunay-Aygun M, Gahl WA. Disorders of lysosome-related organelle biogenesis: clinical and molecular genetics. Annu Rev Genomics Hum Genet. 2008;9:359–86.PubMedPubMedCentral
99.
Campo M, Berliner N. Hemophagocytic lymphohistiocytosis in adults. Hematol Oncol Clin North Am. 2015;29:915–25.PubMed
100.
• Brissot P, Pietrangelo A, Adams PC, de Graaff B, McLaren CE, Loreal O. Haemochromatosis. Nat Rev Dis Primers. 2018;4:18016. Published in 2018, this is a recent overview of hemochromatosis. PubMed
101.
102.
Zamboni P, Tognazzo S, Izzo M, Pancaldi F, Scapoli GL, Liboni A, et al. Hemochromatosis C282Y gene mutation increases the risk of venous leg ulceration. J Vasc Surg. 2005;42:309–14.PubMed
103.
Ozkurt S, Acikalin MF, Temiz G, Akay OM, Soydan M. Renal hemosiderosis and rapidly progressive glomerulonephritis associated with primary hemochromatosis. Ren Fail. 2014;36:814–6.PubMed
104.
Bacon BR, Adams PC, Kowdley KV, Powell LW, Tavill AS. American Association for the Study of Liver D: diagnosis and management of hemochromatosis: 2011 practice guideline by the American Association for the Study of Liver Diseases. Hepatology. 2011;54:328–43.PubMedPubMedCentral
105.
Moini M, Mistry P, Schilsky ML. Liver transplantation for inherited metabolic disorders of the liver. Curr Opin Organ Transplant. 2010;15:269–76.PubMed
106.
Raichlin E, Daly RC, Rosen CB, McGregor CG, Charlton MR, Frantz RP, et al. Combined heart and liver transplantation: a single-center experience. Transplantation. 2009;88:219–25.PubMed
107.
Barg A, Elsner A, Hefti D, Hintermann B. Total ankle arthroplasty in patients with hereditary hemochromatosis. Clin Orthop Relat Res. 2011;469:1427–35.PubMed
108.
Beaton MD, Adams PC. Prognostic factors and survival in patients with hereditary hemochromatosis and cirrhosis. Can J Gastroenterol. 2006;20:257–60.PubMedPubMedCentral
109.
Kowdley KV, Brandhagen DJ, Gish RG, Bass NM, Weinstein J, Schilsky ML, et al. National Hemochromatosis Transplant R: survival after liver transplantation in patients with hepatic iron overload: the national hemochromatosis transplant registry. Gastroenterology. 2005;129:494–503.PubMed
110.
Gerhard GS, Chokshi R, Still CD, Benotti P, Wood GC, Freedman-Weiss M, et al. The influence of iron status and genetic polymorphisms in the HFE gene on the risk for postoperative complications after bariatric surgery: a prospective cohort study in 1,064 patients. Patient Saf Surg. 2011;5:1.PubMedPubMedCentral
111.
Shander A, Berth U, Betta J, Javidroozi M. Iron overload and toxicity: implications for anesthesiologists. J Clin Anesth. 2012;24:419–25.PubMed
112.
• Bandmann O, Weiss KH, Kaler SG. Wilson’s disease and other neurological copper disorders. Lancet Neurol. 2015;14:103–13. A comprehensive overview of Wilson’s and other copper disorders from 2015. PubMedPubMedCentral
113.
Clayton PT. Inherited disorders of transition metal metabolism: an update. J Inherit Metab Dis. 2017;40:519–29.PubMed
114.
Li K, Lindauer C, Haase R, Rudiger H, Reichmann H, Reuner U, et al. Autonomic dysfunction in Wilson’s disease: a comprehensive evaluation during a 3-year follow up. Front Physiol. 2017;8:778.PubMedPubMedCentral
115.
116.
Grandis DJ, Nah G, Whitman IR, Vittinghoff E, Dewland TA, Olgin JE, et al. Wilson’s Disease and Cardiac Myopathy. Am J Cardiol. 2017;120:2056–60.PubMed
117.
118.
Langley A, Dameron CT. Copper and anesthesia: clinical relevance and management of copper related disorders. Anesthesiol Res Pract. 2013;2013:750901.PubMedPubMedCentral
119.
Kobayashi M. The effects of sevoflurane and isoflurane on hepatic blood flow in man. Masui. 1996;45:281–6.PubMed
120.
Baykal M, Karapolat S. Anesthetic management of a pediatric patient with wilsons disease. J Clin Med Res. 2010;2:99–101.PubMedPubMedCentral
121.
Foubert-Samier A, Kazadi A, Rouanet M, Vital A, Lagueny A, Tison F, et al. Axonal sensory motor neuropathy in copper-deficient Wilson’s disease. Muscle Nerve. 2009;40:294–6.PubMed
122.
Blair NF, Cremer PD, Tchan MC. Urea cycle disorders: a life-threatening yet treatable cause of metabolic encephalopathy in adults. Pract Neurol. 2015;15:45–8.PubMed
123.
• Häberle J, Boddaert N, Burlina A, Chakrapani A, Dixon M, Huemer M, et al. Suggested guidelines for the diagnosis and management of urea cycle disorders. Orphanet J Rare Dis. 2012;7:32. Published in 2012, this comprehensive overview of urea cycle disorders incorporates new trends and emerging therapies. PubMedPubMedCentral
Metadata
Title
Metabolic Disorders and Anesthesia
Authors
Cindy Yeoh
Howard Teng
Jacob Jackson
Lee Hingula
Takeshi Irie
Aron Legler
Corrine Levine
Iris Chu
Casey Chai
Luis Tollinche
Publication date
01-09-2019
Publisher
Springer US
Published in
Current Anesthesiology Reports / Issue 3/2019
Electronic ISSN: 2167-6275
DOI
https://doi.org/10.1007/s40140-019-00345-w

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