İ. Aydın ERDEN, Nalan ÇELEBİ, Şennur UZUN, Hüseyin C. TURGUT, Ülkü AYPAR

Homosistinürili hastada anestezi uygulaması komplikasyonların önlenmesi ve tedavisi

Homosistinüri; metiyonin-homosistein metabolizmasında önemli rolü olan enzimlerin eksikliğine bağlı otozomal resesif geçiş gösteren bir hastalıktır. Görme azlığı şikayetiyle göz hastalıkları kliniğine başvuran 8 yaşında kız hastada bilateral ektopia lends tespit edildi. Klinik bulgular ve serum amino asit düzeylerine bakılarak hastaya homosistinüri tanısı konuldu. Homosistein düzeyini azaltmak için hastaya metiyoninden fakir diyet ve medikasyon başlandıktan sonra hasta cerrahiye alındı. Sendromla ilişkili çeşitli komplikasyonların ve mortalitenin önlenmesi için önlemler alındı. Perioperatif ve postoperatif dönemde istenmeyen bir olay gelişmedi, hasta sorunsuz olarak taburcu edildi.

Anaesthesia practice in a patient with homocystinuria preventing and management of complications

Homocystinuria is an autosomal recessive disease associated with the deficiency of enzymes that play important role on metionin-homocystein metabolic process. A 8 year old female child presented with the complaints of diminished vision and was found to have bilateral ectopia lentis. According to clinical findings and serum amino acid levels the patient was diagnosed as homocystinuria. She was started a metionin, reduced diet combined with proper medication to reduce homocysteine levels and then taken up for surgery. Precautions were taken in order to avoid the various complications and mortality associated with this syndrome. During perioperative and postoperative period no complication was detected, the patient was discharged uneventfully.

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1.Rezvani I. Defects in metabolism of amino acids. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF,(eds) Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:529-32.
2.Crooke JW,Towers JF, Taylor WH. Management of patients with homocystinuria requiring surgery under general anaesthesia. Acase report. Br J Anaesth 1971; 43: 96-9.
3.Lowe S, Johnson DA, Tobias JD. Anesthetic implications of the child with homocystinuria. J Clin Anesth 1994; 6: 142-4.
4.Saxena KN, Kapoor S, Chopra N, Dua CK. Anaesthetic management of a case of homocystinuria. Indian J Anaesth 2006; 50 (6): 476-8.
5.McGoldrick KE. Eye, Ear, Nose and Throat Diseases. In: Fleisher L.(ed) Anaesthesia and Uncommon Diseases. 5th ed. Philadelphia Elsevier; 2006: 1-27.
6.Badner NH, Drader K, Freeman D, Spence JD. The use of intraoperative nitrous oxide leads to postoperative increases in plasma homocysteine. Anest Analg 1998; 87: 711-3.
7.Badner NH, Beattie WS, Freeman D, Spencer JD. Nitrous oxide-induced increased homocysteine concentrations are associated with increased postoperative myocardial ischemia in patients undergoing carotid endarterectomy. Anest Analg 2000; 91: 1073-9.