Berrin IŞIK, Mustafa ARSLAN, Nedim ÇEKMEN

HOMOSİSTİNÜRİDE ANESTEZİK YAKLAŞIM

Homosistinüri otozomal resesif geçiş gösteren, metiyonin metabolizmasında rol oynayan enzim ya da enzimlerin defektinden kaynaklanan ve birden çok sistemik bozuklukla seyreden, metabolik bir hastalıktır. Kliniğinde mental retardasyon, iskelet anomalileri, gözle ilgili problemler, hayatı tehdit eden tromboembolik komplikasyonlar görülür. Homosistinürili olgularda genel anestezi altında oküler cerrahiye sıklıkla ihtiyaç duyulmaktadır. Preoperatif iyi bir hazırlık yapılması, perioperatif tromboemboli ve hipogliseminin önlenmesi ve bunlara yönelik tedbirlerin alınması ile anestezi ve cerrahinin neden olabileceği komplikasyonlar azaltılabilir. Yazımızda homosistinüri tanısı konulmuş iki olgunun oküler cerrahisi sırasında uyguladığımız anestezik yaklaşımları bildirmeyi planladık. Operasyon öncesi ve perioperatif dönemde gerekli önlemleri alarak uyguladığımız anestezi sonucunda her iki olgumuzda da intraoperatif ve postoperatif tromboembolizm, hipoglisemi veya başka bir komplikasyon izlenmemiştir

Anahtar Kelimeler:

Homosistinüri, genel anestezi, tromboembolizm

Anaesthetic Management with Homocystinuria

Homocystinuria is a metabolic and multisystemic disorder often caused by cystatyonin β-sentase deficiency. We have noticed mental retardation, skeletal system malformation, oculer disorders, hypoglycemia and severe thromboembolic complication in this disease. Patients with homocystinüria often needs oculer surgery under general anesthesia. A good preoperative preperation, avoiding hypoglycemia and thromboemboli perioperatively and having precautions about these help to reduce the anesthetic or surgical complication. In our document, we aimed to report our anaesthetic approaches that we applied to two patients with homocystinuria for oculer surgery under general anesthesia. It is possible to prevent the expected complications by the help of precautions that are taken in the preoperative period. No complication about thromboemboli or hypoglycemia is observed during operation or postoperative period in both patients

Keywords:

Homocystinuria, general anesthesia, thromboembolism,

PDF

___

Teng YH, Sung CS, Liao WW, Kao SC. General Anesthesia for Patient with Homocystinuria: A Case Report. Acta Anaesthesiol Sin ,40:153-6. Behrman RE, Kliegman RM, Jenson HB. Nel- son textbook of pediatrics. 16th ed. Pladelphia: W.B. Saunders Co, 2000, pp 351.
Finkelstein JD. Methionine metabolism in mammals. J Nutr Biochem 1990, 1:228-237.
Crooke JW, Towers JF, Taylor WH. Management of patients with homocsytinuria requiring surgery under general anaesthesia. Br J Anaesth 1971, 43:96-99.
Lowe S, Johnson DA, Tobias JD. Anesthetic implications of the child with homocystinuria. J Clin Anesth 1994, 6:142-4.
Mudd SH, Skovby F, Levy HL, et al. The naturel history of homocsytinuria due to cystathionine β-synthase deficiency. Am Hum Genet 1985, :1-37.
Ozand PT, Devol EB, Gascon GG. Neurometabolic diseases at a national refered center. Five years experience at the King Faisal Specialist Hospital and Research Center. J Child Neurol 1992,7:4-11.
Falcon CR, Cattaneo M. High prevalence of hyperhomocystinemia with juvenile venous thrombosis. Arterioscler Thromb 1994, 14:1080
Rees MM, Rodgers GM. Homocsytinuria: Association of metabolic disorder with vascular disease and thrombosis. Thromb Res , 71:337. Harker LA, Ross R, Slichter SJ, Scott CR. Homocystine-induced arteriosclerosis. The role of endothelial cell injury and platelet response in its genesis. J Clin Invest 1976, :731-41.
Badner NH, Drader K, Freeman D, Spence JD. The use of intraoperative nitrous oxide leads to postoperative increases in plasma homocysteine. Anesth Analg 1998, 87:711-3.
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
Foschi D, Rizzi A, Zighetti ML, et al. Effects of surgical stres and nitrous oxide anaesthesia on peri-operative plasma levels of total homocysteine. Anaesthesia 2001, 56:670-89.
Harrison AD, Mullaney PB, Mesfer SA, et al. Management of ophthalmic complications of homocystinuria. Ophthalmology 1998, :1886-90.