Mahmut Alp KARAHAN, Şaban YALÇIN, Harun AYDOĞAN, Hasan Hüsnü YÜCE, Mehmet VURAL, Ahmet KÜÇÜK

Anesthetic management of gaucher disease

Gaucher, lizozomal depo hastalıklarının en sık görülen formu olup, kalıtsal beta glukoserebrozidaz enzim eksikliği nedeni ile oluşur. Gaucher hastalığı, obstetrik durumlarda anormal koagülasyon ve çoklu organ tutulumu nedeniyle anestezistler için zorluk oluşturur. Bu faktörler anestezi türünün seçimini etkileyebilir. Bu olgu sunumunda iki olguda Gaucher hastalığında hastaların özel- liklerine göre değişebilen farklı anestezik yaklaşımların bildirilmesini amaçladık.

Gaucher hastalığında anestezik yaklaşım

Gaucher, the most common form of lysosomal storage diseases, is caused by an inherited deficiency of the beta-glucocerebrosidase enzyme. Gaucher disease may present a challenge to the anesthesiologist because of abnormal coagulation and multiorgan disease in obstetric situations. These factors may affect choice of anesthesia type. We aimed to report the diverse anesthetic management of Gaucher Disease depending on patient s characteristics in two different cases.

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1. Jmoudiak M, Futerman AH. Gaucher disease: Pathological mechanisms and modern management. Br J Haematol 2005;129:178-88
2. Chen M, Wang J. Gaucher disease: Review of the litera- ture. Arch Pathol Lab Med 2008;132:851-3
3. Grabowski G: Recent clinical progress in Gaucher disease. Curr Opin Pediatr 2005;17:519-24
4.Beutler E, Gelbart T. Gaucher disease mutations in non- Jewish patients. Br J Haematol 1993 ;85:4015
5.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
6.DellOste C, Vicenti F. Anesthetic management of chil- dren with type II and III Gaucher disease. Minerva Pediatr 1997; 49: 4958
7.Tobias JD, Atwood R, Lowe S, Holcomb GW, 3rd. Anesthetic considerations in the child with Gaucher disease. J Clin Anesth 1993; 5: 1503
8.Cano Ruiz A, Martin Scarpa A, Montescillo Francia A, Moreno Caparros A. Gauchers disease type I. an infre- quent cause of portal hypertension. An Med Interna 1998; 15: 4834
9.Gillis S, Hyam E, Abrahamov A, Elstein D, Zimran A. Platelet function abnormalities in Gaucher disease pa- tients. Am J Hematol 1999;61: 1036
10.Ioscovich A, Briskin A, Lebel E, et al. Anaesthesia for total hip replacement in Gaucher's disease. Eur J Anaesthesiol. 2006;23:265-6
11.Gielchinsky Y, Elstein D, Hadas-Halpern I, Lahad A, Abrahamov A, Zimran A. Is there a correlation between degree of splenomegaly, symptoms and hypersplenism? A study of 218 patients with Gaucher disease. Br J Haematol 1999; 106: 8126