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Hurler Sendromlu Olguda Magnetik Rezonans Görüntüleme Sırasında Anestezi Yaklaşımımız

Anaesthetic Aproach Towards a Patient with Hurler Syndrom in the Magnetic Resonans Imaging Area

Journal Name:

  • İnönü Üniversitesi Tıp Fakültesi Dergisi

Publication Year:

  • 2004

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
Hurler Syndrome is an inherited and congenital disease, disorder of mucopolysaccharide metabolism a-L iduronidase enzyme deficiencyis the causeof the disease which effects many organs and tissues. Present abnormalities accompany to the syndrome frequently cause serious complications during anesthesia which is performed for surgical or diagnostic approaches. One of the major problem for anesthesia is airway difficulty. Multiple factors are present in the mucopolysaccharidoses which make the airway management and trachael intubation potentially hazardous. Cardiovasculary and respiratory pathologies may be the cause of death. In this report we would like to share our experiences, discuss the anesthetic risks and the management of HS patients in the light of literature by presenting our anesthetic approaches towards a 3-year-old patient who is prediagnosed with Hurler Syndrome (Type I Mucopolysaccharidosis).
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Abstract (Original Language): 
Bir mukopolisakkarit metabolizması bozukluğu olan Hurler Sendromu (HS) a-L iduronidase enzim defektinden kaynaklanan, birden fazla doku ve organı etkileyen kalıtsal, konjenital bir hastalıktır. Sendromun klinik seyrinde görülebilecek sorunların saptanması ya da tedavisi amacıyla uygulanacak işlemler sırasında gereken anestezi uygulamalarında mevcut anomaliler nedeni ile ciddi komplikasyonlar yaşanmaktadır. Bu sendromda havayolu güçlükleri en sık karşılaşılan sorundur. Kalp damar ve solunum sistemine ait diğer patolojiler perioperatif mortaliteye neden olabilir. Yazımızda Hurler Sendromu (Tip I Mukopolisakkaridoz) ön tanısı konulmuş olan 3 yaşındaki olguda manyetik rezonans görüntüleme sırasında uyguladığımız anestezi yaklaşımımızı sunarak literatür bilgileri ışığında tartışmayı amaçladık.
FULL TEXT (PDF): 
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  • 4
259-263
  • Turkish

REFERENCES

References: 

1. Hunter CA, A rare disease in two brothers. Proceding of the Royal Society of Medicine, London, 1917;10(15):104-16.
2. Neufeld, E. F., Muenzer, J. (1995) in The Metabolic and Molecular Bases of Inherited Disease (Scriver, C. R., Beaudet, A. L., Sly, W. S., and Valle, D., eds), pp. 2465-508, McGraw-Hill Inc., New York
Hurler
Sendroml
u Olguda Magnetik Rezonans Görüntüleme Sırasında Anestezi Yaklaşımımız
3. Stevenson RE, Howell RR, McKusick VA, Suskind R, Hanson JW, Elliott DE,
Neufeld EF. The ıduronidase
deficien
t mucopolisaccharidoses: clinical and roentgenographic features. Pediatrics 1976:57;111-22.
4. Walker RWM, Colovic M, Robinson DN, Dearlove OR. Postobsructive pulmonary oedema during anaesthesia in children with mucopolysaccharidoses. Paediatr Anaesth 2003:13;441-47.
5. Belani KG, Krivit W, Carpenter BL, Braunlin E, Buckley JJ, Liao JC, Floyd T,
Leonard AS, Summers CG, Levine S, Children with mucopolysaccharidosis: perioperative care, morbidity, mortality, and new findings. J Pediatr Surg. 1993 ;28:403-8; discussion 408-10.
6. Moores C, Rogers G, McKenzie IM, Brown TC. Anaesthesia for children with mucopolysaccharidoses. Anaesth Intensive Care. 1996;24:459-63.
7. Herrick IA, Rhine EJ. The mucopolysaccharidoses and anaesthesia: a report of clinical experience. Can J Anaesth. 1988;35:67-73.
8. Nakayama H, Arita H, Hanaoka K. Anesthesia in a patient with Scheie syndrome.
Masui. 1994;43:1385-8.
9. Peters C, Shapiro EG, Anderson J, Henslee-Downey PJ, Klemperer MR, Cowan MJ, Saunders EF, deAlarcon PA, Twist C, Nachman JB, Hale GA, Harris RE, Rozans
MK, Kurtzberg J, Grayson GH, Williams TE, Lenarsky C, Wagner JE, Krivit W.
Hurler syndrome: II. Outcome of HLA-genotypically identical sibling and HLA-haploidentical related donor bone marrow transplantation in fifty-four children. The Storage Disease Collaborative Study Group. Blood. 1998;91:2601-8. 10. Vellodi, E P Young, A Cooper, J E Wraith, B Winchester, C Meaney, U Ramaswami, A Will. Bone marrow transplantation for mucopolysaccharidosis type I: experience of
two British centres . Arch Dis Child 1997;76:92-9

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