Anesthetic Chalenges And Managment In Patient With Ataxia- Telangiectasia-Reference-Cited by-同舟云学术

Anesthetic Chalenges And Managment In Patient With Ataxia- Telangiectasia

Published:2023-12-01 Issue:3 Volume:44 Page:65-71
ISSN:1857-8985
Container-title:PRILOZI
language:en
Short-container-title:

Author:

Jovanovski-Srceva Marija¹²,Kondov Goran³²,Kartalov Andrijan¹²,Georgiev Antonio⁴²,Kuzmanovski Igor⁵²,Osmani Bujar⁶²,Andov Mishel³²,Gavrilovska-Brzanov Aleksandra¹²,Jakupi Nexhati³,Stevic Marija⁷⁸,Stojanoski Sinisa⁹²

Affiliation:

1. 1 University Clinic for Traumatology, Orthopedic Diseases, Anesthesiology, Reanimation , Intensive Care Medicine and Emergency Department, Clinical center Mother Theresa , Skopje , RN Macedonia

2. 2 Faculty of Medicine “Cyril and Methodious,” , Skopje , RN Macedonia

3. 3 University Clinic for Thoraco-vascular surgery , Clinical center Mother Theresa , Skopje , RN Macedonia

4. 4 University Clinic of Cardiology , Skopje , RN Macedonia

5. 5 University Clinic for Neurological Diseases, Clinical center Mother Theresa , Faculty of Medicine “Cyril and Methodious,” , Skopje , RN Macedonia

6. 6 University Clinic for Abdominal surgery , Clinical center Mother Theresa , Skopje , RN Macedonia

7. 7 University Children’s hospital , Belgrade , Serbia

8. 8 Medical Faculty Belgrade , Serbia

9. 9 Institute for Pathophysiology and Nuclear Medicine , Skopje , RN Macedonia

Abstract

Abstract Louis-Bar Syndrome is a synonym for a very rare complex neurodegenerative disorder ataxia-telangiectasia (A-T). This is an autosomal recessive inherited disease that encompasses abnormalities in the cerebellum, multisystem degeneration, immunodeficiency, increased risk for malignancy and consecutive respiratory insufficiency. Most of the patients are radiosensitive and any exposing to ionization may lead to progression of the disease. Potential risks from anesthesia, mechanical ventilation, and postoperative complications in these patients have been insufficiently discussed in the literature. We present a successful anesthetic and respiratory management with one-lung ventilation in a patient with Louis-Bar Syndrome who underwent video assisted thoracoscopy (VATS) for recurrent pleural effusion.

Publisher

Walter de Gruyter GmbH

Link

https://www.sciendo.com/pdf/10.2478/prilozi-2023-0048

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