
Anaesthetic implications for splenectomy in a child with Gaucher’s disease
Author(s) -
R Praneeth,
Kesavakumar Venkatraj
Publication year - 2021
Publication title -
indian journal of clinical anaesthesia
Language(s) - English
Resource type - Journals
eISSN - 2394-4781
pISSN - 2394-4994
DOI - 10.18231/j.ijca.2021.129
Subject(s) - splenectomy , glucocerebroside , medicine , glucocerebrosidase , gaucher's disease , disease , enzyme replacement therapy , general anaesthesia , mononuclear phagocyte system , intensive care medicine , anesthesia , immunology , pediatrics , spleen
Gaucher’s disease, a lysosomal storage disorder, is caused by an inherited deficiency of beta glucocerebrosidase enzyme leading to the accumulation of glucocerebroside in the reticuloendothelial system. Patients with this disease often manifest coagulation abnormalities and multi-organ complications. These factors present a challenge to the anaesthesiologist, in deciding the type as well as the conduct of anaesthesia. We aim to report the anaesthetic management in a 3-years old child with Gaucher’s disease posted for splenectomy.