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Case Studies | Anaesthesiology | India | Volume 13 Issue 8, August 2024 | Rating: 5 / 10
Anaesthesia Management of Shwachman - Diamond Syndrome Child Posted for Dental Rehabilitation
Dr. Jayprakash Galpalli, Dr. Monali Mahajan
Abstract: Shwachman - Diamond syndrome (SDS) is an autosomal recessive disease which results in inherited bone marrow failure (IBMF) and is characterized by pancreatic dysfunction, skeletal abnormalities. and diverse clinical phenotypes. In the present study, we reviewed the internationally published reports on SDS patient, in order to summarize the clinical features, epidemiology, and intraoperative anaesthesia management of SDS. It is the most common cause of pancreatic insufficiency in children after cystic fibrosis. We report a child with classical SDS who was posted for Complete Dental Rehabilitation
Keywords: Shwachman syndrome, Lipomatosis, Pancreatic insufficiency
Edition: Volume 13 Issue 8, August 2024
Pages: 1260 - 1261