Platelet and blood transfusion in a child with dyskeratosis congenita for dental extraction – a case report

• DOI: 10.21726/rsbo.v15i2.678
• Publication Date: 2019-02-04T18:57:15Z
• Authors: Sayali Mali; P. Keerthi Kundana; Sunita Singh; Sinika Khanchandani; Milind S. Tullu; Mukesh Agrawal; Adesh Kakade
• Keywords: Dyskeratosis congenita; Mucous membrane; Oral mucosa; Bone marrow failure

Introduction and Objective: Dyskeratosis congenita (DC) also known as Zinsser-Engman- Cole Syndrome is a rare inherited disorder with prevalence of less than one per million. Zinsser et al. described an variant ectodermal dysplasia that affected skin, nails mucous membranes in early 1900s.The syndrome eventually came to be DC classified the bone marrow failure syndromes (IBMFS). association three clinical features: dystrophic nails, oral leukoplakia (white spots on tongue mucosa) abnormal skin pigmentation. Case report Conclusion: This case describes dental management DC. Fluctuating vital blood parameters deteriorating overall health status were major challenges delivering treatment. Dental extractions this patient done while maintaining by platelet transfusion.