Skeletal and dental manifestations of Glucose-6-phosphate dehydrogenase deficiency, thalassemia, and sickle cell anemia patients in Saudi Arabia.

Abstract

Glucose-6-phosphate dehydrogenase (G6PD) deficiency, Thalassemia (THL), and Sickle cell anemia (SCA) are the most common inherited hematologic diseases globally. It is important to understand the skeletal and dental manifestations in orthodontic treatment. This study aimed at assessing and comparing skeletal and dental manifestations of G6PD deficiency, THL, and SCA patients with healthy controls in Saudi Arabia. This is a retrospective study of G6PD deficient, THL, and SCA patients' cephalometric records in Saudi Arabia. This study included 136 subjects (G6PD=34, THL=34, SCA=34, and healthy control=34), aged between 18-32 years. 17 skeletal, dental, and soft tissue cephalometric measurements obtained from the G6PD, THL, and SCA were compared to control measurements using ANOVA and Tukey's HSD post-hoc test. Intra-class correlation ranged between (ICC: 0.971-0.996), indicating excellent reliability of measurements. A statistically significant difference in skeletal, dental, and soft tissue measurements between each condition and controls was observed, except for Wits, PP-MP, Y-axis, UI-SN, NLA, and LI-E line in SCA, FMA, ANS-Me, UI-SN, and LI-A Pog in G6PD and Wits, Y-axis, UI-SN, UI-NA, LI-MP, LI-Apog and UI-E line in THL group (p˃0.05). SCA and THL patients showed class II skeletal patterns, while class III skeletal relationship was evident in patients with G6PD deficiency.