Oral Clinical Features and Dental Management of Alazami Syndrome. Case Report

Alazami Syndrome is a rare autosomal recessive genetic disorder caused by biallelic pathogenic mutations in the La Ribonucleoprotein 7 gene, initially identified in a Saudi consanguineous family in 2012. This syndrome presents diverse clinical features, including significant growth restriction, intellectual disability, and distinctive facial characteristics, which contribute to its diagnostic profile. Patients with Alazami Syndrome commonly display severe intellectual disability, growth retardation manifesting as primordial dwarfism, and characteristic facial traits such as a triangular-shaped face, prominent forehead, and dental malocclusion. Additional clinical findings may include microcephaly, skeletal anomalies, thickened skin, and neurobehavioral abnormalities, including hypersensitivity to sensory stimuli and autistic-like behaviors. This case report aims to detail the oral clinical features and specific dental management approaches for a patient with Alazami Syndrome, highlighting the challenges and considerations in providing comprehensive care. The findings provide insights into the dental care needs of patients with Alazami Syndrome and underscore the importance of a multidisciplinary approach for optimal patient outcomes.