BBS1 and Insulin resistance: Manifest a more pronounced clinical phenotype when compared to those with mutations in the BBS1 geneDeleterious variants display more severe phenotypes (3)Often present with early-onset visual impairmentMore commonly associated with polydactyly and renal anomalies than their counterparts with mutations in the BBS1 gene (25)Correlate with a higher prevalence of visceral obesity and insulin resistance (26, 27)(ages 2 to 11) higher BMI than children with BBS1 gene mutation (26)Severe kidney disease is correlated pathogenic variants in BBS10 gene (predominantly truncating variants) (11)