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). This evidence concerns the gene BBS1 and kidney disorder.