A study by Forsyth and his associates identified an increased prevalence of severe kidney disease defined as an estimated glomerular filtration rate (eGFR) <45 ml/min/1.73 m2 in individuals with BBS2, BBS10, and BBS12 compared to individuals with BBS1. Also, in the same study, homozygous and compound heterozygous individuals with truncating variants were more likely to be associated with severe kidney disease than those with two missense variants implicating that neither the type of mutation is prognostically negligible. The gene discussed is BBS1; the disease is kidney disorder.