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. 2021 Oct 22;16:429. doi: 10.1186/s13023-021-02061-3

Table 3.

Unique patient counts and calculated disease percentages by HCS, and estimates from the medical literature

Total patient count NCATS Eversana Sanford OHSU Med lit
4,333,968 * 1,625,685 1,039,213 N/A
Disease Unique patient count Percentage (%) Unique patient count Percentage (%) Unique patient count Percentage (%) Unique patient count Percentage (%) Estimated prevalence
SCD 10,416 0.2400 38,388 0.0340 55 0.0034% 259 0.0250 0.0308% [38]
MD 2763 0.0640 34,956 0.0313 407 0.0250 709 0.0680 0.0769% [38]
CF 2581 0.0600 13,856 0.0120 246 0.0151 567 0.0550 0.0090% [38]
UCD 1487 0.0340 9423 0.0084 58 0.0036 93 0.0090 0.0029% [39]
HHT 1119 0.0260 21,259 0.0190 28 0.0017 341 0.0330 0.0200% [38]
BD 941 0.0220 7,821 0.0070 3 0.0002 145 0.0140 0.0030% [38]
LGS 917 0.0210 42,837 0.0380 229 0.0141 503 0.0480 0.0001% [38]
FSGS 859 0.0200 11,192 0.0100 128 0.0079 153 0.0150 0.0007% [38]
EOE 755 0.0170 55,055 0.0500 581 0.0357 354 0.0340 0.0500% [38]
OI 573 0.0130 5397 0.0050 73 0.0045 122 0.0120 0.0050% [38]
MNGIE 467 0.0110 7144 0.0060 5 0.0003 66 0.0060 0.0010% [41]
Pheo 250 0.0060 15,521 0.0138 19 0.0012 100 0.0100 0.0005% [40]
TA 117 0.0030 1241 0.0011 14 0.0009 24 0.0020 0.0002% [38]

Unique patient counts by disease extracted from each healthcare system database, and estimated disease percentages within each HCS and for the US population using medical literature/published data sources. Unique patient counts which were used to calculate per patient cost and the total disease cost by disease in Figs. 3, 4

SCD sickle cell disease, MD muscular dystrophy, CF cystic fibrosis, HHT hereditary hemorrhagic telangiectasia, BD Batten disease, LGS Lennox Gastaut syndrome, FSGS focal segmental glomerulosclerosis, EOE eosinophilic esophagitis, OI osteogenesis imperfecta, MNGIE mitochondrial neurogastrointestinal encephalopathy, Pheo pheochromocytoma, TA Takayasu’s arteritis, CMT Charcot Marie Tooth disease, NCATS National Center for Advancing Translational Sciences, OHSU Oregon Health and Science University, Med Lit medical literature/public data sources, N/A not applicable

*Drawn from ~ 195 million patients in the healthcare system database. Total patient count varied by disease, see Table 1