Epidemiology and patient journey of Rett syndrome in the United States: a real-world evidence study

May, Damian; Kponee-Shovein, Kalé; Mahendran, Malena; Downes, Nathaniel; Sheng, Kristy; Lefebvre, Patrick; Cheng, Wendy Y.

doi:10.1186/s12883-023-03181-y

BMC Neurology

Table 2 Annual Incidence of RTT among Enrollees between November 1, 2017 and October 31, 2019,^a Overall and by Sex

From: Epidemiology and patient journey of Rett syndrome in the United States: a real-world evidence study

Incidence estimate^b	Overall^c		Female		Male
Incidence estimate^b	2018	2019	2018	2019	2018	2019
At-risk enrollees^d	34,336,947	35,994,590	19,567,060	20,439,789	14,760,187	15,544,934
Incident RTT cases^e	1,167	840	840	631	327	209
Annual incidence proportion estimate per 10,000 enrollees	0.34	0.23	0.43	0.31	0.22	0.13

Abbreviations: RTT Rett Syndrome, ICD-10-CM International Classification of Diseases, 10th Revision, Clinical Modification
^aThe study period spanned from November 1, 2017 to October 31, 2019 for the incidence estimation, as data from the time prior served as a washout period to define the at-risk population and exclude prevalent patients with RTT. Individual years were defined as starting from November 1 of the prior year to October 31 of the year described. For example, year 2018 begins on November 1, 2017 and ends on October 31, 2018
^bA minimum 6-month washout period was used to ensure at-risk enrollees and incident cases did not have any RTT diagnosis ≥ 6 months prior to the start of the study period (November 1 of each year), extending to the start of clinical activity for each enrollee
^cIncludes enrollees identified as female, male, and of unknown sex
^dAt-risk enrollees were identified as enrollees with clinical activity at the start of the study period (November 1 of each year of interest) and with no prior diagnosis of RTT during the washout period. The at-risk enrollee count was calculated by subtracting the number of enrollees with a diagnosis of RTT from the total number of enrollees in the IQVIA database in each given year
^ePatients with ≥ 1 medical claim with a diagnosis of RTT and no prior diagnosis code for RTT during the washout period were considered as incident cases. RTT was identified using the following ICD-10-CM diagnosis codes in any position: F84.2

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ISSN: 1471-2377

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