Estimated prevalence and survival rate of Tuberous Sclerosis in Hong Kong

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Abstract Summary
Abstract ID :
HAC252
Submission Type
Authors (including presenting author) :
Chu CY* (1) , Chiang LLW* (1) , Chan DCC* (1) , Wong WHS (1), Chan GCF (1)
Affiliation :
(1) Department of Pediatrics and Adolescent Medicine, Queen Mary Hospital, Hong Kong
Introduction :
Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder, involving mutation in one of the two TSC genes (TSC1 and TSC2). This mutation leads to upregulation of cell proliferative mechanisms and neoplastic overgrowth, hence causing a number of complications such as epilepsy, developmental delay and kidney dysfunction etc. In Western population, several population-based epidemiological studies of TSC can be found. However, similar local demographic data is lacking. Therefore, we reviewed the prevalence and survival rate of TSC patients in Hong Kong.
Objectives :
To elucidate the disease burden by providing a more comprehensive account of the prevalence and survival rate of TSC patients in Hong Kong.
Methodology :
TSC patients with documented visits to hospitals under the Hong Kong Hospital Authority (HA) between 1995 to 2018 were captured with the International Classification of Diseases (ICD)-9 code of 759.5. Demographics such as age, sex, death were retrieved. Secondary data analysis was conducted to determine the prevalence, age distribution and survival rate of TSC patients in Hong Kong.
Result & Outcome :
Results: We identified a total of 284 surviving TSC patients (55.3% male) in the HA patient database. The age range of local TSC patients was from 4.5 months to 89.9 years, with a median age of 27.2 years (pediatrics to adult ratio was 1:2.84). The overall prevalence of TSC patients was 3.87 in 100,000 (i.e. 1 in 26,455). A total of 37 patients died within the study period. The age of death ranged from 7.6 years to 77.8 years, with a median death age of 36.6 years (IQR: 24.7-51.1 years). Most patients survived till adulthood, the survival rate at 20 and 50 years old was 98.6% and 79.5% respectively. Conclusion: The prevalence of local TSC patients is comparable to other Western studies. Local TSC patients have a good survival rate, but develop a number of serious complications throughout their lifetime. Therefore, further research on patient’s quality of life and a complete patient registry with relevant information is necessary. Such a registry will be of paramount importance for professionals to have a more thorough understanding of the disease, hence facilitates future planning for the care of TSC patients.

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