Prevalence, Age at Diagnosis, Mortality, and Cause of Death in Retinitis Pigmentosa in Korea—A Nationwide Population-based Study

Kun Hoo Na, Hyun Jung Kim, Kyoung Hoon Kim, Seungjin Han, Patrick Kim, Hoo Jae Hann, Hyeong Sik Ahn

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Abstract

Purpose To determine the prevalence and mortality of retinitis pigmentosa (RP) patients in Korea. Design Population-based retrospective cohort study. Methods We used data covering the 2011–2014 period from the Rare Intractable Disease (RID) registry and Health Insurance Review and Assessment (HIRA) service, which include information on all patients diagnosed with RP based on predefined diagnostic criteria. Using the HIRA-RID database, we evaluated the prevalence and age at diagnosis of RP patients across the entire Korean population. We further linked the data from Statistics Korea to the HIRA-RID database to confirm mortality and causes of death. Results The prevalence in the total population across all ages was 11.09 per 105 people, and the prevalence in those over the age of 40 was 16.16 per 105 people. The age at diagnosis ranged from 0 to 95 and, on average, was 44.8. The standardized mortality ratio (SMR) was 1.56 for all ages, peaking at 2.61 in men aged 40–59, which was attributed to 6.6-fold higher suicide rates than the same age group in the general male population. Conclusions This is the first nationwide epidemiologic study of RP patients covering the entire population of all ages. The results suggest that the prevalence of RP in Korea is about 1 in 9000 for all ages and 1 in 6000 for those over 40 years of age. The higher mortality of RP patients than that of the general population is attributable to a high suicide rate in male RP patients of working ages, which necessitates a careful attention to their mental health.

Original languageEnglish
Pages (from-to)157-165
Number of pages9
JournalAmerican Journal of Ophthalmology
Volume176
DOIs
Publication statusPublished - 2017 Apr 1

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Retinitis Pigmentosa
Cause of Death
Mortality
Population
Health Insurance
Korea
Rare Diseases
Suicide
Databases
Registries
Epidemiologic Studies
Mental Health
Cohort Studies
Retrospective Studies
Age Groups

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Prevalence, Age at Diagnosis, Mortality, and Cause of Death in Retinitis Pigmentosa in Korea—A Nationwide Population-based Study. / Na, Kun Hoo; Kim, Hyun Jung; Kim, Kyoung Hoon; Han, Seungjin; Kim, Patrick; Hann, Hoo Jae; Ahn, Hyeong Sik.

In: American Journal of Ophthalmology, Vol. 176, 01.04.2017, p. 157-165.

Research output: Contribution to journalArticle

Na, Kun Hoo ; Kim, Hyun Jung ; Kim, Kyoung Hoon ; Han, Seungjin ; Kim, Patrick ; Hann, Hoo Jae ; Ahn, Hyeong Sik. / Prevalence, Age at Diagnosis, Mortality, and Cause of Death in Retinitis Pigmentosa in Korea—A Nationwide Population-based Study. In: American Journal of Ophthalmology. 2017 ; Vol. 176. pp. 157-165.
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abstract = "Purpose To determine the prevalence and mortality of retinitis pigmentosa (RP) patients in Korea. Design Population-based retrospective cohort study. Methods We used data covering the 2011–2014 period from the Rare Intractable Disease (RID) registry and Health Insurance Review and Assessment (HIRA) service, which include information on all patients diagnosed with RP based on predefined diagnostic criteria. Using the HIRA-RID database, we evaluated the prevalence and age at diagnosis of RP patients across the entire Korean population. We further linked the data from Statistics Korea to the HIRA-RID database to confirm mortality and causes of death. Results The prevalence in the total population across all ages was 11.09 per 105 people, and the prevalence in those over the age of 40 was 16.16 per 105 people. The age at diagnosis ranged from 0 to 95 and, on average, was 44.8. The standardized mortality ratio (SMR) was 1.56 for all ages, peaking at 2.61 in men aged 40–59, which was attributed to 6.6-fold higher suicide rates than the same age group in the general male population. Conclusions This is the first nationwide epidemiologic study of RP patients covering the entire population of all ages. The results suggest that the prevalence of RP in Korea is about 1 in 9000 for all ages and 1 in 6000 for those over 40 years of age. The higher mortality of RP patients than that of the general population is attributable to a high suicide rate in male RP patients of working ages, which necessitates a careful attention to their mental health.",
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