Limits...
Survival of Korean Huntington ’ s Disease Patients

View Article: PubMed Central - PubMed

ABSTRACT

Objective: The survival of Huntington’s disease (HD) patients is reported to be 15–20 years. However, most studies on the survival of HD have been conducted in patients without genetic confirmation with the possible inclusion of non-HD patients, and all studies have been conducted in Western countries. The survival of patients with HD in East Asia, where its prevalence is 10–50-fold lower compared with Western populations, has not yet been reported.

Methods: Forty-seven genetically confirmed Korean HD patients from independent families were included in this retrospective medical record review study.

Results: The mean age at onset among the 47 patients was 46.1 ± 14.0 years. At the time of data collection, 25 patients had died, and these patients had a mean age at death of 57.8 ± 13.7 years. The Kaplan-Meier estimate of the median survival from onset in the 47 patients was 14.5 years (95% confidence interval: 12.3–16.6). None of the following factors were associated with the survival time in the univariate Cox regression analysis: gender, age at onset, normal CAG repeat size, mutant CAG repeat size, and the absence or presence of non-motor symptoms at onset.

Conclusion: This is the first Asian study on survival in HD patients. Survival in Korean HD patients may be shorter than that reported for Western populations, or at least is in the lower range of expected survival. A larger longitudinal observation study is needed to confirm the results found in this study.

No MeSH data available.


A: Kaplan-Meier survival curve for the 47 patients. The dotted line indicates median survival. B: Kaplan-Meier survival curves for men (solid line) and women (dotted line).
© Copyright Policy
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC5035942&req=5

f1-jmd-16022: A: Kaplan-Meier survival curve for the 47 patients. The dotted line indicates median survival. B: Kaplan-Meier survival curves for men (solid line) and women (dotted line).

Mentions: At the time of data collection, 25 patients had died with a mean age at death of 57.8 ± 13.7 (range, 36–80) years (Table 1). The Kaplan-Meier estimate of median survival from onset in the 47 patients was 14.5 years [95% confidence interval (CI): 12.3–16.6] (Figure 1A). No difference in survival was observed between genders in the log-rank test (Figure 1B). In the univariate Cox regression analysis, none of the tested variables were associated with the survival time (Table 2).


Survival of Korean Huntington ’ s Disease Patients
A: Kaplan-Meier survival curve for the 47 patients. The dotted line indicates median survival. B: Kaplan-Meier survival curves for men (solid line) and women (dotted line).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5035942&req=5

f1-jmd-16022: A: Kaplan-Meier survival curve for the 47 patients. The dotted line indicates median survival. B: Kaplan-Meier survival curves for men (solid line) and women (dotted line).
Mentions: At the time of data collection, 25 patients had died with a mean age at death of 57.8 ± 13.7 (range, 36–80) years (Table 1). The Kaplan-Meier estimate of median survival from onset in the 47 patients was 14.5 years [95% confidence interval (CI): 12.3–16.6] (Figure 1A). No difference in survival was observed between genders in the log-rank test (Figure 1B). In the univariate Cox regression analysis, none of the tested variables were associated with the survival time (Table 2).

View Article: PubMed Central - PubMed

ABSTRACT

Objective: The survival of Huntington’s disease (HD) patients is reported to be 15–20 years. However, most studies on the survival of HD have been conducted in patients without genetic confirmation with the possible inclusion of non-HD patients, and all studies have been conducted in Western countries. The survival of patients with HD in East Asia, where its prevalence is 10–50-fold lower compared with Western populations, has not yet been reported.

Methods: Forty-seven genetically confirmed Korean HD patients from independent families were included in this retrospective medical record review study.

Results: The mean age at onset among the 47 patients was 46.1 ± 14.0 years. At the time of data collection, 25 patients had died, and these patients had a mean age at death of 57.8 ± 13.7 years. The Kaplan-Meier estimate of the median survival from onset in the 47 patients was 14.5 years (95% confidence interval: 12.3–16.6). None of the following factors were associated with the survival time in the univariate Cox regression analysis: gender, age at onset, normal CAG repeat size, mutant CAG repeat size, and the absence or presence of non-motor symptoms at onset.

Conclusion: This is the first Asian study on survival in HD patients. Survival in Korean HD patients may be shorter than that reported for Western populations, or at least is in the lower range of expected survival. A larger longitudinal observation study is needed to confirm the results found in this study.

No MeSH data available.