Retrospective multiinstitutional study of the prevalence of XXXX in @entity3148 .

@entity1 @entity131 @entity410 @entity944 @entity8031 @entity302 @entity3148 @entity204 @entity159
PURPOSE: A questionnaire survey was conducted in Japan to investigate the causes and prevalence of death related to @entity3148 . METHODS: A questionnaire was delivered to 246 hospitals at which physicians were treating childhood @entity302 to gain information about the total number of @entity1 with @entity3148 and their prevalence of @entity204 . KEY FINDINGS: Responses to the survey were collected from 91 hospitals, and a total of 63 of 623 @entity1 with @entity3148 died. Data from 59 of these @entity1 were analyzed. The @entity1 ' ages at death ranged from 13 months to 24 years and 11 months, with a median age of 6 years and 8 months. The analysis showed that the risk of mortality remained high up to approximately 12 years of age. The causes of mortality included @entity944 in 31 @entity1 (53%), @entity131 with @entity159 ( @entity159 ) in 21 @entity1 (36%), drowning in 6 @entity1 (10%), and acute hepatopathy in one @entity1 (1%). The incidence of @entity944 reached a first peak at 1-3 years of age and reached a second peak at 18 years and older. In contrast, the incidence of @entity131 with @entity159 reached a sharp peak at 6 years of age. Seven of 10 @entity1 who underwent an @entity8031 mutation analysis exhibited positive mutations without a specific mutation site. SIGNIFICANCE: In the present study, the prevalence of @entity3148 -related mortality was 10.1%. The incidence of @entity944 and @entity131 with @entity159 was the highest in infancy (1-3 years) and at early school ages (with a peak at 6 years), respectively. After approximately 12 years of age, the risk of mortality declined sharply. Neither the treatment nor the number of @entity410 was associated with any cause of mortality. In addition, it is difficult to predict which factors lead to a fatal outcome.
