Significance of extensive XXXX in @entity5684 .

@entity1 @entity30600 @entity5684 @entity2117
BACKGROUND: The importance of early diagnosis in @entity1 with a mild form of @entity5684 should be emphasized. If applied sufficiently early, haematopoietic stem cell transplantation (HSCT) or recombinant enzyme therapy may improve the prognosis. At present, however, diagnosis of the mild form of @entity5684 tends to be delayed, especially in @entity1 with relatively normal intelligence. OBJECTIVES: To investigate the occurrence of @entity30600 in @entity1 with @entity5684 , and to clarify the relationship between the @entity30600 and @entity5684 clinically and histopathologically. METHODS: Seven Japanese @entity1 with @entity5684 who had received HSCT at ages 4-11 years were observed. The cutaneous manifestations of @entity30600 before HSCT were evaluated, and compared with those after HSCT. In two @entity1 , the @entity2117 from the @entity30600 was examined by light and electron microscopy. RESULTS: Pre-HSCT observation revealed that all the @entity1 had an extensive @entity30600 . These were present at birth and have shown no signs of resolution during the post-HSCT period. Electron microscopic findings showed that pigment-bearing dermal melanocytes contained many free melanosomes in stage IV. These were surrounded by extracellular sheaths and encircled by elastic fibres. CONCLUSIONS: Our results indicate a strong clinical correlation between the extensive @entity30600 and @entity5684 . Ultrastructural findings also clearly suggest that the @entity2117 is a long-lasting symptom. The recognition of the extensive @entity30600 is essential as it may lead to early diagnosis in @entity1 with a mild form of @entity5684 .
