XXXX in Macedonia: incidence and outcome after subtotal resection and cranial irradiation.

@entity1 @entity227 @entity410 @entity851 @entity5 @entity4611 @entity1939 @entity1805 @entity1732 @entity976 @entity214 @entity2010
BACKGROUND: @entity976 is a frequent @entity5 in @entity1 with challenging surgical, endocrine, and visual consequences. We evaluated our experience in treating @entity976 and its incidence in Macedonia. METHODS: Thirteen @entity1 (9 male and 4 female) with @entity976 (age 9.55    3.74 years; range 2.90-15.11) who had been treated between 1989 and 2008 in Macedonia were reviewed. RESULTS: initial signs were @entity227 (10 @entity1 ), @entity410 (1), @entity851 (13), and @entity2010 ( @entity2010 ) (2). All @entity1 were subjected to subtotal surgical removal. Cranial irradiation was performed in 12 of the 13 @entity1 , and intracystic @entity1732 was given to one @entity1 . The @entity1 were followed up for 6-229 months (mean    SD: 107.00    74.04 months). All @entity1 had multiple @entity4611 after surgical @entity5 . Body mass index increased from 16.93    6.34 standard deviation scores ( @entity214 ) at diagnosis to 26.33    5.91 @entity214 (P>0.005) at the last follow-up. @entity2010 was permanent in 9 of the 13 @entity1 , and multiple @entity4611 were seen in all @entity1 . Treatment with growth hormone resulted in normalization of adult height from -1.27    1.52 @entity214 at the start of the treatment to -0.13    1.39 @entity214 at the last followup. The final height was not significantly lower than the genetic target height (P>0.005). The permanent deficit was @entity227 : @entity1939 in one or both eyes in 4 @entity1 , @entity1805 in 4, and other defects in 2. Recurrence of the disease was ruled out in one @entity1 after 31 months. No mortality was observed in the observation period of 104.92    76.11 months. CONCLUSIONS: the overall incidence of @entity976 in the period of 1989-2008 in Macedonia was 1.43 per 1 000 000 @entity1 -years. Subtotal resection and systematic irradiation showed good life quality of survivors.
