RESUMO
A 15-year-old girl presented to the pediatrician with complaints of excessive fat distribution on the right side and breast asymmetry. At age 2, she had undergone a left-sided nephrectomy because of a stage III Wilms tumor using a transverse cut supraumbilical approach, followed by systemic chemotherapy and local radiotherapy. In the case of trunk asymmetry, it is questionable which side is deviant. The asymmetry may be an expression of isolated hemihyperthrophy, syndromal hemihypertrophy related to the Wilms tumor, or an expression of left-sided hemiatrophy as a late consequence of radiotherapy, chemotherapy, or surgery. Late clinical manifestations of childhood cancer treatment are difficult to distinguish from other independent diseases but must be considered as explanations for new onset of symptoms in adolescents.
RESUMO
BACKGROUND: Knowledge about the effects of GH treatment on cognitive functioning in children with Prader-Willi syndrome (PWS) is limited. METHODS: Fifty prepubertal children aged 3.5 to 14 yr were studied in a randomized controlled GH trial during 2 yr, followed by a longitudinal study during 4 yr of GH treatment. Cognitive functioning was measured biennially by short forms of the WPPSI-R or WISC-R, depending on age. Total IQ (TIQ) score was estimated based on two subtest scores. RESULTS: During the randomized controlled trial, mean sd scores of all subtests and mean TIQ score remained similar compared to baseline in GH-treated children with PWS, whereas in untreated controls mean subtest sd scores and mean TIQ score decreased and became lower compared to baseline. This decline was significant for the Similarities (P = 0.04) and Vocabulary (P = 0.03) subtests. After 4 yr of GH treatment, mean sd scores on the Similarities and Block design subtests were significantly higher than at baseline (P = 0.01 and P = 0.03, respectively), and scores on Vocabulary and TIQ remained similar compared to baseline. At baseline, children with a maternal uniparental disomy had a significantly lower score on the Block design subtest (P = 0.01) but a larger increment on this subtest during 4 yr of GH treatment than children with a deletion. Lower baseline scores correlated significantly with higher increases in Similarities (P = 0.04) and Block design (P < 0.0001) sd scores. CONCLUSIONS: Our study shows that GH treatment prevents deterioration of certain cognitive skills in children with PWS on the short term and significantly improves abstract reasoning and visuospatial skills during 4 yr of GH treatment. Furthermore, children with a greater deficit had more benefit from GH treatment.