Cardiac angiosarcoma.

Despite cardiac metastases are found in about 20% of cancer deaths, the presence of primary cardiac tumors is rare. Most primary tumors are benign, and malignant tumors comprise about 15%. We report a 21-year-old man with fever, dyspnea, and hemoptysis that was diagnosed with angiosarcoma of the right atrium and pulmonary metastasis. Patient was submitted to surgical tumor resection without adjuvant therapy and died four months after diagnosis.

IGF-I, IGFBP-3 and ALS generation test in Turner syndrome.

OBJECTIVES: The pathophysiology of the short stature in girls with Turner syndrome (TS) is not well understood. The "IGF-I generation test" is used to assess the sensitivity to growth hormone. We compared the biochemical response to four days of growth hormone of TS and controls. STUDY DESIGN: Pre-pubertal TS were recruited to participate in the study. Their siblings served as controls. IGF-I, IGFBP-3 and ALS were measured before and 5 days after using hGH (0.05mg/kg/day). Student-t test was used to compare the differences in their responses. RESULTS: Eleven TS (mean age of 8.5+/-2.4) and 11 siblings (6 females and 5 males) (mean age of 7.0+/-2.0) participated in the study. The basal serum levels of IGF-I, IGFBP-3 and ALS were normal and not different between groups (p=0.62 for IGF-I, p=0.91 for IGFBP-3 and p=0.51 for ALS). The IGF-I generation test was positive in all controls and in 10/11 TS. The IGFBP-3 generation test was positive in 6/11 controls and 4/11 TS. After hGH the mean IGFBP-3 was lower in TS than in controls (p=0.08). The ALS response to hGH was not uniform between groups. CONCLUSIONS: The IGF-I and ALS generation test results were not different between controls and TS. The IGFBP-3 results were higher in the control group but more than 50% of tested children did not pass. The IGF-I/IGFBP-3 generation tests, as presently done, did not help in the understanding of the short stature in TS. The use of different GH dosages and number of doses need to be investigated.

The correlation of the IGF-I, IGFBP-3, and ALS generation test to height velocity after 6 months of recombinant growth hormone therapy in girls with Turner syndrome.

BACKGROUND/AIMS: Girls with Turner syndrome (TS) have short stature and benefit from growth hormone therapy (hGH). Some TS present a significant change in height velocity in response to hGH while others have only a mild increment. Our objective was to correlate the response to hGH (height velocity after 6 months of therapy) to biochemical data prior to and after the beginning of hGH to try to define a tool to predict the response to hGH. METHODS: Thirteen TS participated in the study (ages 3.5-14 years). Levels of IGF-I and IGFBP-3 were measured before and 5, 30 and 90 days after starting hGH (0.05 mg/kg/day), ALS levels were measured only prior and after 5 days. RESULTS: The mean height velocity (+/-SD) increased from 4.27 (+/-1.18) cm/year to 8.46 (+/-2.17) cm/year (p=0.0001). There was no correlation between the height velocity encountered and the expected height velocity using published mathematical models. Basal ALS values correlated to height velocity SDS and IGF-I and IGFBP-3, after 90 days, correlated to height velocity. Most of the data was too scattered to be used individually for each patient. CONCLUSIONS: Even though we observed a relationship between biochemical markers and height velocity in TS treated with hGH, the response to hGH therapy in this condition is highly variable.