Retrospective French nationwide survey of childhood aggressive vascular anomalies of bone, 1988-2009

<p>Abstract</p> <p>Objective</p> <p>To document the epidemiological, clinical, histological and radiological characteristics of aggressive vascular abnormalities of bone in children.</p> <p>Study design</p> <p>Correspondents of the French Society of Childhood Malignancies were asked to notify all cases of aggressive vascular abnormalities of bone diagnosed between January 1988 and September 2009.</p> <p>Results</p> <p>21 cases were identified; 62% of the patients were boys. No familial cases were observed, and the disease appeared to be sporadic. Mean age at diagnosis was 8.0 years [0.8-16.9 years]. Median follow-up was 3 years [0.3-17 years]. The main presenting signs were bone fracture (n = 4) and respiratory distress (n = 7), but more indolent onset was observed in 8 cases. Lung involvement, with lymphangiectasies and pleural effusion, was the most frequent form of extraosseous involvement (10/21). Bisphosphonates, alpha interferon and radiotherapy were used as potentially curative treatments. High-dose radiotherapy appeared to be effective on pleural effusion but caused major late sequelae, whereas antiangiogenic drugs like alpha interferon and zoledrenate have had a limited impact on the course of pulmonary complications. The impact of bisphosphonates and alpha interferon on bone lesions was also difficult to assess, owing to insufficient follow-up in most cases, but it was occasionally positive. Six deaths were observed and the overall 10-year mortality rate was about 30%. The prognosis depended mainly on pulmonary and spinal complications.</p> <p>Conclusion</p> <p>Aggressive vascular abnormalities of bone are extremely rare in childhood but are lifethreatening. The impact of anti-angiogenic drugs on pulmonary complications seems to be limited, but they may improve bone lesions.</p

Carbachol-induced contraction in experimental intestinal atresia.

<p>(<b>A</b>) Typical recording of carbachol dose-response stimulation of contraction in proximal segment (Upper Panel). Proximal segments showed a significant increase in the contractile response starting at 1 µM carbachol (Lower Panel). (<b>B</b>) Typical recording of carbachol dose-response stimulation of contraction in distal segment from the same atresia as in (A) (Upper Panel). Distal segments showed a significant increase in the contractile response starting only at 100 µM carbachol (Lower Panel). n = 8; *p<0.05 as compared to 1 nM carbachol; Kruskal-Wallis analysis of variance. Data are medians, 5–95th percentiles and range. In controls, jejunal segments showed a significant increase in the contractile response starting at 1 µM carbachol (<b>C</b>), whereas ileal segments showed a significant increase in the contractile response starting only at 100 µM carbachol (<b>D</b>). n = 8; *p<0.05 as compared to 1 nM carbachol; Kruskal-Wallis analysis of variance. Data are medians, 5–95th percentiles and range.</p