21 research outputs found

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    Temporal Bone Pathology in Digeorge's Syndrome

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    International collaborative systematic review of controlled clinical trials on pharmacologic treatments for acute pericarditis and its recurrences

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    Background Acute pericarditis is common, yet uncertainty persists on its treatment. We thus aimed to conduct a comprehensive systematic review on pharmacologic treatments for acute or recurrent pericarditis. Methods Controlled clinical studies were searched in several databases and were included provided they focused on pharmacologic agents for acute pericarditis or its recurrences. Random-effect odds ratios (ORs) were computed for long-term treatment failure, pericarditis recurrence, rehospitalization, and adverse drug effects. Results From 2,078 citations, 7 studies were finally included (451 patients); but only 3 were randomized trials. Treatment comparisons were as follows: colchicine versus standard therapy (3 studies, 265 patients), steroids versus standard therapy (2 studies, 31 patients), low-dose versus high-dose steroids (1 study, 100 patients), and statins versus standard therapy (1 study, 55 patients). Colchicine was associated with a reduced risk of treatment failure (OR = 0.23 [0.11-0.49]) and recurrent pericarditis (OR = 0.39 [0.20-0.77]), but with a trend toward more adverse effects (OR = 5.27 [0.86-32.16]). Overall, steroids were associated with a trend toward increased risk of recurrent pericarditis (OR = 7.50 [0.62-90.65]). Conversely, low-dose steroids proved superior to high-dose steroids for treatment failure or recurrent pericarditis (OR = 0.29 [0.13-0.66]), rehospitalizations (OR = 0.19 [0.06-0.63]), and adverse effects (OR = 0.07 [0.01-0.54]). Data on statins were inconclusive. Conclusions Clinical evidence informing decision-making for the management of acute pericarditis and its recurrences is still limited to few, small, and/or low-quality clinical studies. Notwithstanding such major caveats, available studies routinely using nonsteroidal anti-inflammatory agents in both experimental and control groups suggest a beneficial risk-benefit profile for colchicine and a detrimental one for steroids, especially when used at high dosages

    Spectrum of Digeorge Syndrome in Patients with Truncus Arteriosus - Expanded Digeorge Syndrome

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    A study of 26 patients with truncus arteriosus showed a high prevalence of facial dysmorphism, aortic arch abnormalities, extracardiac malformations, and significant prenatal risk factors. There was little evidence of parathyroid or thymic abnormalities. However, there was laboratory evidence of immune deficiency, especially T-helper lymphocytes, and clinical evidence of predilection to infection. These findings suggest that patients with truncus arteriosus belong to the spectrum of the Di George syndrome
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