We report on a patient with Sedaghatian type spondylometaphyseal dysplasia (SSMD) who presented with metaphyseal dysplasia, congenital atrioventricular block, simplified gyral pattern, hypogenesis of corpus callosum, and severe cerebellar hypoplasia. We want to emphasize that in this rare congenital lethal skeletal dysplasia with unknown etiology, central nervous system malformations might be a major component of the disorder and should be evaluated in detail to possibly uncover the underlying pathophysiology.
During acute phase, patients with KD have reduced global left ventricular S and SR which may be more sensitive indicators of myocardial inflammation. This study showed gradual improvements in left ventricular myocardial function during treatment for KD.
Urinary LT-E4 and PG-F2a were both significantly higher in children with migraine during headache than during non-headache periods. The elevation in the levels of these inflammatory mediators was compatible with the hypothesis relating neuroinflammation in trigeminal vascular blood vessels with migraine pathophysiology. Leukotriene antagonists may be effective in the prophylaxis of migraine attacks.
The objective was to compare the efficacy and safety of naproxen (NXN) to acetylsalicylic acid (ASA) in the treatment of acute rheumatic fever (ARF). The data of 338 children were retrospectively analyzed. The patients were grouped according to joint and valve involvement and also drug chosen [methyl prednisolone (mPSL), ASA or NXN]. The treatment results and adverse events in each group were compared. The mean age was 10.3 years and the median follow-up was 62 months. Median time for normalization of acute phase reactants was 1 week in patients given steroids and 2 weeks in patients given ASA or NXN. ASA was replaced with NXN in 18 patients (10.2%) due to hepatic toxicity. The rate of rebound, recurrence and the prevalence of rheumatic valve disease were not different in patients given NXN, ASA or mPSL. In conclusion, NXN is a safe and effective alternative to ASA in the treatment of ARF in children.
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