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Two cases of dyssegmental dysplasia, a rare lethal skeletal dysplasia in the newborn, are reported. Two different forms of dyssegmental dysplasia can be distinguished - the lethal Silverman type and the less severe Rolland-Desbuquois type. In this report, both cases are of the Silverman type. Histopathology of chondro-osseous tissue confirms the similarity in pathologic findings to Kniest dysplasia. [ABSTRACT FROM AUTHOR]

Purpose: Elevated heart rate (70 beats per minute-bpm or more) is a predictor of impaired prognosis in patients with ischemic heart failure. The Austrian Working Group on Heart Failure has established a registry in May 2006 for all patients referred to dedicated heart failure clinics with a planned follow-up after 12 ± 3 months. Here we report an analysis of the prognostic impact of elevated heart rate at referral in a well-defined cohort of heart failure patients., Methods: Between May 2006 and October 2009 1904 patients have been documented in the Austrian Heart Failure Registry. One thousand threehundred and sixty three patients (72%) had sinus rhythm at referral. Kaplan-Meier and Cox proportional hazards regression analyses were used to compare overall and cardiovascular mortality between high (70 bpm or more) and low heart-rate groups. Patients who were lost-to-follow-up (n = 166) were censored at the time of last contact., Results: At baseline in 793 patients (58%) heart rate has been elevated (70 bpm or more) while in 562 patients it has been below 70 bpm, in 8 patients no baseline heart rate has been recorded. Groups were equally balanced regarding age, gender and cardiovascular risk factors with the exception of smokers (more active smokers in the high heart-rate group: 23 vs 14%; p = 0.001) and valvular cause of heart failure (more frequent in the high heart-rate group: 3% vs 1%; p = 0.012). Patients in the high heart-rate group had significantly higher median NT-pro-BNP (1470 pg/ml, IQR 499-4188 pg/ml) compared to patients in the low heart-rate group (784 pg/ml, IQR 314-2162 pg/ml; p < 0.001). NYHA functional classes III and IV have been more frequent in the high heart-rate group than in the low heart-rate group (32% and 22%, respectively; p < 0.001) while reduced left ventricular ejection fraction (39% or less) has been more frequent in the high heart-rate group than in the low heart-rate group (71% and 61%, respectively; p < 0.001). In the high heart-rate group treatment with beta-blockers has been less frequent than in the low heart rate group (76% and 86%, respectively; p < 0.01) while dosage of beta-blocker therapy has been comparable in both groups. Of the 75 patients who died within 3.5 years 38 deaths had a cardiovascular cause. Cox proportional hazards analysis revealed that high NYHA functional class (III and IV) and elevated heart rate (70 bpm or more) were the best predictors of overall mortality while cardiovascular mortality could best be predicted by NYHA functional classes III and IV., Conclusion: Higher NYHA-functional classes and elevated heart rate are predictors of adverse outcome in chronic heart failure patients.

Objective: To investigate the extent and time course of pain intensity upon treatment with a topical diclofenac patch compared with placebo in acute traumatic sport injury based on a validated and established end point., Methods: Post hoc analysis of a randomized, placebo-controlled, double-blind, multicentre, 1-week study in 120 patients with traumatic blunt soft tissue injury. Visual analogue scale (VAS) scores (in millimetres) for pain on movement were analysed. The mean absolute VAS changes in pain intensity from baseline over the study course were calculated for the diclofenac patch formulation (active) and placebo; mean differences between active and placebo were assessed twice daily during the first 3 days after enrolment and then once daily up to day 7., Results: The diclofenac patch was consistently superior to placebo in relieving pain. The mean differences compared with placebo were greatest on day 2 (23.6 - 30.6 mm, p < 0.0001) and day 3 (24.5 - 24.6 mm, p < 0.0001). Diminishing differences were observed over the study course., Conclusion: The investigated diclofenac sodium patch provides clinically relevant pain relief in patients with acute traumatic injuries. Maximum effects versus placebo are detected at 2 - 3 days post-injury. This analysis may serve as useful information for the planning of clinical trials in acute traumatic injury.