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While in adults major advances in heart failure therapy in patients with dilated cardiomyopathy were documented in the last two decades, research on the mechanism and therapies of heart failure in children with left ventricular dilated cardiomyopathy has lagged behind. Despite the lack of sufficient randomized prospective studies, ACE inhibitors are first line and ß-receptor antagonists are second-line strategies in children. Following the adult guidelines, without having data concerning the pediatric population, mineral corticoids are also accepted in the treatment of pediatric heart failure, while diuretics should only be used to achieve a euvolemic status. In cases of complete left bundle bunch block or prolonged QRS duration, cardiac resynchronization is an option. If these instruments are exploited, and the child is still listed for heart transplantation as destination, evolving therapies like pulmonary artery banding in cases of preserved right ventricular function and cardiac cell therapy in cases of localized ventricular dysfunction might represent additional treatment options. This review summarizes the actual guidelines and provides an outlook for evolving therapies. 相似文献
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Summary Myofibrillar ATPase activity, maximum unloaded shortening velocity, and isometric tension development were evaluated in left ventricular preparations of 5-week-old rats with a high endogeneous level of thyroid hormones and hypothyroid rats after 4-week treatment with propylthiouracil (PTU). The range of possible alterations of the above functional parameters was defined in relation to myosin isoenzyme distribution. The mechanical behaviour of the ventricular preparations was investigated in native myocardium as well as in the glycerinated state.The essential result of the present study is that alterations of myofibrillar ATPase activity and mechanical vmax, evaluated in glycerinated preparations, are limited to a well-defined range of similar magnitude for both functional parameters: 32–40% of maximum values (obtained from rat myocardium with homogeneous myosin V1). Isometric tension was only insignificantly decreased in glycerinated preparations of the PTU-treated group.The alteration in the apparent maximum shortening velocity of native myocardium (v0) was of the same magnitude as changes in vmax of chemically skinned preparations. Physical training revealed a shift in the direction of V1-type myosin with increased ATPase activity and shortening velocity; aging and pressure overload showed an opposite effect. The documented mechanical alterations do not contradict an adaptational interpretation of the myosin isoenzyme redistribution in pressure-induced hypertrophy.Supported by the Deutsche Forschungsgemeinschaft. 相似文献
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Ortiz-Alvarez O; Cabral D; Prendiville JS; Stringer D; Petty RE; Malleson PN 《Rheumatology (Oxford, England)》1997,36(2):280-284
Two children are reported in whom intestinal pseudo-obstruction was the
initial manifestation of systemic sclerosis. Gastrointestinal symptoms and
skin changes resolved or improved in both children following treatment with
prednisone and penicillamine (case 1) or methotrexate (case 2), although
radiological changes of the gastrointestinal tract persisted at 3 and 2 yr
of follow-up, respectively.
相似文献
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Disturbed noradrenergic blood pressure control in normotensive members of hypertensive families. 下载免费PDF全文
M G Bianchetti P Weidmann C Beretta-Piccoli U Rupp K Boehringer L Link C Ferrier 《Heart (British Cardiac Society)》1984,51(3):306-311
The possible influence of a family history of hypertension on some variables of adrenergic blood pressure regulation was assessed. Blood pressure, heart rate, plasma renin activity, adrenaline and noradrenaline concentrations, and plasma or urinary electrolyte estimations did not differ significantly between two groups of normotensive subjects matched for age and sex with and without a family history of hypertension. Compared with subjects without a family history, however, an appreciably decreased pressor dose of infused noradrenaline, a distinct shift to the left in the relation between noradrenaline induced changes in mean arterial pressure and concomitant plasma noradrenaline concentrations, and an enhanced pressor response to given increases in plasma noradrenaline concentrations occurred in the group with a family history. These findings suggest that an imbalance between cardiovascular noradrenaline responsiveness and circulating noradrenaline is a common familial disturbance which could possibly predispose to the development of essential hypertension. 相似文献
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Jacqueline AM Smith DL Patil OT Daniels Y-S Ding J-D Gallezot S Henry KHS Kim S Kshirsagar WJ Martin GP Obedencio E Stangeland PR Tsuruda W Williams RE Carson ST Patil 《The international journal of neuropsychopharmacology / official scientific journal of the Collegium Internationale Neuropsychopharmacologicum (CINP)》2015,18(2)