1/4. Continued neurocognitive development and prevention of cardiopulmonary complications after successful BMT for I-cell disease: a long-term follow-up report.I-cell disease or mucolipidosis type II, a rare inherited storage disorder of lysosomal enzyme localization, is characterized by dysostosis multiplex, progressive severe psychomotor retardation and death by 5-8 years from congestive heart failure and recurrent pulmonary infections. A 19-month old girl with I-cell disease received a bone marrow transplant (BMT) from an HLA-identical carrier brother. At the age of 7 years, 5 years after BMT, she has no history of respiratory infections. Her cardiac function remains normal with a shortening fraction of 47%, and she continues to gain neurodevelopmental milestones, albeit at a very slow rate. Musculoskeletal deformities have worsened despite BMT. This is the first report describing neurodevelopmental gains and prevention of cardiopulmonary complications in I-cell disease after BMT.- - - - - - - - - - ranking = 1keywords = heart (Clic here for more details about this article) |
2/4. Common scenarios to clarify the interpretation of cardiac markers.The authors present a practical approach for physicians in clinical practice to use cardiac troponins in the interpretation of heart disease and myocardial damage. Laboratory results that fall within the intermediate area of facility-specific cutoff reference values for elevated troponin levels confer lower risks to patients than do higher levels of cardiac troponin. Perhaps not surprisingly, the actual anatomy of the vessels at cardiac catheterization does not correlate well with the troponin level. In the six cases presented here, the patients' low levels of troponin release are discussed using the new term minimal myocardial infarction, which is synonymous with conditions that would previously have been diagnosed as unstable angina. Elevated levels of cardiac troponin provide a very sensitive measure for clinicians diagnosing patients with myocardial necrosis, but such measures are also useful in defining a broad spectrum of disease. Whenever the troponin levels are elevated (barring laboratory error), the patient has a poorer prognosis. The greatest challenge for physicians is in determining which patients with cardiac troponin elevation will best benefit from heart catheterization and percutaneous intervention.- - - - - - - - - - ranking = 2keywords = heart (Clic here for more details about this article) |
3/4. Anaesthesia for cardiac surgery in children with nemaline myopathy.Nemaline myopathy is a rare congenital myopathy associated with skeletal deformities and respiratory complications. Three children with nemaline myopathy who underwent cardiac surgery are described where the heart rate decreased during induction of anaesthesia and body temperature increased during or after the surgery. The anaesthetic implications in the management of patients with nemaline myopathy are discussed.- - - - - - - - - - ranking = 1keywords = heart (Clic here for more details about this article) |
4/4. Severe musculoskeletal symptoms during continuous infusion of bumetanide.BACKGROUND: The continuous infusion of the loop diuretic, bumetanide, has been shown to be effective in the treatment of severe refractory edema. Musculoskeletal symptoms have been reported with bumetanide following both oral and intermittent i.v. bolus therapy. It has been suggested that these adverse effects do not occur with continuous infusion. We describe, however, a series of patients with heart failure who developed severe disabling musculoskeletal symptoms during the continuous infusion of humetanide. OBJECTIVE: To evaluate the characteristics of bumetanide-induced musculoskeletal symptoms during continuous i.v. infusion in patients with severe heart failure. methods: heart failure patients receiving continuous infusions of bumetanide were monitored for the development of musculoskeletal symptoms. For patients who experienced the adverse reaction, data were collected on demographics, medical diagnoses, concurrent medications, the diuretic regimen, symptoms, time course of the reaction, laboratory findings, alternative therapy, and outcomes. RESULTS: Of 34 patients who received continuous infusions of bumetanide, eight patients experienced a total of 11 episodes of severe musculoskeletal symptoms. All patients had severe congestive heart failure and refractory edema. The reaction was most severe at infusion rates of approximately 2 mg/h and was not associated with any specific laboratory abnormality. The symptoms resolved completely after discontinuing the bumetanide therapy. In two of the patients, the adverse reaction was precipitated again during rechallenge with the bumetanide infusion. patients who were given an equivalent or higher dose of a continuous furosemide infusion, diuresed without experiencing musculoskeletal symptoms. CONCLUSIONS: The continuous i.v. infusion of the loop diuretic, bumetanide, may result in severe, disabling musculoskeletal symptoms. The reaction appears to be dose related, without specific risk factors, and is reversible on discontinuation of the infusion. patients who experience this reaction may be successfully diuresed with equivalent doses of a furosemide infusion.- - - - - - - - - - ranking = 3keywords = heart (Clic here for more details about this article) |