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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present a clinical and morphological study concerning a kindred with slowly progressive X-linked muscular dystrophy (Becker type). Five males were affected; one died of heart failure at age 16. Severe and early cardiac disorder is unusual in this type of muscular dystrophy, and death at such an early age had not been reported previously. In the other patients of the kindred, cardiac abnormalities, if present at all, were nonspecific. Another unusual feature in this kindred was severe muscle pain at an early stage of the disease, a feature that cannot yet be explained.
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PMID:Early myocardial disease and cramping myalgia in Becker-type muscular dystrophy: a kindred. 57

We report 11 cases of bacterial endocarditis with muscular and articular manifestations seen over the past ten years. There was arthralgia in 7 cases, vertebral pain in 7 cases and myalgia in 3 cases. Arthritis consisted of a monoarthritis of the ankle in 2 cases and oligoarthritis in 2 cases. There were also 2 cases of lumbar spondylodiscitis and 1 of finger clubbing in the series. The underlying heart disease was a valvular lesion of the left side of the heart in 10 cases out of 11 and the organism isolated by blood culture was a streptococcus in 9 cases and a staphylococcus in 11. We emphasis the need for early diagnosis and appropriate antibiotic therapy, in the absence of which the course may be fatal in the short term, as it was the case in one of our own patients.
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PMID:[Articular and muscular manifestations of bacterial endocarditis. 11 cases (author's transl)]. 74 39

Chest pain and breathlessness are common somatic symptoms of emotional disorder in ambulatory care. Chronic chest pain has a prevalence of 12% and is associated with high utilization of health care. Of patients with chest pain and breathlessness who are referred to a cardiac clinic but subsequently shown not to have heart disease, the majority continue to report symptoms. Those patients with the worst outcome, in terms of continuing limitation of activity and use of medical resources, are those with chest pain but normal coronary arteries. A number of studies that fail to support a unitary theory of causation of noncardiac chest pain are described. A multifactorial, interactive model is proposed, with contributions from physical factors, such as palpitations and intercostal muscle pain; psychologic factors, which include enhanced awareness of and selective attention to bodily sensation; and environmental factors, such as previous exposure to cardiorespiratory disease in first-degree relatives or significant others. Although there have been few controlled intervention studies in patients with unexplained cardiorespiratory symptoms, there is evidence for the efficacy of both drug treatments and psychologic treatment. The results of intervention studies in patients with chest pain and normal coronary arteries are eagerly awaited. Atypical chest pain and breathlessness are common causes of office consultations and/or functional disability. The diagnoses should be established on the basis of positive evidence of psychiatric illness rather than by exclusion. The etiology is multifactorial, and management is aimed at treating the underlying psychosocial problems and/or psychiatric illness. Cognitive-behavioral treatments are probably as effective as drug treatments in the short-term, and the care of these patients would be improved by a more detailed explanation of noncardiac causes and a greater opportunity for patients to discuss their fears.
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PMID:Chest pain and breathlessness: relationship to psychiatric illness. 173 29

Early in the course of studies of the Spanish toxic oil syndrome it was recognized that vascular lesions were a major problem, most logically attributable to endothelial damage by the toxic oil. However, most clinical attention has been directed to the pulmonary complications and the evolution into a scleroderma-like illness later. In this study of 11 victims of the toxic oil syndrome careful postmortem studies of the coronary arteries and conduction system and neural structures of the heart demonstrated major injury to all those components of the heart. Obliterative fibrosis of the sinus node in four cases resembled findings in fatal scleroderma heart disease, and in eight the cardiac lesions resembled those of lupus erythematosus. The more impressive pathologic features involved the coronary arteries and neural structures, which were abnormal in every heart. The arterial disease included widespread focal fibromuscular dysplasia, but there was also an unusual myointimal proliferative degeneration of both small and large coronary arteries in five patients, four of whom were young women. In two hearts, portions of the inner wall of the sinus node artery had actually detached and embolized downstream. Coronary arteritis was rarely found. Inflammatory and noninflammatory degeneration of cardiac nerves was widespread. Fatty infiltration, fibrosis and degeneration were present in the coronary chemoreceptor. In most respects these cardiac abnormalities resemble those described in the eosinophilia-myalgia syndrome caused by an altered form of L-tryptophan. In both diseases there is good reason to anticipate more clinical cardiac difficulties than have so far been reported, and even more basis for future concern, especially relative to coronary disease and cardiac electrical instability.
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PMID:Cardiac abnormalities in the toxic oil syndrome, with comparative observations on the eosinophilia-myalgia syndrome. 191 15

Experimental and clinical experience with compounds containing antimony have shown that the trivalent compounds are generally more toxic than the pentavalent ones. APT can cause severe pain and tissue necrosis and is therefore not given by intramuscular or subcutaneous injection. APT has the actions and uses of AST, but it is less soluble and more irritating than the sodium salt which is therefore more suitable for intravenous use. Trivalent antimony compounds are toxic when used topically. Adverse effects are similar for all trivalent compounds, and include nausea, vomiting, weakness and myalgia, abdominal colic, diarrhoea, and skin rashes, including pustular eruptions. Hypersensitivity reactions also occur. Respiratory symptoms include cough, dyspnoea, and chronic lung changes. Cardiotoxicity is the most important and may produce arrhythmias, myocardial depression and damage, Stokes-Adams attacks, heart failure, and cardiac arrest. Hepatic damage and necrosis, as well as blood dyscrasias, may occur. Toxic effects on the kidney may follow chronic use. Continuous treatment with small doses of antimony may give rise to symptoms of subacute poisoning, similar to those of chronic arsenic poisoning, due to accumulation of antimony in the body, especially if trivalent compounds are used, because of their long biological half-lives. Reproductive disorders and chromosome damage have been reported; antimony compounds are, therefore, potentially toxic to reproduction and have mutagenic, and oncogenic potential. Antimony compounds should, therefore, not be used during pregnancy or in the presence of hepatic, renal, or heart disease. Pentavalent antimony preparations especially the organic compounds, together with non-metallic synthetic preparations, such as the diamidines, have now replaced APT for use in leishmaniasis. Because of the toxicity of antimony compounds, investigations have been undertaken to reduce their adverse effects by combining them with chelating agents. These preparations appear to have reduced the toxic effects of antimony without affecting the efficacy of the preparations. Liposome-encapsulated antimony products have, more recently, been shown to be much less toxic because of the reduced dose of the antimony compound required for effective therapy. The historical uses of antimony were based on the belief that the topical and systemic adverse effects, for example, skin eruptions and diarrhoea and vomiting, were signs that the condition being treated was responding by being brought to the surface to relieve congestion at the diseased area. There is no evidence in topical use, but there is evidence that such use can cause severe reactions.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Toxicity of antimony and its compounds. 330 36

A multidisciplinary study was conducted in order to assess dystrophin expression in a large series of mild X-linked muscular dystrophy patients, with well-defined clinical phenotype. Patients (104) were divided in 4 clinical groups, according to clinical severity: asymptomatic (sub-clinical), benign, moderate and severe, Cardiopathy was also assessed, and dilated cardiomyopathy was found in 47% of sub-clinical and benign cases. Myoglobinuria, cramps and myalgia were also associated with a sub-clinical or benign clinical status. Dystrophin immunohistochemical pattern of labelling and dystrophin amount decreased gradually across clinical groups. Our study showed a significative correlation between: (1) dystrophin amount and immunohistochemical score (p < 0.05); (2) dystrophin amount and clinical score (p < 0.05). Therefore, the combined use of these different techniques for prognosis of mild X-linked muscular dystrophy patients is useful. Our study assesses the prevalence of the various disease courses in a large cohort of mild X-linked muscular dystrophy patients. From our series, up to 30% of patients may be either asymptomatic or have sub-clinical changes.
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PMID:Clinical-molecular correlation in 104 mild X-linked muscular dystrophy patients: characterization of sub-clinical phenotypes. 798 92

The first case of chronic cardiac toxicity due to an antimalarial agent was reported in 1971 and since then several cases of heart failure, restrictive cardiomyopathy or atrioventricular block have been ascribed to this family of drugs. We report the case of a 43-year-old woman who developed juvenile chronic arthritis at the age of ten, followed in adulthood by sero-positive rheumatoid arthritis. In 1980 she was put under chloroquine sulfate (hydroxychloroquine was not available) in a dose of 200 mg/d (152.66 mg of chloroquine), with 10 mg/day of prednisone. She developed myalgia and increased skin pigmentation, but disregarded recommendations that these symptoms required discontinuation of chloroquine therapy. She was lost to follow-up, but continued the chloroquine therapy of her own accord. In December 1993, she developed a third-degree atrioventricular block with syncopes requiring implantation of a pacemaker. The rare but well-documented myopathy induced by antimalarial agents can produce early severe lesions of the cardiac muscle, which may have a predilection for the interventricular septum, explaining the risk of atrioventricular block. Although histologic studies were not performed in our patient, the clinical evidence of toxicity, absence of underlying heart disease and fairly young age of the patient pointed to chloroquine toxicity. Periodic cardiac investigations including electrocardiography may be warranted in patients under antimalarial therapy.
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PMID:Third-degree atrioventricular block in a patient under chloroquine therapy. 952 87

Patency of the ductus arteriosus (DA) is maintained during gestation by locally produced and circulating prostaglandins (PGE's). As gestation proceeds, the ductus becomes less sensitive to dilating prostaglandins and more sensitive to constricting factors such as PGE's synthetase inhibitors. This case report describes a fetus at term (38 weeks) with signs of severe right ventricular failure due to constriction of DA. Maternal history documented 5 day assumption of a non-steroid antiinflammatory agent to relieve skeletal-muscle pain. Careful echocardiogram ruled out a structural heart disease, such as coarctation of the aorta. A gradient of 41 mmHg across the ductus was recorded. A cesarean section delivery was immediately undertaken. The 3.5 kg newborn delivered appeared to be in good health, with Apgar score of 8/9 at 1 and 5'. There were no signs of congestive heart failure and mild respiratory distress. An echocardiogram showed a dilated, well contractile right ventricle, with a pressure of 50 mmHg. DA was already closed. The fetal echocardiogram was the most relevant investigation in the decision-making process of this case treatment. Any different evaluation of this fetal heart, delaying the delivery would have very seriously compromised the survival of the fetus. Fetal echocardiography is the most important diagnostic tool in the evaluation of the fetal heart; non steroid antiinflammatory drugs to mother at term should be avoided or given with close echocardiographic assessment of DA patency.
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PMID:[Timely detection of premature closure of the ductus arteriosus in a full-term fetus. Important role of fetal echocardiography]. 1043 30

Myoadenylate deaminase deficiency is the most common metabolic disorder of skeletal muscle in the Caucasian population, affecting approximately 2% of all individuals. Although most deficient subjects are asymptomatic, some suffer from exercise-induced myalgia suggesting a causal relationship between a lack of enzyme activity and muscle function. In addition, carriers of this derangement in purine nucleotide catabolism may have an adaptive advantage related to clinical outcome in heart disease. The molecular basis of myoadenylate deaminase deficiency in Caucasians has been attributed to a single mutant allele characterized by double C to T transitions at nucleotides +34 and +143 in mRNA encoded by the AMPD1 gene. Polymerase chain reaction-based strategies have been developed to specifically identify this common mutant allele and are considered highly sensitive. Consequently, some laboratories preferentially use this technique over other available diagnostic tests for myoadenylate deaminase deficiency. We previously identified a G468-T mutation in one symptomatic patient who was only heterozygous for the common AMPD1 mutant allele. In this report, nine additional individuals with this compound heterozygous genotype are revealed in a survey of 48 patients with documented deficiency of skeletal muscle adenosine monophosphate deaminase and exercise-induced myalgia. Western blot analysis of leftover biopsy material from one of these individuals does not detect any immunoreactive myoadenylate deaminase polypeptide. Baculoviral expression of the G468-T mutant allele produces a Q156H substitution enzyme exhibiting labile catalytic activity. These combined results demonstrate that the G468-T transversion is dysfunctional and further indicate that AMPD1 alleles harboring this mutation contribute to the high incidence of partial and complete myoadenylate deaminase deficiency in the Caucasian population. Consequently, genetic tests for abnormal AMPD1 expression designed to diagnose patients with metabolic myopathy, and to evaluate genetic markers for clinical outcome in heart disease should not be based solely on the detection of a single mutant allele.
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PMID:A G468-T AMPD1 mutant allele contributes to the high incidence of myoadenylate deaminase deficiency in the Caucasian population. 1211 80

Hemorrhagic necrosis of a pheochromocytoma is a rare cause of acute presentation that is often devastating to patients. A 44-year-old woman with spontaneous hemorrhage into a previously undiscovered pheochromocytoma is described. The patient presented with acute cardiogenic pulmonary edema, shock, abdominal pain, myalgia and high fever. Her cardiac function recovered with aggressive medical treatment before the tumour was removed. This case illustrates an unusual presentation of pheochromocytoma and emphasizes the importance of aggressive and appropriate medical therapy in pheochromocytoma heart disease.
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PMID:Cardiogenic shock due to acute hemorrhagic necrosis of a pheochromocytoma: a case report and review of the literature. 1271 95


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