Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A syndrome of slowly progressive muscle weakness with scapulo-ilio-peroneal distribution and cardiopathy was identified in 26 members of two families. Inheritance was autosomal dominant. Onset of the disease was between 17 and 42 years. Cardiopathy did not antedate skeletal muscle disease and patients had no symptoms of cardiopathy until a late phase of the disease. Initial ECG changes were non-specific, disturbances of conduction and impulse formation developed subsequently. Skeletal muscle biopsies showed neurogenic and myopathic changes with inflammatory cell reaction and perivascular cuffing. The combination of myopathy with neurogenic-like changes is characteristic of many cases of SPA. The inflammatory cell reaction is considered as part of a secondary polymyositis which is at leart partly responsible for muscle pathology.
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PMID:Inflammatory myopathy in scapulo-ilio-peroneal atrophy with cardiopathy. A study of two families. 121 74

Twenty-four patients presenting an acute stroke with watershed cerebral infarct on CT scan or MRI were included in this retrospective study. Age was 63 +/- 14 years (mean +/- SD), and sex ratio was 2 men for 1 woman. Main clinical features were: in anterior location, lower limb weakness and frontal syndrome with transcortical motor aphasia in left lesions or spatial dyscalculia in right ones; in posterior location, brachiofacial weakness with constant quadranopsia and hypoesthesia, and Gerstmann syndrome in left lesion. There was no distinctive feature for subcortical and multiple infarcts. In bilateral infarcts, there were one pseudobulbar syndrome, and 2 pseudo brainstem syndromes with neuropsychological signs. Aetiologies were severe carotid artery disease in 14 cases, severe cardiopathy in 6, isolated cerebral angiitis in 1, essential thrombocythemia in 1, protein C deficiency with sickle cell disease in 1, and cholesterol emboli in 1 anatomical case. CBF performed in carotid artery occlusions or tight stenoses showed evidence of haemodynamic changes. Microembolic process can be proposed in the case with cholesterol emboli. Preventive treatment is discussed.
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PMID:Watershed cerebral infarcts: retrospective study of 24 cases. 135

The presence of myocardial involvement is rare in benign Duchenne type of progressive muscular dystrophy (Becker's muscular dystrophy). We describe two brothers suffering from Becker's muscular dystrophy, both of whom presented with dilated cardiomyopathy. The first case is a 39-year-old male who had suffered from gait disturbance from the age of 17. When 37 years old, he was found to have heart disease. When he first came to our hospital, pseudohypertrophy of the calves was present. Chest radiography, electrocardiogram, ultrasonocardiography and clinical feature indicated Becker's muscular dystrophy with dilated cardiomyopathy. The second case is the younger brother of the 37-year old male. He suffered from leg weakness. He came to our hospital with the chief complaint of discomfort of the anterior chest. Pseudohypertrophy of the calves was present. Chest radiography, electrocardiogram, ultrasonogram indicated dilated cardiomyopathy.
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PMID:[The two brothers' case of dilated cardiomyopathy with benign Duchenne type of progressive muscular dystrophy (Becker's type)]. 157 Apr 30

A 52-year-old woman with chronic progressive external ophthalmoplegia (CPEO) with familial hypercholesterolemia (FH) was reported. Her mother died from heart disease, and her elder sister has hypercholesterolemia with swelling of Achilles tendons. She had slowly progressive external ophthalmoplegia, bilateral ptosis, swelling of Achilles tendons since twenties. At 40 years of age, she was pointed out hypercholesterolemia. Physical examination was within normal limits except for bilateral swelling of Achilles tendons. Neurological findings showed bilateral ptosis, disturbance of eye movements, mild proximal muscle weakness and dysesthesia in bilateral hands. Routine laboratory findings were within normal limits except for high serum cholesterol level (512 mg/dl). In the biopsied muscle, there was mild variation in fiber size with several ragged-red fibers and focal cytochrome c oxidase defective fibers. Biochemical analysis of the biopsied muscle revealed normal values in the mitochondrial fraction. Southern blot analysis of the mitochondrial DNA (mtDNA) of the muscle disclosed mixed population of mtDNA, consisting of the normal one and partially deleted (4.9-kilobase). Southern blot analysis of the leukocytes from the patient against the cDNA of LDL receptor was normal at least using the restriction enzyme of BglII, XbaI, EcoRI, PvuII and BamHI. This case has CPEO with deleted mtDNA associated with familial hypercholesterolemia, which is caused to nuclear DNA abnormalities, and is thought to be an important case for us to study the relationship between deleted mtDNA and abnormal nuclear DNA in CPEO.
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PMID:[A case of chronic progressive external ophthalmoplegia associated with familial hypercholesterolemia]. 162 35

Depression and chronic fatigue are frequently associated with heart disease. They may precede the onset of myocardial infarction, singly or together, and increase the morbidity and mortality of patients with a history of MI. Virtually all such patients have a transient depression, usually accompanied by anxiety, with onset soon after hospitalization. Although this depression is transient and usually abates spontaneously, it frequently warrants therapeutic intervention. Psychosocial and personality factors play a significant role in the recovery of a patient with a cardiac condition. The clinician must be alert for the effects of changing roles within the family and behaviors that may lead to chronic invalidism. Anxiety disorders, often combined with depression, may mimic cardiac disease and may result from it, leading to chronic fatigue and weakness. Proper diagnosis usually leads to considerable improvement. Cardiac drugs, in addition to many others, may produce depression and fatigue that may be misdiagnosed. Often, discontinuing or changing a medication will lead to marked diminution of such symptoms. Observational and listening skills are key ingredients of the "art" of medicine; they can lead to interventions that are not only therapeutic, but which improve the "quality" of life.
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PMID:Depression and chronic fatigue in the patient with heart disease. 187 16

Digitalis intoxication is among the most common serious adverse drug reactions in clinical medicine. While the recent development of a radioimmunoassay to accurately measure serum concentrations of digoxin has been of assistance, digitalis intoxication remains a difficult diagnosis to make with certainty. The difficulty in diagnosing digitalis intoxication arises from the nonspecificity of its associated signs and symptoms. The most common symptoms include fatigue, weakness, nausea, and anorexia. These symptoms can occur with many illnesses other than digitalis intoxication. Similarly, the electrocardiographic disturbances caused by cardiac glycosides may be nondiagnostic. The arrhythmias commonly associated with digitalis toxicity are often nonspecific and can be a reflection of the patient's underlying heart disease. The measurement of serum digoxin levels is useful, but studies have demonstrated overlap of the levels between groups with and without toxicity. Due to the modulation of the cardiac effects of digitalis glycosides by such clinical variables as underlying myocardial or renal disease, electrolyte and acid-base imbalances, and other factors, the correlation of toxicity with particular serum digoxin concentrations may vary. Because of the inherent difficulties in confirming the diagnosis of digitalis intoxication in some cases, digoxin-specific Fab antibodies may play a role as a diagnostic tool. Certainly, digoxin-specific Fab antibodies play a significant part in the treatment of digitalis intoxication. Fab antibodies have been successfully used to reverse the effects of digoxin, digitoxin, and oleander poisoning. These antibodies are useful in the treatment of acute and chronic digitalis intoxication in all age groups, including geriatric and pediatric populations.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Recognition and management of digitalis intoxication: implications for emergency medicine. 199 19

Of the right aortic arch anomalies, a right arch with isolation of the left subclavian artery is the least common. Herein we describe a 52-year-old woman in whom this anomaly was discovered during cerebral angiography for evaluation of a giant symptomatic intracavernous carotid aneurysm. Isolation of the left subclavian artery may be suggested in a patient with a right arch in whom the blood pressure or pulse in the left upper extremity is diminished. Although the isolated left subclavian artery produces the hemodynamic alterations of a subclavian steal, review of the 39 cases reported in the literature revealed only 5 patients with symptoms suggestive of vertebrobasilar insufficiency and 5 patients with weakness of the left upper extremity. Although the patient we describe had no known heart disease, congenital heart disease was present in 23 of the 39 reported cases (59%), tetralogy of Fallot occurring most frequently.
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PMID:Right aortic arch with isolation of the left subclavian artery: case report and review of the literature. 217 46

A young woman with humeroperoneal muscular dystrophy and contractures received a heart transplant for a severe dilated cardiomyopathy. Cardiac histopathology consisted of myocyte hypertrophy, interstitial fibrosis, and nuclear hyperchromaticity without mitochondrial abnormalities. Myopathy and heart disease were not clinically evident in her family, although three relatives had unexplained shortened Achilles tendons without weakness. Tendon contractures may be a partial expression of this myopathic disorder, suggesting an autosomal dominant inheritance with variable penetrance. A muscular dystrophy clinically similar to that of the Emery-Dreifuss (EDMD) type can thus occur in women. Rather than the cardiac arrhythmias typical of EDMD, a dilated cardiomyopathy may occur and present with severe congestive heart failure. This is the first report of cardiac transplantation in such a case.
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PMID:Cardiac transplantation in female Emery-Dreifuss muscular dystrophy. 223 Aug 49

A sample of people aged 65 and over were interviewed at home and asked a series of questions aimed at identifying episodes of possible transient neurological dysfunction. During follow-up of respondents initially free from manifest cerebrovascular disease, no relationship was found between subsequent stroke and reported episodes of diplopia, transient numbness or weakness, non-rotatory dizziness or blackouts. There was an association of stroke with reported blurring or dimming of vision, statistically significant only for the sexes combined (relative incidence ratio 1.5), and a consistently increased risk in men and women reporting rotatory vertigo (relative incidence ratio 2.5). This relationship remained significant when adjusted for the association of rotatory vertigo with ECG evidence of heart disease. Thus rotatory vertigo is a risk factor for stroke but non-rotatory dizziness is not. Conversely a previous study of falling in the same population sample had shown an association with rotatory vertigo but not with non-rotatory dizziness.
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PMID:Transient neurological dysfunction and risk of stroke in an elderly English population: the different significance of vertigo and non-rotatory dizziness. 231 24

A diagnosis of endocarditis was made in 37 patients (three days to 21 years old) on the basis of the following: histology in 11; at least two positive blood cultures in patients with underlying cardiac disease in 22; less than two positive blood cultures, vegetations seen at echocardiography and a suggestive clinical syndrome in four. Twenty-six patients had primary endocarditis (17 with pre-existing cardiopathy, nine with normal hearts). The 11 others developed secondary endocarditis following heart surgery (early onset in six, late onset in five). The mean delay before diagnosis was prolonged 35.8 days. The clinical and laboratory findings included weakness in 36 patients, fever in 35, new or modified heart murmur in 14, positive blood cultures in 30, anemia in 12, high white blood cell count in 15, increased sedimentation rate in 14, and positive echocardiogram in 11. Etiologic agents isolated were: streptococci in 17, staphylococci in seven, miscellaneous germs in eight, and aspergillus in two. Mortality was greater in patients less than one year old, infected with aspergillus or without underlying heart disease. The present study suggests that childhood endocarditis remains uncommon but presents a poor prognosis with a mortality of 27% and a morbidity of 85.7%.
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PMID:The evolving pattern of pediatric endocarditis from 1960 to 1985. 234 61


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