1/622. Anaesthetic considerations in a patient with lepromatous leprosy. PURPOSE: To consider the anaesthetic problems in a patient with lepromatous leprosy undergoing general anaesthesia. CLINICAL FEATURES: A 52 yr old man with lepromatous leprosy for five years was booked for elective radical nephrectomy. He received 100 mg dapsone per day po. The patient was asymptomatic for cardiovascular disease but his electrocardiogram showed complete left bundle branch block, inferior wall ischaemia with echocardiogram findings of 58% ejection fraction and left ventricular diastolic dysfunction. Other preoperative investigations (haemogram, serum urea and creatinine, liver function tests and chest X-ray) were normal. After premedication with diazepam, meperidine and promethazine, the patient received glycopyrrolate and anaesthesia was induced with thiopentone. atracurium was given to facilitate tracheal intubation. Anaesthesia was maintained with intermittent positive pressure ventilation using N2O in oxygen with halothane. Anaesthesia and surgery were uneventful except that the patient had a fixed heart rate that remained unchanged in response to administration of anticholinergic, laryngoscopy, intubation and extubation. CONCLUSION: patients with lepromatous leprosy may have cardiovascular dysautonomia even when they are asymptomatic for cardiovascular disease. ( info) |
2/622. A novel form of familial congenital muscular dystrophy in two adolescents. We report on two brothers (the product of first-degree consanguineous marriage; aged 15 and 12 years) who presented with severe hypotonia at birth, proximal muscle weakness associated with delayed motor milestones but normal cognitive function. Investigations (at 4 years of age) revealed mildly elevated serum creatine kinase (CK) levels (300 and 824 IU/l; N < or = 210). Muscle biopsies showed minimal change myopathy, no neurogenic atrophy but remarkable type-1 fibre predominance (up to 85.5%) without fibre-type disproportion. Clinical examination at 12 and 9 years, respectively, showed mild facial weakness and high-arched palate in both patients. The younger sibling also had ptosis but otherwise normal external ocular muscles. They showed symmetric proximal muscle weakness and wasting associated with calf-muscle hypertrophy. They could walk independently. A repeat muscle biopsy showed advanced dystrophic changes in the younger patient at the age of 10 years. Virtually all the remaining fibres were type 1. immunohistochemistry revealed normal expression of the dystrophin-glycoprotein complex (DGC), including dystrophin, beta-dystroglycan, alpha-(adhalin), beta-, gamma-, and delta-sarcoglycan, laminin-alpha2 chain (merosin) and syntrophin. Mild dystrophic features and type-1 fibre predominance (92.5%) were seen in the biopsy of the older patient, whereas immunohistochemistry showed normal expression of the DGC. Both cases also showed clear expression of integrin alpha7 at the muscle fibre surface and in the blood vessels. Three years later, they could still walk, but with difficulty, and the older brother showed enlargement of the tongue and echocardiographic features of left ventricular dilated cardiomyopathy. ( info) |
3/622. Evolution of left ventricular diseasein the fetus. Case report. A fetal case is described that showed a rapid progression from the features of initial left ventricular fibroelastosis at 20 weeks of gestation to a more marked dilation at 22 weeks and finally to a hypoplastic left ventricle with aortic stenosis at 24 weeks of gestation. This case confirms the evolutive character of left ventricular disease during fetal life. ( info) |
4/622. Rapid progression of cardiomyopathy in mitochondrial diabetes. Cardiac involvement and its clinical course in a diabetic patient with a mitochondrial tRNA(Leu)(UUR) mutation at position 3243 is reported in a 54-year-old man with no history of hypertension. At age 46, an electrocardiogram showed just T wave abnormalities. At age 49, it fulfilled SV1 RV5 or 6>35 mm with strain pattern. At age 52, echocardiography revealed definite left ventricular (LV) hypertrophy, and abnormally increased mitochondria were shown in biopsied endomyocardial specimens. He was diagnosed as having developed hypertrophic cardiomyopathy associated with the mutation. However, at age 54, SV1 and RV5,6 voltages were decreased, and echocardiography showed diffuse decreased LV wall motion and LV dilatation. Because he had mitochondrial diabetes, the patient's heart rapidly developed hypertrophic cardiomyopathy, and then it seemed to be changing to a dilated LV with systolic dysfunction. Rapid progression of cardiomyopathy can occur in mitochondrial diabetes. ( info) |
5/622. A case of eosinophilic myocarditis complicated by Kimura's disease (eosinophilic hyperplastic lymphogranuloma) and erythroderma. This report describes a patient with eosinophilic myocarditis complicated by Kimura's disease (eosinophilic hyperplastic lymphogranuloma) and erythroderma. A 50-year-old man presented with a complaint of precordial pain. However, the only abnormal finding on examinatioin was eosinophilia (1617 eosinophils/microl). Three years later, the patient developed chronic eczema, and was diagnosed with erythroderma posteczematosa. One year later, a tumor was detected in the right auricule, and a diagnosis of Kimura's disease was made, based on the biopsy findings. The patient developed progressive dyspnea 6 months later and was found to have cardiomegaly and a depressed left ventricular ejection fraction (17%). A diagnosis of eosinophilic myocarditis was made based on the results of a right ventricular endomyocardial biopsy. The eosinophilic myocarditis and erythrodrema were treated with steroids with improvement of both the eosinophilia and left ventricular function. ( info) |
6/622. Decreased left ventricular filling pressure 8 months after corrective surgery in a 55-year-old man with tetralogy of fallot: adaptation for increased preload. A 55-year-old man with tetralogy of fallot underwent corrective surgery. Left ventricular filling pressure increased markedly with increased left ventricular volume one month after surgery, then decreased over the next 7 months, presumably due to increased left ventricular compliance. ( info) |
| A 47-year-old woman with severe prolonged anemia developed heart failure. After treatment of the heart failure and anemia, she showed regional dysfunction of the left ventricular wall and myocardial fatty acid metabolism was disturbed in these sites. Coronary arteriography showed normal images. It took about 4 months to recover both left ventricular wall motion and fatty acid metabolism. Prolonged decrease of oxygen supply to the myocardium, which is caused by severe prolonged anemia, seemed to affect the myocardial function in this case, which could be another model of anemia-related myocardial dysfunction. ( info) |
8/622. Polymyalgia, hypersensitivity pneumonitis and other reactions in patients receiving HMG-CoA reductase inhibitors: a report of ten cases. Ten patients who take hydroxy-methylglutaryl coenzyme a reductase inhibitors, or statin medications, and experience adverse reactions are described. All patients experienced various manifestations of hypersensitivity while receiving the drugs. One patient is described with hypersensitivity pneumonitis, which was graphically demonstrated by both high resolution computerized axial tomography and open lung biopsy. ( info) |
| For unclear reasons, myocardial infarction is rare in childhood sickle cell disease, whereas lung, bone, and brain infarcts are more common. During vasoocclusive crisis and infection, acute myocardial ischemia and chronic volume overload from anemia may result in myocardial dysfunction. We report a child who had reversible cardiac dysfunction that mimicked myocardial infarction. ( info) |
10/622. Left ventricular ischemia due to coronary stenosis as an unexpected treatable cause of paroxysmal atrial fibrillation. We present a patient with exercise-induced paroxysmal atrial fibrillation who was eventually scheduled for a Cox-maze operation due to persistence of his complaints of fatigue, impaired exercise tolerance, and predominantly exercise-related irregular palpitations despite treatment with several antiarrhythmic drugs. A preoperative exercise stress test without antiarrhythmic or negative chronotropic drugs, however, showed clear evidence of myocardial ischemia. After coronary angioplasty of a significant stenosis in the left anterior descending artery, there was no recurrence of atrial fibrillation during a follow-up of 7 months. ( info) |