There have been a few reports of cardiac involvement in the Kugelberg-Welander syndrome. We presented a case of this syndrome complicated with cardiomegaly and conduction disturbance. The patient, a 21-year-old woman, had atrial standstill and A-V junctional rhythm. The chest X-ray film showed marked cardiomegaly. The His bundle electrogram revealed that H-V interval was 40 msec. She was treated with implantation of cardiac pacemaker. Additional Indexing Words: Muscle atrophy Cardiomegaly Atrial standstill functional escape rhythm HE Kugelberg-Welander syndrome was described by Wohlfart et al,1) and Kugelberg and Welander2) as a heredofamilial juvenile muscular atrophy simulating muscular dystrophy. The characteristic features of this syndrome are the followings; onset in childhood or adolescence; atrophy and weakness of mainly proximal limb muscles; slowly progressive clinical course; development of fasciculation; evidence of neurogenic changes in electromyogram and muscle biopsy; nonsex-linked recessive inheritance. Since then, there have been many reports on the Kugelberg-Welander syndrome. In some of them, fasciculation was not seen, and variable modes of inheritance were noted. Sporadic occurrence was also noted. It is well known that cardiac involvements are frequently found in neuromuscular diseases, such as Duchenne's muscular dystrophy,3) myotonic dystrophy,4) Friedreich's ataxia,5) and so on. However, there have been only a few reports on the Kugelberg-Welander syndrome with cardiac lesions. In this paper we presented a case of the Kugelberg-Welander syndrome with cardiac enlargement and abnormalities in the electrocardiogram.
The effect of insulin resistance (IR) on the fatty acid metabolism of myocardium, and therefore on the recovery of left ventricular (LV) wall motion, has not been established in patients with acute myocardial infarction (AMI). A total of consecutive 58 non-diabetic AMI patients who had successfully undergone emergency coronary angioplasty were analyzed retrospectively. They were categorized into 2 groups, normal glucose tolerance (NGT) and impaired glucose tolerance (IGT), based on a 75-g oral glucose tolerance test (OGTT). The parameters of OGTT, myocardial scintigraphy (n=58) (thallium-201 (Tl) and iodine-123-beta-methyl-iodophenylpentadecanoic acid (BMIPP)) and left ventriculography (n=24) were compared in the 2 groups after reperfusion (acute phase) and 3-4 weeks after the AMI (chronic phase). The insulin resistance (IR), estimated by the serum concentration of insulin at 120 min (IRI 120') of the OGTT and by the HOMA (the homeostasis model assessment) index, was higher in the IGT group than in NGT group. An inverse correlation was found between the recovery of regional LV wall motion in the ischemic lesion and the IRI 120' and HOMA index. Although the recovery of BMIPP uptake from the acute to the chronic phase was higher in the IGT group, it was only correlated with the degree of IRI 120', not with the HOMA. IR accompanied by IGT can negatively influence the recovery of regional LV wall motion.
The absorption of nifedipine (10 mg) and haemodynamic response were studied in 10 patients with myocardial infarction before (Phase A) and after (Phase B) a standardized breakfast. In Phase A, the peak nifedipine concentration (Cmax) and maximum haemodynamic changes were found 1 h after drug administration. In Phase B, the Cmax was lower than that in phase A (43 +/- 6 vs 136 +/- 23 ng/ml, p less than 0.001), and both Cmax and maximum haemodynamic changes were delayed to the fourth hour after administration. Nifedipine plasma concentration correlated significantly with the percent changes in systolic and diastolic blood pressure and heart rate. This study suggests that not only dose but also the time intervals between nifedipine administration and food intake are important in determining the haemodynamic effects.
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