[An adult case of angina pectoris as a sequel of Kawasaki disease].
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Biomedical subjects
Publications and source records attributed to M Hongo.
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To determine whether technetium-99m-pyrophosphate (Tc-99m-PYP) scanning or two-dimensional echocardiography can detect amyloid heart disease in an earlier stage of familial amyloid polyneuropathy, 15 patients were examined. Although 10 of the 15 patients had no clinical evidence of congestive heart failure, as well as normal ventricular wall thickness and normal values for left ventricular systolic function, five (50%) of them showed mild or moderate myocardial uptake. On the other hand, none had characteristic highly refractile myocardial echoes on the two-dimensional echocardiographic images (p less than 0.01), and values for diastolic function were reduced in four of the five and normal in the remaining one. In 85 control subjects, diffuse positive pyrophosphate scans of the heart were found in four (5%) of them (three with dilated cardiomyopathy and one with sarcoidosis), and highly refractile granular sparkling echoes were observed in nine (11%) (five with hypertrophic cardiomyopathy, three with aortic stenosis, and one with hypereosinophilic syndrome). We conclude that Tc-99m-PYP scanning is a more sensitive and specific method and may have the potential ability to detect amyloid heart disease in the earlier stage of familial amyloid polyneuropathy than two-dimensional echocardiography.
To clarify the cause of gastrointestinal disorders in systemic amyloidosis we made pathologic and morphometric studies of vagus nerves, celiac ganglia, stomach and rectum in three autopsied cases with type 1 familial amyloid polyneuropathy (FAP) and two with nonhereditary generalized amyloidosis (AL-amyloidosis). The gastric and rectal walls in all cases were affected in the same way by amyloid deposition. On the other hand, there was a great difference between the two diseases in the severity of vagus nerve and celiac ganglion lesions: the vagus nerves in FAP showed very extensive endoneurial deposition of amyloid with severe loss of myelinated nerve fibers, but in AL-amyloidosis there was no loss of myelinated nerve fibers and only slight amyloid deposition in the endoneurium. Similarly, in the celiac ganglion, intraganglionic deposition of amyloid was prominent in FAP and slight in AL-amyloidosis. It is known that bowel symptoms frequently occur in type I FAP and are less prominent in AL-amyloidosis. This study demonstrated that the gastrointestinal autonomic nerves were more markedly disturbed by amyloid in the former than in the latter, and disorder in neural control of the digestive tract may be responsible for the bowel symptoms in systemic amyloidosis, especially in type I FAP.
A clinicopathological study was made on 65 patients from a small area of Nagano Prefecture, Japan, with hereditary generalized amyloidosis with polyneuropathy to clarify the clinical variety of the disease. Forty-five patients from Ogawa village showed similar clinical features. The age of onset ranged widely from 16 to 62 years. The main neurological manifestations were polyneuropathy starting in the legs and autonomic dysfunction. Lower cranial nerves were also affected in the advanced stages. Severe cardiac and renal involvement was uncommon. All these clinical features are consistent with type I familial amyloid polyneuropathy (FAP). The remaining 20 patients from five unrelated kinships showed unique clinical pictures. Two families from Ogawa village had type I FAP, but 4 out of the 5 affected patients showed marked nephropathy with heavy proteinuria from an early stage. Of the three other families, one, with 10 patients, was notable for the involvement of the central nervous system. Most of the patients showed cerebellar ataxia and pyramidal tract signs in addition to a sensorimotor and autonomic peripheral neuropathy. Another family had 2 siblings who had severe amyloid heart disease from the onset and developed polyneuropathy with autonomic features at an advanced stage. In the third family, onset occurred in the sixth decade in all 3 patients and the course was mild in 2, although the clinical features were those of typical type I FAP. Immunohistochemical study revealed that the amyloid fibril proteins in the patients with all four unusual clinical phenotypes were related to plasma prealbumin. The most common form of hereditary generalized amyloidosis in Japan is type I FAP, but the disease shows considerable variety in the age of onset and involves more systemic organs than previously recognized. The newly recognized clinical forms of hereditary generalized amyloidosis with severe amyloid heart disease or central nervous dysfunction indicate clinical heterogeneity of hereditary amyloidosis with polyneuropathy.
Cisapride is a novel gastrointestinal prokinetic drug. The aim of the present study was to investigate gastric emptying time and secretion of gastrointestinal hormones (Gastrin, Motilin, Glucagon) after single oral intake of 5 mg or 10 mg cisapride. The healthy men aged 23-55 years participated the study. The gastric emptying time and gastrointestinal hormones were measured by gamma-camera radioisotopic technique and radioimmunoassay, respectively. The half time of emptying phase was reduced from 31.78 min to 29.88 min, and emptying rate increased in 10 mg cisapride group. There was a tendency that cisapride accelerated a stimulation of Motilin secretion.
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Three patients with dysphagia caused by compression of the distal esophagus by a tortuous nonaneurysmal atherosclerotic aorta are described. All three patients were elderly women; systemic hypertension and cardiomegaly were present in two patients. Barium studies of the esophagus showed displacement and compression of the distal esophagus by the thoracic aorta. Debilitating dysphagia was treated surgically in one patient. The other two patients had milder symptoms and were managed conservatively. Esophageal manometry in these three patients showed superimposed pulsations and elevated intraluminal pressure just proximal to the lower esophageal sphincter. To evaluate the significance of these manometric findings and their correlation with clinical symptoms, we reviewed manometric tracings in 47 normal subjects. Ten of these subjects had an elevation of baseline intraluminal esophageal pressure as a result of superimposed vascular pulsations. We conclude that compression of the distal esophagus by a tortuous atherosclerotic aorta in the appropriate setting can lead to clinically significant dysphagia and a manometric finding of vascular compression of the esophagus does not necessarily correlate symptomatic dysphagia.
Computed tomographic findings in a 71-year-old patient with an infiltrative right ventricular myxoma are described. The findings included multiple lesions in the right ventricle and stenosis of the trunk of the pulmonary artery.
In lactic acid fermentation by Lactobacillus delbrueckii, the produced lactic acid affected the lactic acid productivity. Therefore, for the purpose of alleviating this inhibitory effect, an electrodialysis fermentation method which can continuously remove produced lactic acid from the fermentation broth was applied to this fermentation process. As a result, the continuation of fermentation activity was obtained, and the productivity was three times higher than in non-pH-controlled fermentation. In electrodialysis fermentation, the amount of produced lactic acid was 82.2 g/liter, which was about 5.5 times greater than that produced in non-pH-controlled fermentation. It was concluded that these good results were obtained on account of alleviating the lactic acid inhibitory effect by electrodialysis fermentation. However, the fouling of anion-exchange membranes by cells was observed in electrodialysis fermentation.
To determine the evolution of amyloid heart disease, 28 patients with familial amyloid polyneuropathy (FAP) were studied by echocardiography. The incidence and degree of the abnormalities were correlated with the neurologic disabilities, duration of the illness, and age in cross-sectional studies. Serial studies were performed in 12 patients, who were followed for a mean of 27.7 months. At the initial examinations, left ventricular diastolic function was reduced in six patients, while systolic function was preserved in eight. On follow-up there occurred significant increases in ventricular septal wall thickness (from 10.7 +/- 3.1 to 13.1 +/- 3.2 mm; p less than .01) and posterior wall thickness (from 11.8 +/- 2.5 to 13.5 +/- 2.3 mm; p less than .01), and reductions in the E-F slope of the mitral valve (from 64.4 +/- 18.8 to 43.9 +/- 11.0 mm/sec; p less than .01), percent fractional shortening (from 36.5 +/- 9.4% to 28.8 +/- 8.2%; p less than .02), and left ventricular internal diastolic dimension (from 46.2 +/- 6.1 to 42.4 +/- 6.2 mm; p less than .001). At the final examinations, marked ventricular hypertrophy was found in three patients, reduced left ventricular diastolic function in all, impaired systolic function in nine, and decreased left ventricular internal dimension in three. In addition, highly refractile myocardial echoes had appeared in two patients, pericardial effusion in three, and valve thickening in two. We conclude that amyloid heart disease in patients with FAP develops slowly but progressively.
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A new carbapenem antibiotic, imipenem/cilastatin sodium (MK-0787/MK-0791), was administered by intravenous drip infusion at a dose level of 500 mg/500 mg to 30 patients who underwent total abdominal hysterectomy for uterine myomas with or without benign ovarian tumors. The uterine arteries were clamped bilaterally at 0.25, 0.5, 1, 2, 3, 4, 6 and 8 hours after administration, and plasma samples and uterine tissues were taken for measurements of MK-0787 by bioassay and MK-0791 by HPLC. The measured values were analyzed using a two-compartment open model. Maximum concentrations of MK-0787 at 0.5 hour after the start of intravenous drip infusion were 98.5 micrograms/ml in the antecubital vein, 100.1 micrograms/ml in the uterine artery, 26.5 micrograms/g in the oviduct, 21.2 micrograms/g in the ovary, 19.1 micrograms/g in the endometrium, 19.0 micrograms/g in the myometrium, 22.1 micrograms/g in the cervix uteri and 16.4 micrograms/g in the portio vaginalis. These results suggest that the penetration of MK-0787/MK-0791 into female genital tissues is good, and sufficient concentrations are obtained enough to inhibit organisms which are frequently isolated from patients with pelvic inflammatory disease.
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Only limited work has been reported about the relationships of cardiovascular effects and plasma concentrations of the calcium-channel blocker nifedipine. In this study, placebo and nifedipine in 10-, 20-, 30-, and 40-mg doses were administered sublingually to ten normal subjects with at least three days between dosing periods. Blood pressure and heart rate were monitored every 30 minutes for two hours, and blood samples were taken after each measurement for determination of plasma nifedipine concentration by a sensitive and specific gas chromatographic method. Systolic blood pressure fell significantly (P less than 0.05) although briefly after 10 mg, but the effect persisted with larger doses. Diastolic blood pressure fell significantly only after 30- or 40-mg dosing. Heart rate increased significantly after all doses of nifedipine with the effect lasting longer with higher doses. Systolic blood pressure measurements were significantly related to the log of the concurrently measured plasma nifedipine concentrations (r = -.82, P less than 0.001). Diastolic blood pressure was also related to log nifedipine concentration (r = -.69, P less than 0.01). Heart rate, too, was linearly related to the log of nifedipine plasma levels (r = .75, P less than 0.001). These data indicate that the hemodynamic effects observed after acute nifedipine administration may be used to estimate whether or not significant quantities of the drug are being absorbed and that the intensity of the hemodynamic effects may, therefore, serve as a bioassay to evaluate the appearance of drug in plasma in therapeutic quantities.
The occurrence of gastroesophageal reflux after meals may be related to an increase in the rate of transient lower esophageal sphincter (LES) relaxations, the mechanisms of which are not understood. We investigated the effects of gastric distention on LES pressure in 16 normal subjects and 17 patients with gastroesophageal reflux disease. Intraluminal pressure was measured in the gastric fundus, LES, and esophageal body with a manometric catheter incorporating a sleeve device. Gastric distention was performed by injecting 0, 250, 500, or 750 ml of air in randomized order into a balloon and maintaining each stimulus for 15 min. Gastric distention did not significantly alter resting LES pressure in either group. During the basal period the rate of transient LES relaxation in the reflux patients (1.1 +/- 0.4 per 15 min) was greater than that in the normal subjects (0.6 +/- 0.1 per 15 min). Gastric distention resulted in a significant threefold to fourfold increase in the rate of transient LES relaxations in both groups. The reflux patients had a significantly greater proportion of complete relaxations (87%) than did the normal subjects (73%). We conclude that gastric distention, by significantly increasing the rate of transient LES relaxations in both normal subjects and patients with gastroesophageal reflux disease, may contribute to the postprandial increase in gastroesophageal reflux.
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