Cardiac hypertrophy in juvenile visceral steatosis (jvs) mice with systemic carnitine deficiency.

We have reported the clinical and biochemical findings in juvenile visceral steatosis (jvs) mice with systemic carnitine deficiency. This paper is the first report about cardiomyopathy in jvs mice. Adult jvs mice (at the age of 2-3 months) show cardiac hypertrophy which is caused by enlargement of the cardiac muscle cell associated with increases of non-collagen protein and DNA content. Carnitine administration (2 mg/head, twice a day, from 1 month of age) significantly suppresses the cardiac hypertrophy, showing that carnitine deficiency plays an important role in the development of the cardiac hypertrophy. The discovery of cardiac hypertrophy in carnitine-deficient jvs mice will lead to clarification of the pathophysiology of cardiomyopathy in systemic carnitine deficiency in human beings.

Pathological changes in skeletal muscle in HTLV-I-associated myelopathy.

The main lesion site of HTLV-I-associated myelopathy or tropical spastic paraparesis (HAM/TSP) is the pyramidal tract. In some HAM patients, clinical symptoms and findings indicate neuromuscular involvement, such as muscular atrophy, fasciculation, elevated serum creatine kinase (CK) or significant electrophysiological data. Cases of HAM/TSP complicated with polymyositis or motor neuron disease have been reported. But no investigation has been directed to muscular pathology in many patients of HAM/TSP. We conducted muscle biopsies on 13 HAM patients. Four patients showed neurogenic changes. Six patients showed histological findings indicative of inflammatory myopathy. We investigated surface marker of invading cells in these 6 patients. In all patients, T lymphocytes were more predominant than B lymphocytes and in three of them T helper/inducer cells were more predominant than T suppressor cells. In 2 patients, only slight myopathic change could be seen, such as variation in fiber diameter and increase in the number of internal nuclei. In 1 patient, type 2 fiber atrophy was seen, and was possibly the result of disuse. Disturbance of secondary motor neurons or inflammatory myopathy is thus shown to be possibly associated with HAM/TSP.

A device for controlling odorant stimulation and olfactory evoked responses in humans.

We developed a device for odorous stimuli control to record olfactory evoked responses from the human scalp. The characteristics of the apparatus are as follows. Translating the subject's respiration into electric signals with a sensor attached to the nose. The period and timing of odorous stimuli could be adjusted, so that stimuli could be synchronous with respiration. The interstimulus interval could be arbitrarily selected once every 1 to 9 respiration(s) so that adaptation could be prevented. We obtained evoked responses to odorous stimuli using this apparatus from the human scalp, whose positive peak latencies were approximately 350 and 700 msec. Such responses were not recorded if oxygen stimuli were used instead of odorous stimuli or with click sounds produced by the switching electromagnetic valve. Three types of odorant evoked scalp potentials were obtained in normal human subjects. The first type consisted mainly of two positive peaks with a peak latency of about 350 msec (P350) and about 700 msec (P700). The second type and the third type consisted of only one positive peak with a peak latency of about 350 msec and 700 msec, respectively. Such a P350 or P700 peak as that observed in the normal subjects was not detected in anosmic patients.

[A study of clinical symptoms, muscle pathology, therapeutic response, and prognosis of myositis in patients with HTLV-1 associated myelopathy (HAM)].

Myositis is one of well-known complications of HTLV-1 associated myelopathy (HAM). On twelve HAM patients, 10 women and 2 men complicated with myositis, we examined their clinical symptoms, muscle pathology, and therapeutic response. Clinical examination revealed gait disturbance in 10, weakness of upper limbs in 6, muscle atrophy in 4, and myalgia in 3 patients. Blood test disclosed elevated serum CK level in 6 patients. Muscle biopsy showed marked inflammatory changes with many necrotic and degenerating fibers in 7 patients, and the rest showed only focal invasion of inflammatory cells. Eight patients received corticosteroid or immunosuppressant therapy. The neurological symptoms were improved in 5 of 8 patients 3 months after the treatment. And only one out of 5 patients whom we followed up 3 years after the treatment showed improvement.

[Analysis of postoperative thoracic empyema].

We experienced 15 cases of pyothorax after thoracotomy. Six were pyothorax without bronchial fistula and 9 were with fistula. All of former were cured by the closed drainage and/or open window thoracostomy. Two of pyothorax with fistula, who were treated with the tube drainage, died of respiratory insufficiency. Five cases who underwent the open window thoracostomy survived well. Omentopexy was performed in 2 of pyothorax with fistula and results were excellent.

[Pulmonary arteriovenous fistula (PAVF) diagnosed by two-dimensional Doppler echography].

We report a case of pulmonary arteriovenous fistula (PAVF) with efficiency of the two-dimensional doppler echography for clinical evaluation. A 23-year-old female admitted to our hospital for lips cyanosis, exertional dyspnea and abnormal round shadow by chest X-ray. The two-dimensional echography demonstrated an efferent artery and a drainage vein of the PAVF. Pulmonary arteriography confirmed the diagnosis of a solitary PAVF located in the S10 of the left lung. The fistula was enucleated successfully with the recovery of cyanosis and dyspnea.

[Anesthetic management for a patient with mental retardation and unexamined complex congenital heart disease].

An 18-year old female with mental retardation and unexamined complex congenital heart disease received dental care under general anesthesia. Anesthesia was induced and maintained successfully without any significant hemodynamic changes with inhalation of nitrous oxide, oxygen (FIO2 0.25-0.3) and sevoflurane after a heavy premedication (morphine 10 mg, scopolamine 0.3 mg and midazolam 5 mg i.m.). After induction of anesthesia, cardiac anomaly was diagnosed by transesophageal echocardiography as TGA, VSD, PS, and operation was completed without any problem. Two points are considered important in this case; first, to appropriately estimate preoperative cardiac function and second, to adequately manage anesthesia to avoid any hemodynamic fluctuation.

Effects of starvation and protein deficiency on the acute carbon tetrachloride-induced hepatotoxicity.

The effects of starvation and protein-deficient diet on the acute carbon tetrachloride (CCl4)-induced hepatotoxicity of the mice were studied histologically and histochemically. A total of 69 male mice (C57BL/6) were used and carbon tetrachloride was administered by a single subcutaneous injection at a dose of 0.1 mg/10 g body weight after starvation for 24 hours and feeding with protein-deficient diet for one month. The changes were examined consecutively. It was shown the the CCl4-induced hepatotoxicity was exaggerated by starvation and that the regeneration after centrilobular necrosis due to CCl4 administration was retarded under this condition. In the CCl4-treated group on protein-deficient diet, the centrilobular liver cells seemed to be less responsive to CCl4.

Effect of ornithine concentration on citrulline synthesis in mouse liver mitochondria.

The rate of citrulline synthesis in mitochondria from OTC-deficient spf-ash mice (15% of the normal activity) was found to be the same as that in mitochondria from control mice. The amount of NAG in their mitochondria varied markedly according to whether they had received a high- or low-protein diet, and the rate of citrulline synthesis was found to be affected by the level of NAG. These results indicate that the CPS stage, not the OTC stage, is rate-limiting in the citrulline synthesis process. Kinetic studies on the effect of ornithine concentration on citrulline synthesis in mitochondria showed that the Km for ornithine was very low in the mitochondria from the mice given a low-protein diet. Kinetic studies on the effect of ornithine concentration on mouse OTC at various concentrations of carbamylphosphate showed that OTC has a ping-pong mechanism, i.e., that the Km for ornithine and Vmax decrease with the reduction in carbamylphosphate concentration. This may explain the low Km value observed in citrulline synthesis in the mitochondria. We conclude that in mitochondrial citrulline synthesis the rate of carbamylphosphate synthesis by CPS in the presence of NAG plays a key role in determining the rate of citrulline synthesis and ornithine dependency.

Cardiovascular and renal haemodynamic effects of dopexamine: comparison with dopamine.

We have studied the effects of dopexamine and dopamine on systemic and renal haemodynamics in 20 male patients undergoing elective coronary artery bypass surgery. Patients were allocated randomly to two groups (n = 10) who were treated with incremental doses of either dopexamine 1, 2 and 4 micrograms kg-1 min-1, or dopamine 2.5 and 5 micrograms kg-1 min-1, each dose being maintained for 15 min. Measurements were performed before administration of the drug and at the end of the infusion period at each dose. Fentanyl and midazolam were used as anaesthetic agents. Renal blood flow was measured with the argon washin technique. Dopexamine 4 micrograms kg-1 min-1 produced an increase in cardiac index of 117% caused by a 65% reduction in afterload and an increase in heart rate by 61%. Dopamine 5 micrograms kg-1 min-1 caused a 40% increase in cardiac index as a result of an increase in stroke volume. Renal vascular resistance decreased more than systemic vascular resistance with dopamine. With dopexamine, the increase in renal blood flow (66%) was less than the increase in cardiac index, while renal vascular resistance and systemic vascular resistance declined to almost the same extent. The results show that dopexamine exerts systemic and renal effects mainly via stimulation of beta 2-receptors. An action of dopexamine at renal DA1-receptors could not be demonstrated in this study.

[Surgical treatment of mycotic lung diseases].

Eighteen cases of mycotic lung disease, which included 17 pulmonary aspergillosis and one cryptococcosis, were treated surgically. Lobectomy was performed in 4, segmentectomy in 1, partial resection of lung in 4, cavernostomy in 2, open window thoracotomy in 4 and exploratory thoracotomy in 1. The authors would like to emphasize following points; 1) Of eighteen, cases, there were five patients over 70 years old and results of surgery in those were uneventful. 2) Two cases developed the postoperative empyema which was caused by the remnant lesion invaded with aspergillus fumigotus. From this experience it was considered that in the invasive aspergillosis the limited resection of the lesion was not recommended because of its local recurrence. 3) Omentopexy was effective for the treatment of mycotic empyema.

Antishock effect of gabexate mesilate (FOY).

The antishock effects of gabexate mesilate (FOY) were investigated in rats subjected to hemorrhagic hypotension, with particular attention focused on the stabilizing effects of hepatic lysosomes. The survival time of rats treated with FOY, infused for 2 hours intravenously at a rate of 50 mg/kg/hr, was significantly prolonged, accompanied by a tendency to decreased activity of plasma lysosomal enzymes. FOY, at a concentration of 10(-3) M, exerted a significant decrease in the extralysosomal release of acid phosphatase (15%, P less than 0.05), beta-glucuronidase (25%, P less than 0.05) and cathepsin D (25%, P less than 0.02) following hyposmotic labilization of lysosomes. Although the release of lysosomal enzymes might be counterbalanced by 50 mg/kg/hr of FOY, favorable cytochemical findings indicating stabilization of lysosomal membranes were consistently observed. The data suggest that FOY has antishock properties, and that these properties are caused, at least partially, by the stabilization of lysosomes.

Failure to detect HTLV-I by in situ hybridization in the biopsied muscles of viral carriers with polymyositis.

Direct infection of muscle fibers by human T-lymphotropic virus type I (HTLV-I) has recently been reported in a patient with polymyositis infected with both HTLV-I and human immunodeficiency virus (HIV). Coinfections of these viruses are frequently found in the United States. In Kagoshima, Japan, patients with polymyositis have a significantly increased incidence of seropositivity to HTLV-I alone, when compared with the general population of Kagoshima. In this study, we examined muscle tissue from 6 HTLV-I-positive patients with polymyositis from Kagoshima. To detect HTLV-I products, sensitive immunohistochemistry and in situ hybridization analysis were performed. These were compared with muscle fibers from a well-characterized transgenic mouse model which expressed HTLV-I tax. No specific signals were detected in the biopsied muscles of patients with polymyositis infected with HTLV-I alone. HIV co-infection may, therefore, augment HTLV-I expression through either immunosuppression or direct viral interactions.

Anterior rib strut grafting for the treatment of malignant lesions in the thoracic spine.

Anterior stabilization during thoracotomy, using the patient's own ribs, was carried out seven times in six subjects with malignant lesions of the thoracic vertebrae. The primary malignancy was lung carcinoma in four cases, thyroid carcinoma in one case, and alveolar soft tissue sarcoma in one. Resection of the lung tumor and spinal surgery were carried out simultaneously. Four of the six patients had complete paraplegia and two had partial paraplegia prior to the operation. After the decompression and anterior stabilization, three subjects responded well and three responded poorly because of respiratory insufficiency.

Malignant pheochromocytoma arising in the organ of Zuckerkandl--a clinicopathological study.

An autopsy case of malignant pheochromocytoma arising in the organ of Zuckerkandl is reported, of which distant metastases were found in the liver and lymph nodes. The biochemical and electron microscopic findings from the tumor tissue showed noradrenaline-secreting type. It was revealed that sex ratio in malignant pheochromocytoma was more prominent in the female, and the frequency of extra-adrenal origin was higher in malignant pheochromocytoma than in benign one from comparative study of reported cases of malignant pheochromocytoma.