Non-blood contacting biventricular support for severe heart failure

BACKGROUND: Direct mechanical ventricular actuation (DMVA) is a non-blood contacting method of biventricular support. DMVA employs a vacuum attached, pneumatically regulated, flexible membrane to transfer both systolic and diastolic forces to the ventricular myocardium. The purpose of this study was to determine if DMVA effectively restores pump performance when applied to the severely failing heart. METHODS: Bovines (n = 10) underwent thoracotomy and were instrumented for continuous hemodynamic monitoring. Cardiac failure was induced by beta1-blockade to achieve a cardiac index of < 1.5 l/min/m2 for 1 hour. Heart rate was maintained at 100 bpm by atrioventricular sequential pacing. Synchronous DMVA support was then applied for 3 hours. RESULTS: Eight animals achieved significant reductions in cardiac index and mean arterial pressures (35%* and 43%* control, respectively; *p < 0.05). DMVA restored cardiac index to baseline and significantly increased arterial pressures (p < 0.05; DMVA versus cardiac failure). Pulmonary flow and mean pulmonary artery pressures were similar to baseline during DMVA (p = NS). Pathologic exam did not demonstrate evidence of significant device trauma. CONCLUSIONS: DMVA support can effectively restore pump performance of the acutely failing heart. Synchronization may be inherent to the stimulus of cardiac compression. These data further substantiate DMVA's potential as an adjunct to the field of circulatory support.     

Late onset ataxia phenotype in dentatorubro-pallidoluysian atrophy (DRPLA)

We clinically and genetically studied three patients in a family with dentatorubro-pallidoluysian atrophy (DRPLA). The proband patient had 58/24 CAG repeat alleles of the DRPLA gene (normal ≤ 34 repeats). Cerebellar ataxia first developed in the 6–7^th decades and was the predominant feature for more than 10 years in all three, after which two of them manifested dementia and choreiform movements in the advanced stage. Atrophy of the cerebellum and brain stem an CT or MRI had suggested dominant spinocerebellar ataxia as a diagnosis in their ataxia-predominant stage, with a diagnosis of DRPLA being impossible based on the clinical findings alone. Our experience implies that DRPLA must be taken into account in the differential diagnosis of late onset ataxic disorders, since it can easily be overlooked. Received: 2 April 2001, Received in revised form: 23 July 2001, Accepted: 21 August 2001     

Pre-treatment with ketamine reduces incidence and severity of pain on propofol injection

The purpose of this study was to evaluate the effect of pre-treatment with ketamine on the reduction of pain during injection of propofol in adult patients. We conducted a prospective, randomized, double-blinded trial. Forty-three patients were randomly allocated to one of two groups according to the agents administered before hand; Group C, normal saline 2 ml and Group K, 1% ketamine 2 ml. The pain on injection was rated as none, mild, moderate, or severe. Sixty-eight percent of patients in the C group experienced pain, while 33% of patients experienced pain in the K group. Thirty-six percent of patients in the C group complained moderate to severe pain but only 9% of patients in the K group. The mechanisms of prevention by ketamine of the pain on propofol-injection could not be clarified from our study, but it may be related to central effects of ketamine. In conclusion, ketamine pre-treatment before propofol administration significantly reduces incidence and severity of pain associated with propofol injection.     

Successive cultures of mature hepatocytes for hepatocyte autotransplantation to assist liver function after liver resection for cancer

We developed a method for the rapid successive cultures of adult rat mature hepatocytes on plastic dishes while avoiding viral transformation or co-culture with other cell lines. This method also allows for culturing adult human mature hepatocytes up to the secondary culture. These can be expected to provide a good source for hepatocyte autotransplantation, and, combined with the previously reported methods for the transplantation of hepatocytes into the spleen, a promising option for the support of liver function after liver resection for cancer without the need for immunosuppressive agents.     

Evaluation of preoperative simultaneous radiochemotherapy combined with carboplatin and tegafur.uracil

We performed preoperative radiochemotherapy including local irradiation with total dose of 40 Gy along with concomitant combination chemotherapy with carboplatin and tegafur.uracil for 11 head and neck squamous cell carcinomas and evaluated the clinical and histopathological effects as well as the side effects. Seventy-three percent of patients accomplished significant clinical effects equal to or more than PR and significant histopathological effects in surgically resected tissues. Side effects were seen in only one patient with severe hypoleukocytemia, no patients with renal dysfunction, and 34% of patients with severe oral mucositis. These results suggest that our radiochemotherapy may be useful for preoperative treatment for patients with locally advanced head and neck squamous cell carcinomas.     

Clinicopathological features of genetically confirmed Danon disease

BACKGROUND: Danon disease is due to primary deficiency of lysosome-associated membrane protein-2. OBJECTIVE: To define the clinicopathologic features of Danon disease. METHODS: The features of 20 affected men and 18 affected women in 13 families with genetically confirmed Danon disease were reviewed. RESULTS: All patients had cardiomyopathy, 18 of 20 male patients (90%) and 6 of 18 female patients (33%) had skeletal myopathy, and 14 of 20 male patients (70%) and one of 18 female patients (6%) had mental retardation. Men were affected before age 20 years whereas most affected women developed cardiomyopathy in adulthood. Muscle histology revealed basophilic vacuoles that contain acid phosphatase-positive material within membranes that lack lysosome-associated membrane protein-2. Heart transplantation is the most effective treatment for the otherwise lethal cardiomyopathy. CONCLUSIONS: Danon disease is an X-linked dominant multisystem disorder affecting predominantly cardiac and skeletal muscles.     

The microvasculature of the human cerebellar meninges

The vascular architecture of the human cerebellar meninges was investigated. The surface meninges were poor in vasculature. In the sulci, the meninges were highly vascular but had few capillaries. The venous blood vessels gave long side branches at right angles to the parent vessels in a cruciform pattern, running horizontally along the cerebellar sulci. They were situated at the origin of the secondary or tertiary sulci. Anastomoses between these horizontal branches gave a crosshatched appearance. Short branches often extended to the bases of the sulci, terminating in T-shaped bifurcations with numerous tiny branches, like the roots of a tree. The arteries ran perpendicular to venous branches which were parallel to each other exclusively along the sagittal plane. These arteries bifurcated to straddle the horizontally running veins at the origin of the secondary or tertiary sulci. They gave off many small branches like teeth of a fork from each artery in the secondary or tertiary sulci after they bifurcated to straddle the venous branches and penetrated the cerebellar cortex at the bases of sulci. These fork-like ramifications in the bases of the sulci were most likely responsible for the ready development of pronounced ischemic state. They might also play an important role in the occurrence of ischemic damage at the bases of sulci in cases of severe generalized ischemia.     

Comparison of primary thyroid lymphoma with anaplastic thyroid carcinoma on computed tomographic imaging

PURPOSE: Primary non-Hodgkin's lymphoma (LY) and anaplastic carcinoma (AC) of the thyroid gland are rare malignant tumors, and the initial symptoms of these diseases are very similar. The aim of our study was to compare the characteristics of the two diseases using computed tomographic (CT) scans in order to make an accurate differential diagnosis. MATERIALS AND METHODS: Ten patients with LY and 10 with AC were analyzed. Differences in the CT findings of the two diseases were evaluated before treatment and statistically tested with either Student's t-test or the chi-square test. RESULTS: In the analysis of characteristics of CT imaging, the existence of calcification and necrosis, and heterogeneous tumor were dominant findings in AC, and there was a statistically significant difference in frequency between the two diseases (p < 0.01). Calcification detected in AC was usually multiple and/or gross (mean size: phi8.2 mm). All lymphadenopathies were delineated as having the same homogeneous attenuation as the tumors in the thyroid gland in LY, but were shown as irregular rim enhancement in AC. CONCLUSION: The CT features of the two diseases are characteristic in terms of calcification, necrosis, and tumor composition. Evaluation by means of CT imaging is useful in distinguishing between LY and AC.     

Central core disease and congenital neuromuscular disease with uniform type 1 fibers in one family

We report a family in which the father had central core disease and his son had congenital neuromuscular disease with uniform type 1 fibers. This is the first report of such a combination. Although they had no recognized mutation in the ryanodine receptor gene, it is highly likely that the son also had central core disease but without core structures. The absence of cores may be due to the muscle sample or the young age of the patient since core structures have been reported to increase with age. Although the prevalence of core structures in individual muscles is unknown, there is a possibility of sampling error. In some patients, congenital neuromuscular disease with uniform type 1 fibers is closely related to or identical with central core disease.     

Changes in tissue-plasminogen activator mRNA expression following cortical ablation in the rat brain

Tissue plasminogen activator (tPA) has been used to treat acute thrombotic lesions. Roles other than the activation of fibrinolytic pathways have been suggested for tPA in the mature brain. We used the in situ hybridization technique to investigate the changes in tPA mRNA expression within the brain after cortical ablation. We found that expression of tPA mRNA started to increase diffusely in the cortex ipsilateral to the injury 6 h after ablation. This increase had become prominent 24 h after ablation. On d 5, the expression of tPA mRNA had returned to that of the control animals except for the area near the injury. We also found that administration of MK-801 before injury suppressed the increase of tPA mRNA in the ipsilateral cortex. These results suggest that the increase in tPA mRNA is likely to be mediated via activation of NMDA receptors.