Case of dermatomyositis complicated with massive pleural effusion that preceded the myopathy]

A 36-year-old man was admitted to a hospital with complaints of fever, polyarthralgia and dyspnea. Erythema was observed on his face, extensor surface of the fingers and extremities, and a chest X-ray revealed massive bilateral pleural effusion. He had no sign of myopathy at this point. Pleural fluid was proved to be exudative and contained extremely high levels of hyaluronic acid. He was also complicated with interstitial pneumonitis and was given a pulse therapy with methyl prednisolone followed by daily administration of 55 mg prednisolone (PSL). Twenty days after the commencement of the therapy, pleural effusion decreased but muscle weakness gradually appeared, accompanied by elevation of myogenic enzymes. Myogenic changes on electromyogram, and irregularity of the muscle fibers with slight inflammatory cell infiltrates in a biopsy specimen were demonstrated. He was transferred to our hospital, and a diagnosis of dermatomyositis was made. Later, pleural effusion waxed and waned depending on the dosage of PSL, but no other causative disorder was demonstrated by extensive examinations. This case indicates that the pleuritis could be one of the vasculitic manifestations of dermatomyositis.     

A case report of spinal epidural hematoma complicated after open heart surgery]

The authors reported the first case of acute spinal epidural hematoma (SEH) developed after open heart surgery. The patient was noticed that her legs felt weak and numb on the first postoperative day evening. On the next day morning, neurological examination revealed that flaccid paralysis of both legs and also loss of all sensory perception below the level of Th-6 spine bilaterally. The prolonged effect of anesthesia and painless onset made delayed recognition of the lesion. SEH (Th5-7) was diagnosed with MRI and decompressive surgery was immediately done, sixty hours after the beginning of cardiac operation. But in this case neurological deficits were not changed. We concluded that a routine diagnostic approach was very important procedure to find out this serious complication for all patients underwent open heart surgery in early period of its onset.     

Significance of the BTA Test in Bladder Cancer: A Multicenter Trial

Background :
The BTA test is a latex agglutination assay for the qualitative detection in the urine of analytes that are associated with bladder tumor. We compared the results of the BTA test with those of voided urine cytology (VUC) in patients with bladder cancer.
Methods :
A multicenter trial was performed at 6 institutions. A total of 132 patients with histologically diagnosed bladder cancer were enrolled. Urine samples were split for BTA and VUC testing.
Results :
The sensitivities of the BTA test and VUC were 57.6% and 37.9%, respectively; this difference was significant ( P < 0.001). The BTA test had much higher sensitivity for small, solitary, superficial tumors than did VUC.
Conclusion :
The BTA test is simple to perform, gives rapid results, and is far more sensitive than VUC for detection of bladder cancer. The BTA test has the potential to become an additional tool for detecting bladder cancer.     

Malignant Rhabdoid Tumor of the Breast: A Case Report

A 66-year-old woman time of 10 days. One month after radicalmastectomy, there was local recurrence, followed by multiplepulmonary metastases, and the patient died of respiratory failure5 months after surgery. The gray-white-colored tumor measured13x12x;10 cm, and its border was well defined. The tumor wascomposed of diffusely growing round or polygonal cells withvesicular nuclei, prominent nucleoli, and ample cytoplasm containingeosinophilic inclusions. Lymph node involvement was widespread.Both vimentin and keratin were clearly demonstrated by immunohistochemicalstaining. Ultrastructural studies revealed that the MRT cellscontained cytoplasmic whorls of intermediate filaments.     

Molecular cloning of a novel human papillomavirus (type 60) from a plantar cyst with characteristic pathological changes.

In a plantar cyst composed of the wall of the squamous cell layer and the horny inner substance in the lower dermis, we found characteristic pathological changes, such as cytoplasmic eosinophilic inclusions and vacuolated structure, and, immunohistochemically, the papillomavirus capsid antigen. The human papillomavirus (HPV) DNA cloned from the cyst showed no homology with other known prototypes of HPV (HPV 1 through HPV 59) by Southern blot analysis under stringent conditions and was named as HPV 60. HPV 60 DNA was found in three other cases of plantar cyst with the identical pathological changes, but not in a plantar cyst without such changes. The results suggest that HPV 60 has unique biological properties to induce a plantar cyst as a distinct type of cutaneous HPV.     

Operative results with total composite replacement of the aortic valve and ascending aorta]

From December 1980 to December 1990, ten patients, 9 male and 1 female, ranging in age from 21 to 68 years, were operated on for aortic valve insufficiency associated with an aneurysm of the ascending aorta. The surgical treatment in all cases consisted of total replacement of the ascending aorta with Bentall's procedure (n = 4), or Cabrol's procedure (n = 6). In 5 patients an uncomplicated annulo-aortic ectasia existed. Three of them had annulo-aortic ectasia with an aortic dissection. One had aortitis syndrome, and one had syphilitic aortitis. The operative mortality for the entire group was 0% (0 death). Hospital survivors revealed satisfactory clinical improvement in NYHA class (mean value: 3.2 to 1.0). Late complications developed in 2 of the 10 patients. They had a picture of pseudoaneurysm formation at the anastomoses between the graft and the right coronary 46 months and 15 months, respectively, after the initial operation. Despite the reoperation, one died of hepatic failure 30 days after the operation, and the other died of postoperative bleeding at the anastomosis sites. We, furthermore, considered the difference in aortic cross clamp time and cardiopulmonary bypass time between Bentall's procedure and Cabrol's procedure. Aortic cross clamp time and cardiopulmonary bypass time were significantly shorter in Cabrol's procedure than in Bentall's procedure, if a probability value less than 0.20 was considered to be of statistical significance. We were able to conclude that the treatment of aortic valve regurgitation associated with an aneurysm of the ascending aorta by insertion of a composite graft is a reliable method with low operative mortality and excellent long term results, especially in Cabrol's procedure.     

Serum sialyl Tn antigen as a prognostic marker in patients with epithelial ovarian cancer]

Circulating serum sialyl Tn (STN) antigen levels were measured in 89 patients with epithelial ovarian cancer, 157 benign disease, and in 126 healthy controls. Serum antigen levels were increased in 48.3% of patients with ovarian cancer. The false positive rate is significantly low (4.0% in healthy controls and 9.6% in benign disease). The levels of STN antigen were significantly higher in sera of patients with cancer than in those in benign and healthy controls (p less than 0.05). The rise in serum STN antigen levels correlated to the size of the primary tumors. Of the histological type, it is interesting to note the high sensitivity in mucinous-type ovarian cancer. Survival at 1, 2, 3, 4 and 5 years for patients with STN-negative (serum STN levels less than 50 U/ml) versus STN-positive (serum STN levels greater than or equal to 50 U/mol) was 96.2, 92.3, 86.5, 82.7, and 76.9% versus 59.5, 29.7, 18.9, 10.8, and 10.8%, respectively (p less than 0.05). The overall survival probability was worse in patients with STN-positive sera. Percent progression-free survival at 1, 2, 3, 4 and 5 years for patients with STN-negative versus STN-positive was 90.4, 86.5, 76.9, 59.6, and 51.9% versus 35.1, 16.2, 8.1, 8.1, and 5.4%, respectively (p less than 0.05). The overall progression-free period of survival was shorter in patients with STN-positive sera. Multivariate regression analysis revealed that positive STN, stage, PS and histologic grade were the four variables of most importance in predicting survival. These results indicate that a positive STN antigen level in sera is an independent predictor of poor prognosis in ovarian cancer.     

Clinical results of staged repair with complete unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

OBJECTIVE: Our treatment strategy for pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries is a staged repair that comprises the first complete unifocalization (UF) with 'unification' of intrapulmonary arteries and then the definitive repair. The purpose of this study is to evaluate the outcome of our staged repair strategy with complete UF and to determine the results of our current management strategy. METHODS: From 1982 to 2004, 113 consecutive patients were treated with staged repair at our institute. We evaluated the risk of definitive repair failure or death in the 3 years after definitive repair using logistic regression. Furthermore, we compared the early group (patients who underwent UF before December 1995) and the late group (patients who underwent UF after January 1996). RESULTS: The mean follow-up interval was 8.8 years (0.8 months to 23.3 years), and Kaplan-Meier-estimated overall survival rates after first UF were 80.9, 73.8, and 69.9% at 5, 10, and 15 years, respectively. Survival in patients with an absent central pulmonary artery (PA) was significantly lower than in those with a central PA (p<0.05), and the factor that was significantly associated with definitive repair failure or death in the 3 years after definitive repair was central PA morphology (p<0.05). Higher mean PA pressure after UF was detected in patients with hypoplastic central PA, compared with those without hypoplastic PA (30.9 mmHg vs 23.3 mmHg, p<0.05). In the late group, age (in years) at first UF (3.9 vs 8.4, p<0.01), second UF (4.3 vs 9.2, p<0.01), and definitive repair (5.8 vs 9.1, p<0.01) was significantly younger than in early group, and the survival rate after first UF in the late group was 96.2 and 91.3% at 3 and 7 years, respectively. Systolic right ventricular pressure and the pressure ratio between the right and the left ventricles after definitive repair in the late group were significantly lower than in the early group (53.6 mmHg vs 75.0 mmHg, p<0.01; 61.7% vs 75.9%, p<0.05). CONCLUSIONS: Hypoplastic central PA was a significant risk factor in this disease. The overall survival was improved by our current management strategy. Improved RV pressure after definitive repair appears to affect the long-term outcome.     

Increase of oxidized/total coenzyme Q-10 ratio in cerebrospinal fluid in patients with Parkinson's disease.

The concentrations of oxidized coenzyme Q-10 (CoQ-10) and reduced CoQ-10 in the cerebrospinal fluid (CSF) of patients with Parkinson's disease (PD) was examined in order to determine whether the balance in oxidized and reduced CoQ-10 is related to the pathogenesis of PD. The percentage of oxidized/total CoQ-10 (%CoQ-10) in the CSF was significantly higher in the untreated PD group (80.3+/-17.9%) compared to the normal control group (68.2+/-20.4%) (p<0.05). The %CoQ-10 in the CSF of PD patients showed significant negative correlation with the duration of illness. These findings in living patients provide in vivo evidence for a possible role for %CoQ-10 in the pathogenesis in the early stages of PD development.