Successful Norwood operation for single left ventricle associated with severe subaortic stenosis, coarctation of aorta and hypoplastic aorta]

A 4 month-old boy who had double-inlet left ventricle, severe subaortic stenosis, hypoplastic ascending aorta and coarctation of the aorta revealed severe respiratory failure. An urgent Norwood operation was done. The procedure consisted of enlargement of the ascending aorta and main pulmonary artery and a systemic-pulmonary shunt using 5 mm PTFE tube. Postoperative course was uneventful.     

Statistical analysis of the prostatic cancer patients detected from 1985 to 1989 in and around Gunma prefecture]

Prostatic cancer is one of the most common malignant tumors in the field of urology. The number of patients is increasing rapidly and its importance as a mortal disease is gathering attention. In 1985, we organized a registration system for prostatic cancer patients found in and around Gunma prefecture. In this study, we analyzed the clinical characteristics of the 730 patients registered from 1985 to 1989. The results were as follows. Mean age was 74.0 years old and the number of the patients was the greatest in the eighth decade. Voiding disturbance was the most common chief complaint, followed by pollakisuria, gross hematuria and miction pain. Stage and grade distribution were as follows. Stage A 16.2%, B 21.1%, C 17.0%, D 45.7%, well differentiated 27.4%, moderately differentiated 48.2% and poorly differentiated 24.5%, respectively. A statistically significant relationship between stage and grade was observed. Bone was the most common metastatic site. The highest incidence of bone metastasis was in lumbar vertebra, followed by ribs, ilium, thoracic vertebra and ischium. The value of PAP, ALP and ESR tended to be higher in high stage patients, and that of Hb was lower. Fifty two patients were detected by mass screening. Most of these patients were in an early stage. Most of the patients were treated by hormonal therapy. LH-RH agonists constituted 39.2% of the cases given hormonal therapy.     

The effects of long-term intake of a low concentration of sodium fluoride on bone loss in the growing senescence-accelerated mouse (SAM-P/6)]

The long-term effects of low concentrations of sodium fluoride (0, 1 and 5 mg/l) in drinking water on bone metabolism were examined in the growing senescence accelerated mouse (SAM-P/6) as a spontaneous experimental model of senile osteoporosis. In 4 and 8 months of age respectively, there were almost no differences in body weight, and serum calcium, phosphorus and alkaline phosphatase levels between controls and fluoride groups. Calcium contents per dry weight of femoral bone were higher in fluoride groups than in controls. The bone mass of the trabecular was not affected by the low-concentration sodium fluoride intake. However, sodium fluoride decreased the rate of bone mass loss associated with aging in the cortical bone in SAM-P/6 compared with the control. The results of this study suggest that, in growing SAM-P/6 mice, a long-term low-concentration sodium fluoride intake affects the skeletal metabolism.     

Measurement of the zeta potential of human platelets by the use of laser-light scattering.

An instrument was developed to detect the shift in scattering of laser light that occurs when particles in suspension move in a chamber with an electrical load. The instrument measures the zeta potential of particles. We applied the instrument to study human blood cells. Platelet-rich plasma was used because of the stability of the suspension, without the sedimentation or autoaggregation that is often seen with red or white blood cells. The reproducibility of the measurements was satisfactory when there were enough platelets in the suspension. Platelets from healthy controls (n = 136) had a potential of -14.20 +/- 1.64 mV at the detection angle of 17.1 degrees. Platelets from patients with essential thrombocytosis (n = 16) or polycythemia vera (n = 8) had higher potentials than the healthy controls.     

CCR1 acts downstream of NFAT2 in osteoclastogenesis and enhances cell migration.

We found that a chemokine receptor gene, CCR1, acts downstream of NFAT2 in RANKL-stimulated RAW264 and bone marrow cells. The upstream regulatory region of CCR1 showed RANKL-dependent and CsA-suppressible promoter activity. Downregulation of the expression and function of CCR1 suppressed cell migration. INTRODUCTION: We previously reported that the expression of NFAT2 induced by RANKL is a key process for progression to multinucleated cells in an in vitro osteoclastogenesis system. Identifying the target genes of NFAT2 would thus be informative about the differentiation process. We focused here on chemokine and chemokine receptor genes that act downstream of NFAT2 in RAW264 cells as well as osteoclast precursors prepared from bone marrow cells. MATERIALS AND METHODS: RAW264 mouse monocyte/macrophage line cells were cultured with or without cyclosporin A (CsA) in the presence of RANKL or glutathione S-transferase (GST). Osteoclast precursors were prepared from bone marrow cells. RANKL-inducible and CsA-suppressible genes were searched for by microarray analysis, and expression was confirmed by quantitative RT-PCR. Promoter activity was measured by luciferase gene reporter assay. Short interfering (si)RNA for CCR1 was introduced in RAW264 cells. Cell migration activity was examined using a Boyden chamber assay. RESULTS AND CONCLUSIONS: We identified the chemokine receptor gene CCR1 as a gene showing significant differential expression profiles in osteoclastogenesis in the presence versus the absence of CsA, an inhibitor of NFAT. This property was unique to CCR1 among the chemokine and chemokine receptor genes examined in both RAW264 and bone marrow cells. The upstream regulatory region was isolated from CCR1, and its RANKL-dependent and CsA-suppressible promoter activity was confirmed. The functional significance of CCR1 was assessed by monitoring the migration of cells in a transwell migration assay, and this activity was abolished when either CsA- or CCR1 siRNA-treated cells were used. Moreover, treatment with a Galpha inhibitor pertussis toxin (PTX) or methiolynated-regulated on activation, normal T cells expressed and secreted (Met-RANTES), an antagonist of CCR1, suppressed multinucleated cell formation in the bone marrow cell system. Together, these results suggest that the CCR1 signaling cascade is under the control of NFAT2 and seems to enhance the migration of differentiating osteoclasts.     

Eosinophilic fasciitis associated with Borrelia afzelii infection]

A 58-year-old woman suffered from stiffness, swelling, rubor and muscle pain in the extremities one month after she climbed a mountain in Kyushu, an island in southern Japan. On admission, mild proximal weakness was present in the extremities. Her range of motion in the extremities was limited due to firm skin and subcutaneous stiffness which was similar to scleroderma. She showed peripheral blood eosinophilia and hypergammaglobulinemia as well as a high erythrocyte sedimentation rate. An IgM antibody against Borrelia afzelii was positive. MRI of the skeletal muscles demonstrated enhancing fascia around the biceps brachii muscle, and a biopsy specimen revealed perivascular infiltration of mononuclear cells within the hypertrophic fascia. Eosinophilic infiltration was absent. We treated the patient with prednisolone, doxycycline and amoxicillin, which alleviated the symptoms. This is the first report to show that Borrelia afzelii was involved in eosinophilic fasciitis.     

Radical correction of paracardiac type IIb of total anomalous pulmonary venous connection using pedicled flaps of right atrium and atrial septum

Surgical results of total anomalous pulmonary venous connection (TAPVC) has been improved in recent years, however, late development of pulmonary venous obstruction was our concern in its total correction in early infancy. In the cardiac type of TAPVC, in which the pulmonary veins were connected to the right lateral wall, prosthetic patch is conventionally used in diversion of pulmonary venous flow into left atrium. It seemed favorable to repair this subset without using prosthesis. A 3-month-old female with TAPVCIIb according to Darling's classification underwent total correction on September 22th, 1988. Two pedicled flaps were developed using the right atrial wall and the atrial septum to create a pulmonary venous channel to divert arterial blood into left atrium and absorbable sutures were used throughout. Right atrium was entered through a vertical incision in its body and all the pulmonary veins were found in a recess in the lateral wall of the right atrium. Atrial septal defect in the cranial aspect of the fossa ovalis was enlarged by cutting the primum tissue along the right limbus and its caudal margin so as to form a pedicled flap attached to the left limbic tissue. Then the flap was sutured along the limbus to create a roof of the fossa ovalis. The second flap was made in the middle of the lateral atrial wall and was used to create a tunnel from the recess to the atrial septal defect. The defect in the right atrial wall was closed directly and no prosthetic patch was used. Postoperative course was uneventful and echocardiogram showed wide pulmonary venous channel draining into the left atrium.     

Case of Parkinson's disease presenting with unique dyspneic attacks caused by oromandibular dystonia and sleep apnea syndrome]

A 79-year-old woman with a 4-year history of Parkinson's disease was admitted due to unique dyspneic attacks with cyanosis while eating. Dyspneic attacks with cyanosis occurred mainly during actions such as taking meals or rehabilitation. Due to increased tonus of the orbicularis oris muscle, she was unable to open her mouth and breathe out, and finally experienced hypoxemia as revealed by pulse oxymetry. Dystonic hypertonus was relieved by touching the mandible with the fingers, and she was able to open her mouth again. These symptom was compatible with the sensory trick. Based on these findings, we considered that dyspneic attacks were produced by focal oromandibular dystonia. Polysomnography also showed central sleep apnea. We report herein a rare case of Parkinson's disease presenting with respiratory insufficiency caused by focal dystonia and central sleep apnea.     

Left-side catamenial pneumothorax with endometrial tissue on the visceral pleura.

We present here a rare case of left-side catamenial pneumothorax, in which endometrial tissue pleura was found on the visceral pleura histologically. A chest roentogenogram confirmed the left pneumothorax, but did not reveal bullae or any associated anomalies, in a 41-year-old woman with three documented episodes of left-side pneumothorax occurring every menstruation. Video-assisted thoracoscopic surgery revealed not only tiny holes in the diaphragm, but also a scattered small brown spots on the visceral pleura. Histological examination of the lung sections revealed the existence of endometrial tissue on the visceral pleura with disrupted pleural elastic fibers. Our case suggests that cyclic erosion of the visceral pleura by the implanted endometrial tissue caused air leakage from the lung, in addition to the most accepted concept that air is aspirated into the thoracic cavity via the abdomen through the acquired fenestration of the diaphragm.     

Severely calcified constrictive pericarditis simulating a mediastinal tumor and obstructing the right ventricular inflow tract.

We report a rare case of constrictive pericarditis that stimulated a large mediastinal tumor obstructing the right ventricular inflow tract. A 58-year-old woman was referred to our hospital because of a recent increase of dyspnea and facial edema. Computed tomography revealed severely thickened calcification, including a low-density area, presenting as a mediastinal tumor, compressing the right ventricular inflow tract. A complete resection was performed, and her symptoms dramatically improved. Idiopathic constrictive pericarditis was diagnosed pathologically.