DETECTION OF ANTIRIBOSOMAL P PROTEIN ANTIBODIES IN PATIENTS WITH SYSTEMIC SCLEROSIS

This study investigated the prevalence and clinical significanceof anti-ribosomal P protein (anti-P) antibodies in patientswith systemic sclerosis (SSc). Serum samples from 150 patientswith SSc were examined by indirect immunofluorescence, ELISAand immunoblotting. Anti-P antibodies were detected in four(3%) patients with SSc. Three of the four patients showed SSc/SLE(systemic lupus erythematosus) overlap syndrome, but psychiatricdisorders were not observed in these patients. By longitudinalimmunoblotting analysis one patient, who was initially diagnosedwith SSc, later developed anti-P antibodies along with clinicalmanifestations of SLE. Our data suggest that anti-P antibodiesare uncommon in SSc and that the presence of anti-P antibodiesin patients with SSc indicates an overlap with SLE. KEY WORDS: Anti-ribosomal P protein antibodies, Systemic sclerosis, Systemic lupus erythematosus     

Application of 4D-CTA using 320-row area detector computed tomography on spinal arteriovenous fistulae: initial experience

Time-resolved computed tomography angiography (4D-CTA) using a 320-row area detector CT scanner has recently been applied in the evaluation of cranial vascular disorders. However, application of 4D-CTA to spinal vascular disorder evaluation has never before been described. The authors herein report their initial experience of 4D-CTA in the evaluation of spinal arteriovenous fistulas (AVFs) and compare this novel modality with other imaging modalities. Four consecutive patients with spinal AVF underwent time-resolved contrast-enhanced magnetic resonance angiography (trMRA), 4D-CTA, and selective catheter angiography (CA). In 4D-CTA, volume data was transformed into 3D volume-rendered images and maximum intensity projection. These images were also evaluated by time-resolved serial phases. Then, images of each modality were compared, focusing on the detection of perimedullary draining veins and the prediction of AVF location and drainage flow direction. All modalities successfully detected perimedullary draining veins in all cases. Location of the AVF was detected in all cases by CA. trMRA and 4D-CTA detected the AVF in three out of the four cases. With regard to flow direction, while 4D-CTA successfully depicted ascending or descending drainage flow in the spinal canal, CA failed to detect the flow direction in one case while trMRA failed in two cases. In the case with epidural AVF, 4D-CTA was the only technique to detect the flow direction of perimedullary drainage. Although this is only an initial experience of the application of 4D-CTA to spinal vascular diseases, 4D-CTA was capable of detecting the dynamic vascular flow of spinal AVFs. The authors believe that 4D-CTA can be a useful option in the evaluation of spinal AVFs.     

Currarino triad: a case report

The authors report a case of Currarino triad with a combination of anterior sacral meningocele and mature teratoma, sacral body deformity, anorectal stenosis, and tethered cord. A newborn girl suffered from vomiting, abdominal distension and constipation. Initially, a diverting colostomy was performed at the age of one month. 7 months later, at the age of 8 months, we performed posterior sagittal anorectoplasty (PSARP): As a result, extirpation of teratoma, excision of meningocele, untethering of the spinal cord, and anorectoplasty were achieved simultaneously without complication. We suggest the use of an MRI to specify the presence of anosacral and spinal cord anomalies in patients with intractable constipation and we recommend combined pediatric and neurosurgical assessment and management for all cases of Currarino triad.     

A Study of Malignant Bile Duct Stenosis Using Percutaneous Transhepatic Cholangioscopy

Abstract: From June, 1987 to November, 1989, 11 patients with malignant bile duct stenosis, which was later confirmed by surgery or autopsy, were examined by percutaneous transhepatic cholangioscopy (PTCS) and by an endoscopic biopsy. The endoscopic findings obtained with the usual observation methods and methylene blue staining and the histological findings of the biopsy specimens were compared. A fine vascular proliferation was seen in all of the patients and a granular appearance was noted in patients with carcinoma of the major papilla. The papillary appearance was noted in one case each of pancreatic carcinoma, bile duct carcinoma and carcinoma of the major papilla. Marginal protrusion was not noted in patients with pancreatic carcinoma. A distorted narrow segment was seen only in cases of pancreatic carcinoma. The presence of a granular appearance indicated that the carcinoma was exposed on the surface. A high degree of fine vascular proliferation and a papillary appearance tended to indicate a carcinoma which invaded mainly into the fibromuscular layer without invasion of the mucosa. The methylene blue staining method was simple and effective for better visualization of the surface structure of the abnormal area, normal mucosa and the border zone.     

Three-dimensional analysis of occlusal curvature in healthy Japanese young adults

Summary  Knowledge of the standard value of the occlusal curvature would be helpful when providing oral rehabilitation for patients with or without occlusal derangement. Dentists often use a 4-inch radii arc as the standard based on Monson spherical theory. However, the validity of application of this theory in Japanese has not yet been verified. The objective of this study was to determine the typical shape of the occlusal curvature in Japanese adults and investigate the relative contribution of each factor to the depth of the curvature, as the initial phase, to prove the validity of determination of occlusal curvature. Seventy-nine Japanese adults (42 males and 37 females, aged 18 to 37 years) with intact dental arches were recruited, and the occlusal curvature was estimated by calculating the radius and center position of the approximate sphere. Besides, the relative contribution of gender, age, dental arch length and width, overjet, overbite for the radii was calculated. The median radius of the sphere was 110·6 mm, larger than the 4-inch value advocated by Monson. This indicates the necessity to reconsider the application of the method of occlusal plane analysis in Japanese individuals. Median position of the centre was 64·6 mm anterior to and 68·5 mm above the mid-point between condyles. Relative contribution of overbite for the radii was 10·9%, the highest amongst the factors. Therefore, a typical shape of the occlusal curvature was observed in Japanese subjects. Overbite is considered as one of the principal factors correlated to the depth of the occlusal curvature.     

Synaptic Development and Related Disturbances*

Abstract Characteristics of synaptic development are as follows: 1) an increase-decrease in the number of synapses consisting of an over-production followed by elimination, the end product being the maturing form; 2) with maturation, there is a transformation from the axo-dendritic shaft synapses to the axo-dendritic spine ones. In early development, synaptogenesis probably proceeds simultaneously with myelination. A suggestion that the critical period giving definitive impairments in the organization of synapses exists may be raised. Quantitative analyses of the mitochondrial number in. the presynapse, determined using conventional osmium tetroxide staining and the number of synaptic junction determined using the EPTA preferential procedure reveal the synaptic changes, both in normal development or in response to nutritional and pharmacological factors.     

Hybrid Therapy of Radiofrequency Catheter Ablation and Percutaneous Transvenous Mitral Commissurotomy in Patients With Atrial Fibrillation and Mitral Stenosis

AF Ablation and PTMC. Background: The rhythm control of atrial fibrillation (AF) associated with mitral stenosis (MS) is often difficult using antiarrhythmic drugs (AADs), even after a percutaneous transvenous mitral commissurotomy (PTMC). Few studies have examined the efficacy and safety of simultaneously performing radiofrequency catheter ablation (RFCA) and a PTMC in patients with MS and AF. Methods: Twenty consecutive patients with drug‐resistant AF and rheumatic MS underwent RFCA combined with a PTMC (n = 10; persistent AF‐8, long‐lasting [>1 year] persistent AF‐2; RFCA group) or transthoracic direct cardioversion (DC) following a PTMC (n = 10; persistent AF‐7, long‐lasting persistent AF‐3; DC group). In all patients, the mitral valve morphology was amenable to a PTMC, and more than 2 AADs had been ineffective in maintaining sinus rhythm (SR). In the RFCA group, a segmental pulmonary vein isolation (PVI) was performed in the initial 5 patients, and an extensive PVI was performed in the remaining 5. Results: During a mean follow‐up period of 4.0 ± 2.7 years, 8 patients (80%) in the RFCA group were maintained in SR, as compared to 1 (10%) in the DC group (hazard ratio, 0.16; 95% confidence interval, 0.03 to 0.75; P = 0.008 by the log‐rank test). The prevalence of the concomitant use of class I and/or class III AADs was comparable between the 2 groups (P = 0.70). No complications occurred during the procedure or follow‐up period in either group. Conclusions: The hybrid therapy using RFCA and a PTMC was safe and feasible, and significantly improved the AF free survival rate compared to DC following a PTMC. (J Cardiovasc Electrophysiol, Vol. 21, pp. 284–289, March 2010)     

What does Reelin actually do in the developing brain?

In mammalian brains, most neuronal cells form a layer structure. This structure is established thorough correct migration of neuronal cells, and underlies proper functions of the brain. It is thus important to understand the molecular mechanism that regulates neuronal migration and layer formation. Reelin is a large secreted protein essential for neuronal migration and layer formation in mammals. Since its identification in 1995, a number of models and hypotheses have been proposed regarding Reelin function. However, tbe primary role of Reelin in the developing brain and its underlying molecular mechanism still remain unresolved. In this review, I try to summarize what is known and what we can infer about Reelin. I also mention why studying Reelin is difficult.     

Hemangioendothelioma of the brain presenting with intracerebral hemorrhage: a case report]

Hemangioendothelioma (HE) is an uncommon vascular tumor that is intermediate in histological appearance between a hemangioma and an angiosarcoma. Presently, it is regarded as endothelial tumors of low-grade or intermediate malignancy. It has been reported in the liver, lung, heart, mediastinum, lymph nodes, extremity, and bone. The occurrence in the brain is extremely rare; only 16 cases have so far been reported. We report a 51-year-old woman who presented with transient visual disturbance and weakness of the left upper limb on April 12th 2003. Computed tomography (CT) revealed a high density mass in the right parietal lobe. In magnetic resonance imaging (MRI), the lesion is hyperintense on TIWI, isointense on T2WI, and no enhancement with gadopentetate dimegliumine. Intratumoral hemorrhage was indicated and preoperative diagnosis was cavernous angioma. The tumor was excised completely on April 28th 2003. Pathologically, the tumor cells resembled endothelial cells, positive immunoreactivity for Factor VIII, and grew in small nests or cords. Postoperative MRI showed complete removal of the tumor. There has been no recurrence for 8 months after the surgery, but we have to follow MRI up for a long time. We discussed intracerebral HE clinically and neuroradiologically.     

The usefulness of ureterorenoscopic examination in evaluation of upper tract disease

AIM: The objective of the present study was to clarify the indications, usefulness and limitations of ureterorenoscopy. MATERIAL AND METHODS: From January 1998 to June 2004, 72 consecutive patients (48 men and 24 women) with a mean age of 66 years (range, 27-83 years) underwent ureterorenoscopy to diagnose upper urinary tract tumors (UUT). Median follow-up was 24 months (range, 1-73 months). Patients were divided into four subgroups by voided urine cytology and preoperative radiographic findings. Group A (n=11, 15.3%), positive voided urine cytology and positive preoperative radiographic findings; group B (n=5, 6.9%), positive cytology and negative radiographic findings; group C (n=48, 66.7%), negative cytology and positive radiographic findings and group D (n=8, 11.1%), frank hematuria originating from the UUT but negative cytology and negative radiographic findings. We compared the findings of ureterorenoscopic examination and biopsy with the results of retrograde pyelography and cytology of upper tract urine. For each examination, the following diagnostic indices were assessed: sensitivity, specificity, positive-predictive-value (PPV) and negative-predictive-value (NPV) and accuracy. Statistical analysis was performed using McNemar's test. RESULTS: For ureterorenoscopy, sensitivity was 94%, specificity 59%, PPV 72%, NPV 92% and accuracy 76%. For biopsy, sensitivity was 77%, specificity 100%, PPV 100%, NPV 80% and accuracy 88%. Accuracy of ureterorenoscopy tended to be superior to that of retrograde pyelography. Ureterorenoscopy was most useful in the group which consisted of 48 patients (66.7%) with negative voided urine cytology and positive preoperative radiographic findings. This group was the only group in which accuracy of ureterorenoscopic biopsy was superior to that of urine cytology, significantly (P=0.03). CONCLUSION: Results indicated that ureterorenoscopy is most suitable and gives superior accuracy in patients with positive radiographic findings and negative voiding cytology. Ureterorenoscopic biopsy of the upper urinary tract would provide useful information when considering therapeutic strategies, such as nephron-sparing management.