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Biomedical subjects

T Kondoh

Publications and source records attributed to T Kondoh.

At least 37 records · Page 2Linked to original sources
PubMed2001

Spontaneous in vivo reversion of an inherited mutation in the Wiskott-Aldrich syndrome.

The Wiskott-Aldrich syndrome (WAS) is an X-linked primary immunodeficiency disease, arising from mutations of the WAS-protein (WASP) gene. Previously, we have reported that mononuclear cells from WAS patients showed lack/reduced of the intracellular WASP (WASP(dim)) by flow cytometric analysis, and analysis of WASP by flow cytometry (FCM-WASP) was useful for WAS diagnosis. In this study, we report a WAS patient who showed the unique pattern of FCM-WASP. The patient had the small population of normal expression of WASP (WASP(bright)) mononuclear cells together with the major WASP(dim) population. The WASP(bright) cells were detected in T cells, not in B cells or in monocytes. Surprisingly, the molecular studies of the WASP(bright) cells revealed that the inherited mutation of WASP gene was reversed to normal. His mother was proved as a WAS carrier, and HLA studies and microsatellite polymorphic studies proved that the WASP(bright) cells were derived from the patient himself. Therefore, we concluded that the WASP(bright) cells were resulted from spontaneous in vivo reversion of the inherited mutation. Furthermore, the scanning electron microscopic studies indicated that WASP-positive cells from the patient restored the dense microvillus surface projections that were hardly observed in the WASP(dim) cells. This case might have significant implications regarding the prospects of the future gene therapy for WAS patients.

Adult↗
PubMed2001

NMDA-responsible, APV-insensitive receptor in cultured human astrocytes.

The present study was conducted to assess N-methyl-D-aspartate (NMDA)-responsible receptors in cultured human astrocytes by monitoring whole-cell membrane currents. NMDA generated currents, that are potentiated by glycine and blocked by Mg2+, with the current/voltage relation showing a reversal potential of +/- 0 mV. The currents were not inhibited by either the selective NMDA receptor antagonist, D-2-amino-5-phosphonovaleric acid (APV), or the non-selective ionotropic glutamate receptor antagonist, kynurenic acid. The currents were inhibited only by 19% in Ca2+-free extracellular solution. Furthermore, GDPbetaS, a broad G-protein inhibitor, inhibited NMDA-induced currents to 82% of original levels. The results of the present study thus suggest that an NMDA-responsible, APV-insensitive receptor with low Ca2+ permeability, distinct from the neuronal NMDA receptors, is expressed in human astrocytes and that the receptor is regulated in part by an unknown G-protein-linked receptor.

Astrocytes↗
PubMed2001

Severe cervical kyphosis in osteopathia striata with cranial sclerosis: case report.

We report a 29-month-old girl with osteopathia striata with cranial sclerosis (OS-CS), who showed several unusual manifestations, including short stature, muscular hypotonia, short lingual frenulum, an accessory ear and a granuloma at the oral edge. The most unusual distressing finding was severe cervical kyphosis with a dysplastic cervical spine. MRI revealed impingement of the upper spinal cord. To clarify the mode of inheritance of this disease in this patient, we performed a methylation-specific PCR analysis. It eventually showed random inactivation of X-chromosomes, suggesting an autosomal dominant trait.

Bone Diseases, Developmental↗
PubMed2001

Immunohistochemical localization of inducible nitric oxide synthase in synovial tissue of human temporomandibular joints with internal derangement.

The expression and distribution of inducible nitric oxide synthase (iNOS) was examined in 12 samples of human temporomandibular joint (TMJ) with internal derangement (ID) and four control specimens. In the diseased joints, strong or definite iNOS reactivity was expressed in synovial lining and endothelial cells; weaker activity was present in synovial fibroblasts. In contrast, although there was weak expression of iNOS in synovial fibroblasts and endothelial cells in the two control specimens, there was no iNOS staining in the synovial lining cell layers. This original report that iNOS is expressed in the synovial tissue of the temporomandibular joint indicates that nitric oxide is produced locally at least in the synovial lining in these joints when affected by internal derangement.

Adolescent↗
PubMed2001

Quantification of early blood-brain barrier disruption by in situ brain perfusion technique.

Osmotic disruption is currently being used to circumvent the blood-brain barrier (BBB) and enhance the delivery of therapeutic molecules in human brains. To date, however, the time course during the early phase of disruption has not been clarified. In order to demonstrate the rapid change in cerebrovascular permeability after BBB disruption in rats, we developed a method of in situ brain perfusion to demonstrate the earliest reversibility in cerebrovascular permeability. Osmotic BBB disruption was attained by intracarotid infusion of hypertonic mannitol. Perfusate containing [14C]-sucrose was infused at different time points following osmotic stress followed by measuring cerebrovascular permeability. The earliest BBB disruption was seen to occur 5 min after osmotic stress, after which the exact time course of cerebrovascular reversibility was studied. The protocol reported here, in contrast with those reported in previous studies, was shown to be qualitative, simple, and fast. In addition, the method can be applied to measure any low BBB permeability molecules. This protocol should be helpful for any research focused on enhancing drug delivery into the brain following osmotic BBB disruption.

Animals↗
PubMed2001

Generation of a constitutively active fragment of PKN in microglia/macrophages after middle cerebral artery occlusion in rats.

PKN is a fatty acid- and Rho-activated serine/threonine kinase, which has a catalytic domain highly homologous to that of protein kinase C (PKC). Recent studies have demonstrated that PKN is proteolytically cleaved after apoptotic stimulation and then a constitutively active 55-kDa fragment is generated. However, the role of the 55-kDa fragment are poorly understood. Adult Sprague-Dawley (SD) rats underwent middle cerebral artery occlusion (MCAO), and the temporal and spatial changes in the fragmentation of PKN and of PKC delta were examined by immunoblotting. No proteolytic fragment of PKC delta (about 40 kDa) was detected. The 55-kDa fragment of PKN appeared transiently from 3 days after MCAO at the ipsilateral normal cortex. At the boundary zone of infarction, the 55-kDa fragment was markedly induced from day 5 then peaked on day 21 and persisted until day 28. Analysis of anti-phosphoserine immunoprecipitates with an anti-PKN antibody revealed phosphorylation of the 55-kDa band. Double staining for PKN and Ox42 was used to examine the source of the 55-kDa fragment. PKN immunoreactivity was significantly increased in Ox42-positive cells (microglia/hematogenous macrophages). No DNA laddering and only a few terminal deoxynucleotidyl transferase-mediated dUTP nick end-labeling (TUNEL)-positive cells were observed on day 14 in despite of the high level appearance of the 55-kDa band. These results suggest that the constitutively active 55-kDa fragment of PKN does not contribute to apoptosis, but may contribute to a function of microglia/macrophages.

Animals↗
PubMed2001

Disc mobility and arthroscopic condition of the temporomandibular joint associated with long-term mandibular discontinuity.

PURPOSE: The present study aimed to explore disc mobility and arthroscopically diagnosed morphologic changes in the temporomandibular joint (TMJ) associated with long-term mandibular discontinuity. PATIENTS AND METHODS: Twelve patients (24 TMJs) who had undergone mandibulectomy including the unilateral condyle or segmental mandibulectomy without mandibular reconstruction, more than 8 months before this study were examined. The 24 TMJs were classified into 3 groups as follows: group 1, TMJs with a major mandibular fragment including the mandibular body (n = 11); group 2, TMJs with a mandibular ramus including the coronoid process (n = 5); and group 3, TMJs with only a condylar process, a mandibular ramus not including the coronoid process, or without a condyle (n = 8). Disc mobility was evaluated by magnetic resonance imaging, and arthroscopic observation of the superior joint compartment (SJC) was performed in all TMJs. The relationship between disc mobility and the arthroscopic findings was also studied. RESULTS: The frequency of immobile discs differed significantly among groups 1 (0%), 2 (40.0%), and 3 (100%). Arthroscopic findings were normal in all SJCs of group 1. Various types of fibrous adhesions were observed in 40.0% of group 2 and in 75.0% of group 3. The development of fibrous adhesions in the SJC was significantly related to the presence of an immobile disc. CONCLUSIONS: Long-term immobilization of the TMJ in a nonfunctional state seems to promote the development of fibrous adhesions in the SJC. Preservation of the mandibular fragment including the coronoid process, during mandibulectomy appears to contribute to postoperative TMJ mobility. To ensure recovery of a physiologic TMJ after mandibulectomy, it seems important to re-establish TMJ mobility by establishing mandibular continuity as soon as possible.

Humans↗
PubMed2001

Primary racemose hemangioma of the bronchial artery.

A 44-year-old woman was admitted to our hospital for repeated hemoptysis. Selective angiography of the bilateral bronchial arteries showed them to be enlarged and convoluted, and revealed shunts between the bronchial and pulmonary arteries. The patient was diagnosed as having racemose hemangioma of the bronchial artery. Although embolization of the bronchial artery with gelatin sponges was performed, hemoptysis emerged again about one year later. Finally, segmentectomy of the right lower lung was performed and primary racemose hemangioma of the bronchial artery was histopathologically confirmed.

Adult↗
PubMed2001

[Total removal of infected pacemaker lead under cardiopulmonary bypass in a case of endocarditis, bacteremia and lung abscess].

A 60-year-old man, who had undergone implantation of a transvenous pacemaker system on the left chest wall for sick sinus syndrome 19 years ago, was admitted because of endocarditis with septicemia and lung abscess 2 months after reimplantation of the generator. His blood culture revealed Staphylococcus aureus. Following debridement of the infected pacemaker pocket and antibiotics therapy, we tried to remove the pacemaker system under cardiopulmonary bypass 1 month after admission. In intraoperative inspection, the electrodes had become firmly encased with fibrous tissue within the tricuspid valve and the right ventricle. After the operation, antibiotic therapy was performed for 4 weeks. His postoperative course was uneventful. Patients with pacemaker infection should undergo aggressive total removal of the pacemaker system, particularly incase with endocarditis and bacteremia.

Bacteremia↗
PubMed2001

[Assessment of left internal thoracic artery graft by exercise Doppler echocardiography].

Doppler echocardiography has several advantages such as frequent use, noninvasive approach, and physiological evaluation. Supine bicycle exercise testing was conducted for 30 patients undergoing CABG with LITA to LAD. Doppler echocardiography studies were performed before and after exercise to observe the change. On the basis of the angiographic data, patients were divided into two groups: 27 patients with a patent LITA graft, 3 patients with mildly stenosed LITA graft. In the patients who had patent grafts, diastolic flow velocity were increased higher than systolic after exercise. In the stenotic group, the flow pattern was changed to further systolic one. Doppler echocardiography during exercise is thought to be a reliable method to assess the LITA flow.

Adult↗