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Biomedical subjects

T Kondoh

Publications and source records attributed to T Kondoh.

At least 109 records · Page 6Linked to original sources

A severe case of Moebius syndrome with calcification on the fourth ventricular floor.

We report the case of a Japanese girl with a severe type of Moebius syndrome. Her morphological features were a mask-like face, limitation of horizontal eye movements, severe bulbar palsy, multiple and bilateral arthrogryposis including the elbow, knee, and ankle joints, and clubfeet. After birth, her general condition became worse because of repeated apneic spells and aspiration pneumonias due to dysphagia. She finally required tracheotomy. Computed tomography (CT) of the brain revealed minute calcifications on the fourth ventricle floor; this may have been due to severe damage to the brain stem. It is most likely that the various manifestations in our patient were due to disturbance of the blood supply to arteries perfusing the brain stem and to some other arteries, at a critical stage of fetal development.

Abnormalities, Multiple↗

Large and invasive silent corticotroph-cell adenoma with elevated serum ACTH: a case report.

BACKGROUND: A silent corticotroph-cell adenoma (SCCA) is defined as evidence of immunoreactive ACTH in tumor cells of a pituitary adenoma that is not associated with symptoms of Cushing's disease. Most previous reports have discussed the etiology of this unique entity; however, the precise mechanisms of its "silence" are not clear yet. We discuss the pitfalls of endocrinological diagnosis and surgical management of SCCA. METHODS: A 63-year-old man, without the symptoms of Cushing's disease, demonstrated elevated serum levels of ACTH, but normal levels of cortisol. ACTH was measured by newly developed immunoradiometric assays (RMA), which are more specific for biologically active ACTH than conventional radioimmunoassay (RIA). The tumor was more than 3 cm in diameter and invaded both the cavernous sinus and the sphenoid sinus. RESULTS: Two different types of IRMAs revealed elevated serum ACTH levels. The tumor was removed successfully by staged operations. The high levels of serum ACTH were normalized after the second operation. CONCLUSION: The cause of the silence of SCCA is not clear yet. The elevated serum ACTH levels in the present case were not attributable to "Big ACTH." We suggest one possibility: the cause of the silence might be a minor variant of ACTH, detected by IRMA, leading to less biological activity of ACTH. This type of adenoma is invasive in nature and can potentially grow large because it is free of endocrinological symptoms. We emphasize the appropriate surgical strategy to normalize the serum levels of ACTH in case of SCCA.

Adenoma↗

Prevalence of morphological changes in the surfaces of the temporomandibular joint disc associated with internal derangement.

PURPOSE: The purpose of this study was to determine the prevalence of morphological changes in the superior and inferior surfaces in the temporomandibular joint (TMJ) disc and relate them to disc displacement. MATERIALS AND METHODS: Thirty TMJs obtained from fresh cadavers were studied. The TMJs were dissected, and the superior and inferior surfaces of the disc were inspected and classified as intact, irregular, or perforated. These findings were corrolated to the position of the disc. RESULTS: There was a greater prevalence of morphologic changes in the inferior (57%) than in the superior surface (17%) of the disc (P < .001). This was found for joints both with normal disc position and those with disc displacement. There was no relationship between surface irregularities of the inferior surface and the position of the disc. Perforation was seen more frequently in joints with disc displacement than in those with normal disc position. CONCLUSION: Morphologic changes in the inferior surface of the TMJ disc are more prevalent than those in the superior surface, but this is not related to disc position. However, this needs to be considered when doing arthroscopy of only the superior joint compartment.

Aged↗

Proinflammatory cytokines detectable in synovial fluids from patients with temporomandibular disorders.

OBJECTIVE: To measure the levels of the proinflammatory cytokines, interleukin (IL)-1 beta, IL-6, tumor necrosis factor- (TNF) alpha, IL-8, and interferon- (IFN) gamma in synovial fluid samples taken from patients with temporomandibular disorders (TMD). STUDY DESIGN: We studied 6 asymptomatic volunteers and 51 patients with TMD. The IL-1 beta, IL-6, TNF-alpha, IL-8, and IFN-gamma levels in temporomandibular joint synovial fluid were measured using enzyme-linked immunosorbent assay. RESULTS: Measurable level of at least one cytokine in the synovial fluid was found in 40 (64.5%) of 62 joints in the patients: IL-1 beta and IFN-gamma were each detected in 18 (29.0%) of 62 joints; IL-6 in 13 (21.0%) of 62 joints; IL-8 in 11 (19.3%) of 57 joints; and TNF-alpha in only 5 (8.1%) of 62 joints. None of these cytokines was detectable in the synovial fluid in the control group. Furthermore, there was a strong correlation between the detection of IL-1 beta and pain in the joint area. CONCLUSIONS: These data clearly demonstrate increased levels of several proinflammatory cytokines in certain patients with TMD and suggest that these cytokines may play a role in the pathogenesis of synovitis and degenerative changes of the cartilaginous tissue and bone of the temporomandibular joint.

Adolescent↗

Complete-type DiGeorge syndrome treated by bone marrow transplantation.

BMT was carried out on a patient with DiGeorge syndrome who suffered recurrent infections after birth. At 13 months of age, 8.0 x 10(8)/kg of bone marrow nuclear cells were infused from an HLA-identical sibling using only anti-thymocyte globulin to prevent rejection. Donor DNA was not detected on microsatellite polymorphism by PCR. At 19 months of age, a second BMT from the same donor was carried out using busulfan and cyclophosphamide as conditioning. DNA examination of bone marrow showed chimerism at day 18 and complete donor origin at day 28. Seven months post-BMT, the numbers of CD3-, CD4- and CD8-positive cells were in the normal range. BMT is thus an effective therapy for DiGeorge syndrome.

Antigens, CD↗

Production and characterization of a monoclonal antibody specific for ubiquitin-like polypeptide responsible for nonspecific immune suppression.

Monoclonal nonspecific suppressor factor (MNSF) is a lymphokine product of a murine T cell hybridoma that inhibits the immune response in an antigen nonspecific manner. Recently, we found that a novel ubiquitin-like protein (Ubi-L), a subunit of MNSF, is responsible for its biological activity. We developed a monoclonal antibody with specific activity against Ubi-L. Inhibition experiments showed that this mAb, termed NA4, preferentially recognizes Ubi-L but not irrelevant proteins such as ubiquitin. With the use of NA4, we established an ELISA method for the quantitation of Ubi-L. By this ELISA system, approximately 40 ng/ml of MNSF was detected in the culture supernatants of concanavalin A (Con A)- or interferon gamma (IFN gamma)-activated splenocytes, whereas MNSF in the supernatant of IFN alpha- and IFN beta-stimulated splenocytes was nil. In addition, NA4 could abrogate the action of Ubi-L. Thus NA4 was confirmed to be a pertinent tool for elucidation of the underlying mechanism of action of MNSF.

Animals↗

Relationship between left ventricular hypertrophy and renal and retinal damage in untreated patients with essential hypertension.

We examined the relationship between left ventricular hypertrophy (LVH) and renal and retinal damage in 174 untreated patients with essential hypertension. As an index of renal and retinal damage, we examined proteinuria and retinal vascular change. LVH was diagnosed according to left ventricular mass obtained from echocardiography. Of the hypertensive patients, 111 patients (64%) had LVH. The incidences of proteinuria and advanced retinal vascular change were higher in patients with LVH than in those without LVH. In a multiple regression model, there was a significant positive correlation between left ventricular mass and proteinuria, as well as diastolic blood pressure, sex, age and body mass index. In conclusion, proteinuria is related to elevated left ventricular mass in patients with essential hypertension.

Blood Pressure↗

[An emergent surgical treatment of recurrent Stanford type A thrombosed aortic dissection].

A 71-year-old male was admitted to our hospital with a chief complaint of back pain. Chest CT scan showed so-called Stanford type A thrombosed aortic dissection. Aortography revealed no ulcer like projection. At first, conservative treatment was carried out. But about two hours later, he had another back pain. Repeat chest CT scan demonstrated an enlarged dissecting lumen, necessitating emergent graft replacement. Postoperative course was uneventful and he was discharged on the 33rd postoperative days.

Aged↗

[Effective use of a laboratory database: quality assurance and laboratory workflow applications].

Recent laboratory information systems have usually adopted a client server system. Computing tools which can provide easy access to a database using simple language are now strongly needed. These functions are provided in an End User Computing (EUC) system. An EUC is defined as follows: 1) General end users can easily access the database of the laboraotry system and extract objective data stored in database. 2) The extracted data will be easily converted to files that can be processed by commercially available software. In this paper, we demonstrate the examples how to use the EUC for a quality assurance system and analyses of laboratory workflow. In the case of quality assurance, we demonstrate the setting of reference intervals from stored laboratory data concerning health care examination programs at our university. Secondly, we developed a system of monitoring quality control data, and set parameters for delta checking and actual zone QC method. We can estimate and design an outline of laboratory workflow from extraction of the time currently required for each task. We can measure the turn-around time for laboratory testing and rate of requests for laboratory tests received from physicians via order entry system. Moreover, we can estimate and simulate the waiting time and time required for analyses by outpatient clinics. These time monitoring systems reflect the design of laboratory workflow such as the labor and equipment time required in laboratory work. These uses of laboratory data are currently expanding further and further in the fields of education and laboratory research. We believe that information technology will facilitate future advances of laboratory medicine.

Clinical Laboratory Information Systems↗

The GABAA receptor is expressed in human neurons derived from a teratocarcinoma cell line.

NT2 cells, a human teratocarcinoma cell line, are shown to be differentiated in neuron-like cells (NT2-N cells) by treatment with retinoic acid. The present study identified the neurotransmitter receptors expressed in NT2-N cells using patch-clamp recording. Voltage-sensitive Na+ currents, which are specific for neurons, were observed in NT2-N cells but not in NT2 cells, suggesting that NT2-N cells actually function as neurons. Glutamate receptor agonists, N-methyl-D-aspartate (NMDA) and kainate, evoked whole-cell currents. In addition, gamma-aminobutyric acid (GABA) evoked currents and the currents were inhibited by the selective GABAA receptor antagonist, bicuculline. In outside-out patches, GABA elicited single channel currents with two classes of the slope conductance (26 and 50 pS). No current, however, was induced by ACh, serotonin, or dopamine NT2-N cells, thus, express at least two types of the major excitatory and inhibitory neurotransmitter receptor in the central nervous system, the glutamate and GAGAA receptors, suggesting that these receptors have a crucial role in neurotransmission from the earlier stage of the brain development.

Bicuculline↗

The mechanism of free radical generation in brain capillary endothelial cells after anoxia and reoxygenation.

We studied the mechanism of reoxygenation injury of cerebral microvessels in cultured rat brain capillary endothelial cells (BCECs). BCECs were isolated from rat cerebral cortices by a two step enzymatic treatment. The monolayers of BCECs were subjected to anoxia for 20 minutes followed by reoxygenation for 3 hours. Cell damage was assessed by measuring the leakage of intracellular lactic dehydrogenase (LDH). The control group was anoxia/reoxygenated BCECs without any protective reagents. To study the protective effect of free radical scavengers and antioxidants, superoxide dismutase, catalase, deferoxamine, oxypurinol, indomethacin, or NG-nitro-L-arginine methyl ester (L-NAME) was applied during anoxia/reoxygenation. Thus 7 experimental conditions were established. Lactic dehydrogenase (LDH) leaked from reoxygenated BCECs due to cell membrane damage. This leakage was almost totally suppressed by superoxide dismutase, indicating that reoxygenation injury of BCECs is mediated by superoxide generation. The other scavengers and antioxidants partially suppressed LDH leakage. Reduction of Ca2+ in the culture medium from 1.6 mM to 0.016 mM also suppressed LDH leakage. These results indicate that BCECs subjected to anoxia/reoxygenation become potent generators of superoxide anion, which is thought to be responsible for reoxygenation injury. The superoxide generation partially depends on the xanthine oxidase and cyclooxygenase pathways. As L-NAME partially suppressed LDH leakage peroxynitrite may contribute to reoxygenation injury of BCECs. The extracellular Ca2+ concentration also plays a critical role in the reoxygenation injury of BCECs.

Animals↗

Localization of dopamine receptors and associated mRNA in transplants of human fetal striatal tissue in rodents with experimental Huntington's disease.

Huntington's Disease (HD) is characterized by deficits in motor and cognitive functions. This neurodegenerative disease shows an extensive loss of medium-sized spiny projection neurons (GABAergic) within the neostriatum. With the loss of these neurons, there is a concomitant loss of associated receptors, such as those for GABA, glutamate, and dopamine. In the present study, we have addressed the question of whether dopamine receptors are re-established in the lesioned rodent striatum following the transplantation of human striatal cells. Human striatal cell suspension or saline (transplant controls) was injected into the striatum of rats previously lesioned with quinolinic acid (QA). Three nine months following transplantation, the animals were sacrificed and the brains were processed for receptor autoradiography and in situ hybridization of dopamine D1 and D2 receptor subtypes. Our results demonstrate that animals transplanted with human striatal cells show a significant increase in D1 receptors following transplantation when compared to the lesion area in control animals, while D1 receptor mRNA remains unchanged. In contrast to D1 receptor binding, D2 receptor levels are not increased in the lesioned and transplanted area of the striatum when compared to controls; however, D2 receptor mRNA levels are significantly increased. These results demonstrate that at the times the animals were examined, D1 and D2 receptors were differentially regulated. Our results further indicate that human striatal primordium will survive following transplantation and will express D1 receptors and D2 receptor mRNA that are depleted in the QA lesioned rodent striatum. This study compliments and extends previous findings on human striatal cell transplantation in rodent models of HD.

Animals↗

A functioning black adenoma of the adrenal gland.

A 53-year-old female had clinical and laboratory findings suggestive of Cushing's syndrome. In contrast to the Cushing's syndrome caused by cortical adenoma, a high level of urinary 17-ketosteroids (17-KS) was also noted. Imaging studies revealed a right adrenal tumor. Right adrenectomy was performed; the surgical specimen revealed a black adenoma consisting of compact cells with numerous pigments which seemed to be lipofuscin in nature. The present case indicates that black adenoma as well as adrenocortical carcinoma should be suspected, when patients with Cushing's syndrome show an increased level of urinary 17-KS excretion.

17-Ketosteroids↗

Fatality due to severe Salmonella enteritis associated with acute renal failure and septicemia.

A 66-year-old man was hospitalized for abdominal pain and diarrhea of more than 10 times a day. He had been under regular medication with prednisolone for rheumatoid arthritis. On admission, laboratory data and clinical examination indicated inflammation, dehydration, acute renal failure with a high level of serum musculogenic enzyme (creatine kinase), and ileus. Salmonella enteritidis was isolated from his fecal and blood samples. The patient died within 24 hours after admission, and autopsy showed hemorrhagic necrotic enteritis localized to the ileum. Enterocolitis due to Salmonella enteritidis, which is usually an acute self-limited gastrointestinal illness, may occasionally be a serious and lethal disease.

Acute Kidney Injury↗

Cerebellopontine angle ependymoma with internal auditory canal enlargement and pineal extension--case report.

A 38-year-old male presented with a posterior fossa ependymoma with unusual extension from the cerebellopontine angle to the pineal region. Magnetic resonance imaging clearly demonstrated that these two components were continuous through the right ambient cistern. Computed tomography using a bone algorithm revealed enlargement of the right internal auditory canal. This case suggests that ependymoma can extend anywhere within the subarachnoid space along the path of least resistance.

Adult↗