Search PubMed⌕ Search

Biomedical subjects

M Nitta

Publications and source records attributed to M Nitta.

At least 91 records · Page 5Linked to original sources

Williams syndrome. A middle-aged case of markedly delayed diagnosis.

A rare case of Williams syndrome diagnosed at the age of 41 is documented. The first subjective symptom was chest pain and the patient displayed many other features in addition to severe supravalvular aortic stenosis with a systolic gradient of 60 mmHg. The stenotic lesion had an area of 0.5 cm2, and was associated with dilated and tortuous coronary arteries. Extended aortoplasty was successfully performed and the postoperative course has been excellent without any cardiac symptoms. In spite of the severe cardiac lesions, this case had been largely asymptomatic and presented unusual features related to the diagnosis and management of this syndrome in an adult. The pattern of abnormalities found in this case suggested problems in relation to the calcitonin/calcitonin gene related peptide (CGRP) and the elastin gene occurring in embryonic organogenesis.

Abnormalities, Multiple↗

[Non-Hodgkin's lymphoma with pulmonary involvement and various immunological abnormalities in an elderly patient].

A 85-year-old female was admitted with general fatigue and chest discomfort in July 1991. Her chest X-ray film showed several pulmonary nodules in the bilateral lung fields without hilar lymphadenopathy. Metastatic lung cancer was suspected, but primary cancer was not detected in spite of cancer screening. Hemoglobin was 8.7 g/dl. The patient also had various immunological abnormalities including increased serum cold agglutinin titer, decreased serum complement, positive anti-nuclear antigen and IgM-kappa monoclonal gammopathy detected by immunoelectrophoresis. She was diagnosed as having autoimmune hemolytic anemia with cold agglutinin disease and M-protein. With the administration of corticosteroids, hemolytic anemia improved temporarily, but the patient died of hemorrhagic gastritis. At autopsy, a lung tumor was detected in the left upper lobe, mainly without hilar lymphadenopathy. The autopsy specimens showed Non-Hodgkin's lymphoma (diffuse medium-sized cell type), differentiated from macroglobulinemia by immunohistochemical studies. In elderly patients, with various immunological abnormalities, B-cell lymphoproliferative disorders such as malignant lymphoma should be suspected.

Aged↗

[Chronic myelogenous leukemia complicated with sarcoidosis].

A 79-year-old man who had been diagnosed as having sarcoidosis when he was 63 year old, was admitted to our hospital because of marked thrombocytosis and leukocytosis in July 1991. The low neutrophil alkaline phosphatase (NAP) score, presence of Philadelphia (Ph1) chromosome in the bone marrow cells, and M-BCR rearrangement by Southern blot hybridization were observed. He was diagnosed as having chronic myelogenous leukemia complicated with sarcoidosis. The coexistence of sarcoidosis and leukemia has rarely been reported. It is difficult to discuss that there is not causal association between of them.

Aged↗

[Non-Hodgkin's lymphoma in a patient with sarcoidosis (the sarcoidosis-lymphoma syndrome)].

A-31-year-old man with right cervical and supraclavicular lymphadenopathy was admitted in March, 1991. He was diagnosed as having muscular sarcoidosis at the age 8 year, and was treated with corticosteroids. Since age 18, his skin was erythematous and ulcerous, and later his skin became gradually atrophic. Lymph node biopsy revealed diffused large cell non-Hodgkin's lymphoma. Lymphoma cells showed TCR-beta gene rearrangement by Southern blot hybridization. His lymphoma was refractory to CHOP and CHOP-Bleo regimens. Complete remission was achieved with cisplatin and etoposide. However, early relapse occurred, and he died of pulmonary hemorrhage 4 months after the diagnosis of non-Hodgkin's T-cell lymphoma. The so called "sarcoidosis-lymphoma syndrome" is uncommon in Japan. In 9 of 10 cases previously reported, malignant lymphoma occurred during the course of sarcoidosis. Most of the sarcoidosis cases were chronic active type, and required systemic administration of corticosteroids. Hodgkin's disease coexistent with sarcoidosis as reported in other countries, was not found in Japan. These findings suggest that the low incidence of sarcoidosis-lymphoma syndrome in our country is due to the relative rareness of Hodgkin's disease. The sarcoidosis-lymphoma syndrome possibly appears as a consequence of immunological abnormalities observed in sarcoidosis.

Adult↗

[Chimerism monitoring by VNTRs polymorphism analysis in a patient who received allogenic bone marrow transplantation].

A 39-year-old female diagnosed as acute myelogenous leukemia received allogenic bone marrow transplantation (BMT) pre-conditioned with busulfan and cyclophosphamide regimen from her HLA identical sibling. To distinguish donor and recipient cells, we analyzed variable numbers of tandem repeats (VNTRs) polymorphisms using a YNH-24 probe by Southern blot hybridization. VNTRs polymorphism analysis documented the engraftment of donor cells, relapse of recipient cells, and mixed hematopoietic chimerism. Assessment of the chimerism state is important for determining the prognosis of patients undergoing BMT, and VNTRs polymorphisms analysis is very useful for identifying the chimerism state.

Adult↗

Reliability of Doppler echocardiographic stroke volume measurement.

To evaluate the accuracy and utility of the echocardiographic Doppler method of determining the stroke volume, the stroke volume was measured in 64 instances in 15 patients and compared to the simultaneous thermodilution stroke volume determinations. Stroke volume, varied by atrial pacing at various heart rates, was calculated noninvasively by the product of the cross-sectional area of the aortic orifice determined by echocardiography and the flow velocity integral measured by continuous wave Doppler. While the cardiac output was increased moderately by atrial pacing, the stroke volume was decreased in relation to the increase in the heart rate. The stroke volume calculated by the echo-Doppler method exhibited a significant linear relationship to the stroke volume determined by thermodilution (r = 0.84) and to the cardiac output (r = 0.75, p less than 0.01). Inter-observer and intra-observer variability was 7 +/- 4% and 5 +/- 4%, respectively. The noninvasive echo-Doppler method of estimating the stroke volume is concluded to be accurate, specifically, to be comparable to the values obtained by thermodilution and with a low inter- and intra-observer variability.

Adult↗

[Cricoarytenoid joint involvement in rheumatoid arthritis].

The cricoarytenoid (CA) joint, which controls the vocal cords, is diarthrodial; it is composed of two cartilages with a ligamentous capsule lined by synovial membrane. It has been known that inflammation of CA joint occurs sometimes in patients with rheumatoid arthritis (RA). Yet, so far, no detailed full-report on this subject has ever been made in this country. Here we report four RA cases with CA joint ankylosis or abduction disturbance due to progression of CA arthritis. Episodes of inspiratory dyspnea were observed in all cases and tracheostomy was performed in case 1 and case 2. Further surgical intervention i.e., tracheoplasty was necessary in case 2. Case 3 and case 4 have been followed conservatively thus far. Clinical surgical interventions were necessitated mainly with joint replacement, 3) there exists severe destruction of cervical spines as well as temporomandibular joints. Rheumatologist must be fully aware of how urgent this clinical entity is and not miss-diagnose+ these patients.

Arthritis, Rheumatoid↗

[Neutrophilic dermatosis in a patient with refractory anemia].

A 46-year-old man diagnosed as refractory anemia was hospitalized because of high fever and extensive erythema with ulceration in the femoral region. His peripheral blood examination showed marked leukocytosis (WBC 31,500/microliter:neutrophilic 90%) and anemia (Hb 8.6 g/dl. In spite of administration of antibiotics, the cutaneous ulcer rapidly extended to the right thigh and became necrotic. The bacterial culture of the cutaneous lesion showed no growth and a skin biopsy showed infiltration of neutrophils in the dermis. He became afebrile and his cutaneous lesion improved after administration of corticosteroid. When the dose of corticosteroid was decreased, cutaneous erythema and nodules appeared at other sites repeatedly, and disappeared after the dose of corticosteroid was increased. The cutaneous lesions had characteristics of both Sweet's syndrome and pyoderma gangrenosum. Moreover, the patient had immunological abnormalities and decreased neutrophilic functions (chemotaxis and O2- generation). Thus, it was suggested that the cutaneous lesions of this patient could be diagnosed as "neutrophilic dermatosis of MDS", and corticosteroid was recognized to be very effective in treating these skin lesions.

Anemia, Refractory↗

Endothelin-1 induces hypertrophy with enhanced expression of muscle-specific genes in cultured neonatal rat cardiomyocytes.

To determine whether endothelin-1 (ET-1) induces hypertrophy of cardiomyocytes, the effects of ET-1 on the expression of muscle-specific genes and a proto-oncogene, c-fos, in cultured neonatal rat cardiomyocytes were examined by Northern blot analysis. ET-1 (10(-7) M) induced about twofold to fourfold increases in the gene expression of myosin light chain 2, alpha-actin, and troponin I after 6 hours, which continued up to 24 hours. The ET-1-induced increases in mRNA levels for these muscle-specific genes were dose dependent (10(-9) to 10(-7) M). Run-on transcriptional assay showed that the changes in mRNA level for three muscle-specific genes were regulated, at least in part, at the transcriptional level. 12-O-Tetradecanoylphorbol 13-acetate (TPA), a potent protein kinase C activator, and the Ca2+ ionophore ionomycin also increased mRNA levels of three muscle-specific genes. ET-1, TPA, and ionomycin similarly induced the expression of c-fos after 30 minutes, which returned to an undetectable level after 6 hours. ET-1 remarkably and dose-dependently stimulated accumulation of total inositol phosphates in cardiomyocytes. Morphometrical evaluation showed that ET-1 significantly increased surface area of cardiomyocytes without cell proliferation. ET-1 also dose-dependently stimulated the synthesis of protein and DNA, which was unaffected by the L-type calcium channel blocker nicardipine. These data suggest that ET-1 induces hypertrophy of cardiomyocytes associated with the induction of muscle-specific gene transcripts through the possible involvement of protein kinase C activation or intracellular Ca2+ mobilization.

Animals↗

[Application of Y-chromosome specific DNA analysis for detection of mixed hematopoietic chimerism after allogeneic bone marrow transplantation].

We report a case involving mixed hematopoietic chimerism after an allogeneic bone marrow transplantation (BMT) from a sex mismatched donor. A 31 year-old-man who was diagnosed as having chronic myelogenous leukemia in the accelerated phase received an allogenic BMT from his HLA-identical sister in March, 1989. To determine the mixed chimerism we used the Y-chromosome specific repeated sequence of DNA using a specific probe (PHY 10). The donor's DNA 3.5 kb band appeared in 1-10% of male DNA by Southern blot hybridization in the peripheral blood 21 days after BMT. The Y-chromosome DNA band decreased day by day, and disappeared 110 days after BMT. The Y-chromosome DNA band could be detected, even though few metaphases were obtained immediately after BMT. Thus this method is very sensitive for determining which cells contain the Y-chromosome, and is therefore useful for detecting mixed chimerism after sex-mismatched BMT. Using this method the clinical significance of mixed chimerism can be assessed.

Adult↗

[Blastic form of acute erythremia: report of an autopsy case].

We report an autopsy case of acute erythremia which terminated in generalized infiltration of immature blasts similar to proerythroblasts. A 61-year-old man was admitted because of general fatigue and fever in June, 1990. Mild anemia and severe thrombocytopenia were noted. The bone marrow was hypocellular with 25.5% blasts similar to proerythroblasts and 36.5% erythroblasts, many of which were polynuclear and megaloblastoid. The blasts were cytochemically negative for POX, but positive for PAS staining. Therefore he was diagnosed as having acute erythremia. Partial remission was achieved by BHAC-EV therapy. But three months later, his marrow was replaced by 52.7% blasts as seen in admission. Those blasts were negative for lymphoid, myelocytic, megakaryocytic markers and antiglycophorin A, but positive for OKT 9. Electron microscopy revealed that some of blasts had characteristics of immature erythroblasts. In spite of low dose Ara-C therapy, he died of sudden gastrointestinal bleeding in December, 1990. The autopsy disclosed widespread infiltration of blasts, involving liver, spleen, lung, kidney and stomach. It was interesting that dysplasia had been confined to erythroid lineage throughout his clinical course. He seemed to be a rare case of blastic form of acute erythremia which should be distinguished from erythroleukemia.

Acute Disease↗

[A case of rheumatoid arthritis complicated with auranofin-induced acute interstitial pneumonitis].

A case of auranofin(AF)-induced acute interstitial pneumonitis in a patient with rheumatoid arthritis (RA) is reported and possible mechanism of the incidence is discussed. A 57 year-old woman with a history of RA starting in early April 1989, had been prescribed AF for seven days prior to her referral to this hospital. Because of the severity of her polyarthritis, AF was discontinued; therapy with gold sodium thiomalate (GST) instead was started on 23 June 1989. The patient responded quite favorably. At a total cumulative dose of 160mg of GST, however, she developed generalized exfoliative dermatitis and the drug was discontinued. On 29 Sept. 1989 when her skin lesions cleared up completely, she was put on AF again. Six days after resumption of AF therapy, she developed fever. Nonproductive cough and dyspnea on exertion was observed within the following three days. The chest X-ray film revealed diffuse reticular-linear infiltrates more prominently in both upper lobes. Arterial blood gas studies showed hypoxemia and a restrictive ventilatory defect was demonstrated by pulmonary function studies. AF was stopped. High dose pulse iv corticosteroid(CS) therapy combined with oral CS was instituted. Immediate improvement of both subjective and objective findings was observed. By using the patient's peripheral blood lymphocytes, delayed lymphocyte stimulation test(DLST) against the probable causative agents was performed before the bolus CS therapy. Stimulation Index(shown in percentage) was markedly elevated in AF(724%) as compared with GST(163%). The fiberoptic bronchoscopy with bronchoalveolar lavage(BAL) was performed. Analysis of BAL fluid revealed increased cellularity (9 x 10(5)/ml) with marked lymphocytosis of 79%.(ABSTRACT TRUNCATED AT 250 WORDS)

Arthritis, Rheumatoid↗

[Doppler hemodynamic evaluation of bioprosthetic valve failure in the mitral position].

M-mode and two-dimensional echocardiography (2DE) allows the accurate assessment of primary tissue degeneration of bioprosthetic valves. The Doppler method permits quantitative evaluation of the pressure gradient across the prosthetic valve or detection of regurgitant flow. The present study summarized our clinical experiences of serial cases of mitral valve replacement (MVR) with bioprostheses at the mitral position, and clarified the clinical usefulness and limitations of Doppler and 2DE examinations for the early detection of primary valve dysfunction. Consecutive 65 patients undergoing single mitral valve replacement from April, 1977 to November, 1979 were listed for the study. A survey of the present clinical status was carried out from July, 1988 to July, 1990 (a follow-up period ranged from 84 to 127 months) for all patients, and the information was available from 53 patients (47 adults and six infants). Twenty-four survived patients without re-MVR were examined by Doppler and 2DE. Among the 53 patients, 34 were alive and 19 dead, and the total survival rate was 64.2%. The reasons for death in 19 patients were perioperative death in seven (including four infants with severe calcification of bioprostheses), chronic heart failure in three, cerebral infarction in two, post blood transfusion hepatitis in two, endocarditis in one, and non-cardiac death such as cancers in four. During the long-term observation of 47 adult patients, 14 cases (30%) had re-MVR (one for a stenotic lesion with massive calcification, and 13 for torn leaflets). Thickening and/or torn leaflets were noted in 13 (54%) of the 24 survived patients without re-MVR.2+ suggests that bioprosthetic valve replacement at the mitral position may not be recommended.

Adult↗

[The prevalence and clinical features of pathologically abnormal mitral valve leaflets (myxomatous mitral valve) in the mitral valve prolapse syndrome: an echocardiographic and pathological comparative study].

We studied the prevalence and clinical features of pathologically abnormal mitral valve leaflets (myxomatous mitral valve: MMV) in consecutive 142 patients with the mitral valve prolapse syndrome (MVP). Our echocardiographic criteria for MMV were 1) thick leaflets 3 mm or greater, 2) redundant leaflet-motion, and 3) echo-density lower than that of the aortic walls. The echocardiographic measurements of left ventricular diastolic dimensions (LVDd), percent fractional shortening (%FS), mitral annular diameter (MAD), and LV mass were compared between MMV and non-MMV groups. Twelve patients (8%) were referred for surgery because of congestive heart failure, and two patients died during the observation periods. Gross morphology of the MMV was characterized by increased surface area, dome formation of the leaflet-body, and non-uniform leaflets in thickness, and histologic findings of the MMV were the infiltration of spongiosa layer into the fibrosa layer. The diagnostic accuracy of echocardiography for the MMV was examined in 14 patients underwent either surgery or autopsy, and it was high (78% in sensitivity and 80% in specificity). The progression of mitral regurgitation (MR) from mild to moderate grade or mild to severe grade was found in five of 26 patients during follow-up studies over 12 months (mean = 36 months). All of the five patients were aged 50 years and older. While, MR completely disappeared in a 17-year-old boy with marked physical development within three years of the observation period. Mitral annular diameter significantly increased in MMV with MR when compared to non-MMV with MR (4.1 +/- 0.7 vs 3.5 +/- 0.4). But no significant changes were noted in LVDd and LV mass between non-MMV with MR and MMV with MR. Of the 142 patients with MVP, 96 patients were non-MMV and 46 patients were MMV. Ruptured chordae tendineae were associated in 5/96 patients (5%) with non-MMV and 22/46 patients (48%) with MMV. Intracardiac vegetations were seen in four of the 96 patients (4%) with non-MMV. The prevalence of MMV in MVP was greater in older patients, and it reached nearly as high as 50% of MVP patients aged 60 years and older. In conclusion, the echocardiographic diagnostic criteria for MMV are reliable with high sensitivity and specificity, and are useful to predict the high risk patients in the MVP syndrome. MMV may be a potential etiology causing aggravation of mitral regurgitation and/or ruptured chordae tendineae.

Adolescent↗

[A case showing the infraoptic course of the anterior cerebral artery associated with anterior cerebral artery aneurysm].

A ruptured anterior cerebral artery aneurysm with anomalous right infraoptic course of the anterior cerebral artery (ACA) was reported. A 40-year old male was admitted because of SAH episode, and computerized tomography (CT) on the day of admission revealed hemorrhage in both Sylvian and interhemispheric fissures. Four-vessel angiography was performed on the same day. A saccular aneurysm was demonstrated in the region of the anterior communicating artery (ACoA), and anomalous artery, which originated from the intradural right carotid artery at the level of the right ophthalmic artery was found to be running medially and superiorly. It supplied the bilateral callosomarginal arteries and the unpaired pericallosal artery. The fenestration of the vertebral artery, duplication of bilateral superior cerebellar arteries, and an occipital artery arising from the right internal carotid artery at the C3 level were found. Surgery was performed on the first day of admission, and the anterior interhemispheric approach was selected. The anomalous branch of the right ICA ran beneath the right optic nerve, ascended between the optic nerves, and supplied the ACoA system. The left hypoplastic A1 segment entered the ACoA. However, the right A1 segment could not be found. The aneurysmal neck, situated between the bilateral callosomarginal arteries and the unpaired pericallosal artery, was clipped with Sugitas curved clip. The postoperative course was uneventful and a follow-up angiograph revealed successful obliteration of the aneurysm.

Adult↗

The electrocardiogram at rest and exercise during a simulated ascent of Mt. Everest (Operation Everest II).

To evaluate the effect of extreme altitude on cardiac function in normal young men, electrocardiograms were recorded at rest and during maximal exercise at several simulated altitudes up to the equivalent of the summit of Mt. Everest (240 torr or 8,848 m). The subjects spent 40 days in a hypobaric chamber as the pressure was gradually reduced to simulate an ascent. Changes in the resting electrocardiogram were evident at 483 torr (3,660 m) and were more marked at 282 torr (7,620 m) and 240 torr (8,848 m). They consisted of an increase in resting heart rate from 63 +/- 5 to a maximum of 89 +/- 8 beats/min; increase in P-wave amplitude in inferior leads; right-axis shift in the frontal plane; increased S/R ratio in the left precordial leads; and increased T negativity in V1 and V2. No significant arrhythmias or conduction defects were observed. Most changes reverted to normal within 12 hours of return to sea level, with the exception of the frontal-plane axis and T-wave alterations. Maximal cycle ergometer exercise at 282 torr (7,620 m) and 240 torr (8,848 m) resulted in a heart rate of 138 +/- 7 and 119 +/- 6 beats/min at the 2 altitudes, respectively. No ST depression or T-wave changes suggestive of ischemia occurred despite a mean arterial oxygen saturation of 49% and a mean pH of 8 during peak exercise. Occasional ventricular premature beats were observed during exercise in 2 subjects.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗