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Biomedical subjects

H Sugawara

Publications and source records attributed to H Sugawara.

At least 37 records · Page 2Linked to original sources

Condition of microcephaly, growth retardation, joint contractures, atopic dermatitis, and mental retardation in two Japanese sisters: a new autosomal recessive MCA/MR syndrome?

We report on two sisters in a family with a hitherto undescribed MCA/MR condition characterized by growth retardation, severe microcephaly, a peculiar facies, congenital contractures of the interphalangeal and patellar joints, atopic dermatitis, and growth and developmental delay. The disorder in the family we describe is similar to but clearly distinguished from tricho-rhino-phalangeal syndromes or Bavinck syndrome. We propose that the condition in the sisters represents a new autosomal recessive MCA/MR syndrome.

Abnormalities, Multiple↗

Lymphoma-type adult T-cell leukaemia-lymphoma with a bulky cutaneous tumour showing multiple human T-lymphotropic virus-1 DNA integration.

Human T-lymphotropic virus-1 (HTLV-1) is considered to be the cause of adult T-cell leukaemia-lymphoma (ATL). Monoclonal integration of HTLV-1 proviral DNA, as is analysed by Southern blotting, has been demonstrated in ATL patients. Unusual integration patterns of HTLV-1 proviral DNA have occasionally been described, and it is suggested that the patterns have clinical implications for ATL pathophysiology. Multiple, complete and defective types of integration patterns, in that order, are apparently associated with prognoses from good to poor. We report a 73-year-old Japanese woman with lymphoma-type ATL and a bulky cutaneous tumour on the left thigh. Four bands of slightly differing intensity were seen after EcoRI digestion of skin and lymph node samples on Southern blot analysis of HTLV-1 proviral DNA. Analysis for T-cell receptor-beta gene revealed five novel bands after restriction enzyme digestion with HindIII, indicating that the patient has four separate tumour cell clones, each of which carries one copy of the provirus. She was treated with chemotherapy and radiation and remains under reasonable control despite some relapsing cutaneous nodules. The indolent course in this present case could be related to the multiple integration pattern of HTLV-1 proviral DNA detected.

Aged↗

Inactivation of parvovirus B19 in coagulation factor concentrates by UVC radiation: assessment by an in vitro infectivity assay using CFU-E derived from peripheral blood CD34+ cells.

BACKGROUND: Nonenveloped and thermostable viruses such as parvovirus B19 (B19) can be transmitted to patients who are receiving plasma-derived coagulation factor concentrates treated by the S/D method for inactivating enveloped viruses. Therefore, it is important to develop and validate new methods for the inactivation of nonenveloped viruses. STUDY DESIGN AND METHODS: Suspensions of B19 in coagulation factor concentrates (FVIII) were irradiated with UVC light. B19 infectivity was determined by an indirect immunofluorescence assay using CFU-E, as a host cell, derived from peripheral blood CD34+ cells. The effects of catechins on B19 infectivity and on FVIII activity after UVC illumination were also examined. RESULTS: The indirect immunofluorescence assay estimated the B19 infectivity of samples containing virus copies of 10(5) to 10(11) per 10 microL to be a median tissue culture-infectious dose of 10(0.3) to 10(5.4) per 10 microL. B19 was inactivated by 3 log at 750 J per m(2) of UVC radiation and was undetectable after 1000 or 2000 J per m(2) of irradiation. However, FVIII activity decreased to 55 to 60 percent of pretreatment activity after 2000 J per m(2) of UVC radiation. This was inhibited in the presence of rutin or catechins. Epigallocatechin gallate could maintain FVIII activity at almost 100 percent of pretreatment activity after 2000 J per m(2) of UVC radiation, while B19 infectivity was decreased to undetectable levels, which resulted in >3.9 log inactivation. CONCLUSION: UVC radiation in the presence of catechins, especially epigallocatechin gallate, appears to be an effective method of increasing the viral safety of FVIII concentrates without the loss of coagulation activity.

Antigens, CD34↗

Three-year follow-up study of allergy in workers in a mushroom factory.

Exposure to mushroom spores may cause many respiratory allergic diseases, however, there has been no serial study in a mushroom factory to address this problem. The aim of this study was to investigate the serial changes in respiratory allergy and the incidence of hypersensitivity pneumonitis (HP) in mushroom workers. A 3-year follow-up study, beginning in June 1996, was conducted in a newly operating mushroom factory in which one kind of mushroom is produced: Hypsizigus marmoreus (Bunashimeji). Allergic symptoms, chest roentgenogram, serum precipitins to the spores and soluble adhesion molecules in sera were evaluated once a year in 60 workers and 20 controls. Three out of the 60 subjects were diagnosed as having HP caused by inhalation ofthe mushroom spore and they were therefore excluded from this study, and the 57 non-HP subjects were evaluated. In this study 24 workers quit because of intolerable cough, runny nose, wheezing, sputum, fever elevation and/or shortness of breath at their place of work. During each year of this study as many as 70-80% of employees suffered some ofthe above symptoms, cough being the most frequent, and positive rate of serum precipitins to the spore revealed 30% in 1996, 93% in 1997 and 94% in 1998. From the June 1996 examination until the following May, serum soluble intercellular adhesion molecule-1 levels of the 15 workers who quit during that period were significantly higher than those in the 42 workers still employed in 1997 (P < 0.05). Workers in Bunashimeji mushroom factories might be at critical risk of developing respiratory allergy. In our 3-year study over 90% workers were sensitized to the spore, 40% quit because of the symptoms and 5% developed HP. It was suggested that workers should be counselled about the risk of mushroom allergy and precautionary measures should be taken to prevent its occurrence.

Adult↗

CD2- CD4+ CD56+ hematodermic/hematolymphoid malignancy.

BACKGROUND: CD2- CD4+ CD56+ lymphoid malignancy has been only rarely reported the last 5 years. It is characterized by a high incidence of cutaneous involvement, cytologically agranular cells, aggressive clinical course, and negative Epstein-Barr virus (EBV) involvement. OBSERVATION: We describe a Japanese patient with a unique hematolymphoid malignancy characterized by an involvement of skin, nasopharyngeal region, bone marrow, lymph node, and a CD4+ CD43+ CD56+ CD2- CD3- CD8- and terminal deoxynucleotidyl transferase phenotype. Clinically, the cutaneous eruptions were purplish, hard, multiple nodules. Histologically, a massive proliferation of atypical pleomorphic cells with medium-sized nuclei were observed throughout the dermis. No clonal rearrangement of T-cell receptor (TCR)-beta gene or immunoglobulin heavy chain J gene was found, and no positive identification of EBV by in situ hybridization for EBV-encoded small nuclear RNA was found. The patient underwent high-dose chemotherapy with autografting of peripheral blood stem cells; however, the tumors quickly relapsed. CONCLUSION: We gathered data from 17 cases of lymphoid malignancy from the literature sharing immunophenotypic and genotypic features similar to those of our case, including CD2- CD4+ CD56+ and germline rearrangement of TCR. Although the cellular origin could not be decided, this malignancy was found to have 100% affinity for skin, a short course, and poor prognosis.

CD2 Antigens↗

Zosteriform and epidermotropic metastatic primary cutaneous squamous cell carcinoma.

The first case of primary cutaneous squamous cell carcinoma (SCC) to cause zosteriform and epidermotropic metastasis to skin is reported. The patient is a 72-year-old Japanese woman. A cutaneous SCC appeared on the lateral side of her right knee and was removed. After dissection of the right inguinal lymph nodes, which revealed metastases, and irradiation of the right inguinal region, the patient presented with slightly pruritic and painful erythematous papules on the right hip and small brownish papules and vesicles with crusts on the anterior side of the right thigh. The eruptions were in a zosteriform distribution along the right L1 to L3 dermatomes. Histologically neoplastic squamous cell nests were observed in the epidermis, below the epidermal-dermal junction, and within lymphatic vessels in the deeper reticular dermis. We postulate that neoplastic cells with the ability to fuse with adjacent squamous epithelium may have been carried beneath the basal lamina or to the epidermis via dermal lymphatic backflow, resulting in epidermotropic metastasis.

Aged↗

Pharmacology of SCH00013: a novel Ca2+ sensitizer.

Cardiotonic agents that facilitate cardiac pump function by direct improvement of contractile dysfunction are indispensable for the treatment of hemodynamic disorders in acute myocardial failure and the aggravating phase of congestive heart failure. Cardiotonic agents currently available for the treatment of hemodynamic crisis in congestive heart failure are catecholamines, selective phosphodiesterase (PDE) III inhibitors and digitalis, all of which are Ca2+ mobilizers. Considering the number of serious adverse effects of these clinically available cardiotonic agents, development of agents that act via a novel mechanism of action may contribute to the progress of pharmacotherapy of congestive heart failure. Ca2+ sensitizers that act by increasing in myofilament Ca2+ sensitivity may be able to overcome the disadvantage of Ca2+ mobilizers. Ca2+ sensitizers do not increase activation energy, do not produce Ca2+ overload and may be effective even under pathophysiological states such as acidosis, myocardial stunning and heart failure. SCH00013 ((4,5-dihydro-6-[1-[2-hydroxy-2-(4-cyanophenyl)ethyl]-1,2,5,6-tetrahydropyrido-4-yl]pyridazin-3(2H)-one)) is a novel Ca2+ sensitizer that elicits a moderate positive inotropic effect without significant alteration of Ca2+ transients. SCH00013 does not have a positive chronotropic effect and has a weak PDE III inhibitory action and class III antiarrhythmic action. SCH00013 prolonged the survival in a animal heart failure model with genetic cardiomyopathy. The oral bioavailability of SCH00013 is high and equivalent to that via intravenous administration. The unique pharmacological profiles of SCH00013 imply that this agent may be potentially beneficial for pharmacotherapy of contractile dysfunction in congestive heart failure.

3',5'-Cyclic-AMP Phosphodiesterases↗

Chronic hypersensitivity pneumonitis induced by Shiitake mushroom spores associated with lung cancer.

A 61-year-old man was admitted to our hospital with a 6-month history of productive cough. He, along with his wife, had been involved with Shiitake mushroom cultures for a period of 12 years. On admission, chest radiography showed bilateral fine-nodular shadow and CT scans showed reticulonodular opacities and a ground-glass appearance predominantly in the subpleural area in both lungs, and a mass in the left S6. Resected pathological specimens obtained by left lower lobectomy revealed lung adenosquamous carcinoma (stage IB), interstitial changes accompanied with lymphocyte proliferation and fibrosis, and granuloma with giant cells. Serum precipitins for Shiitake mushroom antigens were positive. The productive cough improved after the hospital admission and occurred again when he returned to work with the Shiitake mushroom production. Therefore, chronic hypersensitivity pneumonitis (HP) caused by Shiitake mushroom spores was diagnosed. Moreover, his wife was found to have HP caused by mushrooms at this time. There are only two previous reports of chronic HP caused by Shiitake mushroom in Japan, and this is the first case of chronic HP associated with lung cancer.

Alveolitis, Extrinsic Allergic↗

Germline mutation of the PTEN gene in a Japanese patient with Cowden's disease.

Cowden's disease (CD) is an autosomal dominant disorder which confers a high susceptibility to diverse benign and malignant tumors. The PTEN (phosphatase and tensin homologue deleted in chromosome ten) gene has been identified as a tumor suppressor gene responsible for cancers of the endometrium, ovary, prostate, and glioblastomas. Recently, germline mutations of this gene were also found in patients with CD, and it is now recognized as a gene responsible for this disease. We identified a germline nonsense mutation at codon 130 in exon 5 of PTEN in a 56-year-old Japanese woman with CD. The patient had adenoid facies and mucocutaneous lesions including multiple facial papules, acral keratoses on neck and shoulders, palmoplantar keratoses, multiple oral papillomas, scrotal tongue, mucosal and cutaneous hemangiomas, and a sclerotic fibroma on the arm. She also had benign and malignant polypoid neoplasms throughout the entire digestive tract, including adenocarcinoma of the colon and submucosal lipomas of the rectum, as well as bilateral breast carcinomas, multinodular goiters, an ovarian cyst with a fibroma-like nodule, hepatic hemangiomas, and abdominal hernia. We searched CD cases with the same genotypic PTEN mutation as the present case and compared their phenotypes. Further studies will disclose a better understanding of the role of mutation in the PTEN gene in the course of tumorigenesis of both benign and malignant tumors developed in patients with CD.

Codon, Nonsense↗

[The estimation of the effectiveness of GRF glue in the respiratory].

We had reported on the basis of experimental findings that the efficacy of protecting the suture line in tracheoplasty by using a self-fascia lata and GRF glue. This time we investigated the effectiveness of GRF glue in the respiratory surgery on the basis of clinical findings. All ten cases in which GRF glue was used resulted in an excellent outcome, namely, GRF glue prevented from air leakage, bleeding and leakage of chyle. Moreover, it was not recognized that any grave side effects occurred in any cases after using GRF glue. We could conclude that GRF glue was a useful material for the respiratory surgery, especially for the case that had a dead space after lobectomy. However its price is not inexpensive, therefore, there seems need to restrict the use of GRF glue--for example--to cases of Giant bulla resection, residual of a large dead space as a postoperative possibility and chemotherapy performed before an operation, and so on.

Aged↗

[Limited thoracotomy as surgical therapy for lung cancer: lobectomy and lymph node dissection by means of 12 cm skin incision].

In the domain of respiratory surgery minimally invasive approaches are currently being used and video-assisted thoracic surgery (VATS) is one of these methods. However VATS has some problems in its use for malignant lung diseases for example, with VATS adequate lymph node dissection and continuation of lobectomy in cases with severe adhesion are both difficult. In consideration of these points, limited thoracotomy has been practiced for treatment of lung cancer in our hospital. This study made comparisons between this new method (group A) and the conventional standard thoracotomy (group B). For the limited thoracotomy group (A) the skin incision and amount of bleeding were smaller than for the standard thoracotomy group (B) and the operation time was shorten. All limited thoracotomy group patients could lift up and move the hand of the affected side without pain immediately after the operation. The limited thoracotomy is an excellent approach as a curative operation for lung cancer, for preservation of post-operative pulmonary function, for movement capacity of the hand of the affected side and from the cosmetic viewpoint.

Adult↗

[Traumatic diaphragmatic herniation after continuous thoracic drainage: a case report].

We report a case of traumatic diaphragmatic hernia (TDH) resulting from continuous thoracic drainage and was successfully treated by surgical procedures. A 45-year-old man was admitted to our department due to shock after a blunt trauma by a traffic accident. As he revealed left hemothorax on admission, continuous thoracic drainage was performed. Soon after the drainage, diaphragmatic hernia occurred as an incarceration of the spleen into the thoracic cavity. In the literature, 80 cases with TDH have been reported in Japan since 1986. The purpose of this study is to discuss the mechanism of TDH in the acute phase and to consider its appropriate diagnostic tools. The following two results were obtained. (1) TDH may be appeared during the clinical course, especially after a continuous thoracic drainage, in patients with damaged diaphragm by blunt traumas. (2) CT is the most effective tool for the diagnosis of TDH.

Drainage↗

[The use of the autologous blood transfusion to the respiratory surgery: autologous blood transfusion or no-blood transfusion surgery?].

We could report the effect of the autologous blood transfusion to the lobectomy, by comparison with no-blood transfusion group and MAP-red cell concentrate transfused group (MAP group). Autologous blood which was stored three days before an operation and preserved at 4 degrees C kept 89.9 +/- 11.7% of the number of erythrocyte and 59.6 +/- 23.4% of platelet. Autologous blood transfusion group and MAP group showed significantly more than no-blood transfusion group on the number of erythrocyte at the time of discharge. These facts suggest that the autologous blood has an ability of coagulation. We concluded that on lobectomy autologous blood transfusion was better than MAP-red cell concentrate transfusion in point of protection from disease transmission and better than no-blood transfusion in point of post-operative recovery.

Aged↗

[Assessment of the therapy for the recurrent lung cancer after pulmonary operation].

As is generally known, pulmonary carcinomas have not good prognosis. Even though a post-operative p-stage is the first, the 5-year survival may be only about 70%. Therefore, the opportunity to encounter recurrent cases after pulmonary operations is increasing more and more. In this research we compared the three groups, group A; re-operations were performed for recurrent pulmonary cancers (eleven cases), group B; chemo- and/or irradiation-therapy were performed (eleven cases), group C; only conservative therapies were received (twenty-one cases). The result showed group A and group B were statistically better than group C on mean survival months (p < 0.001). From that conclusion we could indicate the therapeutic policy for recurrent pulmonary carcinomas as follows. in contralateral recurrent case, lesser resection (wedge/segment) with VATS as possible even if the same side recurrent case, aggressive removal of cancer (GRF glue is useful for protection against air leakage.) without lymph node dissection, as the same concept of metastatic lung tumor in case of non-operative indication, consideration of chemotherapy and/or irradiation, (especially hypotonic therapy was effective against carcinomatous hydrothorax.) in a difficult case for chemotherapy and/or irradiation, BAI is one of methods which ought to be tried.

Aged↗

DNA data bank of Japan (DDBJ) in collaboration with mass sequencing teams.

We at DDBJ (http://www.ddbj.nig.ac.jp) process and publicise the massive amounts of data submitted mainly by Japanese genome projects and sequencing teams. It is emphasised that the collaboration between data producing teams and the data bank is crucial in carrying out these processes smoothly. The amount of data submitted in 1999 is so large that it alone exceeds the total amount submitted in the preceding 10 years. To cope with this situation, we have developed tools not only for processing such massive amounts of data but also for efficiently retrieving data on demand.

Animals↗

Differential alteration of cardiotonic effects of EMD 57033 and beta-adrenoceptor agonists in volume-overload rabbit ventricular myocytes.

BACKGROUND: We investigated the effects of EMD 57033, a prototype Ca2+ sensitizer, and beta-adrenoceptor agonists in ventricular myocytes isolated from the volume-overload (V-O) heart failure model of the rabbit. METHODS AND RESULTS: V-O cardiac hypertrophy was induced in rabbits by the formation of an arterio-venous shunt between the carotid artery and jugular vein 12 to 15 weeks after the operation. Ventricular myocytes were enzymically isolated from normal and V-O rabbit hearts. The myocyte was loaded with a fluorescence Ca2+ dye, indo-1, and Ca2+ transients, and cell lengths were measured simultaneously. V-O myocytes were significantly larger than control myocytes. Duration of Ca2+ transients and cell shortening was significantly longer in the V-O myocytes than in control myocytes. Effects of cardiotonic interventions, including EMD 57033, isoproterenol, and dobutamine, on Ca2+ transients and cell shortening in V-O myocytes were compared with those in control rabbit myocytes. Isoproterenol and dobutamine increased the systolic cell shortening and peak Ca2+ transients and abbreviated the duration of cell shortening and Ca2+ transients. These responses were markedly attenuated in V-O myocytes. By contrast, the response of cell shortening to EMD 57033 was unaltered, and the Ca2+ sensitizing effect of EMD 57033 was rather enhanced in V-O myocytes. CONCLUSION: Our results indicate that the effectiveness of Ca2+ sensitizers is maintained in the V-O rabbit hypertrophy and heart failure model in contrast to the blunted response to beta-adrenoceptor agonists, which provides an insight on therapeutic strategy with Ca2+ sensitizers for the treatment of contractile dysfunction in congestive heart failure.

Adrenergic beta-Agonists↗