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Hiroshi Nemoto

Publications and source records attributed to Hiroshi Nemoto.

18 recordsLinked to original sources

Histological and immunohistological changes of the skeletal muscles in older SJL/J mice.

SJL/J mice have been studied as the model animals for autoimmunological diseases. Recently it was clarified that SJL/J mice have a defect of dysferlin. Human limb girdle muscular dystrophy 2B and Miyoshi myopathy also have a defect of dysferlin. In this study we present the histological and immunohistological changes in the natural course. Histological study revealed that SJL/J mice had inflammatory, degenerative changes, and neurogenic changes in later ages. As for interstitial inflammatory cells, the macrophages were dominant in any age, and in the T cell subset, the CD4+ T cells were more abundant than the CD8+ T cells, and few B cells were seen. The laboratory data showed a high level of creatine kinase in all ages. It is suspected that the inflammatory changes were induced by the primary immunological abnormality or by the defect of dysferlin in SJL/J mice.

Aging↗

Subacute combined degeneration of the spinal cord concomitant with gastric cancer.

We report a rare case of subacute combined degeneration of the spinal cord concomitant with gastric cancer. A 67-year-old man was admitted because of posterior column symptoms, pyramidal tract sign and peripheral neuropathy with severe hyperchromic anemia. He was treated with mecobalamin 1 mg IM, after which his anemia and neurological signs recovered. He was diagnosed as having subacute combined degeneration with pernicious anemia. Subsequent stomach biopsy revealed gastric cancer, and the patient underwent gastrectomy. It is a well known association that chronic atrophic gastritis is associated with gastric cancer or subacute combined degeneration. Our findings suggest that in this case subacute combined degeneration and gastric cancer are independent of each other; rather, both resulted from chronic atrophic gastritis.

Aged↗

High-level tryptophan accumulation in seeds of transgenic rice and its limited effects on agronomic traits and seed metabolite profile.

Metabolic manipulation of plants to improve their nutritional quality is an important goal of plant biotechnology. Expression in rice (Oryza sativa L.) of a transgene (OASA1D) encoding a feedback-insensitive alpha subunit of rice anthranilate synthase results in the accumulation of tryptophan (Trp) in calli and leaves. It is shown here that the amount of free Trp in the seeds of such plants is increased by about two orders of magnitude compared with that in the seeds of wild-type plants. The total Trp content in the seeds of the transgenic plants was also increased. Two homozygous lines, HW1 and HW5, of OASA1D transgenic rice were generated for characterization of agronomic traits and aromatic metabolite profiling of seeds. The marked overproduction of Trp was stable in these lines under field conditions, although spikelet fertility and yield, as well as seed germination ability, were reduced compared with the wild type. These differences in agronomic traits were small, however, in HW5. In spite of the high Trp content in the seeds of the HW lines, metabolic profiling revealed no substantial changes in the amounts of other phenolic compounds. The amount of indole acetic acid was increased about 2-fold in the seeds of the transgenic lines. The establishment and characterization of these OASA1D transgenic lines have thus demonstrated the feasibility of increasing the Trp content in the seeds of rice (or of other crops) as a means of improving its nutritional value for human consumption or animal feed.

Anthranilate Synthase↗

Autoimmune thrombocytopenic purpura, autoimmune hemolytic anemia and gastric cancer appeared in a patient with myasthenia gravis.

We report a case of myasthenia gravis (MG) associated with autoimmune thrombocytopenic purpura (AITP) and autoimmune hemolytic anemia (AIHA), and after that gastric cancer appeared. A 51-year-old man began to suffer from fluctuated muscle weakness in 1985. Muscle weaknesses became exacerbated, and he was admitted to our hospital in 1989. He was diagnosed as MG associated with AITP. After a thymectomy (hyperplasia), prednisolone therapy was started, subsequently his condition was satisfactory. In March 1995, he developed severe anemia and icterus. He was diagnosed as Evans' syndrome (AIHA and AITP) with MG. High-doses of immunoglobulin administration improved the anemia, but thrombocytopenia continued. In November 2002, he suffered marked petechia; the platelet count decreased to 1000/microl. Methylprednisolone pulse therapy and platelet transfusion were started. Gastrofiberscopy was performed and biopsy specimens revealed signet cell-type adenocarcinoma. On December 19, 2002, subtotal gastrectomy and splenectomy were performed. After that, his condition has remained satisfactory, without MG symptoms or thrombocytopenia. This is the first such case report in the literature.

Anemia, Hemolytic, Autoimmune↗

Novel NF1 gene mutation in a Japanese patient with neurofibromatosis type 1 and a gastrointestinal stromal tumor.

Many mutations of the NF1 gene have been reported in patients with neurofibromatosis type 1 (NF1); however, there have been no documented NF1 gene mutations in Japanese NF1 patients. In the present study, we used the polymerase chain reaction (PCR) and DNA sequencing analysis to characterize the NF1 gene in a 53-year-old Japanese patient with NF1 who suffered from neurofibroma, pheochromocytoma, and gastrointestinal stromal tumor (GIST). Direct sequence analyses revealed a single base substitution in the splicing donor site of intron 6 (IVS6 888+1, G --> A) in one NF1 allele, resulting in an altered splice site (ss) in the mutated allele. Splicing at the cryptic 5' ss in the mutated allele generated mRNA with an insertion of 60 nucleotides. In addition, we screened for mutations in exons 9, 11, 13, and 17 of the c-kit gene in GIST and the succinate dehydrogenase subunit D (SDHD) gene in the pheochromocytoma, but we did not detect any somatic mutations. We report here the first case of an NF1 patient with four neoplasms: neurofibroma, pheochromocytoma, astrocytoma and GIST. Our results suggest that the molecular pathogenesis of GISTs in NF1 patients is different from that in non-NF1 patients.

Female↗

Extrathymic tumors in patients with myasthenia gravis: a 35-year retrospective study.

OBJECTIVE: Autoimmune diseases are frequently associated with malignant tumor. In addition, prolonged immunosuppression may favor the development of malignancy. While the coincidence of myasthenia gravis and extrathymic tumor has been reported, the risk and features of these tumors are not well understood. REVIEW SUMMARY: We treated 305 patients with myasthenia gravis from 1968-2003, including 48 thymoma cases. Two hundred twenty-nine patients had undergone thymectomy and 76 had not. We examined cancer risk, tumor characteristics, and associations to medications. We encountered 9 cases of extrathymic tumor. Cancer risk in the thymoma cases was 6.3% and 2.3% in the nonthymoma cases, a statistically insignificant difference. Azathioprine was administered to only 14 in this series of patients; however, 2 patients developed cancer. CONCLUSIONS: Cancer risk in patients with myasthenia gravis is 2.6%, similar to that of the general population in Japan. We neurologists need to be aware that prolonged immunosuppression may favor the development of malignancy.

Adult↗

[Case study--an elderly patient with Stage IV advanced gastric cancer achieved good performance status (PS) without subjective symptoms for more than two years by chemotherapy].

An 83-year-old male with Stage IV gastric cancer of performance status 1 (PS 1) was treated with fluoropyrimidine (TS-1) since January 2003 in our department. As the patient exhibited decreased renal function due to his age, we set the basic dose at 80% of the recommended dose of 80 mg/body/week. We administered 11 four-week cycles, each with a two-week drug-free washout period, and six two-week cycles, each with a two-week drug free washout period. The first cycle of chemotherapy ameliorated the subjective symptoms; the level of a tumor marker then returned to normal, and the ascites disappeared. The patient's condition was maintained at a favorable PS level without any subjective symptoms for approximately 20 months. In late September 2004, however, an exacerbation of the primary foci was confirmed by routine endoscopic examination. As second-line chemotherapy, we initiated weekly administration of paclitaxel (PTX). Due to the patient's age and high PS level (PS 2) at the time of intervention, we utilized a reduced dose of 70 mg/body/dose. After a short premedication period, the patient was administered PTX intravenously for one hour once a week for three weeks, then given a one-week drug-free washout period (one cycle). After the first cycle of PTX administration, all subjective symptoms disappeared, the PS level returned to zero,and the patient was discharged. In total, four cycles of the PTX regimen were given. Throughout the treatment period, we did not observe any significant adverse events (grade 2 or higher). Treatment achieved a favorable PS level. To design an effective chemotherapy regimen for elderly patients, it is important to establish an effective dose and dosing method based on the patient's chronologic age and a thorough evaluation of the patient's systemic conditions, including the PS level.

Adenocarcinoma, Mucinous↗

[A case of primary HIV infection presenting as mononeuritis multiplex].

A 34-year-old man presented with gait disturbance. He had a low grade fever and diarrhea for a few days prior to admission. Two weeks after he started to have diarrhea, he developed gait disturbance with a left foot drop. The cerebrospinal fluid obtained on admission showed pleocytosis (30/microl) and increased protein. The motor nerve conduction velocities (MCV) of the left peroneal nerve and the tibial nerve were slow, but the right peroneal and tibial MCVs were within normal limits. A test for human immunodeficiency virus (HIV) antibody was positive. The Western blot was positive with bands of gp160 and p24, confirming HIV infection. Seroconversion-related neuropathy of HIV was diagnosed. Acute HIV infection should be included in the differential diagnosis of mononeuritis multiplex. Before full-fledged AIDS development, it is important to find early stage of HIV infection in patients, so that we can treat them more effectively by means of anti-HIV drugs.

Adult↗

[A case of Brown-Vialetto-van Laere (BVVL) syndrome in Japan].

Here we report a sixty-year-old woman of Brown-Vialetto-van Laere (BVVL) syndrome in Japan. She had sensorineural deafness, weakness and atrophy of her extremities from 15 years of age. Her neurological symptoms slowly progressed. She first visited our hospital in 1993 when she was 49 years old. At that time, she had distal muscle weakness and atrophy of the four extremities and bulbar palsy. Deep tendon reflexes were absent and the plantar toe reflex was flexor. EMG revealed neurogenic changes and the nerve conduction studied were normal. The vital capacity was marked decreased. On August 10, 2003, she was admitted to our hospital because of CO2 narcosis. She had III, VII, X, XI, XII cranial nerve palsy, distal muscle weakness and atrophy of the four extremities. From her neurological symptoms and signs, we made a diagnosis of BVVL syndrome. MRI revealed no high signal in pyramidal tract by FLAIR image. ABR showed no response, and VEP demonstrated delay of the P100. She was intubated, and was attached to a respirator to improve her CO2 narcosis. After treatment she improved and did not need to be assisted by a respirator during daytime. During night time, she had apnea, and her blood gas showed the retention of CO2, and she still required the respiratory assistance during her sleep. This is the first report of BVVL syndrome in Japanese literature.

Bulbar Palsy, Progressive↗

Bone density in myasthenia gravis patients receiving long-term prednisolone therapy.

Osteoporosis is an adverse effect of prednisolone therapy, although no study has been conducted on myasthenia gravis patients receiving high-dose prednisolone. We measured bone density in 36 patients (26 females and 10 males) who had undergone long-term prednisolone administration, and found a decrease in bone density in 31% of female patients and osteoporosis in only 11.5% (three cases). This frequency is lower than the presumptive rate of the general population in Japan (22.6%). No osteoporosis was detected in male patients. In conclusion, prednisolone-treated patients with myasthenia gravis have an acceptable risk of bone loss if prophylactic medication is administered.

Absorptiometry, Photon↗

Ulcerative colitis presenting as sensorineural deafness, brainstem encephalopathy, and white matter lesions.

BACKGROUND: Several rare neurologic complications of ulcerative colitis have been reported. REVIEW SUMMARY: We report a 69-year-old Japanese woman who developed bilateral sensorineural deafness, 2 attacks of bilateral ophthalmoplegia, and bilateral facial nerve palsy in association with ulcerative colitis. Laboratory data showed elevated cerebrospinal fluid (CSF) protein without pleocytosis, abnormal auditory brainstem evoked potentials, and multiple high signal lesions on magnetic resonance imaging of the brainstem and cerebral deep white matter. Her symptoms improved with corticosteroid therapy except for sensorineural deafness and an exacerbation of cerebral deep white matter lesions without any new clinical signs. CONCLUSION: Immunologic mechanisms may have led to her central and peripheral nervous system findings in addition to her colon disorder.

Aged↗

Skeletal muscle myosin is the autoantigen for experimental autoimmune myositis.

Experimental autoimmune myositis (EAM) is a rodent model for human inflammatory muscle disease (IMD). It can be induced by immunization of rodents with skeletal muscle homogenate and adjuvant. The specific myositogenic autoantigen has not been clearly identified although some evidence points to skeletal muscle myosin. In this report we strengthen this evidence, showing that Lewis rats immunized with purified skeletal muscle myosin develop EAM with the same pattern and severity as EAM induced by whole rabbit skeletal muscle homogenate (WRM). Multiple inflammatory lesions are detected histopathologically in the biceps, quadriceps, and gastrocnemius muscles. Myosin-reactive T cells from animals immunized either with myosin or with WRM have similar patterns of antigen-induced proliferation. The results show that myosin, a component of skeletal muscle, is at least one autoantigen in EAM.

Animals↗

[Complete remission of esophageal undifferentiated carcinoma with nedaplatin and 5-FU chemotherapy and endoesophageal brachytherapy].

The patient was a 74-year-old female. Type 1 undifferentiated carcinoma (non-small cell type) was detected in the middle of the thoracic esophagus in August 1999. Although the lesion was diagnosed as T2, N0, and Stage II, the patient was judged to be a poor risk, inoperable case because of a complex past history of renal and respiratory dysfunctions, and dysbasia. Intravenous administration of nedaplatin at 15.8 mg/m2 and 5-FU 590.6 mg/m2 were carried out for 5 consecutive days as chemotherapy. The second cycle of chemotherapy was performed with nedaplatin reduced to 11.8 mg/m2 on the basis of the adverse reactions observed after the first cycle, and PR was attained. As for radiotherapy, additional extracorporeal irradiation was judged to be too dangerous from her history, so endoesophageal brachytherapy alone was added, and CR was obtained. The patient has maintained a CR for more than 2 years after discharge. In this poor risk case with a highly malignant undifferentiated carcinoma, an "individualization strategy" was effective.

Aged↗

[Assessing liver function by magnetic resonance imaging two-dimensional phase-shift flow measurement of portal venous blood flow after oral intake of glucose].

We have already reported that the ratio of portal venous flow 30 min after oral intake of glucose 75 g to that before intake (PVFR30), measured using pulsed-Doppler ultrasonography (US), correlated significantly with other indicators of liver function and that it could be used to estimate hepatic function before surgery, including liver resection. In this study, to assess the disadvantages of pulsed-Doppler ultrasonography, PVFR30 was measured using two-dimensional (2D) phase-shift (PS) magnetic resonance imaging (MRI). PVFR30 was measured in 17 patients and 7 volunteers: 13 with liver cirrhosis (LC) and 11 without LC (non-LC). Portal venous flow could be measured in all patients without any disturbance of intestinal gas or patient fat, or the high degree of technical skill that Doppler US requires. PVFR30 was significantly lower in the LC group than in the non-LC group. In addition, it correlated significantly with other indicators of liver function, including the indocyanine green clearance test, prothrombin time, hepaplastin test, and cholinesterase activity. These results suggest that PVFR30 measured by 2D PS MRI can be used to estimate liver function, and that this MRI method can be performed more easily than pulsed-Doppler US.

Administration, Oral↗

Microwave coagulation therapy for a liver tumor involving the root of the left hepatic vein of the residual liver after extended right hepatectomy.

A 54-year-old man had received low anterior resection for rectal carcinoma and extended right hepatectomy for a metastatic liver tumor. One year later, imaging studies disclosed a metastatic liver tumor in segment 2 of the liver. The residual left hepatic vein was completely invaded by the tumor from the root of the hepatic vein to the confluence of several branches by intraoperative ultrasonography. To resect partial liver with the involved hepatic vein was deemed impossible. Microwave coagulation therapy was performed on the tumor and the involved hepatic vein, as we had established the safety of microwave irradiation to the main hepatic vein without interference of the hepatic venous flow in an experimental study. The patient remains well 40 months after the surgery without recurrent signs. The hepatic venous flow of the irradiated hepatic vein is maintained well according to serial examination of Doppler ultrasonography.

Carcinoma↗

Is fenestration a safe treatment for adult polycystic liver disease?: A report of refractory complications.

We report a 67-year-old man with highly symptomatic polycystic liver disease. Fenestration was selected to treat symptoms because the cysts were scattered diffusely and the normal liver volume was limited. Although this patient was relieved from symptoms of liver cysts consequently, several severe postoperative complications including disseminated intravascular coagulation, respiratory failure, liver failure, and biliary leakage occurred resulting in a 6-month postoperative hospital stay. Although various treatments for symptomatic adult polycystic liver disease have been advocated, a definitive treatment remains controversial, especially in diffuse adult polycystic liver disease. Fenestration is one of the alternative treatments for the patients whose cysts are difficult to resect. However high morbidity rate should be carefully assessed, if extensive fenestration is needed to treat diffuse adult polycystic liver disease. Further consideration of appropriate treatments for diffuse adult polycystic liver disease is needed.

Aged↗