An infant with severe atopic dermatitis and progressive hepatomegaly due to fatty liver.
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Adolescent↗
Biomedical subjects
R Murata
Publications and source records attributed to R Murata.
Effect of early oral fluoroquinolones in hemorrhagic colitis due to Escherichia coli O157:H7.
During the Sakai outbreak of Escherichia coli O157:H7 infection, which was linked to contaminated cafeteria school lunches, there were several treatment modalities with regard to antimicrobial drugs. Patient outcomes among three hospitals with different modalities were compared retrospectively. Hemolytic uremic syndrome did not develop in any of the 15 patients treated with oral fluoroquinolone therapy; however, HUS did develop in three of 15 patients treated with intravenous (i.v.) fosfomycin and in two of 12 patients treated with i.v. cefotaxime and oral fosfomycin. The results indicate that oral fluoroquinolone therapy administered within 3 days of illness is effective in preventing the development of HUS; however, prospective randomized double-blind studies on early antimicrobial therapy of O157 hemorrhagic colitis are necessary. Several antibiotics, including fluoroquinolones, were reported to induce the production or release of Shiga-like toxins (STX) from E. coli O157:H7 in vitro. Although patients were examined for fecal STX, no STX were detected in the stools of patients treated with oral fluoroquinolones. In fact, treatment with fluoroquinolones for 5 days eradicated E. coli O157 in all patients.
Administration, Oral↗
Fifteen-year follow-up of a heterozygous Fabry's disease patient associated with pre-excitation syndrome.
A 47-year-old woman with heterozygous Fabry's disease with pre-excitation syndrome has been followed up for 15 years. Diagnosis was confirmed by the typical electron microscopic feature of the endomyocardial specimen and a decreased plasma alpha-galactosidase activity. As the disease progressed, the interventricular septum thickened from 11 to 17 mm as measured by echocardiography, while the AH interval was prolonged from 80 to 140 msec. In Fabry's disease, the PR interval has been reported to be variable from short PR to AV block. Therefore, this case may be helpful to understand the time course in the AV conduction abnormalities with the progression of Fabry's disease.
Biopsy↗
Different transcriptional expression of the matrix gene of the two sibling viruses of the subacute sclerosing panencephalitis virus (Osaka-2 strain) isolated from a biopsy specimen of patient brain.
Two sibling viruses of the subacute sclerosing panencephalitis (SSPE) virus Osaka-2 strain were isolated from a small biopsy specimen of the brain of an SSPE patient just before intraventricular interferon treatment by cocultivation with two different cell lines, Vero cells or B95a cells (Ogura et al, 1997). Both the virus-infected cells were found to be indistinguishable from each other in defective production of cell-free virus and in defective expression of the matrix (M) protein. The sequence analysis of the M genes predicted that they were translatable due to a lack of alteration of the translational start and stop codons for the proteins. A different pattern of the M monocistronic transcripts, however, was observed in a Northern blot analysis of the infected cells. This different pattern was confirmed further by a primer extension analysis. The undetectable expressions of the M proteins in the sibling virus-infected cells are most probably different in their molecular mechanisms. All these results indicate the possibility that the two different, replicable variants existed at Jabbour stage III of the disease's progression in a very small portion of the brain, where no lesion had yet been recognized by a magnetic resonance imaging.
Amino Acid Sequence↗
[Efficacy of zonisamide in West syndrome].
The efficacy of zonisamide (ZNS) was studied in 16 patients (11 males, 5 females) with West syndrome (WS), symptomatic in 13 and cryptogenic in 3. They did not respond to pyridoxal phosphate (12 cases) or valproate (16 cases). The mean age of onset of WS was 4.4 (2-9) months. ZNS was administered from 3 to 9 months of age (mean 6.1). Four cases (2 cryptogenic and 2 symptomatic) became seizure free. Two had more than 50% seizure reduction. Ten infants remained unchanged or showed less than 50% seizure reduction. In the 4 responders, the effective dose was 4-8 mg/kg (mean 5.8), and the serum ZNS concentration was 10-21 micrograms/ml (mean 13.8). One had relapse of WS after 4 months. Three with normalized EEG remained seizure-free during the follow-up period (12-26 months). One case developed a transient drowsiness, but no serious side effects were observed. These data suggest ZNS may be regarded as a therapy of choice before synthesized ACTH therapy in the management of WS.
Anticonvulsants↗
A long-term surviving patient with cancer of papilla of Vater after local resection.
We report successful local resection for cancer of papilla of Vater in an 86-year-old woman. She was referred to our hospital because of right hypochondralgia. Abdominal ultrasonography and computed tomography showed marked dilatation of the common bile duct (CBD). Endoscopic retrograde cholangiography disclosed a small shadow defect in the terminal of the dilated CBD. Biopsy of the papilla revealed well-to-moderately differentiated adenocarcinoma. Considering her extreme old age and keeping in mind her quality of life after the operation, and the finding that the tumor was localized within the papilla and highly differentiated, we performed local resection. In addition, the intrapancreatic portion of the CBD and part of the main pancreatic duct (MPD) were further resected to secure a negative margin, confirmed by frozen section. The MPD was reapproximated to the duodenal mucosa and a choledocho-duodenostomy was performed for CBD reconstruction. Histopathological examination showed the tumor was papillary adenocarcinoma, 10 x 15 mm in size; there was no invasion beyond the sphincter of Oddi, it had partly infiltrated the CBD, but had not invaded to the pancreas or duodenum. The patient's postoperative course was not eventful and she has had good quality of life for the past 6 years since the operation, without any evidence of recurrence. Although radical pancreaticoduodenectomy is now the standard procedure in patients with malignant tumor of the papilla of Vater, local resection is a reasonable alternative for high-risk patients with highly differentiated, apparently localized carcinomas.
Adenocarcinoma↗
Nucleotide sequences of the matrix protein gene of subacute sclerosing panencephalitis viruses compared with local contemporary isolates from patients with acute measles.
Measles viruses isolated from brain cells of patients with subacute sclerosing panencephalitis (SSPE) have numerous mutations, especially in the matrix protein (M) gene. To find whether the M genes of these SSPE viruses were mutated randomly or in a pattern, we sequenced this gene from three strains of defective measles virus isolated in Osaka, Japan. We could deduce the sequence of the possible progenitor measles virus for each patient by comparison of the isolate with measles viruses prevailing at roughly the same time and place as the primary infection. Biased hypermutation affected the M genes of all three SSPE viruses, although the molecular mechanisms for the mutations might be various. Replacements of U with C in the plus strand accounted for 76% of all mutations in two of the strains, but in the other strain, replacements of A with G accounted for 52% of the mutations, and the U residues were unchanged.
Acute Disease↗
CT and MR imaging of cerebral tuberous sclerosis.
Tuberous sclerosis is a heredofamilial neurocutaneous syndrome, or phakomatosis, with multisystem involvement including the brain, kidney, skin, retina, heart, lung, and bone. The brain is the most frequently affected organ in tuberous sclerosis. Brain lesions in tuberous sclerosis are of three kinds; cortical tubers, white matter abnormalities, and subependymal nodules. We review the computed tomography (CT) and magnetic resonance (MR) features of the brain lesions in patients with tuberous sclerosis. CT clearly demonstrates calcified subependymal nodules. MR imaging demonstrates more clearly cortical, and white matter lesions than CT, since MR imaging shows excellent image contrast between various normal structures and high sensitivity in detecting pathological states due to intrinsic differences in proton density and in particular, in proton relaxation times of tissues. Possible pathogenesis of this disorder is also discussed.
Brain↗
The matrix gene expression of subacute sclerosing panencephalitis (SSPE) virus (Osaka-1 strain): a comparison of two sibling viruses isolated from different lobes of an SSPE brain.
The Fr/V and Oc/V sibling viruses of the Osaka-1 strain of the subacute sclerosing panencephalitis (SSPE) virus were defective in cell-free virus production. By radioimmunoprecipitation assay, the matrix (M) protein was not detected in cells persistently infected with the Osaka-1 strain. This undetectable expression was consistent with the selective reduction of antibody response to the M protein in the patient from whom the Osaka-1 strain was isolated. The sequence of the M gene, however, predicted that the protein could be synthesized because the translational start and stop codons for the protein were not altered. Northern blot hybridization demonstrated the selective defect of the monocistronic mRNAs for the M protein and the phosphoprotein (P) together with the dominant presence of the P-M bicistronic mRNA. This absence of the M mRNA was further confirmed by primer extension analysis. Therefore, the undetectable expression of the M protein in the infected cells was proved to be caused by a transcriptional defect. The two sibling viruses, isolated from remote portions of an SSPE brain, were indistinguishable in their viral characters, including the M gene sequences, which indicates the possibility of clonal expansion of the strain in the brain.
Animals↗
Encephalopathy and cytomegalovirus colitis in an AIDS child.
A 2-year-old girl, who had prolonged thrush and spastic diplegia, was found to have a mother-to-child vertical transmission of human immunodeficiency virus type-1 (HIV). A brain computed tomography scan revealed a symmetrical calcification on the bilateral basal ganglia and periventricular white matter. She had an acquired immune deficiency syndrome (AIDS) encephalopathy of pure dominant pyramidal tract disorder without an intellectual deficit. Helper cell lymphocyte count (CD4) increased with the beginning of zidovudine (ZDV, also known as AZT) monotherapy but began to decrease after the 4th week to reach the baseline at 20th week. Zidovudine plus didanosine combination therapy was started at the 68th week, but because of intolerance, the combination was changed to ZDV plus lamivudine at the 98th week. By the 80th week, neither severe opportunistic infection nor deterioration of the neurological status was recognized, but chronic diarrhea appeared. The diarrhea advanced to the wasting syndrome at the age of 4 years and cytomegalovirus genome was confirmed in a biopsied specimen of the colon. Ganciclovir treatment was effective in stopping the diarrhea and increasing her bodyweight, but after the age of 5, resumption of diarrhea was followed by progressive emaciation and weakness. This work may provide some clues in treating children's AIDS.
AIDS Dementia Complex↗
[Home mechanical ventilation with nasal intermittent positive pressure ventilation for a boy with congenital central hypoventilation syndrome].
We report a 4-year-old boy with congenital central hypoventilation syndrome (CCHS) successfully treated with home mechanical ventilation with nasal intermittent positive pressure ventilation (NIPPV) during sleep hours. He had had frequent severe apneic attacks from the neonatal period. At 8 months, he was treated with positive pressure ventilation following a tracheostomy. At 4 year and 2 months, NIPPV was attempted because of recurrent respiratory tract infections and cor pulmonale. The tracheostomy was successfully abandoned 6 months later. Adequate ventilation has been maintained for more than 3 years without troubles. NIPPV is an effective and non-invasive treatment of CCHS that it significantly improves the quality of life during daytime.
Child, Preschool↗
[Intraarterial chemotherapy combined with radiation therapy for advanced cancer of the uterine cervix].
The results of intraarterial chemotherapy (IACT) combined with definitive radiation therapy for 23 advanced and bulky carcinomas of uterine cervix are reported. IA-CT with cisplatin 50 mg and doxorubicin 30 mg was administered by one shot method in bilateral internal iliac arteries. The protocol consisted of one to three treatment session every 3 weeks. Nine of eleven patients with clinical stage III achieved a complete local response (82%), and the 3- and 5-year survival in these cases were 72% and 72%, respectively. These results were superior to the response (58%), 3- and 5-year survivals (68%, 58%) obtained in 19 patients treated mainly with radiation therapy alone. The side effect of grade 2 and 3 for the intestine, such as ileus and hemorrhagic colitis, was noted in 3 patients (15%). In addition, 3 of 8 patients with radical surgery and postoperative radiation therapy after IA-CT developed insufficient fracture of pelvic bone. These complications accompanied by IA-CT combined with radiation therapy and/or surgery increased slightly, compared with that by the previous therapy without IA-CT, but were not critical. The results suggest that IA-CT following radiation therapy is effective to improve the prognosis of patients with Stage III cervical cancer.
Antineoplastic Combined Chemotherapy Protocols↗
Radiation therapy for ocular choroidal neovascularization (phase I/II study): preliminary report.
PURPOSE/OBJECTIVE: Choroidal neovascularization (CNV) is a major cause of severe loss of visual acuity in some ocular diseases such as age-related macular degeneration (ARMD) and angioid streaks. Laser photocoagulation has been used to treat patients with subfoveal neovascular lesions with well-demarcated boundaries. However, the treatment method is usually associated with a large decrease in visual acuity. Therefore, indications for this treatment are very limited. Recently, some investigators reported the effect of low dose irradiation on the subretinal neovascular membranes in CNV. We conducted a Phase I/II study to determine the toxicity and efficacy of external photon beam radiotherapy in patients with CNV. METHODS AND MATERIALS: Between April, 1994 and July, 1995, 36 patients with choroidal neovascularization (34 with ARMD and 2 with angioid streaks) were treated with radiation therapy. Treatment planning was performed using a CT simulator that enables real-time treatment planning from multiple CT slices. The clinical target volume that included the macula and optic disc received a dose of 10 Gy/5 fractions/1 week (first 18 eyes) or 20 Gy/10 fractions/2 weeks (last 18 eyes). All eyes were irradiated with a single lateral 6 MV photon beam, angled 10 degrees posteriorly to exclude the ipsilateral lens and the contralateral eye from the radiation field. The ipsilateral lens was irradiated with less than 10% of the total reference dose. The field size averaged 3.0 x 2.5 cm. Records of the 17 eyes with CNV referred to our hospital in 1993, which satisfied the eligibility criteria for this study, were retrospectively analyzed for comparison. RESULTS: There was no significant acute morbidity. All patients were followed regularly by both ophthalmologists and radiation oncologists. Cataract formation after 1 year of the treatment was observed in one patient who had received a dose of 20 Gy. One patient who had received 20 Gy complained of transient dry-eye sensation 2 months after treatment, but this had disappeared spontaneously by the fourth month. The subjective symptoms, visual acuity, and size of the neovascular membrane were evaluated at 6 and 12 months after treatment in each patient. In the group of patients irradiated with 10 Gy and with 20 Gy, respectively, subjective symptoms improved in five and seven eyes, did not change in seven and four eyes, and deteriorated in six and six eyes at 12 months. Although visual acuity was significantly decreased in the control group, the patients in both irradiated groups did not show such a decrease in visual acuity. The size of the neovascular membrane in the control group progressed significantly. However, the patients in the 20 Gy group showed significant regression of the membrane, although those in the 10 Gy group showed no significant change in size. CONCLUSION: This Phase I/II study including a dose escalation study showed that radiation therapy seems to be useful for CNV. The dose of 20 Gy in 10 fractions was more useful in treating neovascular membranes than the dose of 10 Gy in five fractions. These results have encouraged us to start a multicenter randomized prospective study of the treatment of CNV with radiation therapy.
Aged↗
An antiangiogenic agent (TNP-470) inhibited reoxygenation during fractionated radiotherapy of murine mammary carcinoma.
PURPOSE: TNP-470, a synthetic analogue of fumagillin which is a natural product of Aspergillus fumigatus, has been noted as an angiogenesis inhibitor. Combined effects of TNP-470 with fractionated radiotherapy (RT) were investigated using a mouse tumor. METHODS AND MATERIALS: Tumors were early generations of mammary carcinoma in C3H/He mice. Treatments were initiated when tumors reached an average diameter of 4-5 mm. Tumor response was evaluated by tumor growth (TG) time assay and 50% tumor control dose (TCD50) assay. Tumors were irradiated locally under hypoxic conditions or in air. Five fractionated radiation doses were given in the TG time assay, whereas a single dose or 10 fractionated doses were given in the TCD50 assay. TNP-470 (100 mg/kg) was administered subcutaneously twice a week during and/or after RT. RESULTS: In the TG time assay, significant delay of tumor growth was observed by TNP-470 alone (100 mg/kg x 2) compared with control tumors (p < 0.001), indicating that TNP-470 alone has an antitumor effect in vivo. When TNP-470 was administered during fractionated RT, no additional delay of tumor growth was observed. However, additive effects of TNP-470 was noted when it was given after the end of fractionated RT. In the TCD50 assay, no significant difference in TCD50s was observed between RT alone and RT combined with TNP-470 in single dose experiments. Hypoxic fraction of tumors calculated from the TCD50s was not affected significantly by administrating TNP-470 24 h before RT. On the other hand, in 10-fraction experiments, the TCD50 (RT with TNP-470, in air) was significantly higher than the TCD50 (RT alone, in air) (p < 0.005), indicating that TNP-470 given during fractionated RT decreased radiocurability. This negative effect of TNP-470 was not observed when TNP-470 was combined with fractionated RT given under hypoxic conditions. CONCLUSION: The tumor control probability decreased by administrating TNP-470 during fractionated RT in air. This unexpected result may be attributable to partial inhibition of reoxygenation by TNP-470, because no significant difference was noted between the TCD50 (RT with TNP-470) and the TCD50 (RT alone) under hypoxic conditions.
Animals↗
Retrohepatic cavoatrial shunt with a ringed polytetrafluoroethylene graft for the Budd-Chiari syndrome. A case report.
This report describes a patient with Budd-Chiari syndrome who was operated on successfully by means of shunt formation with polytetrafluoroethylene graft between the inferior vena cava (IVC) and right atrium. The patient is a sixty-two-year-old woman suffering from persistent edema of the lower limbs for four years. The examination disclosed complete obstruction of the IVC at the level of the diaphragm with a patent right inferior hepatic vein. Following the operation, edema of the limbs disappeared, hypersplenism improved, and the serum ammonium concentration decreased to the normal range. In conclusion, a retrohepatic cavoatrial shunt is feasible and useful in treating a patient with the Budd-Chiari syndrome who has patent major hepatic veins.
Blood Vessel Prosthesis↗
Effect of non-insulin dependent diabetes on cyclosporin A disposition in Goto-Kakizaki (GK) rats.
We previously reported that the pharmacokinetics of cyclosporin A (CyA), particularly absorption, were altered in diabetic rats treated with streptozotocin. In the present study, the effect of diabetes on pharmacokinetics of CyA after intravenous and oral administration of CyA using the blood and lymph and the gastrointestinal transit were examined in Goto-Kakizaki (GK) rats, a genetic model of non-obese non-insulin-dependent diabetes mellitus (NIDDM), and compared to non-diabetic Wistar rats. Although the systemic and lymphatic availability after intravenous administration of CyA to the GK rats was not significantly different from those of the control Wistar rats, those availability after oral administration of CyA to GK rats was markedly reduced in comparison. These results suggest that the pharmacokinetics of CyA, particularly absorption, was altered in GK rats. Studies on the gastrointestinal transit in GK rats showed that the gastric emptying rate was lower than that of Wistar rats, suggesting that a change in gastrointestinal transit in GK rats may influence the absorption of CyA. The gastric emptying rate in GK rats altered not only the systemic availability but also the lymphatic availability, suggesting that the altered systemic availability may cause adverse effects and that altered lymphatic availability may influence the immunosuppressive effects.
Administration, Oral↗
Efficient isolation of subacute sclerosing panencephalitis virus from patient brains by reference to magnetic resonance and computed tomographic images.
Subacute sclerosing panencephalitis virus has been isolated with difficulty from brains of infected patients. More strains are needed for the study of the pathogenesis of this virus. To make the isolation more efficient, we selected portions to be examined from the brains of three patients with reference to findings of repeated magnetic resonance and computed tomographic imaging. Three cell lines susceptible to measles virus field strains were used. In all three cases viruses were isolated most effectively from recent lesions and with Vero cells. Our results suggested that these imaging methods and Vero cells could be used for improvement in the efficiency of isolation of this virus from patient brains.
Adult↗
[Right-sided intrathoracic bypass grafting for a coarctation of the aorta in an advanced aged woman].
A sixty-two years old woman with a successful bypass grafting for coarctation of the thoracic aorta (Co-A) is presented. She has been suffering from hypertension since her thirties. Preoperative cardiac catheterization demonstrated Co-A with a pressure gradient of 100 mmHg. Angiography revealed the hypoplastic aortic arch with marked calcification and well developed collateral circulation. Right-sided intrathoracic bypass grafting using a 16 mm woven Dacron graft was placed between the ascending and descending aorta via a right sixth intercostal space. The patient had a good postoperative course with reduced pressure gradient of 10 mmHg. We recommend this procedure to be a safe and simple technique that can avoid injury to the collateral circulation to our knowledge, this is the most aged patient who underwent a successful surgical treatment for the Co-A in Japan.
Aorta↗