[Clinical aspect of gastroesophageal reflux disease].
Explore the source record for details and available documents.
Gastroesophageal Reflux↗
Biomedical subjects
M Endo
Publications and source records attributed to M Endo.
Relation between stent expansion and arterial remodeling: a serial intravascular ultrasound study.
Previous studies suggested that stent area (SA) did not change after the Palmaz-Schatz stent implantation. Whether these findings apply to other types of stent, however, is unknown. This study assessed vascular response to stent implantation using intravascular ultrasound (IVUS) studies. Serial (pre-intervention to follow-up) IVUS imagings were used to study 57 native coronary lesions after the GFX stent or the Multilink stent implantation. The vessel area (VA) at lesion site increased at follow-up (16.92 +/- 3.67 mm(2) after intervention to 18.17 +/- 4.66 mm(2) at follow-up, P < 0.01). The SA also increased from 8.39 +/- 1.90 mm(2) after intervention to 8.80 +/- 2.08 mm(2) at follow-up (P = 0.02). Thirty-two percent of lesions showed late stent expansion. The stent expansion [Delta (after intervention to follow-up) SA] correlated significantly with the VA growth [Delta (after intervention to follow-up) VA] (r = 0.59, P < 0.0001). In conclusion, some lesions reveal late stent expansion after both the GFX stent and the Multilink stent implantation. Adaptive vessel remodeling may be followed by stent expansion.
Aged↗
Cardiac function evaluated by transesophageal echocardiography during cardiopulmonary bypass.
OBJECTIVE: To evaluate cardiac function at cardiopulmonary bypass weaning, we applied a new technique clinically to determine the approximated Emax without using a conductance catheter. METHODS: Subjects were 5 patients. The left ventricular end-systolic pressure was obtained by overlaying the radial arterial pressure curve on the left ventricular pressure curve. Left ventricular end-systolic volume was assessed by a transesophageal echographic apparatus. At cardiopulmonary bypass weaning, volume loading was applied to increase left atrial pressure by a few mmHg while fixing the pump flow rate at half flow. Changes in left ventricular end-systolic volume and approximated left ventricular end-systolic pressure for total heart beat were plotted during this period, and the gradient of the regression line was taken as approximated Emax. RESULTS: Approximated Emax ranged from 1.29 to 3.28 (mean 2.13 +/- 0.72), and its correlation coefficient was 0.80 +/- 0.06. CONCLUSION: Our new technique is useful in evaluating cardiac function during cardiopulmonary bypass.
Adult↗
St. Jude Medical prosthetic aortic valve malfunction due to pannus formation.
According to the literature, the incidence of pannus formation with the St. Jude Medical prosthetic aortic valve has been reported at 0.03% and 0.14% (per patient-year), with no case report of St. Jude Medical prosthetic aortic valve malfunction due to pannus formation. Between 1980 and 1999, 1,186 patients underwent aortic valve replacement at our institute. We encountered 2 aortic valve malfunctions due to pannus formation, including the case of a 53-year-old woman who suffered a St. Jude medical aortic valve malfunction 13 years after the initial operation. A second aortic valve replacement was successful and the postoperative course was uneventful. The possibility of pannus formation on St. Jude Medical aortic valves must thus be considered and its mechanism clarified.
Aortic Valve↗
Progress reports on immune gene therapy for stage IV renal cell cancer using lethally irradiated granulocyte-macrophage colony-stimulating factor-transduced autologous renal cancer cells.
There is no effective treatment for patients with stage IV renal cell cancer (RCC), although the introduction of new therapy is imminent. Cancer gene therapy is currently considered to be one of the most promising therapeutic modalities in the field of cancer treatment. Based on the results of animal studies, vaccination using autologous granulocyte-macrophage colony-stimulating factor-transduced renal cancer cells appears promising. Before initiating a clinical study using an ex vivo gene-transduced autologous cell vaccine-based immunogene therapy for RCC in Japan, in 1992 we initially planned a Japanese version of a clinical protocol in collaboration with a US group. In 1993, the original protocol was refined. We performed five preclinical qualification studies using RCC nephrectomy specimens from patients in 1997, and the results showed that preparation of RCC cells for autologous vaccines at the Clinical Cell Technology Facility, Research Hospital of the Institute of Medical Science, University of Tokyo, was feasible. Subsequently in August 1998, the Ministry of Health and Welfare and the Ministry of Education, Science, Culture, and Sport approved our clinical protocol. We have recruited two patients with stage IV RCC to our study so far. Here we report the background to the initiation of cancer gene therapy in Japan.
Adult↗
Acute alteration in bone mineral density and biochemical markers for bone metabolism in nephrotic patients receiving high-dose glucocorticoid and one-cycle etidronate therapy.
It is widely known that glucocorticoids induce and accelerate osteoporosis. High-dose glucocorticoids are administrated daily to patients in the acute phase of nephrotic syndrome. It could be inferred that high-dose glucocorticoids rapidly decrease patients' basal bone mineral density (BMD) and this accelerates the natural progress of osteoporosis associated with aging or menopause. Nine nephrotic patients (male/female: 5/4) without previous prednisolone administration were chosen to measure BMD and the level of the markers for bone turnover before and after treatment for 3 months (total prednisolone administration: 4.5 +/- 0.0 g). Twenty-three patients under remission with prednisolone administration (male/female: 14/9) were included in the long-term treatment group. Patients in this group whose %YAM in the lateral lumbar spine was less than 89% were classified into a low BMD group (n = 10, male/female: 3/7). They were administered etidronate disodium at 200 mg/day for 14 days. BMD and % of young adult mean (YAM) in the lumbar spine (L2-L4 in lateral objection) and other regions were measured by dual-energy X-ray absorptiometry. As markers of bone metabolism, the urinary level of deoxypyridinoline (Dpd) was determined to evaluate osteogenesis, and serum osteocalcin was measured to evaluate bone resorption. BMD of the lumbar spine significantly decreased in the 3-month treatment group (752 +/- 96 mg/cm(2), 7 +/- 4% reduction) compared with the pretreatment group (810 +/- 85 mg/cm(2)). BMD in the long-term treatment group decreased continuously (683 +/- 135 mg/cm(2)). No significant differences were noted in other measurement sites. BMD in the etidronate treatment group increased significantly (597 +/- 55 mg/cm(2)) compared with the pretreatment group (549 +/- 76 mg/cm(2)). Etidronate did not change BMD at the sites with a normal BMD. Among the biochemical markers (BM) examined, the urinary level of Dpd (nMol/liter. Cr) significantly increased in the 3-month treatment group (8.6 +/- 5.1 nMol/liter.Cr) compared with the pretreatment group (5.8 +/- 2.0 nMol/liter. Cr). No significant differences were seen in the BMs measured in the long-term treatment group. The urinary Dpd level of the etidronate treatment group decreased (3.9 +/- 1.4 nMol/liter. Cr) compared with the pretreatment group. These data indicate that etidronate could improve the accelerated bone resorption. In conclusion, high-dose glucocorticoid therapy causes rapid bone resorption and accelerates the natural progress of osteoporosis associated with aging or menopause. Etidronate administration prevents the progress of osteoporosis in nephrotic patients. Preventive treatment should be performed when the estimated BMD in 3 months falls below the baseline by more than 7 +/- 4%, reaching the therapeutic range.
Adolescent↗
Cronkhite-Canada syndrome: report of two cases.
Two cases of Cronkhite-Canada syndrome are reported. In the first case, a 56-year-old woman had an adenoma of the colon, arising within the Cronkhite-Canada polyps, which was removed by endoscopic polypectomy. This suggests possible neoplastic transformation of polyps in this syndrome. She achieved remission with corticosteroids, but the polyposis recurred (only in the stomach) 7 months after the remission. In the recurrent polyposis, corticosteroid therapy resulted again in complete remission, which has lasted for 5 years. In the second case, a 69-year-old man developed typical manifestations of the syndrome while under emotional stress. He had a past history of chronic pityriasis lichenoides, and serum antinuclear antibody was positive. These findings suggested a possible role of autoimmune response in the pathogenesis of the syndrome. Corticosteroids were also effective in this patient.
Adenoma↗
Pulmonary vascular changes induced by congenital obstruction of pulmonary venous return.
BACKGROUND: Pulmonary venous obstruction (PVO) induces pulmonary arterial hypertension, as well as pulmonary venous hypertension, and jeopardizes the repair of cardiac lesions. METHODS: Four cases of congenital mitral stenosis and 4 cases of cor triatriatum (Lucas type A), ages ranging from 2 months to 16 years, were histologically examined on pulmonary vasculature. Histometrical analysis was performed on medial thickness and intimal changes of both pulmonary arteries and veins. For comparison, the examination of pulmonary vasculature in ventricular septal defect (VSD) cases was also performed. RESULTS: Medial thickening and intimal fibrosis, in both pulmonary arteries and veins with widespread lymphangiectasia, were characteristic vascular changes of PVO cases. Medial thickness of pulmonary arteries was correlated with preoperative pulmonary arterial pressure (PAP) (r = 0.77, p = 0.03 for systolic PAP), and greater than that of VSD cases. Medial thickness of pulmonary veins was also greater in PVO cases. Intimal fibrosis of pulmonary arteries and veins was seen extensively at the advanced ages, whereas no plexiform lesions or more advanced stages of pulmonary vascular disease were present. CONCLUSIONS: Congenital PVO induced progressive medial thickening and intimal fibrosis in pulmonary arteries and veins accompanied by lymphangiectasia. However, no plexiform lesions or more advanced stages of pulmonary vascular disease were present, which may explain the reversibility of pulmonary hypertension due to congenital PVO.
Adolescent↗
The effect of atorvastatin on serum lipids and lipoproteins in patients with homozyous familial hypercholesterolemia undergoing LDL-apheresis therapy.
The efficacy of atorvastatin, a new hydroxymethylglutaryl (HMG)-CoA reductase inhibitor, in reducing serum lipid levels, modifying lipoprotein composition, and suppressing cholesterol synthesis was evaluated in patients with homozygous familial hypercholesterolemia (homozygous FH) undergoing LDL-apheresis therapy. Atorvastatin was given in escalating doses (10, 20, and 40 mg/day) to nine patients with homozygous FH. Five of nine patients responded well to atorvastatin; four of these patients were receptor-defective and the remaining one was receptor-negative. The change in LDL-cholesterol in the receptor-defective patients averaged -20.6% compared to the baseline level at the highest dose of atorvastatin. Of five receptor-negative type patients, only one showed good response to atorvastatin therapy with a LDL-cholesterol reduction of 14.9%. Although the other four receptor-negative patients did not show a change in LDL-cholesterol, all of them exhibited a considerable increase in HDL-cholesterol. All patients showed reduced urinary excretion of mevalonic acid, suggesting that atorvastatin decreases LDL-cholesterol by inhibiting cholesterol biosynthesis even where LDL-receptor activity is not present. Atorvastatin also decreased serum triglycerides in both receptor-negative and defective patients, especially in the latter. As cholesterol level rebounds quickly after each apheresis procedure, a combination therapy using atorvastatin and apheresis may increase the efficacy of the apheresis treatment, improving cost-benefit effectiveness by reducing the frequency of the apheresis treatment.
Adolescent↗
Complement activation through the lectin pathway in patients with Henoch-Schönlein purpura nephritis.
Henoch-Schönlein purpura nephritis (HSPN) is considered a form of systemic vasculitis of the small blood vessels with immune pathogenesis. In this disorder, the complement system is recognized as an important mechanism of glomerular injury. The aim of this study is to determine whether the lectin pathway, a novel pathway of complement activation, is related to the pathogenesis of HSPN. Renal biopsy material from 10 patients with HSPN was studied immunohistochemically and examined for a clinicopathologic correlation. Serum levels of complement components, including mannose-binding lectin (MBL), and plasma levels of complement activation products were also evaluated in these patients and compared with levels in patients with immunoglobulin A (IgA) nephropathy or mesangial proliferative glomerulonephritis (GN) without IgA deposition (non-IgA GN). Glomerular deposition of components of the pathway, MBL and MBL-associated serine protease (MASP-1), as well as C3b/C3c, C5b-9, and C4-binding protein (C4-bp), was detected in 8 of 10 patients. Although no significant correlation was found between glomerular deposition of MBL/MASP-1 and histological or clinical findings, the biopsies on all patients with MBL/MASP-1 deposits were performed within 20 weeks from the onset of disease. Levels of plasma C4d, the activation fragment of C4, and C4-bp, a soluble regulatory protein of the pathway, were greater in patients with HSPN than in those with non-IgA GN. However, there was no difference in serum MBL levels between the three groups of patients (HSPN, IgA nephropathy, and non-IgA GN). These results suggest that complement activation through the lectin pathway was involved at the onset of HSPN, and this mechanism might be important in the disease pathogenesis.
Adolescent↗
Heterogeneity of renal cortical circulation in hypertension assessed by dynamic computed tomography.
The aim of this study was to assess the grade of heterogeneous disturbance in the renal cortical circulation using dynamic computed tomography and to investigate the relationship between the heterogeneity of renal cortical circulation and hypertension. We studied 125 patients who underwent dynamic computed tomography (CT) for various abdominal diseases and had no serious hemodynamic abnormalities. In dynamic computed tomography under appropriate conditions, each pixel (image element), less than 1 mm2, has a CT number that is in proportion to the concentration of contrast media, which reflects the blood volume in the pixel. The image was constructed at the hilus level about 50 s after the start of a continuous infusion of contrast medium. The mean and standard deviation were calculated from the CT numbers in the renal cortex. The coefficient of variation, ie, the standard deviation divided by the mean value, was used as the index of the heterogeneity of renal cortical circulation. The coefficient of variation was significantly (P < .001) greater in the hypertensive patients (n = 48, 0.174 +/- 0.006 [mean +/- SE]) than in normotensive subjects (n = 77, 0.140 +/- 0.004). The coefficient increased in parallel with the patient's age and with the grade of renal surface irregularity. In the patients whose serum creatinine levels were normal, this parameter also had a significant relationship (r = 0.367, P < .0001) with serum creatinine. These results suggest that the heterogeneity of renal cortical circulation is increased in hypertension and is also associated with aging. This parameter may become a sensitive indicator to detect slight deterioration in the renal cortical circulation.
Adult↗
Tandem duplication of the FLT3 gene is infrequent in infant acute leukemia. Japan Infant Leukemia Study Group.
Explore the source record for details and available documents.
Acute Disease↗
The PEBP2beta/CBF beta-SMMHC chimeric protein is localized both in the cell membrane and nuclear subfractions of leukemic cells carrying chromosomal inversion 16.
The chromosomal inversion (16)(p13q22), which is associated with the M4-eosinophilia subtype of human acute myeloid leukemia, causes the fusion of two distinct genes. The polypeptide encoded by the chimeric gene, PEBP2p/CBFp-SMMHC, retains the ability to interact with, and dominantly interfere with the function of proteins possessing the Runt homology domain. The Runt protein homologs constitute the DNA binding subunit of the PEBP2/CBF transcription factor. We examined the subcellular localization of PEBP2beta/CBFbeta-SMMHC, as well as that of Runt protein homologs in leukemic cells carrying inversion 16 by immunoblot analysis. A significant amount of the PEBPbeta/CBFbeta-SMMHC protein was recovered from the nuclear fraction along with the Runt protein homologs. Furthermore, some of both polypeptides was retained in the DNA pellet that represents the material remaining after extraction of nuclear fraction with high salt. These observations suggest that the so-called dominant interfering effect of PEBPbeta/CBFbeta-SMMHC on PEBP2/CBF occurs inside the nucleus. In addition, we could detect PEBP2beta/CBFbeta-SMMHC in the cytoplasmic membrane fraction as well. The function of this membrane-located PEBP2beta/CBFbeta-SMMHC, if any, appears to be unrelated to that of Runt protein homologs.
Binding Sites↗
Molecular mode of interaction of plant amine oxidase with the mechanism-based inhibitor 2-butyne-1,4-diamine.
2-Butyne-1,4-diamine (DABI) is a mechanism-based inhibitor of copper-containing plant amine oxidases; the number of turnovers that leads to enzyme inactivation is approximately 20. The product of DABI oxidation is a very reactive aminoallene that reacts with an essential nucleophilic group at the enzyme active site, forming a covalently bound pyrrole and producing an inactive enzyme. The inactivated enzyme shows a new absorption maximum at 295 nm and gives coloured derivatives with p-dimethylaminobenzaldehyde and p-dimethylaminocinnamaldehyde that are spectrally similar to the products of pyrrole treated with the above reagents. Resonance Raman spectra of the p-dimethylaminobenzaldehyde adduct of pyrrole and the inactivated enzyme show very high degree of similarity, supporting the idea that the product of inactivation is indeed a bound pyrrole. The bound pyrrole is formed already in the anaerobic step of the reaction, while the topa semiquinone radical is not affected, as shown by the EPR and stopped-flow absorption measurements. Peptides containing the DABI binding site were obtained by proteolysis of inactivated enzyme, isolated by HPLC and analysed by amino acid sequencing and MS. The crystal structure of the amine oxidase from pea has been determined; inhibition is caused mainly by the highly reactive DABI product, 4-amino-2-butynal, binding to a nucleophilic residue at the entrance to the substrate channel. As other DABI labelled peptides were also found and no free DABI product was detected by MS after complete inhibition of the enzyme, it is likely that the DABI product binds also to other solvent exposed nucleophilic residues on the enzyme surface.
Amine Oxidase (Copper-Containing)↗
Pleural amyloidosis in a patient with intractable pleural effusion and multiple myeloma.
Pleural involvement of systemic amyloidosis has been rarely reported. We report a case with multiple myeloma presenting an intractable right pleural effusion, in which pleural amyloidosis was diagnosed through pleural biopsy using a Cope needle. The diagnosis of pleural amyloidosis is important, because its refractory pleural effusion should be treated with pleurodesis. Since closed pleural biopsy using a Cope needle is much less invasive than thoracoscopy, the former should be attempted first whenever pleural amyloidosis is suspected.
Aged↗
Clinicopathologic analysis of lymph node metastasis in surgically resected superficial cancer of the thoracic esophagus.
We examined lymph node metastasis clinicopathologically in 236 cases of superficial cancer (T1, Tis) of the thoracic esophagus surgically resected at our department without adjuvant treatment. Mucosal cancer was observed in 112 cases (47%) and submucosal cancer in 124 cases (53%). Lymph node metastasis was present in 3% of mucosal cancer cases and 41% of submucosal cancer cases. By the recent pathologic subclassification of the extent of the cancerous invasion in superficial esophageal cancer, mucosal cancer and submucosal cancer were each divided into three subtypes according to the extent of invasion, i.e. m1, m2, m3, sm1, sm2 and sm3 cancers. There was no case of lymph node metastasis in m1 and m2 cases, but it was observed in 8% of m3 cases, in 11% of sm1 cases, in 30% of sm2 cases and in 61% of sm3 cases. The number of involved nodes was three or less in m3 and sm1 cases, however four or more involved nodes were observed in 14% of sm2 cases and in 24% of sm3 cases. Positive lymph nodes were found only in the mediastinum in m3 and sm1 cases. On the contrary, they were found extensively in the mediastinum, the abdomen and the neck and in two or more regions in 27% of sm2 cases and in 38% of sm3 cases. Considering the location of positive nodes, the recurrent nerve lymph nodes were most frequently involved, followed by the cardiac lymph nodes. A similar tendency was observed in cases with single node metastasis. The 5-year survival rate of cases from m1 to sm1 was similar. That of sm3 cases was significantly worse than that of other groups. Based on the clinical results, the therapeutic guidelines for superficial cancer of the thoracic esophagus are considered to be as follows: (i) in m1 and m2 cancer, endoscopic mucosal resection is generally indicated in principle, although transhiatal esophagectomy may be indicated in some cases; (ii) in m3 and sm1 cancer, endoscopic mucosal resection is performed initially, then subsequent treatment is selected if necessary; (iii) in sm2 and sm3 cancer, conventional transthoracic esophagectomy with systematic lymph node dissection is indicated.
Adult↗
Glomerular and extraglomerular immune complex deposits in a bone marrow transplant recipient.
A 44-year-old man developed nephrotic syndrome 9 months after HLA-identical sibling bone marrow transplantation. Membranous changes consisted mainly of alterations of glomeruli, which were interpreted as chronic graft-versus-host disease (GVHD) caused by lodging of the circulating immune complex. In the tubules, a lumpy deposition of IgG and complement breakdown products was distributed along the tubular basement membrane, which coincided with the peculiar deposits ascertained by electron microscopy. These findings suggest that an extraglomerular reaction should be considered in evaluating renal involvement of GVHD.
Adult↗
Surgical treatment of neuroblastoma with micrometastasis.
BACKGROUND/PURPOSE: The aim of this study was to define the role of surgery in neuroblastoma with micrometastasis, which is detectable only by the polymerase chain reaction (PCR) method. METHODS: Fifty samples (peripheral blood 9, bone marrow 41) were harvested sequentially from 27 neuroblastoma patients, and the micrometastases were examined using the previously described single-step PCR method. The results were reviewed with the clinical courses. RESULTS: Radical surgery was performed in 9 patients with bone marrow micrometastasis. Event-free survival was obtained in 2 patients with stage IV disease (25.0%) for a follow-up period of 2 to 6 years in this group. Both patients received intraoperative radiation and subsequent autologous bone marrow transplantation (ABMT) using purged marrow. Radical surgery was performed in 18 patients without micrometastasis, and 6 of 9 advanced patients (66.7%) survived without the disease including 4 patients who received unpurged stem cell transplantation. CONCLUSIONS: Persistent micrometastasis in bone marrow should be considered predictive as a poor prognostic factor in neuroblastoma. Intensive local control with surgery and radiation is important for the patients with micrometastasis and should be followed by ABMT using purged marrow. Unpurged marrow may be safely used if the single-step PCR detects no micrometastasis.
Adrenal Gland Neoplasms↗