Systemic lupus erythematosus and anticardiolipin antibodies in Klinefelter's syndrome.
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Biomedical subjects
Publications and source records attributed to H Uchida.
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Antihypertensive effects of repeated oral administration of cilnidipine in 2K1C renal hypertensive dogs were compared with those of nicardipine. On the first day, oral administration of cilnidipine (3 mg/kg) or nicardipine (3 mg/kg) markedly lowered both systolic and diastolic blood pressure 1 hr after administration. The hypotensive effects of cilnidipine were longer compared with those of nicardipine. Both drugs elevated the heart rate and plasma renin activity. On the 8th and 15th days, similar responses were obtained by repeated administrations of cilnidipine and nicardipine. After withdrawal of these drugs, no rebound phenomena in blood pressure were observed. The changes in mean blood pressure were correlated with plasma cilnidipine or nicardipine concentrations that were obtained at each time of blood pressure measurement (r = -0.598; P < 0.001 and r = -0.594; P < 0.001, respectively). These results suggest that stable and long-acting antihypertensive effects of cilnidipine for 15 consecutive days in renal hypertensive dogs are related to the change in plasma drug concentrations.
We present a unique case of renovascular hypertension due to combined renal artery disease in a 22-year-old woman. Renal angiography revealed renal artery stenosis with poststenotic dilatation and an aneurysm due to fibromuscular dysplasia in the left kidney, and a congenital arteriovenous fistula in the right kidney. The results of a captopril test and plasma renin sampling demonstrated that the renin-angiotensin-aldosterone system was stimulated in both kidneys, accounting for the hypertension in this patient. Almost all cases of renovascular hypertension are due to only one underlying renal artery disease. This is the first case of renovascular hypertension associated not only with renal artery stenosis and an aneurysm due to fibromuscular dysplasia, but also with a congenital arteriovenous fistula.
Color Doppler flow imaging was carried out in 10 multilocular cystic lesions of the kidney. These consisted of three renal cell carcinomas, three hemorrhagic renal cysts, one benign multilocular cystic nephroma, two infected renal cysts, and one benign multilocular renal cyst secondary to von Hippel-Lindau disease. In the patients with renal cell carcinoma, color signals were obtained at the septum and in the solid component within the lesions. A pulsatile wave with a large maximum flow velocity and a high PI value was obtained in two of them. In one of the patients with hemorrhagic renal cysts, color signals were obtained at the region of the septum. In the other benign lesions, however, color signals were obtained only at the peripheral margin, and the flow waveform in these cases was identical to the waveforms found in the interlobar arteries. This study suggested that color display in the lesion well reflects its vascularity in patients with multilocular cystic renal diseases and that measurement of the systolic maximum flow velocity by fast Fourier transform analysis is useful for the differential diagnosis of malignant versus benign lesions.
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A new highly sensitive thermoluminescent (TL) sheet has been studied as a means of in vivo measurement of spatial dose distribution of radiation therapy. This TL sheet (40 cm x 50 cm x 0.2 mm), which is composed of teflon mixed with BaSO4:Eu doped powder, is very flexible and can be cut to the desired size. The TL sheet was found to have a linear response with a very wide dynamic range of at least 0.002 cGy to 5000 cGy absorbed dose. In addition, this sheet does not need be shielded, because of its insensitivity to room light, and therefore the sheet can detect low-energy electrons decreased by a few millimeter thick air for 3H(maximum beta-ray energy: 18 KeV). The spatial dose distribution was printed out with a newly developed digital readout system. In addition, another high-resolution dosimetry system, which exposes X-ray film with TL photons emitted from the irradiated TL sheet at constant room temperature, is reported. Clinically, the in vivo dose distribution on the surface of the rectal cancer for intracavitary radiation therapy was determined. The applicability of TL sheet for in vivo measurement of dose distribution is discussed.
UNLABELLED: This study evaluates the mucociliary transport system in patients with diffuse panbronchiolitis using aerosol inhalation cine-scintigraphy (AICS). METHODS: Forty-one subjects, 10 healthy controls and 31 patients with diffuse panbronchiolitis, were studied. In addition, the mucociliary transport system was evaluated in 11 patients who had received erythromycin therapy for 3-8.3 yr. Following inhalation of 99mTc-human serum albumin aerosol for 3-5 min in a sitting position, the subjects were placed on the imaging table in the supine position and posterior images were obtained dynamically for 20 sec/frame over 2 hr with a gamma camera linked to a digital computer. The 360 20-sec serial frames were edited into a cinematographic presentation at 200-msec intervals. Clinical evaluation of the mucociliary transport system was based on the bolus movement of radioactive aerosol from the main bronchi to the trachea and the movement patterns, which were divided into four types using the movement in the controls as a standard (type I): type I, rapid and smooth movement; type II, slow movement; type III, stagnation at the carina; and type IV, complete stasis. RESULTS: All patients with diffuse panbronchiolitis had types III and IV, indicating that mucociliary transport system was severely impaired. Of the 11 patients on erythromycin therapy, 8 had movement pattern type IV and 3 had movement pattern type III before erythromycin therapy. In eight patients (72.7%), movement pattern was improved to type I or II after therapy. CONCLUSION: Aerosol inhalation cine-scintigraphy helps evaluate the clinical usefulness of erythromycin therapy in diffuse panbronchiolitis.
A rare subcutaneous leiomyosarcoma metastatizing to the sphenoid bone and presenting exophthalmos is reported. A 56-year-old female presented with protrusion of the right eye and a slowly growing lump on the right temporal region. Six years previously, she had undergone removal of a subcutaneous mass in the back, which was histologically diagnosed in another hospital as leiomyosarcoma. She had undergone four other operations, including removal of local recurrences and a right renal metastasis. On admission, physical examination showed no neurological deficits. Craniogram revealed an osteolytic lesion without marginal sclerosis in the right sphenoid bone. CT showed an inhomogeneously enhanced mass with irregular expansion of the diploic space, which was partly invading the right orbit. MRI demonstrated an extradural mass in the right sphenoid region, which was slightly low-intense in T1-weighted image, high-intense in T2-weighted image, and inhomogeneously enhanced by Gd-DTPA. Right external carotid angiogram showed a highly vascular stain fed by meningeal arteries. Radionuclide bone scintigram showed multiple high-uptake areas in the left femoral head, the ribs, and the sphenoid bone. Preoperative embolization of the tumor vessels fed by the external carotid artery was performed. Following this procedure, the tumor stain disappeared completely. The tumor was totally excised with minimal bleeding through an orbitozygomatic approach. The tumor was loosely adherent to the dura and periorbit. The bone defect was covered with a methylmethacrylate resin plate. the histological examination demonstrated fascicular arrangement of the spindle shaped cells with mitotic figures. Immunohistochemical studies showed that most tumor cells were positive for actin and myosin, but negative for desmin.(ABSTRACT TRUNCATED AT 250 WORDS)
Monoclonal gammopathy of undetermined significance (MGUS) denotes the presence of a monoclonal protein (M protein) in patients, without evidence of multiple myeloma, macroglobulinemia, amyloidosis or other related diseases. Differential diagnosis of MGUS from multiple myeloma is difficult in cases of the unusual type of each disease. A serum monoclonal protein concentration of less than 3 g/dl, normal polyclonal immunoglobulin, no or small amount of Bence Jones proteinuria and the infiltration of plasma cells of less than 10% in bone marrow are suggestive of MGUS. In the literature, about 25% of the MGUS have developed multiple myeloma or related diseases in a long term follow-up. This fact calls our attention to the management of MGUS.
We studied the anterior chamber inflammation with a laser flaremeter after transvitreal membrane peeling surgery for posterior vitreous membrane syndrome and cataract intraocular lens surgery, through separate surgery and combined surgery. In 16 eyes with vitrectomy and membrane peeling, mean flare count had one peak of 11 photons/msec at postoperative days 5 through 7. In 10 eyes with phacoemulsification and aspiration with posterior chamber lens implantation, the mean flare count had two peaks of 11 photons/msec at postoperative day 1 and in postoperative week 2. In 25 eyes with combined surgery of vitrectomy and cataract intraocular lens surgery, the mean flare count was similar to the summation of that of each simple surgery. In these 25 eyes, in 9 eyes with sutures, the mean flare count had two peaks of 37 photons/msec at postoperative day 1 and 27 photons/msec at postoperative week 2, and in 16 eyes with self sealing wounds, the mean flare count had one peak of 22 photons/msec at postoperative week two. In 14 vitrectomized eyes with cataract-intraocular lens surgery, the mean flare count was as high as in combined surgery. In eyes with self sealing wounds, the flare count at postoperative day one was significantly lower than that of sutured eyes.
Neoplastic angioendotheliosis (NAE) is a rare neoplastic disease, and its pre-mortem diagnosis is extremely difficult. A 49-year-old male developed vertigo, hearing and visual disturbance, transverse myelopathy below Th 5 and hypercalcemia. These symptoms were markedly improved by VEPA chemotherapy. Thirty-four months after onset, diffuse reticular shadows were noted on chest X-ray. The biopsy specimen of the lung revealed intravascular lymphoid cells differentially stained with L26 and LCA. Southern blot analysis of the DNA from the tissue showed rearranged bands for the immunoglobulin gene (JH). A diagnosis of NAE of B-cell nature was established.
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Sixty patients with diabetes mellitus (DM) antedated pregnancy were enrolled; seven had proliferative retinopathy, 13 had simple retinopathy, and 40 were intact. Diet and/or insulin was prescribed to adjust their glucose control at fasting to < 100 mg/dl, as well as at 2 hours postprandial to < 120 mg/dl. Glycohemoglobin (Hemoglobin A1c) levels ranged between 5.4% and 6.4% in the third trimester in three groups. Incidences of pregnancy complications (toxemia, hydramnios, urinary tract infection and cesarean section) and neonatal complications (low Apgar score, hypoglycemia, jaundice, polycythemia, respiratory distress syndrome and anomaly) did not differ significantly with the grade of retinopathy. Compared with the intact group, the duration of DM was significantly longer in the retinopathy groups and the incidence of fetal distress was significantly higher in the proliferative retinopathy group. In ten of 60 patients (16.7%) the grade of retinopathy progressed during pregnancy. In four patients photocoagulation was performed for neovascularization, and proved to be effective. There was a tendency for those whose retinopathy progressed to the proliferative stage during pregnancy to have larger decreases in glycohemoglobin and for their retinopathy to worsen after delivery. With tight maternal glucose control and intensive fetal surveillance, we obtained good perinatal outcome in pregnancies with diabetic retinopathy, as compared to diabetic pregnancy without diabetic microangiopathy. Careful and frequent monitoring of retinal changes should be required during pregnancy and the postpartum period.
We evaluated the nerve fiber layer defect (NFLD) in glaucomatous eyes imaged by a scanning laser ophthalmoscope (SLO, Rodenstock Gm BH, Munich, Germany) and its relationship to parafoveal visual field defects. Twenty-three eyes of 20 patients with open angle glaucoma were studied. Only those eyes were used in which NFLD reaching the temporal raphe of the nerve fiber layer was observed by the SLO in either the superior or the inferior hemisphere. We defined three topographic parameters of the NFLD: 1. the angle (0, degree) generated by a line A passing through the foveal pit and the disc center, and a line through the disc center to the point of the NFLD at the disc edge and closest to line A, 2. the horizontal distance (D1, mm) along the temporal raphe between the foveal pit and the point of the NFLD nearest to the foveal pit, and 3. the vertical distance (D2, mm) between the foveal pit and the point of NFLD nearest to the foveal pit. We also defined the nearest defect location (in degrees from the fovea) as the stimulus point with sensitivity loss greater than 6 dB in the Humphrey visual field (program central 10-2). A highly significant correlation was observed between each of the three NFLD parameters and the nearest defect location (p < 0.001). The NFLD parameters we have newly defined here may be helpful for evaluating parafoveal visual functional damages in open angle glaucoma.
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Acetylacetonato complexes of lutetium, ytterbium, thulium, and europium ions efficiently hydrolyze the phosphodiester linkage in adenylyl(3'-5')adenosine. The pseudo first-order rate constant (3.2 x 10(-2) h-1) at pH 7.2 and 30 degrees C for the 1:1 lutetium-acetylacetonato complex (0.5 mmol dm-3) is close to the value (3.5 x 10(-2) h-1) for free lutetium ion of the same concentration. Potentialities of these complexes as the catalytic sites of artificial ribonucleases are indicated.
Renal ablation by transcatheter renal arterial embolization (TAE) was performed in 10 patients with benign renal disease (hydronephrosis n = 6; renovascular hypertension n = 3; nephrotic syndrome n = 1). Each affected kidney had little or no renal function. Six patients with hydronephrosis were treated with TAE using absolute ethanol alone in three patients and the combination of absolute ethanol and gelatin sponge in the other three. Each patient was followed by sclerotherapy of the pelvocalyceal system via nephrostomy using absolute ethanol. In four of the six patients, the embolized kidney had no urine, and there was very little urine in the remaining two. The size of the embolized kidney was markedly decreased on CT. The three patients with renovascular hypertension were pre-studied by selective and renal vein sampling for PRA, and the kidney excreting higher renin was embolized by TAE with absolute ethanol. Blood pressure has become manageable without antihypertensive drug in two patients and with a reduced amount of drug in one. The patient with nephrotic syndrome had end stage renal failure and showed significant protein excretion. To prevent further protein loss, both kidneys were embolized with stainless steel coils. Urine output was significantly decreased, and consequently, hypoalbuminemia improved. All patients tolerated the procedure well, and there were no significant complications. Renal ablation by TAE may be an alternative to surgical treatment in selected patients with benign renal disease, particularly in patients with contraindications to surgery and in the elderly. Absolute ethanol and gelatin sponge seem safe and effective for TAE in patients with hydronephrosis and renovascular hypertension. For hydronephrosis, we recommend combining TAE with sclerotherapy of the pelvocalyceal system via nephrostomy using absolute ethanol. Though we successfully applied steel coil for the patient with nephrotic syndrome, absolute ethanol may be equally effective.
BACKGROUND: Several uncontrolled trials have suggested that dose intensity of chemotherapy is a crucial determinant of treatment outcome for patients with non-Hodgkin's lymphoma (NHL). To explore the possibility of increasing dose intensity, a dose-escalation study of cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) using recombinant human granulocyte colony stimulating factor (rhG-CSF) was initiated. METHODS: First, the feasibility of standard dose CHOP (750 mg/m2 cyclophosphamide intravenously [i.v.] on Day 1;50 mg/m2 doxorubicin i.v. on Day 1; 1.4 mg/m2 vincristine i.v. on Day 1; and 100 mg/body prednisolone orally on Days 1-5) repeated biweekly at the original dose was assessed. rhG-CSF was given subcutaneously at doses of 2-5 micrograms/kg every day or every other day on Days 3-13. The safety of increasing the dose of cyclophosphamide during biweekly CHOP then was tested. Besides the standard dose (750 mg/m2), two dose levels of cyclophosphamide were set (1200 mg/m2 and 1500 mg/m2 in patients younger than 61 years of age, and 1200 mg/m2 in patients 61-75 years old). RESULTS: Twenty-seven patients with NHL who had received minimal or no previous treatment were enrolled in this study. In the 750 mg/m2 group, 9 patients received 3-6 cycles of treatment (mean, 3.9 cycles), in the 1200 mg/m2 group, 10 patients received 3-6 cycles (mean, 4.8), and in the 1500 mg/m2 group, all 8 patients received 6 cycles. No significant differences among the groups were observed in the extent and the duration of neutropenia in each cycle, and a leukocyte count of more than 3000/microliters on Day 15 was achieved in all 131 cycles. Hemoglobin values and platelet counts, however, decreased in the later cycles in the 1500 mg/m2 group. Two patients were hepatitis-B virus carriers, one of whom died of fulminant hepatitis after completion of six cycles. Another patient developed a transient increase of transaminases after the second cycle. One other patient developed Grade 4 mucositis (World Health Organization scale). The numbers of patients who achieved complete and partial responses, respectively, were 4 (50%) and 2 (25%) in the 750 mg/m2 group, 8 (80%) and 2 (20%) in the 1200 mg/m2 group, and 8 (100%) and 0 (0%) in the 1500 mg/m2 group. CONCLUSIONS: The dose of cyclophosphamide in biweekly CHOP can be increased up to 1500 mg/m2 with no increase in the incidence of treatment-related early mortalities without any organ damage in younger patients. The efficacy of this dose intensification of CHOP currently is being investigated in a multicenter prospective randomized trial using three different dose levels of cyclophosphamide.