[Clinical statistical data of patients with abnormal hemoglobin].
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Gene Deletion↗
Biomedical subjects
H Fujii
Publications and source records attributed to H Fujii.
Intragenic homozygous deletion of the WT1 gene in Wilms' tumor.
One example of intragenic homozygous deletion of the WT1 gene on chromosome 11p13 was found after screening 42 samples of Wilms' tumor DNA from Japanese patients. After construction of a restriction map for the genomic sequence covering the 3' half of the gene, the deletion was analysed at the nucleotide sequence level. The deletion occurred in the patient's germline on his paternal chromosome, and most of the short arm of his maternal chromosome 11 was subsequently lost in the tumor. The size of the deletion was about 8 kb, removing exons 6 and 7 and resulting in premature termination. The deletion seemed to be created by recombination between short homologous sequences found in an Alu repeat, with a 16-bp duplication left at the junction. This case conforms to a two-hit model for the genesis of a certain group of tumors, and supports the hypothesis that WT1 is one of the recessive oncogenes responsible for Wilms' tumor.
Base Sequence↗
Effects of orally administered beta-adrenergic blockers and calcium-channel blockers on the intraocular pressure of patients with treated hypertension.
Little attention has been paid to the influence of orally administered antihypertensive drugs on intraocular pressure (IOP). Therefore, we evaluated the effects of oral medications on the IOPs and visual fields of 70 patients with systemic hypertension who had no ocular symptoms and had not visited any eye hospital. In patients with systemic hypertension treated with medication, the IOP value (mean, 18.3 +/- 4.2 mmHg; age range, 63.3 +/- 11.6 years) was significantly higher than in a control group with a similar age range who were not receiving oral medication (mean, 13.7 +/- 2.0 mmHg; age range, 62.5 +/- 7.8 years). In the group in which hypertension was controlled by medication, the IOPs were lower when orally administered beta-adrenergic blockers were given than in those patients with uncontrolled hypertension. In the group to whom calcium-channel blockers were administered orally, the IOPs were not lower. Angiotensin-converting enzyme inhibitors made the visual fields worse. This study suggests a modulating influence of orally administered drugs on the IOP and visual fields, which may be affected by whether or not the patient's blood pressure is controlled.
Administration, Oral↗
Successful pancreatojejunal anastomosis for pancreatoduodenectomy.
A series of 100 patients underwent pancreatoduodenectomy for carcinoma of the head of the pancreas (36 patients), the bile duct (19 patients), the gallbladder (14 patients), the papilla of Vater (13 patients), the duodenum (seven patients), the stomach (seven patients) or the colon (one patient), or for chronic pancreatitis (two patients) and adenomyoma of the bile duct (one patient) between October 1983 and December 1990. The operative morbidity and mortality rates were 27 and 6 percent, respectively, and failure of the pancreatojejunal anastomosis was the most common post-operative complication (16 patients). Anastomotic leakage correlated significantly with the quality of the pancreatic remnant and the preoperative technique used (p < 0.01). The leakage rate in 34 patients with fibrotic pancreatic tissue was 5.8 percent (two of 34) and was significantly less than that in 66 patients with a normal pancreatic remnant (21.2 percent). Forty-seven patients underwent direct anastomosis between the main pancreatic duct and the jejunum, using mucosa to mucosa sutures and a pancreatic duct drain tube as well as a single layer of sutures between the pancreatic tissue and the jejunum. Only two patients had a pancreatic fistula (4.2 percent) and the incidence was significantly less than that in 53 patients who received the usual technique of double-layer sutures between the pancreatic remnant and the jejunum without a direct anastomosis to the main pancreatic duct (26.4 percent, p < 0.01).
Adult↗
Clinical characteristics of chronic cutaneous graft-versus-host disease in Japanese leukemia patients after bone marrow transplantation: low incidence and mild manifestations of skin lesions.
We investigated the incidence of chronic graft-versus-host disease (GVHD) and skin and oral lesions in chronic GVHD in 90 Japanese leukemia patients surviving over 100 days after bone marrow transplantation (BMT) from HLA-compatible siblings. The clinical characteristics of chronic cutaneous GVHD occurring in eight of these patients are summarized. There were some differences from previous reports in the USA and Europe. The incidence (8/29: 28%) of skin lesions in chronic GVHD was low in contrast to the previously reported high incidence (79-90%). Cutaneous manifestations in six out of eight patients with chronic GVHD tended to be mild, and lichen-planus-like eruptions in the skin and scleroderma-like lesions, which are well known as representative cutaneous manifestations of chronic GVHD, were rare. Furthermore, our statistical analysis indicated that there was no relationship between pretransplant total body irradiation and GVHD prophylaxis or acute GVHD and the onset of chronic cutaneous GVHD. The present study suggests that the differences from previous reports in the USA and Europe concerning chronic cutaneous GVHD may be attributable to differing genetic backgrounds in Japanese and western populations.
Acute Disease↗
[A case of aortitis syndrome treated by simultaneous procedure of Bentall's operation and aortic arch replacement].
The case is a 22-year-old man, in whom chest X-P pointed out aortic and cardiac shadow enlargements. Aortography revealed extensive aortic aneurysm involving the ascending, arch, and proximal descending portions of the aorta, accompanied with annulo-aortic ectasia, aortic regurgitation, and stenosis of the left common carotid and left subclavian arteries, suggesting lesions due to aortitis syndrome. For this case, Bentall's operation and replacement of the aortic arch and the proximal descending aorta were performed simultaneously, using hypothermic selective cerebral perfusion. Favorable postoperative course and absent neurological abnormality enabled the patient to return to his former social work. Steroid therapy has been continued up to the present, 5 years postoperatively, with neither recurrence of inflammation nor occurrence of cardiac murmur.
Adult↗
[A case report of two mitral valve aneurysms with one perforation after two attacks of infective endocarditis].
Aneurysms of the mitral valve complicating infective endocarditis (IE) are uncommon. The patient was a 57-year-old man who was admitted to our hospital for a precise examination of heart failure. One year before, the first two-dimensional echocardiography showed an aneurysm of the anterior mitral leaflet possibly due to a previous attack of IE. Doppler color flow mapping detected a regurgitant jet from the mitral valve aneurysm into the left atrium during systole, which suggested perforation of the aneurysm, and an aortic regurgitant jet flowing against the anterior mitral leaflet. Because the patient's family refused cardiac catheter examination and surgery, we treated him in the out-patient clinic. A few weeks before his admission, he had discontinued taking diuretics because of uncomfortable urinary frequency. He gradually developed the symptoms of heart failure and entered our hospital. IE was suspected because of leucocytosis and slight fever. Two-dimensional echocardiography revealed a new aneurysm of the anterior mitral leaflet without perforation, located in the distal part of the old aneurysm. This time, his family consented to the surgical treatment. Aortic and mitral valve replacement was successfully performed. It was pathologically confirmed that the two mitral aneurysms had been caused by IE.
Aortic Valve↗
[Primary macroglobulinemia with hypercalcemia, renal failure and systemic amyloidosis].
This report deals with an unusual case of primary macroglobulinemia with hypercalcemia, chronic renal failure and systemic amyloidosis. In May 1990, a 63-year-old male was transferred to our hospital because of hypercalcemia (13.5 mg/dl) and renal failure. Clinical examinations showed anemia, macroglossia, lymph node swellings and hepatomegaly. Laboratory findings included Bence-Jones (kappa type) proteinuria (0.8 g/day), a monoclonal gammopathy of the IgM-kappa type (2.8 g/dl), a proliferation of lymphoid cells in the peripheral blood (5%) and the bone marrow (59.6%), and lymphomatous involvement of an inguinal lymph node. Serum creatinine concentration was 8.5 mg/dl. The serum levels of parathormone and vitamin D3 metabolites were normal. The roentgenogram of bones showed a compression fracture of the lumbar spine and systemic osteoporosis. The treatment included eel calcitonin, prednisolone and the CHOP regimen, followed by hemodialysis and plasmapheresis. The serum level of IgM increased to 4.6 g/dl. The patient died three months later and postmortem examination demonstrated marked systemic amyloidosis.
Amyloidosis↗
[Measurement of blood velocity of rabbit iris by laser speckle flowgraphy].
We developed laser speckle flowgraphy which displays the relative velocity of blood microcirculation as a color map in two dimensions. We applied this method to the iris in 10 eyes of 5 normal white rabbits under general anesthesia. The coefficient of variation was small and averaged 5.6. There was no significant difference in the values measured on 3 consecutive days. The blood velocity in the iris increased significantly during experimental ocular hypotony and decreased during ocular hypertension and 2 hours after instillation of timolol (p less than 0.05). These findings indicate that the present method is of value in assessing the relative velocity of iris microcirculation in vivo and that topical instillation of timolol induces decrease of blood flow in the iris and, possibly, in the ciliary body.
Animals↗
Synchronous cancer of squamous cell carcinoma of the lung and lymphocytic leukemia (B-cell type): a case report.
The patient was an 82-year-old male who consulted us over a lymph node enlargement in the right side of his neck. The WBC was 23,700/microliters (lymphocytes 80%), and a diagnosis of chronic lymphocytic leukemia (CLL) was given based on the findings of a bone marrow puncture and lymph node biopsy. During the observation period, abnormal shadows accompanied by cavitation appeared in the right lower lung field, and CT-guided percutaneous biopsy findings indicated a squamous cell carcinoma of the lung. No metastatic lesions were noted. No aggressive surgical or internal treatments were performed in consideration of the patient's age. The patient was admitted again and died of respiratory failure due to lung carcinoma. Reports of CLL and other malignant tumors arising in one patient are rare in Japan, and those of their occurring concurrently are even rarer. In the present report, a case of synchronous cancer of squamous cell carcinoma of the lung and CLL is described, together with a review of the literature.
Aged↗
[Emergency coronary bypass surgery after failed coronary angioplasty].
Of 1,315 consecutive patients undergoing PTCA, 9 eventually underwent emergency CABG because of angioplasty failure (emergency rate 0.68%). The indication for emergency bypass was dissection with threatening occlusion in 3 patients, acute occlusion with evolving MI in 3 patients, cardiogenic shock in 1 patient, intractable cardiac arrest in 2 patients. Eight patients were taken directly to the operating room from the catheterization laboratory. The overall mortality of emergency CABG was 11%. One of the two patients with cardiac arrest died of LOS postoperatively. Two patients developed perioperative myocardial infarction (25%). The incidence of operative morbidity and mortality were significantly increased among patients with hemodynamic instability. Should PTCA failure become evident, the prompt undertaking of emergency CABG could perhaps decrease the incidence of acute MI and death.
Adult↗
[An unusual case of localized form of primary macroglobulinemia developing from a nodular primary pulmonary amyloidosis].
The authors reported here a case of primary pulmonary amyloidosis, which developed into a localized form of primary macroglobulinemia (PMG) 10 years later. A nodular shadow was pointed out on routine chest x-ray films of a 61-year-old Japanese male in 1977. In 1981, a diagnosis of nodular primary pulmonary amyloidosis was made by percutaneous lung biopsy. At that time, he suffered from signs and symptoms of chronic cold agglutinin disease (CCAD). Cold agglutinins were IgM-kappa antibodies. In 1986, serum immunoelectrophoresis demonstrated the presence of a small amount of monoclonal IgM-kappa. In 1987, the patient was readmitted because of pleural effusions. In the pleural effusion, the IgM level was 3,341 mg/dl and the titer of cold agglutinin was 32,000. Cytological examinations of pleural effusion showed the proliferation of lymphocytes, lymphoid cells and plasma cells. These cells showed the monoclonality of IgM-kappa by the peroxidase-antiperoxidase method. At post-mortem examination in March 1988, PA and PMG were found, but both lesions were localized only in the thoracic cavity. It is suggested that primary amyloidosis (PA) as well as CCAD and monoclonal gammopathy of undetermined significance may be one of the pre-neoplastic conditions of PMG.
Aged↗
[A case of schwannoma of the intrathoracic vagus nerve].
A 42-year-old female was noted to have a mediastinal mass on routine chest roentgenogram. She was asymptomatic and physical examination was unremarkable. Computed tomography of the chest confirmed the presence of a 3.5 cm well circumscribed mass in the middle mediastinum adjacent to the aortic arch. Operation was performed. A solitary middle mediastinal tumor was readily apparent at the aortic arch to the left subclavian artery, originating from the vagus nerve superior to the take-off of the recurrent laryngeal nerve. Excision was possible by enucleation of the mass. The postoperative period was uneventful, although there was paralysis of the left vocal cord as evidenced by hoarseness. Histologically the diagnosis of a schwannoma of the vagus nerve was made.
Adult↗
[Clinical and pathological study of 152 cases of prostatic cancer].
A total of 152 prostatic cancer patients who underwent mainly hormone therapy was conducted. Our histological grading system combined with structure atypsim--SAT and nuclear anaplasia--NAN allowed for more accurate prognosis of prostatic cancer patients. The prognosis of G3 patients was obviously poor. The over-all 5-year survival rate for prostatic cancer patients with G1, G2 and G3 was 80%, 57% and 17%, respectively. The 5-year reactivation rate for patients with G1, G2 and G3 was 14%, 32% and 87%, respectively. The period for reactivation after initial hormone therapy for prostatic cancer patients with G1, G2 and G3 was 11.4, 10.0 and 3.2 years, respectively. Further improvements in survival for the patients with G3 prostatic cancer will require the development of effective systemic chemotherapy and the re-consideration of appropriate use of hormone therapy.
Aged↗
[Multicenter clinical evaluation of red cell concentrates stored up to 6 weeks in MAP, a new additive solution].
The clinical safety and efficacy of transfusion of red cell concentrates stored in MAP solution (MAP-CRC) containing mannitol, adenine, glucose, phosphate and citrate, into 39 anemic patients were evaluated. In 23 patients, infusion of MAP-CRC was alternated with infusion of ordinary CRC as a control. The MAP-CRC and CRC used in this study were stored at 4 degrees C for an average of 38.2 +/- 2.6 days (n = 52) and 18.1 +/- 2.2 days (n = 26), respectively. Red cell recovery was 77.5% for MAP-CRC and 82.5% for CRC, based on calculation of the increase in hemoglobin level one day after transfusion. There were no differences between patients transfused with MAP-CRC and those transfused with CRC in clinical findings or biochemical data. No major side-effects other than pyrexia associated with the underlying infections were seen in patients transfused with MAP-CRC. MAP-CRC stored up to 42 days is apparently as safe and effective as stored CRC. This new additive solution may therefore be useful for the future expansion of the indications for autologous blood transfusion by facilitating the collection and storage of more blood in the liquid state for a longer period, and may also be useful in obtaining more plasma from whole blood as source plasma.
Adenine↗
Polymerase chain reaction (PCR) for detection of ApaI polymorphism at the insulin like growth factor II gene (IGF2).
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Asian People↗
Electron transfer reactions in the NADPH oxidase system of neutrophils--involvement of an NADPH-cytochrome c reductase in the oxidase system.
Membrane-bound NADPH oxidase of pig blood neutrophils was solubilized with heptylthioglucoside in a high yield. The solubilized preparation from myristate-stimulated cells (sample S) showed high O2- generating activity, and the preparation from resting cells (sample R) had no activity, but the two samples had equal amounts of flavins and cytochrome b-558 (cyt b-558). The electron transfer reactions to exogenous cytochrome c (cyt c) or cyt b-558 in samples S and R were examined. Under anaerobic conditions, NADPH-dependent cyt c reductase activity appeared higher in sample S than in sample R, and the addition of FMN and FAD greatly enhanced the reductase activity of sample S, but not that of sample R. No marked difference between the reductase activities of samples S and R was seen with NADH. Photoreduction of the NADPH oxidase system was examined in the absence of NADPH under anaerobic conditions by monitoring the reduction rates of exogenous cyt c using a flashlight with cut-off filters between 400 and 500 nm. Cyt c reduction was much higher in sample S than in sample R on photoexcitation at about 450 nm. Photoreduction was carried out with a band-pass filter for selective irradiation at 450 nm. Marked reduction of exogenous cyt c was observed only in sample S: the small reduction of cyt c by sample R was independent of the light wavelength and was equal to the blank level. In contrast, no difference in the reduction of cyt b-558 by the two samples was found by either NADPH or photoreduction. Under aerobic conditions, no direct reduction of either cyt c or cyt b-558 was observed. These results suggest that an NADPH-cyt c reductase (a membrane-bound flavoprotein) is involved in the NADPH oxidase system of stimulated neutrophils.
Anaerobiosis↗
Characterization of neutrophil NADPH oxidase factors p47-phox and p67-phox from recombinant baculoviruses.
Superoxide production by phagocytic blood cells involves assembly of an active NADPH oxidase complex from components found both in membrane and cytosolic locations in resting cells. We recently cloned cDNAs encoding two cytosolic components (p47-phox and p67-phox) of the oxidase that are deficient in distinct forms of autosomal recessive chronic granulomatous disease. The precise roles of p47-phox and p67-phox were explored further using purified factors produced in large quantities using recombinant baculoviruses to infect cultured Sf9 insect cells. Neither p47-phox nor p67-phox are thought to represent the flavoprotein components of the oxidase, since neither of the purified recombinant factors contained or bound FAD. Recombinant p47-phox and p67-phox are capable of restoring the deficient cytosol from chronic granulomatous disease patient neutrophils to nearly normal levels in a cell-free reconstitution system. Both p47-phox and p67-phox, used together in the absence of neutrophil cytosol, are incapable of supporting cell free production of superoxide, confirming the involvement of other soluble factor(s) in the assembly of an active oxidase in vitro.
Baculoviridae↗