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Biomedical subjects

N Usui

Publications and source records attributed to N Usui.

At least 55 records · Page 3Linked to original sources

[MALT lymphoma of the larynx].

We describe a 25-year-old Japanese woman with a MALT-type lymphoma of the larynx. She presented with a one-year history of hoarseness and increasing pain in the larynx. A small tumor was found on the left side of the false cord, and was biopsied under laryngoscopy in the department of laryngology. Histological examination showed the presence of centrocyte-like cells infiltrating the submucosa and forming lymphoepithelial lesions. The neoplastic cells were CD20+, CD79a+, and CD5-. Staining for keratin with CAM 5.2 highlighted the infiltrated epithelium. Analysis of DNA extracted from the biopsy specimen showed a clonal immunoglobulin heavy chain gene rearrangement, confirming the histological diagnosis of extranodal marginal zone B-cell lymphoma of the MALT type. To our knowledge, only 6 cases of MALT lymphoma of the larynx have been reported previously. The presence of MALT lymphomas arising at rare sites emphasizes the importance of accurate diagnosis and appropriate clinical management. Patients require careful periodic evaluation in order to time the therapy appropriately, and to avoid overtreatment and complications of therapy, including secondary malignancies.

Adult↗

Severe aplastic anemia associated with chronic natural killer cell lymphocytosis.

Chronic natural killer (NK) lymphocytosis involves a persistent increase in CD56+ large granular lymphocytes (LGLs) that is sometimes associated with immune-mediated complications, such as anemia and neutropenia. However, aplastic anemia (AA) is a rare complication. Here we describe 2 patients with severe AA who presented with persistent increases in NK cells. Their LGLs were positive for CD56, CD16, and intracellular interferon (IFN)-gamma but negative for CD3, Fas-ligand, and T-cell receptor rearrangement, findings that are compatible with NK cells. Not only the number of NK cells, but NK activity as well, was increased in both patients. The number of NK cells changed according to hematologic recovery and relapse in 1 case. Thus, there seemed to be a close relationship between NK cells and the progression of AA, at least in this instance. Further investigation of the clinical course of similar cases and the characteristics of NK cells is necessary.

Aged↗

The effect of phospholipids and mucin on bacterial internalization in an enterocyte-cell culture model.

A primary component of the intestinal mucous layer that functions as a barrier to luminal bacteria is mucin, a high-molecular-weight glucoprotein. In addition, the mucous layer also contains other important elements such as phospholipids (PLs), which may effect bacterial translocation (BTL). It has been reported that mucin inhibits Escherichia coli translocation; however, the effect of PLs on intestinal permeability is still controversial. We have recently reported that the concentration of mucous PLs is higher in neonatal as compared to adult rabbits. The functional significance of these biochemical differences on BTL remains to be determined. The aim of this study was to evaluate the effect of PL and mucin composition on BTL in a human enterocyte-cell culture model. Human enterocyte Caco-2 cells were seeded in 24-well tissue-culture plates and grown for 14 days to confluence. The monolayers were pretreated with phosphate buffered saline as control, 10 mg/ml or 20 mg/ml mucin, 0. 5 mM or 1.0 mM PL mixture based on neonatal (NPL) and adult (APL) composition, and 10 mg/ml mucin with 0.5 mM either APL or NPL mixtures 30 min before a 120-min incubation period at 37 degrees C with 10(8) colony forming units (CFU) of E. coli C25. Non-internalized bacteria were killed by the addition of gentamicin. Internalized bacteria were quantified by counting CFU from enterocyte-cell lysates grown on MacConkey's agar. Mucin inhibited bacterial internalization, while both compositions of PLs promoted it. Mucin added to the PL solution also diminished the stimulatory effect of PLs on bacterial internalization. These results indicate that the increased concentration of PLs found in the intestinal mucous layer of neonates, and/or the alteration in the balance between PLs and mucin, may play a role in the increased BTL seen in neonates.

Animals↗

The effect of mucin on bacterial translocation in I-407 fetal and Caco-2 adult enterocyte cultured cell lines.

Although the intestinal mucosa forms a crucial barrier between the host and the environment, bacterial translocation (BT) occurs frequently in neonates and may be a source of sepsis. The intestinal mucous gel layer is thought to be a vital component of the gut barrier and is composed, in part, of a family of glycoproteins known as mucins. Our aim was to study the effects of mucin on BT in an enterocyte cell-culture model using a fetal (I-407) and an adult (Caco-2) intestinal cell line. I-407 and Caco-2 cells were grown to confluence on porous filters in a two-chamber Transwell system. The integrity of the monolayers was confirmed by transepithelial electrical resistance (TEER) and permeability using the macromolecule dextran blue. Cells were treated with mucin (40 mg/ml) prior to inoculation of 1 x 10(6) Escherichia coli C25. The magnitude of BT was determined quantitatively by culturing the samples from the basal chamber of the wells and was expressed as log 10 [Colony Forming Units (CFU)/ml]. Statistical analysis was performed by the Mann-Whitney U test with statistical significance at P < 0.05. Mucin inhibited BT across both fetal and adult cultured enterocyte monolayers; however, the inhibitory effect was less on the fetal cells compared to the adult cells. Dextran-blue studies showed that monolayers were intact throughout the experiments. Despite 98% inhibition of BT, mucin had a statistically significant effect on post-bacterial inoculation TEER in Caco-2 cells and no effect in I-407 cells. The ability of mucin, a mucous-barrier glycoprotein, to inhibit BT across immature intestinal enterocytes, as in the neonate, may be diminished compared to mature adult enterocytes.

Adult↗

Clinical significance of the lung/thorax transverse-area ratio in fetuses with cystic lung disease.

We examined the clinical significance of the lung-thorax transverse-area ratio (L/T) in fetuses with cystic lung disease (CLD). The transverse section of the thorax was analyzed at the level of the 4-chamber view of the heart. L/T was calculated as the bilateral normal lung area divided by the thoracic area. Within a 9-year period, ultrasonography was performed in 15 fetuses with CLD, 13 of which were histologically diagnosed: congenital cystic adenomatoid malformation of the lung (CAM; n = 8), pulmonary sequestration (n = 4), and bronchial atresia (n = 1). Although the initial L/T measurement (29.6 +/- 4.2 weeks) showed significantly lower values than in the controls, the final measurement (36.4 +/- 1. 6 weeks) indicated that the L/T in CLD excluding CAM was significantly higher than that in CAM. Six patients with a final L/T of less than 0.21 required mechanical ventilation immediately after birth. Two of these patients died, and the autopsy findings indicated pulmonary hypoplasia. In these cases, a significant correlation was observed between the maximal value of L/T and preductal alveolar-arterial oxygen difference (A-aDO(2)), although the final L/T was not correlated to A-aDO(2). These results indicate that serial measurements of L/T may be useful in the diagnosis of CLD and may help to predict postnatal respiratory conditions.

Bronchopulmonary Sequestration↗

[Acute vincristine neurotoxicity in a non-Hodgkin's lymphoma patient with Charcot-Marie-Tooth disease].

A 44-year-old, previously healthy man with a diagnosis of non-Hodgkin's lymphoma (NHL, diffuse large B-cell type, stage IIA) was treated with combination chemotherapy including vincristine (VCR). After receiving a cumulative dose of VCR, he experienced rapid and marked weakening which progressed to quadriplegia and bulbar palsy. Prior to this therapy, the patient had no neurological problems, and his siblings were asymptomatic. Physical examination identified pes cavus (hollow foot), and electrodiagnostic studies showed markedly slower nerve conduction velocity of myelinated fibers, with abundant "onion bulb" formations. Chromosomal analysis detected 17p11.2-12 duplication, thus yielding a diagnosis of Charcot-Marie-Tooth (CMT) 1A. CMT disease is a familial neuromuscular disorder, and the incidence is approximately 1 in 2,500. We concluded that if CMT disease is diagnosed, vincristine should be avoided due to the potential severity of neurotoxicity to small doses.

Adult↗

Prolonged preoperative stabilization using high-frequency oscillatory ventilation does not improve the outcome in neonates with congenital diaphragmatic hernia.

Although delayed repair with preoperative stabilization using high-frequency oscillatory ventilation (HFOV) has been advocated in neonates with congenital diaphragmatic hernia (CDH), improved survival has not been reported. We compared survival between neonates undergoing delayed repair after prolonged stabilization for more than 48 h using HFOV and those undergoing immediate repair following short stabilization using HFOV in subjects with high-risk CDH. Patient selection was made by two criteria: fetuses with the lung/thorax transverse area ratio (L/T) below 0.26 and neonates who presented with respiratory distress significant enough to require intubation within 6 h after delivery. Selection of the strategies differed by era and medical center. Stabilization failed in 11 of 18 patients with delayed repair and only 7 patients (38.9%) in this group survived, but 18 of 23 patients (78.3%) in the immediate-repair group survived (P < 0.05). Although lower values of Apgar scores, best postductal PO2 (BPtDPO2), gestational weeks, and L/T and more frequent patch repair were observed in the delayed than in the immediate repair group, significant differences in survival among the subpopulations were also observed in the prenatally diagnosed group, including the group with L/T < 0.15 and the group that required ECMO, the group with the best preductal PO2 (BPrDPO2) >/= 100 torr, and the group with BPtDPO2 < 100 torr. These results indicate that prolonged stabilization for more than 48 h using HFOV does not improve survival in patients with high-risk CDH.

Combined Modality Therapy↗

Successful laparoscopic cholecystectomy in a child with upper transverse scarring: report of a case.

Laparoscopic cholecystectomy (LC) was safely performed for cholelithiasis in a 4-year-old boy who had a long transverse operative scar in the upper abdomen as a result of intestinal surgery performed during the neonatal period. The adhesions beneath the scar were sharply divided and sometimes coagulated, and additional working ports were subsequently placed as the adhesiolysis proceeded. LC was performed in the usual fashion using 5-mm titanium clips, and his postoperative course was uneventful. This case report serves to demonstrate that laparoscopic surgery is feasible even for pediatric patients who have undergone previous major intraabdominal surgery.

Child, Preschool↗

Decondensation of the mouse sperm nucleus within the interphase nucleus.

Sperm nuclei incorporated into the cytoplasm (ooplasm) of fertilised mouse eggs at the pronuclear stage remain condensed, whereas those injected into male or female pronuclei decondense. Similarly, sperm nuclei injected into germinal vesicles of immature oocytes or the nuclei of 2-cell embryos decondense, while those entering the cytoplasm of these oocytes/embryos do not. These facts seem to suggest that factors necessary for the decondensation of sperm nucleus are present in interphase nuclei and are released into the ooplasm during nuclear envelope breakdown. Nucleoplasmin, which is synthesised in the cytoplasm and accumulated within the nucleus, is likely a major candidate for these factors.

Animals↗

Startle epilepsy with infantile hemiplegia: report of two cases improved by surgery.

PURPOSE: To study the effectiveness of surgical therapy on 2 patients with startle epilepsy with infantile brain damage (SEIBD), a rare but distinctive epileptic syndrome characterized by motor seizures resistant to antiepileptic drugs (AEDs). METHODS: The patients with SEIBD both had hemiplegia, due to gross contralateral hemispheric lesions, and suffered from tonic postural seizures, frequently provoked by sudden unexpected somatosensory stimuli on the paretic side of the body. These attacks occasionally caused the patients to drop to the floor, and mild-to-moderate injuries had been sustained; they were resistant to all currently available AEDs. Consequently, these daily drop attacks severely restricted the patients' social lives and school participation. Both patients underwent corpus callosotomy and resection of epileptogenic premotor and supplementary motor lesions. One patient also underwent additional multiple subpial transections of the primary sensorimotor area. RESULTS: Seizures, as well as quality of life, improved dramatically in both patients after surgery. CONCLUSIONS: Startle epilepsy with infantile hemiplegia is a distinct epileptic syndrome characterized by structural brain damage restricted primarily to one hemisphere, large ipsilateral epileptogenic lesions involving the perisensorimotor area, refractory startle-provoked drop attacks, and a good response to epilepsy surgery.

Adolescent↗

Nephrotic syndrome associated with diffuse mesangial hypercellularity: is it a heterogeneous disease entity?

Diffuse mesangial hypercellularity (DMH) is a rare primary mesangial proliferative glomerulonephritis associated with idiopathic nephrotic syndrome (INS). We conducted this study on 15 patients, including 5 patients with repeated specimens, with a follow-up of 0.9-17.5 years and evaluated the clinical course and pathological findings. Seven patients were male. Ten patients were under 14 years of age. All specimens had INS and were diagnosed morphologically with primary diffuse mesangial proliferative glomerulonephritis at initial biopsy; 4 were diagnosed with focal segmental glomerulosclerosis (FSGS) within 3 years by the second biopsy. The remaining 11 patients included 8 initial responders and 3 initial nonresponders to 8 weeks' steroid therapy and had the histologic variant of the minimal-change nephrotic syndrome (MCNS). Ten of the 11 patients had normal renal function during the investigation period. One patient with the MCNS variant who was refractory to steroid therapy developed end-stage renal disease (ESRD) within 6 years. Four patient with the histologic variant of FSGS included 1 initial responder, 2 late responders, and 1 steroid-refractory case. One patient with the FSGS variant developed ESRD within 4 years. The follow-up biopsies documented that the severity of mesangial hypercellularity was associated with the severity of proteinuria or hematuria. We conclude that DMH may be divided into heterogeneous disease entities, whereas morphologic changes in initial biopsies were similar. Each variant as well as the degree of DMH should be recognized routinely by follow-up biopsy, because they are prognostic indicators.

Adolescent↗

Role of daunorubicin in the induction therapy for adult acute myeloid leukemia.

PURPOSE: To evaluate the relationship of total-dose of daunorubicin (DNR) to the induction therapy and treatment outcome, we have administered individualized doses of DNR during induction treatment to patients with acute myelogenous leukemia (AML). PATIENTS AND METHODS: Ninety-two previously untreated adult patients with AML who entered our hospital were analyzed for the dose of DNR required to achieve complete remission (CR), the CR rate, disease-free survival (DFS), and overall survival (OS). Induction therapy consisted of DNR 40 mg/m2 daily intravenously from day 1 until the marrow was hypoplastic, cytarabine (Ara-C), prednisolone (PRD), and/or 6-thioguanine (6-TG). RESULTS: Eighty-three of 92 patients with adult AML were assessable for this study. Sixty-three (76%) patients achieved CR. Fifty-two of 63 CR patients achieved the CR in the first course of induction therapy, and 11 patients required the second course of induction therapy. The 5-year and 10-year DFS rates were 31.2% and 5-year and 10-year OS rates were 45.1% and 42.3%, respectively. The median total dose of DNR in the induction therapy was 280 mg/m2 (120 to 480 mg/m2). DNR dose did not influence the response to therapy and was not influenced by the initial WBC count or French-American-British (FAB) system classification. CONCLUSION: These results indicated that when the dose was linked to observed tumor response, the optimal dose of DNR in the induction therapy was approximately 280 mg/m2 (40 mg/m2 for 7 days), which is greater than the conventional dose of 40 to 60 mg/m2 for 3 days.

Adolescent↗

[Evaluation of nasal obstruction with acoustic rhinometry: a simulated study with a nasal model].

The measurement of nasal resistance is important for understanding the pathophysiology of nasal obstruction. However, it is difficult to define the normal range of nasal resistance because of various physiological factors. Several authors have reported that nasal resistance is not correlated with the sensation of nasal obstruction. On the other hand, acoustic rhinometry was introduced by Hilberg et. al. (1989) to determine the geometry of the nasal cavity. The method based on sound reflection analysis provides an estimate of the cross-sectinal area of the nasal cavity as a function of the distance from the nostril. Recently, the measurement by acoustic rhinometry is becoming popular also in our country. However, the assessment by acoustic rhinometry may be different from that by measuring nasal resistance because the cross-sectional area is measured without studying the air flow. The aim of this study was to investigate the benefit of acoustic rhinometry using a nasal model. First, 4 pieces of nasal model LM005 (KOKEN, Co., Japan) made of silicone were coated with resinous putty. Then, the changes in the area-distance curve were measured with an acoustic rhinometer RHIN 2100 (SRE, Co., Denmark) in which this putty was scraped off gradually from anterior to posterior. In the next study, the quality of coated putty was increased at three points of the nasal area: anterior part, middle part, and posterior part. Thereafter the changes in the area-distance curve were observed in the same way. As a result, neither a decrease or an increase in the putty changed the area-distance curve at the posterior part. Therefore, it is suitable that the degree of the changing of nasal mucosa is defined as the change in the nasal volume between the top the I-notch and the end of the C-notch. In conclusion, acoustic rhinometry is useful to investigate nasal obstruction, especially the change in the nasal mucosa. Therefore, further clinical study is required for the different purpose of measuring nasal resistance.

Airway Resistance↗

[Cytomegalovirus retinitis].

Cytomegalovirus (CMV) retinitis is the most common intraocular infection in patients with Acquired immunodeficiency syndrome (AIDS). Approximately 25% of patients with AIDS will develop CMV retinitis during their life time. It is generally associated with CD4+ lymphocyte counts of less than 50 cells/microliter. Clinically, the most characteristic feature of CMV retinitis is a yellow-white lesion of retinal necrosis with a granular border extending into the surrounding retina. At present, ganciclovir and foscarnet have been approved for the treatment of CMV retinitis in patients with AIDS. Although both drugs arrest the progression of retinitis, relapse often occurs. More than ever, ophthalmologists have a special obligation to alleviate both the physical and psychological suffering of patients with CMV retinitis.

AIDS-Related Opportunistic Infections↗

[EPOCH therapy for relapsed/refractory lymphoid malignancies].

Patients with refractory or relapsed non-Hodgkin's lymphoma (NHL), acute T-cell leukemia (ATL), ATL lymphoma and acute lymphocytic leukemia (ALL) received EPOCH therapy. All were previously treated with doxorubicin (DOX), vincristine (VCR) and other drugs. The EPOCH treatment schedule is consisted with DOX (10 mg/M2/day, 5 days c.i.v.), VCR (0.4 mg/M2/day, 4 days c.i.v.), etoposide (50 mg/M2/day, 4 days c.i.v.), cyclophosphamide (750 mg/M2/day, day 6 i.v.) and prednisolone (60 mg/M2/day, 5 days p.o.). Twenty-one patients (ALL:10, NHL:8, ATLL:2, ATL:1) were assessable for response and toxicity. Two patients with ALL and NHL, respectively, achieved a complete remission and 3 patients obtained partial remission (NHL:2, ATLL:1). The hematological toxicity (grade > 1) included neutoropenia, anemia and thrombocytopenia, which were observed in 83.3%, 76.7% and 76.7% respectively, of total 30 EPOCH courses. The major non-hematological toxicities were nausea/vomiting, constipation and infection, but most of the toxicity were tolerable with sufficient clinical supportive care. These results indicate that continuous infusion of DOX, VCR and ETP might be effective in patients who were treated with, and presumed to be resistant to the same drugs administrated by bolus infusion.

Adolescent↗

[Primary cardiac non-Hodgkin's lymphoma].

A 69-year-old man who initially presented with lumbago developed heart failure during an MRI scan on the day of admission. A chest X-ray showed cardiomegaly and bilateral pleural effusion. Echocardiogram and computed tomography (CT) scan of the chest revealed a large tumor mass encompassing the heart with much pericardial effusion was demonstrated. The cytology of the effusion obtained by pericardiocentesis was consistent with non-Hodgkin's lymphoma, diffuse large B cell type. As CT scans of the abdomen and pelvis were negative, he was considered to have primary cardiac lymphoma. Although he responded remarkably to therapy with vincristine, cyclophosphamide and prednisolone and, he developed acute respiratory failure on the seventh month after admission. Although incidence of primary cardiac lymphoma is very low, it is necessary to investigate the mechanism of this disease in order to establish appropriate diagnostic and therapeutic approaches.

Aged↗