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S Suita

Publications and source records attributed to S Suita.

At least 19 recordsLinked to original sources
PubMed2002

Experience with International Neuroblastoma Staging System and Pathology Classification.

The International Neuroblastoma Staging System and Pathology Classification were proposed in 1988 and in 1999, respectively, but their clinical value has not yet been fully studied in new patients. Six hundred and forty-four patients with neuroblastoma treated between January 1995 and December 1999 were analysed by these classifications. The 4-year overall survival rate of patients <12 months of age with INSS stages 1, 2A, 2B, 3 and 4S disease was 98.5%, which was significantly higher than the 73.1% rate in stage 4 patients <12 months (P<0.0001). When patients were > or = 12 months, the 4-year overall survival rate of patients with neuroblastoma at 1, 2A, 2B and 3 stages was 100% and that of patients at stage 4 was 48.5% (P<0.0001). As to the International Neuroblastoma Pathology Classification histology, the 4-year overall survival rate was 98.8% in patients with favourable histology and 60.7% in those with unfavourable histology in the <12 months group (P<0.0001). In the > or = 12 months group, the 4-year oral survival of patients with favourable histology was 95.3% and that of patients with unfavourable histology was 50.6% (P<0.0001). Among biological factors, MYCN amplification, DNA diploidy and 1p deletions were significantly associated with poor prognosis in patients <12 months, as were MYCN amplification and DNA diploidy in patients > or = 12 months of age. Multivariate analysis showed that the INSS stage (stage 4 vs other stages) and International Neuroblastoma Pathology Classification histology (unfavourable vs favourable) were significantly and independently associated with the survival of patients undergoing treatment, stratified by age, stage and MYCN amplification (P=0.0002 and P=0.0051, respectively).

Age Factors↗
PubMed2001

Mutations/deletions of the WT1 gene, loss of heterozygosity on chromosome arms 11p and 11q, chromosome ploidy and histology in Wilms' tumors in Japan.

Incidence rates of Wilms' tumor (WT) markedly differ in East Asian and Caucasian children. In the present study, we examined WT1 deletions/mutations and loss of heterozygosity (LOH) on 11p and 11q in a large number of WTs and compared our findings with those from 4 series of Caucasian WTs. Incidence rates of the subtle WT1 mutation in 3 of the 5 series of sporadic and unilateral WTs including ours were 4.3-6.2% and similar. However, gross homozygous WT1 deletion was more frequent in our series than in some others. In addition, our series tended to show a higher incidence of LOH limited to 11p13 and a lower incidence of LOH including 11p15 than the Caucasian one. These findings indicate some genetic differences in WT between the 2 regions. One of the 4 Caucasian series reported a correlation of germinal WT1 mutation with the predominantly stromal histology. The present study not only confirms the correlation of germinal WT1 deletion/mutation with predominant stromal histology but also establishes a correlation with somatic WT1 deletion/mutations with predominant stromal histology. While WTs with WT1 abnormalities usually showed pseudodiploidy and predominant stromal histology, those without WT1 abnormalities showed various chromosome numbers and histologic subtypes.

Alleles↗
PubMed2001

Clinical and biologic characteristics for recurring neuroblastoma at mass screening cases in Japan.

BACKGROUND: It is said that most cases detected by neuroblastoma mass screening at 6 months of age tend to have a favorable clinical course after a surgical resection either with or without mild chemotherapy. However, a few cases have an unfavorable outcome. In the current study, the authors analyzed the clinical and biologic characteristics for recurring neuroblastoma in mass screening cases. METHODS: In 245 cases detected through mass screening in the Kyushu area in Japan, the clinical data and biologic features (N-myc status, DNA ploidy, Shimada histology, neuron-specific enolase (NSE), ferritin) were investigated, whereas, in particular, the data for recurring cases also were analyzed. RESULTS: Of 245 cases, 28 tumors had one or more biologically unfavorable prognostic factors, and 6 patients experienced recurrence. Three of the six patients with recurring disease underwent a complete resection of the primary tumor, whereas three cases had undergone an incomplete resection of the tumor. Regarding the initial chemotherapy, three cases received mild chemotherapy, two cases received no chemotherapy, and one case had high-dose multidrug chemotherapy. Regarding biologic prognostic factors, four of six cases with recurring disease had one or more unfavorable factors, whereas two cases had no unfavorable factors. Regarding the outcome after recurrence, four cases are CR, one case has a stable residual tumor, and one case died of disease with N-myc amplification. CONCLUSIONS: Most neuroblastomas detected by mass screening at 6 months of age have biologically favorable factors. However, approximately 10% of the cases had one or more unfavorable factors and thus might have a higher risk of recurrence than the patients with no unfavorable factors. Conversely, some cases with recurring disease had no unfavorable factors; however, the reason for this is still unclear. A long-term follow-up for mass screening cases is important, and it also might be necessary to research the established biologic factors and identify other new prognostic factors.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2001

Quick quantitative analysis of gene dosages associated with prognosis in neuroblastoma.

The amplification of the N-myc gene and a gain of the chromosome 17q arm correlate with an unfavorable outcome in patients with neuroblastoma. In this study, we determined the gene dosage of the N-myc gene (located at 2p24) and Survivin gene (located at 17q25) using the p53 gene (located at 17p13) as the internal control gene by the TaqMan polymerase chain reaction (PCR)-based gene dosage analysis in 25 neuroblastoma samples. Based on the assumption that the gene dosages of each gene of a normal individual lymphocytes are 1.0, 11 of the 25 cases with a corrected gene dosage of N-myc (N-myc/p53) of more than 2.0 had a more unfavorable prognosis than the 14 cases with a N-myc gene dosage of less than 2.0 (5-year survival rate: 18 vs. 71%, P<0.01). Ten of 25 cases with a corrected Survivin gene dosage (Survivin/p53) of more than 2.0 had a more unfavorable prognosis than the 15 cases with a Survivin gene dosage of less than 2.0 (5-year survival rate: 10 vs. 67%, P<0.01). This quantitative PCR system is considered to be useful for quickly and accurately evaluating the degree of malignancy of neuroblastoma in order to select the optimal treatment.

Child↗
PubMed2001

The biological effect of phthalate esters on transabdominal migration of the testis in fetal rats in comparison with the antiandrogen flutamide.

Phthalate esters are commonly used as plasticizers for polyvinyl chloride and are known to be hormone-disrupting chemicals. We previously reported that mono-n-butyl phthalate (MBP) administered to rat fetuses induced cryptorchidism postnatally. The aim of this study was to investigate the biological effect of MBP on the transabdominal migration of the testis in prenatal rats by comparing this with the prenatal effect of the antiandrogen flutamide on testicular descent. Time-pregnant Wistar King A rats were divided into three groups: group I rats (N = 3) were administered MBP 0.3 g/day by gavage from gestational days 15 to 18; group II rats (N = 3) were injected with flutamide (30 mg/day) from gestational days 15 to 18; group III rats (N = 3) were administered solvent as controls. On the 19th gestational day, all rats underwent a cesarean section and the male fetuses were dissected to examine the position of the testis, which was significantly higher in the abdominal cavity in the MBP-treated rats than in either the flutamide-treated or control rats. No significant difference was observed in the position of the testis between the flutamide-treated and control rat fetuses. Our findings suggest that maternal MBP prevented transabdominal migration of the testis in prenatal rats, which may not have been due to either an antiandrogenic or estrogenic effect of MBP, but to a direct toxic effect of MBP on the testis.

Animals↗
PubMed2001

Funnel chest: treatment strategy and follow-up.

Although funnel chest is the most frequently seen deformity of the anterior chest wall in children, there is still considerable controversy regarding three major aspects, namely, the frequency of such deformities, their physiological importance, and the methods available for treatment. We retrospectively analyzed our experience with the 154 patients managed in our department. In 81 of these an operation was performed (OP), and the clinical findings for this group were compared with the 73 patients in whom an operation was not performed (NOP). Evaluation included subjective findings, especially the views of the patients' parents, and objective findings, including chest radiographs, computed tomography (CT), spirogram, electrocardiography, and echocardiography. In all patients the assessment included postoperative respiratory symptoms, appearance of the chest, and psychological aspects related to the deformity. Post-operatively, respiratory symptoms almost invariably subsided. The cosmetic result could initially be regarded as satisfactory or fair during the first 10 years following surgery, but over time there was frequently increasing concern regarding the scar. The NOP patients showed significantly less severity of the funnel index compared with OP patients. However, there was no spontaneous improvement in the deformity in older patients; most of the NOP patients continued to show a cosmetic deformity and 26.7% had psychological problems. This retrospective study confirms that our treatment strategy of objective criteria for operation (functional compression index > 0.2, % vital capacity <80, and CT index less than 0.25) and timing of operation (between 4 and 6 years of age) provides good results. Based on the analysis of long-term follow-up, surgery is considered indicated in patients with severe deformity. However, in the interest of psychological development, the indications for surgery may be extended.

Abnormalities, Multiple↗
PubMed2001

Mutations of the p53 gene in malignant rhabdoid tumors of soft tissue and the kidney: immunohistochemical and DNA direct sequencing analysis.

Malignant rhabdoid tumor (MRT) is characterized by the presence of intracytoplasmic eosinophilic inclusions composed of whorls of intermediate filaments. This tumor was originally described as an entity of the abortive type of Wilms' tumor in childhood. Recently, it has been proved that these rhabdoid cells can be observed in various types of malignant tumors, including soft tissue sarcoma or carcinoma. To investigate the oncogenesis of this tumor, we examined the p53 gene alteration by means of immunohistochemical analysis and DNA direct sequencing in three cases of malignant rhabdoid tumor (MRT) of the soft tissue and three cases of MRT of the kidney. All the cases of MRT of the soft tissue and two of the cases of MRT of the kidney showed immunopositivity for p53 protein. Among them, one of the cases of MRT of the soft tissue and two of the cases of MRT of the kidney showed missense mutations of the p53 gene. These results strongly suggest that p53 gene alterations may have an important role to play in the aggressive biological behavior and poor prognosis of this tumor.

Adult↗
PubMed2001

Moyamoya syndrome with spherocytosis: effect of splenectomy on strokes.

A 3-year-old male patient with hereditary spherocytosis who developed moyamoya syndrome, presenting hemiplegia, and slurred speech is reported. Transient ischemic attacks occurred repeatedly with hemolytic crises. Magnetic resonance imaging and angiography revealed bilateral occlusion of the internal carotid and middle cerebral arteries with the formation of moyamoya vessels and multiple infarctions in the basal ganglia. Although splenectomy can increase the risk of stroke, no stroke occurred after splenectomy. On aspirin and dipyridamole therapy the patient has been free of neurologic deficits and progression of the vasculopathy for 5 years. This rare observation suggests that anemic hypoxia more greatly contributes to the progression of moyamoya syndrome than postsplenectomy thrombocytosis or reduced deformability of spherocytes.

Aspirin↗
PubMed2001

The mechanism for the contraction induced by leukotriene C4 in guinea-pig taenia coli.

The mechanism underlying the LTC(4)-induced contraction of guinea-pig taenia coli was determined using the simultaneous measurements of [Ca(2+)](i) and force in whole muscle preparations. Additional experiments were performed in receptor coupled permeabilized preparation. For comparison purposes, the contraction which was induced by a typical G-protein mediated agonist, carbachol was also characterized. LTC(4) induced a contraction in the guinea-pig taenia coli in a concentration-dependent manner. The maximal response was obtained at 100 nM and the EC(50) value was 5.4+/-1.9 nM. Both LTC(4) and carbachol induced increases in [Ca(2+)](i) and force. The maximum force induced by 100 nM LTC(4) was significantly smaller than that induced by 10 microM carbachol, although an increase in [Ca(2+)](i) produced by both agonists was similar. In the permeabilized preparations, carbachol, but not LTC(4), induced an additional force development at a fixed Ca(2+) concentration. LTC(4) induced no increase in [Ca(2+)](i) and force in the Ca(2+)-free solution, while carbachol induced transient increases in both [Ca(2+)](i) and force in a Ca(2+)-free solution. Both diltiazem and SK&F 96365 significantly inhibited the LTC(4)- and carbachol-induced increases in [Ca(2+)](i) and force in normal PSS. The inhibitory pattern of [Ca(2+)](i) by these drugs was also similar. We thus conclude that LTC(4) induces the contraction of the guinea-pig taenia coli mainly through Ca(2+) influx via both the diltiazem-sensitive and SK&F 96365-sensitive Ca(2+) channels, without affecting either the Ca(2+)-sensitivity or the intracellular Ca(2+) release. These results indicated that the mechanism underlying the LTC(4)-induced contraction differs greatly from that for conventional G-protein mediated agonists, such as carbachol.

Animals↗
PubMed2001

Fourteen-year experience of acetylcholinesterase staining for rectal mucosal biopsy in neonatal Hirschsprung's disease.

BACKGROUND/PURPOSE: Acetylcholinesterase (AChE) staining of rectal mucosal biopsy specimens is the most important and popular examination for making a definite diagnosis of Hirschsprung's disease. This examination often is performed for patients with constipation in the daily clinic. The results of this examination are reflected immediately in the treatment. However, the authors sometimes encountered difficult cases to diagnose, especially in neonates. Therefore, a retrospective investigation was conducted on the benefits and problems of AChE staining of rectal mucosal biopsy specimens in neonates. METHODS: The authors encountered 459 cases (91 neonates) of suspected Hirschsprung's disease, clinically, from April 1986 to March 2000. Mucosal specimens were taken by punch biopsies. Samples were stained by the modified Karnovsky Roots method using rubeanic acid as an amplifier and immediately examined with a light microscope. These results were collected and assessed mainly on neonatal cases. The authors also analyzed the 104 cases of Hirschsprung's disease diagnosed in patients less than 1 year of age to evaluate the relationship between the grade of proliferation of AChE positive fiber and age. RESULTS: Forty-one neonatal cases of Hirschsprung's disease were diagnosed based on the findings of AChE staining. A definite diagnosis of Hirschsprung's disease was confirmed based on the pathologic findings of operative samples. Forty-eight cases that were diagnosed as normal included 4 cases that turned out to be false-negative (3 Hirschspurung's disease cases and 1 case of an allied disorder of Hirschsprung's disease). There were no major complications in mucosal punch biopsy. In the cases of Hirschsprung's disease diagnosed in a patient less than 1 year of age, the grade of AChE-positive fiber tended to increase with the aging of patients. CONCLUSIONS: The specificity of AChE staining was high (100%), but its sensitivity was slightly low (91%). Careful long-term follow-up is required for any cases diagnosed as normal. Mucosal biopsies should be repeated in cases of persistent clinical symptoms.

Acetylcholinesterase↗
PubMed2001

Hazards of hepatic portocholecystostomy in biliary atresia.

A hepatic portocholecystostomy (HPC) has been recommended to avoid postoperative cholangitis in the case of a patent distal extrahepatic bile duct (PDEBD) for the treatment of biliary atresia (BA). We investigated the efficacy and clinical problems of HPC in BA. The clinical records of eight patients with BA and PDEBD were reviewed. The diameter of the common bile duct was compared between the patients with BA and PDEBD and age-matched patients with neonatal hepatitis (NH). Five of 8 patients with PDEBD underwent HPC. One patient had to be converted to a cholecystojejunostomy because of common bile duct stenosis at 19 years of age. The other two patients underwent a reoperation by a hepatic portojejunostomy due to poor bile drainage after HPC. Another patient became jaundice-free one month after HPC, but died of sepsis due to bile leakage 3 months thereafter. The mean diameter of the common bile duct in BA with PDEBD was significantly smaller than that of NH (0.76 +/- 0.16 mm (n = 8) in BA vs. 1.90 +/- 0.39 mm (n = 11) in NH, p<0.01). HPC was thus found to be an excellent operative method for preventing postoperative cholangitis in BA, however, many clinical problems still need to be overcome for such a narrow distal duct.

Biliary Atresia↗
PubMed2001

Frequent deletions and mutations of the beta-catenin gene are associated with overexpression of cyclin D1 and fibronectin and poorly differentiated histology in childhood hepatoblastoma.

Hepatoblastoma (HBL) is the most common malignant liver tumor in young children. Recent reports have shown that the beta-catenin gene was frequently mutated or deleted in HBLS: To elucidate the role of beta-catenin abnormalities in HBLs, we searched for mutations of beta-catenin and APC as well as expression of the target genes, cyclin D1, c-myc, and fibronectin, in 68 primary HBLS: The mutation analysis revealed that 44 (65%) tumors carried missense mutations or deletions of beta-catenin, all of which were somatic and targeted to the exon 3 encoding the amino acid residues involved in its degradation. However, no loss of function mutation of the APC gene was detected by the yeast functional assay. Of interest, beta-catenin mutation was significantly correlated with overexpression of the target genes, cyclin D1 and fibronectin, but not with that of c-myc in HBLs as measured by quantitative real-time reverse transcription-PCR. The immunohistochemical studies in 15 HBLs demonstrated that the nuclear/cytoplasmic accumulation of beta-catenin was positive in 13 tumors, 9 of which had the deletion or mutation of the gene. The significant correlation between the beta-catenin gene abnormality and the positive staining of cyclin D1 was also confirmed. Furthermore, the nuclear accumulation of beta-catenin was strongly associated with the poorly differentiated tumor cell components as well as with the positive staining of cyclin D1 within the tumor. Thus, our present results suggested that the gain of function mutation of beta-catenin played a crucial role in the malignant progression of HBL in vivo.

Adenomatous Polyposis Coli Protein↗