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Biomedical subjects

N Kanazawa

Publications and source records attributed to N Kanazawa.

At least 37 records · Page 2Linked to original sources
PubMed2000

5q- syndrome presenting chronic myeloproliferative disorders-like manifestation: a case report.

A 28-year-old Japanese woman with suspected essential thrombocythemia (ET) had marked thrombocytosis, mild leukocytosis with normal neutrophil alkaline phosphatase activity, and no anemia. She was monitored without being given any medication. Eleven years later, complete blood counts showed no remarkable changes but some non-lobulated mononuclear megakaryocytes were found in the bone marrow. Cytogenetic analysis revealed deletion of the long arm of chromosome 5 (5q-). Subsequently, hemoglobin and platelet counts decreased gradually, splenomegaly appeared and progressed, after which myelofibrosis developed. Acute leukemia developed 16 years after the first documentation of thrombocytosis. 5q- syndrome is known to be a myelodysplastic syndrome (MDS) with unique clinical features and cases with this syndrome presenting with thrombocytosis of more than 1,000 x 10(9)/L but without anemia are rare. Furthermore, it is noteworthy that in this patient transition to acute leukemia occurred following development of myelofibrosis and marked splenomegaly, which are generally observed in blastic crises resulting from chronic myeloproliferative disorders (CMPD). The patient showed features indicative of CMPD rather than of MDS in spite of presenting with 5q- chromosomal abnormality. This case supports the concept of "mixed myelodysplastic and myeloproliferative syndromes" and suggests the possibility of the appearance of CMPD-like manifestations in 5q- syndrome.

Adult↗
PubMed2000

Successful surgical treatment for implanted intraperitoneal metastases of hepatocellular carcinoma.

We report here two patients with hepatocellular carcinoma who experienced implanted metastases in the abdominal cavity after hepatectomy or microwave coagulo-necrotic therapy. Hepatic resection and microwave coagulo-necrotic therapy were successful for these tumors, and the postoperative status was satisfactory in both patients. Implanted metastases were discovered in the abdominal cavity of each of these two patients 6 months after surgery. It is necessary to look not only for the presence of liver metastasis but also for the recurrence of the tumor in the abdominal cavity during the follow-up period. Generally, surgical resection for intraabdominal implanted tumors arising from any other abdominal organs is not indicated for improving the patient's quality of life. However, resection of metastatic tumors that occur in the abdominal cavity, arising from hepatocellular carcinoma may be of value in improving patient survival.

Aged↗
PubMed2000

Resectable carcinoma of the pancreatic head developing 7 years and 4 months after distal pancreatectomy for carcinoma of the pancreatic tail.

A 67-year-old woman was referred with an abnormal finding on an abdominal echogram but presented with no symptoms; a pancreatic tail tumor was detected by ultrasonography. Biochemical examinations showed slight elevation of serum carcinoembryonic antigen level. The lesion was resected by tail and body pancreatectomy and her postoperative course was uneventful. Seven years and 4 months after the initial operation, however, her serum level of carbohydrate antigen 19-9 was found to be elevated, and a recurrence of pancreatic cancer was suspected. Examinations revealed a mass in the head of the remnant pancreas. The lesion was radically resected by total remnant pancreatectomy. Histological examinations showed that the initial tumor was a well differentiated tubular adenocarcinoma, while the second tumor was characterized as a moderately differentiated tubular adenocarcinoma. The surgical margins of the distal pancreatectomy specimen were free of atypical cells. Therefore, the position of the second lesion diminished the likelihood that it had developed by intrapancreatic metastasis. This suggests that the second carcinoma in the head of the pancreas may have been a second primary lesion.

Adenocarcinoma↗
PubMed2000

Insulinoma occurring in association with fatty replacement of unknown etiology in the pancreas: report of a case.

A 66-year-old woman with a 10-year-history of diabetes mellitus was admitted to our hospital for investigation of several recent attacks of hypoglycemia. Her fasting blood glucose level was very low, at 30-40 mg/dl, and abdominal ultrasonography and computed tomography revealed a tumor in the pancreatic tail with fatty changes. Endoscopic retrograde cholangiopancreatography revealed absence of the main pancreatic duct from the body to tail of the pancreas. Abdominal angiography showed a hypervascular tumor stain in the pancreas, and percutaneous transhepatic portal vein sampling demonstrated a step-up of immunoreactive insulin levels in the splenic vein. Based on these clinical findings, we made a preoperative diagnosis of an insulinoma accompanied by fatty changes in the pancreatic body and tail. During laparotomy for the insulinoma, fat tissue was identified in the anatomic location of the pancreatic body and tail, and resected. Pathological examination of the resected specimen revealed a number of Langerhans islets in the adipose tissue, and an islet cell tumor with fatty replacement of the pancreatic tissue around the tumor. The insulinoma was found not to have caused obstruction of the main pancreatic duct. We present herein a rare case of an insulinoma that developed in the pancreas, and was associated with fatty replacement of unknown etiology.

Adipose Tissue↗
PubMed2000

Frequent mutations in Japanese patients with acid maltase deficiency.

We screened 22 Japanese patients with acid maltase deficiency (seven with the infantile type, eight with the juvenile type and seven with the adult type) for three previously described mutations, D645E, S529V and R672Q, and a novel mutation, R600C. Although D645E has been reported to be common in Chinese patients with the infantile type, only three of 44 alleles (two of 14 infantile type alleles) from Japanese patients harbored the D645E mutation. The S529V mutation was identified in six of 14 alleles from adult-onset patients. None of the infantile or juvenile patients harbored the S529V mutation. Therefore, S529V apparently results in the adult type disease and is common in Japanese adult-onset patients. R672Q was identified in two pairs of siblings with the juvenile type. A novel mutation, R600C, was identified in eight of 22 patients (nine of 44 alleles). Therefore, R600C is another common Japanese mutation occurring at a CpG dinucleotide "hot spot". Homozygosity for this mutation apparently results in the infantile phenotype. Genetic diagnosis by detecting these four mutations might be feasible for most Japanese patients with acid maltase deficiency.

Adolescent↗
PubMed2000

Three novel mutations (G27E, insAAC, R179X) in the ORNT1 gene of Japanese patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.

Hyperornithinemia, hyperammonemia and homocitrullinuria (HHH) syndrome presents with various neurological symptoms, including mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia, and episodic disturbance of consciousness or coma caused by hyperammonemia. We report three novel mutations in the mitochondrial ornithine transporter gene (ORNT1) of Japanese patients with HHH syndrome: a nonsense mutation (R179X) associated with exon skipping and a frameshift, a missense mutation (G27E), and an insertion of AAC between codons 228 and 229, leading to an insertion of the amino acid Asn. The ORNT1 gene consists of at least six exons, and all exon-intron junction sequences conform to the GT/AG rule. All 3 patients were homozygous for their respective mutations. This study confirms that defects in the ORNT1 gene cause the HHH syndrome and that the genetic basis in Japanese patients is heterogeneous.

Amino Acid Metabolism, Inborn Errors↗
PubMed1999

Fractalkine and macrophage-derived chemokine: T cell-attracting chemokines expressed in T cell area dendritic cells.

Dendritic cells (DC) are a system of antigen-presenting cells specialized in interaction with T cells. Recently it has been reported that DC can produce CC (beta) chemokines that attract T cells. In this study we isolated mouse fractalkine and macrophage-derived chemokine (MDC) belonging to CX3C (delta) and CC chemokine families, respectively, from bone marrow-derived mature DC. While expression of fractalkine, which has so far been only examined in the brain and in vitro endothelial cells so far, was rather ubiquitous, MDC, which has been reported to be synthesized by macrophages and DC, was expressed specifically in the thymus and lymph node. This is the first report that indicates fractalkine expression by DC. Expression of fractalkine and MDC mRNA increased with maturation of DC during in vitro culture of bone marrow cells. Spleen- and epidermis-derived mature DC in culture also expressed these chemokines. Furthermore, their expression was detected selectively by Northern hybridization in CD11c+ B220- DC freshly purified from lymph nodes, and in large stellate cells in the lymph node T cell areas by in situ hybridization. Conditioned media of 293T cells transfected with these chemokine cDNA were chemotactic to Con A-activated splenic T cells as well as the mouse T cell line EL4. In conclusion, while fractalkine and MDC belong to different families of chemokines, both may be involved in recruitment of T cells for interaction with mature DC in the immune response.

Animals↗
PubMed1999

Primary malignant hemangiopericytoma of the breast: report of a case.

A 70-year-old woman presented with a huge tumor in her left breast, which had rapidly increased in size causing cutaneous erosion. She underwent a simple mastectomy, at which time the tumor was found to be an encapsulated multilocular cystic lesion containing dark fluid. Histological examination disclosed the characteristic features of malignant hemangiopericytoma, including the perivascular lamellar growth of atypical mesenchymal cells with vimentin-positive cytoplasm. Multiple irregular vascular luminal formation was also conspicuous. The characteristics of this lesion are discussed together with a review of the previous literature on this unusual neoplasm.

Aged↗
PubMed1999

A case of Turner's syndrome complicated with desmoid tumor of the transverse colon.

The association of Turner's syndrome and endometrial carcinoma has been previously established, but has never been described in conjunction with a desmoid tumor of the colon. A case is described of a colonic desmoid tumor developing in a 38-year-old female with Turner's syndrome. The association has not been previously reported. In this report, we describe a 38-year-old woman who has Turner's syndrome with a colonic desmoid and review the literature.

Adolescent↗
PubMed1999

Evaluation of curability and prediction of prognosis after surgical treatment for hepatocellular carcinoma by lens culinaris agglutinin-reactive alpha-fetoprotein.

The clinical significance of serum lens culinaris agglutinin-reactive alpha-fetoprotein (AFP-L3), which can distinguish between hepatocellular carcinoma and hepatitis by detecting a sugar chain micro heterogeneity, was evaluated for its possible ability to recognize previously undetectable residual tumors, and for increasing the accuracy of prognosis after surgical treatment for hepatocellular carcinoma. Serum lens culinaris agglutinin-reactive alpha-fetoprotein was measured pre- and post-operatively in 130 patients who underwent curative surgical treatment for hepatocellular carcinoma. The preoperative AFP-L3 positive rate was 35.4%. AFP-L3 remained positive postoperatively in 28 of the 46 preoperative AFP-L3 positive patients, and converted to positive in 4 of the 84 preoperative AFP-L3 negative patients. Regardless to preoperative AFP-L3, the postoperative AFP-L3 positive patients had a poorer recurrence-free rate (p<0.0001). The postoperative L3 positive patients had a high incidence of recurrence due to metastasis, but did not have recurrence due to multicentric origin. Multivariate analysis revealed that AFP-L3 (p<0.0001) was the most independently significant factor for predicting survival after surgery among several conventional prognostic factors. Thus, AFP-L3 is a valuable marker for evaluation of curability of surgical treatment and for improving the accuracy of prognosis.

Aged↗
PubMed1999

[The characteristics of myocardial fatty acid metabolism in patients with left ventricular hypertrophy].

We evaluated the characteristics of myocardial fatty acid metabolism in patients with left ventricular hypertrophy (LVH). Myocardial imaging with 123I-beta-methyl iodophenyl pentadecanoic acid (BMIPP) was performed in 28 patients with hypertrophic cardiomyopathy (HCM), 15 patients with hypertensive heart disease (HHD), 13 patients with aortic stenosis (AS) and 8 normal controls (NC). The patients with HCM consisted of 13 patients of asymmetric septal hypertrophy (ASH), 7 patients of diffuse hypertrophy (Diffuse-HCM) and 8 patients of apical hypertrophy (APH). Planar and SPECT images of BMIPP were acquired 15 minutes and 4 hours after tracer injection. Resting 201Tl SPECT images and echocardiography were also performed on other days. We calculated heart/mediastinum count ratio and washout rate of BMIPP by using planar image. In patients with LVH, the incidence of reduced BMIPP uptake was more frequent than that of reduced 201Tl uptake. In delayed images, more than 60% of patients with LVH reduced BMIPP uptake, especially remarkable for patients with ASH and APH. The washout rate of all cardiac hypertrophic disorders was tended to be higher than that of normal subjects. Reduced BMIPP uptake was frequently found in septal portion of anterior and inferior wall in patients with ASH, in inferior wall in patients with Diffuse-HCM and HHD, in apex in patients with APH and AS. These results suggest that BMIPP scintigraphy can differentiate three types of cardiac hypertrophy.

Aged↗
PubMed1998

Molecular cloning, characterization, and chromosomal localization of FKBP23, a novel FK506-binding protein with Ca2+-binding ability.

We have identified and characterized a cDNA encoding a novel FK506-binding protein (FKBP), named FKBP23, from mouse heart by the signal sequence trap method. The deduced amino acid sequence has significant homology to other FKBP family members around the peptidylprolyl cis-trans-isomerase motifs. FKBP23 also has two Ca2+-binding (EF-hand) motifs, and purified FKBP23 protein was shown to have Ca2+-binding ability. This is the first report of a Ca2+-binding FKBP. FKBP23 is a glycoprotein retained in the endoplasmic reticulum by its carboxyl-terminal tetrapeptide His-Asp-Glu-Leu, as demonstrated by immunostaining, retention, and deglycosylation assays. FKBP23 mRNA is expressed most strongly in heart, lung, and testis, beginning at day 8.5 of embryonic development. The FKBP23 gene was mapped to mouse chromosome 2.

Amino Acid Sequence↗
PubMed1998

Pancreatic carcinoma: correlation between E-cadherin and alpha-catenin expression status and liver metastasis.

BACKGROUND: Dysfunction of the E-cadherin/catenin-mediated cell-cell adhesion system has been associated with invasiveness and poor differentiation of human carcinomas. However, its importance in the genesis of liver metastasis has not been examined sufficiently. METHODS: A series of 26 primary pancreatic carcinomas and the concomitant liver metastases from 15 of them, obtained at autopsy, were analyzed for E-cadherin and alpha-catenin protein expression by immunohistochemistry. RESULTS: Both E-cadherin and alpha-catenin expression were preserved in 15 (58%) and reduced in 11 (32%) of the 26 primary pancreatic carcinomas. In the former 15 primaries, carcinoma cells were attached to each other tightly, whereas the latter 11 primaries showed isolated or loosely connected attachments. The metastatic ratio was higher in tumors exhibiting tight adhesion than in those with loose adhesion: 73% and 36%, respectively (P = 0.059). E-cadherin and alpha-catenin expression patterns in liver metastases basically followed those in the corresponding primaries (P < 0.01). CONCLUSIONS: Reduced E-cadherin and alpha-catenin expression in primary pancreatic carcinoma has no significant predictive value regarding the presence of liver metastasis. Rather, there is a greater tendency for liver metastasis in cases in which the integrity of the E-cadherin/catenin-mediated cell-cell adhesion system is intact.

Adult↗
PubMed1997

Calumenin, a Ca2+-binding protein retained in the endoplasmic reticulum with a novel carboxyl-terminal sequence, HDEF.

We have identified and characterized a cDNA encoding a novel Ca2+-binding protein named calumenin from mouse heart by the signal sequence trap method. The deduced amino acid sequence (315 residues) of calumenin contains an amino-terminal signal sequence and six Ca2+-binding (EF-hand) motifs and shows homology with reticulocalbin, Erc-55, and Cab45. These proteins seem to form a new subset of the EF-hand protein family expressed in the lumen of the endoplasmic reticulum (ER) and Golgi apparatus. Purified calumenin had Ca2+-binding ability. The carboxyl-terminal tetrapeptide His-Asp-Glu-Phe was shown to be responsible for retention of calumenin in ER by the retention assay, immunostaining with a confocal laser microscope, and the deglycosylation assay. This is the first report indicating that the Phe residue is included in the ER retention signal. Calumenin is expressed most strongly in heart of adult and 18.5-day embryos. The calumenin gene (Calu) was mapped at the proximal portion of mouse chromosome 7.

Amino Acid Sequence↗
PubMed1997

Transforming growth factor-alpha (TGF alpha)-producing gastric carcinoma with acanthosis nigricans: an endocrine effect of TGF alpha in the pathogenesis of cutaneous paraneoplastic syndrome and epithelial hyperplasia of the esophagus.

A case of well-differentiated adenocarcinoma (Borrmann type 3) of the stomach in a 76-year-old man associated with the typical skin manifestations of acanthosis nigricans and with multiple protruding lesions showing epithelial hyperplasia of the esophagus is reported. The advanced tumor was located in the cardiac region of the stomach, and measured approximately 8 cm in diameter, with partial invasion to the esophagus. The associated cutaneous lesions were characterized by hyperpigmentation and by protruding verrucous papules on the torso, head, face, neck, upper extremities, perineum, and inguinal region. Histologically, the protruding skin lesions showed keratinocytes proliferation throughout the epidermis, resulting in diffuse hyperkeratosis, papillomatosis, and acanthosis of the skin. Immunohistological analysis showed coexpression of transforming growth factor alpha (TGF-alpha) and epidermal growth factor (EGF) receptors in the tumor from the stomach. It is reasonable to conclude from this evidence that gastric carcinoma cells secrete TGF alpha in an autocrine for auto-stimulation. EGF receptor expression was also noted on the papillomatous hyperplasia of the cutaneous lesion. Serum level of TGF alpha, determined by an enzyme-linked immunosorbent assay, was high (144 pg/ml; normal, 22.0 +/- 16 pg/ml (Mean +/- SD)). Serum TGF alpha abruptly decreased to 49 pg/ml on day 7 after the total gastrectomy, and then gradually increased to 77 pg/ml within 28 days. Amelioration of the cutaneous lesions and the protruding lesions in the esophagus was observed after surgical resection of the gastric carcinoma. This suggests that the TGF alpha stimulates the proliferation of keratinocytes involved with EGF receptor. Large amounts of circulating TGF alpha in the blood over a long period released by the primary tumor seem to act as an endocrine-like mechanism causing epidermal and esophageal epithelial cells to proliferate. There is a possible link in the pathogenesis of the acanthosis nigricans as a cutaneous paraneoplastic syndrome, and epithelial hyperplasia of the esophagus.

Acanthosis Nigricans↗