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Biomedical subjects

F Zeze

Publications and source records attributed to F Zeze.

At least 19 recordsLinked to original sources

Genotype-phenotype correlation of patients with multiple endocrine neoplasia type 2 in Japan.

BACKGROUND: Multiple endocrine neoplasia type 2 (MEN 2) is a hereditary syndrome characterized by medullary thyroid carcinoma (MTC), pheochromocytoma and hyperparathyroidism. MEN 2 is caused predominantly by germ-line mutations of the RET proto-oncogene. This study aimed to clarify the genotype-phenotype correlation in MEN 2 patients in Japan in order to modify the clinical management according to the genotype. METHODS: Constitutive DNA of 64 MEN 2 patients (48 kindreds) were searched for mutations at exons 10, 11, 13, 14 and 16 of the RET proto-oncogene using polymerase chain reaction-single strand conformation polymorphism (PCR-SSCP), direct sequencing and restriction enzyme digestion. The clinical characteristics of the patients were obtained from a previous nationwide questionnaire survey. RESULTS: Overall, 62 (96.9%) out of 64 patients had a germ-line point mutation at the hot spots. MTC and pheochromocytoma occurred equally in every genotype except C630S. Specific genotype had a correlation between tumor size and age at the operation for MTC or extent of MTC, i.e. C618S developed late onset type of MTC as compared with that of C634R, C634Y and M918T. Small MTC in C634R may be less aggressive than those in C634Y and M918T. CONCLUSIONS: DNA testing has good clinical implications for the management of patients with MEN 2 and the timing and operative procedures of thyroidectomy can be modified according to the genotype.

Adrenal Gland Neoplasms↗

[Efficacy and safety of sulbactam/cefoperazone for hepato-biliary infections].

We studied efficacy and safety of sulbactam/cefoperazone (SBT/CPT) in the treatment of biliary tract infections in hospitalized patients at 26 hospitals from February 1993 to March 1995. Secondary to dropout, 273 out of 338 patients entered in the study were evaluated, 127 patients with cholecystitis, 132 patients with cholangitis, and 14 patients with liver abscesses. Of these, 93 patients (34.1% had malignancy as an underlying disease. SBT/CPZ had an efficacy of 79.9% (218 patients; excellent: 52, good: 166), with the efficacy in patients with cholecystitis, cholangitis and liver abscess at 89.0% (113 patients), 77.3% (102 patients and 21.4% (3 patients), respectively. A significant difference (p < 0.05) was observed in the efficacy rates of patients with (59 patients [63.4%]) and without malignancy (159 patients [88.3%]). A total of 84 strains were isolated from bile specimens of 53 patients, and the major isolates were Escherichia coli, Pseudomonas aeruginosa and Enterococcus spp. Two or more bacterial strains were isolated simultaneously in 20 patients. Mild or moderate side effect (allergic reaction including rash etc.) were noted in 4 patients (1.18%), and laboratory abnormalities (increased GOT, etc.) were in 16 patients (4.71%) out of the total 338 patients. This study clearly demonstrated that SBT/CPZ retains its excellent clinical efficacy and safety profile, throughout its use over the past decade.

Adolescent↗

[Hyperplasia and adenoma of the ectopic parathyroid gland].

The frequency of the ectopic parathyroid glands has been reported to be 7-46% by anatomists or surgeons, while the proper frequency is still unknown. Recently the diagnostic methods of hyperparathyroidism have been developed, and many surgeons select total parathyroidectomy for renal hyperparathyroidism. Therefore the frequency of the ectopic glands has been supposed to be higher than that recognized previously. We investigated 97 patients with renal hyperparathyroidism, and detected 60 ectopic glands in 34 patients (35.1%). The ectopic glands existed in the mediastinum, intrathyroid, carotid sheath, post-pharynx, para-esophagus, and other regions. We describe the features of procedures for detecting ectopic glands. Using non-invasive examination, including ultrasonography, scintigraphy (T1 201-Tc 99 m), and MRI, will be a great assistance in the diagnostic procedures.

Adenoma↗

[Experimental studies on endocrine therapy for rat hepatocellular carcinoma].

Androgen receptor (AnR) and estrogen receptor (ER) are known to exist in human hepatocellular carcinoma (HCC), but the low binding capacity casts doubts on the efficacy of endocrinotherapy. However, we focussed on the favorable dissociation constant and displacement of AnR and ER. Efficacy of endocrinotherapy for HCC was investigated using rat HCC cell line (AH66F) resembling the properties of human HCC and sex hormone receptors. In rat HCC AH66F, we confirmed that the AnR and ER were both positive and were mentioned binding capacity, dissociation constant and displacement resembled those of human HCC. In rat HCC AH66F, administrations of Tamoxifen converted AnR and ER responses to negative. Rat HCC AH66F was transplanted intraperitoneally to Donryu rats, various endocrinotherapies administered and the number of survival days compared with a control group. In male and female rats, the number of survival days was both in the orchidectomied (p < 0.01) and the Tamoxifen treated (p < 0.001) group significantly prolonged. However, in the group treated with medroxyprogesterone acetate no significant differences were observed. Also, in experiments with AnR (-) and ER (-) rat HCC cell line AH60C all endocrinotherapies were ineffective. Above results confirmed the efficacy of endocrinotherapy for rat HCC with positive sex hormone receptors.

Animals↗

A kindred of multiple endocrine neoplasia type 2B.

We describe familial cases of multiple endocrine neoplasia (MEN) 2B: A 48-year-old man is the proband. He had pheochromocytoma, medullary thyroid carcinomas (MTCs), parathyroid hyperplasia, mucosal neuromas, eversion of eyelids and Marfanoid appearance, and then underwent adrenalectomy and total thyroidectomy. Family screening revealed that his two daughters (10 and 8 years old) had mucosal neuromas and increased serum calcitonin (CT). Both of them had MTCs but no pheochromocytoma, and their MTCs were surgically removed. The father and his children have been in favorable condition since the operations. Southern blot analysis with 33 polymorphic DNA probes was done in MTCs obtained from two daughters. An RBP3 (10q11.2) locus linked to a predisposing gene on chromosome 10 was uninformative in either patient because of constitutional homozygosity. Loss of heterozygosity at the MYCL1 locus on chromosome 1p32 was observed in MTC from the younger sister, but no loss of heterozygosity was recognized in other loci examined. Deletion of the 1p32 locus may play a role in the development of MEN 2B.

Adrenal Gland Neoplasms↗

[A case of hyperparathyroid bone disease and the review of renal osteodystrophy].

We report here a typical case of hyperparathyroid bone disease associated with CRF on maintenance HD and review on Renal Osteodystrophy. A 39 year-old female patient was admitted because of polyarthralgia and pruritus. She had a history of HD due to CGN for about 13 years. Laboratory data showed an increase in serum PTH and Alkaline phosphatase level. The evidence of osteitis fibrosa was revealed by bone Xp and scintigraphy. Enlarged solid masses were found in her neck by echogram and parathyroid scintigraphy. She was diagnosed as hyperparathyroid bone disease and total parathyroidectomy c autoplantation was done. Shortly after the surgical treatment, subjective symptoms were relieved and PTH level was normalized. The bone Xp findings improved gradually.

Adult↗

Hyperechoic pattern in breast cancer--its bio-acoustical genesis and tissue characterization.

Clinical breast echography is increasingly being used as it offers a high degree of diagnostic accuracy rate. Internal echoes of usual breast cancer is hypoechoic in the majority of cases, however hyperechoic mass lesion may appear in rare instances. This echo-pattern was seen in a 38-year-old housewife with invasive ductal carcinoma. Also, its bio-acoustical mechanism, that is, why hyperechoic pattern appears, was investigated from the viewpoint of ultrasonic tissue characterization and its related papers were reviewed. Tumor heterogeneity in cellularity such as cribriform pattern, tubular structure, solid nests and a scirrhous pattern of cancer cells may play an important role in producing the hyperechoic breast pattern.

Acoustics↗

[Estrogen receptors in human gastric, hepatocellular, and gallbladder carcinomas and normal liver tissues].

Steroid binding assay using the dextran coated charcoal (DCC) method was applied to human tissues including tumors of the digestive organs, and the results were compared with those of enzymeimmunoassay (EIA) and immunocytochemical assay (ICA) with monoclonal antibody against human estrogen receptor of MCF-7 breast cancer cells. Using the DCC method, estrogen receptor activity was detected in 6 of 26 cases (23.1%) with gastric carcinoma, 3 of 16 hepatocellular carcinoma cases (18.8%), 1 of 3 gallbladder carcinoma cases (33.3%), and both of the 2 cases (100%) with normal liver tissue. However, using EIA, no ER activity was detected in any case. Moreover, ER positive cells were not found by immunohistochemical staining in the gastric carcinoma cases or in normal liver tissue, both of which showed ER activity by the DCC method. These results suggest that the estrogen receptor like material exists in cytosol of the human digestive tumors and normal liver tissue, but that the specificity of the antibodies against estrogen receptor molecules in these tumors may be different from that of the breast tumors.

Breast Neoplasms↗

99mTc-methylene diphosphonate uptake by ossifications and calcifications of non-osseous metastatic tumors.

Two cases of extra-osseous uptake of 99mTc-methylene diphosphonate (MDP) by non-osseous metastatic tumors are reported. One was a metastasis with ossification in the abdominal wall from carcinoma of the sigmoid colon and the other was a metastasis with calcification from an ovarian carcinoma. The mechanism of extra-osseous uptake of 99mTc-MDP is discussed. Bone scintigraphy can be a potential means to assess tumor spread with ossifications and calcifications.

Abdominal Neoplasms↗

[Enzyme immunoassay and radioimmunoassay using monoclonal antibody for the determination of estrogen receptors in human breast cancer].

Enzymeimmunoassay (EIA) with monoclonal antibody against human estrogen receptor (ER) from MCF-7 breast cancer cells and radioimmunoassay (RIA) with monoclonal antibody against ER D-5 antigen from human myometrium were applied to human breast tumor, and the results were compared to those of steroid binding assay using the dextran coated charcoal (DCC) method. The rates of coincidence of positivity and negativity of ER between the DCC method and EIA or RIA in 30 human breast tumors were 96.7% (29/30) and 86.7% (26/30), respectively. A highly significant positive correlation was observed between ER values obtained by the DCC method and those by EIA (r = 0.87, P less than 0.001). On the other hand, the correlation between ER values obtained by the DCC method and those by RIA was less significant (r = 0.41, P less than 0.05). The discrepancy between these ER values may be due to the difference of the monoclonal antibody used in each new assay. The above results indicate that EIA and RIA with monoclonal antibody for human ER are useful for clinical use, although the reason for the partial discrepancy of the data between the DCC method and RIA remains to be elucidated.

Antibodies, Monoclonal↗

Hepatocellular carcinoma associated with familial polyposis of the colon. Report of case.

A case is reported of a 33-year-old man with hepatocellular carcinoma associated with familial polyposis of the colon. During subtotal colectomy for diffuse colonic polyposis, a small tumor was excised from the right lobe of the liver. Histologic examination revealed hepatocellular carcinoma. The association of primary hepatoma in familial polyposis of the colon is very rare. However, it is well known that familial polyposis of the colon has a potent oncogenicity not only in the colon but also in the extracolonic organs. The hepatoma may also be one of the extracolonic malignant manifestations in this inherited disease.

Adult↗

Unusual histiocytic tumor of the small intestine.

A case of unusual sarcoma of the small intestine is described. Ultrastructural observation, enzyme histochemical studies, and functional analysis, such as phagocytic capacity and surface markers, were helpful in defining the neoplastic cells as histiocytes. Autopsy findings are presented.

Histiocytoma, Benign Fibrous↗

Ultrastructural, histochemical, and biochemical studies of two cases with amylase, ACTH, and beta-MSH producing tumor.

Two cases of intrathoracic tumor, different in histology and accompanied by hyperamylasemia, were studied ultrastructurally, histochemically, and biochemically. The ultrastructure of the tumor cell cytoplasm showed many zymogen granules in case 1 and smaller cored granules in addition to zymogen granules in case 2. Both tumors contained not only a large amount of amylase, which was electrophoretically of saliva type with three components, but also significant amounts of immunoreactive ACTH and beta-MSH. Starch film and immunofluorescence showed that the tumor cells stored amylase. It was concluded from these findings that the tumor cells ectopically producing amylase, which showed differentiation toward the cells with zymogen production, could differentiate toward the cells of ACTH-MSH system at the same time.

Adenocarcinoma↗

Macroamylasemia: clinical and laboratory features in 7 patients.

The clinical features of patients with macroamylasemia are diverse. A certain correlation between macroamylasemia and the disease of the pancreas or hepato-biliary system may exist, because among 7 patients in this series 4 patients were diagnosed as acute pancreatitis or relapsing pancreatitis and another patient was performed choledochotomy and manipulation of papilla of Vater may had a effect on pancreas. Among other 2 patients, one was completely symptomless and had no abnormal findings and in another the action of anticancer drugs is suspected. The course of macroamylasemia is usually chronic but in this series one patient had acute transient macroamylasemia and another one had intermittent macroamylasemia during exacerbations of pancreatitis. The serum amylase levels are mostly 2-4 times above normal with one exception (192 Somogyi units) and urinary amylase values are low with one exception (1922 Somogyi units) during exacerbation of pancreatitis. Characteristic zymogram of macroamylase on electrophoresis is also reported.

Adult↗