Continuous antiemetic effect of epidural droperidol was evaluated by prospective, double-blinded study using 145 ASA physical status I-II patients undergoing major gynecological surgery. All patients received bolus epidural infusion of buprenorphine at the end of the operation and continuous epidural infusion of buprenorphine for 48 hours. Patients were divided into 3 groups, 49 patients of Group I received placebo, 50 patients of Group II received only bolus doses of droperidol 2.5 mg and the remaining 46 patients of Group III received continuous epidural infusion of droperidol 10 mg in 48 hours. Visual analogue scales for nausea, the incidence of emetic episodes and side effects were evaluated at 3 hr, 24 hr and 48 hr postoperatively. The scores for nausea were significantly lower in Group III (P < 0.05) compared with Group I 24 hr after the operation. The incidence of emesis was significantly lower in Group III (P < 0.05) as compared with Group I and II 48 hr after the operation. Extrapyramidal side effects and sedative effects were not observed in any patients. The continuous epidural administration of droperidol is effective in prevention of postoperative nausea and vomiting and the effect lasts during its infusion with no side effects.

Analgesia, Epidural↗

PubMed2001

[Indication for surgical and microwave coagulation therapy for multiple (> or = 5) bilobar liver metastases from colorectal cancer].

We analyzed methods and clinicopathological factors for multiple (> or = 5) bilobar liver metastases (H3) from colorectal cancer and evaluated the indication of surgical and microwave coagulation therapy (MCT) for them. Twenty-four patients with H3 underwent surgical therapy and were divided into two groups. Group a: 9 patients with a prognosis of more than 700 days. Group b: the remaining 15 patients. There was no significant difference in prognosis between those receiving MCT and resection + MCT as a surgical therapy. The number and maximum diameter of tumors tended to be smaller in Group a. The number of tumors was less than or equal to 9 and the maximum size of the tumors was 38 mm. Moreover, the tumor could be controlled by MCT alone if the tumor size was less than 30 mm. MCT is a useful therapy for these cases and the indication for surgical therapy may depend on the number and maximum size of tumors.

Colorectal Neoplasms↗

PubMed2001

[A symptomatic Rathke's cleft cyst with pituitary apoplexy: a case report].

We encountered a rare case of symptomatic Rathke's cleft cyst associated with pituitary apoplexy. To our knowledge, six cases have been reported in the literature. The mechanism of this symptom is still obscure because of the rare pathological findings concerning Rathke's cleft cysts. Judging from the operative and the histopathologic findings, we speculate that pituitary apoplexy could occur due to hemorrhage from certain vessels such as compressed portal veins or newly organized vessels of the granulation tissue of the cyst wall.

Aged↗

PubMed2001

[Examination concerning dissociation of left ventricular volume calculation value based on difference of analytical algorithm and perfusion tracer in gated myocardial SPECT].

According to improvement of SPECT system, ECG-gated SPECT with 201TlCl have been applied to the left ventricular volumetry. In this study 24 patients without ischemia demonstrated by stress (99mTc-TF) and rest (201TlCl) dual-isotope ECG-gated myocardial SPECT were enrolled. To evaluate left ventricular volumetry using 201Tl ECG-gated SPECT data, the left ventricular end diastolic volumes (EDV) were compared between Quantitative Gated SPECT (QGS) and Emory Cardiac Toolbox (ECT) as well as between dual-isotopes based on the same ECG-gated data. The EDV values with 99mTc data (EDVTc) using QGS were well correlated with those using ECT (r = 0.96, p < 0.0001). Both QGS and ECT demonstrated well correlation between EDVTc (r = 0.98, p < 0.0001) and the EDV value with 201Tl (EDVTl) (r = 0.93, p < 0.0001). However, QGS processing induced significantly lower EDVTl compared with EDVTc. In contrast, EDVTl were significantly higher than EDVTc in ECT performance. The QGS errors subtracting EDVTl from EDVTc were more evident according to the left ventricular volume increase. On the other hand, ECT error showed no tendency associated with the left ventricular volume. From these results, a careful strategy for selection of tracers and softwares should be necessary to assessment of quantitative values derived from ECG-gated SPECT data because of interaction with softwares, tracers, and subjects.

Adult↗

PubMed2001

[Genetic testing and bioethics].

Genetic counseling is fundamental before and after the genetic testing, especially presymptomatic genetic testing. However, there is no official system for genetic counseling in Japan. We propose the multidisciplinary approach to genetic counseling, which might become available in the Japanese medical system. Shinshu University Hospital established a division of clinical and molecular genetics as one of its central service departments in 1996. It was officially approved by Ministry of Education in 2000. Our division is composed of several MDs from the departments of neurology, endocrinology, pediatrics, oncology, laboratory medicine and medical genetics, a clinical psychologist, and a genetic nurse. We have a staff meeting once a week to discuss each case for providing the suitable counseling, and we discuss the ethical, legal and social issues (ELSI). We hope our approach will become familiar to other hospitals, and genetic services in Japan will consequently be improved.

Bioethics↗

PubMed2000

Standard growth curves for Japanese patients with Prader-Willi syndrome.

We constructed the standard growth (length/height and weight) curves for Japanese individuals with Prader-Willi syndrome (PWS). Crude height and weight data were collected from 153 males and 99 females with the syndrome, and the collected data were arranged by a mathematical method to construct the curves. Height growth patterns were quite different between PWS and normal children. Mean height of individuals with the syndrome by puberty is -2 SD for normal children, and it drops off far below -2 SD value after puberty. Final mean height is 141.2 +/- 4.8 cm for females (n = 13) and 147.7 +/- 7.7 cm for males (n = 17), showing 15.8 and 21.9 cm below the average height for normal Japanese girls and boys, respectively. Thus, the degree of shortness is more pronounced in male than in female patients. There was no difference in height between those with chromosome 15q deletion and those without. Mean weight at birth of girls (n = 88) and boys (n = 131) were 2.70 +/- 0.45 Kg and 2.62 +/- 0.47 Kg, respectively. These values were smaller than those for normal neonates (P < 0.05, t-test). The weight of PWS children was under the mean value for normal infants by age 2 years, but gradually increase above the mean values for normal children around ages 2-4 years. Overweight in both males and females becomes obvious during prepuberty. Growth patterns are not different between Japanese and Caucasian children with the syndrome. Short stature is more prominent in boys of both ethnic groups, whereas the degree of overweight appears much more severe in Caucasians.

Adolescent↗

PubMed2000

Role of endogenous endothelins in catecholamine secretion in the rat adrenal gland.

We investigated the role of endogenous endothelins in catecholamine secretion in response to transmural electrical stimulation in the retrogradely perfused rat adrenal gland. (R)2-[(R)-2-[(S)-2-[[1-(hexahydro-1H-azepinyl)]carbonyl]amino-4-++ +methy l-pentanoyl]amino-3-[3-(1-methyl-1H-indoyl)]propionyl]amino-3-(2-+ ++pyridyl) propionic acid (FR139317; 0.03-3 microM), an endothelin ET(A) receptor antagonist, inhibited the electrical stimulation-induced epinephrine and norepinephrine output. Neither N-cis-2, 6-dimethylpiperidinocarbonyl-L-gamma-methylleucyl-D-1- methoxycarbonyl tryptophanyl-D-norleucine (BQ-788; 0.03-3 microM), an endothelin ET(B) receptor antagonist, nor phosphoramidon (1-100 mM), an endothelin-converting enzyme inhibitor, affected the catecholamine output responses. However, the inhibition by FR139317 of the catecholamine output responses was abolished by pretreatment with phosphoramidon (100 mM) or BQ-788 (3 microM). These results indicate that activation of endothelin ET(B) receptors by endogenous endothelins inhibits the catecholamine output responses under the condition in which endothelin ET(A) receptors are blocked. Exogenous endothelin-1 (1-100 nM) did not affect the catecholamine output responses, but it inhibited the responses under treatment with phosphoramidon and FR139317. Activation of endothelin ET(A) receptors may interfere with the endothelin ET(B) receptor-mediated inhibitory action on the neuronally evoked secretion of adrenal catecholamines.

Adrenal Glands↗

PubMed2000

Japanese family with an autosomal dominant chromosome instability syndrome: a new neurodegenerative disease?

We report on a Japanese family having an autosomal dominant neurodegenerative disease with chromosomal instability and radiosensitivity. Clinical manifestations of affected members included short stature, osteoporosis, severe dental caries, and various neurological abnormalities, such as mental retardation, depression, dysarthria, hyperreflexia, and ataxic gait. MRI demonstrated a markedly atrophic spinal cord and degeneration of the white matter. Cytogenetic examination showed spontaneous chromosome rearrangements at 14q11.2 and hypersensitivity to radiation and bleomycin. The degree of these cytogenetic abnormalities was significantly higher in the patients than in normal controls but lower than in patients with ataxia telangiectasia or Nijmegen breakage syndrome. Moreover, genetic anticipation was observed in this family: the age of disease onset became earlier, MRI abnormalities more extensive, and the chromosome hypersensitivity to radiation increased in successive generations. We speculate that a basic defect in this family is a mutation in the gene that is responsible for DNA double-strand breakage repair.

Adult↗

PubMed2000

Molecular genetic study of japanese patients with X-linked alpha-thalassemia/mental retardation syndrome (ATR-X).

X-linked alpha-thalassemia/mental retardation syndrome (ATR-X) is one of the many known X-linked mental retardation syndromes. Mutations in the ATR-X gene (ATRX) that encodes a putative global transcription factor have been identified in patients with ATR-X as well as those with other forms of X-linked mental retardation syndrome. To better understand the genetic basis of ATR-X, we investigated nine patients with the ATR-X phenotype from eight independent Japanese families for mutations in ATRX. We identified seven missense mutations, including six novel mutations, all of which were located either in the N-terminal region corresponding to the putative zinc finger domain (N179S, P190L, V194I, and R246C) or in the C-terminal region corresponding to the helicase domain (V1552F, L1645S, and Y1847C). R246C was found in two independent patients. Furthermore, we investigated the origin of the mutations in seven mothers. Five mothers were found to be carriers, and two were not, indicating de novo origin of the mutations. When we compared clinical manifestations with respective mutations, we could not find apparent phenotype-genotype correlation. Therefore, the putative zinc finger domain and the helicase domains may have similar functional significance for the function of ATRX.

Adult↗