Homozygosity for Machado-Joseph disease gene enhances phenotypic severity.
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Adult↗
Biomedical subjects
S Kawakami
Publications and source records attributed to S Kawakami.
Adnexal masses: transvaginal US and gadolinium-enhanced MR imaging assessment of intratumoral structure.
PURPOSE: To evaluate the accuracy of transvaginal ultrasound (US) and gadolinium-enhanced magnetic resonance (MR) imaging in the assessment of the intratumoral structure of an adnexal mass. MATERIALS AND METHODS: The population comprised 82 patients with an adnexal mass who underwent both transvaginal US and gadolinium-enhanced MR imaging and had surgical confirmation. The findings from both modalities were compared retrospectively with those of surgery. RESULTS: The sensitivity, specificity, and accuracy were 100%, 98%, and 99% with MR imaging and were 97%, 46%, and 68% with transvaginal US for the identification of solid tissue in the masses. Transvaginal US exhibited many (n = 25) false-positive results, especially in evaluation of lesions filled with blood or fatty tissue and small echogenic excrescences adherent to the wall, such as blood or fatty tissue. CONCLUSION: Both transvaginal US and gadolinium-enhanced MR imaging were highly sensitive in identification of solid components within an adnexal mass. Gadolinium-enhanced MR imaging was specific, whereas transvaginal US was nonspecific.
Adnexal Diseases↗
Polycystic ovaries: implications of diagnosis with MR imaging.
PURPOSE: To review the prevalence and clinical usefulness of a previously described magnetic resonance (MR) imaging finding of polycystic ovarian disease. MATERIALS AND METHODS: MR images of the uterine adnexa of 1,074 consecutive female patients suspected of having various gynecologic diseases were retrospectively reviewed for the presence of an MR imaging finding that has been described for polycystic ovarian disease. RESULTS: The prevalence of the previously described MR imaging finding of polycystic ovarian disease was 1.1% (12 of 1,074). Of the 12 patients, five had confirmed polycystic ovarian disease. Seven had no clinical or endocrinologic findings suggestive of polycystic ovarian disease. Six of these patients had endocrinologic findings related to ovarian dysfunction (n = 5) or had undergone hormonal therapy (n = 1). The other patient had vaginal agenesis but no clinical or endocrinologic evidence of abnormal ovarian function. CONCLUSION: The finding of multiple peripheral ovarian cysts at MR imaging may not be specific for polycystic ovarian disease.
Adnexa Uteri↗
Morphometric study on the fetal thyroid gland in the nude mouse (BALB/cAnNCrj-nu/nu).
Fetal thyroid glands of athymic nude mice (BALB/cAnNCrj-nu/nu) were examined morphometrically on day 18 of gestation. Compared to euthymic litter mate controls (BALB/ cAnNCrj-nu/+), both the cell height and diameter of thyroid follicles were significantly smaller; fewer well-developed follicles were found in the peripheral region of the thyroid; the body weight and total volume of the thyroid gland were also smaller in nude mice. These results suggest underdevelopment of the fetal thyroid gland of the athymic nude mouse.
Animals↗
[Results of genitoplasty for virilized genitalia of 41 females with congenital adrenal hyperplasia].
OBJECTIVE: The results of genitoplasty performed at our clinic for 41 female patients with congenital adrenal hyperplasia (CAH) were reviewed. PATIENTS: Among 57 females with CAH (56 with 21-hydroxylase deficiency and 1 with 11 beta-hydroxylase deficiency) diagnosed between 1961 and 1995, 41 underwent genitoplasty. For the rest of 16 patients, 1 received already clitorectomy, 11 (19%) needed no surgical reconstruction, 2 died before surgery, and 2 are in a waiting list for surgery. RESULTS: According to Prader's classification of virilized genitalia, 41 patients who received surgery included 8 of type I, 10 of type II, 17 of type III, and 4 of type IV. There was no patients of type V, and 2 were not classified because of previous surgery. Four patients underwent genitoplasty 7 times in total before visiting us. The age receiving initial genitoplasty at our clinic ranged from one year and 9 months to 20 years (median: 4 years and 5 months). Follow-up period averaged 17 years with the range of 2 months to 33 years. For enlarged clitoris, clitorectomy (amputation of clitoris) was carried out for 22 patients before 1975 and corporal resection for 17 after 1975. Two patients manifested recurrence of clitoromegaly after the surgery because of either incomplete resection of the clitoris or patient's ignorance of taking glucocorticoid regularly. Thirty-six patients needed labiovaginoplasty. Simple midline cutback was performed for 6 patients and Y-V plasty (skin flap labiointroitoplasty) for 30. Vaginal orifice was not identified in 5 patients at the initial surgery. Two patients received successful second introitoplasty at the older age than 10. Post-operative stenosis of vaginal orifice was confirmed in 6 patients and 3 underwent second introitoplasty. Among 23 patients older than 25 years at present, 7 got married and 4 raised 6 children with assistance of Caesarian section. CONCLUSIONS: Most of female patients with CAH needed genital plastic surgery including clitoroplasty and labiovaginoplasty. Corporal resection for enlarged clitoris with sufficient hormone therapy brought a satisfactory result. Y-V plasty for labiovaginoplasty proved to be an excellent method, especially from cosmetic viewpoint, but remained some problems as one stage operation including difficult identification of vaginal orifice and post-operative stenosis of introitus in some patients.
Adrenal Hyperplasia, Congenital↗
[An investigation on the therapeutic effects of tuboplastic catheter system under salpingoscope (FT catheter system) for the patients with bilateral tubal occlusion].
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Adult↗
Different time-course production of peptidic and nonpeptidic leukotrienes and prostaglandins E2 and F2 alpha in the ovary during ovulation in gonadotropin-primed immature rats.
Temporal changes in ovarian leukotrienes (LTs) and prostaglandins (PGs) were examined during ovulation to assess roles they may play in this process. Ovulation was induced in immature rats by injection with human chorionic gonadotropin (hCG; 10 IU, s.c.) 2 days after they had been primed with pregnant mare serum gonadotropin (10 IU, s.c.). The ovaries were extirpated at various intervals after hCG administration and assayed for LTB4, and LTC4/D4/E4, as well as PGE2, and PGF2 alpha. Ovarian concentration of LTB4 increased (P < 0.01) rapidly, reaching a peak at 1 hour after hCG administration, then declined (P < 0.05) by 4 hours and remained low thereafter. In contrast, LTC4/D4/E4 peaked (P < 0.01) between 1 and 2 hours, declined (P < 0.05), and then increased (P < 0.01) to achieve a second, larger peak at 10 hours. Prostaglandin E2 increased (P < 0.01) at 6 hours after hCG and did not decrease from the peak value until (P < 0.01) 14 hours after hCG. Concentrations of PGF2 alpha increased (P < 0.01) at 4 hours after hCG, but decreased (P < 0.01) from the peak by 10 hours. Although LTB4, LTC4/D4/E4, PGE2, and PGF2 alpha all increased during ovulation, the time-course differed. This suggests that each eicosanoid may play a distinct role in the process of follicular rupture.
Animals↗
In vitro evaluation of antithrombogenicity of hybrid-type vascular vessel models based on analysis of the mechanism of blood coagulation.
In vitro evaluation of antithrombogenicity of materials of artificial vascular vessels was carried out based on an analysis of the coagulation mechanism of blood. Coagulation of blood in tubes coated with endothelial cells (ECs) or segmented polyurethane (SPU) was measured by a rheological technique. Coagulation of blood in hybrid-type vascular vessel model tubes consisting of cultured ECs proliferated on carbon-deposited SPU was also examined. Coagulation of blood in these tubes occurred within 40 min, and appears to be due to the interaction between erythrocytes and a coagulant factor in plasma. From the in vitro evaluation test, when the time of onset of the coagulation of blood in contact with a surface of the artificial materials is longer than 20-39 min, the materials can be considered to provide an inert surface against thrombus formation and blood coagulation.
Adult↗
The LHRH pulse generator: a mediobasal hypothalamic location.
The location and mechanism of LHRH pulse generator are discussed based on our series of experiments. Suckling stimulus is a novel stimulus that inhibits LH pulses without any cooperation from ovarian steroids, unlike other stimuli such as stress, photoperiod etc. It is directly involved in suppressing the activity of the LHRH pulse generator. The information from teats suckled by pups or babies is conveyed dorsally to the mediobasal hypothalamus (MBH), where the LHRH pulse generator may be located. Experiments using various types of deafferentation and fetal brain tissue transplantation confirmed that the LHRH pulse generator is located in the MBH and suggested that LHRH pulse generator consists of nonLHRH neurons. Endogenous excitatory amino acid is one of the possible neurotransmitters that regulate LHRH release at the nerve terminal in ME.
Animals↗
Adenylyl cyclase activity in human decidua and myometrium during pregnancy and labour.
Adenylyl cyclase activity was studied in human decidua and myometrium in early pregnancy and at term before and after the onset of labour. Decidual basal, prostaglandin-, catecholamine- and forskolin-stimulated adenylyl cyclase activities at term before the onset of labour were significantly lower than those in early pregnancy. After the onset of labour at term, decidual basal, prostaglandin-, catecholamine-, NaF- and forskolin-stimulated adenylyl cyclase activities significantly increased compared with those at term before the onset of labour. Myometrial prostaglandin- and catecholamine-stimulated activities did not alter during pregnancy, except for basal and forskolin-stimulated activity. Myometrial basal, prostaglandin-, catecholamine-, NaF- and forskolin-stimulated activities at term showed no change after the onset of labour. At term, before the onset of labour, myometrial basal, prostaglandin, catecholamine-, NaF- and forskolin-stimulated activities were the same as those in the decidua. However, after the onset of labour at term, decidual basal and the stimulated activities were significantly higher than those in the myometrium. These results suggest that decidual prostaglandin- and catecholamine-stimulated adenylyl cyclase may play an important role in the initiation or maintenance of human labour or in both processes.
Adenylyl Cyclases↗
Mechanical left ventricular support in patients with Marfan's syndrome: a report of two cases.
Left ventricular assist device (LVAD) was utilized for the treatment of postcardiotomy heart failure in two patients with Marfan's syndrome. Patient 1 (a 22-year-old) with annuloaortic ectasia (AAE) and DeBakey type II dissection had been supported by LVAD for 87h after composite graft replacement of the ascending aorta and aortic valve. Patient 2 (a 52-year-old) with AAE and DeBakey type I dissection had been supported by LVAD for 91 h after aortic valve replacement. During the assist, both patients complicated bleeding from the fragile left atria near the sites of cannulation. Patient 1 died of multiple organ failure on the 62nd postoperative day, but patient 2 returned to work after surgery.
Adult↗
[Clinical study of renal cell carcinoma with brain metastasis].
Of 130 cases with renal cell carcinoma treated at Cancer Institute Hospital from January, 1981 to December, 1992, 14 (10.6%) developed brain metastasis, 12 of whom had had preceding pulmonary metastasis. Interval between the initial treatment of the primary lesion (nephrectomy in 13, embolization in 1) and the diagnosis of brain metastasis ranged 0 to 57 months with a median of 11 months. Twelve patients had clinical symptoms such as headache, vomiting, paralysis or disturbance of consciousness. Eleven patients were treated with external beam irradiation (30-60 Gy linear accelerator). Only 3 (30%) of 10 patients with measurable lesion on CT scan achieved PR but 6 (66.7%) of 9 had symptomatic improvement. Especially, chronic intracranial hypertension such as headache and vomiting disappeared in 5 (83.3%) of 6. Average survival period and one year survival after the diagnosis of brain metastasis were 5 months and 14.3%. Although most of the patients with brain metastasis died of the progression of other organ metastasis, radiation therapy for brain metastasis was useful to palliate the agonizing symptoms.
Adult↗
[Renal cell carcinoma in a patient with malignant lymphoma: a case report].
This is a case report of a 51-year-old male in the late stage of malignant lymphoma who presented with pathological fracture renal cell carcinoma secondary to metastatic. He underwent right nephrectomy followed by immunotherapy consisting of interferon alpha and some cytokines. According to his condition and extension of the disease, OK-432, interleukin-2 and interferon gamma were combined. He was also given irradiation to the bone metastases. He died of progressive disease 8 years and 6 months after the initiation of interferon alpha therapy. The autopsy revealed evidence of pancreatic involvement with renal cell carcinoma and malignant lymphoma as well. Malignant lymphoma also infiltrated into the mesentery and left femur. We reviewed other reports regarding multiple malignant tumors associated with renal cell carcinoma and discussed the occurrence of malignant lymphoma in renal cell carcinoma patients.
Carcinoma, Renal Cell↗
Evidence for the presence of an inactive precursor of human hepatocyte growth factor in plasma and sera of patients with liver diseases.
Human hepatocyte growth factor (hHGF), which is now known to be the same protein as the scatter factor and the tumor cytotoxic factor, is a heterodimeric protein with one heavy chain and one light chain linked together by a disulfide bond, and is thought to be involved in liver regeneration. Using an enzyme-linked immunosorbent assay (ELISA) and Western blot analysis, we found that a significant amount of single chain precursor of hHGF (pro-hHGF) was present in plasma of patients with fulminant hepatic failure (FHF) and that normal human serum contained a protease or proteases that convert pro-HGF to a heterodimeric (mature) form of hHGF. We also showed that the processing protease activity for hHGF was suppressed by such serine protease inhibitors as leupeptin, antipain, and aprotinin, and that sera of patients with liver diseases such as fulminant hepatic failure, acute hepatitis, chronic hepatitis, and cirrhosis contained not only pro-hHGF but also the protease. This is the first report showing the presence of pro-hHGF in human blood, and our observations suggest that hHGF is synthesized and secreted from the hHGF-producing cells as an inactive pro-hHGF after hepatic injuries, and the pro-hHGF is then converted to an active heterodimeric form of hHGF in the blood. It is also suggested that plasma of patients with liver diseases contains an active protease or proteases that convert pro-hHGF to a mature form of hHGF.
Animals↗
[A case of traumatic diaphragmatic hernia accompanied with rupture of pericardium].
We experienced a case of left traumatic diaphragamatic hernia accompanied with rupture of pericardium after blunt trauma by traffic accident. A 55-year-old man who had suffered blunt chest and upper abdominal trauma because of smash of two cars when he had been turning to right in the corner was admitted to our hospital. On arrival at the hospital, the patient complained severe dyspnea that reduced by lying on his right. Another position couldn't be taken because of deterioration of dyspnea. Chest X-ray showed disappeared left diaphragmatic line and abnormal transparent mass that suggest alimentary canal in the left intrapleural space. Cardiac ultrasonography showed abnormal tubular mass close to the left side of mediastinum that deviated to right. The patient was diagnosed as traumatic diaphragmatic hernia and emergency operation was performed. Left diaphragma was lacerated sagitally toward its top for about 12 cm in length. Left pleuropericardium was seen to be lacerated longitudinally for about 8 cm in length in front of the phrenic nerve intraoperatively. Both lacerations were closed by direct suture. The patient discharged on the 37th postoperative day without phrenic nerve palsy. At making diagnosis of patient with chest and abdominal trauma, injure of diaphragma and/or pericardium also must be considered. If it is possible, operation must be performed without hesitation in order to get good prognosis.
Accidents, Traffic↗
[Intra-arterial chemotherapy with cisplatin, vincristine, methotrexate and adriamycin for recurrent penile cancer: a case report].
A 60-year-old man visited our hospital with a chief complaint of penile tumor on November 13, 1984. Circumcision of true phimosis was performed and biopsy of the 27 x 27 mm fixed tumor on the glans revealed well differentiated squamous cell carcinoma. Seventy Gy of electron irradiation to the penis revealed a complete response. On February 1, 1985 bilateral inguinal lymph node dissection was performed and revealed metastasis to a left inguinal node. In May 1990, erosive changes occurred on the irradiated region, and local recurrence was confirmed. Since 2 courses of MBD (methotrexate, bleomycin, cisplatin) chemotherapy was not effective, additional wedge dissection was performed. In January 1992, local recurrence was found. Four courses of intra-arterial chemotherapy (cisplatin, vincristine, methotrexate, adriamycin) was performed with a complete response. No evidence of disease has been recognized for more than two years after completion of the treatment.
Antineoplastic Combined Chemotherapy Protocols↗
Evaluation of facial palsy by Moiré topography.
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Face↗
Docosahexaenoic acid status of patients with extrahepatic biliary atresia.
Docosahexaenoic acid (DHA) is believed to be an important long-chain polyunsaturated fatty acid (LCPUFA), which may be essential for neurofunction in infants. Patients with extrahepatic biliary atresia (EBA) may have DHA deficiency secondary to fat malabsorption. The authors investigated DHA and other LCPUFA levels in plasma and red blood cell (RBC) phospholipids of patients after the Kasai portoenterostomy and after supplementation with essential fatty acids. Ten children aged 8 to 17 months (mean, 12.6 months) comprised the study group. Five were jaundiced and five had a normal bilirubin level. The patients received 1 mL/kg of fat emulsions (10% Intralipid, containing 50% linoleic acid and 9% alpha-linolenic acid) in addition to an age-appropriate diet. Additional supplements were ursodeoxycholic acid (UDCA) (15 mg/kg/d) and taurine (100 mg/kg/d). The percentages of DHA in both plasma and RBC phospholipids of patients in the jaundiced group were significantly lower than those of normal children. Patients in the jaundice-free group had significantly lower levels of DHA and higher levels of linoleic acid in both plasma and RBC phospholipids in comparison to the normal group. This study shows that postoperative EBA patient become DHA-deficient even when supplemented with fat emulsions (largely composed of linoleic acid) that contain DHA's precursor, alpha-linolenic acid. This demonstrates a deficiency in the long-chain acid desaturase activity of these patients. It is recommended that excessive/linoleic acid intake be avoided and that all EBA patients have small amounts of DHA added to their lipid supplementation.
Biliary Atresia↗