[Paget disease of bone].
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Biomedical subjects
Publications and source records attributed to Hiroyuki Nagasawa.
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PURPOSE: The purpose of this study was to evaluate the use of radionuclide angiography and single photon emission computed tomography (SPECT) using Tc-99m hexakis-2-methoxyisobutylisonitrile (Tc-99m MIBI) for analyzing musculoskeletal lesions. MATERIALS AND METHODS: Radionuclide angiography and SPECT using Tc-99m MIBI were performed in 36 patients with various soft tissue and bone pathologies (18 benign and 18 malignant lesions). The ratio of the counts of the lesion to that of the contralateral normal area was calculated from the region of interest drawn on the MIBI scan. The MIBI-uptake ratio was calculated using SPECT and the MIBI-perfusion index was calculated using radionuclide angiography. RESULTS: The MIBI-uptake ratio in malignant lesions (4.80 +/- 4.43) was significantly higher (P < 0.05) than that in benign lesions (1.83+/-2.48). The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of the MIBI-uptake ratio for malignant lesions were 72%, 83%, 78%, 81%, and 75%, respectively. The MIBI-perfusion index in malignant lesions (17.68 +/- 21.19) was significantly higher (P < 0.05) than that in benign lesions (2.25 +/- 2.56). The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of the MIBI-perfusion index for malignant lesions were 87%, 75%, 81%, 77%, and 86%, respectively. CONCLUSIONS: The MIBI-uptake ratio and MIBI-perfusion index using Tc-99m MIBI can yield important additional information with which to differentiate musculoskeletal lesions.
Retinoic acid (RA) plays an important role in bone metabolism in vivo through osteoclast activation and bone resorption. Retinoid X-activated receptor beta (RXRbeta) has been implicated in the genetic spinal defect of ossification of the posterior longitudinal ligament (OPLL). In this study, we examined the effects of 9-cis RA and all-trans RA (ATRA) on the proliferation, differentiation, and RXRbeta expression of the murine preosteoblastic cell line MC3T3-E1. Both 9-cis RA and ATRA dose-dependently inhibited the increase in total soluble protein content at concentrations of 10 and 100 nM after 4 and 8 d co-culture with MC3T3-E1 cells. The inhibitory effect of 9-cis RA was slightly stronger than that of ATRA. Histone H4 mRNA expression was dose-dependently suppressed by both RAs on day 1. Alkaline phosphatase activity was increased by both RAs at 10 and 100 nM concentrations on day 4, with 9-cis RA-induced activity slightly stronger than that of ATRA. Osteopontin mRNA expression was increased by both RAs on day 1, but was suppressed on day 4. Bone Gla protein mRNA expression was inhibited by 10 and 100 nM 9-cis RA and by 100 nM ATRA on day 14. RXRbeta mRNA expression was increased by 9-cis RA, an RXRbeta ligand, in a dose-dependent manner. Our results suggested that while both RAs suppressed proliferation and stimulated the maturation of preosteoblastic MC3T3-E1 cells, 9-cis RA was slightly more potent than ATRA. It also appeared that RAs may contribute to the development of heterotopic ossification, including OPLL.
We describe a case history of a 24-year-old male with osteogenesis imperfecta (OI) who developed osteosarcoma of the left thigh. High-dose ifosfamide therapy caused marked tumor regression of multiple lung metastases. Immunohistochemically, the tumor cells were diffusely positive for the p53 protein. Mutation of the p53 gene was not detected by direct genomic sequencing of exons 4-8. The radiographic characteristics, including irregularly distributed osteolytic lesions and cortical discontinuity, should not be confused with hyperplastic callus formation, a benign process. A biopsy is critical to establish the differential diagnosis between osteosarcoma and common hyperplastic callus formation in OI; however, it must be applied with great care.
We assessed the thickness of the normal left ventricular wall according to gender by performing two-dimensional echocardiography in children. While a few studies have evaluated left ventricular wall thicknesses in children using two-dimensional echocardiography, there is little information related to the influence of gender throughout childhood. We evaluated 251 patients (128 males and 123 females) with innocent murmurs but without congenital heart anomalies, or coronary artery lesions or arrhythmias, as determined by repeated echocardiographic, and electrocardiographic examinations. Intra-ventricular septal thicknesses at end-diastole and end-systole, and left ventricular posterior thicknesses at end-diastole and end-systole were determined in the parasternal short-axis view. Significant gender differences were observed in the thickness of all four measurements. There was a linear relationship between the thickness of the left ventricular wall and height. Height was the most appropriate and practical index for assessing the thickness of the wall of the left ventricle in children.
OBJECTIVE: To report a case of a 47 XYY male neonate with congenital heart disease and short stature. DESCRIPTION: This is the first case report of a 47 XYY male neonate associated with congenital heart disease (total anomalous pulmonary venous return) and small for date. The boy neonate was born at around 32 weeks of gestation with birthweight of 1134 g. An intracranial hemorrhage and pulmonary high flow were discovered at an early neonatal period. His physical and mental development was very retarded. The infant underwent a palliative ligation of ductus arteriosus and a ventriculoperitoneal shunt operation, but subsequently died due to consequent heart failure at 19 month-old. COMMENTS: This combination of XYY male and congenital heart disease may be a fortuitous one. However, we think it is important to report that there was a poor prognosis case of XYY male with congenital heart disease and short-stature.