1/327. Type 1 GM1 gangliosidosis with basal ganglia calcification: a case report.This report concerns a 10-month-old boy, admitted to the veterans General Hospital-Kaohsiung with generalized tonic convulsion and aspiration pneumonia. He was found to have had developmental regression, progressive hypotonia and hepatosplenomegaly since four months of age. physical examination revealed a large head circumference (97th percentile), frontal bossing, depressed nasal bridge, hepatosplenomegaly, broad hands and short fingers. neurologic examination showed poor control of eye movement, profound hypotonia, muscle weakness, brisk deep tendon reflexes and Babinski's sign. Hypoplasia of the vertebral bodies with anterior beaking, wedge-shaped metacarpals, spatulated ribs and a J-shaped sella turcica were displayed on bone radiographs. Cranial computerized tomography scans showed diffuse brain atrophy, dilated ventricles and calcification of the bilateral basal ganglia. Vacuolated lymphocytes were noted in a peripheral blood smear. Type 1 GM1 gangliosidosis was diagnosed based on a deficiency of beta-galactosidase activity. To our knowledge, basal ganglia calcification in type 1 GM1 gangliosidosis has never been reported in the literature. We suggest that type 1 GM1 gangliosidosis be considered in the differential diagnosis of patients with an early onset of neurologic decline, organomegaly and basal ganglia calcification.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
2/327. Gaucher's disease with valve calcification: possible role of Gaucher cells, bone matrix proteins and integrins.Gaucher's disease, an autosomal recessive storage disease, leads to deposition of glucocerebrosides in various organs, especially those of the reticuloendothelial system. The heart is not thought to be frequently involved and studies of patients with cardiac involvement have concentrated on myocardial involvement. Despite careful prior investigation Gaucher cells have never been detected in the valves of these patients. Pathological findings of a patient with Gaucher's disease, type IIIc, with prominent cardiac valvular involvement are reported and, for the first time, the presence of Gaucher cells in the valve tissue is documented. There is evidence that the pathogenesis of the valvular injury may be by way of a cell-mediated mechanism involving bone matrix proteins and integrins.- - - - - - - - - - ranking = 5keywords = bone (Clic here for more details about this article) |
3/327. Localization of a bone imaging agent in a calcified hematoma.A patient with chronic renal failure and secondary hyperparathyroidism had iliac bone biopsy. The procedure was complicated by a soft-tissue hematoma, which had calcified. A 3-4-cm palpable mass was visible in the lower left abdominal wall. Intense uptake of 99mTc-HMDP corresponded with the location of the calcified hematoma in this patient.- - - - - - - - - - ranking = 5keywords = bone (Clic here for more details about this article) |
4/327. Reduced size of liquefaction necrosis of mitral annular calcification in chronic renal failure by using low calcium concentration hemodialysis.A report is presented of a liquefaction necrosis of mitral annular calcification in a patient with chronic renal failure and secondary hyperparathyroidism who had been managed by hemodialysis for 11 years. The mass was echogenic with an echo-lucent area inside, high density on computed tomography and low intensity on magnetic resonance imaging. The uptake of gallium-67 (67Ga)-citrate and the bone agent technetium-99m-methylene diphosphate (99mTc-MDP) was seen in the mass. These findings were compatible with liquefaction necrosis of the mitral annular calcification. After treatment with low calcium concentration hemodialysis, the size of the mass reduced with disappearance of the echo-lucent area on the echocardiography and there was no uptake of 67Ga-citrate or 99mTc-MDP. Liquefaction necrosis might be the early and reversible form of mitral annular calcification. When a tumorlike echogenic mass at the base of mitral leaflets is seen in patients with predisposing factors for mitral annular calcification, consider the possibility of this specific form of mitral annular calcification in order to avoid any unnecessary surgical intervention.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
5/327. Osseous metaplasia in a growth hormone-secreting pituitary adenoma.A case of growth hormone-secreting adenoma of the pituitary gland showing osseous metaplasia is described in a 56-year-old acromegalic female. The tumor was composed of nests of densely granulated cells separated by and intermixed with calcifications, trabeculae of mature bone and fat. Calcifications are seldom found in pituitary adenomas. In rare instances, calcium deposits can be prominent enough to lead to the formation of pituitary stones and bone which replace the entire tumor mass. Analogously with metaplastic meningiomas, we propose using the term metaplastic adenoma to define cases with osseous metaplasia in order to distinguish between lesions containing bone from the more frequently seen calcified adenomas.- - - - - - - - - - ranking = 3keywords = bone (Clic here for more details about this article) |
6/327. association of diffuse idiopathic skeletal hyperostosis (DISH) and calcification and ossification of the posterior longitudinal ligament.Diffuse idiopathic skeletal hyperostosis (DISH) is a common ossifying diathesis in middle-aged and elderly patients characterized by bone proliferation along the anterior aspect of the spine and at extraspinal sites of ligament and tendon attachment to bone. Four patients with DISH revealed extensive calcification and ossification of the posterior longitudinal ligament in the cervical spine. review of cervical spine radiographs in 74 additional patients with DISH demonstrated bony hyperostosis of the posterior aspect of the vertebrae in 41%, posterior spinal osteophytosis in 34%, and posterior longitudinal ligament calcification and ossification in 50%. These ligamentous findings, which have previously been described almost exclusively in Japanese people, appear to be an additional skeletal manifestation of DISH.- - - - - - - - - - ranking = 2keywords = bone (Clic here for more details about this article) |
7/327. Deep soft tissue leiomyoma of the thigh.A case of ossified leiomyoma of the deep soft tissues of the left thigh is presented. The radiographic appearance suggested a low-grade chondrosarcoma. MRI of the lesion showed signal characteristics similar to muscle on both T1- and T2-weighted spin echo sequences with linear areas of high signal intensity on T1-weighted images consistent with medullary fat in metaplastic bone. Histopathological examination of the resected specimen revealed a benign ossified soft tissue leiomyoma.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
8/327. osteosclerosis, hypoplastic nose, and proptosis (Raine syndrome): further delineation.We describe a newborn girl with a lethal sclerosing bone dysplasia leading to prenatal skeletal alterations and microcephaly, proptosis, hypoplastic nose and midface, small jaw, cleft palate, hypertrophied gums, intracranial calcifications, and generalized osteosclerosis. There is a remarkable similarity between our patient and six previously reported infants subsequently categorized as having a distinct entity: Raine syndrome. Autosomal recessive inheritance is postulated based on parental consanguinity in several of the previous cases and in our patient.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
9/327. Malignant large cell calcifying sertoli cell tumor of the testis (LCCSCTT). Report of a case in an elderly man and review of the literature.Malignant Large Cell Calcifying sertoli cell tumor of the testis (LCCSCTT) is a rare histological variant of sex cordstromal tumors. It usually arises in young males, sometimes is associated with endocrine abnormalities and has a benign course. It is exceptional in elderly men and the outcome is rarely fatal. We report a case of LCCSCTT in a 73 year-old man with fatal outcome. The tumor involved the right testis and several areas of the tunica albuginea were grossly invaded. serum levels of HCG, LH and testosterone were normal. Lymphoangiography performed after orchiectomy showed an involvement of the iliac and preaortic lymph nodes. X-ray of the chest showed no lung metastases. A thorough study of the light microscopic, immunohistochemical and ultrastructural appearances was performed. immunohistochemistry revealed positivity to vimentin, S-100 and NSE. Our observations confirm the previous findings concerning malignant LCCSCTT and point out the histogenesis of the tumor from sertoli cells.- - - - - - - - - - ranking = 0.47513104513119keywords = endocrine (Clic here for more details about this article) |
10/327. Aortic calcification contributing to bone densitometry measurement.A 75-yr-old glucocorticoid-dependent asthmatic male had a bone mineral density study to assess possible osteoporosis prior to initiating therapy. A radiograph of the lumbar spine revealed an asymmetrical compression of the second lumbar vertebra, marked scoliosis, vertebral osteopenia, and a highly calcified abdominal aorta. Bone mineral density (dual X-ray absolptiometry [DXA]) revealed low bone mass in L2-L4 and a markedly abnonrnal pattern, with a linear central density representing a calcified aorta. Posterior-anterior measurements of the midlumbar region with and without the overlying aorta indicated that the calcified vessel contributed up to 33% of the measured density. This was a far higher contribution than reported in other studies. Lateral DXA measurements of the L2 vertebra and the overlying aorta were performed to validate this finding. The density of the L2 vertebra was 0.215 g/cm(2), and that of the overlying calcified aorta was 0. 210 g/cm(2). This case suggests that aortic calcifications may contribute sign)ficantly to overall lumbar bone density and, unless recognized, can inadvertently lead to misclassification of osteoporosis.- - - - - - - - - - ranking = 7keywords = bone (Clic here for more details about this article) |
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