1/199. 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 = organ (Clic here for more details about this article) |
2/199. 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 = 1keywords = organ (Clic here for more details about this article) |
3/199. Periarticular calcification in systemic lupus erythematosus.OBJECTIVE: To describe the radiologic manifestations of periarticular calcification in patients with systemic lupus erythematosus (SLE) and to investigate clinical variables associated with its occurrence. methods: hand radiographs and clinical records of 52 patients who had 4 or more features of the 1982 revised criteria for classifying SLE and who had no other collagen vascular diseases were analyzed retrospectively. RESULTS: Periarticular calcifications were found in 7 patients (13.5%) near the distal and proximal interphalangeal (DIP and PIP) joints and metacarpophalangeal (MCP) joints. No significant association with calcification was noted for the following variables: age at disease onset, duration of the disease, sex, the maximum value of the serum calcium, organic phosphate, and uric acid, Raynaud's phenomenon, lupus nephritis, femoral avascular necrosis, central nervous system lupus, proteinuria, or the use of drugs such as corticosteroids, synthetic vitamin d, and nonsteroidal antiinflammatory drugs. However, a significant association was noted with the use of furosemide (p < 0.01 by chi-square). In 5 patients periarticular calcification was observed during or just after hyperuricemia had developed while taking diuretics. CONCLUSION: Periarticular calcification in patients with SLE was seen in the DIP, PIP, and MCP joints, and appeared to be associated with the use of diuretics. If patients with SLE are prescribed a diuretic regimen, crystal associated arthritis should be considered as a possibility when diagnosing oligoarthritis.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
4/199. Initiation of ectopic epithelial calcification in a calcifying odontogenic cyst.Ultrastructural observation was performed on a calcifying odontogenic cyst (COC) associated with an odontoma and arising in the right mandibular region of an 8-year-old Japanese boy. Four types of cells were identified in the epithelial layer of the COC. The basal cells were low columnar in shape and contained some intracellular organelles. They were attached to the neighboring cells with a few desmosomes and resembled inner enamel epithelium of the normal enamel organ. The stellate reticulum-like cells, polygonal in shape, possessed desmosomes and many cytoplasmic projections. Some intracellular organelles and a few bundles of tonofilaments were observed in the cytoplasm. The light oval cells that were pale staining with toluidine blue contained dilated membranous organelles and many relatively evenly distributed tonofilaments. These cells were usually scattered in the vicinity of the focal accumulations of ghost cells, and the cell membrane was discontinuous in parts. The ghost cells contained many bundles of tonofilaments that were 60-240 nm in diameter and arranged in various directions. No intact intracellular organelles were noted in the cytoplasm. They were attached to the neighboring ghost cells with some desmosomes and their cell membrane was discontinuous in parts. A variety of vesicles, 90-450 nm in diameter, were scattered among the tonofilament bundles. Some of these contained needle-like crystals that were considered to be initial calcification sites in ghost cells. These vesicles presented morphological similarities to matrix vesicles, and it is therefore suggested that matrix vesicle-like structures are deeply involved with initiation of calcification of ghost cells in COC.- - - - - - - - - - ranking = 5keywords = organ (Clic here for more details about this article) |
5/199. dna ploidy alterations detected during dedifferentiation of periosteal chondrosarcoma.dna ploidy of a case with dedifferentiated periosteal chondrosarcoma was analyzed by dna cytofluorometry. The diagnosis of primary periosteal chondrosarcoma was made on the basis of the radiographic and histological findings. At 4 years after marginal resection, the tumor recurred locally and metastasized to various organs. The patient died of disease 2 years later. Histologically, there were two components, chondrosarcoma and malignant fibrous histiocytoma, in the recurrent and metastatic tumors. dna ploidy analysis of multiple samples revealed that the primary lesion was composed of many diploid cells with some tetraploid and octaploid cells, whereas in the dedifferentiated area, there were many aneuploid cells which were not recognized in any area of the primary tumors. This case illustrated that dna ploidy alteration of euploidy to aneuploidy is closely correlated with the process of dedifferentiation in chondrosarcoma.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
6/199. Mobile intracardiac calcinosis: a new risk of thromboembolism in patients with haemodialysed end stage renal disease.Cardiac calcinosis is a common complication of end stage renal disease. A newly observed risk of thromboembolism is reported in four patients with mobile cardiac calcinosis, treated with long term dialysis. Rapidly growing mobile calcification was confirmed by echocardiography. Each patient had an imbalance in serum calcium x inorganic phosphate (Ca x P product >/= 50); this imbalance could not be treated due to the sudden death of the patient or the need for surgical resection to prevent recurrent cerebral thromboembolism. Histological examination revealed intracardiac calcinosis in three cases, and each case showed haemodialysis hypoparathyroidism (intact PTH < 160 pg/ml). thromboembolism in such cases is rare, however it indicates a need for cautious echocardiographic monitoring in end stage renal disease in patients with an uncontrolled Ca x P product.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
7/199. Malignant intracerebral nerve sheath tumor with intratumoral calcification. Case report.The authors present the clinical, radiological, and pathological features of a malignant intracerebral nerve sheath tumor that occurred in the right parietooccipital lobe of a 4-year-old girl. Computerized tomography scanning and magnetic resonance imaging demonstrated a 5x5x4-cm multiloculated mass with considerable enhancement of the irregularly shaped septa and clearly calcified areas within the mass. Among five cases reported in the literature, this patient is the youngest and represents the first case in which there is radiological evidence of intratumoral calcification.- - - - - - - - - - ranking = 14.738895937512keywords = nerve (Clic here for more details about this article) |
8/199. Conservative management of a case of placenta praevia percreta.Pregnancies complicated by placenta praevia and a history of caesarean section are associated with increased risk of placenta percreta (1). placenta praevia percreta sometimes involves the bladder or other pelvic organ, invasion leading to genital bleeding or haematuria (2, 3). Bladder injury or uncontrollable profuse haemorrhage occasionally occurs in such patients during surgery. Examination of placental invasion is necessary as this clinical condition is severe. Treatment of placental myometrium invasion is required to prevent uncontrollable profuse haemorrhage during surgery. We present a multiparous patient who was diagnosed prenatally with placenta praevia percreta using magnetic resonance imaging (MRI) and who was treated conservatively with a good prognosis.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
9/199. carpal tunnel syndrome caused by an idiopathic calcified mass.This is a case report of carpal tunnel syndrome caused by an idiopathic calcareous lesion within the carpal canal. The median nerve was trapped between the transverse carpal ligament and the calcified mass. The mass was predominantly composed of calcium phosphate. Surgical release of the transverse carpal ligament and removal of the calcareous mass relieved the symptoms.- - - - - - - - - - ranking = 2.9477791875024keywords = nerve (Clic here for more details about this article) |
10/199. Compression of the ulnar nerve in Guyon's canal by uremic tumoral calcinosis.We describe the case of a 70-year-old woman with chronic renal failure on haemodialysis presenting with an ulnar nerve compression in Guyon's canal secondary to uremic tumoral calcinosis. Excision of calcium deposits and external neurolysis of the ulnar nerve were successfully performed. Simultaneously, the hyperphosphatemia and hypercalcemia were corrected. The pathogenesis of this condition is different from primary tumoral calcinosis. Clinical and radiological features and therapy are discussed. Uremic tumoral calcinosis is an unusual etiology of ulnar nerve compression in Guyon's canal not previously reported in dialysis patients.- - - - - - - - - - ranking = 20.634454312517keywords = nerve (Clic here for more details about this article) |
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