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At least 91 records · Page 5Linked to original sources

Bone disease, hypophosphatemia and hyperparathyroidism after renal transplantation.

UNLABELLED: Long-term survivors after renal transplantation (RT) show: 1) decreased s-phosphorus 2) increased s-PTH 3) no correlation between s-PTH and s-phosphorus 4) increased amount of non-mineralized bone (osteomalacia), inversely correlated to s-phosphorus 5) decreased amount of trabecular bone (osteopenia) in RT patients with aseptic necorsis of bone. CONCLUSION: aseptic necrosis of bone after RT is mainly due to osteoid-induced osteopenia, perhaps aggravated by hypophosphatemic osteomalacia.

Adolescent↗

Massive pelvic osteolysis in the Gorham-Stout syndrome.

Massive osteolysis is a very rare tumour-like lesion usually affecting young adults. There are 132 reported cases which we have reviewed, including 31 single cases of pelvic involvement. The term "massive osteolysis" is based on the typical radiological findings, such as increasing translucency and loss of bone density. The diagnosis of the Gorham-Stout syndrome must be confirmed by the microscopic finding of intramedullary vascular structures. Our patient was a 23 year old white Caucasian, who had the full clinical signs of Gorham-Stout syndrome. She had achieved 2 normal pregnancies and deliveries in spite of her massive pelvic osteolysis.

Adult↗

Vascular anomalies in association with osteodystrophies--a spectrum.

A wide spectrum of osseous abnormalities has been reported in association with vascular lesions. Enchondromas, bone hypertrophy, bone lysis, and sclerotic bone lesions have all been reported in association with hemangiomas, lymphangiomas, varicosities, and arteriovenous fistulae. These associations can be grouped into four major syndromes: Maffucci syndrome, mixed sclerosing bone dystrophy with angiodysplasia, congenital angiectatic hypertrophy (Klippel-Trenaunay-Weber syndrome), and massive osteolysis (Gorham syndrome).

Adult↗

Seventeen year follow-up and autopsy findings in a case of massive osteolysis.

Clinical, radiologic, and pathologic findings in an unusual case of massive osteolysis are described. Peculiar clinical episodes of a foul body odor with fever and erythematous skin lesions, presence of radiographic findings such as acro-osteolysis and periarticular erosions, and the absence of angiomatous or lymphangiomatous dysplasias at autopsy, indicate that this is a new form of massive osteolysis rather than a variant of Gorham disease.

Bone and Bones↗

Gorham massive osteolysis.

Gorham syndrome (massive osteolysis) is a very rare tumour-like lesion characterized by progressive osteolysis. The diagnosis must be confirmed by the microscopic finding of intramedullary angioma-like vascular structures. We report a case of a 15-year-old boy with a pathological fracture in his left humerus. Imaging modalities such as magnetic resonance imaging, computed tomography, angiography and bone scintigraphy failed to disclose to tumorous lesion that filled a cavity in the left humerus. After observing the boy's progress for 6 months, a temporary diagnosis of Gorham syndrome was made, and surgical treatment was chosen. After resection of the left humeral head and the proximal one-quarter of the humerus, thorough curettage was performed in the distal humerus and an intramedullary artificial humeral head fixed with adequate success. Pathological examination of the specimen revealed intramedullary haemangioma of the humerus.

Adolescent↗

Idiopathic acro-osteolysis in an elderly woman. A 10-year follow-up.

We report a case of idiopathic acro-osteolysis in an 80-year-old, otherwise normal woman. The patient first presented at the age of 70 years with a 3-year history of progressive shortening of the left toes. There was no history of trauma or bone disorders. She had osteolysis of the left second to fourth distal phalanges. Angiography was normal. During follow-up over 10 years the osteolysis gradually progressed and spread to affect the right toes.

Age Factors↗

Tetracycline double-labelling in post-traumatic osteopenia in man.

Biopsy specimens were taken from parts of earlier fractured bones in seven patients with post-traumatic osteopenia and one healthy volunteer, who were all administered a double dose of tetracycline for the purpose of prelabelling. In the volunteer, specimens were taken from both the proximal tibia epiphysis and the iliac crest. The fraction of labelled surfaces and the rate of apposition were calculated. The results were also compared with normative data from the iliac crest. At least in the first year after fracture, post-traumatic osteopenia appears to be a condition with an increased appositional activity.

Bone Resorption↗

[On osteolysis].

Explore the source record for details and available documents.

Adolescent↗

Osteopenia in insulin treated diabetes mellitus. Its relation to age at onset, sex and duration of disease.

Bone mineral content was measured by photon absorptiometry in 215 insulin treated diabetic out-patients aged 7--70 years. As bone mass increases until the age of 20--25 years, patients were so selected that all remained within the same phase of bone mineral storage throughout the entire course of their diabetes. Other criteria for exclusion were diseases or drugs interfering with mineral metabolism and previous use of oral antidiabetic agents. As a group the patients demonstrated a bone mineral deficit of 9.8% compared with sex- and age-matched controls (P less than 0.001). Comparison between patients who had developed diabetes before the age of 20 years and after that of 25 years revealed deficits of 14% and 7%, respectively (P less than 0.001). Sex differences were not observed. The initiation of osteopenia seemed to coincide with the onset of clinical diabetes mellitus, and significantly reduced bone mineral content was observed after 2 years of diabetes (P less than 0.001). After 3--5 years the osteopenia appeared to attain a stable level.

Adolescent↗

Massive osteolysis of the pelvis, femur and sacral bone with a Gorham-Stout syndrome.

We report a case of a 49-year-old white woman with Gorham-Stout syndrome. This very rare massive osteolysis occurred in the left pelvis and included the femur and the sacral bone. Histology study revealed typical ectatic vessels covered with endothelium. The therapy of choice in this region is radiotherapy with a dose of 40 Gy. However, in our case this therapy only led to short-term pain relief and cessation of the osteolysis. Stabilization with corticospongious bone did not seem to be effective against the progressing osteolysis.

Female↗

Cytochemical localization of alkaline and acid phosphatase in human vanishing bone disease.

This report is the first cytochemical investigation of vanishing bone disease "Gorham's Disease" (Gorham and Stout 1955). The ultrastructural localization of non-specific alkaline phosphatase and of specific and non-specific acid phosphatase activity was studied in slices of tissue removed from a patient with this rare disorder. Sodium beta-glycerophosphate and phosphorylcholine chloride were used as substrates. Alkaline phosphatase was present around the plasma membranes of osteoblasts and associated with extracellular matrix vesicles in new woven bone. This is consistent with the proposed role for this enzyme (Robison 1923) and for matrix vesicles (Bonucci 1967) in the mineralization of bone (Bernard and Marvaso 1981). Concentrations of specific secretory acid phosphatase reaction product in the cytoplasm of degenerating osteoblasts may contribute to the imbalance between bone formation and resorption. Osteoclasts, while few in number, showed non-specific and specific acid phosphatase activity. The Golgi apparatus and heterophagic lysosomes of mononuclear phagocytes were rich in non-specific acid phosphatase. This was also present in the Golgi lamellae and lysosomes of endothelial cells. Acid phosphatase cytochemistry suggests that mononuclear phagocytes, multinuclear osteoclasts and the vascular endothelium are involved in bone resorption in this disease.

Acid Phosphatase↗

Case report 803: Gorham's disease (Gorham-Stout syndrome) of scapula.

In summary, a 36-year-old man presented with pain and limited motion in the shoulder. Clinical examination revealed obliteration of normal scapular landmarks in the conspicuous absence of any palpable soft tissue mass. Roentgenograms showed progressive osteolysis of the scapula. Biopsy confirmed the diagnosis of Gorham's disease. MRI played a key role in defining the extent of disease involvement and in displaying the distinct soft tissue anatomy. These MRI features are to our knowledge previously undescribed.

Adult↗

Hajdu-Cheney syndrome: MR imaging.

Hajdu-Cheney syndrome is a rare congenital disease with acro-osteolysis, osteoporotic changes of the spine and long bones of extremities and marked basilar invagination with an unusually deformed skull. Magnetic resonance imaging of a 32-year-old male revealed the deformed skull and almost horizontal basal angle and the elongated and upwardly shifted brain stem caused by the tip of the odontoid process of the second cervical vertebra invaginating the base of the skull. In addition there were atrophic pituitary gland, widely open sella turcica and symmetrical fluid collections along the optic nerve sheath.

Adult↗

Abnormal glomerular basement membrane in idiopathic multicentric osteolysis.

The primary cause of nephropathy in idiopathic multicentric osteolysis is as yet unknown. We report a young girl with idiopathic multicentric osteolysis and nephropathy. An abnormal glomerular basement membrane was the only abnormality found in a renal biopsy taken 2 years before the development of end-stage renal failure. We believe that this biopsy finding represents or is related to the unknown primary lesion causing nephropathy in idiopathic multicentric osteolysis.

Basement Membrane↗

Multicentric osteolysis: report of the second successful renal transplant.

A patient with end stage renal disease due to sporadic idiopathic multicentric osteolysis (type 3 multicentric osteolysis) is described. His pre-transplant course was similar to those of previously described patients, while his post-transplant course has been uncomplicated. The pathology and pathogenesis of the nephropathy of sporadic idiopathic multicentric osteolysis is not well characterized. The short term outcome of renal transplantation is excellent in our patient and in the other similar case known to have been transplanted.

Adolescent↗

Massive osteolysis of the skull: long-term follow-up observations after cranioplasty. Report of two cases.

Two cases of massive osteolysis involving the cranial vault are presented. Unlike disappearing bones in other regions, it is vital to protect the brain from injury by performing cranioplasty once the bone loss has reached a reasonable extent. This appears to be the first report on surgical management in massive osteolysis of the skull. Long term follow-up results are given. The diagnosis and differentiation from related conditions are discussed.

Adult↗