1/83. Angiomatous osteolysis of the skull vault.A case is reported of angiomatous osteolysis of the calvarium. This condition normally affects long bones and there is only one previous report in literature of massive osteolysis of the skull. The clinical, radiological and pathological implications of this condition are considered.- - - - - - - - - - ranking = 1keywords = osteolysis (Clic here for more details about this article) |
2/83. Severe osteoporosis in familial hajdu-cheney syndrome: progression of acro-osteolysis and osteoporosis during long-term follow-up.hajdu-cheney syndrome is an autosomal dominant inherited osteodysplastic bone disease with the hallmarks of acro-osteolysis, skull deformations, and generalized osteoporosis. Very few patients have been followed long-term with respect to the prognosis of acro-osteolysis and osteoporosis. Here we describe a 39-year-old woman and her 19-year-old daughter who are both affected with the hajdu-cheney syndrome. Skeletal lesions were followed in the mother between the ages of 22 and 39 years. The acro-osteolytic lesions progressed markedly and caused shortening of several fingers; some end phalanges had completely disappeared. Severe spinal osteoporosis with serial vertebral fractures was found at the age of 22 years. New vertebral fractures developed until the age of 33 years, but did not progress afterward. High turnover osteoporosis was found in the bone histology of iliac crest biopsies performed at the ages of 22 and 34 years. Bone mineral content (BMC) was strikingly decreased at the age of 34 years (T score -5.1 SD) and did not significantly change during further follow-up. In the daughter, BMC failed to increase between the ages of 12 and 19 years and was also markedly decreased (T score -4.4 SD). This suggests that osteoporosis in hajdu-cheney syndrome is related to a low peak bone mass and a high bone turnover, leading to insufficient bone formation compared with the increased bone resorption.- - - - - - - - - - ranking = 1keywords = osteolysis (Clic here for more details about this article) |
3/83. acro-osteolysis prior to diagnosis of leprosy.lysis (bone resorption) has been observed in a heterogeneous group of congenital and acquired bone disorders. leprosy is the main cause of peripheral neuropathy leading to acro-osteolysis in endemic countries. Pure neuritic leprosy, a less common form of the disease, is difficult to diagnose. Two unrelated leprosy patients with acropathy whose disease began as pure neuritic are discussed.- - - - - - - - - - ranking = 0.83333333333333keywords = osteolysis (Clic here for more details about this article) |
4/83. Hajdu--Cheney syndrome: evolution of phenotype and clinical problems.hajdu-cheney syndrome is a rare, autosomal dominant disorder comprising acroosteolysis of the distal phalanges with associated digital abnormalities, distinctive craniofacial and skull changes, dental anomalies, and proportionate short stature. The clinical and radiologic characteristics of hajdu-cheney syndrome develop and progress with age. Many of the medical problems that arise in this syndrome cluster in specific age ranges. case reports of six affected individuals in two additional families and a summary of the English literature is presented with emphasis on the changing physical findings and medical sequelae over time.- - - - - - - - - - ranking = 0.16666666666667keywords = osteolysis (Clic here for more details about this article) |
5/83. Massive osteolysis of the humerus with spontaneous recovery.An elderly woman presented with a pathological fracture of the right humerus. Progressive dissolution of the shaft of this bone took place over six months. No cause could be established and the patient refused biopsy. With only simple splintage for treatment the humeral shaft gradually reformed and re-ossified over a period of two years. The patient has been under review for four and a half years and no further pathology has come to light. The cause of the osteolysis remains obscure.- - - - - - - - - - ranking = 0.83333333333333keywords = osteolysis (Clic here for more details about this article) |
6/83. Posttraumatic osteolysis of the distal clavicle: analysis of 7 cases and a review of the literature.OBJECTIVE: To discuss the clinical manifestation, radiographic features, and treatment of 7 cases of posttraumatic osteolysis of the distal clavicle. Also, to furnish evidence indicating that the current terminology for this disorder is ambiguous and to propose a new classification system. Clinical Features: Three cases resulted from acute trauma, and 4 cases were caused by sports-related repetitive microtrauma. All the cases involved young male patients who had similar clinical presentations that included shoulder pain with decreased shoulder range of motion. Radiographic findings ranged from small erosive changes to aggressive osteolysis of the distal clavicle. Intervention and Outcome: All the patients were treated with conservative care consisting of sling immobilization, ice, various physiotherapeutic modalities, and mobilization exercises. The patients that followed through with care showed clinical improvement within 3 months. Follow-up radiographic examinations, when performed, demonstrated reconstitution of the distal clavicle of various degrees, although lagging behind clinical evidence of improvement. CONCLUSION: Traumatic osteolysis of the distal clavicle may result from acute trauma or repetitive microtrauma. Radiographic changes are varied, including irregularity of the distal clavicle, cystic erosions, and blatant osteolysis. Positive outcomes may be achieved with conservative care; however, patient compliance plays a fundamental role in the overall prognosis.- - - - - - - - - - ranking = 1.3333333333333keywords = osteolysis (Clic here for more details about this article) |
7/83. Carpal and tarsal osteolysis.A 19-year-old girl with carpal and tarsal osteolysis has been presented. The clinical course was characterized by normal early growth and motor development followed by the insidious onset in early childhood of a progressive, peripheral joint destruction, especially in the tarsal and carpal bones. The patient's course was complicated by acquired spasticity from platybasia, scoliosis, generalized muscle weakness, mild growth failure and corneal clouding.- - - - - - - - - - ranking = 0.83333333333333keywords = osteolysis (Clic here for more details about this article) |
8/83. Hadju-Cheney syndrome: response to therapy with bisphosphonates in two patients.Hadju-Cheney syndrome is characterized by short stature, distinctive facies, and a slowly progressive skeletal dysplasia including acro-osteolysis. Autosomal dominant inheritance is typical, but the genetic defect and molecular pathogenesis of the syndrome are unknown. osteoporosis with atraumatic fracture is a frequent finding, and previous studies have documented biochemical and morphometric evidence of high bone turnover. Here, we report the clinical details and response to therapy with bisphosphonates in two patients (mother and son) with Hadju-Cheney syndrome and postulate that osteoclast-mediated bone resorption is important in the generalized osteoporosis commonly associated with this condition.- - - - - - - - - - ranking = 0.16666666666667keywords = osteolysis (Clic here for more details about this article) |
9/83. The acro-osteolysis syndrome: Morphologic and biochemical studies.The acro-osteolysis syndrome consists of dissolution of terminal phalanges of the hands and feet, dolichocephaly with multiple wormian bones, delayed closure of cranial sutures, absence of frontal sinuses, a prominent occipital ridge, skeletal demineralization, vertebral and extremity fractures, joint laxity, and coarse hair. Studies of bone morphology reveal diminished bone density and bone formation. osteoblasts have widely dilated smooth endoplasmic reticulum. It is postulated that an abnormality of a structural protein is the pathogenic basis of this disease.- - - - - - - - - - ranking = 0.83333333333333keywords = osteolysis (Clic here for more details about this article) |
10/83. Distal phalangeal brachydactyly and osteosclerosis in a case of secondary hyperparathyroidism.A case of severe hyperparathyroidism (HPT), secondary to chronic renal failure, with massive distal phalangeal osteolysis is reported. After parathyroidectomy, brachydactyly and osteosclerosis of the distal phalanges were observed; the phalanx of the thumbs healed with shortened and sclerotic beaked appearance. These radiographic findings are actually uncommon because of more efficient treatment of renal osteodystrophy. Pathophysiology of this bone sclerotic lesion is discussed, and other causes of distal phalangeal osteosclerosis are mentioned.- - - - - - - - - - ranking = 0.16666666666667keywords = osteolysis (Clic here for more details about this article) |
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