Lesions of bone in disseminated infection due to the mycobacterium avium-intracellulare group. Report of a case. (57/1036)

A white child with a long history of illness from the age of six was thought at first to have Hodgkin's disease. There followed an acute illness with lesions involving glands, lungs, bone and skin. Mycobacterium avium-intracellulare group (Battey) was isolated from various lesions at the age of thirteen. After six years of continuous treatment the patient, now eighteen, is living a normal life.  (+info)

The use of polymethylmethacrylate in the management of hydatid disease of bone. (58/1036)

Hydatid disease of bone is rare. It probably represents between 0.5% and 4% of all human hydatid disease and, in about 60% of patients, affects the spine or pelvis. Between 1986 and 1998, we treated 15 cases of bone hydatidosis. Curettage, swabbing with povidone iodine and filling the defect with polymethylmethacrylate (PMMA) were carried out in ten patients. Three of these had a recurrence after five years, but seven had no signs of relapse during a mean follow-up of 52 months. We believe that the combination of antihelminthic therapy, wide resection and the use of PMMA gives the best outcome in the treatment of bone hydatidosis.  (+info)

Comparison of Pkd1-targeted mutants reveals that loss of polycystin-1 causes cystogenesis and bone defects. (59/1036)

A high level of polycystin-1 expression is detected in kidneys of all patients with autosomal dominant polycystic kidney disease (ADPKD). Mice that overexpress polycystin-1 also develop renal cysts. Whether overexpression of polycystin-1 is necessary for cyst formation is still unclear. Here, we report the generation of a targeted mouse mutant with a null mutation in Pkd1 and its phenotypic characterization in comparison with the del34 mutants that carry a 'truncation mutation' in Pkd1. We show that null homozygotes develop the same, but more aggressive, renal and pancreatic cystic disease as del34/del34. Moreover, we report that both homozygous mutants develop polyhydramnios, hydrops fetalis, spina bifida occulta and osteochondrodysplasia. Heterozygotes also develop adult-onset pancreatic disease. We show further that del34 homozygotes continue to produce mutant polycystin-1, thereby providing a possible explanation for increased immunoreactive polycystin-1 in ADPKD cyst epithelia in the context of the two-hit model. Our data demonstrate for the first time that loss of polycystin-1 leads to cyst formation and defective skeletogenesis, and indicate that polycystin-1 is critical in both epithelium and chondrocyte development.  (+info)

Bone disease after liver transplantation. (60/1036)

1. Bone disease is a common problem in patients with chronic liver disease and liver transplants. 2. The cause of bone disease in these patients is multifactorial. 3. Bone disease worsens initially after liver transplantation, with subsequent improvement over time. However, bone disease in liver transplant recipients is common with long-term follow-up. 4. Evaluation of these patients should include metabolic and hormonal evaluations in conjunction with dual energy x-ray absorptiometry or bone mineral density evaluation. 5. Treatment with calcium, vitamin D, and hormonal supplements should be considered when appropriate for patients awaiting and after liver transplantation. The use of bisphosphanates and calcitonin also should be considered, although published studies in these populations are few in number.  (+info)

Correlation between osteochondral changes depicted by magnetic resonance imaging and disease progression. (61/1036)

PURPOSE: To determine the consequences of the chronic use of systemic corticosteroids in children with juvenile rheumatoid arthritis by means of evaluating osteochondral effects depicted by magnetic resonance imaging. PATIENTS AND METHODS: We reviewed clinical and magnetic resonance imaging findings in 69 children (72 knees) with juvenile rheumatoid arthritis. Two groups were studied. Group I: 34 (49.3%) children had previous or current use of systemic corticotherapy (22 girls; 12 boys; mean age: 11.3 years; mean disease duration: 5.9 years; mean corticotherapy duration: 2.9 years; mean cumulative dose of previous corticosteroids: 5000 mg); Group II: 35 (50.7%) children had no previous use of corticosteroids (27 girls; 8 boys; mean age: 11.7 years; mean disease duration: 5.3 years). The groups were compared statistically. RESULTS: In the group that had received corticotherapy (Group I), osteochondral abnormalities were significantly correlated to long-standing disease (>3.5 years; p<0.001). This correlation was not found in the group that had no previous history of corticotherapy (Group II). No correlations were established between median dose of corticosteroids and magnetic resonance imaging findings. CONCLUSION: It is important to further investigate the long-term intra-articular effects of systemic corticotherapy to ensure that the side effects of the aggressive therapy will not be more harmful for the joints than the symptoms suffered over the natural course of the disease.  (+info)

The use of plate fixation in anterior surgery of the degenerative cervical spine: a comparative prospective clinical study. (62/1036)

The method of anterior mono- or bisegmental cervical spine fusion is a well-established procedure for degenerative conditions of the cervical spine. While the early reports promote fusion with bone graft alone, recent studies report superior results with the addition of anterior plating. The objective of this study was to evaluate the influence of using plates in anterior cervical spine fusion in a prospective study. Fifty candidates for anterior monoor bisegmental cervical spine fusion were randomly and prospectively selected and assigned to a plated and a non-plated group. After a minimum follow-up of 22 months, patients were clinically and radiologically examined. The reduction in pain, improvement in neurology and functional assessment showed a significant improvement in both groups compared to the preoperative values. The total neurological score improved significantly in both groups, but the changes were greater in the group with plates. There was no significant difference between the groups for fusion rating, but graft quality (graft height) was significantly better in the plated group. We conclude that the overall data do not suggest better results with plating in mono- or bisegmental anterior spine fusions. Indications for additional internal fixation are restricted to special conditions with increased instability, insufficient bone quality or inappropriate graft placing.  (+info)

Hieronymus Bosch (1450-1516): paleopathology of the medieval disabled and its relation to the Bone and Joint Decade 2000-2010. (63/1036)

BACKGROUND: At the start of the Bone and Joint Decade 2000-2010, a paleopathologic study of the physically disabled may yield information and insight on the prevalence of crippling disorders and attitudes towards the afflicted in the past compared to today. OBJECTIVE: To analyze "The procession of the Cripples," a representative drawing of 31 disabled individuals by Hieronymus Bosch in 1500. METHODS: Three specialists--a rheumatologist, an orthopedic surgeon and a neurologist--analyzed each case by problem-solving means and clinical reasoning in order to formulate a consensus on the most likely diagnosis. RESULTS: This iconographic study of cripples in the sixteenth century reveals that the most common crippling disorder was not a neural form of leprosy, but rather that other disorders were also prevalent, such as congenital malformation, dry gangrene due to ergotism, post-traumatic amputations, infectious diseases (Pott's, syphilis), and even simulators. The drawings show characteristic coping patterns and different kinds of crutches and aids. CONCLUSION: A correct clinical diagnosis can be reached through the collaboration of a rheumatologist, an orthopedist and a neurologist. The Bone and Joint Decade Project, calling for attention and education with respect to musculoskeletal disorders, should reduce the impact and burden of crippling diseases worldwide through early clinical diagnosis and appropriate treatment.  (+info)

TBX-3, the gene mutated in Ulnar-Mammary Syndrome, is a negative regulator of p19ARF and inhibits senescence. (64/1036)

Prolonged culturing of rodent cells in vitro activates p19(ARF) (named p14(ARF) in man), resulting in a p53-dependent proliferation arrest known as senescence. The p19(ARF)-Mdm2-p53 pathway also serves to protect primary cells against oncogenic transformation. We have used a genetic screen in mouse neuronal cells, conditionally immortalized by a temperature-sensitive mutant of SV40 large T antigen, to identify genes that allow bypass of senescence. Using retroviral cDNA expression libraries, we have identified TBX-3 as a potent inhibitor of senescence. TBX-3 is a T-box gene, which is found mutated in the human developmental disorder Ulnar-Mammary Syndrome. We have shown that TBX-3 potently represses expression of both mouse p19(ARF) and human p14(ARF). We have also shown here that point mutants of TBX-3, which are found in Ulnar-Mammary Syndrome, have lost the ability to inhibit senescence and fail to repress mouse p19(ARF) and human p14(ARF) expression. These data suggest that the hypoproliferative features of this genetic disorder may be caused, at least in part, by deregulated expression of p14(ARF).  (+info)