[Algodystrophy. Etiology, diagnosis, course, treatment].
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Female↗
Biomedical subjects
D Liens
Publications and source records attributed to D Liens.
Long-term effects of intravenous pamidronate in fibrous dysplasia of bone.
Fibrous dysplasia of bone (FD) is a rare disorder characterized by proliferation of fibrous tissue in bone marrow leading to osteolytic lesions. It causes bone pain and fractures. To date the only treatment is orthopedic. Histological and biochemical similarities between FD and Paget's bone disease related to increased osteoclastic resorption led us to propose treatment with the bisphosphonate pamidronate. The aim of the study was to assess the long-term effects of intravenous pamidronate in FD. In this open label phase III study, 20 patients with FD (11 males and 9 females; mean age 31 years) received courses of 180 mg of intravenous pamidronate every 6 months (60 mg/day during 3 days by infusion). The mean duration of follow-up was 39 months (range 18-64). Severity of bone pain, number of painful skeletal sites per patient, X-rays of all involved areas, serum alkaline phosphatase, fasting urinary hydroxyproline, and urinary type I collagen C-telopeptide were assessed every 6 months. The severity of bone pain and the number of painful sites appeared to be significantly reduced. All biochemical markers of bone remodeling were substantially lowered. We observed a radiographic response in nine patients with refilling of osteolytic lesions. A mineralization defect proven by bone biopsy was observed in one case. Four patients sustained bone stress lines, but no fracture occurred. We suggest that intravenous pamidronate alleviates bone pain, reduces the rate of bone turnover assessed by biochemical markers, and improves radiological lesions of FD. Few side effects were observed.
Adolescent↗
Dual energy X-ray absorptiometry in patients with lower limb reflex sympathetic dystrophy syndrome.
OBJECTIVE: To assess the difference in bone mineral density (BMD) between pathologic and healthy legs in reflex sympathetic dystrophy syndrome (RSDS) using a whole body BMD with dual energy X-ray absorptiometry (DEXA). METHODS: Cross sectional evaluation of BMD in 55 patients with RSDS compared BMD of the affected and the healthy limb with 121 controls. Followup was performed on 21 patients treated with intravenous pamidronate. RESULTS: In the cross sectional study, BMD was reduced in the dystrophic affected extremities and this reduction correlated with the duration of evolution of the disease. In the longitudinal study, BMD remained stable in patients treated with pamidronate. CONCLUSION: The cross sectional study confirms that DEXA is not a diagnostic tool. The longitudinal study confirms DEXA is accurate, nontraumatic, rapid, and safe for longterm quantitative assessment of unilateral bone loss caused by lower limb RSDS.
Absorptiometry, Photon↗
Long-term effects of intravenous pamidronate in fibrous dysplasia of bone.
Nine patients with symptomatic and severe fibrous dysplasia were treated with intravenous pamidronate (60 mg per day over 3 days every sixth month), and were followed up for 18-48 months. The major effect was decreased bone pain (complete remission in 12 of 14 sites). Radiological changes were seen in four patients, with thickening of cortices, refilling of osteolytic lesions, or both. The initial increased bone remodelling was reduced, as shown by decrease of raised serum alkaline phosphatase and urinary hydroxyproline. The treatment was well tolerated, but a 13-year-old patient showed widening of knee growth-plates which is consistent with a transient mineralisation defect.
Adolescent↗
[Biphosphonates in the treatment of osteolytic metastasis. Essential actions on hypercalcemia and important on lytic lesions].
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Bone Neoplasms↗
[Aseptic osteomyelitis].
Based on a series of eight personal cases and a review of the literature, this study tries to define the diagnostic elements of aseptic osteomyelitis. Although sternocostoclavicular sites are immediately suggestive of the diagnosis, lesions of the pelvis and spine and long bones of the limbs are particularly difficult to diagnose. Inflammatory type pain occurred in episodesover a number of years and responded to NSAIs and possibly diphosphonates. Laboratory abnormalities were usually confined to a raised ESR, but alterations of the IgA levels, similar to those observed in ankylosing spondylitis were observed in four cases. Hyperostosis occurred late in the course, preceded by signs of osteomyelitis, periosteitis or enthesitis. Histology does not provide any formal conclusions. The most suggestive lesions are those of nonspecific aseptic osteomyelitis, followed by a Paget-like appearance. The interpretation of these findings may be complicated by the presence of eosinophils, giant cells or mast cells. Two elements are very valuable for establishing the diagnosis: the presence of infraradiological anterior thoracic increased uptake on bone scan and the presence of a skin disorder, either severe acne or, more especially, palmoplantar pustulosis.
Adolescent↗