Topics (9) View all

Publications (9) View all

  • Source
    Article: The diagnosis and management of patients with idiopathic osteolysis.
    [show abstract] [hide abstract]
    ABSTRACT: ABSTRACT: Idiopathic osteolysis or disappearing bone disease is a condition characterized by the spontaneous onset of rapid destruction and resorption of a single bone or multiple bones. Disappearing bone disorder is a disease of several diagnostic types. We are presenting three patients with osteolysis who have different underlying pathological features. Detailed phenotypic assessment, radiologic and CT scanning, and histological and genetic testing were the baseline diagnostic tools utilized for diagnosis of each osteolysis syndrome. The first patient was found to have Gorham-Stout syndrome (non-heritable). The complete destruction of pelvic bones associated with aggressive upward extension to adjacent bones (vertebral column and skull base) was notable and skeletal angiomatosis was detected. The second patient showed severe and aggressive non-hereditary multicentric osteolysis with bilateral destruction of the hip bones and the tarsal bones as well as a congenital unilateral solitary kidney and nephropathy. The third patient was phenotypically and genotypically compatible with Winchester syndrome resulting in multicentric osteolysis (autosomal recessive). Proven mutation of the (MMP2-Gen) was detected in this third patient that was associated with 3MCC deficiency (3-Methylcrontonyl CoA Carboxylase deficiency). The correct diagnoses in our 3 patients required the exclusion of malignant osteoclastic tumours, inflammatory disorders of bone, vascular disease, and neurogenic arthropathies using history, physical exam, and appropriate testing and imaging. This review demonstrates how to evaluate and treat these complex and difficult patients. Lastly, we described the various management procedures and treatments utilized for these patients.
    Pediatric Rheumatology 01/2011; 9:31. · 1.44 Impact Factor
  • Article: Aseptic stem loosening in primary THA: migration analysis of cemented and cementless fixation.
    [show abstract] [hide abstract]
    ABSTRACT: Early migration has reportedly been predictive for later implant failure. Using four different migration patterns, this study aimed to analyse migration behaviour of the two types of implant fixation--cemented and cementless--throughout the process of loosening. Migrational behaviour of 69 revised stems (49 cemented, 20 uncemented) was analysed retrospectively with EBRA-FCA (Einzel-Bild-Röntgen-Analyse, Femoral Component Analysis). Uncemented stems failed after early and late onset migration alike, while late migration was the predominant pattern in cemented stems. Mean prosthetic failure after early migration occurred 5.8 (+/-4.4) years postoperatively due to insufficient primary stability. Initially stable stems with late onset migration were revised after 12.4 (+/-4.5) years. Measurement of early migration was found to be a valuable tool to screen short-term and mid-term failure. In the long run the method's sensitivity decreased. Late onset migration, however, preceded long-term failure by a mean of three years.
    International Orthopaedics 01/2009; 33(6):1501-5. · 2.03 Impact Factor
  • Article: High recurrence after calf lengthening with the Ilizarov apparatus for treatment of spastic equinus foot deformity.
    [show abstract] [hide abstract]
    ABSTRACT: Both gastrocnemius recession and Achilles tendon lengthening lead to scarring in the calf and have high reported recurrence rates when performed under the age of 8 years. Triceps surae lengthening by external fixation seemed to be a valuable alternative. Twelve calf lengthenings have been performed with an Ilizarov device with a mean correction of 27 degrees. No calcaneal gait was observed, but there was a slow continuous loss of dorsiflexion over the observation period. The Ilizarov technique has a higher recurrence rate than most operative procedures for calf lengthening, but carries virtually no risk in producing calcaneus. The technique cannot be recommended for routine clinical use and may only be an alternative for selected cases.
    Journal of Pediatric Orthopaedics B 04/2007; 16(2):125-8. · 0.47 Impact Factor
  • Article: A role of TRAIL in killing osteoblasts by myeloma cells.
    [show abstract] [hide abstract]
    ABSTRACT: In multiple myeloma (MM), neoplastic plasma cells accumulate in the bone marrow where their survival, proliferation, and apoptosis are controlled at multiple levels by interaction with the bone marrow microenvironment. Myeloma cells actively control these interactions by activating stromal and endothelial cells for production of survival factors, such as interleukin-6, and suppressing other cell types such as erythroblasts, normal B cell progenitors, and T-cells. In the present study, we identified primary osteoblasts as additional potential targets for myeloma cell-mediated suppression which was partly dependent on the death receptor ligand TRAIL. Besides killing of osteoblasts, myeloma cell lines sensitized osteoblasts to cell death mediated by recombinant TRAIL, whereas primary osteoblasts protected myeloma cells from TRAIL-mediated apoptosis that was mediated by osteoprotegerin (OPG). Besides increase of osteoclastogenesis and osteoclast activity, suppression of bone-forming cells by myeloma cells might contribute to bone loss in MM patients. In addition, clinical development of recombinant TRAIL as anti-myeloma therapy should include evaluation of potential side effects on viability of normal bone cells.
    The FASEB Journal 05/2006; 20(6):759-61. · 5.71 Impact Factor
  • Article: Assessment of talar flattening in adult idiopathic clubfoot.
    [show abstract] [hide abstract]
    ABSTRACT: The radius/length (R/L) ratio was developed to evaluate the convexity of the talar dome in idiopathic clubfoot. However, the index has not been tested for its reliability and reproducibility. The R/L ratio was determined by three independent observers on the radiographs of 21 adult patients with idiopathic clubfoot and 30 adult subjects with normal feet. The reproducibility and the reliability of the R/L ratio were calculated. For the normal feet the reproducibility and the reliability of the R/L ratio was high (correlation coefficient > 0.87). For the patients with clubfoot, the reliability and reproducibility depended on the severity of talar flattening. For a radius of less than 45 mm the mean intraobserver correlation coefficient was 0.74 (range 0.54 to 0.83) and the mean interobserver correlation coefficient was 0.58 (range 0.49 to 0.75). For a radius of more than 45 mm no statistically significant intraobserver and interobserver correlations were found. The current results indicate that the R/L ratio of talar flattening is reliable and reproducible for mild talar deformity but not for severe flattening (radius of more than 45 mm).
    The Foot and Ankle Online Journal 10/2005; 26(9):754-60. · 1.22 Impact Factor

Following (18) See all

Followers (10) See all