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Biomedical subjects

Christophe Glorion

Publications and source records attributed to Christophe Glorion.

16 recordsLinked to original sources

Lumbar hyperlordosis of neuromuscular origin: pathophysiology and surgical strategy for correction.

Lumbar hyperlordosis of neuromuscular origin is rare and requires surgical treatment in order to preserve a good sitting posture. We report twenty-seven cases of a preponderantly sagittal hyperlordosis deformity of the lumbar spine in patients with neuromuscular disorders and identify the indications and results of treatment. Seventeen males and ten females, aged 13 to 27 years, underwent operations for a lumbar hyperlordosis of neuromuscular origin responsible for major difficulties in sitting. In all patients, the sacrum was horizontal and associated in twenty-six cases with marked pelvic anteversion. Eleven patients were treated surgically by a posterior approach. The sixteen remaining patients had a preliminary discectomy, followed by posterior correction and fusion. Lumbar hyperlordosis was reduced from 8 degrees to 77 degrees between L1 and S1. The horizontal sacrum was partially reduced with an improvement from 8 degrees to 50 degrees . Consequently, patients recovered a comfortable sitting position. One patient died of respiratory complications six weeks after surgery. Surgical correction is a demanding procedure which can be performed by a posterior approach. It is mandatory to analyse the spino-pelvic balance to avoid iliac retroversion and the loss of the role of the ischia in the sitting position.

Adolescent↗

Development of a broad-range 16S rDNA real-time PCR for the diagnosis of septic arthritis in children.

The broad-range PCR has been successfully developed to search for fastidious, slow-growing or uncultured bacteria, and is mostly used when an empirical antibiotic treatment has already been initiated. The technique generally involves standard PCR targeting the gene coding for 16S ribosomal RNA, and includes a post-PCR visualisation step on agarose gel which is a potential source of cross-over contamination. In addition, interpretation of the presence of amplified products on gels can be difficult. We then developed a new SYBR Green-based, universal real-time PCR assay targeting the gene coding for 16S ribosomal RNA, coupled with sequencing of amplified products. The real-time PCR assay was evaluated on 94 articular fluid samples collected from children hospitalised for suspicion of septic arthritis, as compared to the results obtained with bacterial cultures and conventional broad-range PCR. DNA extraction was performed with the automated MagNa Pure system. We could detect DNA from various bacterial pathogens including fastidious bacteria (Kingella kingae, Streptococcus pneumoniae, Streptococcus pyogenes, Salmonella spp, Staphylococcus aureus) from 23% of cases of septic arthritis giving negative culture results. The real-time technique was easier to interpret and allowed to detect four more cases than conventional PCR. PCR based molecular techniques appear to be essential to perform in case of suspicion of septic arthritis, provided the increase of the diagnosed bacterial etiologies. Real-time PCR technique is a sensitive and reliable technique, which can replace conventional PCR for clinical specimens with negative bacterial culture.

Adolescent↗

Severe osteoarticular infection associated with Panton-Valentine leukocidin-producing Staphylococcus aureus.

We report here a case of osteoarticular infection associated with Panton-Valentine leukocidin-producing Staphylococcus aureus. The 3-year-old child presented osteoarthritis associated with toxic shock syndrome, which was complicated by thrombosis, pneumonia, and diaphysitis, despite appropriate antibiotic treatment. Osteitis associated with toxinogenic S. aureus is rarely described. This case highlights the need of early and aggressive surgical treatment, in addition to appropriate antimicrobial therapy.

Arthritis, Infectious↗

[Kyphosis and kyphotic posture in children and adolescents].

Kyphosis is a sagittal deviation of the spine that presents as an anterior spinal curvature. It is a frequent reason for consultation during adolescence. It is necessary to know how to differentiate between a simple postural kyphosis that is flexible and reducible, and requires no further investigation, from a true kyphosis that requires at minimum a radiological evaluation with films of the entire spine, in AP and lateral views. If vertebral epiphysitis is most often at the origins of the sagittal vertebral deviations encountered in daily practice, the other causes pose diagnostic and therapeutic problems that are often more complex. They include congenital, paralytic, infectious, cancerous and post-traumatic kyphosis, or kyphosis due to constitutional bone diseases. Surgical treatments, that have only an exceptional place in treatment of the regular kyphosis of the adolescent, are potentially indicated in all of the other situations.

Adolescent↗

Surgical treatment of high-grade lumbosacral spondylolisthesis in childhood, adolescent and young adult by the "double-plate" technique: a past experience.

Between 1979 and 1996, 40 patients with high-grade lumbosacral spondylolisthesis were treated in our institution using a newly designed osteosynthesis device. The mean age was 13 years and 6 months, and the mean follow-up was 18 years. Combined posterior decompression and anterior reduction, instrumentation and fusion of the slippage were performed in all cases. The technique includes reduction of the slippage by means of an anteriorly placed plate that engages two screws, previously placed during the posterior approach, going through the S1 vertebra. Progressive compression applied on the plate by the screws achieves reduction. Complete fusion was obtained in all 40 patients. Twelve patients presented a postoperative radiculopathy, from which only ten recovered completely. There were six L4-L5 annulus lesions, responsible for instability, produced by the plate. We report five late infections. Thirty-five of the forty patients were asymptomatic at the latest follow-up. The double compressive plate technique proved to be effective in obtaining lumbosacral fusion and optimal slippage reduction. However, the high rates of neurological and infectious complications preclude recommendation of this technique in its present form.

Adolescent↗

Radiological assessment of lumbosacral dystrophic changes in high-grade spondylolisthesis.

OBJECTIVE: To analyse radiographic correlates for the clinical status of patients and the deformation reducibility of high-grade lumbosacral spondylolisthesis. We also clarify the clinical and radiographic correlates of a new parameter for S1 dystrophy, the "S1 index". DESIGN AND PATIENTS: One hundred cases of high-grade isthmic lumbosacral spondylolisthesis were reviewed. We noted the dystrophic changes in the cranial sacral endplate, and the caudal endplate of L5. The severity of the spondylolisthesis was evaluated by measuring the lumbosacral kyphosis. The clinical status and the deformation reducibility (dependent on the stiffness of the deformation) were compared with these dystrophic patterns, the sagittal slope of S1 and S2 endplates and a sacral morphological marker, the S1 index. RESULTS: Lumbosacral kyphosis was less severe in cases with dystrophic changes of the posterior cranial edge of S1 and/or of the posterior caudal edge of L5 but its reducibility was worse. These patients were more functionally impaired. We describe and analyse this situation as a partial lumbosacral disc failure responsible for the less severe L5 slipping. The S1 index was strongly correlated with the grade of slipping, the lumbosacral kyphosis and its reducibility. We noted the same configuration among patients with a smaller S1 index, i.e. vertical S1 and S2 vertebral bodies associated with more severe but more reducible lumbosacral kyphosis. CONCLUSION: Analysing specific criteria, we think it is possible to note progressive dystrophic changes according to the natural history of lumbosacral spondylolisthesis. We think that repeated measurements of these morphological parameters in patients diagnosed with a low-grade lumbosacral spondylolisthesis could be helpful in the early detection of evolving lumbosacral kyphosis and L5 slipping.

Adolescent↗

DOR-1, A novel CD10+ stromal cell line derived from progressive Langerhans cell histiocytosis of bone.

BACKGROUND: Langerhans cell histiocytosis (LCH) is granulomatous proliferative disorder characterized by the presence of activated Langerhans cells admixed with macrophages, lymphocytes, and eosinophils. In an effort to obtain an LCH ex vivo model, we succeeded in establishing the DOR-1 cell line from an LCH lesion of bone in a 3-year-old girl. PROCEDURE: The DOR-1 cell line was established from a CD1a immunoreactive LCH lesion of bone maintained in long-term cell culture. The phenotypic characteristics were assessed by immuno-cytochemistry and fluorescence activated cell sorter (FACS) analysis. Cytogenetic analysis was performed by RHG-banding that was supplemented by fluorescence in situ hybridization (FISH). RESULTS: The DOR-1 cells grew in vitro as a poorly differentiated mesenchymal-like cells with a doubling time between 72 and 96 hr. The cells exhibited pleomorphism and consistent immuno-reactivity for CD10 (50%), CD13 (55%), CD68 (65%), and CD117 (70%) while CD1a, Langerin and HLA-DR were not detected. By RHG-banding, several aberrant chromosomes were detected including the t (9; 17) (p23; p13) translocation and a pair of long dicentric marker chromosomes indicating clonal abnormality. Functionally, exposure to 33 nM 12-O-tetradecanoyl phorbol mirystate-13-acetate (TPA) induced DOR-1 cell differentiation with appearance of cytoplasmic extensions. CONCLUSIONS: The DOR-1 cell line exhibits distinct immuno-cytochemical features and carries the t (9; 17) (p23; p13) translocation suggesting involvement of stromal-like cell lineage in LCH initiation and progression.

Animals↗

Imaging of traumatic dislocation of the hip in childhood.

BACKGROUND: Traumatic hip dislocation in childhood is a rare consequence of violent trauma. After reduction, outcome is usually favourable although epiphyseal necrosis can occur. Reduction must be carried out as soon as possible and is achieved easily, although if the labrum is involved, surgery may be required to achieve complete reduction. OBJECTIVE: To analyze a retrospective series of traumatic hip dislocations in children, describing the therapeutic and imaging strategy. MATERIALS AND METHODS: A total of 42 patients were studied. Their mean age was 10 years 3 months. All relevant radiographic, CT, MRI and radionuclide bone scan examinations were reviewed. Special attention was paid to associated lesions. RESULTS: In 22 patients the dislocation was caused by low-energy trauma. Road traffic accidents accounted for 17 dislocations. An acetabular fracture was present in six patients and the femoral head was fractured in three. Reduction was easily achieved in 31 patients. In 11 patients the postreduction radiograph and CT showed joint space asymmetry secondary to labral entrapment. Only two patients developed epiphyseal necrosis. CONCLUSIONS: It has been difficult to define and evaluate accurate principles for a medical imaging strategy in this group of patients. Analysis of plain radiographs is essential before and after reduction of the joint, and it is important to perform postreduction CT in every patient whose joint space remains widened. A radionuclide bone scan should be performed between the second and third weeks after injury to assess epiphyseal vascularity. With the use of specific sequences, MRI may be an alternative modality to assess epiphyseal vitality.

Acetabulum↗

Imaging of malignant tumours of the long bones in children: monitoring response to neoadjuvant chemotherapy and preoperative assessment.

This review focuses on imaging of osteosarcoma and Ewing's sarcoma of the long bones in children during preoperative neoadjuvant chemotherapy. Morphological criteria on plain films and conventional static MRI are insufficiently correlated with histological response. We review the contribution of dynamic MRI, diffusion-weighted MR and nuclear medicine (18FDG-PET) to monitor tumoural necrosis. MRI is currently the best method to evaluate local extension prior to tumour resection, especially to assess the feasibility of conservative surgery. Quantitative models in dynamic MRI and 18FDG-PET are currently being developed in order to find new early prognostic criteria, but for the time being, treatment protocols are still based on the gold standard of histological response.

Adolescent↗

Osteomyelitis in infants and children.

Infection of the pediatric skeletal system is a dramatic diagnostic and therapeutic challenge. The consequences of a missed diagnosis could lead to long-lasting functional sequelae. This disease has multiple aspects according to the clinical presentation and evolution, the causative microorganism, the site of infection, and, finally, the patient's age and immunological status. The choice of the most appropriate imaging modality according to the clinical presentation is very important to assess the diagnosis, the extent of the disease, and to obtain the causative germ when needed; thus, plain films, ultrasound, nuclear medicine, computed tomography, and MR imaging are discussed, each of them having pros and cons. This multimodality approach of the musculoskeletal infections is mandatory to guide surgery, to deliver an appropriate antibiotic therapy, and to reduce the rate of long-term functional sequelae.

Age Distribution↗

Traumatic dislocation of the hip with separation of the capital epiphysis: 5 adolescent patients with 3-9 years of follow-up.

We reviewed 5 cases of type I epiphyseal fracture with dislocation of the femoral head from the acetabulum in adolescent patients. All children had an open reduction and screw fixation. In all cases, the femoral head developed avascular necrosis. The clinical result after a mean of 3-9 years' follow-up was good according to the Merle d'Aubigné-Postel scale. Despite necrosis, 2 heads developed spherically after treatment: one which had a primary physeal resection and fixation, the other after an autogenous bone graft in the screw track following removal of the screw.

Accidents, Traffic↗

Detection of molecular cytogenetic aberrations in langerhans cell histiocytosis of bone.

Langerhans cell histiocytosis (LCH) is a disorder of unknown etiology that gives rise to clonal expansion of Langerhans-like cells or their precursors. The molecular basis include aberrant expression of several adhesion molecules and elevated expression of p53, c-myc, and H-ras. To identify new locations of LCH-related oncogenes or tumor-suppressor genes, we performed comparative genomic hybridization (CGH) and loss of heterozygosity (LOH) analysis on a series of 7 bone LCH lesions. Recurrent abnormalities were found by the CGH in all cases representing losses of DNA sequences on chromosomes 1p, 5, 6, 7, 9, 16, 17, and 22q. Gain of DNA copy number was seen on chromosomes 2q, 4q, and 12. The CGH data were supplemented by LOH analysis by means of 85 polymorphic microsatellite markers distributed along chromosomes 1, 7, 9, and 22. The highest frequency of LOH was found on 1p region in 3 of 7 informative cases and on chromosome 7 in 4 cases. Allelic loss was also detected on chromosomes 9 in 2 of 7 informative cases and on 22q in 1 of 7 cases. These results indicate that the deleted chromosomal segments may contain genes important in LCH initiation and progression.

Adolescent↗

Another observation with VATER association and a complex IV respiratory chain deficiency.

The VATER association of vertebral anomalies (V), anal atresia (A), esophageal atresia and/or tracheo-esophageal fistula (TE), radial and renal anomalies (R) is a common congenital association of unknown origin with probably heterogeneous causes. Here, we report on a girl presenting with pre- and postnatal growth retardation, esophageal atresia, vertebral and costal anomalies and a unilateral radial defect, consistent with the diagnosis of VATER association. In the first month of life, she presented with failure to thrive, severe episodes of hypoglycemia, liver dysfunction and high levels of lactate, which prompted us to perform metabolic screening. A complex IV respiratory chain deficiency (RCD) was diagnosed on a liver biopsy. The respiratory chain defect was not observed in skin fibroblasts. No mtDNA point mutation or deletion was identified. The girl is now 9 years old and has a normal mental development but persistent feeding difficulties and moderate hyperlactatemia. To our knowledge, this is the second report of VATER association with mitochondrial disorder. In a previous report, a VACTERL association was observed in a girl with the mitochondrial A3243G point mutation. The association of VATER phenotype with a mitochondrial disorder may be coincidental but could also suggest that the presence of multiple malformations is the result of the antenatal expression of RCD.

Abnormalities, Multiple↗

Traumatic hip dislocation in childhood.

Traumatic dislocation of the hip in childhood is uncommon and can be a consequence of minor trauma. The authors report a series of 35 dislocations in skeletally immature patients. Most were isolated posterior dislocations without acetabular lesions. In 75% of cases, reduction of the dislocation was easy. Nine children required surgery to remove interposed joint capsule and/or osteochondral fragments to achieve anatomic reduction. Outcomes were generally good, except in one patient in whom a displaced fracture of the femoral physis was followed by total head avascular necrosis. One case of partial necrosis had a satisfactory outcome. Epiphyseal necrosis, though uncommon, appeared to be inconsistent to prevent and hard to predict. Bone scan seems to be more effective than MRI for the detection of necrosis.

Adolescent↗

Cementless hip arthroplasty in juvenile idiopathic arthritis.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. The hip is usually affected later, and its involvement is the most common cause of disability in JIA. Failure of medical and preventive treatment, significant joint destruction, or multiarticular involvement may necessitate total hip arthroplasty (THA) to restore good function. Related clinical trials show initially good THA results in most of the series, but long-term outcomes are uncertain due to a significant arthroplasty loosening rate in the initial years, particularly with cemented stems. The authors report the results of 62 noncemented THAs in 34 children with JIA after an average follow-up of 6 years (range 3-13). Mean age at surgery was 18.3 years (range 11.8-31) and 14 of the 34 children had active disease. Clinical results were good for hip function but less for global function. There were no infections. Two acetabular cups early in the series failed due to poor primary fixation and had to be revised. Survivorship analysis was performed with the Kaplan-Meier method. At 13 years, the survival rate was 100% for the femoral component and 90.1% for the acetabular component.

Adolescent↗