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1.  Orthopedic Management of Patients with Pompe Disease: A Retrospective Case Series of 8 Patients 
The Scientific World Journal  2014;2014:963861.
Introduction. Pompe disease (PD), a lysosomal storage disease as well as a neuromuscular disorder, is a rare disease marked by progressive muscle weakness. Enzyme replacement therapy (ERT) in recent years allowed longer survival but brought new problems to the treatment of PD with increasing affection of the musculoskeletal system, particularly with a significantly higher prevalence of scoliosis. The present paper deals with the orthopedic problems in patients with PD and is the first to describe surgical treatment of scoliosis in PD patients. Patients and Methods. The orthopedic problems and treatment of eight patients with PD from orthopedic consultation for neuromuscular disorders are retrospectively presented. We analyzed the problems of scoliosis, hip dysplasia, feet deformities, and contractures and presented the orthopedic treatment options. Results. Six of our eight PD patients had scoliosis and two young patients were treated by operative spine stabilization with benefits for posture and sitting ability. Hip joint surgery, operative contracture release, and feet deformity correction were performed with benefits for independent activity. Conclusion. Orthopedic management gains importance due to extended survival and musculoskeletal involvement under ERT. Surgical treatment is indicated in distinct cases. Further investigation is required to evidence the effect of surgical spine stabilization in PD.
doi:10.1155/2014/963861
PMCID: PMC3910124  PMID: 24523658
2.  Natural course of scoliosis in proximal spinal muscular atrophy type II and IIIa: descriptive clinical study with retrospective data collection of 126 patients 
Background
Progressive scoliosis, pelvic obliquity and increasing reduction of pulmonary function are among the most significant problems for patients with SMA type II and SMA type III once they have lost the ability to walk. The aim of this study was to examine and document the development and natural course of scoliosis in patients with spinal muscular atrophy type II and IIIa.
Methods
For the purposes of a descriptive clinical study, we observed 126 patients, 99 with SMA II and 27 with SMA IIIa and the data of scoliosis, pelvic obliquity and relative age-dependent inspiratory vital capacity were evaluated.
Results
Scoliosis and pelvic obliquity were regularly observed already in children under 4 years old in the group with SMA II. The severity and progression of both conditions were much more pronounced in the SMA II group than in the IIIa group. There was already a distinct reduction in relative vital capacity in the group of 4- to 6-year-olds with SMA II.
Conclusions
The differences between the two SMA types II and IIIa described in this study should be taken into consideration when developing new treatments and in management of scoliosis in the childhood years of these patients.
doi:10.1186/1471-2474-14-283
PMCID: PMC3850509  PMID: 24093531
Spinal muscular atrophy; Scoliosis; Pelvic obliquity; Pulmonary function; Natural course
3.  Impaction grafting in the femur in cementless modular revision total hip arthroplasty: a descriptive outcome analysis of 243 cases with the MRP-TITAN revision implant 
Background
We present a descriptive and retrospective analysis of revision total hip arthroplasties (THA) using the MRP-TITAN stem (Peter Brehm, Weisendorf, GER) with distal diaphyseal fixation and metaphyseal defect augmentation. Our hypothesis was that the metaphyseal defect augmentation (Impaction Bone Grafting) improves the stem survival.
Methods
We retrospectively analyzed the aggregated and anonymized data of 243 femoral stem revisions. 68 patients with 70 implants (28.8%) received an allograft augmentation for metaphyseal defects; 165 patients with 173 implants (71.2%) did not, and served as controls. The mean follow-up was 4.4 ± 1.8 years (range, 2.1–9.6 years). There were no significant differences (p > 0.05) between the study and control group regarding age, body mass index (BMI), femoral defects (types I-III as described by Paprosky), and preoperative Harris Hip Score (HHS). Postoperative clinical function was evaluated using the HHS. Postoperative radiologic examination evaluated implant stability, axial implant migration, signs of implant loosening, periprosthetic radiolucencies, as well as bone regeneration and resorption.
Results
There were comparable rates of intraoperative and postoperative complications in the study and control groups (p > 0.05). Clinical function, expressed as the increase in the postoperative HHS over the preoperative score, showed significantly greater improvement in the group with Impaction Bone Grafting (35.6 ± 14.3 vs. 30.8 ± 15.8; p ≤ 0.05). The study group showed better outcome especially for larger defects (types II C and III as described by Paprosky) and stem diameters ≥ 17 mm. The two groups did not show significant differences in the rate of aseptic loosening (1.4% vs. 2.9%) and the rate of revisions (8.6% vs. 11%). The Kaplan-Meier survival for the MRP-TITAN stem in both groups together was 93.8% after 8.8 years. [Study group 95.7% after 8.54 years ; control group 93.1% after 8.7 years]. Radiologic evaluation showed no significant change in axial implant migration (4.3% vs. 9.3%; p = 0.19) but a significant reduction in proximal stress shielding (5.7% vs. 17.9%; p < 0.05) in the study group. Periprosthetic radiolucencies were detected in 5.7% of the study group and in 9.8% of the control group (p = 0.30). Radiolucencies in the proximal zones 1 and 7 according to Gruen occurred significantly more often in the control group without allograft augmentation (p ≤ 0.05).
Conclusion
We present the largest analysis of the impaction grafting technique in combination with cementless distal diaphyseal stem fixation published so far. Our data provides initial evidence of improved bone regeneration after graft augmentation of metaphyseal bone defects. The data suggests that proximal metaphyseal graft augmentation is beneficial for large metaphyseal bone defects (Paprosky types IIC and III) and stem diameters of 17 mm and above. Due to the limitations of a retrospective and descriptive study the level of evidence remains low and prospective trials should be conducted.
doi:10.1186/1471-2474-14-19
PMCID: PMC3556053  PMID: 23311769
Arthroplasty; Hip; Revision; Modular; Impaction bone grafting
4.  Surgical treatment of Duchenne muscular dystrophy patients in Germany: the present situation 
Acta Myologica  2012;31(1):21-23.
In 1988, we familiarised ourselves at Poitiers with the concept of operative treatment of the lower limbs and the spine in Duchenne muscular dystrophy (DMD) patients which Yves Rideau and his collaborators (1, 2) had developed there in the early 1980s. Thereupon, we immediately established the techniques at our home universities, first at the Technische Universität Aachen and, from 1999 on, at the Universitätsklinikum Erlangen, Germany. Since then, we have applied the technique to more than 500 DMD patients in total by performing more than 800 operations on the lower limbs and/or spine. In support of findings reported by Professor Rideau in this issue (3) we observed that, where patients are still ambulatory at the time of operation, the operation delays the point at which patients become wheelchair-bound by about two years. Likewise, patients receiving this treatment were/are also able to perform the Gowers' manoeuvre for around two years longer (4-6).
PMCID: PMC3440800  PMID: 22655513
Duchenne muscular dystrophy; prophylactic surgery; prevention of scoliosis
5.  Excellent results with cementless total hip arthroplasty and alumina-on-alumina pairing: minimum ten-year follow-up 
International Orthopaedics  2010;35(2):195-200.
Ceramic-on-ceramic coupling is thought to be a durable alternative to metal- or alumina-on-polyethylene pairing. No evidence exists suggesting superior clinical and radiological results for hydroxyapatite-coated stems versus uncoated stems. The aim of this study is to report the performance of an alumina-on-alumina bearing cementless total hip arthroplasty and to compare stems with a tapered design with and without hydroxyapatite coating. We prospectively analysed the results of cementless tapered femoral stems (40 hydroxyapatite-coated versus 22 uncoated stems), a metal-backed fibre mesh hydroxyapatite-coated socket and alumina-on-alumina pairing. Of 75 hips studied, 62 were available for follow-up (mean of 10.5 years after surgery). The average Harris hip score was 90. Only one hydroxyapatite-coated stem was revised for aseptic loosening. One instance of non-progressive osteolysis was detected around a screw of a cup. All other components showed radiographic signs of stable ingrowth. Hydroxyapatite coating of the stem had no significant impact on the clinical or radiological results. Total hip arthroplasty with the presented implant and pairing provides a durable standard for all patients requiring hip joint replacement against which all newer generations of cementless implants should be judged.
doi:10.1007/s00264-010-1150-1
PMCID: PMC3032123  PMID: 21079952
6.  Progressive femoral cortical and cancellous bone density loss after uncemented tapered-design stem fixation 
Acta Orthopaedica  2010;81(2):171-177.
Background Aseptic implant loosening and periprosthetic bone loss are major problems after total hip arthroplasty (THA). We present an in vivo method of computed tomography (CT) assisted osteodensitometry after THA that differentiates between cortical and cancellous bone density (BD) and area around the femoral component.
Method Cortical and cancellous periprosthetic femoral BD (mg CaHA/mL), area (mm2) and contact area between the prothesis and cortical bone were determined prospectively in 31 patients 10 days, 1 year, and 6 years after uncemented THA (mean age at implantation: 55 years) using CT-osteodensitometry.
Results 6 years postoperatively, cancellous BD had decreased by as much as 41% and cortical BD by up to 27% at the metaphyseal portion of the femur; this decrease was progressive between the 1-year and 6-year examinations. Mild cortical hypertrophy was observed along the entire length of the diaphysis. No statistically significant changes in cortical BD were observed along the diaphysis of the stem.
Interpretation Periprosthetic CT-assisted osteodensitometry has the technical ability to discriminate between cortical and cancellous bone structures with respect to strain-adapted remodeling. Continuous loss of cortical and cancellous BD at the femoral metaphysis, a homeostatic cortical strain configuration, and mild cortical hypertrophy along the diaphysis suggest a diaphyseal fixation of the implanted stem. CT-assisted osteodensitometry has the potential to become an effective instrument for quality control in THA by means of in vivo determination of periprosthetic BD, which may be a causal factor in implant loosening after THA.
doi:10.3109/17453671003635843
PMCID: PMC2852152  PMID: 20180716
7.  Osteomyelitis of the Ulna Caused by Porphyromonas gingivalis 
Journal of Clinical Microbiology  2006;44(10):3835-3837.
A 41-year-old man was provided with a jacket crown after a root end resection of a molar. Four months later, cortical destruction of the ulnar diaphysis with swelling and pain appeared in his forearm. No microorganism could be grown from an intraoperative tissue specimen, but bacterial 16S rRNA genes were detected by broad-range PCR, revealing Porphyromonas gingivalis as the causative agent of osteomyelitis.
doi:10.1128/JCM.00793-06
PMCID: PMC1594789  PMID: 17021123
8.  A modular femoral implant for uncemented stem revision in THR 
International Orthopaedics  2000;24(3):134-138.
Abstract 
We present the early results of 142 uncemented femoral stem revisions using the modular MRP-Titan system. There were 70 cases with marked preoperative femoral bone defects (Paprosky type 2C and type 3); and bone grafts were used in 31 cases. At a mean follow-up of 2.3 years five cases were re-revised due to dislocation and two due to aseptic loosening. The mean Harris hip score improved from 37.4 preoperatively to 92.4. In 122 cases progressive bone regeneration on X-ray was seen; and no further osteolysis was observed.
doi:10.1007/s002640000135
PMCID: PMC3619872  PMID: 10990382

Results 1-8 (8)