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1.
BMC Musculoskelet Disord ; 19(1): 251, 2018 Jul 25.
Article in English | MEDLINE | ID: mdl-30045704

ABSTRACT

BACKGROUND: Treponema Pallidum (TP), the pathogen of syphilis, commonly infects bones in cases of congenital and tertiary syphilis, but it is rare in the primary and secondary stages. With its mild symptoms and rare clinical findings, it might be easy to dismiss the diagnosis of early syphilis. Usually, effective results can be achieved after the conventional strategy of antibiotic treatments, mainly penicillin. To our knowledge, our case is so far the most serious reported case of destructive bone lesion in secondary syphilis, and our treatment for the case is the first strategy using total hip arthroplasty in secondary syphilis. CASE PRESENTATION: We present the case of a 71-year-old man with local repeated pain and dysfunction in the right hip. Radiologic examinations showed the disappearance of the ipsilateral femoral head and neck. After excluding the aetiologies of cancer metastasis and tuberculosis, we confirmed the diagnosis of syphilitic arthritis. The patient received the medical treatment of antibiotics and the surgical treatment of total hip arthroplasty. At the follow-up of 1, 3, and 5.5 years after the operation, the patient presented with a pain-free and functional hip prosthesis without local signs of infection and loosening. CONCLUSIONS: This report highlights the difficulties of early diagnosis of secondary syphilis with bone involvement. Bone defect of the femur with secondary syphilis, especially at the proximal femur, was an extremely rare complication in the previous reports. Our case was the first case of a patient who experienced the disappearance of femoral head and neck caused by secondary syphilis. Follow-up after the operation proved the successful treatment of the extensive bone defect of femur by total hip arthroplasty.


Subject(s)
Coxa Magna/diagnostic imaging , Femur Neck/diagnostic imaging , Syphilis/diagnostic imaging , Aged , Coxa Magna/etiology , Coxa Magna/surgery , Femur Neck/surgery , Follow-Up Studies , Humans , Male , Syphilis/complications , Syphilis/surgery
2.
Mol Genet Metab ; 121(3): 241-251, 2017 07.
Article in English | MEDLINE | ID: mdl-28552677

ABSTRACT

Hip problems in Mucopolysaccharidosis type VI (MPS VI) lead to severe disability. Lack of data on the course of hip disease in MPS VI make decisions regarding necessity, timing and type of surgical intervention difficult. We therefore studied the development of hip pathology in MPS VI patients over time. Data were collected as part of a prospective follow-up study. Standardized supine AP pelvis and frog leg lateral radiographs of both hips were performed yearly or every 2years. Image assessment was performed quantitatively (angle measurements) and qualitatively (hip morphology). Clinical burden of hip disease was evaluated by physical examination, six minute walking test (6MWT) and a questionnaire assessing pain, wheelchair-dependency and walking distance. A total of 157 pelvic radiographs of 14 ERT treated MPS VI patients were evaluated. Age at first image ranged from 2.0 to 21.1years. Median follow up duration was 6.8years. In all patients, even in the youngest, the acetabulum and os ilium were dysplastic. Coverage of the femoral head by the acetabulum improved over time, but remained insufficient. While the femoral head appeared normal in the radiographs at young age, the ossification pattern became abnormal in all patients over time. In all patients the distance covered in the 6MWT was reduced (median Z scores -3.3). Twelve patients had a waddling gait. Four patients were partially wheelchair-dependent and ten patients had limitations in their maximum walking distance. In conclusion, clinically significant hip abnormalities develop in all MPS VI patients from very early in life, starting with deformities of the os ilium and acetabulum. Femoral head abnormalities occur later, most likely due to altered mechanical forces in combination with epiphyseal abnormalities due to glycosaminoglycan storage. The final shape and angle of the femoral head differs significantly between individual MPS VI patients and is difficult to predict.


Subject(s)
Coxa Magna/etiology , Hip Dislocation/etiology , Mucopolysaccharidosis VI/complications , Acetabulum/abnormalities , Adult , Coxa Magna/diagnosis , Female , Femur/abnormalities , Femur Head/abnormalities , Follow-Up Studies , Hip Dislocation/diagnosis , Hip Joint/diagnostic imaging , Hip Joint/pathology , Humans , Male , Mucopolysaccharidosis VI/diagnosis , N-Acetylgalactosamine-4-Sulfatase/genetics , Pelvis/abnormalities , Pelvis/diagnostic imaging , Prospective Studies , Time Factors
3.
J Orthop Res ; 35(9): 2051-2058, 2017 09.
Article in English | MEDLINE | ID: mdl-27864891

ABSTRACT

The purpose of this study was to quantify femoral head deformity in patients with Legg-Calvé-Perthes disease (LCPD) using a novel three dimensional (3D) magnetic resonance imaging (MRI) reconstruction and volume based analysis. Bilateral femoral heads of 17 patients (mean age 9.9 ± 2.0 years; 12 boys, 5 girls) with LCPD were scanned 1-2 times (n = 33 LCPD heads, 20 normal heads) using a 1.5T MRI scanner. Fourteen patients had unilateral and three had bilateral LCPD with five hips in the Waldenström initial stage, 9 in the fragmentation stage, 14 in the reossification stage, and 5 in the healed stage. 3D digital reconstructions of femoral heads were created using MIMICS software. Deformity was quantified using a 3D volume ratio method based on reference hemisphere volume as well as two surface geometry methods. Intra-observer analysis showed that 97% of the LCPD femoral heads were within 10% of the original value and test shapes had 99.6% accuracy. For normal femoral heads, the volume ratios of all except one were between 95 and 98% (n = 20) of a perfect hemisphere volume. For femoral heads affected with LCPD, the volume ratios ranged from 43% to 96% of a perfect hemisphere (n = 33). The volume ratio method and the two surface geometry comparison methods had high correlation (r = 0.89 and 0.96). In summary, the 3D MRI volume ratio method allowed accurate quantification and demonstrated small changes (<10%) of the femoral head deformity in LCPD. This method may serve as a useful tool to evaluate the effects of treatment on femoral head shape. © 2016 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 35:2051-2058, 2017.


Subject(s)
Coxa Magna/diagnostic imaging , Femur Head/diagnostic imaging , Legg-Calve-Perthes Disease/diagnostic imaging , Child , Coxa Magna/etiology , Female , Femur Head/pathology , Humans , Imaging, Three-Dimensional , Legg-Calve-Perthes Disease/complications , Legg-Calve-Perthes Disease/pathology , Magnetic Resonance Imaging , Male
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