scholarly journals Pathophysiology of Hip Disorders in Patients with Mucopolysaccharidosis IVA

Diagnostics ◽  
2020 ◽  
Vol 10 (5) ◽  
pp. 264
Author(s):  
Zhigang Wang ◽  
Yunlan Xu ◽  
Enze Jiang ◽  
Jianmin Wang ◽  
Shunji Tomatsu ◽  
...  

Patients with mucopolysaccharidoses IVA (MPS IVA) have a progressive accumulation of the specific glycosaminoglycans (GAGs): chondroitin-6-sulfate (C6S) and keratan sulfate (KS), leading to the degeneration of the cartilage matrix and its connective tissue perturbing the regular microarchitecture of cartilage and successively distorting bone ossification and growth. Impaired cartilage quality and poor bone mineralization lead to serious hip disorders in MPS IVA patients. Although hip dysplasia is seen widely in musculoskeletal abnormality of this disorder, the pathophysiology of the hip bone and cartilage morphology in these patients remains unclear. Until now, no systemic study of the hip joints in MPS IVA has been reported by using the combined images of plain film radiographs (PFR) and Magnetic Resonance Imaging (MRI). This study aimed to assess the bony and cartilaginous features of hip joints and to explore the potentially related factors of femoral head osteonecrosis (FHN) and hip subluxation/dislocation in patients with MPS IVA. Hip joints in MPS IVA patients were retrospectively reviewed, based on the findings of PFR and MRI data from 2014 to 2019. Demographic information was also collected and analyzed with imaging measurements. A total of 19 patients (eight boys and 11 girls) were recruited, and 38 hip joints in these patients were examined. Eleven patients (57.9%) had FHN. FHN patients were statistically compared with those without FHN. Correlations between cartilaginous femoral head coverage (CFHC) and acetabular index (AI), cartilaginous AI (CAI), or neck-shaft angle (NSA) were investigated in patients with hip subluxation or dislocation. The greater cartilaginous coverage of the hips than their osseous inherency was observed. Significant correlation was observed between CFHC and AI (r =−0.351, p = 0.049) or CAI (r =−0.381, p = 0.032). Severe subluxations or dislocations were more likely to be present in those with more dysplastic bony and cartilaginous hips. In conclusion, our study provides the first systemic description of bony and cartilaginous characteristics in the hip morphology of MPS IVA patients. We have demonstrated that plain radiography alone leads to a misunderstanding of hip morphology and that MRI measurements with PFR are an essential tool to evaluate the ‘true’ characterization of hips for MPS IVA patients.

2016 ◽  
Vol 1 ◽  
pp. 15-15
Author(s):  
Abdulaziz Almaawi ◽  
Pascal-André Vendittoli

2017 ◽  
Author(s):  
Enis Gursel ◽  
Omer Faruk Bilgen ◽  
Sadik Bilgen ◽  
Basak Erdemli Gursel ◽  
Canan Ersoy ◽  
...  

2017 ◽  
Vol 68 (5) ◽  
pp. 974-976
Author(s):  
Alexandru Patrascu ◽  
Liliana Savin ◽  
Dan Mihailescu ◽  
Victor Grigorescu ◽  
carmen Grierosu ◽  
...  

In recent years, there has been an increase in the number of studies on the etiology of femoral head necrosis. We retrospectively reviewed all patients diagnosed with aseptic necrosis of the femoral in the period of 2010-2015. We recorded a total of 230 cases diagnosed with aseptic necrosis of the femoral head, group was composed of 65.7% men and 34.3% women, risk factors identified was 19.13% (post-traumatic), 13.91% (glucocorticoids), 26.52% (alcohol), 3.47% (another cause) and in 36 95% of the cases no risk factors were found. The results of the study based on the type of surgery performed on the basis of stages of disease progression, 8 patients (3.48%) benefited from osteotomy, 28 patients (12.17%) benefited of bipolar hemiarthroplasty prosthesis and 188 patients (81.74%) benefited of total hip arthroplasty. Osteonecrosis of the femoral head is characteristic to young patients between the age of 30-50 years old. Predisposing factors, alcohol and corticosteroid therapy remains an important cause of the disease. Total hip arthroplasty remains the best option for the patients with osteonecrosis of the femoral head.


2019 ◽  
Vol 7 (18) ◽  
pp. 441-441 ◽  
Author(s):  
Wei Huang ◽  
Xuan Gong ◽  
Steve Sandiford ◽  
Xiaoqiang He ◽  
Feilong Li ◽  
...  

2021 ◽  
pp. 112070002110130
Author(s):  
Leigh-Anne Tu ◽  
Douglas S Weinberg ◽  
Raymond W Liu

Background: While the influences of acetabular dysplasia and overcoverage on hip arthritis have been studied, the impact of femoral neck-shaft angle on hip arthritis is much more poorly understood. The purpose of this study is to determine if a relationship exists between neck shaft angle and the development of osteoarthritis, a better understanding of which would be useful to surgeons planning osteotomies about the hip. Methods: 533 cadaveric femora and acetabulae (1066 total) from the Hamann-Todd Osteological Collection (Cleveland, OH) were acquired. We measured true neck shaft angle using an AP photograph with the femoral neck parallel to the table. Femoral head volume to acetabular volume ratio, representing femoral head coverage, as well as femoral version were utilised. Correlation between neck shaft angle, femoral version, femoral head coverage and osteoarthritis were evaluated with multiple regression analysis. Results: The mean age and standard deviation was 56 ± 10 years. There were 64 females (12%) and 469 males. There were 380 Caucasians (71%) and 153 African-Americans. Mean femoral version was 11° ± 12° and mean true neck shaft angle was 127.7° ± 5.9° There was a strong correlation between age and arthritis (standardised beta 0.488, p < 0.001). There was a significant correlation between increasing true neck shaft angle and decreasing hip arthritis (standardised beta -0.024, p = 0.038). In the femoral head overcoverage subset, increasing true neck shaft angle was still significantly associated with decreasing hip arthritis (standardised beta −0.088, p = 0.018), although this relationship was not significant with femoral head undercoverage subset. Conclusions: With sufficient acetabular coverage, a relative increase in femoral neck shaft angle within the physiologic range is associated with decreased hip osteoarthritis. Clinical relevance: An understanding of the relationship between femoral neck shaft angle and hip osteoarthritis could be useful for surgeons planning pelvic or proximal femur osteotomies in children.


Biomaterials ◽  
2010 ◽  
Vol 31 (14) ◽  
pp. 4048-4055 ◽  
Author(s):  
Chih-Hung Chang ◽  
Tai-Chieh Liao ◽  
Yuan-Ming Hsu ◽  
Hsu-Wei Fang ◽  
Chia-Chun Chen ◽  
...  

2011 ◽  
Vol 131 (11) ◽  
pp. 1585-1590 ◽  
Author(s):  
Young-Kyun Lee ◽  
Yun Jong Lee ◽  
Yong-Chan Ha ◽  
Ki-Choul Kim ◽  
Kyung-Hoi Koo

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Ahmed Salem Eid ◽  
Ahmed Mohamed Mohasseb ◽  
Ramy Mohamed Mohamed El-Bahnasy

Abstract Background Femoral neck fractures in adults younger than age 50 years are uncommon and often the result of high energy trauma. They account for only 2-3% of all femoral neck fractures. Femoral neck fractures are associated with aging and osteoporosis and can occur after relatively trivial trauma in elderly patients. femoral neck fractures are associated with higher incidences of femoral head osteonecrosis and nonunion. Fixation options include cannulated screws, non parallel cannulated screws, medial augmentation plate, and fixed angle femoral neck fixation implants. A systematic review of literature done to evaluate which technique is better. Methods After literature search thirteen articles were found and were reviewed to evaluate final outcome of fixation of neck femur fractures by cannulated screws, non parallel cannulated screws, medial augmentation plate, and fixed angle femoral neck fixation implants. Results After evaluation of all studies the incidence of nonunion, osteonecrosis of femoral head, implant failure & revesion surgery is lower in comparing Targon plate with multiple cannulated screws while we need further studies for application of non parallel screws and medial plate. Conclusion Targon plate recorded low incidence of complications in the final outcome in comparison with multiple cannulated screw while we need further studies for application of Non parallel screws and medial plate.


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