Body composition, muscle strength, and physical function of patients with Bethlem myopathy and Ullrich congenital muscular dystrophy.

OBJECTIVE: To determine the contributions of body mass, adiposity, and muscularity to physical function and muscle strength in adult patients with Bethlem myopathy (BM) and Ullrich congenital muscular dystrophy (UCMD). MATERIALS AND METHODS: Evaluation involved one UCMD and 7 BM patients. Body composition was determined by body mass index (BMI) and dual-energy-X-ray-absorptiometry (DXA), muscle strength by dynamometry, physical function by the distance walked in 6 minutes (6MWD), forced vital capacity (FVC) by a spirometer. RESULTS: Six participants were of normal weight and 2 overweight based on BMI; all were sarcopenic based on appendicular fat free mass index (AFFMI); and 7 were sarcopenic obese based on AFFMI and % fat mass. Average muscle strength was reduced below 50% of normal. The 6MWD was in BM patients 30% less than normal. FVC was reduced in 4 of the BM patients. Muscle strength had a good correlation with the physical function variables. Correlation between muscle strength and BMI was poor; it was very high with AFFMI. AFFMI was the best single explicator of muscle strength and physical function. CONCLUSION: Muscle mass determined by DXA explains most of the variability of the measures of muscle strength and physical function in patients with BM and UCMD.

Stress Fracture of Proximal Femur after Hip Resurfacing Treated with Cannulated Screw.

Stress fractures of the proximal femur are described in athletes and military personnel. In most cases, they are not treated surgically, except when they are at the top of the femoral neck and with cortical involvement. The return to sports is not recommended in patients with hip replacement, especially for the high rate of revision of implants in the younger patients. One of the major complications of hip resurfacing (HR) is the medial fracture of the femoral neck, which usually occurs within 9 weeks after surgery. The causes have to be attributed to a malposition of the femoral component or to an insufficient bone density. The case reported herein is unique because it describes a stress fracture on patient operated with HR, treated with screw fixation. Two years after surgery, the patient returned to his normal life, practicing sports, without progression of varus angulation of the stem.

Clinical and radiographic outcomes of the Birmingham Hip Resurfacing arthroplasty at a minimum follow-up of 10 years: results from an independent centre.

BACKGROUND: Metal-on-metal hip resurfacing (MoMHR) has been proposed as an effective surgical treatment for young and active patients with symptomatic hip disease. Recently, good clinical and radiographic outcomes have been reported by the designer surgeons at a 15.3 years follow-up; however, results at long follow-up by non-designer surgeons are less satisfactory. The aim of the study was to investigate if MoMHR can produce satisfactory clinical and radiographic results and if survival rate can be high even if the procedure is performed by non-designer surgeons. METHODS: All patients were assessed about implant survival. All patients completed an Oxford Hip Score (OHS), Harris Hip Score (HHS) and a University of California Los Angeles (UCLA) activity score preoperatively, at 1 year and at last available follow-up; at this time, a standard anteroposterior weight-bearing radiograph was performed. RESULTS: The survival rate with revision for any reason is 96%, similar to those obtained by designer surgeons. All the clinical scores improved over time: according to the OHS the survivors are asymptomatic and according to the UCLA maintain a high level of function. 6 remodellings of the femoral neck and 2 heterotopic bone formations were seen, but they were asymptomatic. CONCLUSIONS: As designer surgeons have already shown, MoMHR can provide in active patients a durable treatment for hip arthritis, with low risk of revision and good results at 10 years follow-up, even if the procedure is performed by non-designer surgeons.