Increased risk of strontium ranelate-related SJS/TEN is associated with HLA.

UNLABELLED: Severe adverse drug reactions (ADR) of Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) in some patients receiving strontium ranelate have been reported, but the risk factors are unclear. We show that HLA-A*33:03 and B*58:01 are significantly associated with patients who developed SJS/TEN; and provide the first evidence that genetic risk factors are involved in strontium ranelate-associated SJS/TEN. INTRODUCTION: In this study, HLA as a genetic risk factor was assessed among osteoporotic patients prescribed with strontium ranelate that developed severe cutaneous adverse drug reactions (SCARs) compared with those who were tolerant. METHODS: Genomic DNA isolated from peripheral blood mononuclear cells (PBMCs) of patients was HLA typed using sequencing-based typing method to determine their HLA profiles. RESULTS: Osteoporotic patients who are currently on strontium ranelate were enrolled in the study (n = 76). Tolerant controls were defined as patients who received strontium ranelate for a minimum of 3 months (range 3 months to 8 years) with no reports of any cutaneous reactions as these reactions usually occur within the first 12 weeks after starting treatment. Retrospective cases of SJS/TEN were also identified (n = 5). The majority of the accrued samples were of Han Chinese descent: controls (n = 72) and cases (n = 4). All cases and controls were genotyped at four HLA genes, namely HLA-A, HLA-B, HLA-C, and HLA-DRB1. In comparing the samples of Han Chinese descent (72 controls and 4 cases), we found significant associations with HLA-A*33:03 (p = 0.002) and HLA-B*58:01 (p = 0.023). There was no significant association with any HLA-C or HLA-DRB1 alleles. CONCLUSIONS: This study reveals that the occurrence of SJS/TEN in Han Chinese patients receiving strontium ranelate is HLA associated. This has important clinical implications for understanding the underlying mechanisms for this ADR as well as evaluating the potential role of genetic pre-screening for osteoporotic patients who may be prescribed strontium ranelate.

Giant labial fibroepithelial stromal polyp.

We report an18-year-old girl with a four-year history of a slow-growing labial mass with a sudden increase in size in the last year. Examination revealed a large fleshy 20 cm perineal mass centering on the left labia majora and attached to it by a 1cm pedicle. It was associated with pain, ulceration and discharge. The lesion was excised via diathermy at the base of the stalk. The excised specimen weighed 1.112kg and measured 20.5 x 17 x 5cm. The lesion showed a solid, soft whitish, cut surface. Histology revealed a hypocellular tumour with focally oedematous fibrous stroma in which were scattered large and small blood vessels, mast cells and other chronic inflammatory cells. True myxoid matrix was not observed. The stromal cells had a spindle to stellate morphology. There was no significant cytological atypia, mitotic activity or necrosis. The tumour cells were negative for SMA, desmin, CD34, S100 protein, EMA and PR. The diagnosis was clinically and histologically challenging because various vulvovaginal soft tissue tumours often have overlapping clinicopathological features. However, based on strict histological criteria and the absence of worrisome cytological features, a diagnosis of fi broepithelial stromal polyp was rendered despite the unusual size. A review of the literature shows that whilst vulvovaginal fibroepithelial stromal polyps are well described, giant variants are rare. Awareness of the extraordinary size that can be attained by such polyps can facilitate swift clinical and histological diagnosis.

Sonomyography: monitoring morphological changes of forearm muscles in actions with the feasibility for the control of powered prosthesis.

Electromyography (EMG) has been widely used for the assessment of musculoskeletal functions and the control of electrical prostheses, which make use of the EMG signal generated by the contraction of the residual muscles. In spite of the successful applications of EMG in different fields, it has some inherent limitations, such as the difficulty to differentiate the actions of neighboring muscles and to collect signals from deep muscles using the surface EMG. The majority of current EMG controlled prostheses can only provide sequential on-off controls using signals from two groups of muscles, so the users are required to put many conscious efforts in monitoring the speed and range of motion of the terminal devices being controlled. Recently, many alternative signals based on the detection of dimensional changes of muscles or tendons during actions have been reported. The objective of this study was to investigate the potential of the dimensional change of muscles detected using sonography for musculoskeletal assessment and control. A portable B-mode ultrasound scanner was used to collect the dynamic ultrasound images of the forearm muscles of six normally limbed young adults and three amputee subjects. A motion analysis system was used to collect the movement of the wrist angle during the experiments for the normal subjects. It was demonstrated that the morphological changes of forearm muscles during actions can be successfully detected by ultrasound and linearly correlated (R(2)=0.876+/-0.042, mean+/-S.D.) with the wrist angle. We named these sonographically detected signals about the architectural change of the muscle as sonomyography (SMG). The mean ratio between the wrist angle and the percentage deformation of the forearm muscle was 7.2+/-3.7 degrees /% for the normal subjects. The intraclass correlation coefficient (ICC) of this ratio among the three repeated tests was 0.868. The SMG signals from the residual forearms were also successfully detected when the three amputee subjects contracted their residual muscles. The results demonstrated that SMG had potentials for the musculoskeletal control and assessment.