Mirror movements in progressive hemifacial atrophy

Mirror movements are simultaneous, involuntary, identical movements occurring during contralateral voluntary movements. These movements are considered as soft neurologic signs seen uncommonly in clinical practice. The mirror movements are described in various neurological disorders which include parkinsonism, cranio veretebral junction anamolies, and hemiplegic cerebral palsy. These movements are intriguing and can pose significant disability. However, no such observation regarding mirror movements in progressive hemifacial atrophy have been reported previously. We are reporting a teenage girl suffering from progressive hemifacial atrophy and epilepsy with demonstrable mirror movements in hand.     

Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare malignant tumour of the skin, with an estimated incidence of 0.8 to five cases per 1 million people per year. OBJECTIVE: To study epidemiological, immunohistochemical and clinical features, delay in diagnosis, type of treatment and outcome of DFSP from 1982 to 2002. METHODS: Using data from the population-based cancer registry, 66 patients with pathologically proved DFSP were included (fibrosarcomatous DFSP were excluded). Each patient lived in one of the four departments of Franche-Comté (overall population of 1 million people) at the time of diagnosis. The main data sources came from public and private pathology laboratories and medical records. The rules of the International Agency for Research on Cancer were applied. RESULTS: The estimated incidence of DFSP in Franche-Comté was about three new cases per 1 million people per year. Male patients were affected 1.2 times as often as female patients were. The trunk (45%) followed by the proximal extremities (38%) were the most frequent locations. DFSP occurred mainly in young adults between 20 and 39 years of age. Mean age at diagnosis was 43 years, and the mean delay in diagnosis was 10.08 years. Our 66 patients initially underwent a radical local excision. Among them, 27% experienced one or more local recurrences during 9.6 years of follow-up. There was one regional lymph node recurrence without visceral metastases. These recurrences were significantly related to the initial peripheral resection margins. We observed a local recurrence rate of 47% for margins less than 3 cm, vs. only 7% for margins ranging from 3 to 5 cm [P=0.004; OR=0.229 (95%, CI=0.103-0.510)]. The mean time to a first local recurrence was 2.65 years. Nevertheless, there was no death due to the DFSP course at the end of the follow-up, and the final outcome was favourable. CONCLUSION: Our study emphasizes the importance of wide local excision with margins of at least 3 cm in order to prevent local recurrence. However, the recent development of inhibitors of signal transduction by the PDGFB pathway should soon modify the surgical strategy, which is often too mutilating.     

Validity of the 'Ten Questions' for screening serious childhood disability: results from urban Bangladesh

A survey of 2576 children aged two to nine years was carried out in Dhaka, Bangladesh, as part of a collaborative study to test the validity of a questionnaire (the Ten Questions) for screening severe childhood disabilities in community settings. Approximately 7% of the children were positive on the screen and this rate was slightly higher in boys than girls. The sensitivity, specificity and negative predictive value of the Ten Questions were perfect or nearly perfect for severe and moderate (serious) disabilities. The positive predictive value was only 22% for serious disabilities, but 70% of children classified as false positives were found to have mild disabilities or other conditions (such as ear infections) for which early detection and treatment could be beneficial. No major age or gender differences in the validity of the questionnaire were apparent, but this finding needs additional study and confirmation with studies based on larger samples. In general, the results indicate that the Ten Questions is a valid tool for screening serious disabilities in children and can potentially improve the efficiency of health services by reducing the number of children requiring attention from professionals. Future studies using the Ten Questions should foster greater attention to the dimensions of childhood disability as a public health problem in the less developed world.     

REOPERATION FOR RECURRENT CORONARY ARTERY DISEASE: RESULTS OF 200 CONSECUTIVE CASES

Background : It is well known that reoperation for recurrent coronary artery disease is more difficult than primary coronary artery bypass grafting. However, it is possible to reduce the morbidity and mortality of reoperation to the same level as the initial procedure with careful surgical technique. Methods : A retrospective study of the first 200 patients who underwent redo coronary bypass grafting was undertaken. Results : In the first 200 cases of redo coronary bypass grafting at St George Hospital, Sydney (August 1986–January 1995), there were five in-hospital deaths (2.5%). There was one case of sternal infection (0.5%), which required surgical debridement, three cases of stroke (1.5%), one case of postoperative bleeding (0.5%), which required a return to theatre and six cases (3%) required mechanical ventilation for more than 24h. The need for major postoperative support (such as intra-aortic balloon pumping/adrenaline infusion) was significantly affected by the degree of urgency and the degree of pre-operative ventricular impairment. Conclusions : The mortality rate of redo coronary artery bypass grafting in this series is similar to that of primary surgery described in other reports.     

The impact of poverty on educational outcomes for children

Over the past decade, the unfortunate reality is that the income gap has widened between Canadian families. Educational outcomes are one of the key areas influenced by family incomes. Children from low-income families often start school already behind their peers who come from more affluent families, as shown in measures of school readiness. The incidence, depth, duration and timing of poverty all influence a child’s educational attainment, along with community characteristics and social networks. However, both Canadian and international interventions have shown that the effects of poverty can be reduced using sustainable interventions. Paediatricians and family doctors have many opportunities to influence readiness for school and educational success in primary care settings.     

Crown augmentation using modified bilayered restoration for anchorage: A case report

An ankylosed tooth can be suitable for obtaining orthodontic anchorage. However, if such a tooth lacks adequate clinical crown height, the anchorage will not be effective. In those situations surgical luxation or restorative crown augmentation is suggested. This case report is about the restorative treatment of an ankylosed, infraoccluded tooth to enhance the anchorage for forced orthodontic eruption of impacted maxillary canines. A crown augmentation in the form of a modified bilayered (sandwich) restoration using GIC, Composite resin and Silver amalgam on left maxillary first molar (26) was successful in sustaining the anchorage for forced eruption and alignment of impacted canines.     

Failure modes of left ventricular reconstruction or the Dor procedure: a multi-institutional perspective.

BACKGROUND: Complex ventricular reconstruction (CVR) is now being employed increasingly thanks to the pioneering work of Dor. However, little is known about the failure mode of CVR. We present experience from three centres with CVR and an analysis of the failure modes. METHODS: Between January 1997 and February 2005, 284 patients underwent CVR in three centres in Australia and USA. All of the procedures were performed as adjuncts to coronary artery surgery and/or valvular surgery. Patients were followed-up clinically and/or echocardiographically. Failure modes were classified as fatal or non-fatal. Non-fatal failure mode (NFM) was defined as either persistent heart failure, recurrence of LV scar, need for ventricular assistance, persistent ventricular arrhythmia, or a combination. RESULTS: Operative mortality rate (OMR) was 8% (23 deaths). This fatal failure mode was most related to urgency of surgery and cardiogenic shock in 15 patients (5.3% of OMR), stroke in 5 patients (1.8%) or postoperative bi-ventricular failure (1%). Non-fatal failure modes accounted for morbidity in 26 patients (9%). This was predominantly due to persistent septal dyskinesis in 7 patients (2.46%), persistent mitral regurgitation in 5 (1.8%), postoperative ventricular tachycardia in 4 (1.4%), sub-optimal myocardial protection in 4 (1.4%) use of a large, stiff patch in 4 (1.4%). One hundred and ninety-nine of the surviving 261 patients (76%) were in NYHA Class I. CONCLUSIONS: Complex ventricular reconstruction is a robust technique that has lasting benefit. Failure modes have been identified and could be minimized by appropriate patient and procedure selection.