Takayasu's arteritis in children : a review

Takayasu's arteritis is an inflammatory disease of unknown origin involving aorta, its primary branches and pulmonary artery. This article briefly reviews the pathology, clinical features and treatment of Takayasu's arteritis, focusing mainly on the disease in children.peer-reviewe

The caveats in the diagnosis of anomalous origin of left coronary artery from pulmonary artery (ALCAPA)

Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is an infrequent, well described, but important anomaly of the coronary origin. Early diagnosis and prompt surgical treatment of the disease can be life saving. However, there are several potential sources of error in the seemingly simple stereotype diagnostic pattern. This article reports a case of ALCAPA and allude to some of the caveats in the diagnosis of this entity.peer-reviewe

Classical supravalvar aortic stenosis and peripheral pulmonary stenosis

Supravalvar aortic stenosis as well as peripheral pulmonary stenosis are rare forms of congenital heart disease, which occur, however, relatively frequently in association. This article reports and discusses the classical findings of SVAS and PPS upon clinical examination and imaging in a sixyear-old girl that presented with repeated respiratory tract infections and failure to thrive.peer-reviewe

Androgen receptor as a mediator and biomarker of radioresistance in triple-negative breast cancer.

Increased rates of locoregional recurrence have been observed in triple-negative breast cancer despite chemotherapy and radiation therapy. Thus, approaches that combine therapies for radiosensitization in triple-negative breast cancer are critically needed. We characterized the radiation therapy response of 21 breast cancer cell lines and paired this radiation response data with high-throughput drug screen data to identify androgen receptor as a top target for radiosensitization. Our radiosensitizer screen nominated bicalutamide as the drug most effective in treating radiation therapy-resistant breast cancer cell lines. We subsequently evaluated the expression of androgen receptor in >2100 human breast tumor samples and 51 breast cancer cell lines and found significant heterogeneity in androgen receptor expression with enrichment at the protein and RNA level in triple-negative breast cancer. There was a strong correlation between androgen receptor RNA and protein expression across all breast cancer subtypes (R2 = 0.72, p < 0.01). In patients with triple-negative breast cancer, expression of androgen receptor above the median was associated with increased risk of locoregional recurrence after radiation therapy (hazard ratio for locoregional recurrence 2.9-3.2)) in two independent data sets, but there was no difference in locoregional recurrence in triple-negative breast cancer patients not treated with radiation therapy when stratified by androgen receptor expression. In multivariable analysis, androgen receptor expression was most significantly associated with worse local recurrence-free survival after radiation therapy (hazard ratio of 3.58) suggesting that androgen receptor expression may be a biomarker of radiation response in triple-negative breast cancer. Inhibition of androgen receptor with MDV3100 (enzalutamide) induced radiation sensitivity (enhancement ratios of 1.22-1.60) in androgen receptor-positive triple-negative breast cancer lines, but did not affect androgen receptor-negative triple-negative breast cancer or estrogen-receptor-positive, androgen receptor-negative breast cancer cell lines. androgen receptor inhibition with MDV3100 significantly radiosensitized triple-negative breast cancer xenografts in mouse models and markedly delayed tumor doubling/tripling time and tumor weight. Radiosensitization was at least partially dependent on impaired dsDNA break repair mediated by DNA protein kinase catalytic subunit. Our results implicate androgen receptor as a mediator of radioresistance in breast cancer and identify androgen receptor inhibition as a potentially effective strategy for the treatment of androgen receptor-positive radioresistant tumors

Recurrent hemorrhagic pericardial effusion in a child due to diffuse lymphangiohemangiomatosis: a case report

<p>Abstract</p> <p>Introduction</p> <p>Recurrent hemorrhagic pericardial effusion in children with no identifiable cause is a rare presentation.</p> <p>Case presentation</p> <p>We report the case of a 4-year-old Indian girl who presented with recurrent hemorrhagic pericardial effusion. Diffuse lymphangiomatosis was suspected when associated pulmonary involvement, soft tissue mediastinal mass, and lytic bone lesions were found. Pericardiectomy and lung biopsy confirmed the diagnosis of diffuse lymphangiohemangiomatosis. Partial clinical improvement occurred with thalidomide and low-dose radiotherapy, but our patient died from progressive respiratory failure.</p> <p>Conclusion</p> <p>Diffuse lymphangiohemangiomatosis should be considered in the differential diagnosis of hemorrhagic pericardial effusion of unclear cause.</p

Perception of stroke and knowledge of potential risk factors among Omani patients at increased risk for stroke

BACKGROUND: Previous studies have demonstrated poor knowledge of stroke among patients with established risk factors. This study aims to assess the baseline knowledge, among patients with increased risk for stroke in Oman, of warning symptoms of stroke, impending risk factors, treatment, and sources of information. METHODS: In April 2005, trained family practice residents at Sultan Qaboos University Hospital Clinics (cardiology, neurology, diabetic, and lipid clinics), using a standardised, structured, pre-tested questionnaire, conducted a survey of 400 Omani patients. These patients all demonstrated potential risk factors for stroke. RESULTS: Only 35% of the subjects stated that the brain is the organ affected by a stroke, 68% correctly identified at least one symptom/sign of a stroke, and 43% correctly identified at least one stroke risk factor. The majority (62%) did not believe they were at increased risk for stroke, and 98% had not been advised by their attending physician that their clinical conditions were risk factors for stroke. In the multivariable logistic regression analysis, lower age and higher levels of education were associated with better knowledge regarding the organ involved in stroke, stroke symptoms, and risk factors. CONCLUSION: Because their knowledge about stroke risk factors was poor, the subjects in this study were largely unaware of their increased risk for stroke. Intensive health education is needed to improve awareness of stroke, especially among the most vulnerable groups