RESUMO
The early progressive form of the X-linked disorder, Hunter syndrome or mucopolysaccharidosis type II (MPS II) (OMIM #309900), is characterized by cognitive decline, and pulmonary and cardiac complications that often cause death before 20 years of age. Deficiency of the lysosomal enzyme, iduronate-2-sulfatase (EC 3.1.6.13) results in deposition of the glycosaminoglycans, dermatan, and heparan sulfate in various tissues. In recent years, enzyme replacement therapy (ERT) has become the mainstay of treatment, but is expensive and ineffective in arresting cognitive decline. Hematopoietic stem cell transplantation (HSCT) also provides enzyme replacement, and may be effective in stabilizing neurocognitive function if initiated early, though data are limited. We present a case series of four patients who demonstrated neurocognitive stabilization with early HSCT. HSCT is a potentially underutilized treatment strategy for select groups of MPS II patients.
RESUMO
This study presents the development of a hybrid system consisting of an ensemble of Extended Kalman Filter (EKF) based Multi Layer Perceptron Network (MLPN) and a one-pass learning Fuzzy Inference System using Look-up Table Scheme for the recognition of electrocardiogram (ECG) signals. This system can distinguish various types of abnormal ECG signals such as Ventricular Premature Cycle (VPC), T wave inversion (TINV), ST segment depression (STDP), and Supraventricular Tachycardia (SVT) from normal sinus rhythm (NSR) ECG signal.