Pilar Resano Barrios

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This paper presents data collected by a Brazilian center in a multinational multicenter observational study of patients with mucopolysaccharidosis type VI (MPS VI), aiming at determining the epidemiological, clinical, and biochemical profile of these patients. Twenty-eight south-American patients with MPS VI were evaluated through medical interview,(More)
Characteristic cardiac valve abnormalities and left ventricular hypertrophy are present in untreated patients with mucopolysaccharidosis type VI (MPS VI). Cardiac ultrasound was performed to investigate these findings in subjects during long-term enzyme replacement therapy (ERT) with recombinant human arylsulfatase B (rhASB, rhN-acetylgalactosamine(More)
The most common indications of esophageal replacement in childhood are the esophageal atresia with long gap and caustic strictures. Several techniques has been described, using different components of the digestive tract. Total gastric transposition was performed in 4 children. The indications were esophageal atresia without tracheoesophageal fistula in 3(More)
Leukotrienes have been implicated in the pathogenesis of hypoxic pulmonary hypertension in adult animals and in persistent pulmonary hypertension with accompanying hypoxemia in the neonate. In order to elucidate the role of leukotrienes in hypoxic pulmonary hypertension in a young animal model, the effects of a leukotriene antagonist, FPL 57231, were(More)
The cardiovascular and ventilatory effects of high-frequency jet ventilation (HFJV) and conventional ventilation (CV) were evaluated in a saline lung lavage model in piglets. After saline lavage and stabilization on CV, animals were randomized to either mode of ventilation for 4 h. Arterial blood gases, cardiac output, mean pulmonary and arterial blood(More)
1. Lo2-threshold tetanization of ipsilateral cutaneous nerves evoked fusimotor γ-reflexes in 24 out of 90 primary afferents originating from ankle extensors, whereas the same kind of stimulation was ineffective if applied to antagonistic muscle nerves. 2. Muscle spindles supplied by fusimotor α-fibres responded less frequently to low-threshold tetanization(More)
Introduction: Obesity is associated with 2 closely related respiratory diseases: obesity hypoventilation syndrome (OHS) and obstructive sleep apnea–hypopnea syndrome (OSAHS). It has been shown that noninvasive ventilation during sleep produces clinical and functional improvement in these patients. The long-term survival rate with this treatment, and the(More)
Primary endings of cat flexor muscle spindles supplied by fusimotor α-fibres respond to brief repetitive stimulation (20–250 Hz) of ventral root filaments with a train of discharges, which lasts as long as the duration of stimulation. Frequencies between 80 and 120 Hz increase the number of spindle discharges up to an average value of 50 Imp/sec. The most(More)