Respiration; international review of thoracic diseases
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Review Case Reports
Pulmonary fibrosis in hermansky-pudlak syndrome. a case report and review.
Hermansky-Pudlak syndrome (HPS) is a rare heterogeneously inherited autosomal recessive group of disorders presenting with oculocutaneous albinism, bleeding diathesis and pulmonary disease. HPS is thought to occur as a consequence of disturbed formation or trafficking of intracellular vesicles, most importantly, melanosomes, platelet dense granules and lysosomes. The latter finding, in particular, contributes much to the morbidity associated with the disease, as ceroid lipofuscin deposits in lysosomes affect many organ systems. ⋯ Pirfenidone, a novel compound with documented anti-inflammatory, antioxidant and antifibrotic effects, appears to hold promise in delaying or preventing fibrosis. To date, there has been one successful lung transplant performed on a patient with HPS. We present a patient with HPS and review the current literature on our understanding of this rare disorder.
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Multicenter Study Comparative Study
Gender and chronic obstructive pulmonary disease in high-risk smokers.
Data suggest that women are more susceptible to develop airway obstruction compared to men for the same number of cigarettes smoked. ⋯ Using the GOLD criteria, the prevalence of COPD in smokers was higher than previous reports. In this self-selected sample of high-risk smokers having the same smoking history, prevalence was lower in women than in men, suggesting a lower susceptibility for the development of airway obstruction.
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Obstructive sleep apnea (OSA) is the most common form of sleep-disordered breathing, affecting 5-15% of the population. It is characterized by intermittent episodes of partial or complete obstruction of the upper airway during sleep that disrupts normal ventilation and sleep architecture, and is typically associated with excessive daytime sleepiness, snoring, and witnessed apneas. Patients with obstructive sleep apnea present risk to the general public safety by causing 8-fold increase in vehicle accidents, and they may themselves also suffer from the physiologic consequences of OSA; these include hypertension, coronary artery disease, stroke, congestive heart failure, pulmonary hypertension, and cardiac arrhythmias. ⋯ Despite the availability of diagnostic measures and effective treatment, many patients with sleep-disordered breathing remain undiagnosed. Therefore, OSA continues to be a significant health risk both for affected individuals and for the general public. Awareness and timely initiation of an effective treatment may prevent potential deleterious cardiovascular effects of OSA.
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Over a 36-month study period, 10 nonconsecutive neuromuscular pediatric patients (6 infants, mean age 10.16 months, and 4 children, mean age 9.3 years) presenting with acute respiratory failure (ARF) were treated by noninvasive positive pressure ventilation (NPPV). All patients required immediate respiratory support and fulfilled our intubation criteria. ⋯ NPPV was a safe and effective first-line therapeutic approach in hypoxemic ARF children/infants with neuromuscular disease. It seems of importance to identify children with neuromuscular disorders who may be able to achieve residual ventilator-free breathing and to perform an NPPV trial avoiding tracheal intubation. Life-threatening respiratory distress and very young age should not preclude NPPV application in the PICU setting. The new helmet interface represents a promising tool for noninvasive ventilation in older children.
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Randomized Controlled Trial
Repeated pulmonary rehabilitation in severe and disabled COPD patients.
The optimal frequency of delivering a pulmonary rehabilitation program (PR) is not yet a well established issue. It is still unclear whether repeated PR at established intervals will result in effective maintenance or further improvement in the patient's health status. ⋯ In severe and disabled COPD, a more frequently repeated inpatient PR may lead to some additional physiological and clinical benefits over 1 year.