Respiration; international review of thoracic diseases
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Review
Pulmonary vascular manifestations of hereditary hemorrhagic telangiectasia (rendu-osler disease).
Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is a genetic disorder with autosomal dominance and variable penetrance, characterized by epistaxis, telangiectasia and visceral manifestations of the disease. The estimated minimal prevalence is 1/10,000 inhabitants. The diagnosis is established on clinical criteria, and may be further confirmed by the identification of causative mutations in either the ENG or the ACVRL1 gene coding for endoglin and ALK1, respectively. ⋯ All adult patients with HHT should be proposed systematic screening for PAVM, by contrast echocardiography (preceded by anteroposterior chest radiograph) or computed tomography of the chest. Pulmonary hypertension is rare in HHT, and may be due either to systemic arteriovenous shunting in the liver increasing cardiac output or be clinically and histologically indistinguishable from idiopathic pulmonary arterial hypertension. Pulmonary hypertension is detected by systematic examination of right cardiac cavities and tricuspid regurgitation flow at echocardiography, and the diagnosis is established by right heart catheterization.
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Multicenter Study Clinical Trial
Extraction of pins from the airway with flexible bronchoscopy.
Tracheobronchial foreign body aspiration is a worldwide health problem which often results in life threatening complications. Standard flexible bronchoscopy (FB) is used increasingly in the treatment of tracheobronchial foreign body aspiration in adults and older children, especially in the removal of aspirated foreign bodies which have entered into the peripheral bronchi. ⋯ Our study suggests that FB is a safe, easy and successful method used in the removal of foreign bodies, such as pins, from the tracheobronchial trees. By employing FB, indications of thoracotomy and other invasive methods can be reduced especially in the cases of pins localized in distal airways and in the evaluation of suspected foreign bodies.
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Comparative Study
Noninvasive ventilation using a mouthpiece in patients with chronic obstructive pulmonary disease and acute respiratory failure.
Noninvasive positive pressure ventilation (NPPV) delivered via a mouthpiece (mNPPV) has been successfully used in stable chronic restrictive respiratory insufficiency, but not in patients with chronic obstructive pulmonary disease (COPD) and acute respiratory failure (ARF). ⋯ In case of moderate respiratory acidosis, noninvasive ventilation using a mouthpiece significantly reduces the endotracheal intubation rate in comparison with SMT and therefore appears to be a second-line alternative to noninvasive ventilation delivered via a mask, especially when poorly tolerated.
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Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder reflecting abnormalities in the structure and function of motile cilia and flagella, causing impairment of mucociliary clearance, left-right body asymmetry, and sperm motility. Clinical manifestations include respiratory distress in term neonates, recurrent otosinopulmonary infections, bronchiectasis, situs inversus and/or heterotaxy, and male infertility. Genetic discoveries are emerging from family-based linkage studies and from testing candidate genes. ⋯ If only one mutation is identified, the full gene may be sequenced to search for a trans-allelic mutation. As more disease-causing gene mutations are identified, broader genetic screening panels will further identify patients with PCD. Ongoing investigations are beginning to identify genetic mutations in novel clinical phenotypes for PCD, such as congenital heart disease and male infertility, and new associations are being established between 'ciliary' genetic mutations and clinical phenotypes.
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Continuous positive airway pressure (CPAP) is considered as the standard therapy for obstructive sleep apnea syndrome (OSAS), but some patients with OSAS are unable to accept CPAP due to nasal obstruction and poor nasal airflow. ⋯ Nasal resistance before the beginning of CPAP treatment has a significant effect on the acceptance of CPAP in OSAS patients, and hence, could be a predictive parameter for the initial acceptance of CPAP.