Annals of the American Thoracic Society
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Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive genetic disorder characterized by oculocutaneous albinism and a bleeding diathesis due to platelet dysfunction. More than 50% of cases worldwide are diagnosed on the Caribbean island of Puerto Rico. Genetic testing plays a growing role in diagnosis; however, not all patients with HPS have identified genetic mutations. ⋯ Thus, lung transplantation remains the only potentially life-prolonging treatment. At present, two clinical trials are recruiting patients with HPS-PF to identify biomarkers for disease progression. Advances in the diagnosis and management of these patients will require the establishment of multidisciplinary centers of excellence staffed by experts in this disease.
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Multicenter Study
The Challenge of Predicting Pressure Ulcers in Critically Ill Patients. A Multicenter Cohort Study.
Pressure ulcers are preventable events. Critically ill patients are particularly vulnerable. The Braden scale has been used to identify hospitalized patients at high risk for the development of pressure ulcers; however, this predictive tool has not been adequately validated for critically ill patients. ⋯ The original Braden scale measured at ICU admission is a valuable tool for pressure ulcer prediction, although it is not accurate for severely ill patients. To overcome the limitations of the original scale, we derived a modified score with better performance, which may identify high-risk ICU patients and support target interventions. External validation of the proposed clinical prediction score is needed.
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Editorial Comment
Physician Culture and Vasopressin Use in Septic Shock.
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Review Meta Analysis
Diagnostic Yield and Complications of Transbronchial Lung Cryobiopsy for Interstitial Lung Disease. A Systematic Review and Metaanalysis.
The diagnostic usefulness and safety of transbronchial lung cryobiopsy for the evaluation of interstitial lung disease remain unclear. ⋯ The diagnostic accuracy of transbronchial lung cryobiopsy cannot be determined given the absence of studies directly comparing cryobiopsy diagnoses with diagnoses derived from surgical lung biopsies interpreted within multidisciplinary discussions. The histopathological and multidisciplinary discussion-based diagnostic yield of transbronchial cryobiopsy appears high, but with variable frequencies of complications dominated by pneumothorax and moderate-to-severe hemorrhage.
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Randomized Controlled Trial
Montelukast for Children with Obstructive Sleep Apnea: Results of a Double-Blind, Randomized, Placebo-Controlled Trial.
Obstructive sleep apnea (OSA) is highly prevalent in children and is usually treated by adenotonsillectomy. Nonsurgical therapies for OSA consist primarily of antiinflammatory approaches and have gained popularity, but their efficacy remains to be critically examined. ⋯ When compared with placebo, montelukast for 16 weeks effectively reduced the severity of obstructive sleep apnea in children 2-10 years of age. These results support a therapeutic role for leukotriene modifiers in pediatric OSA provided that long-term trials confirm current findings. Clinical trial registered with www.clinicaltrials.gov (NCT 00599534).