Chest
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Pulmonary arterial hypertension (PAH) is a rare disease, and much of our understanding stems from single-center studies, which are limited by sample size and generalizability. Administrative data offer an appealing opportunity to inform clinical, research, and quality improvement efforts for PAH. Yet, currently no standardized, validated method exists to distinguish PAH from other subgroups of pulmonary hypertension (PH) within this data source. ⋯ This set of validated algorithms to identify PAH in administrative data can be used by the PAH scientific and clinical community to enhance the reliability and value of research findings, to inform quality improvement initiatives, and ultimately to improve health for PAH patients.
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A number of genetic markers linked to familial pulmonary fibrosis predict differential survival in interstitial lung disease (ILD) patients. Although genetic testing is not performed routinely for ILD, family history commonly is obtained and may inform outcome risk. ⋯ Patient-reported familial pulmonary fibrosis is predictive of reduced transplant-free survival in IPF and non-IPF ILD patients. Because survival among patients with familial non-IPF ILD approximates that of sporadic IPF ILD, early intervention should be considered for such patients. Until clinical genetic testing is widely available and provides actionable results, family history should be ascertained and considered in risk stratification.
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Primary ciliary dyskinesia (PCD) is a heterogeneous disease with a diverse clinical and genetic spectrum among populations worldwide. Few cases of pediatric PCD have been reported from China. ⋯ Besides typical clinical features, PIBO was observed as the first presentation of pediatric PCD in China. An association of the novel gene DNAH14 with PCD was observed, expanding the PCD genotypic spectrum.
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A 62-year-old African American man was admitted to the hospital with hemoptysis. He had a complicated medical history significant for active tobacco use (>50 pack-year history), coronary artery disease, and heart failure with reduced ejection fraction. He reported intermittent episodes of coughing up streaks of blood in the sputum for the past 3 years. ⋯ Physical examination was significant for decreased bilateral breath sounds with no wheezing, crackles, or rhonchi. Cardiovascular examination revealed normal cardiac rhythm without murmur, rubs, or gallops. There was no clubbing or edema on his extremities.
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A better understanding of the clinical features associated with prolonged hospitalization in acute respiratory failure may allow for better-informed care planning. ⋯ From 2004 to 2014, incidence and mortality decreased among patients with ARF and pLOS, and although per-patient costs rose, the percentage of total cost of care remained stable. There is substantial variation in length of stay for patients with ARF by US region, hospital teaching status, and patient insurance coverage.