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1.
Sci Rep ; 9(1): 4061, 2019 03 11.
Article in English | MEDLINE | ID: mdl-30858405

ABSTRACT

The presence of pulmonary hypertension (PH) significantly worsens outcomes in patients with advanced sarcoidosis, but its optimal management is unknown. We aimed to characterize a large sarcoidosis-associated pulmonary hypertension (SAPH) cohort to better understand patient characteristics, clinical outcomes, and management strategies including treatment with PH therapies. Patients at Duke University Medical Center with biopsy-proven sarcoidosis and SAPH confirmed by right heart catheterization (RHC) were identified from 1990-2010. Subjects were followed for up to 11 years and assessed for differences by treatment strategy for their SAPH, including those who were not treated with PH-specific therapies. Our primary outcomes of interest were change in 6-minute walk distance (6MWD) and change in N-terminal pro-brain natriuretic peptide (NT-proBNP) by after therapy. We included 95 patients (76% women, 86% African American) with SAPH. Overall, 70% of patients had stage IV pulmonary sarcoidosis, and 77% had functional class III/IV symptoms. Median NT-proBNP value was elevated (910 pg/mL), and right ventricular dysfunction was moderate/severe in 55% of patients. Median values for mean pulmonary artery pressure (49 mmHg) and pulmonary vascular resistance (8.5 Woods units) were consistent with severe pulmonary hypertension. The mortality rate over median 3-year follow-up was 32%. Those who experienced a clinical event and those who did not had similar overall echocardiographic findings, hemodynamics, 6MWD and NT-proBNP at baseline, and unadjusted analysis showed that only follow-up NT-proBNP was associated with all-cause hospitalization or mortality. A sign test to evaluate the difference between NT-Pro-BNP before and after PH therapy produced evidence that a significant difference existed between the median pre- and post-NT-Pro-BNP (-387.0 (IQR: -1373.0-109), p = 0.0495). Use of PH-specific therapy may be helpful in selected patients with SAPH and pre-capillary pulmonary vascular disease. Prospective trials are needed to characterize responses to PH-specific therapy in this subset of patients with SAPH.


Subject(s)
Hemodynamics/drug effects , Pulmonary Arterial Hypertension/drug therapy , Sarcoidosis, Pulmonary/drug therapy , Aged , Biomarkers/blood , Cardiac Catheterization , Echocardiography , Epoprostenol/administration & dosage , Epoprostenol/analogs & derivatives , Female , Humans , Iloprost/administration & dosage , Male , Middle Aged , Pulmonary Arterial Hypertension/blood , Pulmonary Arterial Hypertension/complications , Pulmonary Arterial Hypertension/physiopathology , Sarcoidosis, Pulmonary/blood , Sarcoidosis, Pulmonary/complications , Sarcoidosis, Pulmonary/physiopathology , Treatment Outcome , Vascular Resistance/physiology , Ventricular Dysfunction, Right/physiopathology
2.
Clin Pediatr (Phila) ; 58(11-12): 1302-1308, 2019 10.
Article in English | MEDLINE | ID: mdl-31165627

ABSTRACT

Our objective was to use community-based, national databases to evaluate diagnostic imaging and antibiotic prophylaxis practice patterns before and after the release of the 2011 American Academy of Pediatrics guidelines for acute febrile urinary tract infection. Using the National Ambulatory and Hospital Ambulatory Medical Care Surveys, urinary tract infection encounters were identified for patients aged 2 months to 18 years. Primary outcomes were utilization of antibiotics (as proxy for prophylaxis) and diagnostic imaging during encounters. Weighted multivariate logistic regression models were used to examine the association between time period (before and after 2011) and each of the primary outcomes. Among 8 588 035 weighted encounters, adjusting for covariates, there was insufficient evidence to suggest a difference between time periods for antibiotic utilization (odds ratio = 0.66, P = .12) or diagnostic imaging (odds ratio = 1.16, P = .56). Thus, we did not find evidence of changes in antibiotic utilization or diagnostic imaging practice patterns after the release of the 2011 American Academy of Pediatrics guidelines.


Subject(s)
Anti-Bacterial Agents/therapeutic use , Community Health Services/methods , Guideline Adherence/statistics & numerical data , Health Care Surveys/statistics & numerical data , Practice Patterns, Physicians'/statistics & numerical data , Urinary Tract Infections/drug therapy , Child , Cohort Studies , Female , Humans , Male , Pediatrics , Retrospective Studies , Societies, Medical , United States
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