Recent Publications by CFE Educators

BACKGROUND

The current interstitial lung disease (ILD) classification has overlapping clinical presentations and outcomes. Cluster analysis modeling is a valuable tool in identifying distinct clinical phenotypes in heterogeneous diseases. However, this approach has yet to be implemented in ILD.

METHODS

Using cluster analysis, novel ILD phenotypes were identified among subjects from a longitudinal ILD cohort, and outcomes were stratified according to phenotypic clusters compared with subgroups according to current American Thoracic Society/European Respiratory Society ILD classification criteria.

RESULTS

Among subjects with complete data for baseline variables (N = 770), four clusters were identified. Cluster 1 (ie, younger white obese female subjects) had the highest baseline FVC and diffusion capacity of the lung for carbon monoxide (Dlco). Cluster 2 (ie, younger African-American female subjects with elevated antinuclear antibody titers) had the lowest baseline FVC. Cluster 3 (ie, elderly white male smokers with coexistent emphysema) had intermediate FVC and Dlco. Cluster 4 (ie, elderly white male smokers with severe honeycombing) had the lowest baseline Dlco. Compared with classification according to ILD subgroup, stratification according to phenotypic clusters was associated with significant differences in monthly FVC decline (Cluster 4, -0.30% vs Cluster 2, 0.01%; P  .0001). Stratification by using clusters also independently predicted progression-free survival (P  .001) and transplant-free survival (P  .001).

CONCLUSIONS

Among adults with diverse chronic ILDs, cluster analysis using baseline characteristics identified four distinct clinical phenotypes that might better predict meaningful clinical outcomes than current ILD diagnostic criteria.

BACKGROUND

Although surgical patch arterioplasty is the conventional therapy for branch pulmonary artery (PA) stenosis, limited data exist on long-term outcomes. We examined the incidence of and risk factors for reintervention after surgical arterioplasty for branch PA stenosis in biventricular congenital heart disease.

METHODS

This retrospective cohort study included patients with 2-ventricle physiology who underwent patch arterioplasty for PA stenosis at a single high-volume congenital heart center during a 10-year period. Freedom from surgical or percutaneous reintervention for recurrent PA stenosis was evaluated. Univariate and multivariable Cox regression analyses were performed to determine risk factors for reintervention.

RESULTS

Among 135 patients, the median age at patch arterioplasty was 0.9 years. Survival to hospital discharge (or 30 days postoperatively) was 96%. During a median follow-up period of 4.0 years, reintervention for PA stenosis occurred in 38 of 115 patients (33%) at a median time to reintervention of 1.4 years. The overall 10-year reintervention rate was 54%. In univariate analysis, age less than 30 days at initial arterioplasty, congenital PA stenosis (vs acquired), and bilateral PA stenosis (vs unilateral) were significantly associated with reintervention. In multivariable analysis, neonatal age (adjusted hazard ratio, 3.6; p = 0.002) and bilateral PA stenosis (adjusted hazard ratio, 2.8; p = 0.005) remained independently associated with reintervention.

CONCLUSIONS

Long-term reintervention for recurrent PA stenosis after patch arterioplasty is common. Patients with bilateral PA stenosis or age younger than 30 days at the time of the index pulmonary arterioplasty are at higher risk for reintervention. These patients may benefit from frequent monitoring or novel approaches to repair.

PURPOSE

Patient centered data are lacking regarding functional and quality of life improvements after artificial urinary sphincter placement. We analyzed the degree of benefit from artificial urinary sphincter placement using ISI (Incontinence Symptom Index), a validated patient reported outcome measure assessing the severity and bother of urinary incontinence, and IIQ-7 (Incontinence Impact Questionnaire-7), a validated patient reported outcome measure assessing the impact and emotional distress of urinary incontinence.

MATERIALS AND METHODS

We performed a retrospective review at 4 centers participating in TURNS (Trauma and Urologic Reconstruction Network of Surgeons). Data were available on 51 and 45 patients who underwent artificial urinary sphincter placement, and had preoperative and postoperative ISI and IIQ-7 data, respectively.

RESULTS

Mean age was 64.8 years. Median time from surgery to followup questionnaires was 8.5 months. On ISI the median preoperative severity and bother scores were 24 (IQR 20-28.5) and 6 (IQR 4-7), and the median postoperative severity and bother scores were 10 (IQR 4.5-17) and 1 (IQR 0-3), respectively. Improvement on each ISI item was statistically significant. On IIQ-7 the median preoperative impact and distress scores were 9 (IQR 6-13) and 4 (IQR 2-6), and the median postoperative impact and distress scores were 3 (IQR 0-7) and 0 (IQR 0-3), respectively. Improvement on each IIQ-7 item was statistically significant.

CONCLUSIONS

Artificial urinary sphincter implantation significantly reduces the severity and bother of stress urinary incontinence symptoms. Longer followup and development are needed of a patient reported outcome measure targeting male stress urinary incontinence.

Prenatal genetic counselors are health care professionals who counsel women making reproductive decisions which include decisions such as terminating pregnancies due to fetal anomalies. Little is known about the experiences and practices of prenatal genetic counselors working with women who have the option of termination after 24 weeks gestation. In this national survey of 168 genetic counselors who have practiced prenatal genetic counseling, we asked about their general practice patterns, including indications for which termination is offered and types of abortion care services that are coordinated by genetic counselors. We report respondents' self-assessments of level of understanding of federal abortion law and abortion procedures. Seventy-six percent of respondents have offered and counseled on termination after 24 weeks and 93% of respondents believe it is the responsibility of the counselor to discuss this option with patients. However, one-third report that they have some or no understanding of the procedures and three-quarters report that they have some or no understanding of federal abortion law. The results of this study provide insight into knowledge and experiences of genetic counselors working with these patients, allowing for improved genetic counselor training and continuing education to provide better guidance and develop more effective means of assisting patients.

PURPOSE

Long-term health risks of occupational exposures to uranium processing were examined to better understand potential differences with uranium underground miners and nuclear reactor workers.

METHODS

A cohort study of mortality of workers from Port Hope, Canada (1950-1999) and Wismut, Germany (1946-2008) employed in uranium milling, refining, and processing was conducted. Poisson regression was used to evaluate the association between cumulative exposures to radon decay products (RDP) and gamma-rays and causes of death potentially related to uranium processing.

RESULTS

The pooled cohort included 7431 workers (270,201 person-years of follow-up). Mean RDP exposures were lower than in miners while gamma-ray doses were higher than in reactor workers. Both exposures were highly correlated (weighted rho = 0.81). Radiation risks of lung cancer and cardiovascular diseases (CVD) in males were increased but not statistically significant and compatible with risks estimated for miners and reactor workers, respectively. Higher RDP-associated CVD risks were observed for exposures 5-14 years prior to diagnosis compared to later exposures and among those employed 5 years. Radiation risks of solid cancers excluding lung cancer were increased, but not statistically significant, both for males and females, while all other causes of death were not associated with exposures.

CONCLUSIONS

In the largest study of uranium processing workers to systematically examine radiation risks of multiple outcomes from RDP exposures and gamma-rays, estimated radiation risks were compatible with risks reported for uranium miners and nuclear reactor workers. Continued follow-up and pooling with other cohorts of uranium processing workers are necessary for future comparisons with other workers of the nuclear fuel cycle.

PURPOSE

Efforts have been made to reduce use of computerized tomography in children with blunt abdominal injury. Computerized tomography may be overused in pediatric patients with renal trauma.

MATERIALS AND METHODS

We performed a retrospective chart review of all renal trauma patients younger than 18 years old treated at 2 urban trauma centers from 2002 to 2016. We collected demographic and clinical characteristics, renal trauma grades, urological interventions, and timing and use of computerized tomography and renal ultrasound.

RESULTS

During the study period 145 patients presented with blunt renal trauma. During hospitalization 46 patients (32%) underwent repeat computerized tomography. About 20% of repeat computerized tomograms were performed less than 48 hours after the first scan. After controlling for center, isolated injury (yes/no), stent placement, age and surgical interventions (yes/no) patients who underwent delayed imaging on their first scan had decreased odds of undergoing a second computerized tomogram (adjusted OR 0.2, 95% CI 0.05-0.9, p = 0.04). Number needed to treat to prevent 1 repeat scan in high grade renal trauma patients was 3 (95% CI 2-4). Estimated sensitivity and specificity for ultrasound monitoring to detect an abnormality requiring urological intervention are 50% and 94%, respectively.

CONCLUSIONS

Repeat computerized tomography in pediatric patients with renal trauma is common. Obtaining delayed imaging on the initial scan in patients with high grade renal trauma may prevent repeat scans. Renal ultrasound provides diagnostic usefulness in monitoring kidney injuries and should be considered before repeating computerized tomography.

Candida aurisis an emerging multidrug-resistant pathogen that can be difficult to identify using traditional biochemical methods. C. auris is capable of causing invasive fungal infections, particularly among hospitalized patients with significant medical comorbidities. Echinocandins are the empiric drugs of choice for C. auris, although not all isolates are susceptible and resistance may develop on therapy. Nosocomial C. auris outbreaks have been reported in a number of countries and aggressive infection control measures are paramount to stopping transmission.