Combined pulmonary fibrosis and emphysema as a clinicoradiologic entity: Characterization of presenting lung fibrosis and implications for survival.

BACKGROUND: The prevalence of classifiable and unclassifiable causes of lung fibrosis and its implications for survival are mostly unknown in combined pulmonary fibrosis and emphysema (CPFE). MATERIALS AND METHODS: Patients with >10% involvement of both emphysema and lung fibrosis seen over 11 years at our institution were reviewed independently by expert radiologists for fibrotic and emphysematous findings and overall fibrotic CT pattern. Underlying interstitial lung disease (ILD) diagnoses and baseline demographic and clinical characteristics were collated and assessed for predictors of comparative survival. RESULTS: In this retrospective cohort, 179 CPFE patients were identified and categorized as 58 usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF) (32%), 42 secondary ILD (23%), and 79 unclassifiable ILD (44%). The most prevalent (47%) radiologic pattern was 'unclassifiable', followed by 'consistent' and 'possible' UIP pattern in 38%. Adjusted predictors of mortality for the cohort as a whole included age (HR 1.03[1.01-1.06], P = 0.002), percent predicted diffusing capacity for carbon monoxide (unit HR 0.97 [0.96-0.99], P = 0.001), honeycombing (HR 1.58 [1.02-2.43], P = 0.04), and right ventricular dysfunction (HR 2.28 [1.39-3.97], P = 0.002). Survival was similar between CPFE with secondary ILD and CPFE with UIP/IPF, while CPFE with unclassifiable ILD had better comparative survival (Log rank = 0.026). CONCLUSIONS: Our findings suggest only about a third of CPFE patients represent suspected UIP/IPF; the majority were clinically and radiologically unclassifiable ILD whose survival was comparatively better. Identifiable or secondary causes of lung fibrosis in CPFE occurred in about a fifth of presenting patients.

Pulmonary sequestration in adults: a retrospective review of resected and unresected cases.

BACKGROUND: Pulmonary sequestration (PS) is a form of congenital pulmonary malformation that is generally diagnosed in childhood or adolescence and usually resected when diagnosed. We aim to identify the clinical presentation and course of patients diagnosed to have PS during adulthood. METHODS: Using a computer-assisted search of Mayo clinic medical records, we identified adult patients with PS diagnosed between 1997 and 2016. Clinical and radiological data were collected including postoperative course for those who underwent surgical resection. RESULTS: We identified 32 adult patients with PS; median age at diagnosis was 42 years (IQR 28-53); 17 patients (53%) were men. The median sequestration size was 6.6 cm (IQR 4.4-9.3). The type of sequestration was intralobar in 81% and extralobar in 19%. The most common location was left lower lobe posteromedially (56%). Forty-seven percent of the patients presented with no relevant symptoms. The most common radiographic finding was mass/consolidation in 61% and the most common feeding artery origin was the thoracic aorta (54%). Surgical resection was performed in 18 patients (56%) and postoperative complication was reported in 5 patients (28%). There was no surgical mortality. Median duration of follow-up after diagnosis for unresected cases, most of whom were asymptomatic, was 19 months (IQR 4-26) with no complications related to the PS reported. CONCLUSIONS: Nearly one-half of adult patients with PS present with no relevant symptoms. The decision regarding surgical resection needs to weigh various factors including clinical manifestations related to PS, risk of surgical complications, comorbidities, and individual patient preferences.

Utility of autoimmune serology testing in the assessment of uncharacterized interstitial lung disease: a large retrospective cohort review.

BACKGROUND: Autoimmune serologies are often obtained in the initial evaluation of uncharacterized interstitial lung disease (ILD). Whether this practice is helpful in delineating connective-tissue disease related ILD (CTD-ILD) is not well known. We assessed the frequency of incident CTD-ILD as detected by autoimmune serology testing and presenting clinical signs and symptoms. METHODS: Consecutive patients seen at our institution over a four year period with newly diagnosed uncharacterized ILD and autoimmune serologic testing were included. Serologic assessment was performed as a standardized order set of 13 laboratory tests. Presenting demographics and clinical signs or symptoms suggestive of autoimmune disease were correlated with the presence or absence of positive serology studies and final CTD-ILD diagnoses. RESULTS: Overall prevalence of newly diagnosed CTD-ILD was 6.9% (42 of 605). Positive serology was seen in 35.2% (213 of 605) of screened ILD. CTD-ILD was diagnosed in 19.2% of those with positive serology, and 52.8% of those with both positive serology and suggestive clinical signs or symptoms. Only 1.4% of those with positive serology and negative review of systems were diagnosed with CTD-ILD. CTD-ILD diagnoses were made more frequently in younger patients ≤60 years with no diagnoses made after the age of 80 (P = 0.009). Positive serology in non-CTD-ILD cases did not appear to confer any survival advantage. CONCLUSIONS: The yield of autoimmune serology testing in uncharacterized ILD appears greatest in those with suggestive clinical signs or symptoms on presentation for CTD-ILD.

Predictors and outcomes of pneumonia in patients with spontaneous intracerebral hemorrhage.

OBJECTIVES: Pneumonia is an important complication of spontaneous intracerebral hemorrhage (sICH). The purpose of this study was to determine the predictors and outcomes of the development of pneumonia in patients with sICH. METHODS: In total, 290 consecutive patients with sICH admitted within 24 hours of stroke onset were investigated in a single center, retrospective study from January 2006 to July 2009. Clinical, biochemical, and imaging variables were registered. Stroke severity and functional outcomes were evaluated with the Glasgow Coma Scale (GCS) and modified Rankin Scale (mRS). Baseline variables that predicted pneumonia were investigated and outcomes were assessed. RESULTS: The association of our primary exposure variables, such as mechanical ventilation, tube feeding, dysphagia, and tracheostomy, with pneumonia was highly significant (P < .0001, for each variable). For mechanical ventilation, we observed an odds ratio (OR; 95% confidence interval [CI]) of 9.42 (4.24-20.9); for tube feeding, OR = 22.3 (8.91-55.8); for dysphagia, OR = 13.1 (4.66-36.7); and for tracheostomy, OR = 26.8 (8.02-89.3). After adjustment of potential confounders including GCS and mRS on admission, the use of angiotensin-converting enzyme inhibitors, proton pump inhibitors, and H2 blockers, all the adjusted OR (ORa) remained significant. For mechanical ventilation, the minimum ORa was 3.72 (95% CI: 1.68-8.26) when adjusted for GCS. For both dysphagia and tracheostomy, mRS reduced OR to 7.46 (95% CI: 3.34-10.6) in the case of dysphagia with an ORa of 16.2 (95% CI: 4.98 to 52.8) for tracheostomy. For tube feeding, both GCS and mRS reduced ORa; the former to 14.7 (95% CI: 6.16-35.0) and the latter to 15.7 (95% CI: 6.63-37.0). Pneumonia shows a significant effect on the morbidity (P = .003), length of stay (P < .0001), and mortality (P = .041) rate of the patients. CONCLUSION: Mechanical ventilation, tube feeding, dysphagia, and tracheostomy are exposures associated with increased risk of the development of pneumonia in patients with sICH. Pneumonia is associated with an increase in morbidity, length of stay, and mortality among patients with sICH.

Electrocardiographic abnormalities in patients presenting with intracranial parenchymal haemorrhage.

OBJECTIVES: The electrocardiographic abnormalities associated with ischaemic stroke and subarachnoid haemorrhage have been described frequently and studied systematically; however, these changes were not investigated thoroughly in patients with intracranial parenchymal haemorrhage (IPH). METHODS: We retrospectively reviewed the electrocardiograms (ECGs) and medical records of all patients who had been diagnosed with acute intraparynchemal haemorrhage (IPH) between 2006 and 2009. RESULTS: We included 160 patients (56% males). The median age was 71 years (interquartile range (IQR) 59 to 80) and 69% were above the age of 60 years. Most patients were hypertensive (81%).The majority of patients (86%) had at least one ECG abnormality. Sixty-eight (43%) patients had T-wave inversion and 65 (41%) had QTc interval prolongation. There was a significant association between QTc prolongation and the bleeding size and the presence of midline shift; odd ratios were 2.8 (CI 1.4 to 5.5; P 0.003) and 2.2 (CI 1.1 to 4.2; P 0.04), respectively. In addition, sinus tachycardia was found to be significantly associated with the presence of hydrocephalus (OR 4.1; CI 1.3 to 12.8; P 0.02). CONCLUSIONS: ECG abnormalities are a common finding in patients with IPH. Repolarizaion abnormalities occur the most frequently. QTc prolongation was associated with bleeding size and midline shift. Patients who had hydrocephalus were more likely to have sinus tachycardia at presentation.

Hepatic hydrothorax without any evidence of ascites.

Hepatic hydrothorax usually presents in association with ascites, but there are rare cases when it does not. This case helps to support the differential of hepatic hydrothorax in patients who have a history of liver cirrhosis, portal hypertension, and recurrent pleural effusions without ascites. We hope to support the conclusion that a patient with recurrent pleural effusions, without ascites, does not exclude gastrointestinal involvement in its etiology.

Association of hypophosphatemia with failure-to-wean from mechanical ventilation.

Severe hypophosphatemia is known to be associated with respiratory failure, but there are few studies that specifically examine the relationship between serum phosphorus concentration and failure to wean patients from mechanical ventilation. This study investigated the association between hypophosphatemia and weaning failure in patients in two medical intensive care units (ICU). The study was conducted in a prospectively developed cohort of 66 patients being treated with ventilatory support and in whom 193 weaning trials were attempted. Ultimately, all 66 subjects were successfully weaned. A cross-sectional analysis was conducted on serum phosphorus levels and success or failure to wean the patients from ventilators. At the time of the successful weaning attempts (n = 66), the subjects' serum phosphorus concentrations (mean +/- SD) were 1.18 +/- 0.27 mmol/L, whereas at all failed weaning attempts (n = 127) serum phosphorus concentrations averaged 1.06 +/- 0.31 mmol/L (p = 0.008). Subjects with phosphorus concentrations below the reference interval (RI) in our laboratory (<0.80 mmol/L) had greater risk for weaning failure compared to subjects with phosphorus concentrations at or above the RI (relative risk = 1.18; 95% confidence interval = 1.06 to 1.32; p = 0.01). Serum calcium concentrations were not significantly different at the time of successful weaning compared to those at failed weaning attempts. This study indicates that there is an association between hypophosphatemia and failure-to-wean from mechanical ventilation in ICU patients on ventilatory support.

Mounier-Kuhn syndrome: a case report.

Mounier-Kuhn syndrome is a rare clinical entity. It was described in 1932 to refer to the presence of tracheobronchomegaly. The clinical presentation is variable and it can occur in several different age groups. We present the case of a young man who was admitted to the hospital for an asthma exacerbation and was found to have tracheobronchomegaly on his CT scan. A review of the literature for this rare, but interesting, phenomenon is included.