Neurogenic pulmonary edema associated with underlying lung disease after a breakthrough seizure.

Neurogenic pulmonary edema (NPE) can result from various central nervous system disorders such as brain malignancies, traumatic brain injuries, infections, and seizures. Although the pathogenesis is not completely understood, NPE creates an increase in pulmonary interstitial and alveolar fluid. It has been reported with prolonged seizure activity. Treatment for NPE is largely supportive. If unrecognized, it can lead to hypoxia and respiratory arrest. We report a case of NPE in a middle-aged female patient following a breakthrough seizure in whom an immunological cause for respiratory findings was high on the differential list, based on her past medical history and chronicity of symptoms. Rapid symptomatic and radiological improvement following hospitalization led to the correct diagnosis.

Characterization of altered patterns of endothelial progenitor cells in sickle cell disease related pulmonary arterial hypertension.

Endothelial dysfunction plays an important role in the pathogenesis of pulmonary arterial hypertension (PAH) in sickle cell disease (SCD). A variety of evidence suggests that circulating endothelial progenitor cells (EPCs) play an integral role in vascular repair. We hypothesized that SCD patients with PAH are deficient in EPCs, potentially contributing to endothelial dysfunction and disease progression. The number of circulating CD34+/CD14-/CD106+ EPCs was significantly lower in SCD patients with PAH than without PAH (P=0.025). CD34+/CD14-/CD106+ numbers significantly correlated with tricuspid regurgitation velocity (TRV, r=-0.44, P=0.033) 6-minute walk distance (6MWD, r= 0.72, P=0.001), mean pulmonary artery pressure (mPAP, r= -0.43, P=0.05), and pulmonary vascular resistance (PVR, r=-0.45, P=0.05). Other EPC subsets including CD31+/CD133+/CD146+ were similar between both groups. Numbers of EPCs did not correlate with age, sex, hemoglobin, WBC count, reticulocyte count, lactate dehydrogenase (LDH), iron/ferritin levels, and serum creatinine. These data indicate that subsets of EPC are lower in SCD patients with PAH than in those without PAH. Fewer EPCs in PAH patients may contribute to the pulmonary vascular pathology. Reduced number of EPCs in SCD patients with PAH might not only give potential insight into the pathophysiological mechanisms but also might be useful for identifying suitable therapeutic targets in these patients.

Uric Acid as a potential biomarker of pulmonary arterial hypertension in patients with sickle cell disease.

Serum uric acid (UA) is emerging as a strong and independent marker for pulmonary arterial hypertension (PAH). PAH is well recognized as a life threatening complication of sickle cell disease (SCD). However, the association between UA and PAH in SCD is unknown. We reviewed electronic medical records (EMR) of 559 consecutive adult SCD patients from Kings County Hospital Center (KCHC) between January 2005 and February 2010. Patients (n = 96) with measurement of UA in close temporal proximity to the transthoracic echocardiography (TTE) were identified. PAH was defined as pulmonary artery systolic pressure (PASP) ≥30 mm Hg. Patients (n = 16) with other risk factors which may cause PAH and chronic renal insufficiency were excluded. In 18 patients, TTE could not measure PASP. Finally, 62 patients were selected. Statistical analysis was performed using Student t tests, Pearson correlation coefficient and multivariate regression analysis. Out of 62 patients, 30 had PAH. Patients with PAH had a higher UA level (8.67 ± 4.8 vs. 5.35 ± 2.1, P = 0.001). We found strong positive correlation between the UA level and PASP (r = 0.71; P < 0.0001). This correlation was independent of diuretic use. UA could be a potential marker for PAH in SCD. However, its' prognostic and pathophysiologic role in SCD patients with PAH needs to be further investigated.

A new functional status outcome measure of dyspnea and anxiety for adults with lung disease: the dyspnea management questionnaire.

PURPOSE: To develop and psychometrically test the Dyspnea Management Questionnaire (DMQ), a new multidimensional measure of dyspnea in adults with chronic obstructive lung disease. PARTICIPANTS: Eighty-five participants were recruited with diagnoses of chronic obstructive pulmonary disease (COPD, n = 73) and asthma (n = 12). The total sample was predominately female (65%) and married (34%), with 64.9% white and a mean age of 75 years (SD = 9.6, n = 76), diagnosed with pulmonary disease 4.8 years ago (SD = 4.4), 32% requiring the use of supplemental oxygen. Participants were also African American (29.9%), Asian (2.6%), and Hispanic (2.6%); n = 77. METHODS: An initial item pool of 74 items was drawn for the DMQ aided by qualitative interview data, literature review, and pilot testing with 3 adults with COPD. Several analyses were used to reduce the item pool. An interdisciplinary panel of 12 experts evaluated the content validity of the DMQ items. To evaluate test-retest reliability, respondents with stable COPD (n = 26) completed the questionnaire twice within a mean interval of 18 days (SD = 7.17). The DMQ was compared with the Medical Outcomes Study 12-Item Short-Form (SF-12) Health Survey, the Seattle Obstructive Lung Disease Questionnaire, and the Hospital Anxiety and Depression Scale. RESULTS: The resulting DMQ is a 30-item scale that measures 5 conceptually derived dimensions: dyspnea intensity, dyspnea-related anxiety, fearful activity avoidance, self-efficacy for activity, and satisfaction with strategy use. It has a 7-point Likert-type scale and third Flesch-Kincaid reading grade level. A panel of 12 experts supported the content validity of the DMQ. It showed high internal consistency (alpha = .87 to .96) and test-retest reliability over 2.5 weeks (intraclass correlation coefficient = 0.71 to 0.95). Dyspnea intensity, dyspnea-related anxiety, and fearful activity avoidance subscales of DMQ-30 and composite score were moderately to highly correlated with 3 Seattle Obstructive Lung Disease Questionnaire dimensions (r = 0.44-0.83), Medical Outcomes Study 12-Item Short-Form scales (r = 0.41-0.57), and Hospital Anxiety and Depression Scale-Anxiety (r = -0.59 to -0.65). Two of DMQ's subscales, self-efficacy for activity and satisfaction with strategy use, correlated mildly with Seattle Obstructive Lung Disease Questionnaire (r = 0.28 and 0.27, respectively). Some very low correlations for DMQ-30's satisfaction with strategy use compared with the Medical Outcomes Study 12-Item Short-Form provided preliminary support for its divergent construct validity. The DMQ-30 discriminated adults with COPD requiring supplemental oxygen from those not requiring it. CONCLUSIONS: The DMQ addresses the need for a more comprehensive, multidimensional assessment of dyspnea, especially for anxious patients with COPD, in order to better guide the appropriate application of dyspnea management interventions and measure pulmonary rehabilitation outcomes. The DMQ can help add insights into the benefit of adjunctive therapies such as psychoeducation, controlled breathing strategies, and cognitive-behavioral approaches in pulmonary rehabilitation for anxious patients with COPD.