Angioimmunoblastic lymphadenopathy with dysproteinaemia (AILD) is a rare lymphoproliferative disorder characterized by systemic lymphadenopathy, hepatosplenomegaly, loss of body weight, fever, skin eruption, and polyclonal hypergammaglobulinaemia. Occasionally, pulmonary involvement, including pleural effusion, has also been observed. Two cases of AILD accompanied by pleural effusion are reported here. When thoracentesis was performed, an exudative effusion was obtained and there was an increase in soluble interleukin-2 receptor and immunoglobulin G, A, and M in the pleural fluid. Cytologically, atypical plasma cells, and T-cell predominant lymphocytes were also present. These findings are likely to be characteristic of pleural effusions associated with AILD and may prove to be a useful marker for diagnosis.
Aim To evaluate the influence of comorbidities and aging on pulmonary rehabilitation ( PR ) efficacy in patients with chronic obstructive pulmonary disease ( COPD ). Methods This was a retrospective cohort study of patients with COPD attending an outpatient PR program. Comorbidity information was collected with the C harlson Index, BODE index and COPD ‐specific comorbidity test, and also included other common conditions not included in these indexes. The efficacy of PR was defined as a 54‐m increase in 6‐min walk distance or a four‐point decrease in S t. G eorge's R espiratory Q uestionnaire score. Patients were divided into two age groups according to the median age of 72 years. Results A total of 21 of 52 patients (40%) showed a clinically significant benefit by the 6‐min walk distance, and 29 patients (55.8%) by the S t. G eorge's R espiratory Q uestionnaire score. PR efficacy was not different between the elderly group and the younger group by either parameter. A total of 98% of the patients had at least one chronic comorbidity. Hypertension was the most frequently reported comorbidity (28.5%). Higher body mass index, H ospital A nxiety and D epression S cale anxiety score and S t. G eorge's R espiratory Q uestionnaire total score were associated with a good response to PR by the 6‐min walk distance. None of the individual comorbidities or indexes were correlated with the efficacy of PR . Multiple logistic regression analysis showed that body mass index was independently associated with the response to PR . Conclusions PR is equally effective in elderly and younger patients with COPD , with efficacy influenced by body mass index and anxiety. Geriatr Gerontol Int 2016; 16: 934–941.
Intravascular lymphomatosis with primary pulmonary lesion is an extremely rare disease. Although the major clinical symptoms include fever, cough, dyspnea and loss of body weight, these are not diagnostic. Chest radiograph findings are also nonspecific and include bilateral reticular shadow, reticulonodular shadow, ground-glass opacity or wedge-shaped subpleural opacities. Therefore, the antemortem diagnosis is relatively difficult. It is considered that intravascular lymphomatosis is a high-grade malignant lymphoma. However, it has been shown recently that a good response and long-term survival may possibly be obtained through systemic combination chemotherapy. We report a case of intravascular lymphomatosis with primary pulmonary lesion where an early diagnosis was obtained through thoracoscopic lung biopsy and subsequent systemic chemotherapy proved to be quite effective. Because the clinical symptoms or chest radiograph findings are usually nonspecific, it was thought that thoracoscopic lung biopsy could be a useful procedure for early and reliable diagnosis of primary pulmonary intravascular lymphomatosis and that it might contribute to an improved prognosis.
Background: The asthma prevalence of elderly individuals is increasing with aging society worldwide. Elderly asthma is more susceptible to asthma morbidity and mortality than younger adults and children. Controlling asthma and preventing asthma death requires attention to self-management, including correct knowledge of asthma and consistent use of ICS with proper technique. Aims: To examine self-management behaviors, including the knowledge of asthma and ICS treatment, inhaler technique (IT), and to evaluate their association with clinical variables in elderly asthma. Methods: We scored the knowledge and IT using questionnaire and checklist in seventy-eight elderly asthma (mean 73.9 years of age). The correlations with these scores and clinical evaluation (pulmonary function, exhaled nitric oxide, cognitive function, depression status, asthma control) were analyzed. Results: The mean score of the knowledge was 69.0/100. The percentage of patients who answered correct mostly, "What kind of sickness is asthma?" :53.6%, "The name and effect of inhaler which you use": only 14.5%. However, "Times to use the inhaler": 73.0%, "Necessity of consistent inhaler use": 93.0%, showed high percentage of correct answered. The mean score of IT were 77.2/100 (DPI) and 69.4/100 (pMDI). Especially, we found the low percentage of performed correctly "expiration before inhalation" and "breath hold after inhalation" showed each 50%. Multivariate analysis showed significant association between the asthma knowledge score and the ITscore (p=0.005). Conclusion: The study indicates that asthma education is important for performing proper inhalation technique in patients with elderly asthma.
Primary cancer of the appendix is a rare entity. We experienced a patient with the disease who was seen at the hospital because of a lower abdominal tumor and was operated on with a diagnosis of ovarian cancer, but intraoperative frozen section diagnosis resulted in that the ovarian lesion was a metastasis from a cancer of the appendix. This paper describes this rare case with a review of the literature. A 46-year-old woman was admitted to the gynecological & obstetrical department in our hospital because of a lower abdominal tumor. There were an adult's head sized tumor in the lower abdomen, pleural effusion and ascites. Ultrasonography and CT revealed aninternally heterogenous tumor with irregular margin in the ovary. Laparotomy was performed with a diagnosis of ovarian tumor. The tumor existed in the left ovary, and a swelling of the ileocecal lymph nodes and claval hypertrophy of the appendix were noted. In addition intraoperative frozen section diagnosis was ovarian metastasis of signet ring cell carcinoma. The patient was definitely diagnosed as cancer of the appendix with an ovarian metastasis. Excision of the ileocecal region, simple hysterectomy, and bilateral adnexectomy were carried out. The excised specimen revealed mixed-type and moderately differentiated adenocarcinoma arising in the appendix with metastases to the left ovary as well as the uterus and right ovary.
Background. Various factors have been reported to be useful for predicting future exacerbations. Objective. This study was intended to determine a usefulness of a combination of a patient-based questionnaire, such as the Asthma Control Test (ACT) score with objective assessments, such as forced expiratory volume in 1 second (FEV1) and/or exhaled nitric oxide (FENO), for predicting future exacerbations in adult asthmatics. Methods. We therefore enrolled 78 subjects with mild to moderate asthma, who were clinically stable for 3 months who all had been regularly receiving inhaled steroid treatment. All subjects underwent a routine assessment of asthma control including the ACT score, spirometry, and FENO, and then were followed up until a severe exacerbation occurred. The predictors of an increased risk of severe exacerbation were identified and validated using decision trees based on a classification and regression tree (CART) analysis. The properties of the developed models were the evaluated with the area under the ROC curve (AUC) (95% confidence interval [CI]). Results. The CART analysis automatically selected the variables and cut-off points, the ACT score ≤23 and FEV1 ≤ 91.8%, with the greatest capacity for discriminating future exacerbations within one year or not. When the probalility was calculated by the likelihood ratio of a positive test (LP), the ACT score ≤23 was identified with a 60.3% probability, calculated by 1.82 of LP, whereas the combined ACT score ≤23 and the percentage of predicted FEV1 ≤ 91.8% were identified with an 85.0% probability, calculated by an LP score of 5.43, for predicting future exacerbation. Conclusion. These results demonstrated that combining the ACT score and percentage of predicted FEV1, but not FENO, can sufficiently stratify the risk for future exacerbations within one year.
The efficacy of montelukast, a specific cysteinyl leukotriene receptor antagonist, in preventing recurrent asthma attacks was evaluated for post-emergency management of acute asthma exacerbation. Twenty-two patients with a history of chronic asthma whose symptoms were responsive to an inhaled β-adrenergic receptor agonist in an emergency room setting, were randomized into two groups, those with and those without montelukast (n = 11 for each group). Patients in the montelukast group received an oral dose of 10 mg montelukast before leaving the emergency room following rescue treatment with an inhaled β-adrenergic receptor agonist. Patients in both groups were instructed to use an inhaled β-adrenergic receptor agonist for shortness of breath or dyspnea in post-emergency management. Additional β-adrenergic receptor agonist use, subjective asthma symptoms, sleep impairment, additional emergency visits and/or hospitalization were monitored for 24 hours following the emergency room visit. In the montelukast group, the need for a rescue β-adrenergic receptor agonist was significantly decreased; 54.5% of patients in the montelukast group required use of β-adrenergic receptor agonist compared with 100% in the non-montelukast group (P < 0.05). The average number of uses of a β-adrenergic receptor agonist was 2.67 ± 3.58 times/ 24 h in the montelukast group compared with 11.95 ± 3.60 times/24 h in the non-montelukast group (P < 0.01). The average subjective asthma symptom scores were significantly decreased in the montelukast group, whereas no score change occurred in the non-montelukast group. The sleep impairment score was significantly lower in the montelukast group compared with that in the non-montelukast group (P < 0.05). No patients in either group had an emergency visit or hospitalization during this period. The results demonstrate that montelukast can prevent recurrent asthma exacerbations in the home environment.
Exertional dyspnea is the primary symptom that limits exercise in patients with chronic obstructive pulmonary disease (COPD). It is unknown which activated brain area is associated with this symptom in COPD patients.To investigate the activation of cortical areas associated with dyspnea during exercise in COPD patients.COPD patients (n = 10) and age-matched controls (n = 10) performed mild-intensity constant work rate cycle exercise (40% of their symptom-limited peak work rates) for 10 min, while cerebral hemodynamics and oxygenation were measured by near-infrared spectroscopy (NIRS). Ventilatory responses (breathing pattern and pulmonary gas exchange) and Borg scale ratings of dyspnea and leg fatigue were measured during exercise. Three NIRS probes were placed over the prefrontal and temporoparietal cortical regions of the subjects' heads. Changes in cortical oxyhemoglobin (oxy-Hb), deoxyhemoglobin (deoxy-Hb), and total hemoglobin (total Hb) concentrations from baseline recordings were measured. Increased oxy-Hb (oxygenation) was assumed to reflect cortical activation.Oxy-Hb concentration was significantly increased in the prefrontal region during exercise in both groups but not in the temporoparietal regions. The change in prefrontal oxy-Hb concentration of COPD patients was not different from that of controls. Dyspnea scores were positively correlated with changes in oxy-Hb concentrations of the prefrontal regions in both groups. Multivariate analysis showed that oxy-Hb concentration in the prefrontal region was the best predictor of dyspnea in both groups.Exertional dyspnea was related to activation (oxygenation) of the prefrontal cortex in COPD patients and control subjects.
Although the amiloride-sensitive epithelial sodium channel (ENaC) plays an important role in the modulation of alveolar liquid clearance, the precise mechanism of its regulation in alveolar epithelial cells is still under investigation. Protein kinase C (PKC) has been shown to alter ENaC expression and activity in renal epithelial cells, but much less is known about its role in alveolar epithelial cells. The objective of this study was to determine whether PKC activation modulates ENaC expression and transepithelial Na + transport in cultured rat alveolar epithelial cells. Alveolar type II cells were isolated and cultured for 3 to 4 d before they were stimulated with phorbol 12-myristate 13-acetate (PMA 100 nmol/L) for 4 to 24 h. PMA treatment significantly decreased α, β, and γENaC expression in a time-dependent manner, whereas an inactive form of phorbol ester had no apparent effect. This inhibitory action was seen with only 5-min exposure to PMA, which suggested that PKC activation was very important for the reduction of αENaC expression. The PKC inhibitors bisindolylmaleimide at 2 µmol/L and Gö6976 at 2 µmol/L diminished the PMA-induced suppression of αENaC expression, while rottlerin at 1 µmol/L had no effect. PMA elicited a decrease in total and amiloride-sensitive current across alveolar epithelial cell monolayers. This decline in amiloride-sensitive current was not blocked by PKC inhibitors except for a partial inhibition with bisindolylmaleimide. PMA induced a decrease in rubidium uptake, indicating potential Na + -K + -ATPase inhibition. However, since ouabain-sensitive current in apically permeabilized epithelial cells was similar in PMA-treated and control cells, the inhibition was most probably related to reduced Na + entry at the apical surface of the cells. We conclude that PKC activation modulates ENaC expression and probably ENaC activity in alveolar epithelial cells. Ca 2+ -dependent PKC is potentially involved in this response.Key words: alveolar epithelial cells, Na + transport, Na + channel, ENaC, protein kinase C, Na + -K + -ATPase, amiloride, gene expression.