Yield of chest computed tomography angiogram in cystic fibrosis patients with suspected pulmonary embolism

IntroductionIndividuals with cystic fibrosis (CF) may be at increased risk of pulmonary embolism (PE). Symptoms of PE overlap substantially with those of CF respiratory exacerbations. CF patients commonly undergo chest computed tomography (CT) angiograms (CTPA) to evaluate for PE, but little is known about the clinical presentation and diagnosis of PE in this population.ObjectivesThe objectives of this study are to determine the diagnostic yield of CTPA for PE in adult patients with CF and assess the utility of the Revised Geneva Score (RGS) in this population.MethodsRetrospective review of all CTPA results was performed on CF patients with suspected PE at a large CF center from 1 January 2011 through 31 March 2017. Patient demographics, medical history, and presenting signs and symptoms were abstracted by chart review.ResultsA total of 103 unique CTPA studies were performed in 68 patients. Most were hospitalized at the time of CTPA, predominantly for respiratory manifestations of CF. CTPA identified four patients with PE. The small number of positive studies precluded analysis of predictors of PE. Fewer PE were diagnosed than predicted by the Revised Geneva Score, which was intermediate probability in 77/103 (75%) patients.ConclusionThe prevalence of PE in CF patients undergoing CTPA for suspected PE was 4%, which is lower than predicted by the Revised Geneva Score. This may be due to a large overlap in the signs and symptoms of PE and exacerbations of CF lung disease.     

Clinical evaluation of the infertile male with respect to genetic etiologies

Severe male factor infertility may have a presently identifiable genetic basis. Y chromosomal microdeletions (e.g., an AZFc microdeletion), karyotypic anomalies (e.g., Klinefelter Syndrome), and mutations in both alleles of the cystic fibrosis transmembrane conductance regulator (CFTR) gene may be found, depending upon the etiology of the reproductive compromise. Which patients should be tested, what are the tests that can be performed, when should those tests be ordered, and what might a positive outcome mean, are all critically valuable clinical questions for the couple that help guide evaluation and management. It is imperative that they be asked and results discussed prior to any intervention such as testis tissue extraction, microsurgical epididymal sperm aspiration, or intracytoplasmic sperm injection so that the couple can incorporate them in their decisions moving forward vis-à-vis their reproductive choices and options. The role of reproductive medicine clinicians should not be limited to just helping couples establish a pregnancy, but instead be expanded to educating them about the reasons and causes of their reproductive failure that affect not only them as individuals but also may have implications for their offspring.     

Contemporary psychosocial issues in cystic fibrosis: treatment adherence and quality of life

Summary

This review focuses on the two major psychosocial areas currently topical in cystic fibrosis research: treatment adherence and quality of life. First, the paper discusses the importance of evaluating adherence to treatments and medical advice, its measurement, predictors of adherence and non-adherence and whether the data should be used to inform clinical practice and policy decisions. Second, the purpose of evaluating quality of life in cystic fibrosis and an overview of the data are discussed. Both areas have suffered from problems with terminology and definition, inappropriate methodologies and hence difficulties with data interpretation. Future directions for research are advocated.     

Single-incision laparoscopic surgery using an articulating monopolar for juvenile cystic adenomyoma

Abstract

We demonstrate the feasibility of single-incision laparoscopic surgery (SILS) by using a novel monopolar cautery and a technique based on conventional multiport laparoscopic surgery in a patient with Juvenile cystic adenomyoma (JCA). A 20-year-old woman with severe dysmenorrhea, and a 3-cm uterine lesion was diagnosed with JCA. 70 cm of absorbable string tied to the tumor parenchyma was used to extract the tumor. The ends of the strings were positioned extracorporeally and the intra-abdominal parts of the strings adjacent to the anchored tumor were grasped by a laparoscopic needle holder for clarifying the incision line surrounding the tumor. The lesion was completely excised using a single-use articulating hook with monopolar cautery, and the hysterotomy site was sutured using barbed string. No complications occurred intraoperatively, and dysmenorrhea had significantly improved three months after surgery. SILS for JCA is a feasible and minimally invasive surgery for women desiring esthetic benefit.     

The current state of antibacterial research

‵...some would say that the era of once-daily HAART is upon us. Others would advocate for randomized controlled trials including more established twice-daily combinations before such a statement can be made. Although these studies may be interesting, there are a number of individual patients who will not wait.′     

Overlooked aspects concerning development and spread of antimicrobial resistance

miRNAs are short, nonprotein coding RNAs that regulate target gene expression principally by causing translational repression and/or mRNA degradation. miRNAs are involved in most mammalian biological processes and have pivotal roles in controlling the expression of factors involved in basal and stimulus-induced signaling pathways. Considering their central role in the regulation of gene expression, miRNAs represent therapeutic drug targets. Here we describe how miRNAs are involved in the regulation of aspects of innate immunity and inflammation, what happens when this goes awry, such as in the chronic inflammatory lung diseases cystic fibrosis and asthma, and discuss the current state-of-the-art miRNA-targeted therapeutics.     

Inhaled ciprofloxacin for chronic airways infections caused by Pseudomonas aeruginosa

Chronic infection of the airways with Pseudomonas aeruginosa represents a therapeutic challenge. Currently existing approaches are particularly based on systemic approaches and this increases the risk of strain resistance selection and is frequently associated with side effects. Therefore, local delivery of antibiotics may reduce these risks and may provide sustained sterilization of the respiratory tract. In fact, this approach was found to be effective with inhaled tobramycin in cystic fibrosis. Inhaled ciprofloxacin (dry powder inhaled formulation or liposomal inhaled formulation) is currently being investigated in chronic infections of the airways in cystic fibrosis, non-cystic fibrosis bronchiectasis and chronic obstructive pulmonary disease, and the initial clinical data are encouraging. This paper presents a perspective on the potential role of inhaled ciprofloxacin in such infections.     

Differential expression of galectin-3, β-catenin, and cyclin D1 in adenoid cystic carcinoma and polymorphous low-grade adenocarcinoma of salivary glands

Background:  Galectin-3 has been implicated in tumor progression of some malignancies as thyroid, prostate, and salivary gland tumors. Recently, it has been suggested that this protein may be an important mediator of the β-catenin/Wnt pathway. Moreover, nuclear galectin-3 expression has been implicated in cell proliferation, promoting cyclin D1 activation. Thus, the present study aimed to correlate galectin-3 expression with β-catenin and cyclin D1 expressions in adenoid cystic carcinoma (ACC) and in polymorphous low-grade adenocarcinoma (PLGA).
Methods:  Fifteen formalin-fixed paraffin-embedded cases of each tumor were retrieved from the files of the Surgical Oral Pathology Service at the University of São Paulo and the proteins were analyzed by immunohistochemistry.
Results:  Adenoid cystic carcinoma showed galectin-3 immunostaining mainly in the nuclei, while PLGA revealed a positive mostly cytoplasmic reaction to galectin-3 in the largest part of tumor cells. Both tumors showed intense cytoplasmic/nuclear staining for β-catenin in majority of cases. Cyclin D1 immunoreactivity was not detected in 14/15 PLGA and showed specific nuclear staining in 10/15 cases of ACC in more than 5% of the neoplastic cells. Cyclin D1 expression was correlated with cytoplasmic and nuclear galectin-3 expression in ACC ( P  < 0.05).
Conclusions:  These results suggest that in ACC galectin-3 may play a role in cellular proliferation through cyclin D1 activation. In addition, nuclear expression of galectin-3 in ACC may be related to a more aggressive behavior of this lesion. Although β-catenin seems to play a role in carcinogenesis in both lesions, it seems that it does not bind to galectin-3 for cyclin D1 stimulation.     

宁夏西吉县人体两种肝包虫病调查的初步报告

采用梯度筛选后B超检查,对西吉县农民、城镇人员患肝泡球蚴病(LAE)和肝棘球蚴病(LCECE)的情况进行调查。共检查2 389人,检出肝包虫病患者197例,患病率为8.2％,其中LAE 141例,占5.9％;LCR 56例,占2.3％。农民LAE和LCE患病率分别为9.7％和3.8％。城镇的分别为0.4％和0.2％。农民患病率高。LAE与猎狐、捕捉达乌尔黄鼠有密切关系。家犬是LCE的传染源。     

Misdiagnosing cystic fibrosis in the era of gene analysis: case reports

We present a scenario of how gene analysis plays a confusing role in the diagnosis of cystic fibrosis (CF). One of the two siblings we are presenting here was initially misdiagnosed as having CF, based on the two CF gene mutations identified by gene analysis. A CF gene study on the other sibling years later, however, led to further investigation and eventually to a change of diagnosis. As interesting and important as gene analysis is in CF, one must always look at each patient in the big picture. Included in the picture, in addition to the state-of-the-art genotype, is the phenotype, or (to simplify) the back-to-basic clinical manifestations.