肺疾患と治療のジャーナル

Pulmonary Hypertension (PH) is regarded as a mean pulmonary artery pressure greater than 25 mm Hg in the setting of normal or reduced cardiac output and a normal pulmonary capillary wedge pressure. A constellation of permissive and provocative factors exists, various mechanisms are activated that lead to vascular constriction, cellular proliferation, and a prothrombotic state in varying degrees, which results in PH and its clinical sequelae [1-6]

The imaging techniques can be classified into two main groups: Structural/morphological imaging (SMI), which includes X rays (XR), computed tomography (CT), ultrasounds (US) as well as some varieties of magnetic resonance (MRI), and shows anatomic-morphological aspects, and molecular imaging (MI), which includes nuclear medicine (SPECT, PET), fMRI, optical and nanosystems techniques, and provides information about biochemistry/biological activity, often before structural changes. According to Society of Nuclear Medicine and Molecular Imaging, MI “is the visualization, characterization, and measurement of biological processes at the molecular and cellular levels in humans and other living systems”. MI procedures are noninvasive, safe and painless. Its sensitivity is greater than SMI, but it lacks anatomical detail, which has led to the development of multimodal imaging, combining structural and molecular techniques, widely used at present in daily practice. The pillars of MI are biochemistry/biology, instrumentation and software, and its cycle is the following: study of biology/biochemistry of a process, establishment/definition of specific targets, and development of tracers, preclinical imaging, histological validation and finally clinical imaging. This new concept led to the individualized diagnostic and treatment, being the patient the center of the medical activity. “As opposed to the doctor-centric, curative model of the past, the future is going to be patient-centric and proactive” said Dr. Zerhouni (NIH Medline Plus Winter 2007). The doctor must adapt to the needs of the own patient and this fact requires a true change of heart, because MI is intimately tied to the biology of the disease to analyzing. A new and strong interrelationship came into being: a bidirectional system biology-imaging that will allow to be much more effective in the daily practice, not only in relation to diagnosis (specific

Abstract Objective: To systematically review the use of azathioprine as a treatment for connective tissue diseaseassociated interstitial lung disease (CTD-ILD) in terms of effectiveness and safety. Materials and methods: A literature search was performed using the PubMed, EMBASE, CINAHL, Cochrane, and Scopus databases. The search was restricted to articles published in English from 1950 to March 2018 that examined the use of azathioprine in patients with CTD-ILD and determined its effects on a primary or secondary endpoint. This review included studies that measured the impacts of azathioprine in terms of effectiveness and safety. Results: The search identified 15 studies with a total of 424 subjects. Two hundred twenty patients received azathioprine. A majority of the studies failed to provide clear evidence for the effectiveness of azathioprine. The reported adverse events were: death 4.5% (n=10), infection 1.3% (n=3), myelosuppression 0.9% (n=2), and malignancy 0.45% (n=1). The rate of azathioprine discontinuation due to treatment failure was 2.7% (n=6). Conclusions: No clear impacts of azathioprine have been reported, yet this review reveals that the drug is less useful than previously believed. In contrast to our current knowledge, this review suggests that the ILD histopathological pattern appears to be the most important determinant of treatment responses and prognosis, and treatment decisions should be based on this parameter, rather than the background CTD. AZA is a relatively safe option. More well-designed studies are needed. The recruitment of subjects based on the ILD pattern rather than CTD may produce more consistent results

Cystic fibrosis is a life threatening disease resulted by a genetic mutation of the cystic fibrosis conductance regulator (CFTR) gene. CFTR gene encodes for a chloride channel, essential for the normal flow of salt and water movements across the cell membranes. There is an improper regulation which results in thickened secretions in lung, liver, reproductive organs and pancreas. Most men with cystic fibrosis (CF) are sterile due to failure of the seminal vesicle, vas deferens, body and tail of the epididymis to develop and ejaculatory duct. Particularly salty-tasting skin, persistent coughing, shortness of breath, frequent chest and lung infections and poor growth or weight gain are some of the symptoms of cystic fibrosis within the first year of a baby’s life [1]. Diabetes mellitus, episodic and chronic distal intestinal obstruction syndrome, osteoporosis, chronic liver disease, oesophageal reflux and oesophagitis, nasal polyposis, portal hypertension, allergic bronchopulmonary aspergillosis (ABPA), gallstones, respiratory and cardiac fai