• Tuberculosis and Respiratory Diseases
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• v.73 no.5
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• pp.249-257
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• 2012

The frequency of diagnosing bronchiectasis is increasing around the world. Cystic fibrosis is the most common inherited cause of bronchiectasis, but there is increasing recognition of significant numbers of patients with bronchiectasis from various causes. With increasing awareness of bronchiectasis, a significant number of research, concerning the causes and treatments, were published over the past few years. Investigation of the underlying cause of bronchiectasis is the most important key to effective management. The purpose of this report is to review the immunological abnormalities that cause bronchiectasis in those that the cystic fibrosis has been excluded, identify the available evidences of current management, and discuss several controversies in the treatment of this disorder.

• Tuberculosis and Respiratory Diseases
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• v.77 no.5
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• pp.227-229
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• 2014

Segniliparus species is a novel genus that is reported to be the new emerging respiratory pathogens. Here, we report a very rare case of S. rugosus pulmonary infection in an immunocompetent patient with non-cystic fibrosis. The organism was identified by 16S rRNA gene sequencing. The patient was successfully treated with antibiotics.

• Tuberculosis and Respiratory Diseases
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• v.79 no.4
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• pp.267-273
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• 2016

Background: Cystic fibrosis (CF) is an autosomal recessive disorder with several clinical presentations. This study was undertaken in the Azeri Turkish population in Iran, to investigate gender differences in the age at onset and diagnosis, age of death, and duration of illness of CF. Methods: The data of 331 CF patients from 2001 to 2015 was surveyed. Parameters including age, sex, ${\Delta}F508$ mutation, age at onset, age at diagnosis, age of death and clinical presentations were evaluated for both sexes, using descriptive analysis. The association of gender with these variables was studied using logistic regression, chi-square test and Mann-Whitney U test by SPSS version 18. Odds ratio with a confidence interval of 95% and $p{\leq}0.05$ was considered statistically significant. Results: The study included 191 males (57.7%) and 140 females (42.3%), all showing statistically significant difference (p<0.001). Age duration differed between genders. Male and female patients were further under 9 and 4 years, respectively. The occurrence of ${\Delta}F508$ mutation was 0.51 times more in females than in males. Age, diagnosis and sex were closely associated: males were diagnosed at a significantly later age than females (p=0.05). While this compression performed based on clinical presentations, males with respiratory disease had a later median age at diagnosis than females at lifespan (p=0.001). The risk of infertility in males was approximately two times greater than in females (p=0.02). Conclusion: These findings indicate gender differences in CF patients. Future studies are needed to establish other differences and evaluate the causes for the gender variations.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.4
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• pp.306-314
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• 2018

Purpose: The objective of this study was to describe the clinical phenotypes of children and adolescents with cystic fibrosis (CF); and to assess the role of pancreatic insufficiency and neonatal screening in diagnosis. Methods: A cross-sectional study was conducted, which included 77 patients attending a reference center of CF between 2014 and 2016. Epidemiological data, anthropometric measurements, and the presence of pulmonary, pancreatic, gastrointestinal and hepatobiliary manifestations were evaluated based on clinical data and complementary examinations. Results: Of the 77 patients, 51.9% were male, with a median age of 147 months (7.0-297.0 months), and the majority showed adequate nutritional status. The most common phenotype was pulmonary (92.2%), followed by pancreatic (87.0%), with pancreatic insufficiency in most cases. Gastrointestinal manifestation occurred in 46.8%, with constipation being the more common factor. Hepatobiliary disease occurred in 62.3% of patients. The group with pancreatic insufficiency was diagnosed earlier (5.0 months) when compared to the group with sufficiency (84.0 months) (p=0.01). The age of diagnosis was reduced following implementation of neonatal screening protocols for CF (6.0 months before vs. 3.0 months after, p=0.02). Conclusion: The pulmonary phenotype was the most common, although extrapulmonary manifestations were frequent and clinically relevant, and should mandate early detection and treatment. Neonatal screening for CF led to earlier diagnosis in patients with pancreatic failure, and therefore, should be adopted universally.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.4
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• pp.283-292
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• 2022

Purpose: The prevalence of eosinophilic esophagitis (EoE) has been on the rise since it was first described in the 1990s. Several diseases and exogenous factors have been associated with EoE. Our aim was to investigate the epidemiology of EoE in cystic fibrosis (CF) patients. Methods: We identified individuals with CF from September 2014 to September 2019 within a database (IBM Explorys Solutions, Inc.). The prevalence of EoE in patients with CF was compared to the general population. Results: The database included 36,111,860 patients during the 5-year study period: 12,950 with CF (0.036%) and 28,090 with EoE (0.078%). EoE prevalence was higher in CF patients than the general population (46 in 10,000 vs. 7.8 in 10,000, p<0.001). Patients with CF and EoE were more likely to be male (50% vs. 33.5%, p<0.008), children (33.3% vs. 16.5%, p<0.001), and non-Hispanic (100% vs. 88.7%, p<0.001) than CF patients without EoE. CF with EoE patients were more likely to be children than EoE only (33.3% vs. 10.5%, p<0.001). Allergic conditions were generally more prevalent in CF with EoE than CF only (83.3% vs. 68.3%, p=0.01) and EoE only (83.3% vs. 69.3%, p=0.014). Conclusion: EoE is nearly 6-times more prevalent in CF patients. Those patients had higher incidence of other atopic conditions. EoE must be considered in the differential diagnosis of patients with CF presenting with dysphagia, refractory gastroesophageal reflux, vomiting, and other esophagus-related symptoms.

• Journal of Ginseng Research
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• v.45 no.1
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• pp.66-74
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• 2021

Background: Abnormal chloride (Cl-) transport has a detrimental impact on mucociliary clearance in both cystic fibrosis (CF) and non-CF chronic rhinosinusitis. Ginseng is a medicinal plant noted to have anti-inflammatory and antimicrobial properties. The present study aims to assess the capability of red ginseng aqueous extract (RGAE) to promote transepithelial Cl- secretion in nasal epithelium. Methods: Primary murine nasal septal epithelial (MNSE) [wild-type (WT) and transgenic CFTR-/-], fisher-rat-thyroid (FRT) cells expressing human WT CFTR, and TMEM16A-expressing human embryonic kidney cultures were utilized for the present experiments. Ciliary beat frequency (CBF) and airway surface liquid (ASL) depth measurements were performed using micro-optical coherence tomography (μOCT). Mechanisms underlying transepithelial Cl- transport were determined using pharmacologic manipulation in Ussing chambers and whole-cell patch clamp analysis. Results: RGAE (at 30㎍/mL of ginsenosides) significantly increased Cl- transport [measured as change in short-circuit current (ΔISC = ㎂/㎠)] when compared with control in WT and CFTR-/- MNSE (WT vs control = 49.8±2.6 vs 0.1+/-0.2, CFTR-/- = 33.5±1.5 vs 0.2±0.3, p < 0.0001). In FRT cells, the CFTR-mediated ΔISC attributed to RGAE was small (6.8 ± 2.5 vs control, 0.03 ± 0.01, p < 0.05). In patch clamp, TMEM16A-mediated currents were markedly improved with co-administration of RGAE and uridine 5-triphosphate (8406.3 +/- 807.7 pA) over uridine 5-triphosphate (3524.1 +/- 292.4 pA) or RGAE alone (465.2 +/- 90.7 pA) (p < 0.0001). ASL and CBF were significantly greater with RGAE (6.2+/-0.3 ㎛ vs control, 3.9+/-0.09 ㎛; 10.4+/-0.3 Hz vs control, 7.3 ± 0.2 Hz; p < 0.0001) in MNSE. Conclusion: RGAE augments ASL depth and CBF by stimulating Cl- secretion through CaCC, which suggests therapeutic potential in both CF and non-CF chronic rhinosinusitis.

• Journal of Microbiology and Biotechnology
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• v.11 no.5
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• pp.804-811
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• 2001

In many Gram-negative bacteria, autoinducers, such as N-acyl-L-homoserine lactone(acyl-HSL) and its derivative molecules, mediate the cell-density-dependnet expression of certain operons. The current study identified the autoinducers produced by Burkholderia cepacia G4, a trichloroethylene-degrading lagoon isolate, using TLC bioassays with Agrobacterium tumefaciens NT1(pDCI141E33) and Chromobacterium violaceum CVO26, and a GC-MS analysis. The ${R_f}\;and\;{R_t}$ values and mass spectra were compared with those of synthetic compounds. Based on the analyses, it was confirmed that G4 produces N-hexanoyl (C6)-, N-octanoyl (C8)-, N-decanoyl (C10)-, N-dodecanoyl (C12)-HSL, and an unknown active species. The integration of the GC peak areas exhibited a ratio of C8-HSL:C10-HSL:C12-HSL at 3:17:1 with C6-HSL and C10-HSL production at trace and micromolar levels, respectively, in the culture supernatants. Nutants partially defective in producing acyl-HSLs were also partially defective in the biosynthesis of an antibiotic substance. These results indicate that the autoinducer-dependent gene regulation in G4 is dissimilar to the clinical B. cepacia strains isolated from patients with cystic fibrosis.

• Journal of Pharmaceutical Investigation
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• v.30 no.1
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• pp.1-12
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• 2000

Gene therapy is a method for the treatment of diseases with introducing the gene-engineered materials into a patient with gene-deficiency disease (e.g. cystic fibrosis) or cancer to produce a therapeutic protein in a patient's cells. Successful gene therapy requires establishing both gene expression systems and delivery systems. Viral and non-viral vectors have been used for gene delivery. Viral vectors have a high transfection efficiency, but are limited in relations to issues of safety, toxicity and immunogenecity. Non-viral vectors are easy to prepare and relatively safe. However, non-viral vectors have a low transfection efficiency. Cationic liposomes are the most available among non-viral vectors. Cationic liposomes have been used to transfect cells both in vitro and in vivo experiments. Besides, several formulations containing cationic lipid are being used in clinical trials in cases of cystic fibrosis or cancer. A crucial subject to the further development of gene delivery vectors will be a long-term gene expression with following characteristics; protecting and deliverying DNA efficiently, non-toxic and non-immunogenic, and easy to produce in large scale.

• Clinical and Experimental Pediatrics
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• v.45 no.7
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• pp.923-927
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• 2002

Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis associated with renal tubular ectasia in a 3-year-old girl, whose chief complaint was abdominal distension. Her liver function test did not reveal any abnormal findings. Hepatosplenomegaly and multiple dilated bile ducts were seen in the abdominal CT scaning. Esophageal varix was not detected by an endoscopic examination. Microscopically, diffuse portal fibrosis and widening with proliferation of blie ductules in the liver specimen and tubular ectasia in renal cortex were seen.

• Journal of radiological science and technology
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• v.31 no.2
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• pp.129-134
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• 2008

Purpose: We examined the roles of Ultrasonography conductors by analyzing the results of tissue biopsy of complex cystic masse under the guidance of breast US. Objects and methods: This study was performed to a group of 178 who showed breast US indicating complex cystic masses among 342 patients who were definitely diagnosed by tissue biopsies and operations in our hospital from June 30th, 2003 to June 30th, 2007. The evaluation of tissues around, calcification, the distribution state of blood flow were excluded from the analysis subjects and logic 200 made by GE corporation and gun for core biopsy(Kimal corp., K7/MBD23) were used in this study. Results: The biopsy results of 178 subjects showed FCC (fibrocystic change)(n=56 : 31.4%), Fibrosis (n=41 : 23.0%), Fibroadenoma (n=20 : 11.2%), Epithelial hyperplasia (n=17 : 9.6%), Carcinoma (n=15 : 8.4%), Fibroadipose (n=8 : 4.5%), Sclerosing adenosis (n=7 : 3.9%), Duct ectasia (n=5 : 2.8%), Papiloma (n=5 : 2.8%), and Fat necrosis (n=1 : 0.6%), Hemangioma (n=1 : 0.6%), Abscess (n=1 : 0.6%), Dystrophic calcification(n=1 : 0.6%). Conclusion: The US showed that the results of the tissue biopsy of complex cystic masses were mostly carcinoma(8.4%). Most of them were benign and only 9.6% of epithelial hyperplasia which has high progression rate into malignant tumors epidemically showed malignancy. Most of them were included in the spectrum of fibrous cystic nodule. Even though these results are confirmed, further studies are required. As a result, a nodule which is not certified by US should be right to take the tissue biopsy, but if it's difficult due to patients or another reasons, re-check tests in three months are required. And systemic ultrasonography evaluation should be well recognized to conduct more careful and specific tests.