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Deepthi Alapati, Edward E Morrisey
While our understanding of the genetics and pathology of congenital lung diseases such as surfactant protein deficiency, cystic fibrosis and alpha 1 antitrypsin deficiency is extensive, treatment options are lacking. Since the lung is a barrier organ in direct communication with the external environment, targeted delivery of gene corrective technologies to the respiratory system via intra-tracheal or intranasal routes is an attractive option for therapy. CRISPR/Cas9 gene editing technology is a promising approach to repair or inactivate disease causing mutations...
October 25, 2016: American Journal of Respiratory Cell and Molecular Biology
Hui Ren, Nigel P Birch, Vinod Suresh
Robust and reproducible in vitro models are required for investigating the pathways involved in fluid homeostasis in the human alveolar epithelium. We performed functional and phenotypic characterisation of ion transport in the human pulmonary epithelial cell lines NCI-H441 and A549 to determine their similarity to primary human alveolar type II cells. NCI-H441 cells exhibited high expression of junctional proteins ZO-1, and E-cadherin, seal-forming claudin-3, -4, -5 and Na+-K+-ATPase while A549 cells exhibited high expression of pore-forming claudin-2...
2016: PloS One
David M Vock, Michael T Durheim, Wayne M Tsuang, C Ashley Finlen Copeland, Anastasios A Tsiatis, Marie Davidian, Megan L Neely, David J Lederer, Scott M Palmer
RATIONALE: Lung transplantation is an accepted and increasingly employed treatment for advanced lung diseases, but the anticipated survival benefit of lung transplantation is poorly understood. OBJECTIVES: To determine whether and for which patients lung transplantation confers a survival benefit in the modern era of US lung allocation. METHODS: Data on 13040 adults listed for lung transplantation between May 2005 and September 2011 were obtained from the United Network for Organ Sharing...
October 25, 2016: Annals of the American Thoracic Society
C Esposito, M Escolino, B Troncoso Solar, R Iacona, R Esposito, A Settimi, I Mushtaq
OBJECTIVES: To document the imaging follow-up of laparoscopic partial nephrectomy (LPN) in children and to investigate the natural history of cystic lesions post-LPN. MATERIALS AND METHODS: We reviewed the US imaging reports performed during follow-up in 125 children (77 girls, 48 boys - average age 3.2 years) underwent LPN in 2 centers of pediatric surgery in the period 2005-2015. RESULTS: Transperitoneal approach was adopted in 83 cases while retroperitoneoscopy in 42 cases...
October 25, 2016: BJU International
Jeng-Haur Chen, Weiyi Xu, David N Sheppard
Cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP-gated Cl(-) channel defective in the genetic disease cystic fibrosis (CF). The gating behaviour of CFTR is characterized by bursts of channel openings interrupted by brief, flickery closures, separated by long closures between bursts. Entry to and exit from an open burst is controlled by the interaction of ATP with two ATP-binding sites, sites 1 and 2 in CFTR. To understand better the kinetic basis of CFTR intraburst gating, we investigated the single-channel activity of human CFTR at different intracellular pH (pHi ) values...
October 25, 2016: Journal of Physiology
Hiroyuki Isayama, Yousuke Nakai, Tsuyoshi Hamada, Saburo Matsubara, Hirofumi Kogure, Kazuhiko Koike
Self-expandable metallic stent (SEMS) was an effective biliary endoprosthesis. Mechanical properties of SEMS, radial and axial force (RF, AF), may play important roles in the bile duct after placement. RF was well known dilation force and influenced on the occurrence of migration. AF, newly proposed by this author, was defined as the recovery force when the SEMS vended. AF was related with the cause of bile duct kinking, pancreatitis, and cholecystitis due to the compression of the bile duct, orifice of the cystic duct, and pancreatic orifice...
December 2016: Current Gastroenterology Reports
Mayank Roy, Fernando Dip, David Nguyen, Conrad H Simpfendorfer, Emanuele Lo Menzo, Samuel Szomstein, Raul J Rosenthal
BACKGROUND: Intraoperative incisionless fluorescent cholangiogram (IOIFC) has been demonstrated to be a useful tool to increase the visualization of Calot's triangle. This study evaluates the identification of extrahepatic biliary structures with IOIFC by medical students and surgery residents. METHODS: Two pictures were taken, one with xenon light and one with near-infrared (NIR) light, at the same stage during dissection of Calot's triangle in ten different cases of laparoscopic cholecystectomy (LC)...
October 24, 2016: Surgical Endoscopy
Sladjana Skopelja, B JoNell Hamilton, Jonathan D Jones, Mei-Ling Yang, Mark Mamula, Alix Ashare, Alex H Gifford, William F C Rigby
While respiratory failure in cystic fibrosis (CF) frequently associates with chronic infection by Pseudomonas aeruginosa, no single factor predicts the extent of lung damage in CF. To elucidate other causes, we studied the autoantibody profile in CF and rheumatoid arthritis (RA) patients, given the similar association of airway inflammation and autoimmunity in RA. Even though we observed that bactericidal permeability-increasing protein (BPI), carbamylated proteins, and citrullinated proteins all localized to the neutrophil extracellular traps (NETs), which are implicated in the development of autoimmunity, our study demonstrates striking autoantibody specificity in CF...
October 20, 2016: JCI Insight
Sang-Hyun Park, Gyu-Min Kong, Yong-Uk Kwon, Jun-Ho Park
Brown tumor refers to a change of skeletones that develops as a complication of hyperparathyroidism. As osteoclast is activated to stimulate reabsorption and fibrosis of bone, it causes a cystic change of the bone. Parathyroid carcinoma is being reported as a tumor that induces primary hyperparathyroidism. It causes excessive secretion of the parathyroid hormone and increases the blood parathyroid hormone and calcium. Bone deformation due to brown tumor is known to be naturally recovered through the treatment for hyperparathyroidism...
September 2016: Hip Pelvis
Sebastian Jobjörnsson, Martin Forster, Paolo Pertile, Carl-Fredrik Burman
We present a model combining the two regulatory stages relevant to the approval of a new health technology: the authorisation of its commercialisation and the insurer's decision about whether to reimburse its cost. We show that the degree of uncertainty concerning the true value of the insurer's maximum willingness to pay for a unit increase in effectiveness has a non-monotonic impact on the optimal price of the innovation, the firm's expected profit and the optimal sample size of the clinical trial. A key result is that there exists a range of values of the uncertainty parameter over which a reduction in uncertainty benefits the firm, the insurer and patients...
October 5, 2016: Journal of Health Economics
Yoshifumi Noda, Satoshi Goshima, Kimihiro Kajita, Hiroshi Kawada, Nobuyuki Kawai, Hiromi Koyasu, Masayuki Matsuo, Kyongtae T Bae
PURPOSE: To evaluate the association between gadoxetic-acid-enhanced magnetic resonance (MR) imaging measurements and laboratory and clinical biomarkers of liver function and fibrosis. MATERIALS AND METHODS: One hundred thirty nine consecutive patients with suspected liver disease or liver tumor underwent gadoxetic-acid-enhanced MR imaging. MR imaging measurements during the hepatobiliary phase included biliary tract structure-to-muscle signal intensity ratio (SIR)...
November 2016: European Journal of Radiology
M Kadihasanoglu, M Kilciler, O Atahan
A 44-year-old woman, who had had left flank pain for the previous 3 months, was treated successfully for renal hydatid cyst disease by using an endoscopic technique with percutaneous access through an all seeing needle. Abdominal ultrasonography showed a Gharbi type III cyst in the lower pole of the left kidney. Computerised tomography of the abdomen revealed a bulky solid-cystic mass with calcified and well-defined wall and daughter cyst without contrast enhancement. The patient, who refused any renal operation, underwent a percutaneous intervention with access through an all seeing needle access...
October 24, 2016: Aktuelle Urologie
Atsushi Takano, Hiroshi Nakagomi, Kou Ikegame, Atsushi Yamamoto, Hideki Watanabe, Haruka Nakada, Masayuki Inoue, Hidemitsu Sugai, Michiya Yasutome, Kazushige Furuya, Masao Hada, Yoshiaki Miyasaka, Toshio Oyama, Masao Omata
INTRODUCTION: The over-expression of P53 protein in gallbladder carcinoma is a biomarker correlating with a poor survival. However, the significance of P53 expression in peritumor tissues is unknown. We experienced a case of gallbladder carcinoma where the operative specimen showed over-expression of P53 on the peritumor epithelium, and early recurrence developed at the biliary tract. PRESENTATION OF CASE: A 74-year-old female patient was referred to our hospital due to wall thickening of the gallbladder on ultrasonography...
October 18, 2016: International Journal of Surgery Case Reports
Money Gupta, Rashi Chauhan, Yamuna Prasad, Gulshan Wadhwa, Chakresh Kumar Jain
The lack of complete treatments and appearance of multiple drug-resistance strains of Burkholderia cepacia complex (Bcc) are causing an increased risk of lung infections in cystic fibrosis patients. Bcc infection is a big risk to human health and demands an urgent need to identify new therapeutics against these bacteria. Network biology has emerged as one of the prospective hope in identifying novel drug targets and hits. We have applied protein-protein interaction methodology to identify new drug-target candidates (orthologs) in Burkhloderia cepacia GG4, which is an important strain for studying the quorum-sensing phenomena...
October 8, 2016: Computational Biology and Chemistry
Raffaele Pezzilli, Gianvico Melzi dʼEril, Alessandra Barassi
OBJECTIVE: This study aimed to assess the presence of pancreatic hyperenzymemia in patients with pancreatic cystic lesions as compared to other chronic diseases of the pancreas. METHODS: Ninety-one patients were studied: 32 had mucinous cystic lesions, 35 had chronic pancreatitis (CP), and 24 had pancreatic ductal adenocarcinoma (PDAC). Surgery was carried out in 10 of the 32 patients with mucinous cystic lesion (7 of them had severe dysplasia), in 5 patients with CP, and in 9 patients with PDAC...
October 2016: Pancreas
Stijn van Weert, Rinze Reinhard, Elisabeth Bloemena, Jan Buter, Birgit I Witte, Marije R Vergeer, C René Leemans
BACKGROUND: We examined the assumption in conventional teaching about metastatic adenoid cystic carcinoma (ACC) being an indolent type of disease. METHODS: A single center analysis of 105 cases of ACC was performed. Radiographs were reviewed and tumor response to chemotherapy was measured. Distant disease-free survival (DDFS) and time to death since distant metastases diagnosis were analyzed. RESULTS: Forty-two percent of the patients were diagnosed with distant metastases...
October 24, 2016: Head & Neck
Ursula N Broder, Tina Jaeger, Urs Jenal
Virulence of pathogenic bacteria is a tightly controlled process to facilitate invasion and survival in host tissues. Although pathways controlling virulence have been defined in detail, signals modulating these processes are poorly understood. The opportunistic pathogen Pseudomonas aeruginosa causes acute and chronic infections in humans. Disease progression is typically associated with a loss of acute virulence and the emergence of biofilms and chronic behaviour. The acute-to-chronic switch is governed by the global Gac/Rsm pathway...
October 24, 2016: Nature Microbiology
M Oviedo Gutiérrez, S Amat Valero, A Gómez Farpón, C Montalvo Ávalos, L Fernández García, D C Lara Cárdenas, S Barnes Marañón, C Granell Suárez, N Vega Mata, A J López López, M González Guerrero, V Álvarez Muñoz
PURPOSE: Gastrointestinal adenomyoma is a rare benign tumor most frequently located in the stomach. The differential diagnosis is wide because of its large clinical spectrum and unspecific radiological findings. Surgical excision is both diagnostic and therapeutic. CASE REPORT: A 49-days old girl presented with nonbilious vomiting of 48 hours of evolution. Infantile hypertrofic pyloric stenosis was suspected. Ultrasound showed a nonobstructive nodular lesion in the anterior pyloric wall...
April 20, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
G Alonso Ciodaro, J I Santamaría Ossorio, S Sanjuán Rodríguez
INTRODUCTION: Cecal duplications are rare, representing 0.4% of all gastrointestinal duplications, with few cases reported in the literature. CLINICAL CASE: A 48 hours-old newborn presented with bilious vomiting and abdominal distension; the ultrasound revealed a cystic mass in the right abdomen; ileocolic resection was performed with end to end anastomosis. Microscopy confirmed cecal duplication cyst. COMMENTS: In patients with intestinal obstruction, contrast enema allows differential diagnosis with other colonic pathologies in the newborn; when the cecal duplication cyst is connected to the lumen of the colon, enema reveals a filling defect, and cecal duplication can be suspected...
October 10, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
Meredith C Fidler, Jack Beusmans, Paul Panorchan, Frederick Van Goor
Ivacaftor, a CFTR potentiator that enhances chloride transport by acting directly on CFTR to increase its channel gating activity, has been evaluated in patients with different CFTR mutations. Several previous analyses have reported no statistical correlation between change from baseline in ppFEV1 and reduction in sweat chloride levels for individuals treated with ivacaftor. The objective of the post hoc analysis described here was to expand upon previous analyses and evaluate the correlation between sweat chloride levels and absolute ppFEV1 changes across multiple cohorts of patients with different CF-causing mutations who were treated with ivacaftor...
October 20, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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