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https://www.readbyqxmd.com/read/28416135/all-the-rage-in-lung-disease-the-receptor-for-advanced-glycation-endproducts-rage-is-a-major-mediator-of-pulmonary-inflammatory-responses
#1
REVIEW
Elizabeth A Oczypok, Timothy N Perkins, Tim D Oury
The receptor for advanced glycation endproducts (RAGE) is a pro-inflammatory pattern recognition receptor (PRR) that has been implicated in the pathogenesis of numerous inflammatory diseases. It was discovered in 1992 on endothelial cells and was named for its ability to bind advanced glycation endproducts and promote vascular inflammation in the vessels of patients with diabetes. Further studies revealed that RAGE is most highly expressed in lung tissue and spurred numerous explorations into RAGE's role in the lung...
March 18, 2017: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/28404095/type-3c-pancreatogenic-diabetes-mellitus-secondary-to-chronic-pancreatitis-and-pancreatic-cancer
#2
REVIEW
Phil A Hart, Melena D Bellin, Dana K Andersen, David Bradley, Zobeida Cruz-Monserrate, Christopher E Forsmark, Mark O Goodarzi, Aida Habtezion, Murray Korc, Yogish C Kudva, Stephen J Pandol, Dhiraj Yadav, Suresh T Chari
Diabetes mellitus is a group of diseases defined by persistent hyperglycaemia. Type 2 diabetes, the most prevalent form, is characterised initially by impaired insulin sensitivity and subsequently by an inadequate compensatory insulin response. Diabetes can also develop as a direct consequence of other diseases, including diseases of the exocrine pancreas. Historically, diabetes due to diseases of the exocrine pancreas was described as pancreatogenic or pancreatogenous diabetes mellitus, but recent literature refers to it as type 3c diabetes...
November 2016: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28376407/baseline-predictors-of-aortic-stiffness-progression-among-multi-ethnic-asians-with-type-2-diabetes
#3
Mei Chung Moh, Chee Fang Sum, Subramaniam Tavintharan, Keven Ang, Simon Biing Ming Lee, Wern Ee Tang, Su Chi Lim
BACKGROUND AND AIMS: This 3-year prospective study aimed to identify baseline parameters that predicted the progression of carotid-femoral pulse wave velocity (cf-PWV), which was used to evaluate aortic stiffness, among Singapore's multi-ethnic Asians with type 2 diabetes (T2DM). METHODS: The cf-PWV was measured by the gold-standard tonometry method in 994 T2DM subjects at baseline and follow-up. The annual rate of cf-PWV change was calculated, and individuals above the 90(th) percentile with rate≥1...
January 27, 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28325727/effect-of-allergic-bronchopulmonary-aspergillosis-on-fev1-in-children-and-adolescents-with-cystic-fibrosis-a-european-cystic-fibrosis-society-patient-registry-analysis
#4
Athanasios G Kaditis, Michael Miligkos, Anna Bossi, Carla Colombo, Elpis Hatziagorou, Nataliya Kashirskaya, Isabelle de Monestrol, Muriel Thomas, Meir Mei-Zahav, George Chrousos, Anna Zolin
OBJECTIVE: To evaluate the effect of allergic bronchopulmonary aspergillosis (ABPA) on FEV1 percent predicted in children and adolescents with cystic fibrosis. DESIGN: Longitudinal data analysis (2008-2010). SETTING: Patients participating in the European Cystic Fibrosis Society Patient Registry. PARTICIPANTS: 3350 patients aged 6-17 years. MAIN OUTCOME MEASURE: FEV1percent predicted was the main outcome measure (one measurement per year per child)...
March 21, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28284526/association-between-glucose-intolerance-and-bacterial-colonisation-in-an-adult-population-with-cystic-fibrosis-emergence-of-stenotrophomonas-maltophilia
#5
C Lehoux Dubois, V Boudreau, F Tremblay, A Lavoie, Y Berthiaume, R Rabasa-Lhoret, A Coriati
BACKGROUND: Diabetes is common in cystic fibrosis (CF). Glucose can be detected in the airway when the blood glucose is elevated, which favours bacterial growth. We investigated the relationship between dysglycemia and lung pathogens in CF. METHODS: Cross-sectional and prospective analysis of CF patients (N=260) who underwent a 2h-oral glucose tolerance test. Clinical data was collected. RESULTS: Stenotrophomonas maltophilia (S. maltophilia) was the sole bacteria increased in dysglycemic (AGT: 20...
March 8, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28273890/cftr-is-involved-in-the-regulation-of-glucagon-secretion-in-human-and-rodent-alpha-cells
#6
Anna Edlund, Morten Gram Pedersen, Andreas Lindqvist, Nils Wierup, Malin Flodström-Tullberg, Lena Eliasson
Glucagon is the main counterregulatory hormone in the body. Still, the mechanism involved in the regulation of glucagon secretion from pancreatic alpha cells remains elusive. Dysregulated glucagon secretion is common in patients with Cystic Fibrosis (CF) that develop CF related diabetes (CFRD). CF is caused by a mutation in the Cl(-) channel Cystic fibrosis transmembrane conductance regulator (CFTR), but whether CFTR is present in human alpha cells and regulate glucagon secretion has not been investigated in detail...
December 2017: Scientific Reports
https://www.readbyqxmd.com/read/28254466/impaired-rib-bone-mass-and-quality-in-end-stage-cystic-fibrosis-patients
#7
Geneviève Mailhot, Natalie Dion, Delphine Farlay, Sébastien Rizzo, Nathalie J Bureau, Valérie Jomphe, Safiétou Sankhe, Georges Boivin, Larry C Lands, Pasquale Ferraro, Louis-Georges Ste-Marie
BACKGROUND: Advancements in research and clinical care have considerably extended the life expectancy of cystic fibrosis (CF) patients. However, with this extended survival come comorbidities. One of the leading co-morbidities is CF-related bone disease (CFBD), which progresses with disease severity and places patients at high risk for fractures, particularly of the ribs and vertebrae. Evidence that CF patients with vertebral fractures had higher bone mineral density (BMD) than the nonfracture group led us to postulate that bone quality is impaired in these patients...
May 2017: Bone
https://www.readbyqxmd.com/read/28247443/acute-kidney-injury-in-patients-treated-with-iv-beta-lactam-beta-lactamase-inhibitor-combinations
#8
W Cliff Rutter, David S Burgess
STUDY OBJECTIVE: Increased acute kidney injury (AKI) incidence has been reported in patients receiving piperacillin-tazobactam (PTZ) therapy compared with other β-lactams. The authors sought to determine if the addition of β-lactamase inhibitors impacts AKI incidence by comparing patients treated with PTZ or ampicillin-sulbactam (SAM). DESIGN: Retrospective cohort study. SETTING: Large academic tertiary care hospital. PATIENTS: Overall, 2448 patients received PTZ (n=1836) or SAM (n=612) for at least 48 hours between September 1, 2007, and September 30, 2015...
March 1, 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/28245190/cystic-fibrosis-related-diabetes-cfrd-is-preceded-by-and-associated-with-growth-failure-and-deteriorating-lung-function
#9
Nicolas Terliesner, Mandy Vogel, Anna Steighardt, Ruth Gausche, Constance Henn, Julia Hentschel, Thomas Kapellen, Sabine Klamt, Julia Gebhardt, Wieland Kiess, Freerk Prenzel
BACKGROUND: Impaired glucose metabolism and cystic fibrosis (CF)-related diabetes (CFRD) are associated with insufficient weight gain and impaired lung function in children and adolescents with CF. We have asked whether imminent CFRD may be a cause of poor growth in children and adolescents. METHODS: A retrospective case control study including 32 patients with CF with or without diabetes was conducted. Sixteen pairs, matched according to age, gender and exocrine pancreatic insufficiency, were analysed...
February 28, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28236043/cystic-fibrosis-in-austria
#10
Thomas Frischer, Ernst Eber, Helmut Ellemunter, Angela Zacharasiewicz, Ingrid Kaluza, Josef Riedler, Sabine Renner
Registry data for patients with cystic fibrosis (CF) are increasingly used to evaluate the natural history, for benchmarking of therapy and in order to identify eligible patients for clinical studies. So far, no data on frequency and clinical status of CF patients have been available for Austria on a national level. We collected data of CF patients treated 2014 in Austrian CF outpatient clinics by means of a European CF registry and on an individual search basis. A total of 773 CF patients with a median age of 18...
February 24, 2017: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/28229097/pediatric-hypovitaminosis-d-molecular-perspectives-and-clinical-implications
#11
Rafiu Ariganjoye
Vitamin D, a secosteroid, is essential for the development and maintenance of healthy bone in both the adult and pediatric populations. Low level of 25-hydroxy vitamin D (25-(OH)-D) is highly prevalent in children worldwide and has been linked to various adverse health outcomes including rickets, osteomalacia, osteomalacic myopathy, sarcopenia, and weakness, growth retardation, hypocalcemia, seizure and tetany, autism, cardiovascular diseases, diabetes mellitus, cancers (prostate, colon, breast), infectious diseases (viral, tuberculosis), and autoimmune diseases, such as multiple sclerosis and Hashimoto's thyroiditis...
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/28213469/insulin-signaling-via-the-pi3k-akt-pathway-regulates-airway-glucose-uptake-and-barrier-function-in-a-cftr-dependent-manner
#12
Samuel A Molina, Hannah K Moriarty, Danny T Infield, Barry R Imhoff, Rachel J Vance, Agnes H Kim, Jason M Hansen, William R Hunt, Michael Koval, Nael A McCarty
Cystic fibrosis-related diabetes (CFRD) is the most common co-morbidity associated with cystic fibrosis (CF) and correlates with increased rates of lung function decline. Since glucose is a nutrient present in the airways of patients with bacterial airway infections and since insulin controls glucose metabolism, the effect of insulin on CF airway epithelia was investigated to determine the role of insulin receptors and glucose transport in regulating glucose availability in the airway. The response to insulin by human airway epithelial cells was characterized by qPCR, immunoblot, immunofluorescence, and glucose uptake assays...
February 17, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28167560/nephrotoxicity-in-patients-with-or-without-cystic-fibrosis-treated-with-polymyxin-b-compared-to-colistin
#13
Ryan L Crass, W Cliff Rutter, Donna R Burgess, Craig A Martin, David S Burgess
Nephrotoxicity is the primary adverse effect of the polymyxins. The relative rates of toxicity of polymyxin B and colistin have not been fully elucidated, especially in patients with cystic fibrosis (CF). A retrospective cohort study of adults treated with polymyxin B or colistin for at least 48 h was conducted. The primary endpoint was the incidence of kidney injury assessed by RIFLE (i.e., risk, injury, failure, loss, end-stage renal disease) criteria. Risk factors for kidney injury were evaluated using multivariate Cox regression...
April 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28164596/genetic-expression-in-cystic-fibrosis-related-bone-disease-an-observational-transversal-cross-sectional-study
#14
Ioana M Ciuca, Liviu L Pop, Alexandru F Rogobete, Dan I Onet, Bogdan Guta-Almajan, Zoran Popa, Florin G Horhat
BACKGROUND: Cystic fibrosis (CF) is the most frequent monogenic genetic disease with autosomal recessive transmission and characterized by important clinical polymorphism and significant lethal prospective. CF related bone disease occurs frequently in adults with CF. Childhood is the period of bone formation, and therefore, children are more susceptible to low bone density. Several factors like pancreatic insufficiency, hormone imbalance, and physical inactivity contribute to CF bone disease development...
September 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28115168/heterogeneity-in-survival-among-adult-cystic-fibrosis-patients-with-fev1-30-of-predicted-in-the-united-states
#15
Kathleen J Ramos, Bradley S Quon, Sonya L Heltshe, Nicole Mayer-Hamblett, Erika D Lease, Moira L Aitken, Noel S Weiss, Christopher H Goss
BACKGROUND: Lung transplantation (LTx) is frequently considered for patients with cystic fibrosis (CF) when FEV1 reaches <30%. This study estimated transplant-free survival for CF patients and FEV1 <30% and identified predictors of death without LTx. METHODS: We conducted a retrospective cohort study using the CF Foundation Patient Registry, 1/1/2003-12/31/2013. Adult patients (≥18 years) with FEV1 <30% prior to LTx were included. We performed Kaplan-Meier survival estimates censored at LTx...
January 20, 2017: Chest
https://www.readbyqxmd.com/read/28106773/determinants-of-serum-glycerophospholipid-fatty-acids-in-cystic-fibrosis
#16
Sławomira Drzymała-Czyż, Patrycja Krzyżanowska, Berthold Koletzko, Jan Nowak, Anna Miśkiewicz-Chotnicka, Jerzy A Moczko, Aleksandra Lisowska, Jarosław Walkowiak
The etiology of altered blood fatty acid (FA) composition in cystic fibrosis (CF) is understood only partially. We aimed to investigate the determinants of serum glycerophospholipids' FAs in CF with regard to the highest number of FAs and in the largest cohort to date. The study comprised 172 CF patients and 30 healthy subjects (HS). We assessed Fas' profile (gas chromatography/mass spectrometry), CF transmembrane conductance regulator (CFTR) genotype, spirometry, fecal elastase-1, body height and weight Z-scores, liver disease, diabetes and colonization by Pseudomonas aeruginosa...
January 18, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28102546/ataluren-and-similar-compounds-specific-therapies-for-premature-termination-codon-class-i-mutations-for-cystic-fibrosis
#17
REVIEW
Aisha A Aslam, Colin Higgins, Ian P Sinha, Kevin W Southern
BACKGROUND: Cystic fibrosis is a common life-shortening genetic disorder in the Caucasian population (less common in other ethnic groups) caused by the mutation of a single gene that codes for the production of the cystic fibrosis transmembrane conductance regulator protein. This protein coordinates the transport of salt (and bicarbonate) across cell surfaces and the mutation most notably affects the airways. In the lungs of people with cystic fibrosis, defective protein results in a dehydrated surface liquid and compromised mucociliary clearance...
January 19, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28054445/anti-diabetic-activities-of-cis-and-trans-2-3-5-4-tetrahydroxystilbene-2-o-%C3%AE-glucopyranoside-from-polygonum-multiflorum
#18
Wenping Tang, Shiming Li, Yue Liu, Jia-Ching Wu, Min-Hsiung Pan, Mou-Tuan Huang, Chi-Tang Ho
SCOPE: Functional foods can be used alone or in combination with existing therapies in preventing and treating type 2 diabetes (T2D). Trans-2,3,5,4'-tetrahydroxystilbene 2-O-β-glucopyranoside (trans-THSG), a dominant bioactive compound from Polygonum multiflorum (PM)-a popular medicinal food in Asia, has attracted increasing research interests due to its strong antioxidant activity. The content of naturally occurring cis-THSG (cis-2,3,5,4'-tetrahydroxystilbene 2-O-β-glucopyranoside) was very low in PM root, but was prepared in this study by mimicking the traditional process of PM...
January 5, 2017: Molecular Nutrition & Food Research
https://www.readbyqxmd.com/read/28024941/orthostatic-hypotension-after-continuous-flow-left-ventricular-assist-device-implantation-in-a-patient-with-longstanding-diabetes-mellitus
#19
Derek S Chew, Brett H Shaw, Debra L Isaac, Jonathan G Howlett, Satish R Raj
A continuous-flow left ventricular assist device (CF-LVAD) benefits patients with advanced heart failure as a bridge to cardiac transplantation. However, unanticipated complications may occur. We report a patient with end-stage heart failure and longstanding diabetes who experienced functionally debilitating orthostatic hypotension from autonomic insufficiency after CF-LVAD implantation. This case demonstrates a role for comprehensive autonomic function testing in the workup of orthostatic hypotension after LVAD implantation...
October 28, 2016: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28018696/alagille-syndrome-a-case-report-highlighting-dysmorphic-facies-chronic-illness-and-depression
#20
James J Bresnahan, Zachary A Winthrop, Rabia Salman, Salman Majeed
Alagille syndrome is a rare multisystem disorder affecting the liver, heart, vertebrae, eyes, and face. Alagille syndrome shares multiple phenotypic variants of other congenital or chronic childhood illnesses such as DiGeorge syndrome, Down syndrome, spina bifida, type 1 diabetes mellitus, and cystic fibrosis. All of these chronic illnesses have well-established links to psychiatric conditions. There are few community resources for Alagille patients, as it is an extremely rare condition. Despite the overlap with other chronic childhood illnesses, the psychiatric manifestations of Alagille syndrome have not been previously discussed in literature...
2016: Case Reports in Psychiatry
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