tbuckley/cabot-search huggingface dataset:
P91 Nitisinone treatment reduces the need for liver transplantation in children with tyrosinaemia type 1 and is associated with improved post-transplant renal tubular function
Authors: D Bartlett, C Lloyd, D Mirza, P McKiernan, P Newsome
Journal: Gut (2010)

Introduction  Tyrosinaemia Type 1 (TT1) is a disorder of tyrosine metabolism which may lead to liver failure and a high risk of hepatocellular carcinoma (HCC). Treatment previously consisted of dietary restriction and orthotopic liver transplantation (OLT) but was transformed by the introduction of Nitisinone in 1992. Here we report how Nitisione has altered the outcome of and need for OLT in patients with TT1 in our centre.  Method  A retrospective analysis was performed of patients treated for TT1 at Birmingham Children9s Hospital from 1989 to 2009.  Results  38 patients were treated with no significant difference in the annual number of patients seen before and after 1992 (p=0.47). 6/7 (85.7%) seen prior to 1992 and 7/31 (22.6%) initially treated with Nitisinone underwent OLT. The primary indication for OLT prior to 1992 was hepatic dysplasia in all with rising α-fetoprotein in 4. Post 1992 indications were suspected/high risk of HCC in five patients, proven HCC in 1 and failure to respond to Nitisinone in 1. In patients treated with Nitisinone who subsequently required OLT, treatment was started at a median age of 428 days compared to 52 days in those who have not required OLT (p=0.03). Survival following OLT was 4/6 (66.7%) pre- and 7/7 (100%) post-Nitisione. Early complications included acute rejection in 4, hepatic artery thrombosis in 1, biliary reconstruction in 1, redo portal vein anastomoses in 1, burst abdomen in 1 and primary non function in 1 patient. Late complications included chronic rejection in 3, hypertension in 3, post transplant lymphoproliferative disease in 2, de novo hepatitis in 2, pulmonary metastasis in 1 and renal failure in 1 patient. 3 patients required a second transplant. Mean calculated glomerular filtration rate decreased post OLT with no significant difference between the pre- and post-Nitisinone groups. Mean tubular reabsorption of phosphate remained within the normal range for both groups up to 5 years post OLT. With the non-responder to Nitisinone excluded, mean urinary protein:creatinine ratio normalised post OLT in the Nitisinone group and was significantly lower than the non-treated group in which it remained raised up to 5 years post OLT (p=0.0046). Quality of life following transplant is good with unrestricted diet in all.  Conclusion  OLT remains an effective treatment for TT1 but since the introduction of Nitisinone the need for OLT has been reduced and the likelihood of requiring OLT is minimised if Nitisinone therapy is instigated early. Although mean cGFR remains reduced post OLT, prior treatment with Nitisinone may improve tubular function as evidenced by normal protein:creatinine ratios.
AB1059 2D Histogram Ultrasound and 3D Ultrasound Correlation in Rheumatic Diseases
Authors: J.A. Mendonça, J.R. Provenza, V.R. Guissa, C.V. Vierhout, L.E. Pedri, P.G.U. Frazon, A.M. Lyrio, F. Peron Filho, R. Bonfiglioli, N.B. Schincariol
Journal: Annals of the Rheumatic Diseases (2015)

<h3>Background</h3> High frequency linear probe ultrasound (US), besides allowing a better resolution in the gray scale (GS), is able to characterize colored images standards through a RGB image histogram<sup>1</sup>. These images develop through the information of additive primary colors, such as Red (R), Green (G) and Blue (B). On the other hand, the 3D US<sup>2</sup> can better define the anatomical cuts, with more details, yielding more precise measures, together with the 2D US. These two methods are able to define echotextural damages, thus enhancing the detection of such changes in rheumatic diseases. <h3>Objectives</h3> To detect echotextural damages through 2D and 3D US correlating these area and volumes measures. <h3>Methods</h3> A total of 16 patients have been assessed (37.5% males and 62.5% females); 75% were affected by synovitis in their wrists, knees, metacarpophalangeal joint, distal interphalangeal joint, hips, ankle, metatarsophalangeal joint; 6.3% exhibited tenossynovitis, 6.3% tophus, 6.3% synovial cyst, 6.3% femoral-condyle cartilage, including 2 rheumatoid arthritis, 5 juvenile idiopathic arthritis, 4 gouts, 1 psoriatic arthritis, 2 osteoarthritis, 1 non-differentiated spondiloarthritis and 1 reactive arthropathy. A MyLab 50 -Esaote equipment was used with frequency transducer that ranged between 6.0 and 18.0 MHz for 2D, and with frequency that ranged from 10.0 to 12.0 MHz for 3D. A total of 630 images was obtained, 470 were in 2 D and 160 images in 3D. For each GS measurement there were 10 measures of 2D standard colored images as well as 3D correlation images of the original GS image, taken at different times in order not to remind prior measurements. <h3>Results</h3> Average age 39.93±25.98 years of age; average measurement of 2D image area: (GS) 159.23±174.98 cm<sup>2</sup>, (Indigo) 126.86±156.98 cm<sup>2</sup>, (RGB) 117.78±142.71 cm<sup>2</sup> and average measurement of 3D images 0.588±0.812 cm<sup>3</sup>. <i>Correlations 2D:</i> (GS) with (Indigo): <i>r</i>=0.948; <i>p</i>&lt;0. 001; (GS) with (RGB) <i>r</i>=0.979; <i>p</i>&lt;0. 001; (Indigo) with (RGB) <i>r</i>=0.987; <i>p</i>&lt;0.001. <i>Correlations 3D and 2D:</i> (GS) <i>r</i>=0.757; <i>p</i>=0.001; (Indigo) <i>r</i>=0.758; <i>p</i>=0.002 and (RGB) <i>r</i>=0.764; <i>p</i>=0.001. <h3>Conclusions</h3> The 2D colored image standards, together with 3D images, may improve the precision of the echotextural damage boundaries in rheumatologic diseases. <h3>References</h3> Marques Filho, O; Vieira Neto, H. Processamento Digital de Imagens, Rio de Janeiro: Brasport, 1999. ISBN8574520098. Mendonça JA. O US Tridimensional (3D US). Ultrassonografia em Reumatologia: Uma Extensão do Exame Físico. Rio de Janeiro: Revinter, 2014. p. 70-76. <h3>Disclosure of Interest</h3> None declared
Pulmonary complications of measles.
Authors: P Boroń, I Barszczewska, C Jezyna, R Kossakowski, K Lotocka
Journal: BMJ (1976)

Measles is a viral disease that we have chosen to talk about because of the progressively increasing number of cases, the respiratory complications that progress toward respiratory failure in children and because of the costs that could be lowerd if vaccinations were carried out according to the proposed Schedule by the Ministry of Health. Our research was conducted during 05.2016-31.12.2018 analyzed according to several criteria (age group, sex, personal history, complications, evolution, treatment, etc.). There are 3 particular clinical cases. From the pathophysiological point of view, we consider the focus on respiratory complications to be useful, most complications being alveolar pneumonia in children under 2 years of age, reaching to acute respiratory failure. Can we prevent this? Keywords: Measles, vaccination, respiratory complications, children
Molecular genetic approaches to the identification of genes involved in the development of nephropathy in insulin-dependent diabetes mellitus.
Authors: A S Krolewski, A Doria, J Magre, J H Warram, D Housman
Journal: Journal of the American Society of Nephrology (1992)

Genetic factors contribute significantly to the development of diabetic nephropathy in patients with insulin-dependent diabetes mellitus. This report discusses some models of diabetic nephropathy that incorporate genetic susceptibility and presents strategies for identifying the responsible genes. To identify variation at a locus, newly developed methods are discussed that employ denaturing gradient gel electrophoresis to study sequence differences in both polymerase chain reaction-amplified DNA fragments and genomic DNA. These techniques are illustrated with studies of the angiotensinogen gene and the insulin receptor gene. In preliminary data from a comparison between individuals with and without diabetic nephropathy, no DNA sequence difference in that part of the angiotensinogen gene that codes for angiotensin I was found. However, with a probe corresponding to exons 7 and 8 of the insulin receptor gene and denaturing gradient gel electrophoresis of Rsal digestions of genomic DNA, different distributions of a DNA polymorphism were found in patients with fast as compared with slowly progressing nephropathy. The interpretation of this finding and the need for further studies are discussed. In conclusion, the advent of methods of molecular genetics makes possible studies on genetic determinants of diabetic nephropathy. However, more clinical and epidemiologic data are needed to find out how many genes are involved and how they interact with exposure to diabetes. Foremost, DNA from families with two or more siblings with diabetic nephropathy must be collected to permit the necessary genetic studies.
Bovine Viruses and Bacteriophages
Authors: Thomas B. Friedman
Journal: JNCI Journal of the National Cancer Institute (1975)

Journal Article Bovine Viruses and Bacteriophages Get access Thomas B. Friedman Thomas B. Friedman Department of Biological Sciences Oakland University, Rochester, Michigan 48063 Search for other works by this author on: Oxford Academic PubMed Google Scholar JNCI: Journal of the National Cancer Institute, Volume 54, Issue 6, June 1975, Page 1495, https://doi.org/10.1093/jnci/54.6.1495 Published: 01 June 1975
Platelet-activating factor mediates angiotensin II-induced proteinuria in isolated perfused rat kidney.
Authors: N Perico, R Lapinski, K Konopka, S Aiello, M Noris, G Remuzzi
Journal: Journal of the American Society of Nephrology (1997)

Isolated kidney preparations (IPK) from male Sprague Dawley rats perfused at constant pressure were used to evaluate the effect of angiotensin II (AII) and platelet-activating factor (PAF) on renal function and urinary protein excretion. Compared with basal, intrarenal infusion of AII at 8 ng/min caused a progressive increase in protein excretion (11 +/- 6 versus 73 +/- 21 micrograms/min) in parallel with a decline in renal perfusate flow (RPF) (29 +/- 3 versus 18 +/- 3 ml/min). Addition to the perfusate of PAF at 50 nM final concentration also induced proteinuria (9 +/- 4 versus 55 +/- 14 micrograms/min) but did not change RPF (29 +/- 3 versus 30 +/- 3 ml/min). Preexposure of isolated kidneys to the PAF receptor antagonist WEB 2086 prevented the increase in urinary protein excretion induced by AII infusion (basal: 13 +/- 6; post-AII: 12 +/- 7 micrograms/min) but failed to prevent the vasoactive effect of AII (RPF, basal: 30 +/- 2; post-AII: 21 +/- 3 ml/min). In additional experiments, dexamethasone reduced the proteinuric effect of PAF remarkably. These results indicate that in isolated kidney preparation: (1) AII infusion induced proteinuria and decreased RPF; and (2) the effect of AII in enhancing urinary protein excretion was completely prevented by a specific PAF receptor antagonist, which, however, did not influence the AII-induced fall in RPF. It is suggested that PAF plays a major role in AII-induced changes in the permselective function of the glomerular capillary barrier.
PWE-351 Acromegaly and colonic polyps revisited: time to change the guidelines?: Abstract PWE-351 Table 1
Authors: D Corrigall, BA Ziolkowski, P Woodland, M Hannon, J ChinAleong, P Jenkins, P Fairclough, W Drake, S Preston
Journal: Gut (2015)

<h3>Introduction</h3> Previous studies, including an important study from our own unit published nearly 20 years ago, have highlighted an increased risk of adenomatous colonic polyps in patients with acromegaly.<sup>1</sup>Current clinical guidelines suggest intense colonoscopic surveillance from the age of 40 for these patients.<sup>2</sup> Advances in detection and improved treatment of acromegaly may have influenced the prevalence of colonic neoplasms in this group, and may necessitate revision of current surveillance guidelines. We <b>aimed</b>to re-evaluate polyp prevalence in a more recent cohort of patients with acromegaly undergoing colonoscopic surveillance at the Royal London Hospital. <h3>Method</h3> We interrogated our database to identify patients with acromegaly undergoing surveillance colonscopy between 2010 and 2015. In these patients we reported detection of premalignant and malignant lesions. <h3>Results</h3> We identified 89 patients (41 males, age range 36–82, median age 64) who had undergone colonoscopy. In this 2015 cohort we identified 16 patients (18%) with at least one adenomatous polyp. Of these polyps, 69% were tubular adenomas and 31% were tubulovillous adenomas. Polyps in only two patients displayed high-grade dysplasia, the remainder displayed low grade dysplasia. No cancers were found, compared to 6 (5%) of the 1996 cohort. <h3>Conclusion</h3> In comparison to the 1996 cohort we show a significantly (p = 0.04) lower detection rate of premalignant polyps in our patient group. In fact adenoma detection rates are similar to those seen in healthy colonoscopic screening populations. The reasons for this are likely to be multifactorial, but may in part be accounted for by advances in the diagnosis, management and monitoring of acromegaly (and thereby lower levels of GH and IGF-1). Consideration may be given to revisiting surveillance guidelines in these patients, resulting in fewer, perhaps more targeted procedures. <h3>Disclosure of interest</h3> None Declared. <h3>References</h3> Jenkins PJ <i>et al</i>. Clin Endocrinol. 1997 Jenkins PJ, Fariclough PD. <i>Gut</i>. 2002
Abrogation of the Hematological and Biological Activities of the Interleukin-3/Granulocyte-Macrophage Colony-Stimulating Factor Fusion Protein PIXY321 by Neutralizing Anti-PIXY321 Antibodies in Cancer Patients Receiving High-Dose Carboplatin
Authors: Langdon L. Miller, Edward L. Korn, Diane S. Stevens, John E. Janik, Barry L. Gause, William C. Kopp, Jon T. Holmlund, Brendan D. Curti, Mario Sznol, John W. Smith, Walter J. Urba, Sarah E. Donegan, Thelma M. Watson, Dan L. Longo
Journal: Blood (1999)
AB0829 Demographics of Patients with New-Onset Psoriatic Arthritis: Real Life Data from an Inception Cohort: Table 1.
Authors: U. Kalyoncu, B. Yılmazer, Ö. Bayındır, K. Aksu, E. Dalkılıç, M.F. Öksüz, E.F. Tarhan, M. Can, O. Küçükşahin, G. Kimyon, L. Akyol, A.M. Onat, B. Kısacık, A. Erden, A. Omma, E.D. Ersözlü Bozkırlı, C. Özişler, E. Gönüllü, S. Masatlıoğlu Pehlevan, D. Solmaz, M. Çınar, G. Yıldırım Çetin, A. Tufan, M. Aydın Tufan, L. Kılıç, S. Erten, T. Kaşifoğlu, M. Özgen, E. Kasapoğlu Günal, F. Arslan, S. Pay, Y. Kabasakal, S.Z. Aydın
Journal: Annals of the Rheumatic Diseases (2015)

<h3>Background</h3> Psoriatic arthritis is a complex disease with a wide range of manifestations. In this inception cohort of PsA patients we aimed to identify the initial manifestations as well as disease activity characteristics and compare with an unselected group of patients with longstanding disease. <h3>Methods</h3> PsART (Psoriatic Arthritis Registry of Turkey) is a prospective, multicentre study in Turkey on patients with PsA. Patients are consecutively recruited to this registry. Patients who have been newly diagnosed with PsA have been identified, and their characteristics were compared with longstanding disease. <h3>Results</h3> Within 746 patients recruited, 111 (14.9%) had a new diagnosis for PsA. Patients in the inception cohort were significantly younger than the rest of the cohort (p=0.03) (table). Females were 53.2% of the inception and 66.8% of the non-inception cohort (p=0.007). For types of joint patterns, 14.4% of the inception cohort had only axial involvement whereas this was seen in 8.2% for the other group (p=0.05). The other joint patterns were comparable among groups. Both groups had similar tender and swollen joint counts, BASDAI and BASFI. Patient (p=0.002) and physician global assessments (p&lt;0.001), fatigue (p=0.02), duration of morning stiffness (p=0.02) and CRP (p=0.05) were higher in the inception cohort. Female patients had higher patient global assessment and fatigue scores despite similar physician global assessment, swollen and tender joint counts, BASDAI, BASFI and CRP with males in non-inception cohort. For the inception cohort, there were no differences between the 2 genders. <h3>Conclusions</h3> More patients present with sole axial involvement at the beginning, which may explain the higher disease activity agreed by the patient and the physician despite similar tender and swollen joint counts. Females may have a different perception of the disease with worse patient global assessments and fatigue in longstanding disease. <h3>Disclosure of Interest</h3> None declared
Treatment of depression in general practice.
Authors: F C Clarke
Journal: BMJ (1973)

of dealing with the problem of anaesthetic contamination.This venting system has been in use for over six monthes in two operating theatres.Although the whole problem of operating theatre pollution seems to merit further in- vestigation, it is clear that until such time as the agent causing the possible deleterious effect on operating theatre personnel is identified all the anaesthetic gases and vapours should be vented from the operating room.-Iam, etc.
SP0001 Getting Around Congress, Newsletter, Resources and Important Facts for Attendees
Authors: K. Betteridge
Journal: Annals of the Rheumatic Diseases (2015)

The presentation will provide practical information about getting around the Congress Centre and extend a warm invitation to visit the health professional stand. The health professional newsletter and membership directory will be introduced. People will learn how they can contribute to future issues and help raise awareness of their work throughout the network. <h3>Disclosure of Interest</h3> None declared
末梢動脈疾患(PAD)における経皮的末梢インターベンション(PPI)に対する環大西洋間学会コンセンサスII(TASC-II)層別の長期成績
Authors: Araki Yoshihiro, Kumakura Hisao, Koizumi Akira, Kanai Hiroyoshi, Kasama Shu, Itoh Toshio, Sumino Hiroyuki, Iwasaki Toshiya, Takayama Yoshiaki, Itikawa Syuuiti, Kurabayashi Masahiko
Journal: Circulation (2008)
A New, Inexpensive Circling Device for Marking Specific Areas on Tissue Smears or Sections
Authors: P R DEL VECCHIO, E E SIEGLER, A W HILBERG
Journal: JNCI Journal of the National Cancer Institute (1956)

Journal Article A New, Inexpensive Circling Device for Marking Specific Areas on Tissue Smears or Sections Get access Pasco R. Del Vecchio, Pasco R. Del Vecchio Search for other works by this author on: Oxford Academic PubMed Google Scholar Edward E. Siegler, M.D., Edward E. Siegler, M.D. Search for other works by this author on: Oxford Academic PubMed Google Scholar Albert W. Hilberg, M.D. Albert W. Hilberg, M.D. Search for other works by this author on: Oxford Academic PubMed Google Scholar JNCI: Journal of the National Cancer Institute, Volume 16, Issue 4, February 1956, Pages 985–987, https://doi.org/10.1093/jnci/16.4.985 Published: 01 February 1956 Article history Received: 03 October 1955 Published: 01 February 1956
Water Content and Proton Spin Relaxation Time for Malignant and Nonmalignant Tissues From Mice and Humans
Authors: C F Hazlewood
Journal: JNCI Journal of the National Cancer Institute (1974)

Journal Article Water Content and Proton Spin Relaxation Time for Malignant and Nonmalignant Tissues From Mice and Humans Get access JNCI: Journal of the National Cancer Institute, Volume 52, Issue 2, February 1974, Page 625, https://doi.org/10.1093/jnci/52.2.625 Published: 01 February 1974
CBFβ/MYH11 Hypomethylation Signature In Patients With Acute Myeloid Leukaemia
Authors: Hana Hajkova, Markus H.-Y. Fritz, Jiri Schwarz, Jana Markova, Martin Vostry, Zdenek Krejcik, Cyril Salek, Ota Fuchs, Petr Cetkovsky, Jaroslav Cermak, Cedrik Haskovec, Vladimir Benes
Journal: Blood (2013)
Studies on Mammary Tumors in the O20 Amsterdam Strain of Mice
Authors: O. Mühlbock, Th. G. van Rijssel
Journal: JNCI Journal of the National Cancer Institute (1954)

Studies on Mammary Tumors in the O20 Amsterdam Strain of Mice Get access O. Mühlbock, O. Mühlbock Laboratory of the Antoni van Leeuwenhoekhuis, The Netherlands Cancer Institute, Amsterdam, Holland Search for other works by this author on: Oxford Academic PubMed Google Scholar Th. G. van Rijssel Th. G. van Rijssel Laboratory of the Antoni van Leeuwenhoekhuis, The Netherlands Cancer Institute, Amsterdam, Holland Search for other works by this author on: Oxford Academic PubMed Google Scholar JNCI: Journal of the National Cancer Institute, Volume 15, Issue 1, August 1954, Pages 73–97, https://doi.org/10.1093/jnci/15.1.73 Published: 01 August 1954 Article history Received: 25 March 1954 Published: 01 August 1954
PWE-211 Evaluation of clinical and radiological features that predict malignant transformation in small cystic lesions of the pancreas
Authors: MG Keane, HR Dadds, G El Sayed, T Luong, GK Fusai, BR Davidson, D Thorburn, SP Pereira
Journal: Gut (2015)

<h3>Introduction</h3> Cystic lesions of the pancreas (CLP) are being detected with increasing frequency. Current methods of stratifying risk of malignant transformation are imperfect. The aim of this study was therefore to determine the incidence of high-risk lesions and define clinical and radiological features that predict malignancy in a large cohort of patients with CLP managed by surgery or surveillance. <h3>Method</h3> A retrospective cohort study from January 2000 to December 2013 of patients over 18 years of age with a confirmed CLP evaluated in a tertiary referral hepatobiliary centre. <h3>Results</h3> Of the 1,090 patients diagnosed with a CLP during the study period, 768 patients were included in the study. A total of 141 patients were referred for immediate pancreatic resection, 570 entered surveillance while 57 had a malignant CLP which was unresectable at diagnosis (N=47) or were unfit for surgery (N=10). In those who underwent immediate resection, malignancy was present in 38% (54/141). During follow-up 2% (10/570) of those entering a surveillance programme underwent malignant transformation, although most instances were after discharge from active surveillance. Only two patients in the surveillance cohort underwent surgery, of which only one case was curative. Clinical and radiological features associated with a high-risk CLP included older age, associated symptoms, presence of a solid component or a dilated main pancreatic duct. Larger size did not correlate consistently with malignant transformation, particularly in IPMNs where the median size of benign lesions was larger than malignant IPMNs (30mm (range: 11–130) vs. 23mm (range: 15–56)). <h3>Conclusion</h3> The sensitivity of diagnostic tests leading to immediate surgery for high risk CLP (malignant or mucinous CLP) was 92%, with a specificity of just 5%. Surveillance of CLP without high-risk features was associated with a low incidence of cancer development. This study supports the stratification of CLP based on worrisome clinical and radiological features. <h3>Disclosure of interest</h3> None Declared.