tbuckley/cabot-search huggingface dataset:
Clinical Features of Childhood Primary Ciliary Dyskinesia by Genotype and Ultrastructural Phenotype
Authors: Stephanie D. Davis, Thomas W. Ferkol, Margaret Rosenfeld, Hye-Seung Lee, Sharon D. Dell, Scott D. Sagel, Carlos Milla, Maimoona A. Zariwala, Jessica E. Pittman, Adam J. Shapiro, Johnny L. Carson, Jeffrey P. Krischer, Milan J. Hazucha, Matthew L. Cooper, Michael R. Knowles, Margaret W. Leigh
Journal: American Journal of Respiratory and Critical Care Medicine (2014)

Rationale: The relationship between clinical phenotype of childhood primary ciliary dyskinesia (PCD) and ultrastructural defects and genotype is poorly defined.Objectives: To delineate clinical features of childhood PCD and their associations with ultrastructural defects and genotype.Methods: A total of 118 participants younger than 19 years old with PCD were evaluated prospectively at six centers in North America using standardized procedures for diagnostic testing, spirometry, chest computed tomography, respiratory cultures, and clinical phenotyping.Measurements and Main Results: Clinical features included neonatal respiratory distress (82%), chronic cough (99%), and chronic nasal congestion (97%). There were no differences in clinical features or respiratory pathogens in subjects with outer dynein arm (ODA) defects (ODA alone; n = 54) and ODA plus inner dynein arm (IDA) defects (ODA + IDA; n = 18) versus subjects with IDA and central apparatus defects with microtubular disorganization (IDA/CA/MTD; n = 40). Median FEV1 was worse in the IDA/CA/MTD group (72% predicted) versus the combined ODA groups (92% predicted; P = 0.003). Median body mass index was lower in the IDA/CA/MTD group (46th percentile) versus the ODA groups (70th percentile; P = 0.003). For all 118 subjects, median number of lobes with bronchiectasis was three and alveolar consolidation was two. However, the 5- to 11-year-old IDA/CA/MTD group had more lobes of bronchiectasis (median, 5; P = 0.0008) and consolidation (median, 3; P = 0.0001) compared with the ODA groups (median, 3 and 2, respectively). Similar findings were observed when limited to participants with biallelic mutations.Conclusions: Lung disease was heterogeneous across all ultrastructural and genotype groups, but worse in those with IDA/CA/MTD ultrastructural defects, most of whom had biallelic mutations in CCDC39 or CCDC40.
Atypical varicella-zoster virus infection in an immunocompromised patient: Result of a virus-induced vasculitis
Authors: Helmut Erhard, Thomas M Rünger, Matthias Kreienkamp, Justus Müller, Hans-Konrad Müller-Hermelink, Eva-Bettina Bröcker
Journal: Journal of the American Academy of Dermatology (1995)
Filaggrin-dependent secretion of sphingomyelinase protects against staphylococcal α-toxin–induced keratinocyte death
Authors: Anne M. Brauweiler, Lianghua Bin, Byung Eui Kim, Michiko K. Oyoshi, Raif S. Geha, Elena Goleva, Donald Y.M. Leung
Journal: Journal of Allergy and Clinical Immunology (2012)
COMPARISON OF OPTIMAL PHARMACOLOGICAL TREATMENT ALONE AND COMBINED WITH CARDIAC RESYNCHRONIZATION THERAPY IN PATIENTS OVER 75 YEARS
Authors: Marcelino Cortes, Elena de la Cruz, Julia Anna Palfy, Angelica Maria Romero Diaz, Paloma Avila Barahona, Ignacio Hernandez, Juan Antonio Franco Pelaez, Juan Benezet-Mazuecos, Jose Rubio Campal, Jeronimo Farre
Journal: Journal of the American College of Cardiology (2014)
IgM antibody to hepatitis C virus in acute and chronic hepatitis C
Authors: Juan Antonio Quiroga, María Luz Campillo, Inmaculada Catillo, Javier Bartolomé, Juan Carlos Porres, Vicente Carreño
Journal: Hepatology (1991)

To assess possible role of testing for IgM-specific antibody in the diagnosis and monitoring of patients with hepatitis C, we tested sera from 14 patients with acute and 97 patients with chronic non-A, non-B hepatitis for IgG and IgM antibody to hepatitis C virus. IgG antibody to hepatitis C virus was detected in 93% of acute cases and 91% of chronic cases. Of the 101 patients with IgG antibody to hepatitis C virus, 57% had IgM antibody to hepatitis C virus. None of the 20 healthy subjects or 40 patients with acute or chronic hepatitis A or hepatitis B had IgM antibody to hepatitis C virus. At the onset of clinical symptoms in acute hepatitis C, IgG antibody to hepatitis C virus was detected in 8 (57%) and IgM antibody to hepatitis C virus in 9 of 14 patients (64%). Eventually, both IgG and IgM antibody to hepatitis C virus became detectable in 13 of 14 patients with acute hepatitis C. Seven patients with antibody to hepatitis C virus resolved the acute infection within 6 mo and all seven cleared IgM antibody to hepatitis C virus, whereas two cleared IgG antibody to hepatitis C virus. Six patients had a chronic outcome of the acute infection and IgM antibody to hepatitis C virus persisted in detectable amounts for more than 6 mo in all (mean = 15.5 mo). Among 88 patients with chronic non-A, non-B hepatitis with IgG antibody to hepatitis C virus, IgM antibody to hepatitis C virus was detected in 45(51%). Twenty-four chronic cases were followed for at least 1 yr: IgM antibody to hepatitis C virus was detected in 13 (54%) at baseline and 8 of these remained positive for at least 1 yr. Patients who lost IgM antibody to hepatitis C virus did not have remission of disease activity. In summary, IgM antibody to hepatitis C virus persists after acute infection in patients who contract chronic hepatitis C; therefore, testing for this antibody may be useful in early identification of patients for antiviral therapy. (HEPATOLOGY 1991;14:38-43.)
Refined cytogenetic-risk categorization for overall and leukemia-free survival in primary myelofibrosis: a single center study of 433 patients
Authors: D Caramazza, K H Begna, N Gangat, R Vaidya, S Siragusa, D L Van Dyke, C Hanson, A Pardanani, A Tefferi
Journal: Leukemia (2010)

We have previously identified sole +9, 13q- or 20q-, as 'favorable' and sole +8 or complex karyotype as 'unfavorable' cytogenetic abnormalities in primary myelofibrosis (PMF). In this study of 433 PMF patients, we describe additional sole abnormalities with favorable (chromosome 1 translocations/duplications) or unfavorable (-7/7q-) prognosis and also show that other sole or two abnormalities that do not include i(17q), -5/5q-, 12p-, inv(3) or 11q23 rearrangement are prognostically aligned with normal karyotype, which is prognostically favorable. These findings were incorporated into a refined two-tired cytogenetic-risk stratification: unfavorable and favorable karyotype. The respective 5-year survival rates were 8 and 51% (hazard ratio (HR): 3.1, 95% confidence interval (CI): 2.2-4.3; P<0.0001). Multivariable analysis confirmed the International Prognostic Scoring System (IPSS)-independent prognostic value of cytogenetic-risk categorization and also identified thrombocytopenia (platelets <100 × 10(9)/l) as another independent predictor of inferior survival (P<0.0001). A similar multivariable analysis showed that karyotype (P=0.001) and platelet count (P=0.04), but not IPSS (P=0.27), predicted leukemia-free survival; the 5-year leukemic transformation rates for unfavorable versus favorable karyotype were 46 and 7% (HR: 5.5, 95% CI: 2.5-12.0; P<0.0001). This study provides the rationale and necessary details for incorporating cytogenetic findings and platelet count in future prognostic models for PMF.
Genetic control of T cell receptor BJ gene expression in peripheral lymphocytes of normal and rheumatoid arthritis monozygotic twins.
Authors: T Nanki, H Kohsaka, N Mizushima, W E Ollier, D A Carson, N Miyasaka
Journal: Journal of Clinical Investigation (1996)

The amino acids encoded at the junctions of T cell receptor (TCR) V and J genes directly interact with MHC bound peptides. However, the regulation of the human TCRBJ gene repertoire has been difficult to analyze, because of the potentially complex number of BJ gene rearrangements. To overcome this problem, we developed a PCR-ELISA method to study BJ gene expression, and compared peripheral T lymphocytes from 12 pairs of monozygotic twins, including 6 rheumatoid arthritis (RA) discordant pairs, and 5 normals. Analyses of the TCRBV5, 13 and 17 gene families, which have been reported to be increased in RA patients, showed: (a) the three TCRBV transcripts have common features of BJ gene usage; (b) TCR transcripts from each TCRBV family display a distinctive BJ gene profile, which is displayed better by CD4+ than CD8+ lymphocytes; (c) the BJ gene repertoires of monozygotic twins are more similar than those of unrelated individuals; and (d) the inflammation of RA does not induce specific changes in the genetically determined pattern of BJ expression. These results indicate that the frequency of expression particular TCRBV-TCRBJ recombinants in human lymphocytes is controlled genetically, and is maintained despite the presence of a chronic inflammatory disease.
Hypercalcemia and Carcinoma of the Esophagus
Authors: JAIME BENREY
Journal: Annals of Internal Medicine (1974)

Letters1 March 1974Hypercalcemia and Carcinoma of the EsophagusJAIME BENREY, M.D., DAVID Y. GRAHAM, M.D., RAJ K. GOYAL, M.D.JAIME BENREY, M.D.Search for more papers by this author, DAVID Y. GRAHAM, M.D.Search for more papers by this author, RAJ K. GOYAL, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-80-3-415 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptTo the editor: Squamous cell carcinoma of the esophagus accounts for approximately 4% of all cancer deaths in the United States. Most of these patients die within a year of diagnosis in the "terminal" phase of the illness, with stupor, inanition, and dehydration. These symptoms are usually believed to arise from debility and malnutrition associated with carcinoma. On occasion, they can be secondary to a reversible superimposed metabolic complication, such as hypercalcemia. The association of hypercalcemia with squamous cell carcinoma of the esophagus has been reported infrequently. We report here a retrospective study to emphasize that hypercalcemia may be more...References1. MYERS W: Hypercalcemia in neoplastic disease. Arch Surg 80:308-318, 1969 CrossrefGoogle Scholar2. SHERWOODO'RIORDANAURBACK LJG: Production of parathyroid hormone by non-parathyroid tumors. J Clin Endocrinol Metab 27:140-146, 1967 CrossrefMedlineGoogle Scholar3. SNEDECORBAKER PH: Pseudohyperparathyroidism due to malignant tumors. Cancer 17:1492-1496, 1964 CrossrefMedlineGoogle Scholar4. MUGGIAHEINEMANN FH: Hypercalcemia associated with neoplastic disease. Ann Intern Med 73:281-290, 1970 LinkGoogle Scholar5. POWELLSINGERMURRAY DFT: N Engl J Med 289:176-181, 1973 CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Baylor College of Medicine Ben Taub General Hospital Houston, Texas 77025 PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited ByHypercalcemia in patients with esophageal carcinoma. The pathophysiologic role of parathyroid hormone-related proteinHypercalcemia related to the poor prognosis of patients with squamous cell carcinoma of the esophagusHypercalc�mie bei malignen ErkrankungenSystemic Manifestations of Gut MalignancyEctopic hyperparathyroidism (pseudohyperparathyroidism) in esophageal malignancy 1 March 1974Volume 80, Issue 3Page: 415-416KeywordsEsophagusHypercalcemiaMalnutritionRetrospective studiesSquamous cell carcinoma Issue Published: 1 March 1974 PDF DownloadLoading ...
Tissue Blood Flow to the Heart
Authors: Lloyd D. MacLean, Paul H. Hedenstrom, Ralph R. Rayner
Journal: Circulation Research (1962)

Regional tissue blood flow to the heart was measured in the dog using deuterium oxide as a tracer substance. The method assumes that the rate of uptake of the tracer by a tissue is limited by the blood flow through the tissue and that the exchange of the tracer between blood and the extravascular space in the organ is instantaneous by comparison. This assumption has had Previous confirmation. Six groups of animals were studied and the results revealed: (1) a uniform distribution of blood flow per unit of weight of tissue between the right ventricle and various parts of the left ventricle; (2) a decrease in tissue blood flow to (0.1 ml./Gm./min., or less), ap proximately 7 per cent of normal in the area supplied by the left anterior descending coronary artery after acute occlusion of this vessel; (3) a failure of two operative procedures designed to increase intercoronary anastomoses to influence this greatly de creased flow, when these procedures were performed six weeks to six months prior to testing; (4) an increase in tissue blood flow to an ischemie area of myocardium to 50 per cent of normal four to six months after coronary occlusion; (5) a failure of olerative procedures to increase significantly the blood flow to the ischemic area over that which developed naturally, indicating no additional collateral vessel development due to the operations. The results of this investigation were discussed in relation to experiments which deal with myocardial function and vulner ability to ventricular fibrillation after coronary occlusion.
Cirrhotic Ascites
Authors: VlTO K. Rocco, Athol J. Ware
Journal: Annals of Internal Medicine (1986)

Cirrhotic ascites occurs via both overflow and underfill mechanisms. Intrahepatic hypertension activates a hepatic baroreceptor reflex that enhances renal sodium absorption; plasma volume is expanded. As cirrhosis progresses, the hepatoportal Starling forces become sufficiently disturbed to sequester this "overflow" in the peritoneal cavity, which results in ascites formation. "Underfill" of the vascular system occurs and eventually dominates the clinical picture. Finally, intrahepatic hypertension also activates the renin-angiotensin system, which causes renal vasoconstriction; the increase in renal prostaglandin synthesis maintains renal blood flow. Although cirrhotic ascites is traditionally classified as a transudate, the serum-ascites albumin gradient may be a better indicator of ascites secondary to portal hypertension than other causes. General management of patients with cirrhotic ascites includes severe restriction of dietary sodium intake and bed rest; diuretics are added if spontaneous diuresis does not occur after 3 to 4 days.
Superoxide production by Crohn's disease neutrophils.
Authors: F T Curran, R N Allan, M R Keighley
Journal: Gut (1991)

<h3>Aim</h3> To identify the underlying genetic defect in Egyptian patients with macular corneal dystrophy (MCD). <h3>Methods</h3> A clinical and molecular genetic study was performed on 11 patients from six families with MCD. Clinical diagnosis was confirmed by slit-lamp biomicroscopy and histopathological examination of corneal buttons following keratoplasty. The coding region of the carbohydrate sulfotransferase (<i>CHST6</i>) gene was amplified by polymerase chain reaction (PCR) in all affected subjects. This was followed by direct sequencing and restriction digest analyses. Enzyme-linked immunosorbent assay of antigenic keratan sulfate (KS) in patients9 serum was also performed. <h3>Results</h3> Six homozygous mutations, of which three are novel, were identified within the coding region of <i>CHST6</i> in six unrelated MCD families. The barely detectable level of antigenic KS in the serum of the affected individuals indicated that they all have MCD type I, including the subtype IA. <h3>Conclusions</h3> This is the first report of a molecular genetic analysis of MCD in the Egyptian population. These data indicate the extensive allelic heterogeneity within <i>CHST6</i> and further support its essential role in maintaining corneal transparency.
Breastfeeding and Childhood Obesity
Authors: Marit L. Bovbjerg, Carolina Amador, Adrienne E. Uphoff
Journal: JAMA Pediatrics (2013)

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA Pediatrics HomeNew OnlineCurrent IssueFor Authors Podcast Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA Pediatrics journal
324 HEV SERO-VIROLOGICAL PREVALENCE IN HIV-INFECTED PATIENTS IN FRANCE: A SOUTH–NORTH GEOGRAPHIC COMPARISON
Authors: C. Renou, A. Lafeuillade, J.-F. Cadranel, A. Pariente, N. Pavio, T. Allegre, C. Wartelle, J. Gaillat, F. Heluwaert, C. Poggi, G. Pénaranda, P. Dumouchel, F. Cordier, E. Nicand
Journal: Journal of Hepatology (2010)
Pulmonary administration of a doxorubicin-conjugated dendrimer enhances drug exposure to lung metastases and improves cancer therapy
Authors: Lisa M Kaminskas, Victoria M McLeod, Gemma M Ryan, Brian D Kelly, John M Haynes, Mark Williamson, Neeranat Thienthong, David J Owen, Christopher J H Porter
Journal: Journal of Controlled Release (2014)
Aprt/Opn double knockout mice: Osteopontin is a modifier of kidney stone disease severity
Authors: Hilary J. Vernon, Christine Osborne, Eleni G. Tzortzaki, Min Yang, Jianmen Chen, Susan R. Rittling, David T. Denhardt, Steven Buyske, Sharon B. Bledsoe, Andrew P. Evan, Lynette Fairbanks, H. Anne Simmonds, J.A.Y.A. Tischfield, Amrik Sahota
Journal: Kidney International (2005)
Creative use of existing clinical and health outcomes data to assess NHS performance in England: Part 2—more challenging aspects of monitoring
Authors: Azim Lakhani, James Coles, Daniel Eayres, Craig Spence, Colin Sanderson
Journal: BMJ (2005)

2][3][4] However, this will require new data collection that could take several years.In the meantime, creative and informed use of existing data, with clear admission of the known shortcomings, may give some indication of how outcomes are changing. 5The main challenges are to measure health validly and to judge how much any improvements are due to NHS interventions.In this, the second of our two articles, we explore some of the technical issues involved and make practical illustrative suggestions about how best to use existing data regarding mental health care, potentially avoidable deaths, and forecasting coronary heart disease outcomes. 6Many other quality indicators could be produced along similar lines.
Colonoscopy, tumors, and inflammatory bowel disease
Authors: J. East
Journal: Endoscopy (2009)

This single-center study from the USA examined the ability of narrow band imaging (NBI), a novel “blue light” advanced endoscopic imaging technique, to reduce adenoma miss rates and thereby increase detection. The study was performed in a back-to-back fashion using wide angle (170°) colonoscopes. With the advent of national bowel cancer screening programs and competing technologies such as computed tomography (CT) colonography, the issue of colonoscopic miss rates has been recently highlighted. This has led to a focus on colonoscopic quality, as assessed by adenoma detection rates [2]. This study showed that NBI did not reduce the miss rate. The overall miss rate was 12.3 % (95 % confidence interval [CI] 8.7 – 16.8) for adenomas of all sizes; one 10-mm tubular adenoma was missed but no carcinomas were missed. Nor did NBI significantly increase adenoma detection, with 50 % of patients having at least one adenoma detected with NBI versus 44 % with white light.
Chronic endothelin receptor blockade prevents renal vasoconstriction and sodium retention in rats with chronic heart failure
Authors: S Ding
Journal: Cardiovascular Research (2002)

Objective: Importance of endothelin in mediating the chronic renal alterations of chronic heart failure was studied in rats chronically treated with bosentan after myocardial infarction. Methods: Rats were subjected to coronary artery ligation and were treated for 8 weeks with placebo or bosentan, a dual ETA and ETB receptor antagonist, (∼100 mg/kg/day) as food admix. Sham-operated rats served as normal controls. Cardiac and renal functions were measured at the end of 8-week treatment. Results: Bosentan significantly reduced the elevated left ventricular end-diastolic pressure (from 26.6±3.3 to 11.4±2.2 mmHg, P<0.001) and the increased heart-to-body-weight ratio seen in untreated rats with myocardial infarction. Bosentan prevented the marked increase in renal vascular resistance (bosentan, 7.7±0.6; untreated, 15.6±2.5 mmHg/ml/min; P<0.001). This led to a significant increase in renal plasma flow resulting in a decrease in filtration fraction. Bosentan furthermore increased urinary sodium excretion. Conclusions: Prolonged ET receptor blockade in rats with myocardial infarction has chronic renal vasodilatory effect and improves renal sodium excretory function. Thus, dual ET antagonists such as bosentan might be useful in the treatment of the progressive renal failure associated with human chronic heart failure.
Diet and Prevention of Dental Disese
Authors: H. Sinclair
Journal: BMJ (1965)

Treatment of Localized Myxoedema SIR,-We were surprised that your leading article (13 November, p. 1 138) did not mention the possible role of the long-acting thyroid stimulator (L.A.T.S.) in the pathogenesis of pretibial myxoedema.This thyroid stimulating agent, a 7S gamma-globulin,' present in
TESTING FOR OCCULT BLOOD IN FAECES: A STUDY OF ALLEGED FALLACIES
Authors: A. G. Ogilvie
Journal: BMJ (1927)

THE great clinical value of a reliable test for oceLlt blood in the faeces is generally recognized, but there is divergence of opinion as to what constitutes a reliable result.A number of fallacies' have been alleged to occur, the chief among them being due to diet, and untilcomparatively recently it was generally accepted that the tests were valueless unless performed under special conditions.The great majority of British observers still hold this
Gender and survival: a population-based study of 201,114 men and women following a first acute myocardial infarction
Authors: Kate MacIntyre, Simon Stewart, Simon Capewell, James W.T Chalmers, Jill P Pell, James Boyd, Alan Finlayson, Adam Redpath, Harper Gilmour, John J.V McMurray
Journal: Journal of the American College of Cardiology (2001)
Isolation of Measles Virus from Urine
Authors: Ion Gresser, Samuel L. Katz
Journal: New England Journal of Medicine (1960)

THE etiologic agent has reportedly been recovered from the urine of patients with a number of different viral infections (rabies,1 lymphocytic choriomeningitis,2 Russian spring-summer encephalitis,3 Newcastle disease,4 mumps,5 cytomegalic-inclusion disease6 and infections due to Coxsackie virus7 , 8 and adenovirus9). To our knowledge, however, viruria has not been demonstrated in measles. Isolation of measles virus from the urine in several typical cases of this disease is described in this preliminary communication.MethodsSpecimens of urine ranging from 2 to 250 ml. in volume were obtained from children with measles. An attempt was made to process these specimens as rapidly as possible . . .