tbuckley/cabot-search huggingface dataset:
A child with laryngo-onychocutaneous syndrome partially responsive to treatment with thalidomide
Authors: R.M. Strauss, J. Bäte, K.K. Nischal, T. Clayton, J. Gooi, J.C. Darling, J.A. Newton-Bishop
Journal: British Journal of Dermatology (2006)

Laryngo-onychocutaneous syndrome (LOCS) is a condition characterized by erosive or ulcerative skin lesions associated with excessive granulation tissue, at sites of trauma such as the digits, elbows and knees. Similar lesions can occur within the conjunctival mucosa, leading to corneal scarring and blindness. The main complications, however, occur in the respiratory tract, where a similar process of erosions and subsequent formation of granulation tissue causes airway obstruction which may lead to premature death. LOCS is now believed to be a nonblistering variant of junctional epidermolysis bullosa and to date there are no efficacious treatments available. We report a 16-year-old girl with LOCS who failed to respond to methylprednisolone and cyclophosphamide, but had a partial response to oral thalidomide with marked decrease in granulation tissue and tracheal secretions. Interruption of treatment resulted in prompt resurgence of the granulation tissue which was again controlled by reintroduction of thalidomide. We propose that in the absence of effective therapies for LOCS, a trial of thalidomide in these patients should be considered.
Physician Tolerance for Uncertainty
Authors: Richard M. Allman
Journal: JAMA (1985)

To gain insight into diagnostic test use, we interviewed 42 physicians who ordered 62 liver-spleen scans to detect possible metastases. Before receiving the actual scan results, physicians gave (1) pretest probability estimates for liver metastasis; (2) probability estimates and management plans given the most common scan results; and (3) probabilities above and below which they would stop the diagnostic workup (their decision thresholds). After learning the actual scan results, physicians gave their posttest probabilities and management plans. Thirteen physicians (31%) desired absolute certainty to rule in metastasis. Fifty percent planned further evaluations even when probability estimates of metastasis reached one of their probability targets for ending the workup. Greater attention to decision thresholds may improve physicians' understanding of their tolerance for diagnostic uncertainty and the ability of diagnostic tests to reduce it. (<i>JAMA</i>1985;254:246-248)
Continuous Subcutaneous Insulin Infusion and Postprandial Exercise in Tightly Controlled Type I (Insulin-Dependent) Diabetic Patients
Authors: Mariella Trovati, Quirico Carta, Franco Cavalot, Sara Vitali, Gabriella Passarino, Giuseppe Rocca, Giorgio Emanuelli, Gianfranco Lenti
Journal: Diabetes Care (1984)

The aim of this study was to investigate the influence of short-term submaximal postprandial exercise on plasma glucose concentrations in tightly controlled insulin-dependent diabetic patients treated by means of continuous subcutaneous insulin infusion (CSII). Two hours after breakfast, five diabetic patients and five healthy control subjects followed this protocol: 30 min of mild exercise, 30 min rest, 30 min of moderate exercise, 150 min rest. Serial determinations of plasma glucose, free insulin, and growth hormone (GH) were made. Similar control studies without exercise were also performed. In the diabetic patients, analysis of variance and covariance did not reveal any significant difference between the 2-h postbreakfast concentrations of plasma glucose and free insulin and postexercise values. A significant GH increase was observed after the exercise periods. Plasma glucose and insulin concentrations throughout the exercise study were not significantly different from the control study concentrations. Plasma free insulin concentrations of the diabetic patients were higher than the concentrations of healthy subjects. We conclude that CSII-treated, tightly controlled, insulin-dependent diabetic patients performing short-term mild and moderate exercises 2 and 3 h after breakfast do not have a high risk of hypoglycemia in spite of mild hyperinsulinemia.
734. Association of DNA Polymerase μ with Recombinant Adeno-Associated Viral Vectors
Authors: David C. Brooks, Fayaz R. Khazi, Katherine A. High
Journal: Molecular Therapy (2005)

Recombinant adeno associated viral (rAAV) vectors have been successfully used for efficient delivery and stable transgene expression in animal models and clinical trials. Transduced vector genomes primarily persist as extra-chromosomal concatamers, with a low frequency of integration into host chromosomes. Investigations into possible mechanisms involved in vector genome integration and analyses of integration junctions reveal actively transcribed regions of chromosomes to be the hotspots for vector integration via a proposed nonhomologous end joining (NHEJ) mechanism of DNA repair (Nakai et.al 2003). Although the primary sensors of DNA damage, Ku, ATM and DNA-PK have been implicated in rAAV integration, there is limited information about the involvement of proteins that are directly involved in NHEJ. A recently discovered mammalian DNA polymerase |[mu]| (Pol|[mu]|) has been shown to be an important component of the NHEJ ligation complex and to have a functional interaction with Ku-DNA complex. In this study, we investigated the effect of rAAV on induction of Pol|[mu]| expression and the possible interaction with vector genomes. HEK293 cells transduced with rAAV2 at an MOI of 20,000 were incubated at 37|[deg]|C for 0, 0.5, 1, 3, 6, 12 and 24 hours. Total proteins were harvested at each time point and the lysates were analyzed by Western blotting using anti-Pol|[mu]| antibody. The results showed a nine-fold increase in Pol|[mu]| expression in cells incubated for 12h post transduction. A three fold increase in expression was observed at the 3h time point. The 0.5 and 1hr treatments did not show a significant increase over basal levels. Expression levels dropped down to 2 fold at the 24h time point.
Osteogenic Sarcoma Associated With the Rothmund-Thomson Syndrome
Authors: KATARZYNA J. MACURA, GEORGE BURKE, VINCENT J.B. ROBINSON
Journal: Clinical Nuclear Medicine (1998)

The Rothmund-Thomson syndrome (RTS), also called poikiloderma congenitale, is a rare autosomal recessive disorder characterized by photosensitivity, poikiloderma of the face and extremities, juvenile cataracts, skeletal abnormalities, and a higher incidence of malignancy. Presented is the case of a 6-year-old boy with RTS who developed an osteogenic sarcoma of the tibia. A bone scan showed bilateral radius agenesis and a hot bone lesion in the proximal tibia metaphysis. An incisional bone biopsy led to a diagnosis of osteogenic sarcoma. There is an association between RTS and osteosarcoma, and there should be a high index of suspicion when patients with RTS develop bony pain.
714 COLLABORATIVE UK STUDY OF CHRONIC HEPATITIS B VIRUS INFECTION
Authors: L. Fries, A. Rodger, G. Dusheiko, S. Ijaz, R. Tedder, J. Banatvala, R. Williams, N. Naoumov, S. Puranik, S. Chokshi, T. Wong, W. Rosenberg, S. Moreea, A. Bathgate, M. Jacobs, P. Mills, D. Mutimer, M. Bassendine, S. Ryder, M. Thursz, A. Johnson
Journal: Journal of Hepatology (2010)
Tecemotide in unresectable stage III non-small-cell lung cancer in the phase III START study: updated overall survival and biomarker analyses
Authors: P. Mitchell, N. Thatcher, M.A. Socinski, E. Wasilewska-Tesluk, K. Horwood, A. Szczesna, C. Martín, Y. Ragulin, M. Zukin, C. Helwig, M. Falk, C. Butts, F.A. Shepherd
Journal: Annals of Oncology (2015)
Acquired immunobullous disease: a cutaneous manifestation of IgM macroglobulinaemia
Authors: S.J. WHITTAKER, B.S. BHOGAL, M.M. BLACK
Journal: British Journal of Dermatology (1996)

Waldenström's macroglobulinaemia has been associated with a variety of cutaneous manifestations. We report a patient with an acquired bullous disorder associated with skin fragility and IgM kappa paraprotein. Immunofluorescence studies demonstrated bright linear deposition of IgM along the basement membrane zone (BMZ), around dermal blood vessels and on arrector pili muscles. Weak keratinocyte cell surface staining with IgM was also noted. There was no deposition of complement or other immunoglobulins. Indirect immunofluorescent studies revealed a circulating IgM anti-BMZ antibody (titre 1/200), which bound to both the roof and the floor of a 1 mol/l NaCl split skin substrate. Immunoblotting analysis of the patient's serum, with both epidermal and dermal extracts, was negative. Treatment with a combination of prednisolone and azathioprine was successful. We suggest that this IgM kappa paraprotein has a low affinity for an undefined epitope within the lamina lucida, which has produced a subepidermal bullous disorder associated with skin fragility.
Methaqualone Abuse
Authors: Darryl S. Inaba
Journal: JAMA (1973)

The "Love Drug," "Heroin for Lovers,"<sup>1,2</sup>"The Dr. Jekyll and Mr. Hyde Drug,"<sup>3</sup>"Hottest Drug on the Streets,"<sup>2</sup>"bigger than marihuana—methaqualone sweeping west."<sup>4</sup>With such grandiose publicity within the past few months, methaqualone, "pharmacologically equivalent"<sup>4</sup>to the short-and intermediate-acting barbiturates, has entered the emerging arena of desired (and commonly available) drugs of abuse. First introduced to the American public by William H. Rorer, Inc, in 1965 under the trade name of "Quaalude," it has at this writing become the sixth most popular sedativehypnotic in the United States, and in 1972 "Quaalude" sales alone increased 360%.<sup>1,2,3</sup>Motivated by such a success story many other drug companies have leaped into competition for this prosperous market and methaqualone is now marketed under the various trade names of Sopor (Arnar-Stone), Parest (Parke-Davis), Optimil (Wallace), and Somnafac (Smith, Miller &amp; Patch). The major selling point of methaqualone is that
Perioperative heart-type fatty acid binding protein is associated with acute kidney injury after cardiac surgery
Authors: Jennifer A. Schaub, Amit X. Garg, Steven G. Coca, Jeffrey M. Testani, Michael G. Shlipak, John Eikelboom, Peter Kavsak, Eric McArthur, Colleen Shortt, Richard Whitlock, Chirag R. Parikh
Journal: Kidney International (2015)
The role of in vivo T-cell depletion on reduced-intensity conditioning allogeneic stem cell transplantation from HLA-identical siblings in patients with follicular lymphoma
Authors: J Delgado, C Canals, M Attal, K Thomson, A Campos, R Martino, T Littlewood, G Jackson, N Milpied, M Boogaerts, A Hunter, J J W M Janssen, S Montoto, A Sureda
Journal: Leukemia (2010)
The Effects of Mitral Regurgitation Alone Are Sufficient for Leaflet Remodeling
Authors: Elizabeth H. Stephens, Tom C. Nguyen, Akinobu Itoh, Neil B. Ingels, D. Craig Miller, K. Jane Grande-Allen
Journal: Circulation (2008)

Background—— Although chronic mitral regurgitation results in adverse left ventricular remodeling, its effect on the mitral valve leaflets per se is unknown. In a chronic ovine model, we tested whether isolated mitral regurgitation alone was sufficient to remodel the anterior mitral leaflet. Methods and Results— Twenty-nine sheep were randomized to either control (CTRL, n=11) or experimental (HOLE, n=18) groups. In HOLE, a 2.8- to 4.8-mm diameter hole was punched in the middle scallop of the posterior mitral leaflet to create “pure” mitral regurgitation. At 12 weeks, the anterior mitral leaflet was analyzed immunohistochemically to assess markers of collagen and elastin synthesis as well as matrix metalloproteinases and proteoglycans. A semiquantitative grading scale for characteristics such as intensity and delineation of stain between layers was used to quantify differences between HOLE and CTRL specimens across the heterogeneous leaflet structure. At 12 weeks, mitral regurgitation grade was greater in HOLE versus CTRL (3.0±0.8 versus 0.4±0.4, P &lt;0.001). In HOLE anterior mitral leaflet, saffron-staining collagen (Movat) decreased, consistent with an increase in matrix metalloproteases throughout the leaflet. Type III collagen expression was increased in the midleaflet and free edge and expression of prolyl-4-hydroxylase (indicating collagen synthesis) was increased in the spongiosa layer. The proteoglycan decorin, also involved in collagen fibrillogenesis, was increased compared with CTRL (all P ≤0.05). Conclusions— In HOLE anterior mitral leaflet, the increased expression of proteins related to collagen synthesis and matrix degradation suggests active matrix turnover. These are the first observations showing that regurgitation alone can stimulate mitral leaflet remodeling. Such leaflet remodeling needs to be considered in reparative surgical techniques.
A2.21 The role of HCK, FGR and LYN in<i>in vivo</i>inflammation in mice
Authors: M Kovács, T Németh, Z Jakus, C Sitaru, E Simon, K Futosi, B Botz, Z Helyes, Lowell CA, A Mócsai
Journal: Annals of the Rheumatic Diseases (2015)

<h3>Background and objectives</h3> Although Src family kinases participate in leukocyte function <i>in vitro</i>, such as integrin signal transduction, their role in inflammation <i>in vivo</i> is poorly understood. The role of Src family kinases in b2 integrin signalling and the requirement for b2 integrins during autoantibody-induced <i>in vivo</i> inflammation prompted us to test the role of Src family kinases in autoantibody-induced inflammatory disease models. <h3>Materials and methods</h3> To determine the role of Src family kinases in autoantibody-induced autoimmune diseases Hck-/-Fgr-/-Lyn-/- triple knockout and wild type mice were tested in autoantibody-induced K/B×N serum transfer arthritis, autoantibody-induced skin blistering disease and reverse passive Arthus reaction. Autoimmune disease development was carefully monitored, and inflammatory cytokine and chemokine production was determined <i>in vivo</i> and <i>in vitro</i>. Furthermore, <i>in vivo</i> migratory capacity of leukocytes was monitored in mixed radiation chimaeras. <h3>Results</h3> We found that Src family kinases play a critical role in myeloid cell–mediated <i>in vivo</i> inflammatory reactions. Mice lacking the Src family kinases Hck, Fgr, and Lyn in the hematopoietic compartment were completely protected from autoantibody-induced arthritis and skin blistering disease, as well as from the reverse passive Arthus reaction, with functional overlap between the three kinases. Though the overall phenotype resembled the leukocyte recruitment defect observed in b2 integrin–deficient (CD18-/-) mice, Hck-/-Fgr-/-Lyn-/- neutrophils and monocytes/macrophages had no cell autonomous <i>in vivo</i> or <i>in vitro</i> migration defect. Instead, Src family kinases were required for the generation of the inflammatory environment <i>in vivo</i> and for the release of proinflammatory mediators from neutrophils and macrophages <i>in vitro</i>, likely due to their role in Fcg receptor signal transduction. <h3>Conclusions</h3> Our results suggest that infiltrating myeloid cells release proinflammatory chemokine, cytokine, and lipid mediators that attract further neutrophils and monocytes from the circulation in a CD18-dependent manner. Src family kinases are required for the generation of the inflammatory environment but not for the intrinsic migratory ability of myeloid cells.
A CLINICAL STUDY OF THE SABULA FOOD POISONING EPIDEMIC.
Authors: A. CRAWFORD
Journal: JAMA (1897)

The most extensive and serious outbreak of food poisoning ever recorded in the history of Iowa, occurred in the eastern part of Jackson County, in the fall of 1895. On September 11, at a farmhouse three miles from Sabula, there assembled about three hundred guests to witness a marriage ceremony and partake of the good things provided for the occasion. Within a few weeks thereafter, over one-third of the entire company were sick and seven deaths had occurred. That the disease had its origin at the wedding, could not be doubted, as there was absolutely no serious sickness among the scores of families who were not present on that occasion, nor had the health of the community been better for years, than during the month preceding the epidemic. The house at which the wedding occurred is well located, and there is no history of disease having existed there for many
A Multimodal Analysis of Antipsychotic Effects on Brain Structure and Function in First-Episode Schizophrenia
Authors: Tyler A. Lesh, Costin Tanase, Benjamin R. Geib, Tara A. Niendam, Jong H. Yoon, Michael J. Minzenberg, J. Daniel Ragland, Marjorie Solomon, Cameron S. Carter
Journal: JAMA Psychiatry (2015)

IMPORTANCE-Recent data suggest that treatment with antipsychotics is associated with reductions in cortical gray matter in patients with schizophrenia.These findings have led to concerns about the effect of antipsychotic treatment on brain structure and function; however, no studies to date have measured cortical function directly in individuals with schizophrenia and shown antipsychotic-related reductions of gray matter.OBJECTIVE-To examine the effects of antipsychotics on brain structure and function in patients with first-episode schizophrenia, using cortical thickness measurements and administration of the AX version of the Continuous Performance Task (AX-CPT) during eventrelated functional magnetic resonance imaging.
Cusum plotting of temperature charts for assessing antimicrobial treatment in neutropenic patients.
Authors: S. E. Kinsey, F. J. Giles, J. Holton
Journal: BMJ (1989)

2 methodes de «somme cumulative» sont appliquees a l'analyse statistique des courbes de temperature chez 25 malades atteints de neutropenie et de fievre. Grande utilite pour controler les effets des traitements par antibiotiques et eventuellement pour les modifier
HISTORY TAKING IN BRONCHIAL ASTHMA
Authors: GRAFTON TYLER BROWN
Journal: JAMA (1927)

Bronchial asthma may be due to many different causes and an exhaustive history is of fundamental importance in the specific diagnosis of this disease. I will endeavor to outline the method of taking such a history, indicating, as I go along, the significance of the various factors. <h3>AGE</h3> The first point to be considered is that of age, although the actual age of the patient is nothing like as important as the age of onset of the asthma. Naturally, the prognosis is not as good in an asthmatic patient past middle life as it is in a younger one, as in the older patient there is much more likelihood of organic changes in the lungs and heart, such as emphysema, bronchiectasis and myocarditis. The age of onset, on the other hand, is of importance in that the earlier in life the asthma starts, the more likely it is to be
Coronary artery passivation: a mechanism for the prevention of recurrent ischemia after thrombolysis
Authors: Mark H. Rubenstein, Harry D. Garabedian, Luis Guerrero, Suzanne M. Sullivan, Thomas Aretz, Renu Virmani, Stanley Hollenbach, Robert C. Leinbach, Herman K. Gold
Journal: Journal of the American College of Cardiology (2002)
Field Trial of a Contraceptive Foam in Puerto Rico
Authors: Manuel E. Paniagua
Journal: JAMA (1961)

Free supplies of Emko Foam were distributed to 142 Puerto Rican couples over a 27-month period, comprising 143 couple-years of experience. The people were primarily low-income individuals who had not voluntarily sought contraception. Thus, they probably had varying degrees of motivation and the results are probably lower than would be expected if the contraceptive were used correctly at each sexual intercourse. Prior to use of the foam, the pregnancy rate for the couples was 80 per 100 couples per year of exposure. When all months of the study are included, the pregnancy rate is 29 per 100 couples per year of exposure, a drop of 64%. The rate rises in the second 6 months, when postpartum amenorrhea is a minor factor, and falls to 17 after 12 months, when some of the less regular users have become pregnant. When only the menstrual months are considered, the rate decreases from 45 in the first 6 months to 17 after 12 months. The commonest reason for discontinuing the foam was unplanned pregnancy. 12 couples separated, 5 stopped for planned pregnancy, 5 for religious reasons, 3 because of husband's objections, 1 because of urethral irritation, and 1 because of lost interest. The fact that 69% of the fertile couples who were approached to try out the foam accepted indicates a latent desire for contraception. A public health program directed toward general distribution of contraceptives would appear to be acceptable to low income groups in Puerto Rico.
Management of women referred to early pregnancy assessment unit: care and cost effectiveness.
Authors: M A Bigrigg, M D Read
Journal: BMJ (1991)

To assess the efficiency of an early pregnancy assessment unit in the care of women with bleeding or pain in early pregnancy.Analysis of women attending in the first year of the unit's operation and in the six months immediately before its introduction.Early pregnancy assessment unit in a district general hospital serving a population of 310,000.1141 women referred with bleeding or pain in early pregnancy.Length of stay in hospital required for diagnosis and treatment.Before the unit was established the mean admission time was one and a half (range half to three) days for women who required no treatment and three (one and a half to five) days in women requiring evacuation of uterus. These times were reduced to two hours as an outpatient and one day respectively for most women after the unit was established. Between 318 and 505 women were estimated to have been saved from unnecessary admission, and 233 had their stay reduced; the associated saving was between pounds 95,000 and pounds 120,000 in one year.The early pregnancy assessment unit improved the quality of care and also produced considerable savings in financial and staff resources.
Pulmonary complications after oesophagogastroscopy using diazepam.
Authors: P A Taylor, P B Cotton, R M Towey, A E Gent
Journal: BMJ (1972)

infarct, and it resolved completely after aspiration and corticosteroid drugs with a fall to normal of the E.S.R.A hypersensitivity reaction to autologous heart tissue, possibly altered antigenically by ischaemia, has been postu- lated as the cause of the postmyocardial infarction syndrome.This case was remarkable because of the severity of the pericardial reaction observed, which we think may have been related to the patient's strong personal history of allergy and her ability to develop auto-antibodies, but immunological studies failed to elucidate the pathogenesis.We wish to thank Mrs.