tbuckley/cabot-search huggingface dataset:
Response to Letter Regarding Article, “Statin Use and Adrenal Aldosterone Production in Hypertensive and Diabetic Subjects”
Authors: Rene Baudrand, Luminita Pojoga, Anand Vaidya, Amanda Garza, Paul Vöhringer, Xavier Jeunemaitre, Paul Hopkins, Tham Yao, Jonathan Williams, Gail Adler, Gordon Williams
Journal: Circulation (2016)
The invested partner in sexual disorders: a profile
Authors: L R Derogatis, J K Meyer
Journal: American Journal of Psychiatry (1979)

To determine the relevant psychosexual characteristics of invested partners of patients with sexual disorders, the authors administered the Derogatis Sexual Functioning Inventory (DSFI) to 51 invested partners and 200 normal volunteers. Female invested partners (N = 18) participated in a significantly more constricted range of specific sexual activities and revealed significantly lower sexual drive than the normal women. Tbey also had significantly lower scores on the femininity component of the gender role definition subtest. Male invested partners (N = 33) also showed significantly lower femininity scores and had somewhat lower scores on endorsement of specific sexual experiences. Degree of sexual satisfaction in the invested partners was comparable to that of their sexually dysfunctional partners.
Thyroid Function in Patients with Aspermiogenesis and Testicular Tubular Sclerosis
Authors: Thomas E. Davis, Craig J. Canfield, Robert H. Herman, David Goler
Journal: New England Journal of Medicine (1963)

IN 1942 Klinefelter et al.1 described a syndrome of micro-orchidism, azoospermia, hyalinization of seminiferous tubules, elevated urinary follicle-stimulating hormone (FSH) and gynecomastia. Since then it has become apparent from numerous reports that Klinefelter's syndrome may be variously manifested but at least consists of micro-orchidism, oligospermia, hyalinization of seminiferous tubules2 (even though occasional areas of spermatogenesis may be seen3 4 5 6 7), an apparent increase in Leydig cells and an elevated FSH level.1 , 8 , 9 There may or may not be gynecomastia,2 , 7 the body habitus may range from the eunuchoid to the male type,5 6 7 , 10 the I.Q. may be normal to subnormal,10 11 12 13 14 15 and the chromosomal pattern . . .
Autoantibodies to Glutamic Acid Decarboxylase in a Patient with Stiff-Man Syndrome, Epilepsy, and Type I Diabetes Mellitus
Authors: M Solimena, F Folli, S Denis-Donini, G C Comi, G Pozza, P De Camilli, A M Vicari
Journal: New England Journal of Medicine (1988)

Stiff-man syndrome is a rare disorder of the central nervous system consisting of progressive, fluctuating muscle rigidity with painful spasms. It is occasionally associated with endocrine disorders, including insulindependent diabetes, and with epilepsy. We investigated the possible existence of autoimmunity against the nervous system in a patient with stiff-man syndrome associated with epilepsy and Type I diabetes mellitus. Levels of IgG, which had an oligoclonal pattern, were elevated in the cerebrospinal fluid. The serum and the cerebrospinal fluid produced an identical, intense staining of all gray-matter regions when used to stain brain sections according to an indirect light-microscopical immunocytochemical procedure. The staining patterns were identical to those produced by antibodies to glutamic acid decarboxylase (the enzyme responsible for the synthesis of gamma-aminobutyric acid). A band comigrating with glutamic acid decarboxylase in sodium dodecyl sulfate–polyacrylamide gels appeared to be the only nervoustissue antigen recognized by cerebrospinal fluid antibodies, and the predominant antigen recognized by serum antibodies. These findings support the idea that an impairment of neuronal pathways that operate through gamma-aminobutyric acid is involved in the pathogenesis of stiffman syndrome, and they raise the possibility of an autoimmune pathogenesis. (N Engl J Med 1988; 318: 1012–20.)
Clinical Characteristics of Non-IgE-Mediated Gastrointestinal Food Allergy: Analysis of Nation-Wide Web-Based Online Patient Registry
Authors: Ichiro Nomura, Hiroko Suzuki, Tetsuo Shoda, Hideaki Morita, Kanami Orihara, Yukihiro Ohya, Hirohisa Saito, Kenji Matsumoto
Journal: Journal of Allergy and Clinical Immunology (2016)
Clinical Application of Cobalt<sup>60</sup>-Labeled Vitamin B<sub>12</sub>Urine Test
Authors: Melvin I. Klayman, Lloyd Brandborg
Journal: New England Journal of Medicine (1955)

SCHILLING1 has observed that normal patients excrete definite amounts of radioactive substances in the urine after an oral dose of 2 microgm. of vitamin B12 labeled with cobalt60 and a parenteral "flushing" dose of 1000 microgm. of the nonradioactive vitamin B12. In contrast, patients with pernicious anemia in remission excrete no detectable radioactivity in the urine when the labeled vitamin is administered with water or boiled gastric juice. However, the simultaneous administration of active gastric juice with the oral dose of vitamin B12 labeled with cobalt60 results in easily detectable amounts of radioactive substances in the . . .
Three Years' Experience with Electric Convulsive Therapy
Authors: Jacob Norman, John T. Shea
Journal: New England Journal of Medicine (1946)

ELECTRIC convulsive therapy has been used for the last three years at the Foxborough State Hospital and has become the chief form of physical therapy in the treatment of psychoses of psychogenic origin and of severe psychoneuroses. During this period 266 patients have been treated, as follows: schizophrenia, 175 cases; manic-depressive psychosis, 51 cases; involutional melancholia, 24 cases; and psychoneurosis, 16 cases. The majority of the schizophrenic patients had been in the hospital for periods ranging from one to thirty years and showed definite deterioration.In all newly admitted functional cases, an attempt was made to obtain permission for shock . . .
Increased plasma nicotinamide phosphoribosyltransferase is associated with a hyperproliferative phenotype and restrains disease progression in MPN‐associated myelofibrosis
Authors: Vittorio Rosti, Rita Campanelli, Margherita Massa, Gianluca Viarengo, Laura Villani, Valentina Poletto, Elisa Bonetti, Paolo Catarsi, Umberto Magrini, Ambra A. Grolla, Cristina Travelli, Armando A. Genazzani, Giovanni Barosi
Journal: American Journal of Hematology (2016)

Myeloproliferative neoplasm (MPN)-associated myelofibrosis is a clonal, neoplastic disorder of the hematopoietic stem cells, in which inflammation and immune dysregulation play an important role. Extracellular nicotinamide phosphoribosyltransferase (eNAMPT), also known as visfatin, is a cytokine implicated in a number of inflammatory and neoplastic diseases. Here plasma levels of eNAMPT in patients with MPN-associated myelofibrosis and their effects on disease phenotype and outcomes were examined. The concordance of eNAMPT levels with the marker of general inflammation high-sensitivity C-reactive protein (hs-CRP) was also studied. A total of 333 MPN-associated myelofibrosis patients (187 males and 146 females) and 31 age- and gender-matched normal-weight healthy subjects were enrolled in the study main body. Levels of eNAMPT and hs-CRP were simultaneously assayed in 209 MPN-associated myelofibrosis patients. Twenty-four polycythemia vera or essential thrombocythemia patients were used as controls. eNAMPT was over expressed in MPN-associated myelofibrosis, and eNAMPT expression was correlated with higher white blood cell count, higher hemoglobin, and higher platelet count, suggesting that eNAMPT is an indispensable permissive agent for myeloproliferation of MPN-associated myelofibrosis. The lack of correlation between eNAMPT and hs-CRP revealed that eNAMPT in MPN-associated myelofibrosis does not behave as a canonical inflammatory cytokine. In addition, higher levels of eNAMPT predicted longer time to blast transformation, and protected against progression toward thrombocytopenia and large splenomegaly. In conclusion, in MPN-associated myelofibrosis high levels of eNAMPT mark the myeloproliferative potential and, at variance with a high number of cancers, are protective against disease progression. Am. J. Hematol. 91:709-713, 2016. © 2016 Wiley Periodicals, Inc.
28P Different genetic profiling in lung adenocarcinoma of smokers with and without chronic obstructive pulmonary disease (COPD): An exploratory analysis by next generation sequencing (NGS)
Authors: L. Bonanno, M. Schiavon, F. Lunardi, M. Tebaldi, D. Calistri, G. Pasello, P.F. Conte, F. Rea, S. Indraccolo, F. Calabrese
Journal: Journal of Thoracic Oncology (2016)
Control of a multi-hospital outbreak of KPC-producing Klebsiella pneumoniae type 2 in France, September to October 2009
Authors: A Carbonne, J  M Thiolet, S Fournier, N Fortineau, N Kassis-Chikhani, I Boytchev, M Aggoune, J C Séguier, H Sénéchal, M P Tavolacci, B Coignard, P Astagneau, V Jarlier
Journal: Eurosurveillance (2010)

An outbreak of Klebsiella pneumoniae carbapenemase (KPC)-producing Klebsiella pneumoniae type 2 was detected in September 2009 in two hospitals in a suburb south of Paris, France. In total, 13 KPC-producing K. pneumoniae type 2 cases (four with infections and nine with digestive-tract colonisations) were identified, including a source case transferred from a Greek hospital. Of the 13 cases, seven were secondary cases associated with use of a contaminated duodenoscope used to examine the source case (attack rate: 41%) and five were secondary cases associated with patient-to-patient transmission in hospital. All isolated strains from the 13 patients: (i) exhibited resistance to all antibiotics except gentamicin and colistin, (ii) were more resistant to ertapenem (minimum inhibitory concentration (MIC) always greater than 4 mg/L) than to imipenem (MIC: 1–8 mg/L, depending on the isolate), (iii) carried the blaKPC-2 and blaSHV12 genes and (iv) had an indistinguishable pulsed-field gel electrophoresis (PFGE) pattern. These cases occurred in three hospitals: some were transferred to four other hospitals. Extended infection control measures implemented in the seven hospitals included: (i) limiting transfer of cases and contact patients to other wards, (ii) cohorting separately cases and contact patients, (iii) reinforcing hand hygiene and contact precautions and (iv) systematic screening of contact patients. Overall, 341 contact patients were screened. A year after the outbreak, no additional case has been identified in these seven hospitals. This outbreak emphasises the importance of rapid identification and notification of emerging highly resistant K. pneumoniae strains in order to implement reinforced control measures.
Pneumococcal conjugate vaccine for adults: “It's tough to make predictions, …”
Authors: Mathias W. Pletz, Tobias Welte
Journal: European Respiratory Journal (2015)

"… especially about the future." This frequently used Yogi Berra quote also holds true for the paper by Mangen et al. [1] from the Netherlands, which modelled the cost-effectiveness of a potential vaccination programme for elderly adults using the 13-valent pneumococcal conjugate vaccine. Do the results of the CAPITA study justify the general vaccination of adults with PCV13? <http://ow.ly/RW74T> We would like to thank Alexander Kuhlmann (Centre for Health Economics, Hannover, Germany) for intense and fruitful discussions.
Inherited Variants of Factor-VIII-Related Protein in von Willebrand's Disease
Authors: Ian R. Peake, Arthur L. Bloom, John C. Giddings
Journal: New England Journal of Medicine (1974)

Three patients from each of two families suffering from von Willebrand's disease were re-evaluated to characterize more clearly the bleeding disorders included in this classification. In all six patients, male and female, the bleeding times were grossly prolonged, but levels of procoagulant factor VIII were normal or only moderately reduced. In five of the patients levels of factor-VIII-related protein measured by an immunoelectrophoretic method were normal, and in the sixth, they were normal or moderately reduced. The plasma of these patients, however, did not support ristocetin platelet aggregation, and factor-VIII-related protein showed abnormal mobility on two dimensional crossed immunoelectrophoresis. The results indicate that these patients synthesize a variant of this protein and that they represent a distinct phenotype or phenotypes of von Willebrand's disease. (N Engl J Med 291:113–117, 1974)
Demonstration of an Intrathymic Defect in a Case of Severe Combined Immunodeficiency Disease
Authors: Kenneth W. Pyke, Hans-Michael Dosch, Moshe M. Ipp, Erwin W. Gelfand
Journal: New England Journal of Medicine (1975)

An in vitro system has been developed to elucidate the nature of the cellular defect in primary immunodeficiency diseases. Incubation, on human thymic epithelial monolayer cultures, of peripheral blood lymphocytes and bone-marrow cells from a child with documented severe combined immunodeficiency disease resulted in the appearance of a population of cells that formed rosettes with sheep erythrocytes. The same cell preparation permitted the synthesis of antigen-specific, complement-dependent antibodies after in vitro education, as demonstrated in a plaque assay system. In addition, thymic tissue from the same child gave morphologic and functional evidence of maturation when cultured in vitro. The experimental results suggest that in this case, lymphoid precursor cells were present in the bone marrow but failed to differentiate to functional maturity due to a defect in maturation of thymic tissue.
SURG-09RESULTS OF A DOSE ESCALATION TRIAL OF TOCA 511 WITH TOCA FC IN RECURRENT HGG UNDERGOING REPEAT RESECTION
Authors: Michael A. Vogelbaum, Santosh Kesari, Steven N. Kalkanis, Tom Mikkelsen, Joseph Landolfi, J. Bradley Elder, Clark C. Chen, Joan Robbins, Derek Ostertag, Doug J. Jolly, Mary R. Keller, Jamey Skillings, Alice Chu, Timothy F. Cloughesy
Journal: Neuro-Oncology (2015)
Clinical Assay of a New Synthetic Estrogen: Vallestril
Authors: M. I. Sturnick, S. L. Gargill
Journal: New England Journal of Medicine (1952)

IN 1947 and 1948 Courrier, Horeau and Jacques1 2 3 4 5 published a series of chemical and biologic studies of certain derivatives of a compound that they called "allenolic acid," in honor of Dr. E. Allen.2 This compound is known chemically as 6-hydroxy-2-naphthaleneproprionic acid. The name allenolic acid has been used widely in France and is recognized, although not officially accepted, by Chemical Abstracts. The derivatives found to possess strong estrogenic acitivity were 3- (6-methoxy-2-naphthyl)-2–2-dimethylpentanoic acid and the corresponding hydroxy compound, referred to by the French interchangeably as dimethyl-ethyl allenolic acid. The authors studied the biologic effects of these two dimethylpentanoic acid . . .
Treatment of Aplastic Anemia with Antilymphocyte Globulin and Methylprednisolone with or without Cyclosporine
Authors: N Frickhofen, J P Kaltwasser, H Schrezenmeier, A Raghavachar, H G Vogt, F Herrmann, M Freund, P Meusers, A Salama, H Heimpel
Journal: New England Journal of Medicine (1991)

Immunosuppression is the most effective treatment for patients with aplastic anemia, except for bone marrow transplantation. The best results are achieved with antilymphocyte globulin or cyclosporine. Patients have been treated successfully with a combination of both agents, but there has been no controlled evaluation of its efficacy. We conducted a randomized, multicenter trial in 84 patients not eligible for bone marrow transplantation, comparing treatment with antilymphocyte globulin and methylprednisolone (41 patients — control group) with antilymphocyte globulin, methylprednisolone, and cyclosporine (43 patients — the cyclosporine group).
Infantile Metachromatic Leukodystrophy
Authors: Michael M. Kaback, R. Rodney Howell
Journal: New England Journal of Medicine (1970)

A profound deficiency of arylsulfatase A activity (ARA) is detectable in skin fibroblasts developed from patients with infantile metachromatic leukodystrophy (about 3 to 4 per cent of normal). Fibroblasts were cultivated from skin biopsies of parents and siblings in four unrelated families in which a child with infantile metachromatic leukodystrophy had been documented. Fibroblast ARA activities of seven parents ranged from three to 15 times higher than those found in the affected patients, but were below the activity measured in control fibroblasts, confirming an autosomal recessive mode of inheritance for this disease. ARA specific activity in cultured cells from normal mid-trimester amniotic-fluid samples is significantly lower than that found in control skin fibroblast cultures. Hence values found in amniotic-fluid cell cultures do not necessarily identify the fetus with metachromatic leukodystrophy.
Clinical and laboratory evaluation of infants and children with Epstein- Barr virus-induced infectious mononucleosis: report of 32 patients (aged 10-48 months)
Authors: CA Horwitz, W Henle, G Henle, M Goldfarb, P Kubic, RC Gehrz, HH Jr Balfour, GR Fleisher, W Krivit
Journal: Blood (1981)
SP0012 What to do After 5 Years of Treatment of Osteoporosis?
Authors: K. Saag
Journal: Annals of the Rheumatic Diseases (2015)

Bisphosphonates are the most commonly used drugs to manage osteoporosis. They reduce fractures at both non-vertebral and vertebral sites by 20 to 70 percent, respectively over a 3 to 10 year period based on results of randomized controlled trials. As a class, bisphosphonates have prolonged skeletal retention and persistent suppression of bone resorption persists, well beyond their period of initial use. Potent IV bisphosphonate may be dosed less often then annually with near similar effects on bone mineral density and fracture risk reduction. Further, concerns have emerged about rare bisphosphonate associated adverse effects including osteonecrosis of the jaw and atypical femoral fractures. Based on the skeletal properties of these drugs and new safety concerns, many clinicians have moved towards a bisphosphonate drug holiday after fixed period of drug use, typically 3 to 5 years. Which patients are optimal candidates for these holidays and when to reinitiate therapy remains controversial. Balancing the potential risk and benefits of these agents is a primary goal improve osteoporosis public health. <h3>Disclosure of Interest</h3> None declared
S62 Using Drosophila melanogaster to Study Pathogenic Mutants of Surfactant Protein C
Authors: E Malzer, SJ Marciniak
Journal: Thorax (2015)

<h3>Introduction and objectives</h3> Surfactant protein C (SFTPC) is secreted by type II pneumocytes to reduce alveolar lining fluid surface tension and thus prevent alveolar collapse at low lung volumes. The immature form of SFTPC must undergo proteolytic processing before being secreted as the mature form, but several pathogenic <i>SFTPC </i>mutations associated with familial interstitial lung disease impede this process. Mutations in the C-terminal BRICHOS domain of SFTPC (ΔEx4 and L188Q) lead to retention of the protein within the endoplasmic reticulum (ER), while other mutations (e.g. I73T) cause SFTPC mis-trafficking. <h3>Methods</h3> To study these mutants <i>in vivo</i> in a genetically tractable organism, we generated lines of <i>Drosophila melanogaster</i> expressing wild type or mutant human SFTPC. The transgenic proteins could be tagged with green fluorescent protein (GFP) to facilitate <i>in vivo</i> visualisation. These fusion proteins were expressed under the control of tissue-specific drivers. Components of the ER associated degradation (ERAD) machinery or of the autophagy pathway were depleted in those tissues by RNA interference. Lines expressing an ER stress reporter or autophagy reporter were used as readouts for these phenomena. <h3>Results</h3> Expression of the BRICHOS mutants ΔEx4 and L188Q led to the progressive deposition of protein aggregates when expressed in the fly eye. In contrast, the I73T mutant accumulated in a more diffuse distribution. When expressed in the larval salivary gland, the BRICHOS mutants where retained within the cell, in contrast to the wild type protein that was trafficked to the cell surface. The I73T mutant showed low-level cell surface and weak intracellular fluorescence. Depletion of the ERAD E3 ubiquitin ligase Hrd1 or its associated E2 ligases failed to affect mutant protein levels arguing against an important role of ERAD in the degradation of SFTPC in this model. In contrast, inhibition of autophagy by depletion of Atg8 enhanced the accumulation of L188Q SFTPC. Accordingly, robust activation of autophagy was detected in L188Q SFTPC-expressing tissue. Interestingly, ER stress was not detected. <h3>Conclusion</h3> In a <i>Drosophila </i>model of hSFTPC trafficking, autophagy was the major degradation pathway for L188Q mutant SFTPC.
Care of Female Psychiatric Patients, Including the Acutely Disturbed, on an Open Medical and Surgical Ward
Authors: Pietro Castelnuovo-Tedesco
Journal: New England Journal of Medicine (1957)

DURING the last two decades greater attention has been paid by the medical profession to what Stanton and Schwartz1 have called the problem of "institutional participation" in the treatment and management of psychiatric illness. More and more, the trend has been away from the "asylum" that separated the patient emotionally as well as geographically from the social community and from his customary activities. More and more, efforts have been made to integrate the treatment of the patient with his daily life and with the accepted fabric of community organization, and to prevent psychiatric therapy from becoming an occasion that robs . . .
Arterial Changes in the Lungs in Cirrhosis of the Liver — Lung Spider Nevi
Authors: P. Berthelot, J.G. Walker, Sheila Sherlock, Lynne Reid
Journal: New England Journal of Medicine (1966)

THE association of hepatic cirrhosis with cyanosis and finger clubbing1 2 3 4 and with arterial oxygen desaturation4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 has been recognized for many years, and the possible basis investigated. Abnormalities in the oxygen dissociation curve in cirrhotic patients have been reported6 but not confirmed.11 , 12 More recently, Caldwell, Fritts and Cournand20 again called attention to abnormal oxygen tension-saturation relations in cirrhosis but concluded that the contribution of these toward oxygen unsaturation was probably small. Physiologic evidence for an admixture of venous to arterial blood has been presented.8 9 10 11 12 13 14 15 16 17 18 19 The possibility of anastomoses between the pulmonary veins and either the pulmonary artery or the portal venous . . .