tbuckley/cabot-search huggingface dataset:
Calculation of cut‐off values based on the Autoimmune Bullous Skin Disorder Intensity Score ( <scp>ABSIS</scp> ) and Pemphigus Disease Area Index ( <scp>PDAI</scp> ) pemphigus scoring systems for defining moderate, significant and extensive types of pemphigus
Authors: C. Boulard, S. Duvert Lehembre, C. Picard‐Dahan, J.S. Kern, G. Zambruno, C. Feliciani, B. Marinovic, P. Vabres, L. Borradori, C. Prost‐Squarcioni, B. Labeille, M.A. Richard, S. Ingen‐Housz‐Oro, E. Houivet, V.P. Werth, D.F. Murrell, M. Hertl, J. Benichou, P. Joly, S. Hofmann, B. Camaioni, B. Didona, L. Fania, R. Della Torre, F. Caux, M. Alexandre, C. Paul, C. Bulai Livideanu, E. Delaporte, A. Dupuy, M. Avenel‐Audran, E. Schmidt, N. Schumacher, M. Bagot, E. Tancrede‐Bohin, J.D. Bouaziz, M. D'Incan, G. Quereux, F. Skowron, M. Beylot‐Barry, M.S. Doutre, C. Bedane, P. Bernard, L. Machet, . Jullien, S. Debarbieux, O. Dereure, C. Hünefeld, S. Schanz, N. Franck, H. Maillard, L. Misery, B. Sassolas, C. Abasq, A.C. Bouyeure, P. Lagoutte, V. Ferranti
Journal: British Journal of Dermatology (2016)

Two pemphigus severity scores, Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Pemphigus Disease Area Index (PDAI), have been proposed to provide an objective measure of disease activity. However, the use of these scores in clinical practice is limited by the absence of cut‐off values that allow differentiation between moderate, significant and extensive types of pemphigus. To calculate cut‐off values defining moderate, significant and extensive pemphigus based on the ABSIS and PDAI scores. In 31 dermatology departments in six countries, consecutive patients with newly diagnosed pemphigus were assessed for pemphigus severity, using ABSIS, PDAI, Physician's Global Assessment (PGA) and Dermatology Life Quality Index (DLQI) scores. Cut‐off values defining moderate, significant and extensive subgroups were calculated based on the 25th and 75th percentiles of the ABSIS and PDAI scores. The median ABSIS, PDAI, PGA and DLQI scores of the three severity subgroups were compared in order to validate these subgroups. Ninety‐six patients with pemphigus vulgaris (n = 77) or pemphigus foliaceus (n = 19) were included. The median PDAI activity and ABSIS total scores were 27·5 (range 3–84) and 34·8 points (range 0·5–90·5), respectively. The respective cut‐off values corresponding to the first and third quartiles of the scores were 15 and 45 for the PDAI, and 17 and 53 for ABSIS. The moderate, significant and extensive subgroups were thus defined, and had distinguishing median ABSIS (P < 0·001), PDAI (P < 0·001), PGA (P < 0·001) and DLQI (P = 0·03) scores. This study suggests cut‐off values of 15 and 45 for PDAI and 17 and 53 for ABSIS, to distinguish moderate, significant and extensive pemphigus forms. Identifying these pemphigus activity subgroups should help physicians to classify and manage patients with pemphigus.
Effect of Cocaine Use on the Fetus
Authors: Franklin H. Epstein, Joseph J. Volpe
Journal: New England Journal of Medicine (1992)

THE effect of cocaine use by pregnant women on their fetuses and then newborn infants is a topic of major public health importance. Cocaine has profound effects on the central and peripheral nervous systems of adults and can cause major alterations in fetal brain structure and subsequently in neurologic, cognitive, and behavioral functions. From 10 to 45 percent of women cared for at urban teaching hospitals take cocaine during their pregnancies, considerably more than did so 10 to 15 years ago.1 2 3 4 5 6 7 8 9 10 The problem of fetal exposure to cocaine is not confined to urban hospital populations; in a recent study 6 . . .
The Leukocyte Count as a Predictor of Myocardial Infarction
Authors: Gary D. Friedman, Arthur L. Klatsky, A. B. Siegelaub
Journal: New England Journal of Medicine (1974)

The multiphasic-examination findings of 464 persons in whom a first myocardial infarction later developed were compared with those of two control groups, one matched for age, sex and race (ordinary controls), and the other matched in addition for standard coronary risk factors (risk controls). The total leukocyte count, measured, on the average, 16.8 months before the myocardial infarction, was strikingly related to development of infarction. The mean leukocyte count in cases was significantly higher (p<0.001) than in either control group. Ascending from lowest to highest quartile in the cases and ordinary controls the increase in risk of myocardial infarction associated with the leukocyte count was similar to that found for cholesterol and blood pressure. Cigarette smoking, which was strongly related to the leukocyte count, may account for about two thirds of the relation of the count to infarction. The leukocyte count may prove valuable in the routine assessment of risk of myocardial infarction. (N Engl J Med 290:1275–1278, 1974)
Alteration of Blood on Storage: Measurement of Adhesiveness of Aging Platelets and Leukocytes and Their Removal by Filtration
Authors: Roy L. Swank
Journal: New England Journal of Medicine (1961)

DURING studies of blood viscosity with a method designed to determine resistance to flow in very small blood vessels, it was unexpectedly found that very high pressures became necessary to force blood-bank blood through a microfilter with multiple openings 20 microns square after storage in acid-citrate-dextrose (A.C.D.) solution for two to ten days or longer or in heparin for only one day. This phenomenon, the apparatus that demonstrated it and a method by which the filtration characteristics of old stored blood can be returned to normal are described below.Material and MethodsBlood was routinely collected in A.C.D. solution or . . .
Microparticles in Nasal Lavage; Potential Biomarkers for Chronic Rhinosinusitis and Aspirin Exacerbated Respiratory Disease
Authors: Toru Takahashi, James E. Norton, Lydia Suh, Roderick G. Carter, Robert C. Kern, Bruce K. Tan, Stephanie S. Smith, Kevin C. Welch, David B. Conley, Anju T. Peters, Leslie C. Grammer, Kathleen E. Harris, Whitney W. Stevens, Kathryn E. Hulse, Bruce S. Bochner, Atsushi Kato, Robert P. Schleimer
Journal: Journal of Allergy and Clinical Immunology (2016)
Characterisation of patients with interstitial pneumonia with autoimmune features
Authors: Justin M. Oldham, Ayodeji Adegunsoye, Eleanor Valenzi, Cathryn Lee, Leah Witt, Lena Chen, Aliya N. Husain, Steven Montner, Jonathan H. Chung, Vincent Cottin, Aryeh Fischer, Imre Noth, Rekha Vij, Mary E. Strek
Journal: European Respiratory Journal (2016)

Patients with interstitial lung disease (ILD) may have features of connective tissue disease (CTD), but lack findings diagnostic of a specific CTD. A recent European Respiratory Society/American Thoracic Society research statement proposed criteria for patients with interstitial pneumonia with autoimmune features (IPAF). We applied IPAF criteria to patients with idiopathic interstitial pneumonia and undifferentiated CTD-ILD (UCTD). We then characterised the clinical, serological and morphological features of the IPAF cohort, compared outcomes to other ILD cohorts and validated individual IPAF domains using survival as an endpoint. Of 422 patients, 144 met IPAF criteria. Mean age was 63.2 years with a slight female predominance. IPAF cohort survival was marginally better than patients with idiopathic pulmonary fibrosis, but worse than CTD-ILD. A non-usual interstitial pneumonia pattern was associated with improved survival, as was presence of the clinical domain. A modified IPAF cohort of those meeting the clinical domain and a radiographic or histological feature within the morphological domain displayed survival similar to those with CTD-ILD. IPAF is common among patients with idiopathic interstitial pneumonia and UCTD. Specific IPAF features can identify subgroups with differential survival. Further research is needed to replicate these findings and determine whether patients meeting IPAF criteria benefit from immunosuppressive therapy.
Number of Pregnancies and the Subsequent Risk of Cardiovascular Disease
Authors: Roberta B. Ness, Tamara Harris, Janet Cobb, Katherine M. Flegal, Jennifer L. Kelsey, Albert Balanger, Albert J. Stunkard, Ralph B. D'Agostino
Journal: New England Journal of Medicine (1993)

Whether increasing parity or gravidity is a risk factor for coronary heart disease has been debated, but the question remains unresolved.
Lack of Immune Responsiveness to Bovine Serum Albumin in Insulin-Dependent Diabetes
Authors: Mark A. Atkinson, Mark A. Bowman, Kuo-Jang Kao, Lalita Campbell, Paula J. Dush, Shirish C. Shah, Olli Simell, Noel K. Maclaren
Journal: New England Journal of Medicine (1993)

Epidemiologic studies have implicated the ingestion of cow's milk in the pathogenesis of insulin-dependent diabetes mellitus (IDDM). Moreover, in a recent study, 100 percent of patients with new-onset IDDM had antibodies against bovine serum albumin (BSA), with a majority directed against a 17-amino-acid BSA peptide (ABBOS). Cellular immune mechanisms are thought to be the principal mediators of pancreatic beta-cell destruction in IDDM.
Neuropsychological Outcome of Zidovudine (AZT) Treatment of Patients with AIDS and AIDS-Related Complex
Authors: F A Schmitt, J W Bigley, R McKinnis, P E Logue, R W Evans, J L Drucker
Journal: New England Journal of Medicine (1988)

Two hundred eighty-one patients with the acquired immunodeficiency syndrome (AIDS) or advanced AIDS-related complex were enrolled in a double-blind, placebo-controlled trial of the efficacy and safety of orally administered zidovudine (azidothymidine or AZT). Significant clinical benefits and adverse experiences have been reported from this trial. Because neuropsychiatrie dysfunction is often associated with human immunodeficiency virus (HIV) infection, a brief affective and neuropsychological examination was administered over 16 weeks of the trial to evaluate any changes in neuropsychological function that occurred with drug administration. Patients receiving zidovudine, particularly those with AIDS, showed improved cognition as compared with patients receiving placebo. There were no changes in affective symptoms. The zidovudine recipients also had a statistically significant reduction in the intensity of symptomatic distress during the trial that may account in part for the observed cognitive changes. Some improvement in various cognitive measures was also seen in patients with AIDS-related complex. The results of this study suggest HIV-associated cognitive abnormalities may be partially ameliorated after the administration of zidovudine. (N Engl J Med 1988; 319: 1573–8.)
Modification of Chicken Pox in Family Contacts by Administration of Gamma Globulin
Authors: Avron H. Ross, Edna Lenchner, George Reitman
Journal: New England Journal of Medicine (1962)

SINCE 1944, when the use of gamma globulin was introduced for the modification and prevention of measles,1 , 2 numerous attempts to prevent, attenuate or treat chicken pox with this agent have been reported.3 4 5 6 7 8 9 10 Before that time convalescent varicella serum had been used with similar objectives.11 In recent years, the need for a method of modification of chicken pox has become increasingly apparent, owing to the publication of descriptions of a variety of serious and sometimes fatal sequelae under certain well defined circumstances. These have included primary varicella pneumonia in adults,12 13 14 15 visceral dissemination,16 , 17 death from neonatal chicken pox,18 19 20 21 fatal chicken pox in . . .
Successful Bone-Marrow Transplantation in a Lymphopenic Immunologic Deficiency Syndrome
Authors: A. Rubinstein, B. Speck, M. Jeannet
Journal: New England Journal of Medicine (1971)

Abstract A 12-year-old boy with lymphopenic immunologic deficiency syndrome (common variable type) was treated with an infusion of bone-marrow cells from a 10-year-old HL-A identical brother. Despite the HL-A identity and a negative mixed lymphocyte culture, a severe graft-versus-host reaction developed from which he recovered, except for persisting hepatitis. The transplant resulted in red-cell chimerism and in an immunologic reconstitution that was almost complete but of an unusual pattern. IgM and IgG concentration increased and exceeded those of normal adults.
P34 Sarcoidosis and co-existent Aspergillus lung disease
Authors: S Gudur, E Nuttall, C Harris, N Chaudhuri, C Leonard, E Muldoon
Journal: Thorax (2015)

<h3></h3> Sarcoidosis is a multisystem disorder which affects the lungs and in a small percentage of cases may result in fibrosis and cystic cavitating lesions. Chronic pulmonary aspergillosis (CPA) typically affects patients with underlying lung conditions; immunosuppressive therapy is not recommended due to risk of progression or spread of Aspergillus infection. Sarcoid patients are often significantly debilitated when they develop co-existent Aspergillus lung disease. They can present with worsening breathlessness, weight loss and significant haemoptysis. There is no consensus on how to best treat patients diagnosed with Sarcoid and CPA. <h3>Methods</h3> A retrospective review of patients diagnosed with Sarcoidosis and CPA was performed. Cases were identified from a database held of patients diagnosed with CPA group in our specialist clinic. Patient demographics, presentation, laboratory parameters, radiology results were recorded and data analysed. <h3>Results</h3> 38 patients with sarcoidosis were diagnosed with CPA from 2009 – March 2015. 23/36 (63%) were male. 14/36 (38%) patients were affected by breathlessness with MRC dyspnoea score ≥3. 15/36 (41%) of patients were on maintenance Prednisolone between 5 – 10 mg/day and 3 patients on other steroid sparing agents. 12/28 patients had obstructive spirometry and average predicted gas transfer factor at 49% (range 20 – 75%). 33/36 (91%) had CPA defined by presence of serum precipitins and 50% of patients had aspergillomas detected in the cavities on radiology. 27/36 (75%) of patients were on antifungal therapy. On regular follow-up, antifungal therapy was titrated based on serum azole levels and as tolerated. 4/36 (11%) patients had been treated for proven TB and 2/36 were on empirical treatment until TB diagnosis was excluded when there was clinical and radiological deterioration. <h3>Conclusions</h3> Although sarcoidosis has slight female predominance, in this cohort of patients with CPA and sarcoid 63% were males. Many of the patients had significant lung disease with high MRC dyspnoea scores, and obstructive spriometry often with low gas transfers. The management of CPA in the setting of sarcoidosis is complex due to the risk of immunosuppression and interactions with antifungal therapy. Close working between specialties is important in optimising such patients, to ensure the best patient outcomes.
Case 26-1970
Authors: Richard C. Cabot, Benjamin Castleman, Betty U. McNeely, Barry G. W. Arnason, Manuel Ramírez-Lassepas, Benjamin Castleman
Journal: New England Journal of Medicine (1970)

Presentation of Case First admission. A 70-year-old man entered the hospital because of dyspnea.He had previously had excellent health except for mild hypertension, for which he took methyldopa twice daily. Repeated physical examinations had shown no cardiac abnormality. On the day before admission he abruptly became dyspneic, sweaty and nauseated but had no chest pain. On admission to another hospital he was found to be in pulmonary edema, with atrial fibrillation and a rapid ventricular response. After digitalis, aminophylline, morphine and oxygen were given the cardiac rhythm reverted to normal, but dyspnea persisted. On the following day, because . . .
Development of Engineered T Cells Expressing a Chimeric CD16-CD3ζ Receptor to Improve the Clinical Efficacy of Mogamulizumab Therapy Against Adult T-Cell Leukemia
Authors: Hiroki Tanaka, Hiroshi Fujiwara, Fumihiro Ochi, Kazushi Tanimoto, Nicholas Casey, Sachiko Okamoto, Junichi Mineno, Kiyotaka Kuzushima, Hiroshi Shiku, Takashi Sugiyama, A. John Barrett, Masaki Yasukawa
Journal: Clinical Cancer Research (2016)

Mogamulizumab (Mog), a humanized anti-CC chemokine receptor 4 (CCR4) mAb that mediates antibody-dependent cellular cytotoxicity (ADCC) using FcγR IIIa (CD16)-expressing effector cells, has recently been approved for treatment of CCR4-positive adult T-cell leukemia (ATL) in Japan. However, Mog failure has sometimes been observed in patients who have accompanying chemotherapy-associated lymphocytopenia. In this study, we examined whether adoptive transfer of artificial ADCC effector cells combined with Mog would overcome this drawback.We lentivirally gene-modified peripheral blood T cells from healthy volunteers and ATL patients expressing the affinity-increased chimeric CD16-CD3ζ receptor (cCD16ζ-T cells). Subsequently, we examined the ADCC effect mediated by those cCD16ζ-T cells in the presence of Mog against ATL tumor cells both in vitro and in vivocCD16ζ-T cells derived from healthy donors killed in vitro Mog-opsonized ATL cell line cells (n = 7) and primary ATL cells (n = 4) depending on both the number of effector cells and the dose of the antibody. cCD16ζ-T cells generated from ATL patients (n = 3) also exerted cytocidal activity in vitro against Mog-opsonized autologous ATL cells. Using both intravenously disseminated model (n = 5) and subcutaneously inoculated model (n = 4), coadministration of Mog and human cCD16ζ-T cells successfully suppressed tumor growth in xenografted immunodeficient mice, and significantly prolonged their survival (P < 0.01 and P = 0.02, respectively).These data strongly suggest clinical feasibility of the novel combined adoptive immunotherapy using cCD16ζ-T cells and Mog for treatment of aggressive ATL, particularly in patients who are ineligible for allogeneic hematopoietic stem cell transplantation. Clin Cancer Res; 22(17); 4405-16. ©2016 AACR.
Trimetrexate for the Treatment of<i>Pneumocystis carinii</i>Pneumonia in Patients with the Acquired Immunodeficiency Syndrome
Authors: C J Allegra, B A Chabner, C U Tuazon, D Ogata-Arakaki, B Baird, J C Drake, J T Simmons, E E Lack, J H Shelhamer, F Balis
Journal: New England Journal of Medicine (1987)

Preclinical studies have demonstrated that trimetrexate is a potent inhibitor of dihydrofolate reductase from Pneumocystis carinii. On the basis of this evidence, this lipid-soluble antifolate was used as an antipneumocystis agent in 49 patients with the acquired immunodeficiency syndrome (AIDS) and pneumocystis pneumonia. Simultaneous treatment with the reduced folate leucovorin was used as a specific antidote to protect host tissues from the toxic effects of the antifolate without affecting the antipneumocystis action of trimetrexate. Patients were assigned to three groups and treated for 21 days: in Group I, trimetrexate with leucovorin was used as salvage therapy in patients in whom standard treatments (both pentamidine isethionate and trimethoprim-sulfamethoxazole) could not be tolerated or had failed (16 patients); in Group II, trimetrexate with leucovorin was used as initial therapy in patients with a history of sulfonamide inefficacy or intolerance (16 patients); and in Group III, trimetrexate with leucovorin plus sulfadiazine was used as initial therapy (17 patients). The response and survival rates were, respectively, 69 percent and 69 percent in Group I; 63 percent and 88 percent in Group II; and 71 percent and 77 percent in Group III. Trimetrexate therapy had minimal toxicity; transient neutropenia or thrombocytopenia occurred in 12 patients and mild elevation of serum aminotransferases in 4. We conclude that the combination of trimetrexate and leucovorin is safe and effective for the initial treatment of pneumocystis pneumonia in patients with AIDS and for the treatment of patients with intolerance or lack of response to standard therapies.
Michael Bernard Matthews
Authors: Philippa Matthews
Journal: BMJ (2015)

Michael Bernard Matthews—variously known as Ginger, Mick, MBM, and Mike—took an egalitarian and inclusive approach to all he worked with. He was patient centred long before it was a phrase and was loved for his gentle humour, compassion, and skills in communication and clinical examination.

Mike was born to Charles and Gertrude Matthews in Broadstairs, Kent, the youngest of three boys. After attending Marlborough College he gained an exhibition to Trinity Hall, Cambridge, then upgraded to a scholarship in 1940. His clinical training was at St Thomas’ during the war, and there were frequent bombings, sometimes by buzz bombs (or doodlebugs). It was known that when the buzzing of the motor stopped, the bomb would then fall and explode on impact. On one occasion Mike and his colleagues were playing golf in an evacuated ward at the top of the hospital, chipping a ball from one end of the ward into an open fireplace at the other end. They heard a buzz bomb, which subsequently …
Spontaneous Compression of the Median Nerve in the Carpal Tunnel
Authors: J. A. Mayer, D. M. Barry
Journal: New England Journal of Medicine (1954)

RELATIVELY little has been written about spontaneous compression of the median nerve in the carpal tunnel. Nevertheless, this condition is probably much more common than is realized. Undoubtedly, some patients with minor paresthesias do not seek medical attention, and, on the other hand, physicians unfamiliar with this condition may miss the diagnosis.LiteratureIn 1913 Marie and Foix1 presented a case of bilateral atrophy of the thenar muscles. Autopsy revealed bilateral median-nerve neuromas just proximal to the transverse carpal ligaments. In 1947 Brain, Wright and Wilkinson2 reported in some detail 6 cases of spontaneous compression of the median nerve at . . .
Immune Response of a Patient with Deficiency of the Fourth Component of Complement and Systemic Lupus Erythematosus
Authors: Charles G. Jackson, Hans D. Ochs, Ralph J. Wedgwood
Journal: New England Journal of Medicine (1979)

The clinical details of a five-year-old boy with systemic lupus erythematosus and an inherited deficiency of the fourth component of complement (C4) have been reported elsewhere. In this study of his immune responses, immunization with bacteriophage φ X 174 demonstrated diminished antibody formation, abnormal immunologic memory and failure to switch from IgM to IgG during secondary response. We also noted persistent lymphopenia and reductions in peripheral-blood T lymphocytes, lymphocyte responses to mitogens and allogeneic cells and granulocyte chemotaxis. Kinetic studies revealed that delayed activation of the alternative pathway was corrected by purified C4 only if the classical pathway was not blocked. This finding is consistent with the concept that minute amounts of C3b provided through the classical pathway are necessary to prime the properdin system. Inability to activate the classical complement pathway, abnormal kinetics of alternative-pathway activation and depressed antibody responses to a T-cell-dependent antigen may predispose C4-deficient patients to viral infection or immune-complex formation. (N Engl J Med 300:1124–1129, 1979)
Vancomycin-Resistant Enterococci in Turkeys and Farmers
Authors: Anthony E. van den Bogaard, Lars Bogø Jensen, Ellen E. Stobberingh
Journal: New England Journal of Medicine (1997)

To the Editor: In enterococci vancomycin resistance of the VanA phenotype is usually due to the presence of seven genes organized in a cluster on a transposon (Tn1546).1,2 The recent emergence of v...
Methods of Induction in Pediatric Anesthesia
Authors: Robert M. Smith
Journal: New England Journal of Medicine (1949)

IN INFANTS and children the technic of induction of anesthesia demands careful consideration, if optimal results are to be obtained.The success of the induction depends upon several factors, one of the most important of which is the preoperative handling of the child. The first objective is the elimination of fear. Intelligent parents can help in this by instilling confidence in their children, and convincing them that the doctors and nurses are going to help them. Much of this reassurance can be given at home before departure for the hospital. The hospital routine should be planned to avoid frightening the . . .