tbuckley/cabot-search huggingface dataset:
Factor VIII brand and the incidence of factor VIII inhibitors in previously untreated UK children with severe hemophilia A, 2000-2011
Authors: Peter W. Collins, Benedict P. Palmer, Elizabeth A. Chalmers, Daniel P. Hart, Ri Liesner, Savita Rangarajan, Katherine Talks, Michael Williams, Charles R. M. Hay
Journal: Blood (2014)

The effect of recombinant factor VIII (rFVIII) brand on inhibitor development was investigated in all 407 severe hemophilia A previously untreated patients born in the United Kingdom (UK) between 1 January 2000 and 31 December 2011. Eighty-eight (22%) had been in the RODIN study. Information was extracted from the National Haemophilia Database. Because exposure days (EDs) were not known for some patients, time from first treatment was used as a surrogate for rFVIII exposure. An inhibitor developed in 118 (29%) patients, 60 high and 58 low titer, after a median (interquartile range) of 7.8 (3.3-13.5) months from first exposure and 16 (9-30) EDs. Of 128 patients treated with Kogenate Bayer/Helixate NexGen, 45 (35.2%, 95% confidence interval [CI] 27.4-43.8) developed an inhibitor compared with 42/172 (24.4%, 95% CI 18.6% to 31.4%) with Advate (P = .04). The adjusted hazard ratio (HR) (95% CI) for Kogenate Bayer/Helixate NexGen compared with Advate was 2.14 (1.12-4.10) (P = .02) for high titer and 1.75 (1.11-2.76) (P = .02) for all inhibitors. When excluding UK-RODIN patients, the adjusted HR (95% CI) for high-titer inhibitors was 2.00 (0.93-4.34) (P = .08). ReFacto AF was associated with a higher incidence of all, but not high-titer, inhibitors than Advate. These results will help inform debate around the relative immunogenicity and use of rFVIII brands.
The Myth of Otomycosis
Authors: Joseph J. Littell
Journal: JAMA (1965)

<h3>To the Editor:—</h3> I believe Dr. Kingery is "myth"staken in seeming to exclude otomycosis as an entity. It is my belief that he is failing to recognize mycotic involvement of the ear canal because he feels that the dermatitis on the external part, or pinna, is not due to mold. I, as an otologist, am in agreement with him here. Mold infections are located in the ear canal where it is warm and moist; these conditions are not found on the pinna. When the black mold, or Aspergillus niger, is found in the canal, and it is readily seen, it produces a burning, itching, most irritating set of symptoms. Surely, this can be called otomycosis. It promptly clears with an antifungal preparation (Salicresin, basically 2% salicylic acid in alcohol). This is not so with the more external dermatitis, well shown in his photographs. These latter, I refer to a dermatologist.
Combined factors V and VIII deficiency climbs onto the map.
Authors: J E Sadler
Journal: Journal of Clinical Investigation (1997)

Combined Factors V and VIII Deficiency Climbs onto the Map EditorialInherited bleeding diseases have lit the path to our current understanding of hemostasis.An affected patient usually has a defect in one gene that causes deficiency of one blood clotting factor, simultaneously proving the existence of the factor and demonstrating its biological importance.Combined Factors V and VIII deficiency deviates from this pattern because a single autosomal recessive defect reduces the blood levels of two proteins encoded by unlinked genes on chromosomes 1 (Factor V) and X (Factor VIII).This rare disorder has substantial theoretical importance because it exposes an uncharacterized metabolic connection between these critical factors.In this issue of The Journal , Nichols et al. ( 1) make progress toward understanding this connection by mapping combined Factors V and VIII deficiency to the long arm of chromosome 18.Combined Factors V and VIII deficiency was recognized first in 1954, when Oeri et al. ( 2) studied two young brothers, born of a consanguineous mating, with bruising, nosebleeds, gum bleeding, and intramuscular hematomas; both had decreased plasma Factor V and Factor VIII.Since then, at least 91 homozygous patients belonging to 60 unrelated families have been reported (3).About 40% of the families live in the Mediterranean basin, and consanguinity is present in twothirds.The populations with the highest disease frequency are Oriental and Sephardic Jews in Israel.Almost all heterozygous carriers are asymptomatic and have normal levels of Factor V and Factor VIII.Affected homozygotes commonly have excessive bleeding after surgical procedures, deliveries, and menorrhagia.They have approximately equal decreases of Factors V and VIII, usually 5-25% of normal, and the residual Factor V and Factor VIII molecules appear to be normal (3).Many mechanisms have been proposed to explain this mysterious dual deficiency.The most intriguing recent model was based on the observation that four unrelated patients with combined Factors V and VIII deficiency had no protein C inhibitor (PCI) in their blood (4).A little background explains the appeal of this finding.Protein C is a protease zymogen that is cleaved and activated by thrombin.Activated protein C degrades Factors V and VIII, thereby acting as a physiologic anticoagulant.Activated protein C is neutralized by PCI, so that absence of PCI could result in unopposed destruction of Factors V and VIII, causing deficiency of both.Unfortunately, as Thomas Huxley said of another attractive scenario, "the great tragedy of Science-the slaying of a beautiful hypothesis by an ugly fact-was played, almost immediately."The clearance of transfused Factor V and Factor VIII was found to be normal, and the endogenous Factor VIII released by a dose of 1-desamino-[8d -arginine]-vasopressin (DDAVP) also had normal survival; thus, accelerated catabolism of Factors V and VIII is not the problem.Other patients were shown to have normal
Discontinuance of Routine Smallpox Vaccination
Authors: William H. Foege
Journal: JAMA (1972)

<h3>To the Editor.—</h3> The immunity status of the US population by age and geographic area is well known from previous immunization surveys. However, a study of European importations and control efforts in west Africa have shown that population immunity is not the single most important factor in determining whether an importation spreads widely. Instead, the important factors appear to be the specific population immunity in the hospital admitting the patient, the speed with which smallpox is recognized, and appropriate control procedures. The administration of attenuated smallpox vaccine or killed vaccine prior to smallpox vaccination cannot be recommended at this time. While it is known that attenuated vaccines can reduce fever and local reactions at the time of vaccination, information is not available regarding life-threatening complication rates with attenuated vaccine. Also, there is no information concerning the protective effect of attenuated vaccine alone or followed by calf lymph vaccine against smallpox
Ketoacidosis Associated with Alcoholism in Nondiabetic Subjects
Authors: LEONARD J. LEVY
Journal: Annals of Internal Medicine (1973)

Six episodes of metabolic acidosis in five nondiabetic, chronically alcoholic patients have been documented. All patients had a history of chronic alcoholism and appreciable alcohol intake before admission. Protracted vomiting and prolonged abstention from food were common features. The acidosis resulted from the accumulation of β-hydroxybutyrate, acetoacetate, and lactate in the blood plasma. The acidotic state was promptly corrected with intravenous fluids containing glucose, NaCl, and modest amounts of NaHCO3. Analysis of the plasma at admission showed low concentrations of insulin and extremely elevated free fatty acid and cortisol concentrations. It is postulated that increased ketone body synthesis in these patients is a direct consequence of enhanced release of free fatty acids from adipose tissue stores. Exaggerated lipolysis could be secondary to low levels of circulating insulin, to increased concentrations of plasma cortisol and growth hormone, or to the response of other lipolytic hormones.
The role of heme oxygenase 1 in rapamycin-induced renal dysfunction after ischemia and reperfusion injury
Authors: G.M. Gonçalves, M.A. Cenedeze, C.Q. Feitoza, P.M.H. Wang, A.P.F. Bertocchi, M.J. Damião, H.S. Pinheiro, V.P. Antunes Teixeira, M.A. dos Reis, A. Pacheco-Silva, N.O.S. Câmara
Journal: Kidney International (2006)
Ferroportin1 in hepatocytes and macrophages is required for the efficient mobilization of body iron stores in mice
Authors: Zhuzhen Zhang, Fan Zhang, Xin Guo, Peng An, Yunlong Tao, Fudi Wang
Journal: Hepatology (2012)

The liver is a major site of iron storage where sequestered iron can be actively mobilized for utilization when needed elsewhere in the body. Currently, hepatocyte iron efflux mechanisms and their relationships to macrophage iron recycling during the control of whole-body iron homeostasis are unclear. We hypothesized that the iron exporter, ferroportin1 (Fpn1), is critical for both iron mobilization from hepatocytes and iron recycling from macrophages. To test this, we generated hepatocyte-specific Fpn1 deletion mice ( Fpn1 Alb/Al ) and mice that lacked Fpn1 in both hepatocytes and macrophages ( Fpn1 Alb/Alb;LysM/LysM ). When fed a standard diet, Fpn1 Alb/Alb mice showed mild hepatocyte iron retention. However, red blood cell (RBC) counts and hemoglobin (Hb) levels were normal, indicating intact erythropoiesis. When fed an iron-deficient diet, Fpn1 Alb/Alb mice showed impaired liver iron mobilization and anemia, with much lower RBC and Hb levels than Fpn1 flox/flox mice on the same diet. Using a strategy where mice were preloaded with differing amounts of dietary iron before iron deprivation, we determined that erythropoiesis in Fpn1 Alb/Alb and Fpn1 flox/flox mice depended on the balance between storage iron and iron demands. On a standard diet, Fpn1 Alb/Alb;LysM/LysM mice displayed substantial iron retention in hepatocytes and macrophages, yet maintained intact erythropoiesis, implying a compensatory role for intestinal iron absorption. In contrast, when Fpn1 Alb/Alb;LysM/LysM mice were fed an iron-deficient diet, they developed severe iron-deficiency anemia, regardless of their iron storage status. Thus, Fpn1 is critical for both hepatocyte iron mobilization and macrophage iron recycling during conditions of dietary iron deficiency. Conclusion : Our data reveal new insights into the relationships between Fpn1-mediated iron mobilization, iron storage, and intestinal iron absorption and how these processes interact to maintain systemic iron homeostasis.
Homologous Serum Jaundice in Infancy
Authors: J. Apley, H. R. E. Wallis
Journal: BMJ (1948)

hospital stores all remaining bottles.We are reminded, of course, of the great importance of clearly and accurately iiecording in the patient's notes the batch numbers of all transfusion fluids given, for without a record of these numbers it is not possible to detect and withdraw from circulation the infected batches.
Regulatory T cells control the CD8 adaptive immune response at the time of ductal obstruction in experimental biliary atresia
Authors: Celine S. Lages, Julia Simmons, Claire A. Chougnet, Alexander G. Miethke
Journal: Hepatology (2012)

CD8 T-lymphocytes are effector cells of cholangiocyte injury in human and in rhesus rotavirus (RRV)-induced experimental biliary atresia (BA). Here we hypothesize that neonatal deficiency in CD25 + CD4 + regulatory T cells (Tregs) leads to aberrant activation of hepatic T-lymphocytes in BA. We found that adoptive transfer of total CD4 cells, but not of CD25-depleted CD4 cells, prior to RRV inoculation reduced expansion of CD8 cells, plasma bilirubin levels, ductal inflammation, and bile duct epithelial injury at 7 days postinfection (dpi) compared with age-matched infected controls without adoptive transfer. Searching for mechanisms, we found that in vitro production of interferon-gamma (IFN-γ) by naïve CD8 cells upon polyclonal stimulation was enhanced in coculture with hepatic dendritic cells (DCs) from RRV-infected, but not with DCs from noninfected mice, which was correlated with an increased proportion of CD11b + myeloid (m)DCs and up-regulation of the costimulatory molecule CD86 on RRV-primed DCs. Furthermore, DC-dependent T-lymphocyte activation was blocked by anti-CD86 antibody in dose-dependent fashion. Importantly, expression of CD86 on mDCs was down-regulated by Tregs in vitro , and adoptive transfer of Treg-containing CD4 cells decreased expression of CD86 on hepatic mDCs at 7 dpi. On the contrary, in mice resistant to experimental BA, CD25+ cell depletion aggravated bile duct injury at 12 dpi after RRV inoculation, as plasma bilirubin levels were elevated by &gt;20-fold compared with nondepleted infected controls. Increased susceptibility to hepatobiliary injury in Treg-depleted mice was linked to hepatic CD8 expansion and enhanced stimulatory capacity of hepatic DCs. Conclusion : Activation of hepatic T-lymphocytes driving biliary obstruction in BA is regulated by mDCs by way of CD86-dependent costimulation and is susceptible to inhibition by Tregs. (HEPATOLOGY 2012;56:219–227)
Evaluating the Changing Role of Respiratory Therapy Services at Two Hospitals
Authors: Sheldon R. Braun
Journal: JAMA (1981)

An analysis of respiratory therapy services at two general hospitals showed a notable change from 1971 to 1979. There was a large decrease in intermittent positive-pressure breathing (IPPB) treatments at both hospitals. These were partially replaced with general chest physiotherapy maneuvers at one hospital and with incentive spirometry at another. Both hospitals demonstrated a substantial increase in time spent in management of mechanical ventilators. Staffs had increased along with time spent per patient. The reduction in IPPB was not associated with lower costs of respiratory therapy services at either hospital but, rather, a shift in the types of service performed. These changes must be considered in determining cost-effectiveness of respiratory therapy services. (<i>JAMA</i>1981;245:2033-2037)
The Resource-Based Relative Value Scale
Authors: Karl A. Poterack
Journal: JAMA (1989)

To the Editor.—  The results of the Resource-Based Relative Value Scale study provide some thought-provoking comparisons. 1 Table 2 in that article reveals that both carpal tunnel surgery (427 resource-based relative values) and a vasectomy (472 resource-based relative values) are considered to be of significantly more value than the resuscitation of the high-risk neonate in the delivery room (276 resource-based relative values). Cervicocerebral angiography (621 resource-based relative values) is more than twice as valuable as neonatal resuscitation.  Without in any way minimizing the skill, time, or risk that my colleagues invest in any of these procedures, I ask: Valuable to whom? To the patients who benefit from these services? To the physicians who perform them? Or to the government that pays for them?—a truly frightening, albeit most likely, answer. It would be interesting to see this concept applied to other areas of government expenditure, for instance, welfare and social security
Coronary zero flow pressure and intramyocardial pressure in transiently arrested heart
Authors: S. Satoh, Y. Maruyama, J. Watanabe, M. Keitoku, K. Hangai, T. Takishima
Journal: Cardiovascular Research (1990)

Journal Article Coronary zero flow pressure and intramyocardial pressure in transiently arrested heart Get access Shoichi Satoh, Shoichi Satoh First Department of Internal Medicine, Tohoku University School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Yukio Maruyama, Yukio Maruyama First Department of Internal Medicine, Tohoku University School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Jun Watanabe, Jun Watanabe First Department of Internal Medicine, Tohoku University School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Mitsumasa Keitoku, Mitsumasa Keitoku First Department of Internal Medicine, Tohoku University School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Katsuyuki Hangai, Katsuyuki Hangai First Department of Internal Medicine, Tohoku University School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Tamotsu Takishima Tamotsu Takishima First Department of Internal Medicine, Tohoku University School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Cardiovascular Research, Volume 24, Issue 5, May 1990, Pages 358–363, https://doi.org/10.1093/cvr/24.5.358 Published: 01 May 1990 Article history Received: 31 July 1989 Accepted: 18 December 1989 Published: 01 May 1990
Predictive Value of Pulmonary Function Parameters for Sleep Apnea Syndrome
Authors: FRANÇOISE ZERAH-LANCNER, FREDERIC LOFASO, MARIE PIA D'ORTHO, CHRISTOPHE DELCLAUX, FRANÇOISE GOLDENBERG, ANDRÉ COSTE, BRUNO HOUSSET, ALAIN HARF
Journal: American Journal of Respiratory and Critical Care Medicine (2000)

Nocturnal polysomnography is the standard diagnostic test for sleep apnea syndrome (SAS) but is both expensive and time-consuming. We developed a predictive index for SAS based on pulmonary function data, including respiratory resistance determined by the forced oscillation technique, from 168 obese snorers with suspected SAS. Our model used logistic regression to obtain case-by-case predictions of the probability of SAS, defined as an apnea– hypopnea index (AHI) ⩾ 15 during overnight polysomnography. We then tested our model in a prospective group of 101 similar patients. Specific respiratory conductance and daytime oxygen saturation contributed significantly to the model. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of the index computed from these parameters were 98%, 86%, 90%, and 97%, respectively. In the prospective group, the model proved repeatable, with 100% sensitivity, 84% specificity, 86% PPV, and 100% NPV. The high NPV may help to identify obese snorers with a SAS risk that is so low as to make polysomnography unnecessary. Based on the 50% prevalence of SAS in our study and on the fact that polysomnography is required in all patients with daytime somnolence, we calculated that using our model would have obviated the need for polysomnography in 38% of our patients.
Personality Disorder Comorbidity with Major Depression and Response to Fluoxetine Treatment
Authors: Maurizio Fava, Evan Bouffides, Joel A. Pava, Mary K. McCarthy, Ronald J. Steingard, Jerrold F. Rosenbaum
Journal: Psychotherapy and Psychosomatics (1994)

The relationship between depression and comorbid personality disorders is still poorly understood. The aims of this study were to examine differences in depression severity between depressed outpatients with and without comorbid personality disorders, to determine the effect of a fixed dose of fluoxetine on personality disorders, and to assess the predictive value of personality disorder diagnoses at baseline with regard to response to fluoxetine. Eighty-three outpatients with major depressive disorder (MDD) were assessed with a self-rating scale, the Personality Diagnostic Questionnaire-Revised (PDQ-R), before and after 8 weeks of treatment with fluoxetine 20 mg/day. The presence of a cluster B diagnosis before treatment predicted positive outcome as measured by the change in score on the modified 17-item Hamilton Rating Scale for Depression (HAM-D-17*). Following treatment, we found significant reductions in the frequency of most individual personality disorder diagnoses and total PDQ-R score. While patients no longer meeting criteria for cluster B personality disorders after treatment had similar reductions in depressive symptoms compared to those maintaining the diagnoses, subjects no longer meeting criteria for cluster A and cluster C diagnoses after treatment exhibited significantly greater decreases in depression severity than those who maintained the diagnoses. Overall, these results suggest that fluoxetine may be beneficial in the treatment of certain personality disorder traits in patients with MDD.
Inhibition Of Human IgE-Allergen Interaction By Antibodies Of Rabbits Immunized With Depigmented-polymerized Allergen Extracts
Authors: V. Iraola, M.T. Gallego, T. Aranda, M.A. Lopez-Matas, M. Morales, R. Saez, C. Marquez, J.R. Leonor, J. Carnes
Journal: Journal of Allergy and Clinical Immunology (2012)
Role of cysteinyl-leukotrienes and histamine in mediating intrinsic tone in isolated human bronchi.
Authors: J L Ellis, B J Undem
Journal: American Journal of Respiratory and Critical Care Medicine (1994)

The intrinsic isometric tone of human isolated intralobar bronchi was evaluated in vitro. Human bronchi (inner diameter, 3 to 12 mm) were obtained from patients undergoing lung resection and from organ donors. The organ donor tissue was studied approximately 24 h after resection, during which time it was shipped to the laboratory in RPMI 1640 medium at 4 degrees C. The bronchi obtained from patients with cancer was placed in the same medium and studied within 4 h of resection. All tissues were suspended in 10-ml organ baths containing oxygenated Krebs' solution at 37 degrees C, at an initial load of 2 g. Tissues were washed every 15 min until they exhibited a stable tension (usually 60 min). Under these conditions the amount of active tension in the bronchi studied on the day of resection averaged 65 +/- 9% of the maximal response to BaCl2 (30 mM), whereas after overnight incubation it averaged 31 +/- 6%. Neither indomethacin nor atropine influenced the intrinsic tone in these tissues. By contrast, the LTD4 antagonists SKF 104353 and ICI 198615 and the histamine H-1 antagonists pyrilamine and chlorpheniramine effectively relaxed the bronchi. The 5' lipoxygenase inhibitor zileuton also produced a small fall in tension. Studies in which pyrilamine was combined with the leukotriene receptor antagonists indicated an additive effect. These results indicate that human bronchial smooth muscle exhibits a high degree of intrinsic tone, averaging more than 50% of available tone in fresh tissues studied in vitro under isometric conditions. The results suggest that this tone is due to the continual production and release of histamine and cysteinyl-leukotrienes.