tbuckley/cabot-search huggingface dataset:
Trichothiodystrophy and fragile hair: the distinction between diagnostic signs and diagnostic labels in childhood hair disease
Authors: S. Cheng, J. Stone, D. de Berker
Journal: British Journal of Dermatology (2009)

Hair typically becomes fragile when there are structural abnormalities and/or a reduction in the sulphur-containing amino acids cystine or methionine. This finding in the setting of a neuroectodermal complaint is usually labelled trichothiodystrophy (TTD). The spectrum of features within this diagnostic grouping tests the validity of using sulphur-deficient hair as a central characteristic.To determine what diagnoses were found within a group of subjects with fragile hair and whether low cystine or methionine were relevant central characteristics.We examined cases referred to us from 12 U.K. centres for hair microscopy over 10 years where hair fragility or clinical characteristics raised the possibility of TTD. All samples underwent amino acid analysis. This was achieved through cation exchange chromatography coupled with spectrophotometric quantification.Twenty-five patients (11 male, 14 female) with a mean age of 11 years (0.3-37) were evaluated. Nineteen patients had features of hair damage. Of these, five patients had abnormalities on microscopy only and four patients had microscopic changes and tiger-tail pattern but normal amino acid content. The remaining 10 patients had reduced cystine content, two of whom also had low methionine. All but one had the tiger-tail pattern. Among the wide range of phenotypes there were only three cases matching a diagnosis of TTD.Our data suggest that clinically apparent fragile hair in childhood is only rarely associated with a diagnosis of TTD. The tiger-tail change is sensitive but not wholly specific to TTD. We propose that the term trichothiodystrophy be limited in its use to define sulphur-deficient hair rather than as a diagnostic term in a heterogeneous and incoherent multisystem disorder, where sulphur-deficient hair is one feature.
Autophagy Is Important in Islet Homeostasis and Compensatory Increase of Beta Cell Mass in Response to High-Fat Diet
Authors: Chie Ebato, Toyoyoshi Uchida, Masayuki Arakawa, Masaaki Komatsu, Takashi Ueno, Koji Komiya, Kosuke Azuma, Takahisa Hirose, Keiji Tanaka, Eiki Kominami, Ryuzo Kawamori, Yoshio Fujitani, Hirotaka Watada
Journal: Cell Metabolism (2008)
The Interactions of Peptides with the Innate Immune System Studied with Use of T7 Phage Peptide Display
Authors: Alex V. Sokoloff, Guofeng Zhang, Magdolna G. Sebestyén, Jon A. Wolff, Ildiko Bock
Journal: Molecular Therapy (2000)

The icosahedral T7 phage (diameter ∼65 nm) displaying random peptides at the carboxy-terminus of the phage coat proteins was used as a model for drug and gene delivery vehicles containing peptide ligands. We found that displayed peptides were recognized by natural antibodies and induced complement activation. Strikingly, the phage inactivation by complement was peptide-specific that implied the existence of numerous natural antibodies with different peptide specificity. Selection of phage that avoided inactivation by complement allowed the identification of peptides that protected the phage by binding to serum proteins. In rat blood, peptides with carboxy-terminal lysine or arginine residues protected the phage against complement-mediated inactivation by binding C-reactive protein. In human serum, a number of protective peptides with tyrosine residues were selected. The recognition of displayed peptides by natural antibodies appears to represent a universal mechanism for activation of complement at sites that contain identical or homologous proteins with exposed carboxy-termini.
Isolation of Mycoplasma hominis From the Blood of Men With Multiple Trauma and Fever
Authors: T. Y. Ti
Journal: JAMA (1982)

<i>MYCOPLASMA hominis</i>is a common inhabitant of the human genitourinary and upper respiratory tracts.<sup>1,2</sup>Its full role in the pathogenesis of disease in man is unclear. Infections associated with<i>M hominis</i>have been reported mainly in postpartum women<sup>1</sup>and neonates.<sup>2</sup>Isolation of the organism from the bloodstream following vaginal delivery or abortion is well documented<sup>1</sup>; however, its recovery from the blood of adult men has been described in only a single report.<sup>3</sup>We present two patients from whom<i>M hominis</i>was isolated in blood cultures following multiple trauma. One of these also had septic arthritis. <h3>Report of Cases</h3><h3>Case 1.—</h3> A 26-year-old man, found unconscious following a motor vehicle accident, was admitted with multiple lacerations and fracture of the left femur and fracture dislocation at C2-C3. Following initial supportive therapy he regained consciousness. The hospital course was characterized by intermittent fever spikes (temperatures of 39
Computed tomography and cystic fibrosis: promises and problems
Authors: Z A Aziz, J C Davies, E W Alton, A U Wells, D M Geddes, D M Hansell
Journal: Thorax (2007)

Computed tomography (CT) has two potential roles in the evaluation of patients with cystic fibrosis (CF) lung disease: as a diagnostic test primarily for the detection of supervening complications and as a monitoring tool in clinical research. Interest in the latter role has gained momentum in the last 5 years because of two factors: (1) therapeutic options for CF lung disease are developing rapidly, hence the need for an outcome measure that can be applied in clinical intervention trials; and (2) it has become clear that traditional outcome measures such as pulmonary function tests are relatively insensitive to the early structural damage that occurs in CF. Several recent studies have shown that CT can be used as a potential surrogate outcome measure, although its suitability for this specific role is controversial and still under investigation. This review summarises current concepts relating to the research applications of CT in CF, with particular emphasis on the evidence supporting the use of CT as a surrogate outcome measure in clinical trials.
Plasma acetate turnover and oxidation.
Authors: C L Skutches, C P Holroyde, R N Myers, P Paul, G A Reichard
Journal: Journal of Clinical Investigation (1979)

Plasma acetate turnover and oxidation were determined in 11 healthy subjects by the constant infusion of a trace amount of [1-14C]acetate for 6 h. The subjects ages ranged from 22 to 57 yr. There was a positive correlation (P less than 0.001) between plasma acetate concentration and turnover rate, and a negative correlation (P less than 0.001) between turnover and age. The plasma acetate concentration in the subjects 22--28 yr old was 0.17 vs. 0.13 mM (P less than 0.02) in subjects 40--57 yr old. The plasma acetate turnover rate was also greater in the younger age group (8.23 +/- 0.66 vs. 4.98 +/- 0.64 mumol/min . kg, P less than 0.01). Approximately 90% of the plasma acetate turnover was immediately oxidized to CO2 in both age groups, however, 13.2 +/- 0.89% of the CO2 output in the younger group was derived from plasma acetate oxidation compared to 7.9 +/- 0.94% in the older group (P less than 0.01). The mean plasma acetate concentration, turnover, and oxidation in six cancer patients 47--63 yr old were similar to the values observed in the age-matched healthy subjects. Uptake or output of acetate by various tissues was measured by arterial-venous plasma acetate concentration differences. In seven of eight subjects undergoing elective surgery, the arterial-portal venous concentration difference was negative, which indicated that the gastrointestinal tract can contribute to plasma acetate production. Uptake of plasma acetate by both the leg and liver appeared to be dictated by the arterial acetate concentration. Net production of acetate by both the leg and liver was most often observed at arterial plasma acetate concentrations less than 0.08 mM.
Cellular Reactivity Studies to Streptococcal Antigens MIGRATION INHIBITION STUDIES IN PATIENTS WITH STREPTOCOCCAL INFECTIONS AND REHEUMATIC FEVER
Authors: Stanley E. Read, Vincent A. Fischetti, Virginia Utermohlen, Rudolf E. Falk, John B. Zabriskie
Journal: Journal of Clinical Investigation (1974)

The question of whether hypersensitivity to streptococcal antigens plays a role in the pathogenesis of the nonsuppurative sequelae of streptococcal infections remains at present unclear. As a first step in the approach to this question, the degree of cellular reactivity of peripheral blood leucocytes to streptococcal antigens was investigated in a number of rheumatic fever patients, patients with uncomplicated streptococcal infections, as well as normal healthy subjects.
What About the Future?
Authors: Albert H. Schwichtenberg
Journal: JAMA (1965)

Most physicians tend to think of computers in medicine as having a "far out into the wide blue yonder" aspect. To some this is stimulating; however, many tend to resent things which are hard to understand and the normal reaction is one of conservatism. The purpose of this symposium has been to provide a basic understanding of computers, what they are and how they work. As understanding and experience improve, physicians can move from conservatism to active use of these splendid instruments in the practice of medicine. <h3>Computers in Medicine</h3> Computers are not monsters; they have no black magic; they do not "think." It should be apparent that a computer is in fact incredibly stupid, doing only what it has been directed to do. However, when properly directed or programmed, a computer can carry out, in seconds or less and without errors or getting tired, extremely difficult computational tasks which
Primary Sellar Neuroblastoma Presenting with Syndrome of Inappropriate Secretion of Anti-diuretic Hormone
Authors: Bishan Radotra, Vasileios Apostolopoulos, Ann Sandison, Emma C. I. Hatfield, Nigel Mendoza, Jill Moss, Amrish Mehta, Mark Glaser, Karim Meeran, Federico Roncaroli
Journal: Endocrine Pathology (2010)
Prevalence of gall stones at necropsy in nine British towns: a collaborative study.
Authors: D J Barker, M J Gardner, C Power, M S Hutt
Journal: BMJ (1979)

A prospective study of the prevalence of gall stones at necropsy in nine towns in England and Wales showed considerable geographical variations. The age- and sex-standardised prevalence ranged from 20.6% in Ipswich to 9.2% in Wakefield. The distribution of gall stones differed from that of all-cause mortality and was negatively correlated with that of mortality from ischaemic heart disease. Socioeconomic influences related to affluence do not appear to be major determinants of the distribution of gall stones.
FURTHER STUDIES ON THE ULTRASTRUCTURE OF DIMERIC I<scp>G</scp>A OF HUMAN ORIGIN
Authors: Björn Bloth, Sven-Eric Svehag
Journal: The Journal of Experimental Medicine (1971)

Human colostral IgA and myeloma dimer IgA were purified and examined in the electron microscope using a modified technique of negative staining. Both types of preparation contained double Y-shaped structures of the dimensions: Fab region, 35 x 70 A, and the sum of the two Fc regions, 40 x 140–155 A. Colostral IgA as well as myeloma dimer IgA molecules showed a tendency of bending at the point where the Fc regions joined. Secretory component bound to dimer IgA produced no visible alteration of the molecule. Mild reduction and alkylation of colostral IgA yielded single Y-shaped 7S monomers with the dimensions, Fab, 35 x 70 A, and Fc, 40 x 65–70 A.
Effect of Reminders of Personal Sacrifice and Suggested Rationalizations on Residents' Self-Reported Willingness to Accept Gifts
Authors: Sunita Sah, George Loewenstein
Journal: JAMA (2010)

Despite expanding research on the prevalence and consequences of conflicts of interest in medicine, little attention has been given to the psychological processes that enable physicians to rationalize the acceptance of gifts.To determine whether reminding resident physicians of the sacrifices made to obtain training, as well as suggesting this as a potential rationalization, increases self-stated willingness to accept gifts from industry.Three hundred one US resident physicians from 2 sample populations (pediatrics and family medicine) who were recruited during March-July 2009 participated in a survey presented as evaluating quality of life and values.Physicians were randomly assigned to receive 1 of 3 different online surveys. The sacrifice reminders survey (n = 120) asked questions about sacrifices made in medical training, followed by questions regarding the acceptability of receiving gifts from industry. The suggested rationalization survey (n = 121) presented the same sacrifice questions, followed by a suggested possible rationalization (based on sacrifices made in medical training) for acceptance of gifts, before the questions regarding the acceptability of gifts. The control survey (n = 60) asked about the acceptability of gifts before asking questions about sacrifices or suggesting a rationalization.Physician self-stated acceptability of receiving gifts from industry.Reminding physicians of sacrifices made in obtaining their education resulted in gifts being evaluated as more acceptable: 21.7% (13/60) in the control group vs 47.5% (57/120) in the sacrifice reminders group (odds ratio, 1.81; 95% confidence interval, 1.27-2.58; P = .001). Although most residents disagreed with the suggested rationalization, exposure to it further increased the perceived acceptability of gifts to 60.3% (73/121) in that group (odds ratio relative to sacrifice reminders group, 1.45; 95% confidence interval, 1.22-1.72; P < .001).Providing resident physicians with reminders of sacrifices increased the perceived acceptability of industry-sponsored gifts. Including a rationalization statement further increased gift acceptability.
Substance P induces intracellular calcium increase and translocation of protein kinase C in epidermis
Authors: H. KOIZUMI, H. TANAKA, T. FUKAYA, A. OHKAWARA
Journal: British Journal of Dermatology (1992)

Substance P is a neuropeptide present in, and released from, peripheral C nerve endings. The presence of substance P-positive nerve fibres in the epidermis has been reported. We investigated the effect of substance P on the transmembrane signalling system of pig epidermal keratinocytes. Treatment of pig epidermis with substance P resulted in an increase in inositol 1,4,5-trisphosphate (IP3), and in intracellular free calcium. The treatment also resulted in translocation of protein kinase C from a cytosol to a membrane fraction. Substance P, however, did not affect the beta-adrenergic- or histamine (H2)- adenylate cyclase responses of the epidermis. Neither forskolin-induced, nor cholera toxin-induced cyclic AMP accumulation were affected by substance P treatment. These results consistent with the view that substance P stimulates phosphatidylinositol-4,5-bisphosphate (PIP2) hydrolysis of keratinocytes, resulting in IP3-Ca2+ and diacylglycerol-protein kinase C signal activation. Although protein kinase C is known to affect the epidermal adenylate cyclase system, no evidence for such 'cross-talk regulation' was detected in keratinocytes by substance P treatment.
Hypericum perforatum (St John's Wort) for Attention-Deficit/Hyperactivity Disorder in Children and Adolescents
Authors: Wendy Weber, Ann Vander Stoep, Rachelle L. McCarty, Noel S. Weiss, Joseph Biederman, Jon McClellan
Journal: JAMA (2008)

Stimulant medication can effectively treat 60% to 70% of youth with attention-deficit/hyperactivity disorder (ADHD). Yet many parents seek alternative therapies, and Hypericum perforatum (St John's wort) is 1 of the top 3 botanicals used.To determine the efficacy and safety of H. perforatum for the treatment of ADHD in children.Randomized, double-blind, placebo-controlled trial conducted between March 2005 and August 2006 at Bastyr University, Kenmore, Washington, among a volunteer sample of 54 children aged 6 to 17 years who met Diagnostic and Statistical Manual of Mental Disorders (Fourth Edition) criteria for ADHD by structured interview.After a placebo run-in phase of 1 week, participants were randomly assigned to receive 300 mg of H. perforatum standardized to 0.3% hypericin (n = 27) or a matched placebo (n = 27) 3 times daily for 8 weeks. Other medications for ADHD were not allowed during the trial.Performance on the ADHD Rating Scale-IV (range, 0-54) and Clinical Global Impression Improvement Scale (range, 0-7), and adverse events.One patient in the placebo group withdrew because of an adverse event. No significant difference was found in the change in ADHD Rating Scale-IV scores from baseline to week 8 between the treatment and placebo groups: inattentiveness improved 2.6 points (95% confidence interval [CI], -4.6 to -0.6 points) with H. perforatum vs 3.2 points (95% CI, -5.7 to -0.8 points) with placebo (P = .68) and hyperactivity improved 1.8 points (95% CI, -3.7 to 0.1 points) with H. perforatum vs 2.0 points (95% CI, -4.1 to 0.1 points) with placebo (P = .89). There was also no significant difference between the 2 groups in the percentage of participants who met criteria for improvement (score < or = 2) on the Clinical Global Impression Improvement Scale (H. perforatum, 44.4%; 95% CI, 25.5%-64.7% vs placebo, 51.9%; 95% CI, 31.9%-71.3%; P = .59). No difference between groups was found in the number of participants who experienced adverse effects during the study period (H. perforatum, 40.7%; 95% CI, 22.4%-61.2% vs placebo, 44.4%; 95% CI, 25.5%-64.7%; P = .78).In this study, use of H. perforatum for treatment of ADHD over the course of 8 weeks did not improve symptoms.clinicaltrials.gov Identifier: NCT00100295.
People with learning disabilities are being let down by NHS
Authors: Zosia Kmietowicz
Journal: BMJ (2007)

Sweeping changes are needed in the organisation and ethos of the NHS’s dedicated inpatient facilities and care homes for people with learning disabilities, the health watchdog for England has said.

Care at NHS facilities for people with learning disabilities falls short of modern safety and quality standards, says the Healthcare Commission in a new report, and many people live in bleak accommodation far away from their families.

Institutional failings mean that many people are being deprived of their human rights and dignity and have little access to advocacy services, few choices about how they live their lives, and limited activities, the report says. Services are too reliant on drug treatment to …
1140-169 Heavy coronary plaque calcification is associated with only minor degrees of plaque macrophage and neovessel density than plaques with less calcification: Solving the calcium paradox?
Authors: Silvio Litovsky, Deborah Vela, Allen Burke, Mohammad Madjid, Morteza Naghavi, Saeed Payvar, Renu Virmani, Ward Casscells
Journal: Journal of the American College of Cardiology (2004)
Lymphocytic Hypophysitis with Isolated Corticotropin Deficiency
Authors: Michael D. Jensen, Barry S. Handwerger, Bernd W. Scheithauer, Paul C. Carpenter, Rita Mirakian, Peter M. Banks
Journal: Annals of Internal Medicine (1986)

In a 32-year-old woman, acquired, isolated corticotropin deficiency resulted from postpartum lymphocytic hypophysitis. The literature suggests that lymphocytic hypophysitis may cause acquired deficiencies of anterior, and possibly posterior, pituitary hormones. Immunologic evaluation of our patient failed to uncover anticorticotroph antibodies. Prompt recognition of this potentially fatal condition is important because of the availability of effective treatment.
Early biochemical response to ursodeoxycholic acid and long-term prognosis of primary biliary cirrhosis: Results of a 14-year cohort study
Authors: Li-Na Zhang, Tian-Yan Shi, Xu-Hua Shi, Li Wang, Yun-Jiao Yang, Bin Liu, Li-Xia Gao, Zong-Wen Shuai, Fang Kong, Hua Chen, Wei Han, Shao-Mei Han, Yun-Yun Fei, Quan-Cai Cui, Qian Wang, Min Shen, Dong Xu, Wen-Jie Zheng, Yong-Zhe Li, Wen Zhang, Xuan Zhang, Feng-Chun Zhang
Journal: Hepatology (2013)

Abstract The biochemical response to ursodeoxycholic acid (UDCA) in primary biliary cirrhosis is a strong predictor of long-term outcome and thus facilitates the rapid identification of patients needing new therapeutic approaches. Numerous criteria for predicting outcome of treatment have been studied based on biochemical response to UDCA at 1 year. We sought to determine whether an earlier biochemical response at 3 or 6 months could as efficiently identify patients at risk of poor outcome, as defined by liver-related death, liver transplantation, and complications of cirrhosis. We analyzed the prospectively collected data of 187 patients with a median follow-up of 5.8 years (range, 1.3-14 years). The survival rates without adverse outcome at 5 years and 10 years were 86% and 63%. Under UDCA therapy, laboratory liver parameters experienced the most prominent improvement in the first 3 months ( P &lt; 0.0001) and then stayed relatively stable for the following months. The Paris, Barcelona, Toronto, and Ehime definitions, but not the Rotterdam definition, applied at 3, 6, and 12 months significantly discriminated the patients in terms of long-term outcome. Compared with biochemical responses evaluated after 1 year of UDCA therapy, biochemical responses at the third month demonstrated higher positive predictive value (PPV) but lower negative predictive value (NPV) and increased negative likelihood ratio (NLR) by all definitions; biochemical responses at the sixth month showed higher or the same PPV and NPV and lower NLR by all definitions. Conclusion : For the previously published criteria, biochemical responses at the sixth month can be used in place of those evaluated after 1 year of UDCA therapy. Our findings justify a more rapid identification of patients who need new therapeutic approaches. (HEPATOLOGY 2013)
COCAINE; ITS USES IN GYNECOLOGY.Read in the Section of Obstetrics and Diseases of Women, at the Forty-fourth Annual Meeting of the American Medical Association.
Authors: WM. H HUMISTON
Journal: JAMA (1893)

Having now had five years' experience in the use of cocaine in gynecological operations, I feel better prepared to eulogize its merits now than I could do two years ago, when I first brought this subject to the notice of the profession at the Washington meeting. I stated at this time that the only annoying symptom that frequently arose was the shortness of breath, and the anxiety connected with it—this I have been able to obviate by the use of strychnia or tincture of nux vom.; one-thirtieth grain of the former, or fifteen minims of the latter, administered with one-half ounce of whisky thirty minutes before operation. I do the following operations with no other anæsthetic than cocaine, viz.: dilating, curetting, trachelorraphy, anterior and posterior, colporraphy, and perineorrphay. Frequently making two of these operations at One sitting. In dilating and curetting primiparæ, I resort to cocaine in preference to chloroform
British cosmetic regulations inadequate.
Authors: C M Ridley, S J Adams, I Leigh
Journal: BMJ (1984)

established treatment with 3 blockers, although a high incidence of intolerance has been reported with primidone.We have encountered adverse reactions (nausea, vomiting, dizziness) in some patients after a single small dosefor example, 62-5 mg orally.The cause of this acute sensitivity (toxicity) is not clear.There is, however, evidence to indicate that this problem could be circumvented by prior exposure to phenobarbitone or other drugs to induce the liver enzymes involved in the metabolism of primidone.10In our experience tranquillising and sedative drugs can be useful in attenuating essential tremor.Their effectiveness is, however, proportional to the degree ofsedation produced.Alcohol, thouah strikingly effective in some patients, cannot be considered as realistic treatment.