tbuckley/cabot-search huggingface dataset:
Unusual molecular findings in Kindler syndrome
Authors: K. Arita, V. Wessagowit, A.C. Inamadar, A. Palit, H. Fassihi, J.E. Lai-Cheong, C. Pourreyron, A.P. South, J.A. McGrath
Journal: British Journal of Dermatology (2007)

Journal Article Unusual molecular findings in Kindler syndrome Get access K. Arita, K. Arita Genetic Skin Disease Group, St John's Institute of Dermatology, Division of Genetics and Molecular Medicine, The Guy's, King's College and St Thomas' School of Medicine, St Thomas' Hospital, Lambeth Palace Road, London SE1 7EH, U.K.Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan Correspondence: Ken Arita. E‐mail: ken.arita@kcl.ac.uk Search for other works by this author on: Oxford Academic Google Scholar V. Wessagowit, V. Wessagowit Genetic Skin Disease Group, St John's Institute of Dermatology, Division of Genetics and Molecular Medicine, The Guy's, King's College and St Thomas' School of Medicine, St Thomas' Hospital, Lambeth Palace Road, London SE1 7EH, U.K.Institute of Dermatology, Bangkok, Thailand Search for other works by this author on: Oxford Academic Google Scholar A.C. Inamadar, A.C. Inamadar Department of Dermatology, Venereology & Leprosy, BLDEA's SBMP Medical College, Hospital & Research Centre, Bijapur, Karnataka, India Search for other works by this author on: Oxford Academic Google Scholar A. Palit, A. Palit Department of Dermatology, Venereology & Leprosy, BLDEA's SBMP Medical College, Hospital & Research Centre, Bijapur, Karnataka, India Search for other works by this author on: Oxford Academic Google Scholar H. Fassihi, H. Fassihi Genetic Skin Disease Group, St John's Institute of Dermatology, Division of Genetics and Molecular Medicine, The Guy's, King's College and St Thomas' School of Medicine, St Thomas' Hospital, Lambeth Palace Road, London SE1 7EH, U.K. Search for other works by this author on: Oxford Academic Google Scholar J.E. Lai‐Cheong, J.E. Lai‐Cheong Genetic Skin Disease Group, St John's Institute of Dermatology, Division of Genetics and Molecular Medicine, The Guy's, King's College and St Thomas' School of Medicine, St Thomas' Hospital, Lambeth Palace Road, London SE1 7EH, U.K. Search for other works by this author on: Oxford Academic Google Scholar C. Pourreyron, C. Pourreyron Centre for Cutaneous Research, Bart's and The London, Queen Mary's School of Medicine and Dentistry, London, U.K. Search for other works by this author on: Oxford Academic Google Scholar A.P. South, A.P. South Centre for Cutaneous Research, Bart's and The London, Queen Mary's School of Medicine and Dentistry, London, U.K. Search for other works by this author on: Oxford Academic Google Scholar J.A. McGrath J.A. McGrath Genetic Skin Disease Group, St John's Institute of Dermatology, Division of Genetics and Molecular Medicine, The Guy's, King's College and St Thomas' School of Medicine, St Thomas' Hospital, Lambeth Palace Road, London SE1 7EH, U.K. Search for other works by this author on: Oxford Academic Google Scholar British Journal of Dermatology, Volume 157, Issue 6, 1 December 2007, Pages 1252–1256, https://doi.org/10.1111/j.1365-2133.2007.08159.x Published: 01 December 2007
Treatment of Diffuse Histiocytic Lymphoma
Authors: William P. Sheridan, Graeme N. Brodie
Journal: Annals of Internal Medicine (1985)

Letters and Corrections1 September 1985Treatment of Diffuse Histiocytic LymphomaWILLIAM P. SHERIDAN, F.R.A.C.P., GRAEME N. BRODIE, F.R.A.C.P., F.R.C.P.A.WILLIAM P. SHERIDAN, F.R.A.C.P.Search for more papers by this author, GRAEME N. BRODIE, F.R.A.C.P., F.R.C.P.A.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-103-3-471_2 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptTo the editor: Klimo and Connors (1) have achieved impressive results with their chemotherapy for diffuse histiocytic lymphoma in a group of 61 patients, with response rates being equally good in patients with and without adverse prognostic features. However, the only therapy-related death occurred in a patient with extensive gastric invasion by lymphoma, as a result of hemorrhage from an area of tumor necrosis. Thus, one third of the patients with gastrointestinal lymphoma in this study died.There are data to suggest that surgical resection is feasible in most patients with gastric lymphoma without compromising our ability to deliver effective...References1. KLIMOCONNORS PJ. MACOP-B chemotherapy for the treatment of diffuse large-cell lymphoma. Ann Intern Med. 1985;102:596-602. LinkGoogle Scholar2. SHERIDANMEDLEYBRODIE WGG. Non-Hodgkin's lymphoma of the stomach: a prospective pilot study of surgery plus chemotherapy in early and advanced disease. J Clin Oncol. 1985;3:495-500. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Prince Henry's Hospital Melbourne, Victoria Australia PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics 1 September 1985Volume 103, Issue 3Page: 471-472KeywordsChemotherapyDiffuse large B-cell lymphomaHemorrhageLymphomaNecrosisSurgical resection Issue Published: 1 September 1985 PDF downloadLoading ...
825 RESULTS OF A RANDOMIZED CONTROLLED TRIAL ON THE IMPACT OF PROLONGED COMBINATION ANTI-HCV TREATMENT IN HIV/HCV CO-INFECTED PATIENTS
Authors: M. Puoti, B. Zanini, A. De Luca, G.P. Quinzan, R. Allegri, R. Bruno, A. Orani, T. Quirino, T. Santantonio, G. Pastore, G. Cristini, F. Suter, R. Cauda, G. Carosi
Journal: Journal of Hepatology (2008)
Pure RBC Aplasia Associated With Chronic Granulocytic Leukemia
Authors: Mitsuo Kitahara
Journal: JAMA (1978)

PURE RBC aplasia is characterized by severe anemia, reticulocytopenia, and an almost complete absence of marrow erythroblasts. The bone marrow contains normally active granulopoietic and megakaryopoietic elements. Both congenital and acquired forms have been described—the latter in association with a variety of conditions and drugs.<sup>1</sup>Red blood cell aplasia has been listed as one form of metamorphosis that leads to terminal transformation in chronic granulocytic leukemia,<sup>2</sup>but to my knowledge, this rare association has not been reported in the literature. <h3>Report of a Case</h3> A 54-year-old woman was referred to the University of Utah Hematology Clinic in July 1973 because of splenomegaly, anemia, and leukocytosis. Physical examination showed hepatosplenomegaly. The hematocrit value was 32%; leukocyte count, 79,000/cu mm; and platelet count, 175,000/cu mm. Bone marrow examination showed myeloid hyperplasia, with a myeloid to erythroid ratio (M:E ratio) of 6:1. Leukocyte alkaline phosphatase score was 0. Philadelphia chromosome was
Doctor allegedly infected patients with hepatitis C
Authors: J. Siegel-Itzkovich
Journal: BMJ (2003)

Twenty five Israelis have been infected with hepatitis C–and hundreds more are being tested at Soroka University Medical Centre in Beersheba–allegedly from a 37 year old hospital doctor who was reportedly addicted to morphine.

Dr Sergei Pontus, who was training to be an anaesthesiologist, is suspected of injecting patients with the same syringes he used for himself. He was arrested by police and remanded …
Constitutive activation of Akt contributes to the pathogenesis and survival of mantle cell lymphoma
Authors: Martina Rudelius, Stefania Pittaluga, Satoshi Nishizuka, Trinh H.-T. Pham, Falko Fend, Elaine S. Jaffe, Leticia Quintanilla-Martinez, Mark Raffeld
Journal: Blood (2006)
Comparison of hospital episode statistics and central cardiac audit database in public reporting of congenital heart surgery mortality
Authors: Stephen Westaby, Nicholas Archer, Nicola Manning, Satish Adwani, Catherine Grebenik, Oliver Ormerod, Ravi Pillai, Neil Wilson
Journal: BMJ (2007)

<b>Objective</b> To verify or refute the value of hospital episode statistics (HES) in determining 30 day mortality after open congenital cardiac surgery in infants nationally in comparison with central cardiac audit database (CCAD) information. <b>Design</b> External review of paediatric cardiac surgical outcomes in England (HES) and all UK units (CCAD), as derived from each database. <b>Setting</b> Congenital heart surgery centres in the United Kingdom. <b>Data sources</b> HES for congenital heart surgery and corresponding information from CCAD for the period 1 April 2000 to 31 March 2002. HES was restricted to the 11 English centres; CCAD covered all 13 UK centres. <b>Main outcome measure</b> Mortality within 30 days of open heart surgery in infants aged under 12 months. <b>Results</b> In a direct comparison for the years when data from the 11 English centres were available from both databases, HES omitted between 5% and 38% of infants operated on in each centre. A median 40% (range 0-73%) shortfall occurred in identification of deaths by HES. As a result, mean 30 day mortality was underestimated at 4% by HES as compared with 8% for CCAD. In CCAD, between 1% and 23% of outcomes were missing in nine of 11 English centres used in the comparison (predominantly those for overseas patients). Accordingly, CCAD mortality could also be underestimated. Oxford provided the most complete dataset to HES, including all deaths recorded by CCAD. From three years of CCAD, Oxford9s infant mortality from open cardiac surgery (10%) was not statistically different from the mean for all 13 UK centres (8%), in marked contrast to the conclusions drawn from HES for two of those years. <b>Conclusions</b> Hospital episode statistics are unsatisfactory for the assessment of activity and outcomes in congenital heart surgery. The central cardiac audit database is more accurate and complete, but further work is needed to achieve fully comprehensive risk stratified mortality data. Given unresolved limitations in data quality, commercial organisations should reconsider placing centre specific or surgeon specific mortality data in the public domain.
Activation of the Endothelial Store-Operated <i>I</i> <sub>SOC</sub> Ca <sup>2+</sup> Channel Requires Interaction of Protein 4.1 With TRPC4
Authors: Donna L. Cioffi, Songwei Wu, Mikhail Alexeyev, Steven R. Goodman, Michael X. Zhu, Troy Stevens
Journal: Circulation Research (2005)

Store-operated calcium (SOC) entry represents the principal Ca 2+ entry pathway into nonexcitable cells. Despite intensive investigation, mechanisms underlying activation of SOC entry have remained elusive. The endothelial I SOC channel is a Ca 2+ -selective SOC entry channel to which the transient receptor potential (TRP) proteins TRPC1 and TRPC4 contribute subunits. Activation of I SOC is specifically regulated by the spectrin–actin membrane skeleton; however, the nature of coupling between the I SOC channel and membrane skeleton is unknown. Here we demonstrate that protein 4.1 is an essential component of the I SOC channel gating mechanism. Protein 4.1 interacts with TRPC4 and the membrane skeleton. Deletion of the protein 4.1 binding domain on TRPC4 or peptide competition to the protein 4.1 binding domain prevents I SOC activation. These findings reveal that interaction of protein 4.1 with TRPC4 is required for activation of the endothelial I SOC channel.
Pyoderma Gangrenosum, Peptic Ulcer, and a $625,000 Damage Award
Authors: Isadore Kaplan
Journal: JAMA (1964)

ONE OF THE highest damage awards for personal "injury" of a single person on record—$625,000-was assessed against the Baltimore and Ohio Railroad (B &amp; O) in a negligence suit brought by one of its employees. The bizarre relationship established by the judgment between an insect bite and a railroad's negligence, and the nature of the disease which became the ground for the award have kept alive an intense interest in the case in medical, legal, and industrial circles. <h3>Report of a Case</h3> On Aug. 10, 1954, a 53-year-old yard helper, while waiting for a diesel switch engine, was standing under a bridge in the flats of Cleveland, opposite a pool of stagnant water. Upon feeling a sting on the back of his left thigh, just above the knee, he squeezed his trouser at that point and heard a "crack or pop." He insisted that a bug about 2 inches long
A Central Role for CD68(+) Macrophages in Hepatopulmonary Syndrome
Authors: Thenappan Thenappan, Ankush Goel, Glenn Marsboom, Yong-Hu Fang, Peter T. Toth, Hannah J. Zhang, Hidemi Kajimoto, Zhigang Hong, Jonathan Paul, Christian Wietholt, Jennifer Pogoriler, Lin Piao, Jalees Rehman, Stephen L. Archer
Journal: American Journal of Respiratory and Critical Care Medicine (2010)

The etiology of hepatopulmonary syndrome (HPS), a common complication of cirrhosis, is unknown. Inflammation and macrophage accumulation occur in HPS; however, their importance is unclear. Common bile duct ligation (CBDL) creates an accepted model of HPS, allowing us to investigate the cause of HPS.
Bicycle injuries in children.
Authors: J R Sibert, R G Newcombe
Journal: BMJ (1977)

normal."No murmurs were heard and there was no clubbing, splinter haemorrhages or splenomegaly.Thus, at this stage there was no clear evidence that the heart wvas infected.The haemoglobin on admission was 12 5 g,dl, WBC 5 9 1091 (5500 mm3), ESR 68 mm in the first hour, and blood urea 5 6 mmol 1 (34 mg 100 ml).Blood, faeces, and urine cultures all yielded a growtth of S enteritidis sensitive to cephalosporins, ampicillin, tetracycline, gentamicin, trimethoprim, sulphonamides, chloramphenicol, and, on later testing, mecillinam (FL 1060).Many antibiotics were given without clinical response (see figure).As his general condition was worsening chloramphenicol was started.This was followed by a prompt clinical improvement and he became afebrile.Nevertheless, an early diastolic murmur was noted on 3 October and signs of rapidly increasing aortic reflux appeared over the next few days.Endocarditis was now diagnosed.Replacement of the prosthetic valve was considered necessary, but surgery postponed because the patient developed bone marrow depression with a low platelet count.Chloramphenicol was stopped; blood and platelet transfusions were given.The fever recurred and mecillinam was started on 21 October.I his was followed by a fall in the temperature.Gentamicin (riqg) Chloramphenicol (72qJ t( 245Mecillinam Cephazolin (39) Cot Ampkillin Am icillin () 39 2 38 I! Blood cultures, 3 7 all 5 sets, positive Endocarditis (aortic refIux) 36 10 12 14 1 18 2224 228 30 2 4 68 10 12 14 16 18 20 22 24 26 28 September October Effects of antibiotics on the course of Salmtioniella enteritidis infection.Cot Cotrimoxazole, four tablets daily.Figures in parentheses indicateintravenous daily dosage of the drug, changes of which are explained in the text.Ampicillin was added tNwo days later as there was in-vitro evidence of synergy between mecillinam and ampicillin against the salmonella.On this combination of antibiotics the patient remained afrebile, the bone marrow depression gradually resolved, and open heart surgery was performed on 28 October.At operation infected granulations were observed on the struts and sewing ring of the Starr-Edwards valve.A large false aneurysm was also present posterior to the prosthetic valve suture line and this contained a mass of infected clot.The valve and clot were removed and an aortic Bjork-Shiley valve inserted; but the patient died without regaining consciousness.The excised Starr-Edwards valve was cultured in nutrient broth containing serum and S enteritidis was isolated.
Alkaline Trypan Blue as a stain for superficial fungi
Authors: I. Ikeda, K. Nishimoto, K. Sasamoto, K. Nagira, T. Ono, H. Ihn
Journal: British Journal of Dermatology (2008)

Conflicts of interest none declared. Sir, For staining superficial fungi, Parker Blue‐Black Super Quink® (Sanford L.P., Oakbrook, IL, USA) ink has been used as a standard dye with potassium hydroxide (KOH) for a long time. Recently comments citing precipitation and colour change of Parker ink in KOH solution have appeared in Japanese journals, and the Chlorazol black E (CbE) method1 is now employed as a major substitute. CbE belongs to the azo dye group and is presumed to be an established fungal stain, although the colour of CbE staining differs from classic Parker ink. In order to produce a stain resembling Parker ink, we examined the literature about old Parker Blue‐Black Superchrome® ink2 and realised that the dye responsible for staining fungi was highly similar to Direct Blue 1, a typical azo dye. Therefore, we attempted to discover a better stain from the azo dye group. Considering commercial availability and structural similarity to Parker ink, we selected three azo dyes as candidates. Direct Blue 1 [Sigma’s Chicago Sky Blue 6B (C8679)] (Sigma‐Aldrich, St Louis, MO, USA) was quite stable in an alkaline environment and had good ability of staining, but the specificity was insufficient. Evans Blue (Sigma‐Aldrich 206334, dye content 85%) was stable and could stain fungi clearly, but it lacked potential for staining fungi embedded in skin scrapes. Trypan Blue (Wako 207‐03252, Wako Special Grade, Wako, Osaka, Japan), which was reported to be a potent stain for fungi in the botanical field by Boedijn,3 could stain fungus elements in clear blue colour in 5–10 min and represent their nuclei. An increase in staining over time was also observed. Candida and Malasssezia stained as well as dermatophytes. By adjusting the dye concentration to 0·3%, we could obtain the best contrast to detect fungus elements easily (Fig. 1).
Perimenopausal risk of falling and incidence of distal forearm fracture.
Authors: S. J. Winner, C. A. Morgan, J. G. Evans
Journal: BMJ (1989)

A postal survey of 2000 women and 2000 men sampled from the electoral roll in Oxford was undertaken to ascertain whether changes with age in the risk of falling might explain the stepwise increases in age specific incidence rates of distal forearm fracture which occur in women at around the age of 50. Corrected response rates were 83% for women and 72% for men. In women, but not in men, there was a rise in the risk of falling from 45 years, peaking in the 55-59 year age group, and sinking to a nadir at ages 70-74. In both sexes rates rose in extreme old age. These variations were not attributable to preferential response from people who had suffered a fracture. It is concluded that changes in the risk of falling interact with osteoporosis to produce a perimenopausal rise in the incidence of forearm fractures and contribute to the fluctuations in incidence of these fractures in old age.
T1 TNFα mediates ectodomain shedding of BMPR-II: a mechanism for inflammation as a trigger for pulmonary arterial hypertension
Authors: LA Hurst, BJ Dunmore, PD Upton, NW Morrell
Journal: Thorax (2013)

<h3>Background</h3> Mutations in BMP type II receptor (BMPR-II) account for 70% of heritable pulmonary arterial hypertension (PAH) cases, but low penetrance (~20%) in mutation carriers implies a 'second hit' is required for disease initiation. Inflammation has been implicated, yet the molecular mechanisms by which it influences pathology are unclear. Tumour necrosis factor alpha (TNFα) has been reported to influence BMPR-II expression, yet the molecular interplay remains unclear. <h3>Methods</h3> Human pulmonary arterial smooth muscle cells (PASMCs) were stimulated with TNFα (1ng/mL). Biochemical (immunoprecipitation, western blotting), molecular biology (quantitative PCR, plasmid DNA transfection, site-directed mutagenesis, RNA interference) and pharmacological (metalloprotease, proteasome and lysosome inhibitors) approaches were used to assess the impact of TNFα on BMPR-II and BMP signalling pathways. To assess this <i>in vivo</i>, two animal models of PAH were utilised: a mouse model overexpressing TNFα specifically in the lung as well as a moncrotaline induced PAH (MCT-PAH) rat model. <h3>Results</h3> TNFα stimulation reduced BMPR-II mRNA and protein expression, leading to loss of BMP signalling, as evidenced by abrogated Smad 1/5 and ID1 activation. Notably, a low molecular weight form of BMPR-II accumulated in PASMC lysates following prolonged TNFα exposure: identified as a C-terminal cleavage product of BMPR-II. Furthermore, the N-terminal ectodomain of BMPR-II could be immunoprecipitated from PASMC conditioned media and was quantified by ELISA. TNFα increased expression of two A Disintegrin and Metalloproteinase Domain-containing proteins (ADAMs); ADAM10/17. Pharmacological blockade and RNA interference revealed both proteases were capable of BMPR-II cleavage. Mutation of the putative cleavage site restored BMP signalling. The cleaved ectodomain acted as a ligand trap, sequestering BMP ligands and inhibiting their signalling capacity. Proliferation assays revealed loss of BMP2/4 induced PASMC anti-proliferation in the presence of BMPR-II ectodomain. Finally, both animal models revealed reduced BMPR-II and c-terminal cleavage product in lung tissue - highlighting this event occurs <i>in vivo</i>. <h3>Conclusions</h3> We identified a novel mechanism by which TNFα impairs BMP signalling and promotes PASMC proliferation in the lung vasculature. TNFa may provide the critical link between inflammation and disease initiation in PAH. Our <i>in vivo</i> observations highlight TNFα as a potential therapeutic target in PAH.
Recurrent Hemoperitoneum Reversed by Ascorbic Acid
Authors: William L. Cooke
Journal: JAMA (1977)

HEMOPERITONEUM, excluding those episodes resulting from surgically reversible conditions, is usually associated with a fatal outcome.<sup>1-3</sup>Recurrent hemoperitoneum is, therefore, exceedingly rare, and medically reversed recurrent hemoperitoneum has not been reported.<sup>4</sup> In the following account, a patient with multiple episodes of hemoperitoneum appears to have been cured by ascorbic acid therapy. <h3>Report of a Case</h3> A 48-year-old white woman, gravida 2, para 2, was hospitalized in April 1967, complaining of hypermenorrhea for one year and abdominal pain for five weeks. No unusual bleeding had occurred in association with dental extractions or episiotomies during vaginal deliveries. No easy bruisability had been noted, and no relative had experienced unusual bleeding. Her only medication had been aspirin as treatment for intermittent headaches. Examination disclosed minimal pallor and moderate abdominal distention. No angiomas were noted in the skin or mucous membranes. The uterus was enlarged. Hemoglobin level was 10.7 gm/100 ml, and
THE ELIMINATION OF THE DIGITALIS BODIES
Authors: R. A. HATCHER
Journal: JAMA (1913)

The digitalis bodies show such wide differences in the duration of their action, even after intravenous administration, that one would be inclined to expect similar variations in their rate of elimination. The difficulties attending the quantitative chemical estimation of these bodies have interfered with the investigation of their elimination, and we know almost nothing of their fate in the organism.  Numerous observers have investigated the question of the storage of these glucosids in various organs, and, while I also have considered that phase of the question, the present paper will be limited almost entirely to a discussion of the disappearance of these agents from the circulation. It may be remarked parenthetically, however, that the rat sometimes excretes ouabain almost quantitatively after its subcutaneous injection, and that no one has been able to demonstrate either the fixation or the destruction of notable amounts of any of the digitalis bodies in the