tbuckley/cabot-search huggingface dataset:
An unexpectedly bizarre blood film in hemoglobin H disease
Authors: S. Chakravorty, M.J King, B.J Bain
Journal: American Journal of Hematology (2012)

A 3-year-old boy from Saudi Arabia had been followed up with a diagnosis of hemoglobin H disease, identified in a neonatal screening programme. His parents were second cousins. Alpha globin genotyping at birth had revealed homozygosity for the non-deletional Saudi-T mutation (αTα/αTα), both parents being carriers. He had remained clinically asymptomatic. Red cell indices at the age of three years were: RBC 3.84 x 1012/l, hemoglobin concentration 80 g/l, MCV 61 fl, MCH 20.9 pg, MCHC 34 g/l, red cell distribution width 34.8 (normal range 11.8-14.3) and reticulocyte count 447 x109/l. His blood film, in addition to microcytosis, showed gross poikilocytosis with the abnormal red cells including elliptocytes and many bizarre poikilocytes, some very small (image). Although poikilocytosis can be prominent in hemoglobin H disease, we have not seen a case with this degree of abnormality. The film was reminiscent of hereditary pyropoikilocytosis. For this reason red cell membrane studies were initiated. An eosin-5 maleimide (EMA)-binding assay [1] gave a twin peak fluorescence profile: 57% of phenotypically normal red cells (MCF = 64.7 units) and 42% having reduced fluorescence (MCF = 32.4 units). Spectrin (Sp) analysis showed a Sp dimer content of 25.6 %, compared with 7.4 % for a normal control within the same assay. In limited trypsin digestion of spectrin, SpαI/50 variant was detected in the child, and absent in the mother. Neither had an increase of SpαV/41 tryptic peptide, thus excluding the presence of the polymorphic low expression allele, SpαLELY. Maternal red cells were not elliptocytic and had normal spectrin dimer content and normal EMA binding. A paternal sample was not available for testing. Although the EMA-binding assay has high specificity and sensitivity for hereditary spherocytosis, it can also be abnormal in hereditary pyropoikilocytosis, Southeast Asian ovalocytosis, cryohydrocytosis, and some cases of congenital dyserythropoietic anemia type II [2]. Hereditary elliptocytosis usually gives a single fluorescence peak within the range for normal red cells but occasionally twin peaks are observed. In this child with co-inheritance of hemoglobin H disease and hereditary elliptocytosis, the severity of the hemolytic anaemia is that expected in uncomplicated hemoglobin H disease but the bizarre blood film reflects an interaction of the hemoglobinopathy and the membrane defect. 1
A DIETARY REGIMEN IN THE TREATMENT OF PSORIASIS
Authors: HARRY SPIERA, ALBERT M. LEFKOVITS, IRWIN ORESKES
Journal: British Journal of Dermatology (1971)

SUMMARY.— A dietary regimen employing turkey meat as the major protein source has been evaluated in patients with severe psoriasis. These patients had been selected for failure to remit spontaneously and failure to respond to other methods of therapy. Of 12 patients studied, 7 exhibited excellent response, 3 moderate response, and 2 no response to the dietary regimen. Two patients, who responded initially, were studied further. Upon resumption of a normal diet, their disease exacerbated. Reinstitution of the study diet again resulted in complete clearing of their psoriasis. Addition of turkey to a normal diet, however, did not prevent re-exacerbation. These results suggest that turkey meat may lack a substance potentially harmful to patients with psoriasis.
Load Dependency of Right Ventricular Performance Is a Major Factor to be Considered in Decision Making Before Ventricular Assist Device Implantation
Authors: Michael Dandel, Evgenij Potapov, Thomas Krabatsch, Alexander Stepanenko, Alexandra Löw, Juliane Vierecke, Christoph Knosalla, Roland Hetzer
Journal: Circulation (2013)

Background— Left ventricular assist devices (LVADs) provide better outcome than biventricular devices, but it is a challenge to predict the impact of LV mechanical unloading on postoperative right ventricular (RV) function preoperatively. We assessed the load dependency in RV performance before and after LVAD implantation aiming to improve preoperative decision making. Methods and Results— Laboratory, echocardiography, and right heart catheterization data collected from 205 patients before LVAD implantation were tested for relationship with postoperative RV function. Comparing patients with different time-course of RV function after LVAD implantation, we found significant differences ( P &lt;0.01) in preoperative RV end-diastolic short-/long-axis and long-axis/length-area ratios, tricuspid annulus peak systolic velocity, RV peak longitudinal global systolic strain rate, systolic pressure gradient between RV and right atrium (Δ P RV−RA ), tricuspid regurgitation velocity-time integral, and pulmonary arterial pressure between patients with and without postoperative RV failure. High predictive values for postoperative RV failure were found for end-diastolic short-/long-axis ratio ≥0.6, tricuspid annulus peak systolic velocity &lt;8 cm/s, and peak systolic longitudinal strain rate &lt;0.6/s in patients with maximum Δ P RV−RA &lt;35 mm Hg. These parameters also seemed predictive for RV failure in patients with tricuspid regurgitation grade &gt;2 and pulmonary arterial pressure &lt;50 mm Hg. End-diastolic short-/long-axis ratio &lt;0.6, tricuspid annulus peak systolic velocity ≥8 cm/s, and peak systolic longitudinal strain rate ≥0.6 in patients with maximum Δ P RV−RA ≥35 mm Hg showed high predictive values for postoperative freedom from RV failure. The RV load adaptation index seemed particularly predictive for RV function after LVAD implantation. Conclusions— RV geometry and velocity of contraction before LVAD implantation become more predictive for postoperative RV function and can improve decision making before VAD implantation if preoperative RV pressure load and tricuspid regurgitation are also considered.
A woman with episodic headaches, sweating, and palpitations
Authors: A. Jones, M. Bull, B. Vaidya
Journal: BMJ (2011)

A 39 year old woman was referred to the headache clinic with a 10 year history of episodic headaches, sweating, and palpitations. The symptoms had intensified over the past one to two years. Since then she has been experiencing three to four episodes a day, mostly at night, of severe headache, chest tightness, sweating, palpitations, and “churning stomach.” She had no medical history of note and took no regular drugs. Her mother had been diagnosed with metastatic cancer of unknown source in her early 60s.

On examination, her blood pressure was 100/80 mm Hg, with no postural drop; pulse was 86 beats/min and regular. She had a palpable left thyroid nodule but no associated lymphadenopathy, and other systemic examinations were unremarkable.

Magnetic resonance imaging of the head showed no abnormalities. Thyroid function tests were normal; 24 hour urinary catecholamines performed twice showed markedly raised noradrenaline (628 nmol/L and 613 nmol/L; reference range 0-450) and adrenaline (722 nmol/L and 915 nmol/L; 0-100), suggestive of phaeochromocytoma. Magnetic resonance imaging of the adrenals showed a large mass in the right upper quadrant and a small mass in the left adrenal. An iodine-123-meta-iodobenzylguanidine (123I-MIGB) single photon emission computed tomography (SPECT) scan (fig 1⇓) was performed.



Fig 1 Coronal 123I-MIBG SPECT fusion scan





### 1 What does the 123I-MIBG SPECT scan show?

#### Short answer

The 123I-MIBG SPECT scan shows increased uptake of marker in the right upper abdomen, and in the regions of the left adrenal gland and left thyroid lobe.

#### Long answer

MIBG is a precursor of catecholamines. Radiolabelled 123I-MIBG or 131I-MIBG enters the catecholamine synthesis pathway and concentrates within phaeochromocytomas and other neuroendocrine tumours.1 In suspected …
Transesophageal Echocardiography for the Diagnosis and Management of Nonobstructive Thrombosis of Mechanical Mitral Valve Prosthesis
Authors: Pascal Gueret, Philippe Vignon, Pierre Fournier, Jean-Marc Chabernaud, Marie Gomez, Philippe LaCroix, Julien Bensaid
Journal: Circulation (1995)

Background Diagnosis of a mechanical mitral valve prosthesis thrombosis is currently made with transthoracic Doppler echocardiography and occasionally with fluoroscopy. However, identifying a thrombus on a valve prosthesis may be difficult, especially if the thrombus is nonobstructive. To prospectively define the role of transesophageal echocardiography for identification of nonobstructive thrombi, we studied a series of patients in whom the prosthetic valve was considered to function normally on clinical examination and transthoracic echocardiography. Methods and Results One hundred fourteen consecutive patients with mechanical mitral valve prosthesis were investigated by both transthoracic echocardiography and transesophageal echocardiography. These examinations were performed for recent systemic emboli (15 patients), fever of unknown etiology (11 patients), routine postoperative evaluation (56 patients), and other reasons (32 patients). Based on transthoracic echo diagnosis, all prostheses were considered normal. Yet, in 20 patients transesophageal echocardiography revealed the presence of a 2- to 15-mm-long mobile thrombus localized on the atrial surface of the prosthesis. When compared with the remaining 94 patients with no visible thrombi, there was no significant difference between the two groups in terms of incidence of atrial fibrillation (65% versus 52%), left atrial size (48±9 versus 51±13 mm), left ventricular end-diastolic diameter (49±10 versus 51±13 mm) and fractional shortening (28±9% versus 31±10%), presence of spontaneous contrast in the left atrium (40% versus 41%), transprosthetic mean pressure gradient (4.0±1.4 versus 3.9±1.5 mm Hg), or the type of prosthesis used. After we discovered a nonobstructive thrombosis, patients were treated with heparin (n=9) or oral anticoagulation (n=11). The presence of a localized thrombus was confirmed in 3 patients who were operated on. In the present study, evolution appeared to depend on thrombus size: of 14 patients exhibiting a small (&lt;5 mm) thrombus, 10 had an uneventful course, whereas 5 of 6 patients with a large (≥5 mm) thrombus developed complications or died. Conclusions Transesophageal echocardiography appears to be a reliable method to diagnose thrombi on a mechanical mitral valve prosthesis, even when transthoracic Doppler echocardiographic parameters appear to be normal. Transesophageal echo assessment of thrombus size may be helpful in deciding whether a patient with mitral prosthesis should be treated by anticoagulation, thrombolysis, or valve rereplacement. .
Other brain effects of words
Authors: Herman T. Epstein
Journal: Behavioral and Brain Sciences (1999)

Pulverm&amp;uuml;ller's discussion needs more explanation of how the proposed assemblies remain assembled after formation and how they can be accessed later among all the possible assemblies, many of which involve many of the same neurons. Alternative Hebbian strengthening mechanisms may provide additional information, and, developmental studies of the assemblies might provide insights into their evolution.
Swapping Ilford for Baghdad
Authors: Fiona Fleck
Journal: BMJ (2004)

Iraq's health minister, Dr Khudair Abbas, is determined to transform Iraq's health services even though it leaves him exposed to danger 

Iraq's interim health minister, Dr Khudair Abbas—like any other Iraqi working closely with the US led Coalition Provisional Authority—is a natural target for attacks.

Far from the east London suburbs, where the 54 year old worked as a general and orthopaedic surgeon until he was appointed to Iraq's ministerial council in September, his four mile journey to work in Baghdad is a reminder that he can never feel safe.

“There are guards at home. I travel to the ministry in a convoy, taking different routes and different timing,” Dr Abbas said in an interview in Geneva, where he was attending the World Health Assembly. “Constant awareness of security has become part of my life,” he added.

Dr Abbas was born and raised in the southern city of Basra. He qualified at the University of Baghdad in 1972 and came to Britain in 1979, where he became a fierce opponent of the Iraq-Iran war, which led to his 23 years' exile.

In 1990, he became a fellow of the Royal College of Surgeons, Edinburgh, and took up a surgeon's …
Inhibition of Vasopressin-Stimulated Urea Transport Across the Toad Bladder by Thiourea
Authors: Patrick Eggena
Journal: Journal of Clinical Investigation (1973)

The mammalian antidiuretic hormone, 8-arginine-vasopressin, was found to increase net mucosal-to-serosal urea flux across the isolated toad urinary bladder 13-fold. This urea flux was accompanied by a 24-fold increase in solute-linked water movement across the membrane. Net urea flux and urea-linked volume flux were inhibited by 50% or more when thiourea was added to the mucosal medium at concentrations equal to those of urea. In contrast, thiourea did not inhibit osmotic water flux across the bladder in the presence of vasopressin. These observations are consistant with the view that thiourea and urea compete for a common site on a membrane carrier molecule. When bladders were exposed to vasopressin on the serosa and subsequently fixed with 1% glutaraldehyde on the mucosa, they were found to retain 74% of their prefixation permeability to urea. Net urea flux across these fixed bladders (in the absence of vasopressin) was markedly inhibited by thiourea, whereas osmotic water flux was not inhibited. These studies suggest that vasopressin induces the formation of "urea-channels" in the membrane that can be preserved by glutaraldehyde and blocked by thiourea.
Large Medical Databases, Population-Based Research, and Patient Confidentiality
Authors: Gregory E. Simon, Jürgen Unützer, Barbara E. Young, Harold Alan Pincus
Journal: American Journal of Psychiatry (2000)

OBJECTIVE: This article is a discussion of the use of large clinical databases in population-based research on psychiatric disorders. METHOD: The authors review uses of large clinical databases in research on the etiology, impact, and treatment of psychiatric disorders. They also describe existing privacy safeguards applicable to use of medical records data in research. RESULTS: The growth of large medical databases has prompted increasing concern about the confidentiality of patient records. Efforts to restrict access to computerized medical data, however, may preclude use of such data in important and legitimate research. Prior research using large medical databases has made important contributions across a broad range of topics, including epidemiology, genetics, treatment effectiveness, and health policy. Continued population-based research will be essential in order to preserve the accessibility and quality of treatment for people with psychiatric disorders. CONCLUSIONS: Public domain research should be distinguished from proprietary or commercial uses of health information, and existing privacy safeguards should be vigorously applied. In our efforts to protect patient privacy, however, we should take care not to endorse or reinforce prejudices against psychiatric treatment and people who suffer from psychiatric disorders. Neither should we ignore important opportunities to improve quality of care and influence public policy through population-based research.
Hepatocyte caspase-8 is an essential modulator of steatohepatitis in rodents
Authors: Maximilian Hatting, Gang Zhao, Fabienne Schumacher, Gernot Sellge, Malika Al Masaoudi, Nikolaus Gaβler, Mark Boekschoten, Michael Müller, Christian Liedtke, Francisco Javier Cubero, Christian Trautwein
Journal: Hepatology (2013)

In human and murine models of nonalcoholic steatohepatitis (NASH), increased hepatocyte apoptosis is a critical mechanism contributing to inflammation and fibrogenesis. Caspase 8 (Casp8) is essential for death-receptor-mediated apoptosis activity and therefore its modulation might be critical for the pathogenesis of NASH. The aim was to dissect the role of hepatocyte Casp8 in a murine model of steatohepatitis. We generated hepatocyte-specific Casp8 knockout (Casp8(Δhep) ) mice. Animals were fed with a methionine-choline-deficient (MCD) diet. Liver injury was assessed by histopathological analysis, apoptotic death, serum alanine aminotransferase (ALT), fluorescent-activated cell sorter (FACS), analysis of liver infiltration and inflammation, reactive oxygen species (ROS), and liver fibrosis. MCD feeding triggered steatosis, hepatic lipid storage, and accumulation of free fatty acid (FFA) in wildtype (WT) livers, which were significantly reduced in Casp8(Δhep) animals. Additionally, lack of Casp8 expression in hepatocytes reduced the MCD-dependent increase in apoptosis and decreased expression of proinflammatory cytokines as well as hepatic infiltration. As a consequence, ROS production was lower, leading to a reduction in the progression of liver fibrosis in Casp8(Δhep) livers.Selective ablation of Casp8 in hepatocytes ameliorates development of NASH by modulating liver injury. Casp8-directed therapy might be a plausible treatment for patients with steatohepatitis. (HEPATOLOGY 2013;57:2189-2201).
Circadian variation in disease activity in rheumatoid arthritis.
Authors: J A Harkness, M B Richter, G S Panayi, K Van de Pette, A Unger, R Pownall, M Geddawi
Journal: BMJ (1982)

Disease activity in rheumatoid arthritis as measured by repeated estimation of joint pain, stiffness, articular index, and grip strength was shown to have a circadian rythm, maximal activity being seen between 0200 and 0400 and minimal activity in the early afternoon. This variation in disease activity may be related to circadian alterations in immune and inflammatory responses (such as immune complexes and neutrophil function) dependent on alterations in circulating concentrations of steroids. The circadian variation in disease activity has important implications in assessment of patients, prescription of drugs, and performance of drug trials.
Enzyme Profiles in Pulmonary Infarction
Authors: Eugene L. Coodley
Journal: JAMA (1969)

<h3>To the Editor:—</h3> In reply to the letter of Dr. Stead, our studies have indicated that the CPK and HBD were primarily elevated in patients with myocardial infarction. We indicated that the study of isoenzymes in general was not of value in distinguishing pulmonary from myocardial infarction. We also indicated that the LDH alone was often not useful since it was elevated in pneumonia, heart failure, and other diseases. Our conclusion was that in the presence of an increased LDH and clinical manifestations suggesting pulmonary infarction, the finding of a normal CPK would effectively eliminate myocardial infarction as the cause of the symptom and the finding of an elevated CPK would strongly suggest such myocardial infarction.
Low-dose alpha-tocopherol improves and high-dose alpha-tocopherol worsens endothelial vasodilator function in cholesterol-fed rabbits.
Authors: J F Keaney, J M Gaziano, A Xu, B Frei, J Curran-Celentano, G T Shwaery, J Loscalzo, J A Vita
Journal: Journal of Clinical Investigation (1994)

Abnormalities in endothelium-dependent arterial relaxation develop early in atherosclerosis and may, in part, result from the effects of modified low-density lipoprotein (LDL) on agonist-mediated endothelium-derived relaxing factor (EDRF) release and EDRF degradation. alpha-Tocopherol (AT) is the main lipid-soluble antioxidant in human plasma and lipoproteins, therefore, we investigated the effects of AT on endothelium-dependent arterial relaxation in male New Zealand White rabbits fed diets containing (a) no additive (controls), (b) 1% cholesterol (cholesterol group), or 1% cholesterol with either (c) 1,000 IU/kg chow AT (low-dose AT group) or (d) 10,000 IU/kg chow AT (high-dose AT group). After 28 d, we assayed endothelial function and LDL susceptibility to ex vivo copper-mediated oxidation. Acetylcholine-and A23187-mediated endothelium-dependent relaxations were significantly impaired in the cholesterol group (P < 0.001 vs. control), but preserved in the low-dose AT group (P = NS vs. control). Compared to the control and cholesterol groups, vessels from the high-dose AT group demonstrated profound impairment of arterial relaxation (P < 0.05) and significantly more intimal proliferation than other groups (P < 0.05). In normal vessels, alpha-tocopherol had no effect on endothelial function. LDL derived from both the high- and low-dose AT groups was more resistant to oxidation than LDL from control animals (P < 0.05). These data indicate that modest dietary treatment with AT preserves endothelial vasodilator function in cholesterol-fed rabbits while a higher dose of AT is associated with endothelial dysfunction and enhanced intimal proliferation despite continued LDL resistance to ex vivo copper-mediated oxidation.
Extrahepatic Manifestations of Hepatitis C Virus Infection
Authors: Tjaard U. Hoogenraad
Journal: Annals of Internal Medicine (1996)

Letters15 August 1996Extrahepatic Manifestations of Hepatitis C Virus InfectionTjaard U. Hoogenraad, MD, Gerard H. Jansen, MD, and Jan van Hattum, MDTjaard U. Hoogenraad, MDUniversity Hospital, Utrecht 3508 GA, the NetherlandsSearch for more papers by this author, Gerard H. Jansen, MDUniversity Hospital, Utrecht 3508 GA, the NetherlandsSearch for more papers by this author, and Jan van Hattum, MDUniversity Hospital, Utrecht 3508 GA, the NetherlandsSearch for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-125-4-199608150-00019 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail TO THE EDITOR:In their review of the extrahepatic manifestations of HCV infection, Gumber and Chopra [1] did not mention the possible association between HCV and progressive multifocal leukoencephalopathy (PML). Pearce and colleagues [2] first proposed this association, but thus far their observation has not been confirmed.We describe a 62-year-old male dentist who had a 6-year history of chronic hepatitis C and cirrhosis. Within a few months, the patient became severely confused, aphasic, and demented. Magnetic resonance imaging showed large white-matter lesions in the frontal lobe. Examination of a biopsy specimen obtained from this area showed abnormal astrocytes with ...References1. Gumber SC, Chopra S. Hepatitis C: a multifaceted disease. Review of extrahepatic manifestations. Ann Intern Med. 1995; 123:615-20. Google Scholar2. Pearce DA, Wong V, Miloszewski KJ. Hepatitis C: a novel cause of progressive multifocal leucoencephalopathy. Br J Hosp Med. 1993; 50:193. Google Scholar3. Von Ho β lin C, Alzheimer A. Ein Beitrag zur Klinik und pathologischen Anatomie der Westphal-Strumpellschen Pseudosclerose. Zeitschr Gesamte Neurol Psychiatr. 1912; 8:183-209. Google Scholar4. Aksamit AJ. Nonradioactive in situ hybridization in progressive multifocal lcukoencephalopathy. Mayo Clin Proc. 1993; 68:899-910. Google Scholar Author, Article, and Disclosure InformationAffiliations: University Hospital, Utrecht 3508 GA, the Netherlands PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited ByWilson disease 15 August 1996Volume 125, Issue 4Page: 345KeywordsBrain diseasesChronic liver diseaseCirrhosisHodgkin lymphomaInfectious hepatitisLymphocytic leukemiaMagnetic resonance imagingMalignant tumorsProgressive multifocal leukoencephalopathySarcoidosis Issue Published: 15 August 1996 CopyrightCopyright © 1996 by American College of Physicians. All Rights Reserved.PDF DownloadLoading ...
TCT-484 Prognostic value of simple standard 12-lead electrocardiographic parameters for predicting clinical outcomes in hospital survivors after acute myocardial infarction
Authors: Jang Hoon Lee, Myung Hwan Bae, Shung Chull Chae, Yongkeun Cho, Hyun Jun Cho, Won Suk Choi, Se Yong Jang, Jae-Eun Jun, Kyun Hee Kim, Sun Hee Park, Hun Sik Park, Dong Heon Yang
Journal: Journal of the American College of Cardiology (2012)