tbuckley/cabot-search huggingface dataset:
Hereditary renal amyloidosis with a novel variant fibrinogen.
Authors: T Uemichi, J J Liepnieks, M D Benson
Journal: Journal of Clinical Investigation (1994)

Two families with hereditary renal amyloidosis were found to have a novel mutation in the fibrinogen A alpha chain gene. This form of amyloidosis is an autosomal dominant condition characterized by proteinuria, hypertension, and subsequent azotemia. DNAs of patients with amyloidosis were screened for a polymorphism in fibrinogen A alpha chain gene by single-strand conformation polymorphism analysis, and affected individuals from two kindreds were found to have a mutation. Both of these kindreds are American of Irish descent presenting with non-neuropathic, nephropathic amyloidosis in the fifth to the seventh decade of life. DNA sequencing showed a point mutation in the fibrinogen A alpha chain gene that is responsible for substitution of valine for glutamic acid at position 526. By restriction fragment length polymorphism analysis, 7 affected individuals and 14 asymptomatic individuals in these two kindreds were positive for the fibrinogen A alpha chain Val 526 gene. Fibrinogen was isolated from plasma of a heterozygous gene carrier and shown to contain approximately 50% variant fibrinogen. Discovery of this new mutation confirms the association between fibrinogen A alpha chain variant and hereditary renal amyloidosis and establishes a new biochemical subtype of amyloidosis.
In situ follicular lymphoma associated with overt B‐ or T‐cell lymphomas in the same lymph node
Authors: Antonino Carbone, Duilio Della Libera, Laura Zannier, Antonella Selva, Paola Ceolin, Ambra Gualeni, Barbara Canal, Annunziata Gloghini
Journal: American Journal of Hematology (2011)

In situ follicular lymphoma (FL) is usually an incidental finding in otherwise reactive lymph node [1–3]. However, it may be associated with overt FL, or with lymphomas other than FL or with other malignancies,in other sites or, less commonly, in the same lymph node [2,4–8]. Here we describe two cases of in situ FL, one with concurrent overt FL(Case 1), and one with concurrent peripheral T-cell lymphoma (PTCL),NOS (Case 2) in the same lymph node. Immunohistochemistry, polymerase chain reaction for B and T-cell clonality, and double-staining chromogenic in situ hybridization for BCL2 translocation were performed.In both cases, the in situ FL foci were characterized by strong expression of BCL2 and CD10 in the germinal center B cells of the affected follicles. Case 1 showed the concurrence of an overt B-cell FL with IgH@ rearrangement and expression of B-cell markers, but not BCL2. Case 2 demonstrated the concurrence of a PTCL, NOS with TCRG@ rearrangement and expression of T-cell markers. In conclusion,the association of in situ FL with PTCL expands the spectrum of lymphoproliferations that may coexist with in situ FL and suggests that in situ FL may not behave like a simple precursor for overt FL.
TRANSPLANTABLE EPITHELIOMAS OF THE LIP AND MOUTH OF CATFISH
Authors: Balduin Lucké, H. Schlumberger
Journal: The Journal of Experimental Medicine (1941)

The catfish (Ameiurus nebulosus) taken from streams near Philadelphia, is commonly afflicted with an epithelial tumor bearing some resemblance to epithelioma of the lip in man. This neoplasm usually occurs as solitary or multiple, large, red, fleshy masses upon the lips or dental plates, and by reason of its size, may prevent closure of the mouth. The tumor is comprised of epithelial cells, often in papillary arrangement, supported by a delicate vascularized connective tissue stroma. The larger growths frequently invade adjacent normal tissues and force their way into vessels where they are found as emboli. The clinical course of the tumor is one of relatively slow but progressive growth. This neoplasm has been observed from the time of its inception in a number of animals. Thus it has been learned that the proliferative stage of the neoplastic process is preceded and accompanied by a striking vascular reaction. Intense hyperemia invariably occurs in that region of the mucosal surface which later becomes the site of neoplastic proliferation. Furthermore, by direct microscopic observation of the living tumors the atypical structure and arrangement of the blood vessels become apparent. A study of the significance of these vascular phenomena in their relation to the inception and growth of the tumor is now in progress. It has been found possible to transmit the catfish tumor to fish of the same species by implanting fragments of the tumor into the anterior chamber of the eye. Also, by taking advantage of an anatomical peculiarity of the catfish cornea, it has been possible to embed the tumor fragments in normal tissue where it could still be readily observed both in the gross and microscopically. The growth of the transplants in the eye has been followed by periodic examination of the living tumor by means of the slit lamp microscope. In the anterior chamber, the tumor characteristically forms dense membranes which spread over the inner surface of the cornea. In this manner growth continues until the tumor fills the chamber. Between the two layers of the cornea, tumor growth is expansive. Attempts to transplant the tumor to the anterior chamber of two other species of fish and to frogs, were unsuccessful. Implantation of the catfish epithelioma in alien species of fish excited no exudative response, whereas in a less closely related species of animal, the leopard frog, a pronounced exudative reaction resulted.
CARDIOVASCULAR DISEASE AND HARDNESS OF WATER
Authors: Walter Menaker
Journal: JAMA (1960)

<h3>To the Editor:—</h3> The paper by Schroeder on mortality from cardiovascular disease in relation to treated water supplies (<i>J. A. M. A.</i><b>172</b>:1902 [April 23] 1960) does not state the magnitude of the difference in mortality rates for white men ( aged 45 to 64 years ) living in hard water and soft water states. The 12 states with softest water (lowest quartile) show a death rate for cardiovascular diseases that is only 10% higher than the 12 states with hardest water (when each state's population is given due weight). Furthermore, the 12 softest water states show an average death rate for these diseases (if each state is given equal weight) barely above the national average. Six of these 12 states are below the national average in mortality rate for these diseases. Such facts do not lend support to the hypothesis that soft or softened water increases cardiovascular mortality; indeed, the
OBSTRUCTIVE SLEEP APNEA AND CARDIOEMBOLIC RISK ASSESMENT IN PATIENTS WITH ATRIAL FIBRILLATION: CHADS2 OR CHADS2S?
Authors: Dmitry M. Yaranov, Athanasios Smyrlis, Natalia Usatii, Amber Butler, Joann Petrini, Jose Mendez, Mark Warshofsky
Journal: Journal of the American College of Cardiology (2014)
Examining Life's (Genomic) Code Means Reexamining Society's Long-Held Codes
Authors: Jody W. Zylke
Journal: JAMA (1992)

MUCH of the scientific community views the Human Genome Project as a path of tremendous promise. But ethicists and lawyers are warning that this gene map may lead down pothole-filled roads. This mapping and sequencing of the genome is unique among scientific projects in its recognition of the need to consider ethical, legal, and social issues and in providing funds to do so. At $5 million, the project has "the largest biomedical ethics program in this country and probably in the world," according to Nancy Wexler, PhD, the chair of the National Center for Human Genome Research's Committee on Ethical, Legal, and Social Issues. She is also president of the Hereditary Disease Foundation and associate professor of clinical and neuropsychiatry at Columbia University, New York, NY. <h3>Insight From Campuses</h3> In 1991, the ethics program sponsored 19 programs across the country. Participants at two of these, at the University of Michigan,
Ingestion of button batteries: hazards and management.
Authors: B Kiely, D Gill
Journal: BMJ (1986)

3 cas d'ingestion accidentelle de piles par de jeunes enfants. Controles radiographiques et evacuation sans problemes. Revue d'autres observations publiees. Les piles bloquees dans l'œsophage peuvent provoquer brulures, necroses, fistules et morts. Les piles depassant l'œsophage et se fissurant provoquent une intoxication par mercure
The “Epidemic” of Eosinophilic Esophagitis (EE) is due to Increased Recognition of a Chronic Disorder
Authors: C.W. DeBrosse, M.H. Collins, B. Buckmeier, A. Greenburg, C. Allen, J. Abonia, A. Assa'ad, J.P. Franciosi, M.E. Rothenberg
Journal: Journal of Allergy and Clinical Immunology (2010)
GALACTOSE FOR DIABETIC PATIENTS
Authors: Joseph H. Roe
Journal: JAMA (1933)

To the Editor:  —InThe Journal, Nov. 19, 1932, appeared a communication by Dr. Harry Shay, which contained such incorrect interpretations of our report on galactose tolerance of normal and diabetic subjects ( J. Biol. Chem.  96 :717 [June] 1932) that we find it necessary to submit the following rebuttal of Shay's statements:  Shay pointed out that, in our tests on normal and diabetic subjects in which 1 Gm. of galactose per kilogram of body weight was administered by mouth, there occurred in the samples of blood collected one hour after ingestion an average drop of 36 mg. of fermentable blood sugar per hundred cubic centimeters for the normal subjects, and an average increase of 22 mg. of fermentable sugar for the diabetic patients, making an average observed difference in fermentable blood sugar of 58 mg. per hundred cubic centimeters.  We object to the calculations made by Shay in the first
Hyperacute GVHD: risk factors, outcomes, and clinical implications
Authors: Rima M. Saliba, Marcos de Lima, Sergio Giralt, Borje Andersson, Issa F. Khouri, Chitra Hosing, Shubhra Ghosh, Joyce Neumann, Yvonne Hsu, Jorge De Jesus, Muzaffar H. Qazilbash, Richard E. Champlin, Daniel R. Couriel
Journal: Blood (2006)
Decoupling of normal CD40/interleukin-4 immunoglobulin heavy chain switch signal leads to genomic instability in SGH-MM5 and RPMI 8226 multiple myeloma cell lines
Authors: W Y K Hwang, C A Gullo, J Shen, C K Poh, S C Tham, G Cow, M Au, E W E Chan, G Teoh
Journal: Leukemia (2006)
Fundamentals of Magnetic Resonance Imaging
Authors: Donald W. Chakeres, Petra Schmalbrock
Journal: JAMA (1987)

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA HomeNew OnlineCurrent IssueFor Authors Podcasts Clinical Reviews Editors' Summary Medical News Author Interviews More Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA journal
A10.15 IgA Rheumatoid Factor is more Predominant than anti-CCP in Sudanese Rheumatoid Arthritis Patients, whereas IgG RF is a Strong Prognostic Marker and Associated with Early Onset
Authors: Amir I Elshafie, Sahwa Nourein, Vivek Anand Manivel, Azita Sohrabian, Mawahib IE Elidrisi, Elnour M Elagib, Musa AM Nur, Johan Rönnelid
Journal: Annals of the Rheumatic Diseases (2013)

<h3>Background and Objectives</h3> The aim was to investigate the diagnostic and prognostic impact of the conventionally used autoantibodies (IgG) anti-CCP and IgM rheumatoid factor (RF) as well as IgA and IgG RF in the first ever collected cohort of Sudanese rheumatoid arthritis (RA) patients. <h3>Materials and Methods</h3> 264 consecutive RA patients (87% females) diagnosed according to the 1987 ACR criteria attending two rheumatology centres in Khartoum between December 2008 and September 2010 were included, together with 168 healthy Sudanese blood donor controls. Autoantibody levels were investigated in Uppsala, and RF specificity levels aligned to the anti-CCP specificity (97.6%). <h3>Results</h3> Anti-CCP was elevated in 52% (131/252) of the patients, a figure not different from what has been found in Sweden (57%, Rönnelid ARD 2005; p = 0.2). Among the Sudanese RA patient, 57.2%, 51% and 49.8% had IgA, IgM and IgG RF, respectively. The areas under the Receiver Operator Characteristics (ROC) curves were 0.94 for anti-CCP, and 0.95, 0.82 and 0.85 for IgA, IgG and IgM RF, respectively. IgG RF was associated with young age (p = 0.0005) and lower age of disease onset (p &lt; 0.0001), as well as with higher total number of affected joints (p = 0.03). Hand deformities like swan neck deformity (p = 0.0001) and boutonnière deformity (p = 0.02) were also primarily associated with IgG RF. Association with the other investigated autoantibodies were weaker or absent. The prognostic impact of IgG RF was not secondarily dependent on anti-CCP, as the correlation between anti-CCP was stronger for IgM RF (r = 0.49) and IgA RF (r = 0.31) than for IgG RF (r = 0.23). <h3>Conclusions</h3> The occurrence of anti-CCP in Sudanese RA patients does not differ from Sweden. In contrary to what has been found in Caucasian RA populations, IgA RF is a diagnostically more sensitive marker than anti-CCP IgG RF is the strongest marker for bad prognosis, and associated with early disease onset.
Controlling Abuse of Drugs From the Anesthesia Department
Authors: Richard B. Patt
Journal: JAMA (1985)

<h3>To the Editor.—</h3> The recent report of Dr Adler et al, "Narcotics Control in Anesthesia Training,"<sup>1</sup>was of great interest. Hard data on the magnitude of this problem and its consequences are minimal. It is clear, however, that physician drug abuse is a devastating phenomenon, and in no subspecialty does it seem more rampant than in anesthesia: one study revealed that 1% to 2% of anesthesia personnel had come to the attention of their chairman because of drug or alcohol abuse, and that seven of 44 abusers were reported to have died as a result of problems related to abuse.<sup>2</sup>Given the nature of the drugs available (super-potent synthetic narcotics) and the anesthesiologists' responsibility for the custody of critically ill and anesthetized (helpless) patients, the consequences of drug abuse in this field are particularly devastating. The authors' efforts to address and in some measure to control the problem
T cell receptor repertoire in polymyositis: clonal expansion of autoaggressive CD8+ T cells.
Authors: A Bender, N Ernst, A Iglesias, K Dornmair, H Wekerle, R Hohlfeld
Journal: The Journal of Experimental Medicine (1995)

In polymyositis (PM), CD8+ T cell receptor (TCR) alpha/beta + cells invade and destroy major histocompatibility complex class I-positive muscle fibers. We combined polymerase chain reaction (PCR) and double-fluorescence immunocytochemistry to analyze the T cell receptor (TCR) repertoire expressed in muscle of PM patients. In patient 1, inverse PCR revealed a preferential usage of TCR V alpha 33.1, V beta 13.1, and V beta 5.1. Six of six TCR V alpha 33.1+ clones and five of seven V beta 13.1+ clones had identical nucleotide sequences. In contrast, the V beta 5.1+ TCRs were more heterogeneous. Similar results were obtained with an independent PCR method using primers specific for TCR V alpha 33, V beta 13, or V beta 5. No TCR sequences could be amplified from noninflammatory control muscle. Furthermore, none of the TCR sequences found in PM muscle could be detected in blood from the same patient or from a normal control subject. Immunohistochemistry confirmed that V beta 5.1 and V beta 13.1 were overrepresented in the muscle lesions of this patient. 32% of all CD8+ T cells were V beta 13.1+, and 16% were V beta 5.1+. However, approximately 60% of the CD8+ T cells that invaded muscle fibers were V beta 13.1+, whereas 10% were V beta 5.1+. In patient 2, 50% of the T cells were V beta 5.1+, and as in patient 1, these T cells were mainly located in interstitial areas. In patient 3, &amp;gt; 75% of the autoinvasive T cells stained with an anti-V beta 3 mAb. Sequence analysis of 15 PCR clones amplified with a V beta 3-specific primer showed that 9 (60%) sequences were identical. The results suggest that (a) a strikingly limited TCR repertoire is expressed in PM muscle; (b) there is a dissociation between the TCR usage of autoinvasive and interstitial T cells; and (c) the autoinvasive T cells are clonally expanded.
Evaluation of Structure and Function of Spleen With Radioactive Tracers
Authors: Henry N. Wagner
Journal: JAMA (1967)

Thevolvement of the spleen is well recognized in some diseases, such as lymphomas, and very likely in others, such as hemolytic anemias. Structural abnormalities, such as enlargement or functional disturbances, such as accelerated removal of red blood cells (RBCs), are often present. The combination of radioactive tracers and external radiation detectors permits measurement of the rate of uptake and spatial distribution of labeled RBCs in the spleen. We can already visualize splenic structure by scanning, and it may soon be possible to evaluate splenic function in a manner similar to the measurement of the rate of uptake of radioactive iodine by the thyroid. The method of labeling RBCs with radioactive chromium (<sup>51</sup>Cr) was discovered in 1950 by Gray and Sterling.<sup>1</sup>This technique made possible extensive study of the fate of normal and abnormal erythrocytes. The technique consists of mixing a few milliliters of venous blood with sodium chromate
INSTRUMENT CASE, STERILIZER, SPONGE BASINS AND TRAYS COMBINED.
Authors: E. C. DUDLEY
Journal: JAMA (1895)

The apparatus herein described is designed to lighten the burden and add to the safety of surgical work in private houses, especially in the country. From an experience of about two years in its use, the author offers it with confidence in place of the septic instrument bags, the unwieldly sterilizer and the cumbersome trays and sponge basins which make up the usual<i>impedimenta</i>of surgical practice outside of hospitals. The apparatus fulfills the requirements, first, of an aseptic instrument case; second, of a steam sterilizer; third, of instrument trays and sponge basins. It consists of two rectangular sterilizers made of copper, nickel plated, in which may be packed all instruments and other appliances requisite for an abdominal section, or for any other ordinary surgical operation. Its component parts may further be used separately as pans, sponge basins and trays. The whole outfit, inclosed in a washable canvas cover, is
Catecholamines, a Dream Sleep Model, and Depression
Authors: TREVOR N. ISKANDER, RUDOLF KAELBLING
Journal: American Journal of Psychiatry (1970)

Chemically reduced catecholamine levels in cats unexpectedly resulted in an increase in paradoxical sleep. This and the probability that catecholamine disturbances are involved in depressive illness led the authors to theorize on the role of paradoxical sleep in the mechanisms maintaining emotions and behavior. A new theoretical model, based on physiological data, is proposed in which paradoxical sleep is considered to provide a release mechanism for the dissipation of the inhibited activity that results from the modification of behavior by discrimination. The authors review some features of sleep and depression in light of these theoretical considerations.
Dysregulated interleukin 6 expression produces a syndrome resembling Castleman's disease in mice.
Authors: S J Brandt, D M Bodine, C E Dunbar, A W Nienhuis
Journal: Journal of Clinical Investigation (1990)

Interleukin 6 (IL-6) is an important regulator of the acute phase response, T cell function, and terminal B cell differentiation. Excessive or inappropriate production of this cytokine may be involved in a variety of autoimmune and neoplastic disorders. To investigate the consequences of dysregulated synthesis of IL-6 in vivo, a high-titer recombinant retroviral vector produced in psi-2 packaging cells was used to introduce the coding sequences of murine IL-6 into mouse hematopoietic cells. Congenitally anemic W/Wv mice reconstituted with bone marrow cells transduced with the retroviral vector developed a syndrome characterized by anemia, transient granulocytosis, hypoalbuminemia, and polyclonal hypergammaglobulinemia, with marked splenomegaly and peripheral lymphadenopathy. Extensive plasma cell infiltration of lymph nodes, spleen, liver, and lung was noted. The similarity of these findings to those of multicentric Castleman's disease, taken together with the observation that lymph nodes from these patients elaborate large amounts of this cytokine, suggest that the inappropriate synthesis of IL-6 has a primary role in the pathogenesis of this systemic lymphoproliferative disorder.
Absence of Detectable HL—A Antigens on Cultured Fibroblasts in Progeria
Authors: Dharam P. Singal, Samuel Goldstein
Journal: Journal of Clinical Investigation (1973)

HL-A phenotypes were determined by a cytotoxicity assay on circulating lymphocytes of two boys with the premature aging syndrome, progeria. The antigenic phenotypes were not unusual inasmuch as they are frequently seen in the normal population. However, none of these antigens could be detected by the same assay on cultured skin fibroblasts from either individual, even when a significant mitotic potential remained before cessation of growth. Fibroblasts from normal donors were concordant with corresponding lymphocytes for HL-A antigens and maintained these antigens until mitotic division had virtually ceased. Absorption studies on fibroblasts with two HL-A2 antisera revealed that HL-A antigens are either absent or have a drastically reduced expression on progeric fibroblasts. The data are in accord with the concept of an immunological role in the pathogenesis of progeria.
Exacerbation of antigen-induced arthritis in urokinase-deficient mice.
Authors: N Busso, V Péclat, K Van Ness, E Kolodziesczyk, J Degen, T Bugge, A So
Journal: Journal of Clinical Investigation (1998)

In rheumatoid arthritis, synovial expression of urokinase (uPA) activity is greatly increased (Busso, N., V. Péclat, A. So, and A. -P. Sappino. 1997. Ann. Rheum. Dis. 56:550- 557). We report the same effect in murine antigen-induced arthritis. uPA-mediated plasminogen activation in arthritic joints may have deleterious effects via degradation of cartilage and bone matrix proteins as well as beneficial effects via fibrin degradation. We evaluated these contrasting effects in vivo by analyzing the phenotype of uPA-deficient (uPA-/-) and control mice during antigen-induced arthritis. Joint inflammation was comparable in both groups up to day 3 and subsequently declined in control mice, remaining significantly elevated in uPA-/- mice on days 10 and 30 after arthritis onset. Likewise, synovial thickness was markedly increased in uPA-deficient mice persisting for up to 2 mo, whereas it subsided in control animals. Bone erosion was exacerbated in uPA-/- mice on day 30. By contrast, no difference in articular cartilage proteoglycan content was found between both groups. Significantly increased accumulation of fibrin was observed by day 30 in arthritic joints of uPA-/- mice. We hypothesized that synovial fibrin deposition plays a role in joint inflammation. Accordingly, defibrinogenation of uPA-/- mice by ancrod significantly decreased the sustained joint inflammation. All the above observations were reproducible in plasminogen-deficient (Pln-/-) mice. In conclusion, synovial fibrin deposition plays a role as a nonimmunological mechanism which sustains chronic arthritis.