tbuckley/cabot-search huggingface dataset:
Expression and Phenotype Analysis of the Nephrocystin-1 and Nephrocystin-4 Homologs in Caenorhabditis elegans
Authors: Matthias T.F. Wolf, Jeeyong Lee, Franziska Panther, Edgar A. Otto, Kun-Liang Guan, Friedhelm Hildebrandt
Journal: Journal of the American Society of Nephrology (2005)

Nephronophthisis (NPHP), an autosomal-recessive cystic kidney disease, is the most frequent genetic cause of end-stage renal failure in children. NPHP types 1 and 4 are caused by mutations in NPHP1 and NPHP4, encoding the proteins nephrocystin-1 and nephrocystin-4, respectively. Nephrocystin-1 and nephrocystin-4 are expressed in primary cilia of renal epithelial cells. NPHP1 and NPHP4 are highly conserved in Caenorhabditis elegans. However, this species does not have a kidney but an excretory system that consists of an excretory cell, an excretory gland cell, a duct cell, and a pore cell. Therefore, cell type-specific expression pattern and function of the nephrocystin homologs in C. elegans were of interest. Expression of green fluorescence protein fusion constructs that contain the C. elegans promoter regions for nph-1 and nph-4 was not found in the excretory system but in ciliated sensory neurons of the head (amphid neurons) and the tail in hermaphrodites (phasmid neurons) and males (sensory ray neurons). As the knockout phenotype for the PKD homologs lov-1 and pkd-2 shows impaired male mating behavior, RNAi knockdown animals were analyzed for this phenotype. A similar phenotype was found in the nph-1 and nph-4 RNAi knockdown animals compared with the lov-1 and pkd-2 knockout phenotype. Thus, it is suggested that renal cyst-causing genes may be part of a shared functional module, highly conserved in evolution. The NPHP homologs may be necessary for initial assembly of the cilium, whereas the polycystic kidney disease homologs may function as sensory transducers.
Nobody's Fault
Authors: Ellen R. Strahlman
Journal: JAMA (1987)

The call came at 1 AM. My drowsiness disappeared as the tentative voice of the first-year resident informed me: "I think I have a lady here with postop endophthalmitis." "How long ago was the surgery?" "Five days," he said, "so I guess she needs to be admitted." I nodded my assent in reply, as if he were in my presence. "She'll need a diagnostic tap tonight. Make sure she's NPO, start IV and topical antibiotics, and call the OR. I'm on my way." Postoperative endophthalmitis. Words an ophthalmologist dreads, loses sleep over, prays will never happen. The most devastating complication of ophthalmic surgery, surgery designed to restore vision, not take it away. I found Mrs Lang in our emergency room, quiet and alert, attended by the one of her three daughters who lives with her. A petite woman, she moved easily to the slit lamp for examination. "We will do
Risk of death from heart attack varies according to when patients arrive at hospital, study finds
Authors: David Spurgeon
Journal: BMJ (2005)

Restoration of normal blood flow is delayed and risk of death is higher for some heart attack patients if they arrive at hospital between 5pm and 7am or on a weekend, a new study shows.

This is the finding of a study from the US healthcare organisation Kaiser Permanente, headed by Dr. David J. Magid, Clinical Research Unit, Kaiser Permanente Denver. It looked at data on 68 439 patients with an electrocardiography finding known as an ST-segment elevation myocardial infarction who were treated with fibrinolytic drugs at 1015 hospitals across the country and 33 647 patients with the same electrocardiography finding who were treated with …
Autoimmunity: a risk factor for allosensitisation to blood products?
Authors: A. S. Wierzbicki, D. M. Cousins
Journal: BMJ (1989)

3 observations de transfusions (sang, hematies etc.) ayant amene la formation de nombreux iso-anticorps persistants chez des malades atteints de troubles auto-immuns (vitiligo, hypothyroidisme, anemie) et traites par prednisolone
THE AFTER-EFFECTS OF DIPHTHERIA ON THE HEART.
Authors: FRANKLIN W. WHITE
Journal: JAMA (1905)

It is well known that in diphtheria the heart muscle and the nerves which control it are the seat of certain changes which more or less seriously disturb its function, and the clinical signs of circulatory disturbance at the height of the disease and during convalescence have been carefully observed by many authorities. Much less is known, however, of the after-effects of the poison of diphtheria on the heart. Veronese<sup>1</sup>says that lasting effects on the heart—ex-except a moderate hypertrophy—do not follow diphtheria. Romberg<sup>2</sup>has seen the mitral insufficiency present in convalescence in several cases last several months and then disappear. Steffen<sup>3</sup>reports a case of mitral insufficiency following diphtheria which has been under observation for over four and a half years, and which he considers not a case of relative insufficiency, but of genuine valvular disease. Baginsky<sup>4</sup>says that the disturbance of the heart in
Research on Children
Authors: William J. Curran
Journal: New England Journal of Medicine (1978)

IT has been four or five years since the Department of Health, Education, and Welfare began working on new regulations for the control of medical and behavioral research on children. Now, a definitive stage seems to have been reached. A complete set of proposed new regulations has been issued.1 These provisions are the legally required response to the earlier recommendations of the National Commission for the Protection of Human Subjects.2 In large measure, the Department has accepted the Commission's recommendations, but there are important areas of difference and compromise.During the entire 1970's the Department has been under heavy pressure . . .
Abstract P5-13-09: Evaluation on the risk factors and prognosis for patients with synchronous bilateral breast cancer in a Chinese cohort
Authors: J-J Chen, Y Wang, J-Y Xue, B-L Yang, S Huang, Y Chen, J-Y Chen, W-T Yang, Z-M Shao, J Wu
Journal: Cancer Research (2013)

Abstract Background: There has been an increasing rate of synchronous bilateral breast cancers, mainly because of the increased morbidity, improved detection abilities, better prognosis and higher life expectancy of breast cancer patients. However, evidences in the literature regarding the risk factors of bilateral breast cancers are scare, and the survival of synchronous bilateral breast cancers still remain controversies. The purpose of the present study is to investigate the risk factors and prognosis of bilateral breast cancers in a Chinese cohort. Methods: The present study included 181 patients with synchronous bilateral breast cancer and 10,831 patients with unilateral breast cancer treated in Fudan University Shanghai Cancer Center between Jan 2007 and Dec 2012. Patients with metastases or inflammatory diseases, or metachronous bilateral breast cancer (after 6 months of first cancer) were excluded. Age of the cases, family history, menopausal status, histology types, pathological stages, types of accompanied benign lesions, and patterns of surgeries and adjuvant therapies were examined to investigate the risk factors of bilateral breast cancers. Results: Patients with synchronous bilateral breast cancer were significantly elder than those with unilateral breast cancer (mean age 53 yrs vs. 51yrs, p&amp;lt;0.001). Presence of sclerosing adenoma and lobular carcinoma component were the two independent risk factors of early-stage synchronous bilateral breast cancer, with the relative risk rate at 5.52 (95% CI: 3.04-10.01) and 5.00 (95% CI: 2.64-9,47), respectively. The rate of in situ carcinoma with or without microinvasion was significantly higher in patients with sclerosing adenoma associated synchronous bilateral breast cancers at 64.3%. With a median follow-up of 35 months, the disease free survival for synchronous bilateral breast cancer was 95.0%, similar to that of unilateral breast cancer. However, for synchronous bilateral breast cancer patients with N3 disease or previously received neoadjuvant therapy (20 cases), the 1.5 year-disease free survival was merely 50%, significantly worse than that of unilateral breast cancer with N3 disease or previous neoadjuvant therapy. Conclusion: The rate of synchronous bilateral breast cancer was higher in patients with sclerosing adenosis or lobular carcinoma component. The prognosis for early-stage synchronous bilateral breast cancer was similar to those with early-stage unilateral breast cancer. However, among patients with N3 disease or previously received neoadjuvant therapy, synchronous bilateral cohort was associated to worse survival compared with unilateral cohort. Citation Information: Cancer Res 2013;73(24 Suppl): Abstract nr P5-13-09.
Atrioventricular sequential pacing: differential effect on retrograde conduction related to level of impulse collision.
Authors: R Mahmud, M Lehmann, S Denker, C J Gilbert, M Akhtar
Journal: Circulation (1983)

Patients with DDD pacemakers who have intact retrograde conduction are known to be at risk of developing ventricular and "endless loop" tachycardia. To address this problem, a pacing protocol was designed in which V2A2 conduction was assessed in 16 patients during ventricular pacing alone (standard method) and during paced atrioventricular (AV) sequential drive (AV sequential method); the results were then compared. In eight of 16 patients who had intact retrograde conduction with both methods (group 1), the V2A2 intervals were significantly shorter (by 60 to 340 msec) with the AV sequential method. In the remaining eight patients, who demonstrated V2A2 block with the standard method, no V2A2 block occurred with the AV sequential method. In this study, two sets of AV intervals were programmed to produce collision of the two impulses (atrial and ventricular), either in the AV node or the His-Purkinje system (HPS). The site of V2A2 facilitation was related to the site of impulse collision. These results can be explained by earlier excitation by the atrial impulse (of AV node and/or HPS) during AV sequential pacing. However, in some cases it was evident that antegrade propagation of the atrial impulse was responsible for subsequent facilitation. The data suggest that assessment of retrograde conduction in candidates for DDD pacemakers can be made most accurately by the AV sequential method.
Migraine and Epilepsy
Authors: Fritz E. Dreifuss
Journal: JAMA (1987)

In<i>Borderland of Epilepsy</i>, Gowers prefaces the section on migraine with the remark, "Some surprise may be felt that migraine is given a place in the Borderland of Epilepsy but the position is justified by many relations, and among them by the fact that the two maladies are sometimes mistaken, and more often their distinction is difficult." Later he states, "The most frequent relation of migraine to epilepsy is as a source of error." But he does allow that "in extremely rare instances one affection may develop while the other goes on, and, as we have seen, the same premonitory disturbance may even be attached to each." This book pulls together in a single volume a wealth of information that illuminates a corner of the realm of the Borderland of Epilepsy. It is badly needed. Since the days of Gowers, tantalizing overlapping relationships have been described between migraine and epilepsy,
Viral Vector–mediated Delivery of Estrogen Receptor-α to the Hippocampus Improves Spatial Learning in Estrogen Receptor-α Knockout Mice
Authors: Thomas C Foster, Asha Rani, Ashok Kumar, Li Cui, Susan L Semple-Rowland
Journal: Molecular Therapy (2008)

Estrogen, which influences both classical genomic and rapid membrane-associated signaling cascades, has been implicated in the regulation of hippocampal function, including spatial learning. Gene mutation studies suggest that estrogen effects are mediated by estrogen receptor-alpha (ER-alpha); however, because gonadal steroids influence the organization of the hippocampus during development, it has been difficult to distinguish developmental effects from those specific to adults. In this study we show that lentiviral delivery of the gene encoding ER-alpha to the hippocampus of adult ER-alpha-knockout (ER-alphaKO) mice restores hippocampal responsiveness to estrogen and rescues spatial learning. We propose that constitutive estrogen receptor activity is important for maintaining hippocampus-dependent memory function in adults.
Mechanisms of Abnormal Calcium Homeostasis in Mutations Responsible for Catecholaminergic Polymorphic Ventricular Tachycardia
Authors: Vivek Iyer, Roger J. Hajjar, Antonis A. Armoundas
Journal: Circulation Research (2007)

Catecholaminergic polymorphic ventricular tachycardia is a heritable arrhythmia unmasked by exertion or stress and is characterized by triggered activity and sudden cardiac death. In this study, we simulated mutations in 2 genes linked to catecholaminergic polymorphic ventricular tachycardia, the first located in calsequestrin (CSQN2) and the second in the ryanodine receptor (RyR2). The aim of the study was to investigate the mechanistic basis for spontaneous Ca2+ release events that lead to delayed afterdepolarizations in affected patients. Sarcoplasmic reticulum (SR) luminal Ca2+ sensing was incorporated into a model of the human ventricular myocyte, and CSQN2 mutations were modeled by simulating disrupted RyR2 luminal Ca2+ sensing. In voltage-clamp mode, the mutant CSQN2 model recapitulated the smaller calcium transients, smaller time to peak calcium transient, and accelerated recovery from inactivation seen in experiments. In current clamp mode, in the presence of beta stimulation, we observed delayed afterdepolarizations, suggesting that accelerated recovery of RyR2 induced by impaired luminal Ca2+ sensing underlies the triggered activity observed in mutant CSQN2-expressing myocytes. In current-clamp mode, in a model of mutant RyR2 that is characterized by reduced FKBP12.6 binding to the RyR2 on beta stimulation, the impaired coupled gating characteristic of these mutations was modeled by reducing cooperativity of RyR2 activation. In current-clamp mode, the mutant RyR2 model exhibited increased diastolic RyR2 open probability that resulted in formation of delayed afterdepolarizations. In conclusion, these minimal order models of mutant CSQN2 and RyR2 provide plausible mechanisms by which defects in RyR2 gating may lead to the cellular triggers for arrhythmia, with implications for the development of targeted therapy.
Giant Splenomegaly in Sarcoidosis Demonstrated by Radionuclide Scintiphotography
Authors: Robert W. Burt
Journal: JAMA (1971)

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 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) Podcasts Clinical Reviews Editors' Summary Medical News Author Interviews More 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
The Essential Role of Atrioventricular Conduction Delay in the Initiation of Paroxysmal Supraventricular Tachycardia
Authors: BRUCE N. GOLDREYER, ANTHONY N. DAMATO
Journal: Circulation (1971)

Studies have shown that in patients with paroxysmal supraventricular tachycardia (SVT), spontaneous or stimulated atrial premature depolarizations (APD) falling within a specific portion of the relative refractory period of the atrioventricular (A-V) conduction system initiate SVT. The present study was designed to determine whether the A-V nodal conduction delay these APDs exhibit, or their coupling interval, is essential for the initiation of SVT. Multiple episodes of SVT were initiated and terminated by single APDs in four patients with a history of paroxysmal SVT. The atria were then paced at numerous fixed rates in excess of the spontaneous sinus rate. In each patient, at atrial rates where progressive A-V nodal conduction delay occurred from beat to beat (Wenckebach cycles), a specific degree of A-V nodal conduction delay always resulted in atrial reentry or SVT. Although the atrial coupling interval was considerably longer during Wenckebach cycles initiating SVT than for single APDs resulting in the arrhythmia, the prolongation of A-V nodal conduction was identical. Independent of atrial coupling interval, a requisite degree of A-V nodal conduction delay always resulted in atrial echoes or SVT. This supports the conclusion that SVT results from atrial reentry via the A-V node.
Abnormalities in Plasma and Cerebrospinal-Fluid Arginine Vasopressin in Patients with Anorexia Nervosa
Authors: Philip W. Gold, Walter Kaye, Gary L. Robertson, Michael Ebert
Journal: New England Journal of Medicine (1983)

Previous studies have indicated that many patients with anorexia nervosa have defects in urinary concentration or dilution suggestive of abnormal secretion of the antidiuretic hormone arginine vasopressin. To explore this possibility, we examined the response of plasma vasopressin to intravenous hypertonic saline in anorexic patients before and after correction of their weight loss. We also measured basal levels of the hormone in the cerebrospinal fluid. In all four subjects studied before correction of weight loss, the response to hypertonic saline was abnormal: in one, the plasma level of arginine vasopressin increased subnormally relative to the plasma sodium level; in the other three, it fluctuated erratically, with no relation to plasma sodium. These defects persisted in the three patients studied three to four weeks after recovery of body weight. In two patients who were initially studied when they were underweight, the defects were gone six months after recovery; in five of seven other patients studied at least six months after recovery but not while they were underweight, the response was normal. Abnormalities in the osmoregulation of plasma arginine vasopressin were not accounted for by nonosmotic stimuli and were almost always associated with an absolute increase in the level of arginine vasopressin in the cerebrospinal fluid or a reversal of the normal (less than 1.0) cerebrospinal fluid/plasma ratio of arginine vasopressin. These results indicate that most if not all patients with anorexia nervosa have abnormal levels of arginine vasopressin in their plasma and cerebrospinal fluid that are corrected very slowly with weight gain. The cause and consequences of these abnormalities remain to be determined.
Vascular Reactivity in Sepsis: Importance of Controls and Role of Nitric Oxide
Authors: Asma Yaghi, Nigel A.M. Paterson, D.G. McCormack
Journal: American Journal of Respiratory and Critical Care Medicine (1995)

We have previously reported differential impairment of pulmonary and systemic vascular contractility in hyperdynamic sepsis. The objectives of this study were (1) to determine whether the magnitude of this phenomenon depends on the control group chosen for comparison, and (2) to examine the role of nitric oxide (NO) in this altered vascular contractility. Rats were randomized to sepsis induced by cecal ligation and perforation (CLP) or to one of two control procedures. The Sepsis group had a jugular venous line for fluid administration, laparotomy, and CLP. Control group 1 (Control) had only a jugular venous line inserted, while group 2 (Sham) had a jugular venous line inserted and an abdominal incision. All rats were killed 24 h after surgery. Vascular contractility of small pulmonary arterial and thoracic aortic rings was assessed in vitro by obtaining cumulative dose-response curves to the contractile agonists potassium chloride (KCI), phenylephrine (PE), and prostaglandin F2α (PGF2α). Pulmonary vessels from animals in the Sepsis and Sham groups exhibited significant attenuation of the contractile responses to KCI, PE, and PGF2α compared with the Control group. In contrast, contractility of the aortic rings to KCI, PE, and PGF2α was not significantly different in the three groups studied. Incubation of pulmonary and aortic vessels with NG-nitro-L-argine methyl ester (L-NAME, 10 µM) caused an increase in the response to KCI, PE, and PGF2α in pulmonary vessels in Sepsis and Sham rats but not in Control rats. We concluded that (1) sepsis affects vascular contractility differently in vessels from different vascular beds; (2) incision of the abdomen has a direct effect on vascular reactivity and may therefore not be an appropriate control for studies of abdominal sepsis; and (3) the effect of L-NAME on contractile responses of pulmonary artery rings supports the hypothesis that NO synthesis is induced in the pulmonary circulation in sepsis.