tbuckley/cabot-search huggingface dataset:
Diagnostic Delay in Duchenne's Muscular Dystrophy and Cystic Fibrosis
Authors: Beryl J. Rosenstein
Journal: JAMA (1982)

<h3>To the Editor.—</h3> In an analysis of diagnostic delays in patients with Duchenne's muscular dystrophy (DMD), Crisp et al (1982;247:478) found that the average elapsed time between initial parental concern and final diagnosis was 3.0 years. These delays occurred even though massive elevation of serum creatine kinase levels is a reliable biochemical marker for the early detection of DMD. It is of interest to note the similarities between DMD, which is transmitted as an X-linked recessive, and cystic fibrosis (CF), which is transmitted as an autosomal recessive. In the case of both diseases, the basic defect is unknown, there is no specific therapy, a reliable test is available for early diagnosis (sweat test for CF), the course is marked by recurrent pulmonary infections and eventual respiratory failure, and average survival is 20 years. There is also a similarity in diagnostic delays. We have reviewed the records of 194 patients with
Colestipol-Niacin Therapy and Coronary Atherosclerosis
Authors: Samuel J. Mann
Journal: JAMA (1987)

<h3>To the Editor.—</h3> In the article entitled "Beneficial Effects of Combined Colestipol-Niacin Therapy on Coronary Atherosclerosis and Coronary Venous Bypass Grafts," Blankenhorn et al<sup>1</sup>presented exciting data concerning the possible stabilization of and improvement in coronary lesions in patients with a previous history of coronary bypass grafting who were treated with diet and cholesterol-reducing drugs. According to the design of the study, in a prerandomization screening phase, patients who could not tolerate the medications or who failed to achieve a significant lowering of cholesterol level (≥15%) were excluded from the randomized, placebo-controlled study. The number of excluded patients was not given. I believe it is important to note that the patients who took the drugs for two years were very unusual in being motivated to tolerate the high incidence of side effects that were noted. I also believe that they were more likely to follow a strict diet. I
Cynicism Among Medical Students-Reply
Authors: Loretta Kopelman
Journal: JAMA (1984)

<h3>In Reply.—</h3> Dr Oken has misunderstood my view if he believes I argue that the studies cited in "Cynicism Among Medical Students" show that medical students become cynical during their training. By their poor design or lack of adequate consent from students, however, they offer unintentional support for some students' claims that cynicism arises from the lack of attention given to the way important ideals are expressed in practice. This "confirmation" does not come from the studies' findings but from their flaws. Thus, there is no contradiction because I do not hold that the studies'<i>findings</i>confirm that students become more cynical during their training. Rather, I argue that they are too flawed to draw any conclusions. As I say in the article, we do not know "if, how, why, or when attitudes change"<sup>1(p2007)</sup>; I urge that "it is important to study, systematically, if and why students become cynical."
Long-Acting HIV Drug May Be Effective as a Prophylactic Agent
Authors: Tracy Hampton
Journal: JAMA (2014)

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
GADD45A methylation predicts poor overall survival in acute myeloid leukemia and is associated with IDH1/2 and DNMT3A mutations
Authors: M Perugini, D G Iarossi, C H Kok, N Cummings, S M Diakiw, A L Brown, S Danner, P Bardy, L Bik To, A H Wei, I D Lewis, R J D'Andrea
Journal: Leukemia (2012)
Is there a Need for Dual Sphincterotomy in Patients with Chronic Pancreatitis?
Authors: R. Jakobs, J. F. Riemann
Journal: Endoscopy (2003)

Kim et al. raise the interesting question of whether biliary sphincterotomy is mandatory to prevent patients from developing acute cholangitis due to edema after endoscopic pancreatic sphincterotomy (EPS) alone, in patients with chronic pancreatitis.
French's Index of Differential Diagnosis
Authors: J. William Hollingsworth
Journal: JAMA (1986)

<i>French's Index of Differential Diagnosis</i>is a title long familiar to me, but I had never used it or even perused a copy. The book originated with Dr French in 1912, and Dr Dudley Hart edits this 12th edition of the classic London-based text. Basically, in the preface of the first edition, Dr French describes the book as "a treatise on the application of differential diagnosis to all the main signs and symptoms of disease." In 1985, the book's purpose is the same, but with some laboratory tests added as indicators of disease. The contributors are eminent London physicians, surgeons, gynecologists, ophthalmologists, dermatologists, psychiatrists, etc—representing all likely ailments of adults. The format is readily apparent as the book is skimmed. Topics are listed alphabetically. Examples from the subjects I read about: heel, pain in; crackling, eggshell; stridor; Cheyne-Stokes respiration; finger, clubbed; and bearing-down pain. Each of these topics is discussed
Relationships Between Myocardial Bioenergetic and Left Ventricular Function in Hearts With Volume-Overload Hypertrophy
Authors: Jianyi Zhang, Cynthia Toher, Mark Erhard, Yi Zhang, Kamil Ugurbil, Robert J. Bache, Thomas Lange, David C. Homans
Journal: Circulation (1997)

Left ventricular (LV) hypertrophy secondary to volume overload can result in alterations in myocardial bioenergetics and LV dysfunction. This study examined whether bioenergetic abnormalities contribute to the pump dysfunction.Severe mitral regurgitation (MR) was produced in 10 dogs by disruption of the chordal apparatus. Hemodynamics and ventricular function were examined 11.7 months later under baseline conditions and during treadmill exercise. Myocardial high-energy phosphates were measured by using magnetic resonance spectroscopy at rest, during coronary vasodilation with adenosine, and during oxidative stress induced by rapid pacing and dobutamine. Chronic MR caused a 30% increase in LV mass and a 65% increase in LV volume. In MR animals, the hemodynamic and LV function were normal at rest, but abnormalities developed during beta-blockade and exercise. Myocardial creatine phosphate-to-ATP ratios were significantly lower in each layer across the LV wall in MR hearts than normal hearts. Myocardial blood flow and coronary reserve were normal in MR hearts. Moreover, hyperperfusion did not correct the abnormal bioenergetics. Despite altered bioenergetics at rest, the MR hearts tolerated rapid pacing and dobutamine infusion well.In volume-overloaded LV hypertrophied hearts, alterations in myocardial high-energy phosphate levels do not induce abnormal mechanical performance at rest but may be related to a decreased contractile reserve during exercise.
Sore Throats and Tonsillectomy
Authors: Robert J. Haggerty
Journal: New England Journal of Medicine (1978)

"But Doctor, Johnny had seven bouts of sore throat last year. Wouldn't it help to take out his tonsils?" This plaintive cry for help is known to most physicians dealing with children and is the most common reason given to justify the nearly one million tonsillectomies done in this country per year. Even the most anti-tonsillectomy physician (and I count myself as one) has difficulty turning a deaf ear to such a worried mother. The article in this issue of the Journal by Paradise et al. is an important clinical observation to assist perplexed physicians and worried parents faced with . . .
Enhanced Sympathetic and Ventilatory Responses to Central Chemoreflex Activation in Heart Failure
Authors: Krzysztof Narkiewicz, Catherine A. Pesek, Philippe J. H. van de Borne, Masahiko Kato, Virend K. Somers
Journal: Circulation (1999)

Background —Sympathetic activation and respiratory abnormalities may each be implicated in the pathophysiology of congestive heart failure (CHF). Chemoreflexes are an important mechanism regulating both sympathetic drive and breathing. We therefore tested the hypothesis that chemoreflex function is altered in CHF. Methods and Results —We compared ventilatory, sympathetic, heart rate, and blood pressure responses to hypoxia, hypercapnia, and the cold pressor test in 9 patients with CHF and 9 control subjects matched for age and body mass index. Baseline muscle sympathetic nerve activity (MSNA) was higher in the patients with CHF compared with control subjects (47±8 versus 23±3 bursts per minute, P &lt;0.01). During hypercapnia, patients with CHF had greater increases in minute ventilation (6.7±1.4 versus 2.7±0.9 L/min, P =0.03) and heart rate (7.0±2.1 versus 0.6±1.2 bpm, P =0.02). Despite higher ventilation, which inhibits sympathetic activity, the MSNA increase in patients with CHF was also greater than that in control subjects (58±12% versus 21±9%, P =0.03). Ventilatory, autonomic, and blood pressure responses to hypoxia and the cold pressor test in CHF patients were not different from those in control subjects. Conclusions —Chronic heart failure is characterized by a selective potentiation of ventilatory and sympathetic responses to central chemoreceptor activation by hypercapnia.
Give a disease a bad name
Authors: H V Wyatt
Journal: BMJ (2004)

HELLP syndrome post partum, there are no concerns about harm to the fetus.In treatment antepartum, the duration of corticosteroid treatment is relatively brief, and the time to delivery should be rapid.The complications from repeated corticosteroid use should therefore not be an issue.Given the preponderance of current evidence, we recommend that women with HELLP syndrome both antepartum and post partum should be given two doses of dexamethasone 10 mg 12 hours apart followed by 5 mg at 24 and 36 hours.The results of the Cochrane systematic review should further clarify this issue.
A missense mutation in the cholesteryl ester transfer protein gene with possible dominant effects on plasma high density lipoproteins.
Authors: K Takahashi, X C Jiang, N Sakai, S Yamashita, K Hirano, H Bujo, H Yamazaki, J Kusunoki, T Miura, P Kussie
Journal: Journal of Clinical Investigation (1993)

Plasma HDL are a negative risk factor for atherosclerosis. Cholesteryl ester transfer protein (CETP; 476 amino acids) transfers cholesteryl ester from HDL to other lipoproteins. Subjects with homozygous CETP deficiency caused by a gene splicing defect have markedly elevated HDL; however, heterozygotes have only mild increases in HDL. We describe two probands with a CETP missense mutation (442 D:G). Although heterozygous, they have threefold increases in HDL concentration and markedly decreased plasma CETP mass and activity, suggesting that the mutation has dominant effects on CETP and HDL in vivo. Cellular expression of mutant cDNA results in secretion of only 30% of wild type CETP activity. Moreover, coexpression of wild type and mutant cDNAs leads to inhibition of wild type secretion and activity. The dominant effects of the CETP missense mutation during cellular expression probably explains why the probands have markedly increased HDL in the heterozygous state, and suggests that the active molecular species of CETP may be multimeric.
HRT no longer first choice for preventing osteoporosis
Authors: D. Singh
Journal: BMJ (2003)

Hormone replacement therapy (HRT) should no longer be the treatment of choice for preventing osteoporosis in women aged over 50, the United Kingdom's regulatory agency on medicines and devices has advised.

In addition, women with severe menopausal symptoms should be prescribed only the “minimal effective dose… for the shortest duration,” recommended the Medicines and Health-care Products Regulatory Agency, last week.

The move follows a Europe-wide review of …
Making Optimum Use of the Microbiology Laboratory
Authors: Raymond C. Bartlett
Journal: JAMA (1982)

UNIQUE pitfalls may be encountered by the physician in making optimum use of the microbiology laboratory. A request for a "culture" is nonspecific compared with a request for a blood glucose determination. The request for a culture is an implied request to search for "anything in the specimen that could contribute to the diagnosis and treatment of an infectious disease." Physicians usually do not know exactly how this request will be construed by laboratory personnel. What would happen if blood were submitted to the chemistry laboratory with a comparable request for "chemical abnormalities" that might suggest the presence of disease? What microorganisms will the laboratory seek in a specimen submitted for "culture"? Will a Gram-stained direct smear be performed, and if so, will the results of this examination be reported? The request for culture usually leads to a search for bacteria that would commonly be expected at the site represented.
Potential for the Transmission of HIV-1 despite Highly Active Antiretroviral Therapy
Authors: Ashley T. Haase, Timothy W. Schacker
Journal: New England Journal of Medicine (1998)

The use of highly active antiretroviral therapy to control infection with human immunodeficiency virus type 1 (HIV-1) is in many ways a success story. Under optimal circumstances, combinations of drugs that inhibit the viral protease and reverse transcriptase suppress replication to below the limits of detection in the circulation. The production of the virus is stifled at its source in lymphatic tissues, and the large pools of virus stored in that reservoir are reduced to undetectable levels.1,2 The control of viral replication benefits the ravaged immune system, even in the late stages of infection. The numbers of CD4+ T . . .
OK, OK, OK: The Facts of Kew—More on Squatter's Palsy
Authors: William C. Caccamise
Journal: JAMA (1987)

<h3>To the Editor.—</h3> Dr Kumaki's<sup>1</sup>conclusion that it was probable that his patient's foot drop was causally related to the use of a squat-type toilet should not be included in the facts of Kathmandu without at least some reservation. The patient presented with a history of ten to 15 bowel movements daily. The use of the squat-type toilet was probably maximal at that time, but the foot drop did not appear until eight days later, when the patient's dysentery was improving. Apparently, Dr Kumaki did not consider that the dysentery itself might have caused the palsy. Textbook accounts of severe dysentery caused by<i>Shigella</i><sup>2</sup>regularly mention that one of the unusual complications of this disease may be a palsy—most probably due to toxic neuritis. This possibility must be considered in this patient. Two types of common peroneal nerve palsy have been described—acute and tardy.<sup>3</sup>The acute type
Splenectomy for Pure Red-Cell Hypoplastic (Aregenerative) Anemia Associated with Autoimmune Hemolytic Disease
Authors: Gustave Eisemann, William Dameshek
Journal: New England Journal of Medicine (1954)

THIS report deals with an apparently unique case in which there was simultaneously selective red-cell aplasia of the bone marrow and an autoimmune hemolytic mechanism. The combination of continued lack of erythrocyte production with excessive blood destruction eventually led to the need for weekly blood transfusions, which were made increasingly difficult by thrombosed veins and the development of transfusion reactions. After a period during which there was complete absence of reticulocytes in the blood and of nucleated red cells in the marrow, splenectomy was performed. No response whatever took place for seventeen days, but on the eighteenth day, the first . . .
Control of Postprandial Hyperglycemia
Authors: Mary F. Carroll, Ahmad Izard, Katrina Riboni, Mark R. Burge, David S. Schade
Journal: Diabetes Care (2002)

OBJECTIVE—This study was designed to compare the efficacy of acute premeal administration of glipizide versus nateglinide in controlling postprandial hyperglycemia in subjects with non-insulin-requiring type 2 diabetes. RESEARCH DESIGN AND METHODS—A total of 20 subjects (10 female, 10 male) with non-insulin-requiring type 2 diabetes were admitted overnight to the General Clinical Research Center on four occasions. In random order, 10 mg glipizide (30 min premeal), 120 mg nateglinide (15 min premeal), 10 mg glipizide plus nateglinide (30 and 15 min premeal, respectively), or placebo pills (30 and 15 min premeal) were administered in a double-blind fashion before a standardized breakfast. Blood was drawn for analysis of glucose, insulin, and C-peptide at −0.05, 0, 0.5, 1, 2, 3, and 4 h relative to the meal. RESULTS—The subjects were aged 56 ± 2 years and were moderately obese (BMI 31 ± 1 kg/m2), with a mean HbA1c of 7.4 ± 0.4%. The peak postprandial glucose excursion above baseline was higher with placebo (6.1 ± 0.5 mmol/l) than glipizide (4.3 ± 0.6 mmol/l, P = 0.002), nateglinide (4.2 ± 0.4 mmol/l, P = 0.001), or glipizide plus nateglinide (4.1 ± 0.5 mmol/l, P = 0.001). The area under the curve for the glucose excursion above baseline was also higher with placebo (14.1 ± 1.8 mmol/h · l) compared with glipizide (6.9 ± 2.4 mmol/h · l, P = 0.002), nateglinide (9.7 ± 2 mmol/h · l, P = 0.004), or glipizide plus nateglinide (5.6 ± 2.2 mmol/h · l, P &amp;lt; 0.001). Peak and integrated glucose excursions did not differ significantly between glipizide and nateglinide. However, by 4 h postmeal, plasma glucose levels were significantly higher with nateglinide (9 ± 0.9 mmol/l) compared with the premeal baseline (7.8 ± 0.6 mmol/l, P = 0.04) and compared with the 4-h postprandial glucose level after administration of glipizide (7.6 ± 0.6 mmol/l, P = 0.02). Integrated postprandial insulin levels were higher with glipizide (1,556 ± 349 pmol/h · l) than nateglinide (1,364 ± 231 pmol/h · l; P = 0.03). Early insulin secretion, as measured by insulin levels at 30 min postmeal, did not differ between glipizide and nateglinide. CONCLUSIONS—Acute premeal administration of nateglinide or glipizide has equal efficacy in controlling postbreakfast hyperglycemia in type 2 diabetes when each drug is administered at the optimum time before the meal. Glipizide causes a more pronounced and sustained postmeal insulin secretory response compared with nateglinide. Glipizide facilitates the return to near-fasting glucose levels at 4 h postmeal, but with the possible risk of increased frequency of postmeal hypoglycemia in drug-naive patients. The clinical decision to use glipizide versus nateglinide should be based on factors other than the control of postprandial hyperglycemia in type 2 diabetes.
Kwashiorkor and marasmus are both associated with impaired glucose clearance related to pancreatic β-cell dysfunction
Authors: Martijn N. Spoelstra, Andrea Mari, Marijke Mendel, Edward Senga, Patrick van Rheenen, Theo H. van Dijk, Dirk-Jan Reijngoud, Remco G.T. Zegers, Geert Tom Heikens, Robert H.J. Bandsma
Journal: Metabolism (2012)
Accuracy of Blood Glucose Estimation by Children With IDDM and Their Parents
Authors: Linda A Gonder-Frederick, Andrea L Snyder, William L Clarke
Journal: Diabetes Care (1991)

Objective To evaluate the accuracy of blood glucose symptom recognition and subjective blood glucose estimation in insulin-dependent diabetic (IDDM) children and their parents. Research Design and Methods Blood glucose estimation questionnaires were completed 4 times/day at home during routine activities. A sequential sample of 19 families, who attended a pediatric diabetes clinic, with IDDM children &amp;lt;2265; 12 yr old and IDDM duration of &amp;gt; or equal to 9 mo comprised the study. Results Error grid analysis showed that both children and parents demonstrated poor accuracy, making clinically significant errors as frequently as clinically accurate estimates. The most common error was the failure to detect extreme blood glucose levels, with a significant tendency to underestimate hyperglycemia. Children often reported hypoglycemia when blood glucose was hyperglycemic. Confidence in the ability to estimate blood glucose was unrelated to measured accuracy. Conclusions IDDM children and their parents demonstrated a higher rate of blood glucose estimation errors than IDDM adolescents and adults in previous studies. Even in families who use self-monitoring of blood glucose frequently, self-reported ability to recognize symptoms and estimate blood glucose should be viewed with caution. Families with IDDM children need more education about errors in symptom recognition and blood glucose estimation. They should also be encouraged to use self-monitoring of blood glucose before treating children's reported hypoglycemic symptoms whenever possible.
Mechano-oxidative coupling by mitochondria induces proinflammatory responses in lung venular capillaries
Authors: Hideo Ichimura, Kaushik Parthasarathi, Sadiqa Quadri, Andrew C. Issekutz, Jahar Bhattacharya
Journal: Journal of Clinical Investigation (2003)

Elevation of lung capillary pressure causes exocytosis of the leukocyte adhesion receptor P-selectin in endothelial cells (ECs), indicating that lung ECs generate a proinflammatory response to pressure-induced stress. To define underlying mechanisms, we followed the EC signaling sequence leading to P-selectin exocytosis through application of real-time, in situ fluorescence microscopy in lung capillaries. Pressure elevation increased the amplitude of cytosolic Ca(2+) oscillations that triggered increases in the amplitude of mitochondrial Ca(2+) oscillations and in reactive oxygen species (ROS) production. Responses to blockers of the Ca(2+) oscillations and of mitochondrial electron transport indicated that the ROS production was Ca(2+) dependent and of mitochondrial origin. A new proinflammatory mechanism was revealed in that pressure-induced exocytosis of P-selectin was inhibited by both antioxidants and mitochondrial inhibitors, indicating that the exocytosis was driven by mitochondrial ROS. In this signaling pathway mitochondria coupled pressure-induced Ca(2+) oscillations to the production of ROS that in turn acted as diffusible messengers to activate P-selectin exocytosis. These findings implicate mitochondrial mechanisms in the lung's proinflammatory response to pressure elevation and identify mitochondrial ROS as critical to P-selectin exocytosis in lung capillary ECs.