tbuckley/cabot-search huggingface dataset:
Localized Disorders in Myocardial Contraction
Authors: Michael V. Herman, Robert A. Heinle, Michael D. Klein, Richard Gorlin, Eric Hammond, Nelson Tsao-Wu, Richard Levy
Journal: New England Journal of Medicine (1967)

CONGESTIVE heart failure has usually been considered to be a global affection of the myocardium in which disturbance of contraction in one or both ventricles is generalized. On the other hand, abnormal myocardial contraction may be caused by local areas of malfunctioning myocardium interacting with other areas of completely normal muscle. The possibility that unco-ordinated contraction of the heart results from such a combination of normal and abnormal muscle has received little attention.In 1925 Wiggers1 described the pattern of left ventricular contraction as a "series of sequential fractionate contractions of muscle bundles." He suggested that disturbance in this temporal . . .
Intragenic Deletion of the<i>KALIG-1</i>Gene in Kallmann's Syndrome
Authors: David Bick, Brunella Franco, Richard J. Sherins, Babette Heye, Lisa Pike, John Crawford, Anne Maddalena, Barbara Incerti, Antonella Pragliola, Thomas Meitinger, Andrea Ballabio
Journal: New England Journal of Medicine (1992)

KALLMANN'S SYNDROME is an inherited disorder characterized by hypogonadism and anosmia.1 The hypogonadism is due to deficiency of gonadotropin-releasing hormone (GnRH),2 and the anosmia is due to hypoplasia or aplasia of the olfactory bulbs and tracts.3 , 4 In addition to hypogonadotropic hypogonadism and anosmia, other abnormalities may be present in patients with Kallmann's syndrome. They include neurologic deficits such as synkinesia,1 , 5 spatial attentional abnormalities,6 spastic paraplegia,7 cerebellar dysfunction, gaze-evoked horizontal nystagmus, pes cavus,5 , 8 mental retardation,9 color-vision disturbance and hearing loss,10 and other somatic defects, such as unilateral renal agenesis,9 horseshoe kidney,11 cryptorchidism,12 and cleft lip and palate.10 Several of these abnormalities . . .
Focal Photodamage on the Occipital Scalp
Authors: Howa Yeung, Kevin Man-Hin Luk, Suephy C. Chen
Journal: JAMA Dermatology (2016)

differences in hair coverage. 3,5The natural photoprotective effect offered by hair is quantitated as exponential to scalp hair density. 5Therefore, patients with alopecia should be counseled on using additional sun protection by wearing hats with complete coverage for the scalp; applying SPF 30+ sunscreen lotion, spray or stick; or using handheld umbrellas to further minimize exposure to UV radiation.Since baseball caps remain a popular style among US adults, the general public, particularly men at risk of developing androgenetic alopecia, should be made aware of their shortcomings in photoprotection and should be counseled to use additional means to protect against skin cancer development, especially on the scalp.
Intracellular tortuosity underlies slow cAMP diffusion in adult ventricular myocytes
Authors: Mark Richards, Oliver Lomas, Kees Jalink, Kerrie L. Ford, Richard D. Vaughan-Jones, Konstantinos Lefkimmiatis, Pawel Swietach
Journal: Cardiovascular Research (2016)

3′,5′-Cyclic adenosine monophosphate (cAMP) signals in the heart are often confined to concentration microdomains shaped by cAMP diffusion and enzymatic degradation. While the importance of phosphodiesterases (degradative enzymes) in sculpting cAMP microdomains is well established in cardiomyocytes, less is known about cAMP diffusivity (DcAMP) and factors affecting it. Many earlier studies have reported fast diffusivity, which argues against sharply defined microdomains. [cAMP] dynamics in the cytoplasm of adult rat ventricular myocytes were imaged using a fourth generation genetically encoded FRET-based sensor. The [cAMP]-response to the addition and removal of isoproterenol (β-adrenoceptor agonist) quantified the rates of cAMP synthesis and degradation. To obtain a read out of DcAMP, a stable [cAMP] gradient was generated using a microfluidic device which delivered agonist to one half of the myocyte only. After accounting for phosphodiesterase activity, DcAMP was calculated to be 32 µm2/s; an order of magnitude lower than in water. Diffusivity was independent of the amount of cAMP produced. Saturating cAMP-binding sites with the analogue 6-Bnz-cAMP did not accelerate DcAMP, arguing against a role of buffering in restricting cAMP mobility. cAMP diffused at a comparable rate to chemically unrelated but similar sized molecules, arguing for a common physical cause of restricted diffusivity. Lower mitochondrial density and order in neonatal cardiac myocytes allowed for faster diffusion, demonstrating the importance of mitochondria as physical barriers to cAMP mobility. In adult cardiac myocytes, tortuosity due to physical barriers, notably mitochondria, restricts cAMP diffusion to levels that are more compatible with microdomain signalling.
Percutaneous Transluminal Coronary Angioplasty
Authors: C Landau, R A Lange, L D Hillis
Journal: New England Journal of Medicine (1994)

The nonsurgical treatment of arteries narrowed by atherosclerosis was introduced in 1964, when Dotter and Judkins performed transluminal angioplasty of femoral arterial stenoses1. In the 1970s, Gruntzig modified the dilation catheter to allow its use in coronary arteries,2 and in September 1977 he performed the first percutaneous transluminal coronary angioplasty (PTCA) in a patient. PTCA has since been used in many patients with stable angina, unstable angina, or acute myocardial infarction. Its use was initially limited to the treatment of discrete stenoses in proximal segments of a coronary artery, but improvements in equipment and technique have led to its . . .
Increased High-Density Lipoprotein Levels Caused by a Common Cholesteryl-Ester Transfer Protein Gene Mutation
Authors: Akihiro Inazu, Maryanne L. Brown, Charles B. Hesler, Luis B. Agellon, Junji Koizumi, Koki Takata, Yoshisuke Maruhama, Hiroshi Mabuchi, Alan R. Tall
Journal: New England Journal of Medicine (1990)

The plasma cholesteryl-ester transfer protein (CETP) catalyzes the transfer of cholesteryl esters from high-density lipoprotein (HDL) to other lipoproteins. We recently described a Japanese family with increased HDL levels and CETP deficiency due to a splicing defect of the CETP gene. To assess the frequency and phenotype of this condition, we screened 11 additional families with high HDL levels by means of a radioimmunoassay for CETP and DNA analysis.We found the same CETP gene mutation in four families from three different regions of Japan. Analysis of restriction-fragment-length polymorphisms of the mutant CETP allele showed that all probands were homozygous for the identical haplotype. Family members homozygous for CETP deficiency (n = 10) had moderate hypercholesterolemia (mean total cholesterol level [+/- SD], 7.01 +/- 0.83 mmol per liter), markedly increased levels of HDL cholesterol (4.24 +/- 1.01 mmol per liter) and apolipoprotein A-I, and decreased levels of low-density lipoprotein cholesterol (1.99 +/- 0.80 mmol per liter) and apolipoprotein B. Members heterozygous for the deficiency (n = 20), whose CETP levels were in the lower part of the normal range, had moderately increased levels of HDL cholesterol and apolipoprotein A-I and an increased ratio of HDL subclass 2 to HDL subclass 3, as compared with unaffected family members (1.5 +/- 0.8 vs. 0.7 +/- 0.4). CETP deficiency was not found in six unrelated subjects with elevated HDL cholesterol levels who were from different parts of the United States.CETP deficiency appears to be a frequent cause of increased HDL levels in the population of Japan, possibly because of a founder effect. The results that we observed in heterozygotes suggest that CETP normally plays a part in the regulation of levels of HDL subclass 2. There was no evidence of premature atherosclerosis in the families with CETP deficiency. In fact, the lipoprotein profile of persons with CETP deficiency is potentially antiatherogenic and may be associated with an increased life span.
Protein losses in continuous renal replacement therapies.
Authors: M H Mokrzycki, A A Kaplan
Journal: Journal of the American Society of Nephrology (1996)

Continuous renal replacement therapy (CRRT) has become a popular treatment modality but may have the disadvantage of producing substantial protein losses. With use of the Biuret method, a relatively insensitive assay, dialysate/ultrafiltrate protein losses have been reported to be as high as 1.3 g/L. With CRRT outputs of up to 50 L/day, these values would amount to protein losses of up to 65 g/day. In this study, dialysate/ultrafiltrate protein losses were reanalyzed by using a highly sensitive microprotein reagent (pyrogallol red) considered to be more accurate than previously available methods. Twenty-two dialysate/ultrafiltrate samples were obtained from Amicon-20 Diafilters or Fresenius F-80 dialyzers during continuous venovenous hemofiltration (CVVH) or continuous venovenous hemodialysis/hemodiafiltration (CVVHD/F). Mean hourly output for all treatments was 1637 +/- 694 mL/h. Mean protein concentration for all 22 dialysate/ultrafiltrate samples was 4.2 +/- 4.0 mg/dL. Mean dialysate/ultrafiltrate protein concentrations were similar for the Amicon 20 (3.4 +/- 4.4 mg/dL, N = 9) and the Fresenius F-80 (4.7 +/- 3.9 mg/dL, N = 13) (P = not significant). Protein losses were higher during convection-based CVVH (6.0 +/- 5.1 mg/dL, N = 10; range, 1 to 15 mg/dL) than during the mixed convection and diffusion-based CVVHD/F (2.7 +/- 1.9 mg/dL, N = 12; range, 0 to 6 mg/dL) (P = 0.049). Mean serum protein concentration at the time of dialysate/ultrafiltrate sampling was 4.7 +/- 1.8 g/dL. There was a weak, but statistically significant correlation between the dialysate/ultrafiltrate samples and the corresponding value for serum protein (r = 0.468, P < 0.03). It was concluded that protein losses during CRRT treatments are substantially lower than previously reported, are dependent on the serum protein concentration and the predominant nature of solute removal (convection versus diffusion), and can vary between 1.2 and 7.5 g/day.
Natural history of sleep disordered breathing in prepubertal children transitioning to adolescence
Authors: Edward O. Bixler, Julio Fernandez-Mendoza, Duanping Liao, Susan Calhoun, Sol M. Rodriguez-Colon, Jordan Gaines, Fan He, Alexandros N. Vgontzas
Journal: European Respiratory Journal (2016)

Because there is a lack of agreed upon diagnostic criteria, it is critical to understand the natural history of obstructive sleep apnoea (OSA) in children in order to establish treatment strategies based on objective data. The Penn State Child Cohort is a representative, general-population sample of 700 elementary school children at baseline, of whom 421 were reassessed 8 years later, during adolescence. The remission of childhood apnoea–hypopnoea index (AHI) ≥2 events per h in adolescence was 52.9%. Using the higher threshold of AHI ≥5 events per h, remission was 100.0%, with 50.0% partially remitting to AHI 2– &lt;5 events per h and the other half remitting to AHI &lt;2 events per h. The incidence of adolescent AHI ≥2 events per h in those with childhood AHI &lt;2 events per h was 36.5%, while the incidence of AHI ≥5 events per h in those with childhood AHI &lt;5 events per h was 10.6%. This longitudinal study confirms that prepubertal OSA tends to resolve naturally during the transition to adolescence, and that primary snoring and mild sleep disordered breathing (SDB) do not appear to be strongly associated with progression to more severe SDB. The key risk factors for SDB in adolescence are similar to those found in middle-aged adults ( i.e. male sex, older age and obesity). Moreover, consistent with recent studies in adults, this study includes the novel cross-sectional finding that visceral fat is associated with SDB as early as adolescence.
Infection with Pathogens Transmitted Commonly Through Food and the Effect of Increasing Use of Culture-Independent Diagnostic Tests on Surveillance — Foodborne Diseases Active Surveillance Network, 10 U.S. Sites, 2012–2015
Authors: Jennifer Y. Huang, Olga L. Henao, Patricia M. Griffin, Duc J. Vugia, Alicia B. Cronquist, Sharon Hurd, Melissa Tobin-D’Angelo, Patricia Ryan, Kirk Smith, Sarah Lathrop, Shelley Zansky, Paul R. Cieslak, John Dunn, Kristin G. Holt, Beverly J. Wolpert, Mary E. Patrick
Journal: MMWR Morbidity and Mortality Weekly Report (2016)

To evaluate progress toward prevention of enteric and foodborne illnesses in the United States, the Foodborne Diseases Active Surveillance Network (FoodNet) monitors the incidence of laboratory-confirmed infections caused by nine pathogens transmitted commonly through food in 10 U.S. sites. This report summarizes preliminary 2015 data and describes trends since 2012. In 2015, FoodNet reported 20,107 confirmed cases (defined as culture-confirmed bacterial infections and laboratory-confirmed parasitic infections), 4,531 hospitalizations, and 77 deaths. FoodNet also received reports of 3,112 positive culture-independent diagnostic tests (CIDTs) without culture-confirmation, a number that has markedly increased since 2012. Diagnostic testing practices for enteric pathogens are rapidly moving away from culture-based methods. The continued shift from culture-based methods to CIDTs that do not produce the isolates needed to distinguish between strains and subtypes affects the interpretation of public health surveillance data and ability to monitor progress toward prevention efforts. Expanded case definitions and strategies for obtaining bacterial isolates are crucial during this transition period.
ATCT-04RETROSPECTIVE ANALYSIS OF TUMOR TREATING FIELDS (TTFIELDS) IN ADULTS WITH GLIOBLASTOMA: SAFETY PROFILE OF THE OPTUNE™ MEDICAL DEVICE IN PATIENTS WITH IMPLANTED NON-PROGRAMMABLE SHUNTS, PROGRAMMABLE SHUNTS, AND PACEMAKERS/DEFIBRILLATORS
Authors: Lynn Ashby, Michael Hasselle, Sajeel Chowdhary, Hassan Fathallah-Shaykh, Jay-Jiguang Zhu
Journal: Neuro-Oncology (2015)

BACKGROUND: Tumor Treating Fields (TTFields) are low intensity, intermediate frequency alternating electric fields used to inhibit tumor cell division by impeding microtubular spindle formation during mitosis. Treatment is delivered by a portable medical device (Optune™) approved in the U.S. for adults with recurrent glioblastoma. Regulatory labelling states that Optune™ should not be used in patients who have implanted electronic devices such as pacemakers, defibrillators, or shunts (programmable and non-programmable) due to insufficient data regarding potential tissue damage or potential interference with implanted device function. METHODS: We reviewed the clinical information for 1,402 brain tumor patients treated with TTFields in the U.S. between November 2011 and May 2015. 44 had non-programmable shunts (NPS), 2 had programmable shunts (PS), and 3 had pacemakers/defibrillators (PM/DF). The safety data available through post-market surveillance in all 49 patients were analyzed to look for implantable device related adverse events. RESULTS: No infections or malfunctions were reported in any of the 44 patients with NPS or 2 patients with PS. No arrhythmia or other cardiac adverse events were reported for the 3 patients with PM/DF. Adverse events reported for these 49 patients did not raise any new safety concerns regarding concurrent use of Optune™ with their electronic implanted devices. Common skin irritations and ulcerations were seen in 27% and 2% of NPS patients, respectively. In the NPS patients, neurological symptoms (43%) and seizures (14%) were related to the underlying tumor rather than TTFields treatment. CONCLUSIONS: The use of TTFields in patients with programmable shunts and pacemaker/defibrillators has not been sufficiently evaluated to conclude safety, however, no issues were observed in the patients analyzed here. The number of patients with recurrent glioblastoma who require shunt implantation warrants further clarification of this safety issue. Therefore, a prospective trial formally studying the effects of TTFields on programmable shunts is planned.
Improvement in Long-Term Survival among Patients Hospitalized with Acute Myocardial Infarction, 1970 to 1980
Authors: Orlando Gomez-Marin, Aaron R. Folsom, Thomas E. Kottke, Shu-Chen H. Wu, David R. Jacobs, Richard F. Gillum, Stanley A. Edlavitch, Henry Blackburn
Journal: New England Journal of Medicine (1987)

The mortality rate associated with coronary heart disease in the United States has declined steadily since 1968, but the reasons for this favorable trend have not been completely elucidated. In particular, it is not clear to what extent the decline reflects decreasing incidence as opposed to improved survival. To assess whether improved survival after myocardial infarction has contributed to the decline, the Minnesota Heart Survey compared the four-year survival rate in patients discharged with a diagnosis of acute myocardial infarction from hospitals in the Twin Cities area in 1970 and 1980. After adjustment for clinical characteristics related to outcome, the survival rate among patients with a definite myocardial infarction was significantly higher in the 1980 than in the 1970 group. The four-year survival for men was 35 percent better in the 1980 than in the 1970 group (95 percent confidence interval, 21 to 50 percent), and for women it was 27 percent better (95 percent confidence interval, 1 to 46 percent). Improvement in survival during the period of hospitalization accounted for 70 percent of the overall gain in survival between 1970 and 1980 in men and for virtually all of the gain in women. We conclude that improved long-term survival among patients with acute myocardial infarction has made an important contribution to the decline in mortality from coronary disease.
Case 22-2011
Authors: Daniela Kroshinsky, John H. Stone, Rosalynn M. Nazarian
Journal: New England Journal of Medicine (2011)

A 79-year-old man was seen in the clinic because of a rash, painful and swollen hands, and red eyes. There was scleral erythema, urticaria on the torso, palpable purpura on the legs, and swelling and tenderness of the hand joints. A diagnostic procedure was performed.
Patterns of care among veterans with colon cancer: Insights from California SEER, Medicare and VA-linked data
Authors: D. M. Hynes, E. Tarlov, R. Perrin, Q. Zhang, C. Bennett, M. Ferreira, T. Lee, A. Benson
Journal: Journal of Clinical Oncology (2007)

6586 Background: U.S. veterans have been shown to be a vulnerable population with high cancer rates, yet practice pattern information is lacking. Linkage of cancer registry, Medicare, and VA data provides a more complete view of health status and healthcare received among veterans with cancer. Methods: As part of a national study, a retrospective cohort of incident colon cancer patients from the California Cancer Registry data, who were = 66 years old and eligible to use VA and Medicare between 1999 and 2001, were followed for three years through 2004. We examined practice patterns for stages I-IV colon cancer, specifically, surgery and use of chemotherapy, and trends in one-year mortality using descriptive and multivariate regression models. Results: Among 633 veterans with colon cancer, 93% were male; 16% were African American; 28% were diagnosed at a VA facility compared to 72% at a Medicare facility, and 553 (87%) had colectomy. One year mortality rate was 29%. Among the 553 colectomy patients, prevalence of chemotherapy use, according to stage was 8%, 27%, 61% and 52% for stages I-IV, respectively (p-value &lt;0.0001). Regression analyses of factors affecting whether chemotherapy was received revealed that Stage III patients were most likely to receive adjuvant chemotherapy (Odds Ratio (OR) 19.80, 95% Confidence Interval (CI): 9.68–40.50). However stage II and stage IV patients were also highly likely to receive chemotherapy following surgery (OR: 4.41 CI: 2.18–8.91 and OR: 13.21; CI: 6.01–29, respectively). Patients =76 years and those =86 years were less likely to receive chemotherapy following surgery (OR: 0.61, CI: 0.40–0.94 and OR: 0.25, CI: 0.094–0.64, respectively) compared to patients 66–75 years. Patients with Charlson Comorbidity Score of 2 or greater were less likely to receive chemotherapy (OR: 0.49; CI: 0.29–0.84). Treating facility (VA or Medicare), did not affect chemotherapy use. Conclusions: Among veterans with colon cancer in California, older patients were less likely to receive chemotherapy even when stage and comorbidity status were considered. Efforts focused on improving guideline consistent adjuvant chemotherapy use among older veterans with stage III colon cancer across treating facilities may yield the greatest benefit. No significant financial relationships to disclose.
Uremia in Sickle-Cell Anemia Treated by Maintenance Hemodialysis
Authors: Eli A. Friedman, T. K. Sreepada Rao, Charles L. Sprung, Albert Smith, Thomas Manis, Rita Bellevue, Khalid M. H. Butt, Richard D. Levere, Dorothy M. Holden
Journal: New England Journal of Medicine (1974)

Abstract Renal failure, which developed in two patients with sickle-cell anemia, was treated by maintenance hemodialysis for six and seven months. Medullary cystic disease appeared to cause the uremia in a 32-year-old black man with sickle-cell anemia; in the second patient, a 24-year-old black woman, histologic changes in a renal biopsy were consistent with So-Called sickle nephropathy. When uremia supervened each patient's transfusion requirement, which had been stable at 2 units per month, increased abruptly to 5 and 6 units per month, and then fell, upon initiation of maintenance hemodialysis, to 1 unit per month. Vascular access for dialysis was provided by an internal arteriovenous fistula in one patient and an arterioarterial bovine carotid heterograft in the other. There were neither hemorrhagic nor thrombotic complications of the dialysis regimen. Coincident congestive heart failure in both patients responded well to digitalis. (N Engl J Med 291: 431–435, 1974)
HOXC4, HOXC5, and HOXC6 Expression in Non-Hodgkin's Lymphoma: Preferential Expression of the HOXC5 Gene in Primary Cutaneous Anaplastic T-Cell and Oro-Gastrointestinal Tract Mucosa-Associated B-Cell Lymphomas
Authors: Janet J. Bijl, Johan W. van Oostveen, Jan M.M. Walboomers, Anja Horstman, Adriaan J.C. van den Brule, Rein Willemze, Chris J.L.M. Meijer
Journal: Blood (1997)