tbuckley/cabot-search huggingface dataset:
Cutis laxa in hereditary gelsolin amyloidosis
Authors: S. Kiuru-Enari, J. Keski-Oja, M. Haltia
Journal: British Journal of Dermatology (2005)

Background Hereditary gelsolin amyloidosis (AGel amyloidosis) is an age‐associated systemic disease with global distribution, caused by a G654A or G654T gelsolin gene mutation. Cutis laxa is a principal clinical manifestation of this disease. However, only few data on the dermatological involvement are available, and the pathogenesis of this amyloidosis‐associated form of cutis laxa has remained unknown. Objectives To elucidate the pathomechanism of this less well‐known genodermatosis. Methods We performed systematic clinical, histological, immunohistochemical and ultrastructural skin biopsy studies in 12 patients with a G654A gelsolin gene mutation. For comparison, skin specimens from 10 control subjects were analysed. Results All patients had clinically characteristic cutis laxa, and frequently other signs of symptomatic skin disease such as increased fragility and risk for intracutaneous bleeding. All patients showed cutaneous deposition of gelsolin amyloid (AGel), mainly attached to basement membranes or basal laminae of various cutaneous structures, dermal nerves and blood vessel walls, and elastic fibres, particularly in the lower reticular dermis. AGel often encircled the elastic fibres, and colocalized with amyloid P component (AP), an elastic fibre microfibrillar sheath‐associated protein. Fragmentation and loss of elastic fibres, epidermal atrophy, and reduction of dermal appendages were also common. Antibodies to wild‐type gelsolin bound to S‐100‐positive epidermal dendritic cells, a previously unrecognized immunoreaction. Patients had fewer gelsolin‐positive dendritic cells than controls. Conclusions Widespread skin involvement with AGel deposition and elastic fibre involvement are essential pathological features in AGel amyloidosis, and contribute to the characteristic cutis laxa, dramatic in old age. Codistribution of AGel and AP, with demonstrated specific binding affinity for amyloid fibrils, suggests that elastic fibre‐associated AP acts as a matrix for cutaneous amyloid deposition in AGel amyloidosis.
Abstract 2685: Imaging the various mechanisms of cancer-cell killing by <i>Salmonella typhimurium</i> A1-R
Authors: Ming Zhao, Yong Zhang, Aisada Uchugonova, Renee Salz, Fang Liu, Atsushi Suetsugu, Lei Zhang, Robert M. Hoffman
Journal: Cancer Research (2012)

Abstract We have developed a genetically-modified bacterial strain of Salmonella typhimurium, auxotrophic for leucine and arginine, which also expresses green fluorescent protein (GFP), termed S. typhimurium A1-R. S. typhimurium A1-R was found to be effective against metastatic human prostate, breast, and pancreatic cancer cell lines as well as osteosarcoma, fibrosarcoma and glioma cell lines in clinically-relevant nude mouse models. To understand the tumor-killing mechanism of S. typhimurium A1-R-GFP, we have studied their interaction with fluorescence-labeled cancer cells in vitro and in vivo. S. typhimurium-GFP invasion, proliferation, and virulence in the cancer cells were visualized by confocal fluorescence microscopy with the Olympus FV1000. We found that A1-R-induced cancer cell death had different mechanisms in different cancer cell lines. Some S. typhimurium A1-R-treated cancer cell lines became apoptotic and necrotic, and other cell lines burst after extensive intracellular bacterial growth. The time for S. typhimurium A1-R-GFP to kill the majority of the cancer cells varied from line to line, ranging from 2 hours to 72 hours. The understanding of the various mechanisms of cell killing by S. typhimurium A1-R will be important for its use as a general therapeutic for cancer. Citation Format: {Authors}. {Abstract title} [abstract]. In: Proceedings of the 103rd Annual Meeting of the American Association for Cancer Research; 2012 Mar 31-Apr 4; Chicago, IL. Philadelphia (PA): AACR; Cancer Res 2012;72(8 Suppl):Abstract nr 2685. doi:1538-7445.AM2012-2685
Prevention of Herpesvirus Infections in Renal Allograft Recipients by Low-Dose Oral Acyclovir
Authors: Linda Seale
Journal: JAMA (1985)

Forty patients with serum antibody against herpes simplex virus (HSV) were enrolled in a randomized, placebo-controlled, double-blind investigation of acyclovir given orally in a low dosage as prophylaxis against recurrent HSV infection after renal transplantation. During 30 postoperative days of medication, 14 of 21 placebo-treated and one of 19 acyclovir-treated patient(s) developed reactivation of HSV infection. Eleven of the former, but not the latter, had herpetic lesions. The protection against active infection with HSV during the period of prophylaxis with acyclovir is statistically highly significant. From 30 to 90 days after transplantation when no antiviral medicine was given, 60% (3/5) of the remaining placebo recipients and 44% (7/16) of the acyclovir patients developed active HSV infections. Herpetic lesions occurred in two of three and two of seven of infected people in the respective groups. No adverse effects of the drug were observed. The results show that HSV infections in immunosuppressed renal allograft recipients can be safely prevented, deferred, and ameliorated by an initial period of prophylaxis with a low dose of oral acyclovir. (<i>JAMA</i>1985;254;3435-3438)
Abnormal copper metabolism and deficient lysyl oxidase activity in a heritable connective tissue disorder.
Authors: H Kuivaniemi, L Peltonen, A Palotie, I Kaitila, K I Kivirikko
Journal: Journal of Clinical Investigation (1982)

Biochemical abnormalities were studied in two brothers with bladder divericulas, inguinal hernias, slight skin laxity, and hyperelasticity and skeletal abnormalities including occipital exostoses. Lysyl oxidase activity was low in the medium of cultured skin fibroblasts, this abnormality being accompanied by reduced conversion of the newly synthesized collagen into the soluble form. Copper concentrations were markedly elevated in the cultured skin fibroblasts, but decreased in the serum and hair. Serum cerulophasmin levels were also low. The reduced lysyl oxidase activity is suggested to be responsible for ther clinical manifestations, but the deficiency in this copper-dependent enzyme may be secondary to the abnormalities in the metabolism of the cation. Nevertheless, a mutation directly affecting both lysyl oxidase and an intracellular copper transport protein cannot be excluded. The disease is tentatively classified as one subtype of the Ehlers-Danlos syndrome.
Erectile Dysfunction and Incidence of Cardiovascular Disease—Reply
Authors: Ian M. Thompson, Catherine M. Tangen, Phyllis J. Goodman, Jeffrey L. Probstfield, Carol M. Moinpour, Charles A. Coltman
Journal: JAMA (2006)

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
Keeping an Eye on Distracted Driving
Authors: Jeffrey H. Coben, Motao Zhu
Journal: JAMA (2013)

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 2022 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
Chemical wastes, children's health, and the Superfund Basic Research Program.
Authors: P J Landrigan, W A Suk, R W Amler
Journal: Environmental Health Perspectives (1999)

Three to 4 million children and adolescents in the United States live within 1 mile of a federally designated Superfund hazardous waste disposal site and are at risk of exposure to chemical toxicants released from these sites into air, groundwater, surface water, and surrounding communities. Because of their patterns of exposure and their biological vulnerability, children are uniquely susceptible to health injury resulting from exposures to chemical toxicants in the environment. The Superfund Basic Research Program, funded by the U.S. Environmental Protection Agency and directed by the National Institute of Environmental Health Sciences, is extremely well positioned to organize multidisciplinary research that will assess patterns of children's exposures to hazardous chemicals from hazardous waste disposal sites; quantify children's vulnerability to environmental toxicants; assess causal associations between environmental exposures and pediatric disease; and elucidate the mechanisms of environmental disease in children at the cellular and molecular level.
Covert bronchial foreign body.
Authors: S Ahmad
Journal: BMJ (1984)

Tapping fracture of tibia An 18 year old man had a painful right shin after having been kicked playing football.The area was swollen, bruised, and tender, but no abnormality was seen on x ray examination.He was discharged.Pain per- sisted and increased while he was dancing eight days after the injury.Later his right leg gave way with a loud snap.X ray examination showed a tibial fracture.The fracture was undoubtedly caused by the kick, but intact, thick periosteum prevented displacement until the dancing.
Studies on the fibronectin receptors of human peripheral blood leukocytes. Morphologic and functional characterization.
Authors: C G Pommier, J O'Shea, T Chused, K Yancey, M M Frank, T Takahashi, E J Brown
Journal: The Journal of Experimental Medicine (1984)

We have investigated the interactions between plasma fibronectin (Fn) and human peripheral blood phagocytic cells. As shown by studies of the binding of Fn-coated fluorescent microspheres (Fn-ms), both polymorphonuclear leukocytes (PMN) and monocytes had specific binding sites for Fn at the plasma membrane. However, as purified from blood, only monocytes were stimulated by Fn to become more actively phagocytic. This increase in phagocytosis was reflected by an Fn-induced increase in the ingestion of IgG-coated erythrocytes and, more dramatically by an Fn-dependent initiation of phagocytosis of C3b-coated erythrocytes. Despite this difference between PMN and monocytes in the functional consequences of Fn binding, the cell surface molecules responsible for Fn binding on the two cell types shared many characteristics. On both cells, binding of Fn-ms was inhibited by sufficient concentrations of fluid-phase Fn; both PMN and monocytes bound fewer Fn-ms at 4 degrees C than at 37 degrees C; both achieved maximal binding at similar Fn-ms/cell ratios; and phenylmethylsulfonyl fluoride did not inhibit Fn-ms binding to either cell type. Most dramatically, monoclonal anti-Fn antibodies that inhibited binding of Fn-ms to one cell type inhibited binding to both; conversely, monoclonal anti-Fn antibodies that did not inhibit Fn-ms binding to either cell type did not inhibit binding to the other. Fn will stimulate PMN to a more actively phagocytic state, like that induced in monocytes, if the PMN are first exposed to C5a or N-formyl-methionyl-leucylphenylalanine. This effect occurs without apparent change in the number of Fn receptors. We conclude that the PMN and monocyte receptors for Fn are very similar, but that their milieu is very different in the two cells as purified from peripheral blood. Whereas Fn induces increased phagocytosis in monocytes, PMN must be activated before the Fn can be effective.
Methylmercury in fish: a review of residue levels, fish consumption and regulatory action in the United States.
Authors: L Tollefson, F Cordle
Journal: Environmental Health Perspectives (1986)

The dangers associated with the consumption of large amounts of methylmercury in fish are well recognized, and there is some evidence to suggest that methylmercury may be the cause of subtle neurological impairments when ingested at even low to moderate levels, particularly the prenatal and early childhood periods. This concern has prompted a continuing assessment of the risk of methylmercury toxicity among fish consumers in the United States as well as other countries. The toxicokinetics of methylmercury in humans are reviewed and used to estimate body burdens associated with toxic effects. To determine seafood consumption patterns among the continental U.S. population the Food and Drug Administration (FDA) has analyzed data from a diary study commissioned by the Tuna Research Foundation. Mercury residue levels in domestic fish sampled by the FDA were used to determine the level of exposure to methylmercury. Until evidence is presented that substantially lowers the known body burden of methylmercury which causes toxicity, calculations indicate that the current 1.0 ppm regulatory level provides adequate protection for the average fish consumer, for young children, and for a significant number of consumers exceeding the acceptable daily intake. However, additional studies are being carried out in a continuing process to ensure that safe levels of prenatal exposure to mercury residues in fish are maintained.
WHO report says countries should do more to prevent suicides
Authors: G. Iacobucci
Journal: BMJ (2014)

<h3>Abstract</h3> Decreased cost of human exome and genome sequencing provides new opportunities for diagnosing genetic disorders, but we need better and more robust methods for interpreting sequencing results including determining whether and by which mechanism a specific missense variants may be pathogenic. Using the protein PTEN (phosphatase and tensin homolog) as an example, we show how recent developments in both experiments and computational modelling can be used to determine whether a missense variant is likely to be pathogenic. One approach relies on multiplexed experiments that enable determination of the effect of all possible individual missense variants in a cellular assay. Another approach is to use computational methods to predict variant effects. We compare two different multiplexed experiments and two computational methods to classify variant effects in PTEN. We distinguish between methods that focus on effects on protein stability and protein-specific methods that are more directly related to enzyme activity. Our results on PTEN suggest that ~60% of pathogenic variants cause loss of function because they destabilise the folded protein which is subsequently degraded. Methods that quantify a broader range of effects on PTEN activity perform better at predicting variant effects. Either experimental method performs better than the corresponding computational predictions, so that e.g. experiments that probe cellular abundance perform better at identifying pathogenic variants than predictions of thermodynamic stability. Our results suggest that loss of stability of PTEN is a key driver for disease, and we hypothesize that experiments and prediction methods that probe protein stability can be used to find variants with similar mechanisms in other genes.
Inhibition of cellular ras prevents smooth muscle cell proliferation after vascular injury in vivo
Authors: Ciro Indolfi, Enrico V. Avvedimento, Antonio Rapacciuolo, Emilio Di Lorenzo, Giovanni Esposito, Eugenio Stabile, Antonio Feliciello, Evelina Mele, Paola Giuliano, GianLuigi Condorelli, Massimo Chiariello
Journal: Nature Medicine (1995)
Studies on tissue distribution of scutellarin and methyl polyethylene glycol (mPEG)-scutellarin prodrug in mice
Authors: Juan Lu, Xia Zhan, Qingfei Liu, Xi Chen, Xinge Zhao, Yiming Wang, Guoan Luo
Journal: Acta Pharmaceutica Sinica B (2012)

This study was to determine tissue distribution and pharmacokinetics of mPEG-scutellarin prodrug (7e), a chemical entity previously shown to have a beneficial effect in cerebral ischemia/reperfusion (I/R) injury. After injecting scutellarin or prodrug 7e, the concentrations of scutellarin and 7e in tissues were determined and the pharmacokinetic parameters were calculated. The results showed that the distribution of scutellarin in tissues was enhanced by PEGylation. The distribution of 7e in brain was approximately 2.1-fold higher than that of scutellarin, indicating that PEGylation increased the brain penetration of scutellarin. We conclude that 7e could exert more effective protection on cerebral I/R injury in mice. This study also provided a simple and convenient strategy to identify novel drugs with potential protective function for I/R injury in mice.
National Standards for Diabetes Self-Management Education
Authors: Ann T.Y. Shiu
Journal: Diabetes Care (2007)

Evidence indicates that diabetes self-management education using empowerment models improves patient outcomes (1). It also shows that a variety of pathways have been used for achieving patient empowerment. Two pathways, general self-efficacy (2) and sense of coherence (3), have shown high utility. Self-efficacy is an individual’s confidence in their capabilities to perform a specific action that has influence over …
CULLEN'S SIGN IN ECTOPIC PREGNANCY: A SUGGESTION FOR ITS DETERMINATION
Authors: F. H. Jackson
Journal: JAMA (1922)

The diagnosis of ectopic pregnancy before the onset of the tragic stage is sometimes difficult. But it is needless to point out that diagnosis made before this period will result in a much better prognosis. Cullen has called attention to the dark appearance of the umbilicus in those cases in which rupture has occurred and free blood is found in the abdominal cavity. Naturally, such a sign is simply one of the methods that enable one to come to a definite conclusion. Busch<sup>1</sup>reports the case of a woman, aged 60, with an ovarian cyst the size of a child's head, that gave this sign with no intra-abdominal hemorrhage. He points out that the sign is not always evidence of intra-abdominal hemorrhage, let alone ectopic pregnancy. Such an isolated example does not make the sign of any less value, for one would hardly be confused or in a quandary
Connexin 26 R143W Mutation Associated with Recessive Nonsyndromic Sensorineural Deafness in Africa
Authors: George W. Brobby, Bertram Müller-Myhsok, Rolf D. Horstmann
Journal: New England Journal of Medicine (1998)

To the Editor: Recently, the pathologic process underlying nonsyndromic forms of inherited hearing impairment has been studied at the molecular level.1 The previously localized DFNA3 and DFNB1 loci...
A NOTE ON THE ADMINISTRATION OF ETHER BY THE OPEN METHOD
Authors: G. H. Colt
Journal: BMJ (1908)

BY THE STOMACH.In the next two experiments 25 c.cm. of chloroform in oil were administered by the stomach on two successive -days.In; bothi experiments a distinct increase in the total nitrogen excreted was observed, and in both there was a distinct fall in the nitrogen in urea, a rise in the nitrogen in ammonia and in uric acid-no marked change in the proportion of nitrogen in creatinin, and a very marked -rise in the nitrogen not in these combinations.This -'remainder nitrogen" may be taken as probably chiefly znitrogen in amino-acids, and it is of interest to observe that its chief rise preceded the chief rise of .ammonia:nitrogen.The proportion of oxidized sulphur showed a lfall.In both experiments proteins, granular cells, and tube casts were present in the urine, and in one experiment jaundice was present. HYPODERMICALLY.In the next two experiments 10 c.cm. of chloroform were given hypodermically on two successive doses.No marked Tise-in the total nitrogen excretion was found.The pro--portion of urea nitrogen fell markedly in the first experi- ment, but only to a small extent in the-second.The ammonia nitrogen showed a rise, the proportion of uric acid nitrogen was increased, but the proportion of creatinin nitrogen was not markedly altered.The nitrogen not in these combinations rose markedly in the experiment in -which the urea nitrogen was decreased.The sulphur was estimated in only one experiment.and the oxidized sulphur showed a marked decrease.J'mt¢in-and granular cells were prose 4inheurine.ASSUMPTION AND EWIMINATJON IN THE BLOOD.Is this difference in the mode of action of chloroform administered-by these different channels merely the result
Poison Ivy, Mangoes, Cashews, and Dermatitis
Authors: Mario Geller
Journal: Annals of Internal Medicine (1989)

Letters and Corrections15 June 1989Poison Ivy, Mangoes, Cashews, and DermatitisMario Geller, MDMario Geller, MDSearch for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-110-12-1036_2 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptTo the Editor: I would like to report two cases of antigenic cross-reactivity between members of the Anacardiaceae plants related to the poison ivy group (1, 2). They occurred in two Americans living in Brazil, both with a previous history of rhus dermatitis (poison ivy) in the United States. This type of contact dermatitis does not exist among native Brazilians, because poisonous rhus plants do not exist in Brazil. The sap of rhus plants contains catechols that cross react with oleoresins from mango rind (Mangifera indica) and cashew oil (Anacardium occidentale).A 43-year-old white woman and a 45-year-old white man...References1. Fisher A. Dermatitis due to plants and spices. In: Contact Dermatitis 1975. 2d ed. Philadelphia: Lea and Febiger; 1975:260-6. Google Scholar2. Adverse Reactions to Foods. American Academy of Allergy and Immunology Committee on Adverse Reactions to Foods and the National Institute of Allergy and Infectious Diseases. Washington DC: U.S. Department of Health and Human Services; 1984: 34-5. N.I.H. Publication no. 84-2442. Google Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byAnacardiaceae Lindl.RHUS (TOXICODENDRON) DERMATITISMan Shall Not Live by Peanut Alone!Pistachio nut hypersensitivity: identification of pistachio nut allergensSelected Environmental Skin Disorders 15 June 1989Volume 110, Issue 12Page: 1036-1037KeywordsAntigensContact dermatitisOils ePublished: 1 December 2008 Issue Published: 15 June 1989 PDF downloadLoading ...