tbuckley/cabot-search huggingface dataset:
Transplantation of Umbilical-Cord Blood in Babies with Infantile Krabbe's Disease
Authors: Maria L. Escolar, Michele D. Poe, James M. Provenzale, Karen C. Richards, June Allison, Susan Wood, David A. Wenger, Daniel Pietryga, Donna Wall, Martin Champagne, Richard Morse, William Krivit, Joanne Kurtzberg
Journal: New England Journal of Medicine (2005)

Infantile Krabbe's disease produces progressive neurologic deterioration and death in early childhood. We hypothesized that transplantation of umbilical-cord blood from unrelated donors before the development of symptoms would favorably alter the natural history of the disease among newborns in whom the disease was diagnosed because of a family history. We compared the outcomes among these newborns with the outcomes among infants who underwent transplantation after the development of symptoms and with the outcomes in an untreated cohort of affected children.Eleven asymptomatic newborns (age range, 12 to 44 days) and 14 symptomatic infants (age range, 142 to 352 days) with infantile Krabbe's disease underwent transplantation of umbilical-cord blood from unrelated donors after myeloablative chemotherapy. Engraftment, survival, and neurodevelopmental function were evaluated longitudinally for four months to six years.The rates of donor-cell engraftment and survival were 100 percent and 100 percent, respectively, among the asymptomatic newborns (median follow-up, 3.0 years) and 100 percent and 43 percent, respectively, among the symptomatic infants (median follow-up, 3.4 years). Surviving patients showed durable engraftment of donor-derived hematopoietic cells with restoration of normal blood galactocerebrosidase levels. Infants who underwent transplantation before the development of symptoms showed progressive central myelination and continued gains in developmental skills, and most had age-appropriate cognitive function and receptive language skills, but a few had mild-to-moderate delays in expressive language and mild-to-severe delays in gross motor function. Children who underwent transplantation after the onset of symptoms had minimal neurologic improvement.Transplantation of umbilical-cord blood from unrelated donors in newborns with infantile Krabbe's disease favorably altered the natural history of the disease. Transplantation in babies after symptoms had developed did not result in substantive neurologic improvement.
Three-dimensional intravascular ultrasound assessment after the implantation of estrogen-eluting PC-coated stents in human coronary arteries
Authors: Marco A. Costa, Jose Eduardo M. Sousa, Alexandre A. Abizaid, Andrea S. Abizaid, Rodolfo Staico, Luiz A. Mattos, Fausto Feres, Ibraim Pinto, Theodore A. Bass, Amanda Sousa
Journal: Journal of the American College of Cardiology (2003)
Promissory notes, genetic clocks, and epigenetic outcomes
Authors: Annette Karmiloff-Smith
Journal: Behavioral and Brain Sciences (1997)

I respond to three continuing commentaries on Beyond modularity, two concerning the representational redescription (RR) framework and its attempts to account for the growing flexibility of human intelligence, and one relating to the putative mysteries of developmental timing. I discuss misunderstandings about the RR framework as well as some of its shortcomings. I strongly reject the notion of a genetic clock and go on to argue for epigenetic outcomes in which genes and environment interact during the protracted period of postnatal brain development.
The infectious aetiology of tropical ulcer—a study of the role of anaerobic bacteria
Authors: BEVERLEY ADRIAANS, R. HAY, B. DRASAR, D. ROBINSON
Journal: British Journal of Dermatology (1987)

We have cultured anaerobic bacteria from patients with tropical ulcers. Fusobacteria were isolated most frequently. Anaerobes were always present, together with aerobes or facultative anaerobes, particularly in early phase ulcers, suggesting that the combination of organisms is important in the pathogenesis of the disease. Spirochaetes were identified in material examined by light and electron microscopy, but were not cultured.
COMPARISON OF AMNIOTIC-FLUID AND MATERNAL SERUM ALPHA-FETOPROTEIN LEVELS IN THE EARLY ANTENATAL DIAGNOSIS OF SPINA BIFIDA AND ANENCEPHALY
Authors: R. Harris, R.F. Jennison, A.J. Barson, K.M. Laurence, E. Ruoslahti, M. Seppälä
Journal: The Lancet (1974)
Clinical efficacy, radiographic and safety findings through 2 years of golimumab treatment in patients with active psoriatic arthritis: results from a long-term extension of the randomised, placebo-controlled GO-REVEAL study
Authors: Arthur Kavanaugh, Iain B McInnes, Philip J Mease, Gerald G Krueger, Dafna D Gladman, Désirée van der Heijde, Surekha Mudivarthy, Weichun Xu, Michael Mack, Zhenhua Xu, Anna Beutler
Journal: Annals of the Rheumatic Diseases (2012)

<h3>Objectives</h3> To assess long-term golimumab efficacy/safety in patients with active psoriatic arthritis (PsA). <h3>Methods</h3> Adult PsA patients (≥3 swollen, ≥3 tender joints, active psoriasis) were randomly assigned to subcutaneous injections of placebo, golimumab 50 mg or 100 mg every 4 weeks (q4wks) through week 20. All patients received golimumab 50 or 100 mg beginning week 24. Findings through 2 years are reported. Efficacy evaluations included ≥20% improvement in American College of Rheumatology (ACR20) response, good/moderate response in Disease Activity Scores incorporating 28 joints and C-reactive protein (DAS28-CRP), ≥75% improvement in Psoriasis Area and Severity Index (PASI75) and changes in PsA-modified Sharp/van der Heijde scores (SHS). <h3>Results</h3> Golimumab treatment through 2 years was effective in maintaining clinical response (response rates: ACR20 63%–70%, DAS28-CRP 77%–86%, PASI75 56%–72%) and inhibiting radiographic progression (mean change in PsA-modified SHS in golimumab-treated patients: −0.36), with no clear difference between doses. No new safety signals were identified through 2 years. With the study9s tuberculosis screening and prophylactic measures, no patient developed active tuberculosis through 2 years. <h3>Conclusions</h3> Golimumab 50 and 100 mg for up to 2 years yielded sustained clinical and radiographic efficacy when administered to patients with active PsA. Increasing the golimumab dose from 50 to 100 mg q4wks added limited benefit. Golimumab safety through up to 2 years was consistent with other antitumour necrosis factor α agents used to treat PsA. Treatment of patients with latent tuberculosis identified at baseline appeared to be effective in inhibiting the development of active tuberculosis.
ATHEROGENICITY OF BLOOD SERUM FROM PATIENTS WITH CORONARY HEART DISEASE
Authors: E.I. Chazov, A.N. Orekhov, N.V. Perova, Kh.A. Khashimov, V.V. Tertov, A.A. Lyakishev, Kh.A. Kurdanov, I.D. Novikov, V.N. Smirnov
Journal: The Lancet (1986)
POST-HEMORRHAGIC ANEMIA
Authors: Lindsay S. Milne
Journal: The Journal of Experimental Medicine (1912)

In rabbits and cats in which a hemorrhagic anemia has been produced, the hemoglobin is regenerated much more slowly than the red corpuscles, but the index, relative to the red corpuscles, never rises above one. The resistance of the corpuscles is not increased. In erythrocytes developing in the bone marrow, the nucleus of the erythroblasts seems to lose its chromatin, but is apparently not extruded. Punctate basophilia of the erythrocytes is a common feature and seems to result from a diffusion of nuclear chromatin through the cell. Extramedullary myeloid metaplasia is generally slight, but in moderately severe and long continued anemia from bleeding it may be very extensive. Lipemia usually occurs when the blood is reduced below a certain limit. The greater part of the fat in the serum can be extracted with ether and is stained by osmic acid. Extensive fatty infiltration of the organs may occur and also widespread necrosis in the central parts of the liver lobules. Pigmentation of the internal organs is usually only slight, but may be considerable, particularly in the spleen. Although there are minor differences, all the essential features of anemia produced by toxins can be reproduced by hemorrhage.
SPECIAL ARTICLE
Authors: HENRY A. SCHROEDER
Journal: Circulation (1967)

Observations made in the last 15 years by the author and others concerning the effect of trace metals, particularly cadmium and chromium, on animals and man are reviewed with special reference to hypertension and atherosclerosis. These trace metals come from the soil or sea. Among the topics covered are cadmium hypertension in rats, diabetes mellitus and chromium deficiency, deficiency sources, and possible losses of chromium, and the influence of drinking water on death rates from atherosclerosis. The conclusions are that one common form of hypertension is probably an example of accumulation of cadmium in the kidney and that deficiency of chromium may influence glucose and lipid metabolism and affect atherosclerosis. Other trace elements may contribute also.
CS-17 * UPREGULATION OF THE ENDOPLASMIC RETICULUM CHAPERONE, PROLYL 4-HYDROXYLASE, BETA POLYPEPTIDE (P4HB) PROMOTES INVASION, ANGIOGENESIS AND GROWTH VIA EGFR/MAPK (ERK) SIGNALLING PATHWAY IN MALIGNANT GLIOMA
Authors: G. K. K. Leung, S. Sun, A. S. W. Ho, D. Lee, K. M. Y. Kiang, F.-f. Xu, J. K. S. Pu, X.-q. Zhang
Journal: Neuro-Oncology (2014)

BACKGROUND: Tumor invasion and vascularization are typical hallmarks in malignant glioma with the majority showing elevated expression and activation of the epidermal growth factor receptor (EGFR). Our group has previously shown that prolyl 4-hydroxylase, beta polypeptide (P4HB), an endoplasmic reticulum (ER) chaperone, was intimately related to chemoresistance in malignant glioma. The role of P4HB in determining other glioblastoma malignant phenotypes, however, remains unknown. METHODS: We studied both the in vitro and in vivo behaviors of globlastoma cells with established P4HB-overexpression. We also studied P4HB expression profiles in clinical specimens and publicly available microarray database. Hierarchical clustering graphically was performed to detect differential gene expression patterns between low and high P4HB tumors. The effect of P4HB in determining EGFR signaling and functioning were also studied. RESULTS: We found that inhibition of P4HB would dyregulate EGFR/MAPK-mediated angiogenic and invasive phenotypes of malignant glioma. Clinical analysis on human glioma specimens and Gene Expression Omnibus (GEO) expression profiles showed that excessive P4HB were correlated with high-grade malignancy, angiogenesis and invasion. Functionally, upregulation of P4HB conferred glioma cells with malignant characteristics including proliferation, invasion and angiogenesis in vitro, and tumor growth in orthotopic mouse model. Genetic and pharmacologic inhibitions of oncogenic P4HB were found to suppress EGFR/MAPK signaling. CONCLUSION: Our study is the first to describe a mechanistic link between P4HB-EGFR-mediated MAPK activation and glioma progression, invasion and angiogenesis. The findings uncovered a novel and promising anti-glioma mechanism related to P4HB-mediated retardation of EGFR/MAPK signal transduction. Targeting P4HB with small molecular agents may provide an alternative therapeutic approach by deactivating several critical downstream events that mediate the aggressive and resistant behaviors of glioblastoma.
Serum Mucoids in Lymphoma
Authors: Henry R. Bartos
Journal: JAMA (1968)

<h3>To the Editor:—</h3> The management of the patient with lymphoma depends upon the clinical stage. Among currently available procedures, lymphangiography has been shown to be the only accurate method of staging lymphomas.<sup>1</sup>This procedure, however, has been associated with complications, and mortality has been reported.<sup>2</sup> We have been interested in finding a chemical test which might be helpful in staging lymphomas. Serum mucoid valves have been reported to be increased in a variety of diseases, including malignancies.<sup>3</sup>In some studies, levels of serum mucoids paralleled the extent of the malignancy.<sup>4</sup>The present study was undertaken to determine whether measurement of serum mucoids would give an accurate assessment of lymphoma stage. <h3>Materials and Methods:—</h3> The diagnosis in all patents were confirmed by biopsy. Patients were categorized into stages I, II, III, and IV as recommended by the Committee on Staging of the American Cancer Society.<sup>5</sup> Lymphangiograms
21<sup>st</sup> Century tablet
Authors: Kamran Abbasi
Journal: Journal of the Royal Society of Medicine (2012)

I recently returned from a trip to Asia where I ran a workshop on publishing in medical journals. This is, of course, a standard routine for a medical journal editor, although it is very much a personal performance since all editors have their peculiarities. How relevant my experiences might be, for example, to a crowd of Chinese geneticists is open for discussion, but there is always a thrill in sharing ideas with people from different cultures. Medicine can be a parochial career, in terms of locality and specialty, if it suits your bent, or it can be an international, multicultural adventure.

JRSM has steered a clear course in recent years: to establish itself as the leading clinical journal for UK medicine. That journey continues. This month, we introduce an important series on lessons in leadership which will feature articles from people with leadership experiences to share from medicine and beyond.1 ‘Without fit for purpose leaders and proper leadership development of the over 250 clinical commissioning groups who will assume £60bn of funding from 2013,’ warns Aidan Halligan, ‘there is real potential for the undermining of public trust in the NHS and for clinical catastrophe at scale.’

But the reality of medicine in our interdependent world is that medical and surgical specialties are international enterprises. In our ambition to bring readers the best possible clinical articles, JRSM will increasingly feature research papers and reviews of international relevance that will offer valuable insights and learning for a UK readership as well as an international one. In this issue, Allyson Pollock and colleagues warn of the dangers of rolling out a large vaccination programme without data to support the initiative, as is being considered in two populous regions of India.2

The primary enablers of internationalism, of course, are affordable international air travel and information technology. My voyage to the Far East was more tolerable for a personal interactive video screen offering films, drama, music, and games. In between, a laptop, electronic reader, and other handheld electronic devices provided e-comfort. Without Powerpoint the deep meaning of my workshop might have been lost on my Eastern friends.

Some of you might add your iPad to that list? Clinicians are familiar to marketing companies as early adopters of technology, and if you believe an essay in this month's issue you will accept that tablet devices, of which an iPad is merely one, will quickly transform the practice and delivery of every aspect of clinical care.3 Indeed, I'll stick my neck out—my in-flight viewing of Game of Thrones taught me that necks are there for slicing off—and say that tablet devices will have an equally transformational effect on medical journals. We are already seeing newspapers and weekly journals moving successfully to tablet versions. Where they lead, monthly journals will follow—albeit in our own peculiar manner.
Remarks ON THE EARLY SYMPTOMS OF MENTAL DISORDER: Read before the Lambeth Division of the British Medical Association
Authors: W. H. B. Stoddart
Journal: BMJ (1907)

CHAIRMAN AND GENTLEMEN,-I have thought that tlle subject which I have chosen would be of greater interest to you than any other because the early symptoms of mental disorder are those which occur in your own practice.The early symptoms have usually passed off before the patient is brought to the notice of a consulting physician.Yet it is important that mental disorder should be recognized in its early stages, so that treatment may be adopted with a view to cutting short the disease, if possible, and sparing the patient the stigma of certification and confinement in en asylum for the insane.Consciousness, mentation, or whatever you like to call mind, is the faculty or function by which we become aware of our surroundings; and it is obvious that we can only become aware of our surroundings by means of sensations aroused by them.A man in whom no senea- tions can be aroused is unconscious, he has no conscious- ness; and vice versa, a conscious individual is constantly experiencing sensations.DISORDERS OF SENSATION.Disorder of sensation is a very common symptom of mental disorder; it is either excessive (hyperaest4iesia) or deficient (anaesthesia).Hyperaesthesia occurs most frequently in maniacal states.If a patient suffering from acute mania be lightly touched with the point of a pin, he screams.His hearing is also more acute than usual; one such patient heard every word of a conversation between a nurse and myself at a distance of forty yards; another complained of the ringing of church bells, which I could only just hear by a strain of attentioD, and which his attendant could not hear at all.In these cases hyperaesthesia was a well marked symptom; and it is to be inferred from them that slight general hyperaesthesia is sometimes to be regarded as an early symptom of mental disorder.
MYD88 (L265P) mutation is an independent risk factor for progression in patients with IgM monoclonal gammopathy of undetermined significance
Authors: Marzia Varettoni, Silvia Zibellini, Luca Arcaini, Emanuela Boveri, Sara Rattotti, Cristiana Pascutto, Silvia Mangiacavalli, Manuel Gotti, Lara Pochintesta, Marco Paulli, Mario Cazzola
Journal: Blood (2013)
Real Help and Red Herrings in Spinal Imaging
Authors: Richard A. Deyo
Journal: New England Journal of Medicine (2013)

Visual evidence is compelling. Unlike an abstract measure of plasma glucose or blood pressure, a herniated disk — or a picture of one, thanks to the wonders of modern imaging — can be seen by doctors and patients. Furthermore, conventional wisdom would have it that knowing imaging results can only be good. Most people are uncomfortable with uncertainty, especially regarding medical diagnosis. To reduce anxiety, patients, family members, and physicians alike often request imaging tests “just to be sure.”Spine imaging plays an essential role in confirming diagnoses and planning surgery. However, with ever-improving resolution, modern imaging uncovers ever more . . .
Gastrointestinal Bleeding: Diagnosis and Management
Authors: Duane W. Taebel
Journal: JAMA (1978)

The management of gastrointestinal (GI) bleeding is one of the more common medical emergencies, the successful outcome of which depends on close collaboration between several specialty groups, and for this reason a protocol for management is desirable for any medical facility. The authors of this monograph, the first of a planned series entitled "Clinical Gastroenterology," present in a succinct fashion such protocols, with supporting discussion and annotation for the management of overt GI bleeding, either upper or lower, as well as occult GI bleeding. In other chapters they outline their approach for specific disease entities such as peptic ulcer and varices. A separate chapter is devoted to a discussion of shock. All are presented in an easily readable style, and the bibliography is reasonably current and complete. As the authors admit, the management of such bleeding is perhaps more art than science. It will, therefore, be subject to individual artistic
Sputum microbiota in moderate versus severe patients with COPD
Authors: Antonio Galiana, Estefania Aguirre, Juan Carlos Rodriguez, Alex Mira, Miguel Santibañez, Inmaculada Candela, Juana Llavero, Pedro Garcinuño, Francisco López, Montserrat Ruiz, Eduardo Garcia-Pachon, Gloria Royo
Journal: European Respiratory Journal (2013)

Sputum microbiota in moderate versus severe patients with COPDTo the Editor:The emergence of new massive sequencing methods has proved revolutionary for the study of complex microbial populations such as the microbiota of the respiratory tract in patients with chronic obstructive pulmonary disease (COPD) [1].Using this powerful methodology, our objective was to compare the microbiota in two groups of patients with COPD of different severity in order to detect potential microbiological markers that could help to provide a better understanding of the pathogenesis of this disease and the role played by the microbiota in its severity.
INAPPARENT MEASLES AFTER GAMMA GLOBULIN ADMINISTRATION
Authors: Francis L. Black
Journal: JAMA (1960)

The appearance of antibodies and development of immunity to measles was observed in 19 children in whom signs of measles had been prevented, during the course of epidemics, by the administration of gamma globulin. The epidemics occurred in two cottages of an institution for mentally retarded children. Both virus-neutralizing and complement-fixing techniques were used for the serologie tests, and human infant kidney tissue cultures were used for virus isolation. Of 38 susceptible children exposed to measles under these conditions and given gamma globulin, 19 developed measles antibodies without overt signs of the disease. Five of these 19 children failed to contract measles when exposed again seven months later.
Editorial: Nephrogenous ascites
Authors: H. H. Hussey
Journal: JAMA (1974)

End-stage renal failure with uremia is sometimes attended by bizarre accumulations of fluid, the pathogenesis of which is not altogether clear. Examples include pulmonary edema in which the fluid is located at the hilar regions, giving a roentgenographic picture characterized as "bat wing" or "butterfly wing," and pericarditis with effusion. At times, the pericardial effusion is so extreme as to cause cardiac tamponade, requiring emergency treatment by paracentesis or pericardiectomy. A recent issue of<i>Archives of Internal Medicine</i>contains two communications that recount extraordinarily large and persistent accumulations of ascitic fluid in uremic patients. During a two-year period, Craig et al<sup>1</sup>observed six patients with chronic renal failure and persistent ascites resistant to hemodialysis in five instances. (The sixth case must be discounted since the patient refused treatment and was lost to follow-up.) All six patients underwent peritoneoscopy that failed to disclose any factor other than the uremia to