tbuckley/cabot-search huggingface dataset:
Sickle cell anemia: Erythrokinetics, blood volumes, and a study of possible determinants of severity
Authors: Martin H. Steinberg, Bernard J. Dreiling
Journal: American Journal of Hematology (1977)

Abstract The variability in the clinical expression of sickle cell anemia led us to study factors which might influence the course of this disease. We examined erythrokinetics, blood volumes, and variables which influence hemoglobin function in a group of adults with sickle cell anemia of varying degrees of clinical severity. We were unable to correlate any single measurement with the clinical course; however, our patient sample was small and the data suggested areas for further study. An expansion of plasma volume was noted in all patients. This made it difficult to predict red cell mass from the hemoglobin level, which consistently underestimated its magnitude. The red cell production index and iron turnover values indicated that there is often a suboptimal erythropoietic response to anemia in sickle cell disease.
Active management of labour: care of the fetus.
Authors: K O'Driscoll, M Coughlan, V Fenton, M Skelly
Journal: BMJ (1977)

A prospective study of 1000 consecutive primigravidae was conducted to assess the relevance of the colour of the liquor to the welfare of the child. The results showed that clear liquor early in labour virtually ensures the birth of a healthy infant, provided the duration of labour is limited and delivery is effected without trauma; and, conversely, that meconium--or no liquor--marks the fetus who may suffer death or brain damage during normal labour. Special techniques were reserved for the second group of cases.
Time-dependent changes in the density and hemoglobin F content of biotin-labeled sickle cells.
Authors: R S Franco, J Lohmann, E B Silberstein, G Mayfield-Pratt, M Palascak, T A Nemeth, C H Joiner, M Weiner, D L Rucknagel
Journal: Journal of Clinical Investigation (1998)

Sickle red blood cells (RBC) are subject to a number of important cellular changes and selection pressures. In this study, we validated a biotin RBC label by comparison to the standard 51Cr label, and used it to study changes that occur in sickle cells as they age. Sickle RBC had a much shorter lifespan than normal RBC, but the two labels gave equivalent results for each cell type. A variable number of sickle, but not normal, RBC disappeared from the circulation during the first few hours after reinfusion. The number of biotinylated sickle reticulocytes was decreased by 50% after 24 h and 75% after 48 h, with a gradual decrease in the amount of reticulum per cell. The labeled sickle cells exhibited major density increases during the first 4-6 d after reinfusion, with smaller changes thereafter. A small population of very light, labeled sickle RBC was essentially constant in number after the first few days. Fetal hemoglobin (HbF) content was determined in isolated biotinylated sickle RBC after reinfusion, allowing an estimate of lifespan for RBC containing HbF (F cells) and non-F cells. The lifespan of sickle biotinylated RBC lacking HbF was estimated to be approximately 2 wk, whereas F cells survived 6-8 wk.
Increased respiratory burst activity of monocytes and polymorphonuclear leukocytes in psoriasis
Authors: R.E. SCHOPF, P. ALTMEYER, E.-M. LEMMEL
Journal: British Journal of Dermatology (1982)

Journal Article Increased respiratory burst activity of monocytes and polymorphonuclear leukocytes in psoriasis Get access R.E. SCHOPF, R.E. SCHOPF Institute for Medical Microbiology, Johannes Gutenberg‐University, D‐6500 Mainz Dr R.E.Schopf, Department of Dermatology, Johannes Gutenberg‐University, Langenbeckstr. 1, D‐6500 Mainz, F.R.G. Search for other works by this author on: Oxford Academic Google Scholar P. ALTMEYER, P. ALTMEYER Department of Dermatology, Johann Wolfgang von Goethe‐University, Theodor‐Stern‐Kai 7, D‐6000 Frankfurt a.M., F.R.G. Search for other works by this author on: Oxford Academic Google Scholar E.‐M. LEMMEL E.‐M. LEMMEL Institute for Medical Microbiology, Johannes Gutenberg‐University, D‐6500 Mainz Search for other works by this author on: Oxford Academic Google Scholar British Journal of Dermatology, Volume 107, Issue 5, 1 November 1982, Pages 505–510, https://doi.org/10.1111/j.1365-2133.1982.tb00399.x Published: 01 November 1982 Article history Accepted: 20 February 1982 Published: 01 November 1982
The National Institute for Health and Clinical Excellence and Its Role in Assessing the Value of New Cancer Treatments in England and Wales
Authors: Rebecca Trowman, Helen Chung, Carole Longson, Peter Littlejohns, Peter Clark
Journal: Clinical Cancer Research (2011)

The boundaries of medical science in the treatment of cancer are constantly extending. Developments of existing treatments, innovative approaches, new discoveries, and more targeted therapeutic options are translating into practice. With advances come increasing costs, often of a magnitude that stretches finite financial resources. When decisions about funding are made on behalf of a population, standardized processes and methods are needed in order to produce robust guidance in a fair, consistent, and transparent way. The challenges of making these difficult decisions are brought into particularly stark relief when potentially life-extending treatments for patients with a short life expectancy are appraised. The National Institute for Health and Clinical Excellence (NICE) produces guidance on the clinical- and cost-effectiveness of medicines compared with current standard practice. Approximately 40% of the technologies appraised by NICE are indicated for cancer, and the majority of these are pharmaceuticals, mostly biological agents. This article provides an overview of the current role of NICE in making new technologies for cancer available in England and Wales. This includes a summary of experiences with end-of-life treatments and the supplementary advice regarding such treatments that was issued by NICE to its decision-making committees in 2009.
Bearing the News
Authors: Saul S. Radovsky
Journal: New England Journal of Medicine (1985)

According to surveys, at least 70 per cent of doctors now believe in telling people the truth about their cancers, as compared with 82 per cent who practiced oppositely only 24 years ago.Surveys often show quick turnabouts of public opinion, especially toward issues and leaders, but the attitude of physicians in this area changed little in the 160 years before the past 20. Thomas Percival, in his Medical Ethics, first published in 1803, argues that "to a patient... who makes inquiries which, if faithfully answered, might prove fatal to him, it would be a gross and unfeeling wrong to . . .
An unfortunate teenager
Authors: Z. Sheikh, S. Khan, A. Khan
Journal: BMJ (2011)

A 19 year old man presented to the emergency department with sudden onset shortness of breath and sharp left sided chest pain that was worse on inspiration. He had no history of trauma and had been otherwise well before the onset of these symptoms. However, a year ago he had twice presented to hospital with similar symptoms. He had also undergone surgery on one of his heart valves two years previously but was unclear about the details. The only drug he was taking was warfarin for long term anticoagulation.

The patient was tall, thin, and visibly short of breath but was able to complete full sentences. Closer examination showed a respiratory rate of 22 breaths/min, a non-deviated trachea, and reduced air entry over the left hemithorax with a resonant percussion note throughout. Precordial auscultation showed a soft systolic murmur and mechanical S2, which was loudest over the cardiac apex. The jugular venous pulse was not visibly raised, the patient was normotensive, and pulse oximetry measured his oxygen saturation at 95% on room air.

Twelve lead electrocardiography showed normal sinus rhythm with no other abnormalities. Full blood count, renal function, electrolytes, and clotting were all normal, with an international normalised ratio of 1.1 reflecting poor compliance with warfarin. Chest radiography was performed (fig 1⇓).





### 1 What abnormalities are visible on the chest radiograph?

#### Short answer

A large left sided pneumothorax is present. A mechanical valve is seen in situ in the aortic position with some overlying metallic sternal sutures. Allowing for rotation, there is a degree of thoracic spine scoliosis.

#### Long answer

A large left sided …
Somatostatin-Receptor Imaging of Medullary Thyroid Carcinoma
Authors: Y KRAUSZ, S ISH-SHALOM, R B J DEJONG, N SHIBLEY, M LAPIDOT, Y MAARAVI, B GLASER
Journal: Clinical Nuclear Medicine (1994)

In-111 pentetreotide scintigraphy of 10 patients with residual or metastatic medullary thyroid carcinoma is described. Six patients had sporadic tumor and 4 had MEN IIB. Foci of increased tracer uptake were observed in 9 patients: in the thyroid bed (4 patients), the mediastinum (3 patients.), the shoulder area and left lower abdomen (1 patient), and the left upper abdomen (1 patient). The 10th patient had no abnormal uptake. CT confirmed 2 mediastinal lesions and 2 out of 3 thyroid masses, but did not detect the thyroid remnants or the lesions in the shoulder area and abdomen. Lung lesions < or = 1 cm in diameter and ill-defined liver foci (2 patients) were seen on CT, but not on scintigraphy. Small liver metastases not demonstrated on CT or on scintigraphy were identified at surgery in a MEN IIB patient. Elevated urinary epinephrine was found in 2 out of 4 MEN IIB patients. In one, tracer uptake in the left adrenal corresponded to a mass on CT, to pathological uptake of MIBG and DMSA, and to a tumor removed at surgery. The second patient had peritoneal spread of malignant pheochromocytoma (at surgery), but negative CT and only a single focus in the left lower abdomen on scintigraphy. Somatostatin-receptor imaging is useful for the detection of residual and recurrent medullary thyroid carcinoma, and may identify pheochromocytoma in MEN IIB patients.
Points: Anthropometric classification of fat distribution shown by computed tomography
Authors: J. Cox
Journal: BMJ (1985)

<h3>Abstract</h3> The development of Parkinson’s disease (PD) causes dysfunction of the frontal cortex, which contributes to the hallmark motor symptoms and is regarded as one of the primary causes of the affective and cognitive impairments observed in PD. Treatment with L-3,4-dihydroxyphenylalanine (L-DOPA) alleviates motor symptoms but has mixed efficacy in restoring normal cognitive functions, which is further complicated by the psychoactive effects of the drug. We investigated how L-DOPA affects gene expression in the frontal cortex in an animal model of unilateral PD. We performed RNA sequencing (RNA-Seq) analysis of gene expression in the frontal cortex of rats with 6-hydroxydopamine (6-OHDA)-induced unilateral dopaminergic lesions treated with L-DOPA, for two weeks. The analysis of variance identified 48 genes with a significantly altered transcript abundance after L-DOPA treatment. We also performed a weighted gene coexpression network analysis (WGCNA), which resulted in the detection of five modules consisting of genes with similar expression patterns. The analyses led to three primary observations. First, the changes in gene expression induced by L-DOPA were bilateral, although only one hemisphere was lesioned. Second, the changes were not restricted to neurons but also appeared to affect immune or endothelial cells. Finally, comparisons with databases of drug-induced gene expression signatures revealed multiple nonspecific effects, indicating that a part of the observed response is a common pattern activated by multiple types of drugs in different target tissues. Taken together, our results identify cellular mechanisms in the frontal cortex that are involved in the response to L-DOPA treatment.
Effects of a Ketogenic Diet on Brain Metabolism in Epilepsy
Authors: Kirsten Korsholm, Ian Law
Journal: Clinical Nuclear Medicine (2012)

For a subpopulation of drug-resistant epilepsies, a ketogenic diet constitutes the treatment of choice. A ketogenic diet is a high-fat, low-protein, and low-carbohydrate diet, which induces ketosis. Despite the use in treatment of epilepsy since 1924, the clinical efficacy was not demonstrated in a controlled, randomized trial until 2008, showing its capability of reducing seizure frequency with more than 50%. However, the exact mechanism of this form of treatment is still unknown. We report here a patient with drug-resistant epilepsy on a ketogenic diet, where a brain 18F-FDG PET examination demonstrated a severely decreased uptake in the cerebral cortex bilaterally.
The poly(A)-binding protein partner Paip2a controls translation during late spermiogenesis in mice
Authors: Akiko Yanagiya, Geraldine Delbes, Yuri V. Svitkin, Bernard Robaire, Nahum Sonenberg
Journal: Journal of Clinical Investigation (2010)

Translational control plays a key role in late spermiogenesis. A number of mRNAs encoding proteins required for late spermiogenesis are expressed in early spermatids but are stored as translationally inactive messenger ribonucleoprotein particles (mRNPs). The translation of these mRNAs is associated with shortening of their poly(A) tail in late spermiogenesis. Poly(A)-binding protein (Pabp) plays an important role in mRNA stabilization and translation. Three Pabp-interacting proteins, Paip1, Paip2a, and Paip2b, have been described. Paip2a is expressed in late spermatids. To investigate the role of Paip2 in spermiogenesis, we generated mice with knockout of either Paip2a or Paip2b and double-KO (DKO) mice lacking both Paip2a and Paip2b. Paip2a-KO and Paip2a/Paip2b-DKO mice exhibited male infertility. Translation of several mRNAs encoding proteins essential to male germ cell development was inhibited in late spermiogenesis in Paip2a/Paip2b-DKO mice, resulting in defective elongated spermatids. Inhibition of translation in Paip2a/Paip2b-DKO mice was caused by aberrant increased expression of Pabp, which impaired the interaction between eukaryotic initiation factor 4E (eIF4E) and the cap structure at the 5' end of the mRNA. We therefore propose a model whereby efficient mRNA translation in late spermiogenesis occurs at an optimal concentration of Pabp, a condition not fulfilled in Paip2a/Paip2b-DKO mice.
Detection of Ferritin in the Plasma of Guinea Pigs in Experimental Shock
Authors: AARON JANOFF, BENJAMIN W. ZWEIFACH, ARNOL L. NAGLER, ZOLTAN OVARY
Journal: Circulation Research (1961)

Guinea pigs were subjected to shock by hemorrhage or by infusion of either E. coil or S. enteritidis endotoxin, and changes in levels of plasma-bound iron and ferritin were assayed. Of 12 bled animals, all showed increases in plasma-bound iron and half gave a positive test for ferritin. Of 12 guinea pigs treated with LD 80 doses of E. coli endotoxin, only 4 showed an elevation in plasma-bound iron. Of these only one was positive for ferritin. Of 10 guinea pigs treated with LD 80 doses of S. enteritdis endotoxin, only five showed elevations in plasma-bound iron, and three of these also showed detectable increases in plasma ferritin. Immunologically detect able increases in plasma ferritin were not found in any of the endotoxin-infused animals showing decreases in plasma-bound iron. It was concluded: (a) that, in the guinea pig (as in the rabbit), hyperferremia is more frequent after hemorrhage than after the in fusion of Gram-negative bacterial endotoxin (b) that significant increases in ferritin do not appear simultaneously with decreases in total plasma-hound iron in endotoxic guinea pigs; and (c) that hyperferremia and hyperferritinemia do not play a direct role in the lethal progression of endotoxic shock in the guinea pig.
Inflammatory bowel disease in the West Indies.
Authors: C Bartholomew, A Butler
Journal: BMJ (1979)

Inflammatory bowel disease is generally assumed to be rare among negroes and Indians. Over 10 years 34 cases of ulcerative colitis and 14 cases of Crohn's disease were seen in one medical and one surgical unit in Port-of-Spain, Trinidad. Twenty-six patients were Negroes, 18 were Indians, three were of mixed race, and one was Caucasian. In many of these patients the disease was extensive and several of those with Crohn's disease suffered severe complications. The assumption that inflammatory bowel disease is rare among West Indians of African and Indian origin therefore seems to be wrong.
Snipping away at hepatitis C
Authors: Nicholas A. Shackel, David G. Bowen, Geoffrey W. McCaughan
Journal: Hepatology (2010)

Abstract Chronic infection with hepatitis C virus (HCV) affects 170 million people worldwide and is the leading cause of cirrhosis in North America. Although the recommended treatment for chronic infection involves a 48-week course of peginterferon-alpha-2b (PegIFN-alpha-2b) or-alpha-2a (PegIFN-alpha-2a) combined with ribavirin (RBV), it is well known that many patients will not be cured by treatment, and that patients of European ancestry have a significantly higher probability of being cured than patients of African ancestry. In addition to limited efficacy, treatment is often poorly tolerated because of side effects that prevent some patients from completing therapy. For these reasons, identification of the determinants of response to treatment is a high priority. Here we report that a genetic polymorphism near the IL28B gene, encoding interferon-lambda-3 (IFN-lambda-3), is associated with an approximately twofold change in response to treatment, both among patients of European ancestry (P = 1.06 x 10(-25)) and African-Americans (P = 2.06 x 10(-3)). Because the genotype leading to better response is in substantially greater frequency in European than African populations, this genetic polymorphism also explains approximately half of the difference in response rates between African-Americans and patients of European ancestry.
DICK TEST AND IMMUNIZATION AGAINST SCARLET FEVER
Authors: O. B. NESBIT
Journal: JAMA (1925)

The staff of Mercy Hospital, composed of practically all the physicians and surgeons of Gary, asked the board of education to offer, through its medical inspection department, diphtheria and scarlet fever immunization, and endorsed a program which included an invitation to Drs. George F. and Gladys H. Dick to conduct a clinic on scarlet fever prevention. Accordingly, July 24, 1924, Dr. Gladys Dick administered seventy-seven Dick tests to a selected group, including children from all parts of the city and a number of adults. The following day Dr. George Dick read a paper on their work on scarlet fever, and Dr. Gladys Dick interpreted the tests. The immunization of the susceptible was commenced. For school work, the Dicks and I worked out the accompanying blank forms, printed on a 3 by 5 card, the front of the card being used for a request from the parent, and the back for
Fibroblast-keratinocyte interactions in psoriasis: failure of psoriatic fibroblasts to stimulate keratinocyte proliferation in vitro
Authors: G.C. PRIESTLEY, R. LORD
Journal: British Journal of Dermatology (2006)

We have tested in two ways the hypothesis that dermal fibroblasts direct the hyperproliferation of the overlying epidermis in psoriasis. First, culture medium from psoriatic and from normal skin fibroblasts was added to monolayer cultures of foreskin keratinocytes. Second, psoriatic and normal fibroblasts embedded in hydrated collagen lattices were co-cultured with monolayers of foreskin keratinocytes. There was no evidence in either study that psoriatic fibroblast products could stimulate the proliferation of the keratinocytes, or that normal fibroblast products inhibited their proliferation. A positive control for the fibroblasts was provided by leucocyte supernatants, which stimulated keratinocyte proliferation by up to 65%. Our data do not support a primary role for dermal fibroblasts in psoriasis.
Cutaneous manifestations of fucosidosis
Authors: C. FLEMING, A. RENNIE, M. FALLOWFIELD, P.M. McHENRY
Journal: British Journal of Dermatology (1997)

Angiokeratoma corporis diffusum (ACD) is still often thought to be synonymous with Anderson-Fabry disease, a deficiency of alpha-galactosidase. It is important, however, to consider other possible enzyme deficiencies in patients with ACD. We report an 8-year-old boy with neurodevelopmental delay who was diagnosed as having fucosidosis following recognition of ACD in the dermatology department. Other cutaneous features in this patient included distal transverse purple nail bands, acrocyanosis and a naevus anaemicus. Histology and electron microscopy of skin papules was consistent with angiokeratoma. Skeletal survey demonstrated dysostosis multiplex. The diagnosis was confirmed by leucocyte oligosaccharide enzyme analysis. There are only three previous reports of fucosidosis in the U.K.
Dual echocardiographic determination of atrial contraction sequence in atrial flutter and other related atrial arrhythmias.
Authors: J Fujii, J R Foster, P G Mills, S Moos, E Craige
Journal: Circulation (1978)

We have applied the new technique of dual echocardiography to determine the sequence of atrial contraction as reflected in the simultaneously recorded movements of the tricuspid and mitral valves. The study group included 29 normal subjects and 23 patients with either atrial flutter, coarse atrial fibrillation or atrial tachycardia with block. In normal individuals, right atrial contraction preceded left atrial contraction, with an average interatrial contraction time of 17 +/- 8 msec. In contrast, the atrial contraction sequence was reversed in atrial flutter, with left preceding right atrial contraction and a prolonged interatrial contraction time of 82 +/- 20 msec. In two patients with atrial tachycardia with block, atrial contraction was either simultaneous or left preceded right atrial contraction by a brief interval. The sequence of atrial excitation, as determined by electrode catheter recordings from the right and left atria in one patient with atrial flutter and one patient with normal sinus rhythm, was the same as the contraction sequence. Left atrial pacing reversed both excitation and contraction sequences. After cardioversion of three patients from atrial flutter to normal sinus rhythm, interatrial contraction time was shortened but remained longer than in normal subjects, suggesting an interatrial conduction disturbance in patients with atrial flutter. In coarse atrial fibrillation, the contraction sequence varied. Significant motion of both mitral and tricuspid valves coincident with fibrillary waves occurred frequently, especially when the fibrillary waves were coarse and regular. Dual echocardiography permits the noninvasive determination of the sequence of atrial contraction and excitation, and may be useful in studying the characteristics of atrial arrhythmias.
Decitabine as “bridge therapy” to a MUD transplant in relapsed AML postautologous stem cell transplantation
Authors: Celalettin Ustun, Abhishek Kalla, Stephanie Farrow, David L. Deremer, Anand Jillella
Journal: American Journal of Hematology (2008)

RAD51 and XRCC3 polymorphism frequency and risk of myelodysplastic syndromes P value for w 2 or Fisher's exact test for allele frequency where appropriate.
Myelodysplastic syndrome and acute myeloid leukemia after treatment with fludarabine, mitoxantrone, and dexamethasone
Authors: Wing-Yan Au, Li-Chong Chan, Raymond Liang, Yok-Lam Kwong
Journal: American Journal of Hematology (2006)

Induction Chemotherapy and Post-Remission Imatinib Therapy for de Novo BCR-ABL-Positive AMLTo the Editor: Translocation t(9;22)(q34;q11) is found in 1-2% of newly diagnosed patients with de novo AML.The prognosis of Ph þ AML is very poor with a median survival time of only 7 months.We present two patients with de novo Ph þ AML who received induction chemotherapy and post-remission imatinib therapy.A 73-year-old man was admitted in November 2003 with fatigue and palpitation.Physical examination showed only pallor.The Hb was 83 g/L, platelets 145 Â 10 9 /L, and WBC 1.4 Â 10 9 /L, 0.12 Â 10 9 /L blast cells.FACS analysis of marrow mononuclear cells showed 22% blast cells positive for HLA-DR, CD34, CD117, CD13, CD33, MPO, and CD38 and partially CD11b.Karyotype analysis of bone