tbuckley/cabot-search huggingface dataset:
A132: Farber Disease Explains Subset of Juvenile Idiopathic Arthritis
Authors: Edward Schuchman
Journal: Arthritis & Rheumatology (2014)

Background/Purpose: Farber Lipogranulomatosis (Farber Disease; FD) is an ultra‐;rare lysosomal storage disorder due to the inherited deficiency of the enzyme acid ceramidase, and the resultant accumulation of the lipid substrate, ceramide. Ceramide is a proinflammatory and pro‐;apoptotic lipid that has been implicated in the pathogenesis of cartilage disorders. Farber Disease has a heterogeneous presentation ranging from a severe phenotype with respiratory and CNS involvement with an average life expectancy of 1.3 years (Type 1) to a moderate phenotype which generally includes joint swelling, contractures and pain (Type 3). Awareness of FD in the pediatric arthritis community is limited due to the rarity of the disease. The clinical similarity between the moderate Farber phenotype and Juvenile Idiopathic Arthritis (JIA) suggests that moderate Farber Disease cases would be classified as JIA cases. Methods: To test the hypothesis that moderate Farber Disease is diagnosed as Juvenile Idiopathic Arthritis, we conducted a literature search of Farber Disease case studies since 1990 using search terms “Farber Disease,” “Farber's Disease” and “Farber lipogranulomatosis” in PubMed published in English. Moderate Farber Disease patients were defined as surviving into their third year of life without hepatosplenomegally or significant neurological involvement. Cases where idiopathic arthritis was suspected were noted. Results: The literature search identified 84 unique papers with 35 comprising Farber Disease case studies. Of the overall case studies, 14 or 40% described patients with moderate disease. Five or 36% of these patients were diagnosed as having Juvenile Idiopathic Arthritis. Conclusion: A high percentage of moderate Farber Disease patients are diagnosed as having Juvenile Idiopathic Arthritis. Several of the patients described were referred to secondary or tertiary centers for treatment, which suggests that this percentage may be understated. Given this finding, it is important to increase the awareness of Farber Disease in the pediatric rheumatology community. Differential diagnosis can be made by accounting for the comparatively early onset of FD, the progressive severity of arthritis in FD, the presence of subcutaneous nodules in the joints, scalp and/or spine of FD patients and an unusual, hoarse cry or voice in FD patients, whichi is due to nodule formation on the larynx. Plexcera Therapeutics LLC is a new company focused on developing acid ceramidase enzyme therapy for disorders with ceramide accumulation ( www.plexcera.com ). Based on experience with bone marrow transplant in Farber Disease patients, enzyme replacement therapy could resolve arthritis phenotype in patients. We propose that FD can account for specific cases of JIA, and that genetic screening of the JIA population will reveal FD patients that will benefit from future treatment with rhAC.
How<i>Staphylococcus aureus</i>Adapts to Its Host
Authors: Franklin D. Lowy
Journal: New England Journal of Medicine (2011)

Why Staphylococcus aureus, like some other bacterial pathogens, is able to target some animals and not others remains a puzzle. Recent research provides new insights into pathogenesis by focusing on the way in which the bacteria acquires iron from its host's hemoglobin.
Reply to the Letter of Dr. Cho et al.
Authors: R. Lambert
Journal: Endoscopy (2006)

We are happy to thank the Korean team from Seoul for their very pertinent comment. They draw attention to the limited reliability of the assessment of the depth of invasion of a cancer in a specimen resected during endoscopy. The established cutoff limits for safety of endoscopic treatment (200 µm in esophagus, 500 µm in stomach and 1000 µm in colon) are based on surgical series, and are preferred to the semiquantitative estimation in three sectors of the submucosa (superficial, middle, and deep, or sm1, sm2, and sm3 sectors). In fact, when surgical specimens, with full thickness of the submucosa and full thickness of the digestive wall, are stretched in a support, prior to fixation in formalin, their elasticity is limited. Pathology studies have confirmed that the risk of lymphatic metastases is very low, when invasion is less than the cutoff limit.
Back Diffusion of Hydrogen Ions across Gastric Mucosa of Patients with Gastric Ulcer and Rheumatoid Arthritis
Authors: K. J. Ivey, J. A. Clifton
Journal: BMJ (1974)

Ionic permeability of the gastric mucosa was measured in six patients with an acute exacerbation of severe generalized rheumatoid arthritis receiving either aspirin and prednisone or aspirin and indomethacin as therapy. The results were compared with those in four patients with benign gastric ulcer and nine normal subjects. Compared with controls H(+) concentration was decreased and Na(+) concentration increased while corrected H(+) flux out of the lumen and Na(+) flux into the lumen were significantly increased in the patient groups, indicating increased mucosal permeability. Abnormality of the gastric mucosal barrier persisted in two patients despite healing of their ulcers. Mucosal permeability of patients with rheumatoid arthritis and gastric ulcer did not differ significantly from one another. One rheumatoid patient with a gastric ulcer showed no difference in mucosal permeability to that of the other rheumatoid patients. These studies suggest that increased H(+) ion loss contributes to the apparent hyposecretion of acid in patients gastric ulcer; persistence of an abnormal gastric mucosal barrier to H(+) ions may explain the high recurrence rate of gastric ulcers; and an abnormal gastric mucosal barrier may be a precursor to gastric ulceration in rheumatoid arthritis.
The effects of chenodiol on biliary lipids and their association with gallstone dissolution in the National Cooperative Gallstone Study (NCGS).
Authors: S M Grundy, S P Lan, J Lachin
Journal: Journal of Clinical Investigation (1984)

The National Cooperative Gallstone Study was a double-masked trial conducted to determine the efficacy and safety of chenodeoxycholic acid (chenodiol) for dissolution of cholesterol gallstones. Patients with radiolucent gallstones were randomly allocated to either a high dose (750 mg/d, n = 305) or low dose (375 mg/d, n = 306) of chenodiol or placebo (n = 305) administered for 2 yr. Specimens of gallbladder bile were obtained for biliary lipid analysis on 50% of all white obtained for biliary lipid analysis on 50% of all white patients at base line and after 3-mo therapy, on 45% at 12 mo, and on 36% at 24 mo. Among these specimens, 20% were inadequate for analysis. For analysis of data, available values during therapy were averaged up to time of dissolution, study exit, or study termination. In the high-dose group, percent chenodiol (molar percent of all bile acids) increased markedly and remained high during the 2 yr of follow-up. Also, molar percent cholesterol decreased significantly and remained low during the 2 yr of follow-up. In the low-dose group, percent chenodiol increased and remained significantly increased. Percent cholesterol saturation decreased at 3 mo, but at 24 mo it was not different from that in the placebo group, suggesting a physiological adaptation to the low dose by 2 yr. 79% of patients on high dose had greater than 70% chenodiol. Among these, half showed unsaturated bile (less than 100% cholesterol saturation) while the remainder were supersaturated; in the former group with unsaturated bile, 23% had complete dissolution and 51% had partial (greater than 50% reduction in stone size) or complete dissolution. In contrast, those with over 70% chenodiol and supersaturated bile had only 5% complete dissolution. Thus, development of unsaturated bile was a major factor associated with gallstone dissolution. The data also indicate that values for percent cholesterol saturation were a better predictor of gallstone dissolution than molar percent chenodiol, although a high percent chenodiol usually was required to obtain unsaturation.
Peptic Ulcer Disease and Neuroticism in the United States Adult Population
Authors: Renee D. Goodwin, Murray B. Stein
Journal: Psychotherapy and Psychosomatics (2002)

&lt;i&gt;Background:&lt;/i&gt; The goal of the current study was to determine the association between personality factors and peptic ulcer disease (PUD) among adults in the general population. &lt;i&gt;Method:&lt;/i&gt; Data were drawn from the Midlife Development in the United States Survey (MIDUS), a representative household survey of the adult population (ages 25–74; n = 3,032) of the United States. Multiple logistic regression analyses were used to determine the association between the big five personality factors and PUD, adjusting for differences in sociodemographic characteristics, psychiatric and physical comorbidity. &lt;i&gt;Results:&lt;/i&gt; Neuroticism was associated with significantly increased odds of PUD [OR = 1.5 (95% CI: 1.03, 2.4)], which persisted after controlling for differences in sociodemographic characteristics, cigarette smoking, perception of poor health, comorbid mental disorders and physical illnesses. This relationship was specific to neuroticism. &lt;i&gt;Conclusions:&lt;/i&gt; These findings are consistent with and extend previous clinical and epidemiologic data by providing evidence of an independent association between neuroticism and PUD among adults in the general population. Future work investigating the relationship between neuroticism and the development of PUD in prospective data, including objective measures of physical and mental health status, may contribute to our understanding of this association.
Stellate Red-Brown Patches on the Distal Extremities
Authors: Sapna Desai, Emily Keimig
Journal: JAMA Dermatology (2015)

This case ofA woman in her 40s presented with a 1-year history of episodic muscle cramping, numbness involving the lower extremities, and stellate red-brown patches on her extremities and buttocks without induration, scale, or ulceration. What is your diagnosis?
DIAGNOSIS OF HERPES SIMPLEX INFECTIONS BY THE COMPLEMENT FIXATION TEST
Authors: D. Carleton Gajdusek
Journal: JAMA (1952)

Herpes simplex is among the most ubiquitous of viruses capable of infecting man, and the clinical manifestations of the infection it induces are various. Until recently no simple and rapid serologic test has been available by which the antibody response to infection with this virus could be determined. When serologic studies have been carried out, the virus neutralization test usually has been employed, although antigens for use in complement fixation and skin tests have been described. The present study was undertaken to explore the value as antigens in the complement fixation test of amniotic and allantoic fluid from infected chick embryos. When such materials were shown to be effective in this respect, the reliability of the test as a diagnostic procedure and as a tool for the epidemiological study of herpes infections was investigated. Earlier workers who applied complement fixation tests to the assay of herpes antibody used antigens prepared
INHBA Overexpression Promotes Cell Proliferation and May Be Epigenetically Regulated in Esophageal Adenocarcinoma
Authors: Christopher W. Seder, Wibisono Hartojo, Lin Lin, Amy L. Silvers, Zhuwen Wang, Dafydd G. Thomas, Thomas J. Giordano, Guoan Chen, Andrew C. Chang, Mark B. Orringer, David G. Beer
Journal: Journal of Thoracic Oncology (2009)
Chronic dermatophytosis in lamellar ichthyosis: relevance of a T-helper 2-type immune response to Trichophyton rubrum.
Authors: R.J. Ludwig, J.A. Woodfolk, M. Grundmann-Kollmann, R. Enzensberger, U. Runne, T.A.E. Platts-Mills, R. Kaufmann, T.M. Zollner
Journal: British Journal of Dermatology (2001)
Angiography in the Diagnosis of Parathyroid Carcinoma
Authors: Plinio Rossi, Ferdinand J. Carillo, Barbara Johnston
Journal: New England Journal of Medicine (1971)

ANGIOGRAPHY has been shown to be useful in the diagnosis and localization of parathyroid adenoma, but it has never been described for parathyroid carcinoma. It is our purpose to present the angiographic findings in a case of parathyroid carcinoma and to indicate its usefulness in correlating the clinical history with the laboratory data for a definite preoperative diagnosis.Case ReportA 51-year-old man was in good health until 1 1/2 years before the present admission, when he was admitted to the hospital with a pathologic fracture of the left humerus. Multiple radiographs taken at this time revealed, in addition to . . .
Abstract P1-03-08: Targeting Neurotensinergic Receptors for Bone Cancer Pain Relief
Authors: R Geneviève, M Lemire, K Belleville, N Beaudet, J Martinez, S. Philippe
Journal: Cancer Research (2010)

Abstract Metastatic breast cancer spreads principally to long bones like femur. The presence of breast cancer metastasis in bone microenvironment results in severe pain increasing with disease progression. Opioids, including morphine, are a standard for bone cancer pain management despite their adverse effects at high doses. Our aim here was to examine the effects of opioidergic-independent neurotensin (NT) receptor (NTS1, NTS2) activation in alleviating bone cancer pain in a new clinically relevant mouse model. To this end, neurotensinergic agonists (PD149163, JMV-2012 or JMV-431) were intrathecally administrated to C57BL/6 mice implanted with syngenic E0771 metastatic breast cancer cells in the femoral intramedullary canal. Compounds were analysed for touch-evoked (von Frey), forced movement-evoked (Rotarod) and ambulatory (dynamic weight bearing) pain 18 days following E0771 implantation. The intrathecal injection of PD149163, a NTS1-selective agonist, induced a marked decrease of touch-evoked pain reaching 64 ± 9%. On the Rotarod, PD149163 treatment failed to improve either time spent on the rotating cylinder or the visual pain score. The injection of a non-selective agonist, JMV-2012, reduced touch-evoked pain of 16 ± 5%, but was not efficient in reversing pain under a physical effort on the Rotarod. JMV-431, a NTS2-selective agonist, produced a significant reduction of touch-evoked pain of 70 ± 9% in cancer mice, which also spent more time (10 ± 13%) on the Rotarod and expressed less movement-evoked pain (97 ± 20%) than saline-treated cancer mice. However, acute spinal JMV-431 failed to restore ipsilateral weight bearing toward basal values. The analysis of spinal neuronal activity of mice injected with JMV-431 showed a significant reduction of 55 ± 6% of c-Fos expression in laminae V-VII of the ipsilateral horn 18 days post implantation. In conclusion, these results indicate that the NTS2 receptor represents a promising target for treating bone cancer pain. Further investigations of continuous delivery of NTS2-selective agonists at the central and peripheral levels are under way to evaluate the real potential of these compounds for prophylactic and curative treatments against cancer pain. Supported by CIHR/Canadian Pain Society/AstraZeneca and Cancer Research Society grants. Citation Information: Cancer Res 2010;70(24 Suppl):Abstract nr P1-03-08.
Bulimic eating patterns should be stabilised in polycystic ovarian syndrome
Authors: J. F Morgan
Journal: BMJ (1999)

EDITOR—Hopkinson et al have reviewed the polycystic ovarian syndrome and emphasised that it represents more than a purely gynaecological disorder.1 They also emphasised the link between insulin resistance and obesity in its pathogenesis. They made no mention, however, of the role of bulimia nervosa. McCluskey et al found that three …
Acute Renal Colic from Ureteral Calculus
Authors: Joel M H Teichman
Journal: New England Journal of Medicine (2004)

A 39-year-old man reports an eight-hour history of colicky pain in the right lower quadrant radiating to the tip of his penis. He had previously had a kidney stone, which passed spontaneously. Examination shows he has tenderness of the right costovertebral angle and right lower quadrant. Urinalysis shows microhematuria. Helical computed tomography shows a 6-mm calculus of the right distal ureter and mild hydroureteronephrosis. How should this patient be treated?
Oxygen Transport in Vivo by Means of Perfluorochemical Preparations
Authors: Robert P. Geyer
Journal: New England Journal of Medicine (1982)

For many years a search has been under way for oxygen-transporting materials that can be used in blood replacement. At one time it was natural to consider hemoglobin the only practical solution to this problem. Hemoglobin preparations free of insoluble residues of red cells have been produced, and they have greatly reduced the kidney damage associated with earlier products. Other compounds, such as polyhemoglobin, are being developed to circumvent the rapid loss of hemoglobin from the circulation that occurs when it is given intravenously. Little clinical work has been done with hemoglobin or its derivatives to date, but a number . . .
A retired shipyard worker with rapidly progressive pulmonary interstitial fibrosis.
Authors: E V Moy, H Hu, D C Christiani
Journal: Environmental Health Perspectives (1999)

We present a case of progressive interstitial fibrosis in a retired shipyard worker who was exposed to asbestos during the postwar era of the late 1940s and 1950s, when asbestos exposures in the workplace were not regulated. Forty years later, at 63 years of age, the patient presented with restrictive lung disease. The patient was diagnosed with asbestos-related pleural disease and parenchymal asbestosis. He remained stable for the next 7 years, but then he began to manifest rapid clinical progression, which raised the possibility of an unusual variant of asbestosis, a concomitant interstitial process, or an unrelated disease. Lung biopsy was not undertaken because of the patient's low pulmonary reserve and limited treatment options. An empiric trial of oral steroids was initiated, but his pulmonary status continued to deteriorate and he died of pulmonary failure at 72 years of age. Many diseases result in pulmonary interstitial fibrosis. Ideally, open lung biopsy should be performed, but this procedure inevitably causes complications in many patients with end-stage restrictive lung disease. Furthermore, while the presence of asbestos bodies in tissue sections is a sensitive and specific marker of asbestos exposure, neither this finding nor any other charge is a marker indicative of asbestosis or the severity of asbestosis. With the enactment of the Asbestos Standard in the United States, asbestos exposures have been decreasing in this country. However, industries that produce asbestos products and wastes continue to expand in developing countries. Prevention of asbestos-related lung disease should be a global endeavor, and asbestos exposures should be regulated in both developed and developing countries.
<b><i>Immediate Early Response Gene X-1</i></b>, a Stress-Inducible Antiapoptotic Gene, Encodes Cytotoxic T-Lymphocyte (CTL) Epitopes Capable of Inducing Human Leukocyte Antigen-A33-Restricted and Tumor-Reactive CTLs in Gastric Cancer Patients
Authors: Tetsuro Sasada, Hiroko Takedatsu, Koichi Azuma, Makoto Koga, Yoshiaki Maeda, Shigeki Shichijo, Hiroki Shoumura, Tatsuya Hirai, Arimichi Takabayashi, Kyogo Itoh
Journal: Cancer Research (2004)

Abstract Peptide-based vaccine therapy, which is designed to elicit T-cell immunity against tumors, is an attractive approach for the treatment of cancer patients. To provide a scientific basis for peptide therapy, an increasing number of CTL-directed peptides have been identified, and some of them have been tried as antigen-specific immunotherapy in the past decade. Only a few studies, however, have been performed on such peptides restricted with alleles other than HLA-A2 and −A24. In the present study, we show that immediate early response gene X-1 (IEX-1), a stress-inducible protein associated with the regulation of cell proliferation and apoptosis, produces antigenic epitopes recognized by 850B-CTLs, HLA-A33-restricted CTLs newly established from T cells infiltrating into gastric adenocarcinoma. The IEX-1 gene was highly expressed in most cell lines and tissues from various types of cancer at both the mRNA and protein levels. However, it was not expressed at the protein level in any normal epithelium or connective tissues tested. Three IEX-1-derived peptides at positions 47–56, 61–69, and 65–73, which were recognized by the 850B-CTLs, could induce CD8+ peptide-specific CTL reaction to tumor cells from HLA-A33+ gastric cancer patients and other epithelial cancer patients, but not from healthy donors, in an HLA class I-restricted manner. Because increased expression of IEX-1 is suggested to be involved in the resistance to apoptosis and in the proliferation of cancer cells, these antigenic peptides could be potent candidates for peptide-based specific immunotherapy against HLA-A33+ gastric cancer and other epithelial cancers.
Preventive Effect of β-Adrenoceptor Blockade on Glucocorticoid-Induced Memory Retrieval Deficits
Authors: Dominique J.-F. de Quervain, Amanda Aerni, Benno Roozendaal
Journal: American Journal of Psychiatry (2007)

Objective: Elevated glucocorticoid levels impair retrieval of emotional information, and animal studies indicate that this effect depends on concurrent emotional arousal-induced increases in noradrenergic transmission within the brain. The authors investigated whether the β-adrenoceptor antagonist propranolol blocks glucocorticoid-induced memory retrieval impairments in human subjects. Method: In a double-blind, placebo-controlled study, 42 healthy volunteers were presented a set of words with variable emotionality and asked to learn them for recall. A day later, cortisone (25 mg), propranolol (40 mg), or both drugs were administered orally 1 hour before a free-recall test. Results: Cortisone selectively impaired the recall of emotionally arousing words by 42%. This impairment was blocked by the concurrent administration of propranolol. Propranolol alone did not affect recall of either emotional or neutral words. Conclusions: A pharmacological blockade of β-adrenoceptors prevents glucocorticoid-induced memory retrieval deficits in human subjects. This finding may have important implications for the treatment of memory deficits in hypercortisolemic states, such as stress and depression.
Radiography, Bone Scintigraphy, SPECT/CT and MRI of Fibrous Dysplasia of The Third Lumbar Vertebra
Authors: Zhen Zhao, Lin Li, Fang-lan Li
Journal: Clinical Nuclear Medicine (2009)

A 32-year-old woman, with destruction of bone in the left pedicle of L3 on radiographs, was referred for Tc-99m methylene diphosphonate bone scintigraphy. Planar imaging showed a focal area of increased radioactivity in the left side of the L3 vertebral body. Single photon emission computed tomography/computed tomography (SPECT/CT) fusion imaging disclosed an osteolytic lesion and intense focal uptake at L3, suggesting giant cell tumor of bone. Magnetic resonance imaging (MRI) revealed heterogeneous signal intensities in the left posterior part of L3, suggesting an aneurysmal bone cyst or giant cell tumor. Subsequent surgery and histopathologic examination confirmed fibrous dysplasia (FD) of bone. The diagnosis was based on multiple imaging modalities and was difficult because of the unusual location of the lesion and the absence of characteristic signs.