tbuckley/cabot-search huggingface dataset:
Defective Granulocyte Regulation in the Chediak–Higashi Syndrome
Authors: Ralph S. Blume, John M. Bennett, Ronald A. Yankee, Sheldon M. Wolff
Journal: New England Journal of Medicine (1968)

The serum muramidase activities of four granulocytopenic patients with the Chediak—Higashi syndrome were always strikingly elevated, indicating a markedly increased rate of granulocyte turnover. Two of the patients, both with histologic and clinical evidence of the accelerated (infiltrative) phase of the syndrome, had intermittent hypersplenism manifest by splenomegaly, thrombocytopenia and hemolytic anemia with splenic sequestration. The administration of prednisone to these two patients corrected manifest hypersplenism but only partly corrected the granulocytopenia and elevation of serum muramidase activity. The other two patients were shown to have normal peripheral granulocyte survival, with no evidence of hypersplenism. None of the patients responded to the administration of etiocholanolone with the expected granulocytosis. All four exhibited marked cytoplasmic abnormalities in bone-marrow granulocyte precursors, different from those seen in peripheral granulocytes. These findings are consistent with intramedullary granulocyte destruction.
Salmonellosis in Massachusetts
Authors: Robert A. MacCready, Joseph P. Reardon, Ivan Saphra
Journal: New England Journal of Medicine (1957)

DURING the sixteen-year period from 1940 through 1955, the Diagnostic Laboratory of the Massachusetts Department of Public Health cultured for bacterial enteric pathogens 144,538 specimens consisting of stools, bloods, urines, pus, exudates and related material. The New York Salmonella Center at the Beth Israel Hospital typed 2625 cultures, or most of the salmonellas other than Salmonella typhi (not including repeat specimens) that have been isolated by the Diagnostic Laboratory during this period.By means of questionnaires answered by numerous health officers and their assistants, as well as physicians and nurses, the bare records of the salmonella types reported have been . . .
Pneumatosis of the Descending Colon
Authors: Kennedy Creevey, Jerome L. Cohen, George J. Riley
Journal: New England Journal of Medicine (1958)

PNEUMATOSIS cystoides intestinalis is a rare condition characterized by the presence of gas-filled cysts involving the intestinal wall and its attachments. It is known under a variety of names: gas cysts of the intestine, pseudocyst and intestinal emphysema. The condition is so unfamiliar that in most cases it is diagnosed on the operating table. Since the etiology and natural evolution of this disease are not well understood, the therapeutic indications may be quite uncertain.In a review published in 1952 Koss1 gathered reports of only 213 cases of pneumatosis intestinalis from the world literature. Excluding 32 heterogeneous cases these fell . . .
Use of Surveillance Systems in Detection of a Ciguatera Fish Poisoning Outbreak — Orange County, Florida, 2014
Authors: Benjamin G. Klekamp, Dean Bodager, Sarah D. Matthews
Journal: MMWR Morbidity and Mortality Weekly Report (2015)

What is already known on this topic? Ciguatera fish poisoning (CFP), caused by the ingestion of predatory reef-dwelling fish harboring ciguatoxins is one of the most commonly reported fish-associated marine intoxications. Ciguatoxin retains toxicity regardless of freezing or cooking. Prompt treatment can reduce debilitating neurologic symptoms that are associated with CFP.What is added by this report? Syndromic surveillance systems in Florida identified six adults with CFP following consumption of black grouper. Five patients sought medical attention; health care providers did not make a diagnosis of CFP or report the cases to public health authorities, and none of the patients received treatment. Close collaboration among several investigating agencies allowed traceback efforts to link black grouper consumed by all patients to a common international distributor.What are the implications for public health practice? Syndromic surveillance systems capable of detecting CFP are essential public health tools to identify outbreaks and enhance investigations. Medical and public health practitioners should be educated to inquire about recent fish consumption when evaluating patients with clinically compatible signs and symptoms to allow for prompt treatment, and report suspected CFP cases to public health authorities to facilitate source-food traceback efforts. Public education on avoidance of consumption of relatively large predatory reef fish species known to be from ciguatoxic-endemic areas might reduce the risk for CFP.
Sepsis in a Newborn Due to<i>Pseudomonas aeruginosa</i>from a Contaminated Tub Bath
Authors: Matthias Vochem, Manfred Vogt, Gerd Döring
Journal: New England Journal of Medicine (2001)

The Sounding Board by Bedell et al. on the doctor's letter of condolence (April 12 issue) 1 was distressing to read.My wife of 40 years died at a prestigious teaching hospital.For nearly four years she had struggled with carcinoma of the fallopian tube.There were 26 hospital admissions for surgery, chemotherapy, or emergency rehydration.When standard approaches ultimately failed, she managed, largely through her own research, to become enrolled at another distinguished hospital in a high-risk experimental protocol: high-dose chemotherapy followed by transplantation of autologous bone marrow.But the regimen turned out to be lethal.She died after three days in the emergency cardiac unit.Neither the hospital management nor the attending physician or anyone from the house staff ever troubled to write or telephone to express sympathy or offer an account of what had gone so wrong those last disastrous days.The effect of that bizarre silence was to make me wonder whether some monumental mistake might have been made in preparing the dose that was to end her life.The next of kin are entitled to some expression of sympathy or concern, even when it is not deeply felt.Those close to the deceased can only be baffled, resentful, or suspicious when no condolences are expressed.
S15 Measuring bronchodilator response by interrupter technique to predict response to inhaled steroid therapy in wheezy preschool children
Authors: R Willson, C Olden, L Symes, N Beydon, E Lombardi, D Wertheim, P Seddon
Journal: Thorax (2015)

<h3></h3> Managing recurrent wheezing in preschool children is problematic, with conflicting evidence on the effectiveness of asthma therapy. One reason is the heterogeneous nature of preschool wheeze: distinguishing “transient viral wheeze” from asthma at presentation is currently impossible. Bronchodilator response (BDR) measured by interrupter resistance (Rint) is greater in preschool children with diagnosed asthma than in healthy controls, but the usefulness of Rint in clinical practice has not been studied. We aimed to assess whether measuring BDR using Rint can predict clinical response to inhaled corticosteroids (ICS). We studied children aged 2 years to 1. Response to ICS was defined as reduction in daily symptom score of at least 0.26.<sup>2</sup> Association between BDR and ICS response was assessed using chi-square test. We also assessed the relationship between measured BDR and the caregiver’s assessment. 121 children were recruited, 59 completed the full study (27 withdrew, 10 were unable to perform Rint, 18 failed to meet 4w symptom threshold, 7 commenced ICS or Montelukast by clinician before 4w). Positive BDR at baseline was demonstrated by 33 children, and was significantly associated with a clinical response to ICS (p &lt; 0.05). There was no relationship between objective BDR and caregiver’s rating of bronchodilator efficacy. Our findings suggest that measuring BDR using Rint may help in making pragmatic decisions on commencing asthma preventer therapy in wheezy preschool children. Further work is needed to assess whether Rint BDR measurement may have the potential to distinguish those with transient wheeze from those with asthma. <h3>References</h3> 1 <i>Pediatr. Pulmonol</i>. 2010;<b>45</b>:633–638 2 <i>Pediatrics</i> 2001;<b>108</b>:E48
The Effect of Prenatal Exposure to Heavy Metals on Atopic Dermatitis: A Population-Based, Prospective Birth Cohort Study (COCOA)
Authors: Jihyun Kim, Sook-young Woo, Sun-Woo Kim, Jaehee Choi, Jin-Yong Chung, Young-Seoub Hong, Youngshin Han, Se-Young Oh, Suk-Joo Choi, Soo-Young Oh, Kyung Won Kim, Youn Ho Shin, Hye-Sung Won, Kyung-Ju Lee, Hee Jin Park, Soo-Jong Hong, Kangmo Ahn
Journal: Journal of Allergy and Clinical Immunology (2016)
MPTH-21MOLECULAR CHARACTERISTICS AND CLINICAL OUTCOME OF GLIOMA PATIENTS.: EXPERIENCE OF KANSAI NETWORK FOR MOLECULAR DIAGNOSIS OF CENTRAL NERVOUS SYSTEM TUMORS
Authors: Kanji Mori, Yonehiro Kanemura, Yosiko Okita, Yuzo Terakawa, Naohiro Tsuyuguchi, Junya Fukai, Yusuke Tomogane, Shusuke Moriuchi, Takuyu Taki, Masahiro Nonaka, Kenichi Ishibashi, Manabu Kinoshita, Naoya Hashimoto, Namiko Nishida, Yoshikazu Nakajima
Journal: Neuro-Oncology (2015)

BACKGROUND: Novel molecular aberrations are found and their prognostic or predictive values are reported. We have established a network for genetical analysis for central nervous system tumors in Kansai area, Japan. METHODS: We retrospectively reviewed clinical records of histologically proven 144 glioma patients (grade4: 88, grade3; 30, grade2: 30) treated by institutes accompanied with Kansai network for molecular diagnosis of central nervous system tumors between 2004-2014. Surgical specimen which were obtained at an initial surgery were used for molecular characterization. We investigated methylation status of MGMT promoter by methylation specific realtime PCR. Mutation of IDH1(R132H), IDH2 and TERT promoter were analyzed by Sanger method. Survival proportion was calculated by Kaplan Meyer method. RESULTS: Median time of overall survival (OS) of grade 4 and grade3 gliomas were 77 and 190 weeks, respectively, OS of grade4 and grade3 patients with methylated MGMT promoter was significantly longer than those without methylation status (151 versus 75.6 weeks, p = 0.0027). IDH1 mutation was found in 23/30, 16/30 and 5/88 cases of grade2, grade3 and grade, respectively. Mutation of TERT promoter was detected in 16/30, 13/30 and 40/87 cases of grade2, grade3 and grade, respectively. CONCLUSION: These data bring us important information to decide how to treat glioma patients. And analysis of glioma patients include who do not match for clinical trial may reveal more practical clinical course and aspects.
Evaluation of the neurobehavioral functioning of patients before, during, and after bone marrow transplantation.
Authors: C A Meyers, M Weitzner, K Byrne, A Valentine, R E Champlin, D Przepiorka
Journal: Journal of Clinical Oncology (1994)

To evaluate the cognitive and emotional functioning of patients undergoing bone marrow transplantation (BMT) in the protected environment (PE).Patients were given tests of cognition and mood before their hospitalization in the PE, after 2 weeks, at discharge, and at 8 months post-BMT. Locus of control, degree of social support, previous biotherapy, and on-treatment psychiatric consultation were also analyzed.Before BMT, 20% of patients had mild cognitive dysfunction, and nearly 40% had significant anxiety. Although few patients developed problems with cognition or mood during the study, short-term memory deficits nearly doubled at follow-up compared with baseline. Anxiety decreased significantly during hospitalization and remained low at follow-up. In contrast, depression increased throughout hospitalization, but decreased at follow-up. Pre-BMT emotional status and cognitive functioning were highly related to long-term outcome. Type of BMT, locus of control, and degree of social support were related to psychologic distress and cognitive functioning, both during and after BMT. Patient age was not a predictor of neurobehavioral symptoms during or after BMT.Pretransplant emotional and cognitive functioning are important determinants of long-term outcome and quality of life (QOL) in BMT patients. In addition, a few patients undergoing BMT develop short-term memory difficulties and mood disturbance that may persist. Pretransplant identification of patients at risk for neurobehavioral difficulties may guide early interventions during hospitalization. Posttransplant assessment may then be used to develop rehabilitation programs and other interventions for individuals with persisting complaints.
SAT0431 A Meta-Analysis of the Diagnostic Value of Minor Salivary Gland Biopsy for Primary Sjogren's Syndrome
Authors: Y.H. Lee, G.G. Song
Journal: Annals of the Rheumatic Diseases (2015)

<h3>Background</h3> Detection of inflammation in minor salivary gland biopsy (MSGB) is an important diagnostic method for primary Sjogren9s syndrome (pSS) in both the American-European Consensus Group (AECG) and American College of Rheumatology (ACR) criteria. Thus, the diagnostic accuracy of MSGB has been studied in the context of pSS. However, the published results are controversial. This may be due to the small sample sizes, low statistical power, and/or the presence of clinical heterogeneity. <h3>Objectives</h3> The purpose of this study is to evaluate the diagnostic performance of MSGB for patients with pSS. <h3>Methods</h3> We searched the Medline, Embase, and Cochrane Library databases, and performed a meta-analysis on the diagnostic accuracy of MSGB in pSS patients. <h3>Results</h3> A total of eight studies including 583 pSS and 627 non-pSS patients were available for the meta-analysis. The pooled sensitivity and specificity of MSGB were 75.7% (95% confidence interval [CI], 72.0–79.1) and 90.7% (88.1–92.9), respectively. The positive likelihood ratio, negative likelihood ratio, and diagnostic odds ratio were 9.475 (4.051–22.16), 0.266 (0.208–0.340), and 38.92 (19.12–72.21), respectively. The area under the curve was 0.901 and the Q<sup>*</sup> index was 0.902, indicating a high diagnostic accuracy. Some between-study heterogeneity was found in the meta-analyses. However, there was no evidence of a threshold effect (Spearman correlation coefficient =0.419; p=0.301). Meta-regression showed that the study quality, sample size, study design, and diagnostic criteria were not sources of heterogeneity, and subgroup meta-analyses did not change the overall diagnostic accuracy. <h3>Conclusions</h3> Our meta-analysis of published studies demonstrates that MSGB has a high diagnostic accuracy and plays an important role in the diagnosis of pSS. <h3>Disclosure of Interest</h3> None declared
Validation of a prognostic model for metastatic castrate-resistant prostate cancer (mCRPC) patients receiving abiraterone acetate (AA).
Authors: Praful Kumar Ravi, Joaquin Mateo, Zafeiris Zafeiriou, Amelia Altavilla, Roberta Ferraldeschi, Spyridon Sideris, Emily Grist, Alan D. Smith, Sophia Wong, David Lorente, Diletta Bianchini, Gerhardt Attard, Johann Sebastian De Bono
Journal: Journal of Clinical Oncology (2014)

5013 Background: A prognostic model based on clinical parameters has been derived from the population of the COU-AA-301 Phase 3 trial for mCRPC patients treated with AA after docetaxel. It relies on the presence of six clinical parameters: ECOG-PS=2, presence of liver metastases, time from start of initial LHRH agonist therapy to AA therapy ≤36mths, low albumin, high alkaline phosphatase and high LDH (1 point per parameter) to stratify patients into 3 risk groups. We sought to validate this model in an independent cohort of patients treated with AA post-docetaxel, and to explore its utility in patients treated with AA in the pre-chemotherapy setting. Methods: We reviewed all mCRPC patients who received AA between January 2006 and June 2013 at our centre who either received AA post-docetaxel outside of a clinical trial (group A, validation set) or were treated with AA prior to chemotherapy (group B, exploratory analysis). Clinical data at the time of starting treatment with AA and outcome was collected from electronic patient records. Patients were assigned into ‘good’ (0-1), ‘intermediate’ (2-3), and ‘poor’ (4-6) prognostic groups as per the published model (Chi KN et al, J Clin Oncol 2013;abstr 5013). Differences in median overall survival (mOS) were assessed using the log-rank test. Results: A total of 158 patients were eligible for this analysis (group A: n=94, group B: n=64). Median duration of AA therapy and mOS were 4.7mths and 13.3mths (group A), and 15.3mths and 40.4mths (group B) respectively. When considering the validation set of patients post-docetaxel, mOS was significantly different across the three prognostic groups (good: n=39, mOS=21.8mths; intermediate: n=44, mOS=10.6mths; poor: n=7, mOS=6.8mths; p=0.0001). Analysis of group B confirmed the ability of the model to prognosticate for OS in the pre-chemotherapy setting: (good: n=44, mOS=45.6mths; intermediate or poor: n=20, mOS=34.5mths; p=0.042). Conclusions: These results serve to validate the prognostic model in an independent, unselected population treated with AA post-docetaxel and support clinical implementation of the score. Additionally, the model remains valid in the pre-chemotherapy setting.
Conjugated Estrogens for the Management of Bleeding Associated with Renal Failure
Authors: Manuela Livio, Pier Mannuccio Mannucci, Gianluigi Viganò, Giulio Mingardi, Rossana Lombardi, Giuliano Mecca, Giuseppe Remuzzi
Journal: New England Journal of Medicine (1986)

Bleeding is a major complication of uremia. Both cryoprecipitate and desmopressin effectively shorten the prolonged bleeding time and favorably influence clinical bleeding, but the former carries the risk of transmitting blood-borne infectious diseases, and both cryoprecipitate and desmopressin have a short duration of action. Preliminary evidence has suggested that estrogens may be useful, and we therefore performed a randomized, double-blind, crossover trial comparing the effect of conjugated estrogens with that of placebo on hemorrhagic tendencies and the bleeding time in six patients with uremia who were on maintenance hemodialysis. Five daily infusions of placebo or conjugated estrogens were administered at the beginning of one-month trial periods. Estrogen shortened the bleeding time in all six patients. The effect was detectable six hours after the first infusion, reached its maximum in all patients between days 5 and 7, and lasted for 14 days. By day 16 after the last infusion, the bleeding time had returned to base line in four of the six patients. No side effects were noted during or after estrogen infusion. Estrogens did not influence the circulating level of von Willebrand factor or change its multimeric structure. Moreover, the defective platelet aggregation and thromboxane formation observed in the patients were not corrected by estrogens. We conclude that conjugated estrogens are an adequate alternative to cryoprecipitate or desmopressin for the treatment of bleeding associated with renal failure, especially when a longer duration of action is needed and immediate onset of the effect is not essential. The mechanism of action of estrogens remains to be clarified.
Changes in a Transplanted Fibrosarcoma Associated with Ascorbic Acid Deficiency
Authors: W V B ROBERTSON, A J DALTON, W E HESTON
Journal: JNCI Journal of the National Cancer Institute (1949)

Changes in a Transplanted Fibrosarcoma Associated with Ascorbic Acid Deficiency Get access William Van B. Robertson, William Van B. Robertson National Cancer Institute, National Institutes of Health, Public Health Service, Bethesda, Md. Search for other works by this author on: Oxford Academic PubMed Google Scholar A. J. Dalton, A. J. Dalton National Cancer Institute, National Institutes of Health, Public Health Service, Bethesda, Md. Search for other works by this author on: Oxford Academic PubMed Google Scholar W. E. Heston W. E. Heston National Cancer Institute, National Institutes of Health, Public Health Service, Bethesda, Md. Search for other works by this author on: Oxford Academic PubMed Google Scholar JNCI: Journal of the National Cancer Institute, Volume 10, Issue 1, August 1949, Pages 53–60, https://doi.org/10.1093/jnci/10.1.53 Published: 01 August 1949 Article history Received: 08 December 1948 Published: 01 August 1949
Snoring and Sleep-Related Breathing Abnormality during Partial Sleep Deprivation
Authors: Riccardo A. Stoohs, William C. Dement
Journal: New England Journal of Medicine (1993)

To the Editor: Snoring may decrease daytime alertness in the absence of repetitive or sustained hypoxia1. Acute total sleep deprivation can worsen sleep-disordered breathing in patients with obstructive sleep apnea syndrome2. No data are available on the influence of prolonged partial sleep deprivation on snoring and sleep-disordered breathing. Prolonged partial sleep deprivation is a common feature of many occupations, particularly shift-work (around the clock) operations. We subjected six persons (two normal controls, two subjects with mild obstructive sleep apnea, and two subjects with moderate obstructive sleep apnea) to the same protocol of prolonged partial sleep deprivation over a . . .
EPID-24IMAGING PATTERNS AND OUTCOME OF POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME DURING CHILDHOOD CANCER TREATMENT
Authors: Zsila Sadighi, Raja Khan, Jennifer Zabrowski, Amar Gajjar, Sima Jeha
Journal: Neuro-Oncology (2015)

INTRODUCTION: We present the long term clinical and radiologic outcomes of posterior reversible encephalopathy syndrome (PRES) in children with cancer. METHODS: We performed an IRB approved retrospective review from 1994-2014 of children with cancer diagnosis less than 18 years old with presentation of mental status change, visual impairment, headache, or seizure with concurrent T2 hyperintensity in subcortical regions. RESULTS: We identified PRES in 37 patients (21 males and 16 females). Median age at cancer diagnosis was 9 years (1 - 17.5 years) and median time from cancer diagnosis to PRES was 6.6 months (0 - 101.4 months) in leukemia (26), brain tumor (5), and solid tumor (7) patients. Clinical symptoms at onset were seizures (97%), hypertension (97%), headaches (42%), altered mental status (69%), and visual impairment (27%). Patients ranged from single seizure (43%), 2 seizures (14%), 3 or more seizures (41%), or status epilepticus (14%) at onset. 17% had epilepsy requiring long-term anti-epileptic medication. Headaches, visual disturbances, and mental status resolved in all within median 2.5 days, 4 days, and 2 days respectively. Initial MRI within 96 hours showed T2 signal hyperintensity in occipital (100%), temporal (47%), parietal (75%), and frontal (55%) lobes; cerebellum (22%), and basal ganglia (5%). Follow-up MRI in 34 patients (median 29 days) and third MRI in 22 patients (median 20.5 months) showed improvement in most areas, except laminar necrosis (5 at 2nd MRI, 1 at 3rd MRI), microhemorrhage (6 at 2nd MRI, 3 at 3rd MRI), and focal atrophy (3 at 2nd and 3rd MRI). 57% patients were alive at a median follow-up of 27.5 months. CONCLUSION: PRES may be recognized by presence of seizures, hypertension> altered mental status> headaches with T2 signal hyperintensities in subcortical regions occipital > parietal > frontal > temporal lobes. Most MRI changes resolve, but brain injury and epilepsy requiring anti-seizure medications may persist.