tbuckley/cabot-search huggingface dataset:
Abstract 12090: Modern Treatments Can Abolish Mortality in Pediatric Patients With Hypertrophic Cardiomyopathy
Authors: Aadhavi Sridharan, Barry J Maron, Christopher Madias, Hassan Rastegar, Martin S Maron, Ethan J Rowin
Journal: Circulation (2019)

Background: Despite recognition that hypertrophic cardiomyopathy (HCM) can be compatible with extended longevity in the modern treatment era, the perception remains that children with HCM remain at...
Clinical Cancer Advances 2021: ASCO's Report on Progress Against Cancer
Authors: Sonali M. Smith, Kerri Wachter, Howard A. Burris, Richard L. Schilsky, Daniel J. George, Douglas E. Peterson, Melissa L. Johnson, Merry Jennifer Markham, Kathryn F. Mileham, Muhammad S. Beg, Johanna C. Bendell, Robert Dreicer, Vicki L. Keedy, Randall J. Kimple, Miriam A. Knoll, Noelle LoConte, Helen MacKay, Jane Lowe Meisel, Timothy J. Moynihan, Daniel A. Mulrooney, Therese Marie Mulvey, Olatoyosi Odenike, Nathan A. Pennell, Katherine Reeder-Hayes, Cardinale Smith, Ryan J. Sullivan, Robert Uzzo
Journal: Journal of Clinical Oncology (2021)

First Page
mtor Haploinsufficiency Ameliorates Renal Cysts and Cilia Abnormality in Adult Zebrafish tmem67 Mutants
Authors: Ping Zhu, Qi Qiu, Peter C. Harris, Xiaolei Xu, Xueying Lin
Journal: Journal of the American Society of Nephrology (2021)

Significance Statement Zebrafish embryos are well recognized for their value in studying polycystic kidney disease, but they cannot fully recapitulate the pathogenesis of the disease. The establishment of tmem67 e3/e3 mutants provides the first adult zebrafish model for cystogenesis. The model exhibits progressive renal cysts and enables the discernment of compensational effects from pathogenic events. Development of a tissue-clearing, whole-mount imaging protocol allows the analysis of renal cysts and cilia abnormality at a single-nephron resolution. Evidence also indicates potential benefit of mammalian target of rapamycin inhibition in tmem67 -based cystogenesis. Combining powerful genetics and phenotyping tools, this novel adult zebrafish model shall facilitate mechanistic studies and therapeutic development for cystic diseases. Background Although zebrafish embryos have been used to study ciliogenesis and model polycystic kidney disease (PKD), adult zebrafish remain unexplored. Methods Transcription activator-like effector nucleases (TALEN) technology was used to generate mutant for tmem67 , the homolog of the mammalian causative gene for Meckel syndrome type 3 (MKS3). Classic 2D and optical-clearing 3D imaging of an isolated adult zebrafish kidney were used to examine cystic and ciliary phenotypes. A hypomorphic mtor strain or rapamycin was used to inhibit mTOR activity. Results Adult tmem67 zebrafish developed progressive mesonephric cysts that share conserved features of mammalian cystogenesis, including a switch of cyst origin with age and an increase in proliferation of cyst-lining epithelial cells. The mutants had shorter and fewer distal single cilia and greater numbers of multiciliated cells (MCCs). Absence of a single cilium preceded cystogenesis, and expansion of MCCs occurred after pronephric cyst formation and was inversely correlated with the severity of renal cysts in young adult zebrafish, suggesting a primary defect and an adaptive action, respectively. Finally, the mutants exhibited hyperactive mTOR signaling. mTOR inhibition ameliorated renal cysts in both the embryonic and adult zebrafish models; however, it only rescued ciliary abnormalities in the adult mutants. Conclusions Adult zebrafish tmem67 mutants offer a new vertebrate model for renal cystic diseases, in which cilia morphology can be analyzed at a single-nephron resolution and mTOR inhibition proves to be a candidate therapeutic strategy.
Abstract 16940: Missed Opportunities for Screening and Management of Dysglycemia After Myocardial Infarction: The Prospective NORIN STEMI Registry
Authors: Muthiah Vaduganathan, Girish MP, Puneet Gupta, Michael Hendrickson, Arman Qamar, Sameer Arora, Ambarish Pandey, ANKIT BANSAL, Vishal Batra, Bhawna Mahajan, Safal Safal, Sanjay Tyagi, Deepak L Bhatt, mohit gupta
Journal: Circulation (2020)

Background: Dysglycemia is a major and increasingly prevalent cardiometabolic risk factor worldwide, but often is undiagnosed even among high-risk patients with cardiovascular disease. New risk reduction strategies for DM are available, but little is known about their uptake in low middle-income countries. Methods: We conducted a prospective NORIN STEMI registry-based study of patients presenting with STEMI to 2 government-funded tertiary care medical centers in New Delhi, India from January to November 2019. Hemoglobin A1c was collected as a part of the study protocol, irrespective of baseline glycemic status. Prediabetes and DM were defined as screening HbA1c 5.7-6.4% and ≥6.5%, respectively. Results: Among 3,635 patients with STEMI (median age 55[45-62] with 33% ≤50 years, 16% women, 54% BMI≥30kg/m 2 ), 855 (24%) reported a prior history of DM. Among patients with known DM, median HbA1c (available in n=826) was 7.4% (6.4-8.2%). At presentation, 1 patient (0.1%) and 21 patients (2%) were treated with an SGLT2i and GLP-1RA, respectively. Among patients without known DM, median HbA1c (available in n=2,668) was 5.5% (5.1%-5.9%) with 737 (28%) newly identified to have prediabetes and 339 (13%) to have DM. Compared with patients with euglycemia (42%), patients with known or newly detected prediabetes (49%) and DM (49%) experienced higher rates of post-MI LV dysfunction EF≤40% (P&lt;0.0001). In-hospital mortality (4.3%, 5.6%, 8.8%) and 30-day mortality (6.6%, 8.1%, 11.7%) were similarly higher among patients with dysglycemia (P&lt;0.0001 for both). Conclusion: Among patients presenting with STEMI in North India, protocolized HbA1c screening doubled the proportion of patients with known dysglycemia. These patients faced higher rates of LV dysfunction and mortality out to 30 days. Treatment with SGLT2i or GLP-1RA was infrequent.
Outcomes of SARS-CoV2 infection in STAT3 and PGM3 Deficiency
Authors: Muhammad Khalid, Amanda Urban, Dirk Darnell, Alexandra Freeman
Journal: Journal of Allergy and Clinical Immunology (2021)

While SARS-CoV2 continues to spread globally, our understanding of the disease continues to be limited in patients with underlying immune dysregulation. Four subjects with known monogenic immunodeficiency with HyperIgE phenotype and confirmed or clinically suspected SARS-CoV2 infection were tele-interviewed to assess for symptoms, duration, severity and complications of illness. Three patients had STAT3 deficient HyperIgE syndrome and one had PGM3 deficiency. All were on antimicrobial prophylaxis during SARS-CoV2 infection. 8-year-old male with PGM3 deficiency related lymphopenia, neutropenia, and reactive airway disease tested positive for SARS-CoV2 and experienced 2 days of low-grade fever and cough. 26-year-old male with STAT3 deficiency working in a high-exposure environment tested positive and experienced symptoms for 2 weeks including headache, myalgias, low-grade fever and dyspnea on exertion. He received additional antibiotics around day 7 of illness when his course worsened briefly. 45-year-old male with STAT3 deficiency, with exposure to confirmed positive family member, developed intermittent low-grade fever, chills, myalgias, severe fatigue, and dyspnea on exertion, lasting for 20 days. His 14-year-old daughter with STAT3 deficiency experienced 14 days of fatigue, low-grade fever, anosmia and ageusia. All 4 patients were managed at home without requiring supplemental oxygen, hospitalization or residual complications. Despite concern for worse outcomes for patients with immunodeficiency including HyperIgE syndromes, our patients exhibited favorable outcomes with relatively mild COVID-19 clinical course. This may underscore the absence of excessive immune response to SARS-CoV2 in this patient population; however, number of patients was limited, and more research is required to understand significance of these underlying diseases.
Differential Contribution Of TNFSF14/LIGHT-Receptor Mediated Pathways To Inflammatory Fibroblast Phenotypes In Eosinophilic Esophagitis
Authors: Mario Cabrero Manresa, Elaine Pham, Loan Duong, Haruka Miki, Michael Croft, Seema Aceves
Journal: Journal of Allergy and Clinical Immunology (2021)
High-fiber diet mitigates maternal obesity-induced cognitive and social dysfunction in the offspring via gut-brain axis
Authors: Xiaoning Liu, Xiang Li, Bing Xia, Xin Jin, Qianhui Zou, Zhenhua Zeng, Weiyang Zhao, Shikai Yan, Ling Li, Shufen Yuan, Shancen Zhao, Xiaoshuang Dai, Fei Yin, Enrique Cadenas, Rui Hai Liu, Beita Zhao, Min Hou, Zhigang Liu, Xuebo Liu
Journal: Cell Metabolism (2021)
Phase 1b Study of Sintilimab Plus Anlotinib as First-line Therapy in Patients With Advanced NSCLC
Authors: Tianqing Chu, Runbo Zhong, Hua Zhong, Bo Zhang, Wei Zhang, Chunlei Shi, Jialin Qian, Yanwei Zhang, Qing Chang, Xueyan Zhang, Yu Dong, Jiajun Teng, Zhiqiang Gao, Huiping Qiang, Wei Nie, Yiming Zhao, Yuchen Han, Ya Chen, Baohui Han
Journal: Journal of Thoracic Oncology (2021)
Association between Clinical Frailty Scale score and hospital mortality in adult patients with COVID-19 (COMET): an international, multicentre, retrospective, observational cohort study
Authors: Roos S G Sablerolles, Melvin Lafeber, Janneke A L van Kempen, Bob P A van de Loo, Eric Boersma, Wim J R Rietdijk, Harmke A Polinder-Bos, Simon P Mooijaart, Hugo van der Kuy, Jorie Versmissen, Miriam C Faes, LA Agnoletto, J Aleman, S Andreassi, LM Andrews, L Ashfield, H Bell, AKB Bengaard, SB Berlinghini, KB Bini, ZB Bisoffi, KB Blum, E Boemaars, GB Boni, TM Bosch, BE Bosma, F Boutkourt, C Bufarini, A Bulsink, RC Cabuk, GC Callens, MC Candela, MC Canonici, EC Capone, IC Carmo, FC Caruso, PC Chessa, GC Cohet, I Cornelissen-Wesseling, KML Crommentuijn, FM de Stoppelaar, HAJM de Wit, DS Deben, LJJ Derijks, MDC Di Carlo, J Diepstraten, B Dilek, DMK Duchek-Mann, MM Ebbens, LJ Ellerbroek, M Ezinga, MF Falcao, FF Falcao, LF Fantini, HF Farinha, PMG Filius, NJ Fitzhugh, G Fleming, TF Forsthuber, GG Gambarelli, MG Gambera, CGY García Yubero, Z Getrouw, CN Ghazarian, N Goodfellow, MQG Gorgas, RG Grinta, K Guda, DH Haider, J Hanley, KH Heitzeneder, WL Hemminga, LC Hendriksen, DL Hilarius, FEF Hogenhuis, IC Hoogendoorn-de Graaf, MBH Houlind, MAH Huebler, KPGM Hurkens, PKC Janssen, E Jong, MHW Kappers, KFM Keijzers, MK Kemogni, EM Kemper, RA Kranenburg, LL Krens, JL G Le Grand, J Liang, S Lim, NL Lindner, EL Loche, AL Lubich, B Maat, CM Maesano, AM Maiworm, M Maragna, FM Marchesini, IM Martignoni, G M Martini, CM Masini, R Mc Menamin, DM Mendes, M Miarons, R Moorlag, MR Müller, FN Nagele, KN Nemec, GO Oka, AG Otten-Helmers, SP Pagliarino, FP Pappalardo, M Patel, PM Peverini, FP Pieraccini, EMP Platania, NPK Pons-Kerjean, LPH Portillo Horcajada, GR Rametta, JR Rijo, EE Roelofsen, E Roobol-Meuwese, LR Rossi, SAH Russel, Z Safipour, FS Salaffi, L Saleh, AMS Schimizzi, JMGA Schols, MS Schwap, MG Scott, EAM Slijfer, EMA Slob, JS Soares, MS Solano, F Sombogaard, GS Stemer, MT Tardella, PGJ ter Horst, RT Tessari, J Tournoy, RB van den Berg, L Van der Linden, PD van der Linden, SC van Dijk, RW Van Etten, IMM van Haelst, M van Heuckelum, HJM van Kan, C van Nieuwkoop, HAW van Onzenoort, P van Wijngaarden, JDJ Verdonk, Fv Verri, JAMC Verstijnen, MV Veyrier, EV Viegas, LE Visser, A Vos, MAM Vromen, PC Wierenga, DR Wong, CZ Zenico, TZ Zuppini
Journal: The Lancet Healthy Longevity (2021)
Abstract IA011: Coordinated T and B cell responses exert immune pressure against malignant progression
Authors: Jose R. Conejo-Garcia
Journal: Clinical Cancer Research (2021)

Abstract Memory B-cell and plasma cell infiltrates have been associated with better outcome in ovarian cancer, but the nature and functional relevance of these responses in other gynecologic malignancies remains elusive. Using a cohort totaling 107 endometrial cancers of different histological subtypes we show that the density of T cell infiltrates is associated with improved outcome in low-grade endometrial cancer, while better survival is associated with increased B cell and plasma cell infiltrates in high-grade endometrioid tumors. IgA and IgG are similarly abundant in endometrial tumors, but play opposite roles, whereby IgA:tumor cell co-localization is associated with accelerated malignant progression in clear cell and high-grade endometrioid tumors, while binding of IgG is associated with improved outcome in the same histological subtypes. Our data indicate that T cell and B cell responses work in coordination against endometrial cancer, but their role in tumor progression depends on the flavor of these responses and the histological subtype of endometrial cancer. Citation Format: Jose R. Conejo-Garcia. Coordinated T and B cell responses exert immune pressure against malignant progression [abstract]. In: Proceedings of the AACR Virtual Special Conference: Endometrial Cancer: New Biology Driving Research and Treatment; 2020 Nov 9-10. Philadelphia (PA): AACR; Clin Cancer Res 2021;27(3_Suppl):Abstract nr IA011.
Newborn Dried Blood Spot Testing for Congenital Cytomegalovirus Screening
Authors: Gail J. Demmler-Harrison
Journal: JAMA Pediatrics (2021)

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA Pediatrics HomeNew OnlineCurrent IssueFor Authors Podcast Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA Pediatrics journal
Omenn Syndrome with Autoreactive T-cell development after viral infections in two patients with Leaky Severe Combined Immunodeficiency (SCID)
Authors: Maria Chitty-Lopez, Jessica Trotter, Carla Duff, Gretchen Vaughn, Benjamin Oshrine, Gauri Sunkersett, Jennifer Leiding
Journal: Journal of Allergy and Clinical Immunology (2021)
Early Steroid Cessation After Kidney Transplant
Authors: Arthur J. Matas
Journal: JAMA Surgery (2021)

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA Surgery HomeNew OnlineCurrent IssueFor Authors Podcast Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA Surgery journal
Aromatase x Her-2/neu double transgenic mice model: Importance of local estrogen and Her-2 in breast cancers
Authors: Ya-Guang Liu, Hareesh B. Nair, Rao P. Perla, Nameer B. Kirma, Rajeshwar Rao Tekmal
Journal: Cancer Research (2005)

5250  A majority of breast cancers are hormone-responsive and will require estrogen for growth, and respond to hormonal therapy that blocks estrogen receptor action. Breast tumors lacking estrogen receptor fail to respond to antiestrogen therapy yet require estrogen for tumor initiation. To address directly the importance of breast tissue aromatase in initiation and progression of breast cancer, we have generated aromatase transgenic mice (MMTV-aromatase) and shown that aromatase overexpression resulted in the induction of hyperplastic and dysplastic lesions and they are persistent even without circulating estrogens. Aromatase transgenic mice form ER-positive mammary tumors when exposed to DMBA within four to five months. To our knowledge, this is the first in vivo model that clearly demonstrated the direct involvement of breast tissue aromatase/estrogen in tumorigenesis. An ideal hormone-responsive breast cancer model should give rise to both ER positive and negative tumors. However, there is no such model. To address this issue we have crossed MMTV-aromatase transgenic mice that form estrogen-dependent ER-positive tumors with MMTV-neu which forms ER-negative tumors in response to estrogen. Double transgenic mice show increased proliferation even at very young age (twelve weeks) and increased TGF beta (5 fold), EGFR (2 fold), cyclin D1 (∼ 10 fold), and estrogen receptor alpha levels as well as other biochemical differences. Ongoing studies are focused on determining tumor incidence, estrogen receptor status of all tumors and potential use of chemoprevention approaches with letrozole or EGFR tyrosine kinase inhibitor (AEE788) alone or combined. (Supported by NIH/NCI grant CA75018)
Progression of Cabozantinib-Related Osteonecrosis Mimicking Metastases on Bone Scan
Authors: Guofan Xu, Yang Lu
Journal: Clinical Nuclear Medicine (2021)

Patient was a 73-year-old woman with metastatic renal cell carcinoma. Bone scan showed multifocal bone metastases. The patient received cabozantinib treatment for more than 1 year. On the follow-up bone scan, the previously biopsy-proven left pelvic bone lesion has improved, whereas the right maxillary lesion showed increased extent and intensity of abnormal radiotracer uptake. Given the different change pattern of these lesions, the right maxillary lesion was further evaluated. Biopsy results confirmed devitalized bone with bacterial colonies overgrowth and without tumor cell, suggestive of medication-related osteonecrosis.
Abstract PO011: Spatial immune cell and transcriptome profiling uncover differential cellular and molecular signatures in primary and metastatic tumor sites in patients with advanced uterine serous cancer
Authors: Li Zhang, Angela Rynne-Vidal, Karen H. Lu, Samuel C. Mok
Journal: Clinical Cancer Research (2021)

Abstract Uterine serous carcinoma (USC) is the most aggressive and lethal subtype of endometrial cancer with a low overall patient survival rate of 18%-27%. Extra-uterine metastasis is frequently found in lymph nodes and omentum. The high lethality and poor understanding of the underneath mechanisms that drives USC progression lead to an urgency to develop novel therapeutic tools for the treatment of USC patients. Metastasis is proven to be the leading cause of death from USC and the immune system plays a vital role for this process. However, differential cellular and molecular profiles in the primary and metastatic tumor microenvironment have not been thoroughly examined. To identify the immune cell profile of USC, a panel of 7 immune cell markers together with functional markers granzyme B and Ki-67 were used to examine the spatial localization of a variety of tumor-infiltrating immune cells by multiplex immunofluorescence IHC assay using OpalTM immunology detection kit (Perkin Elmer). Formalin fixed paraffin embedded (FFPE) tissue sections from both primary and metastasis sites (lymph node metastasis) of 10 USC patients were used. Vetra 3.0 microscope was used to take photos of stained slides. Images were quantified and analyzed by inForm software. Transcriptome profiling of microdissected tumor cells from the same tissue samples was performed using the human Clariom D assay platform. Our results demonstrated that primary USC sites had significantly higher density of intratumoral activated tumor-infiltrating cytotoxic T cells (CD8+granzyme B+), and CD8 and LCK double positive T cell subpopulation (CD8+LCK+) than the metastatic sites in lymph nodes. In addition, we found that the density and spatial distribution of activated CD8+ T cells correlate with the overall survival of USC patients. Furthermore, differentially expressed genes and signaling networks associated immune cell activation and tumor cell metastasis were identified in primary sites compared with those in the metastatic sites. Taken together, our study suggested that the immune cell and molecular phenotypes in the lymph nodes provide a permissive niche that support USC progression, which may lead to poor survival rates in patients with advanced USC. Targeting these altered signaling networks may provide novel therapeutic strategies in the treatment of USC patients. Citation Format: Li Zhang, Angela Rynne-Vidal, Karen H. Lu, Samuel C. Mok. Spatial immune cell and transcriptome profiling uncover differential cellular and molecular signatures in primary and metastatic tumor sites in patients with advanced uterine serous cancer [abstract]. In: Proceedings of the AACR Virtual Special Conference: Endometrial Cancer: New Biology Driving Research and Treatment; 2020 Nov 9-10. Philadelphia (PA): AACR; Clin Cancer Res 2021;27(3_Suppl):Abstract nr PO011.
Differences in Diet Quality Among Food-Allergic Black and White Children
Authors: Eileen Vincent, Jialing Jiang, Jamie Fierstein, Alexandria Bozen, Isabel Galic, Pamela Newmark, Christopher Warren, Lucy Bilaver, Jacqueline Pongracic, Annika Chura, Amal Assa'ad, Aame Andy-Nweye, Susan Fox, Mahboobeh Mahdavinia, Mary Tobin, Adam Robinson, Amaziah Coleman, Hemant Sharma, Andrea Pappalardo, Ruchi Gupta
Journal: Journal of Allergy and Clinical Immunology (2021)
Essential Thrombocythemia Versus Pre-Fibrotic/Early Primary Myelofibrosis: Discrimination By Laboratory and Clinical Data
Authors: Martin Schalling, Andreas Gleiss, Bettina Gisslinger, Albert Wölfler, Veronika Buxhofer-Ausch, Georg Jeryczynski, Maria-Theresa Krauth, Ingrid Simonitsch-Klupp, Christine Beham-Schmid, Jurgen Thiele, Heinz Gisslinger
Journal: Blood (2017)