tbuckley/cabot-search huggingface dataset:
Antimicrobial Prophylaxis
Authors: Eugene Friedman
Journal: JAMA (1978)

<h3>To the Editor.—</h3> I believe a comment is necessary with respect to the letter from Chinh T. Le, MD, on antimicrobial prophylaxis (238:852, 1977). Dr Le's remarks re "detrimental omissions or inadequacies" as they relate to dentists could be misinterpreted to indicate a deficiency on the part of the dental profession. Despite reference to a random phone survey, it must be maintained that the dental profession has been keenly aware both educationally and clinically of the necessity for antimicrobial prophylaxis for many years, and some of the more important research papers on transient bacteremia are from the dental literature. The American Heart Association can attest to this by their continuous dialogue and well-received efforts to provide dental practitioners with current standards. What Dr Le is probably referring to is the result of the deference that dentists normally exhibit to the patient's personal physician who has failed to elaborate guidelines to
Zidovudine. Does it work for everyone?
Authors: M. D. Smith
Journal: JAMA (1991)

Over 85 000 (45%) of reported acquired immunodeficiency syndrome (AIDS) cases in the United States have occurred among racial and ethnic minorities including blacks, Hispanics, Asian-Pacific islanders, and native American or Alaskan native people.<sup>1</sup>This number, of course, represents only those reported with the later stages of the human immunodeficiency virus (HIV) infection; studies in a variety of populations have also demonstrated rates of asymptomatic HIV infection substantially higher in people of color than in whites. Women also represent a growing number of those with AIDS—9% of adult and adolescent cases diagnosed before August 1990 and 12% in the last year. But the representation of minorities and women among AIDS cases stands in stark contrast to their underrepresentation in the clinical trials of drugs directed against HIV. Most clinical trials have been overwhelmingly composed of gay white men; minorities, women, and injecting drug
JHEP at a glance (May 2018)
Authors: Christophe Hezode, Nancy Reau, Evguenia Svarovskaia, Jacqueline O'leary, Stephen Shafran, Stefan Zeuzem, Jonas Söderholm, Charlotta Millbourn, Katharina Büsch, Robert Schvarcz, Karin Lindahl, Annette Bruchfeld, Li Li, Vivian Barry, Stephane Daffis, Robert Lanford, William Delaney, Simon Fletcher, Nadine Gehrke, Nadine Hövelmeyer, Ari Waisman, Marcus Wörns, Peter Galle, Jörn Schattenberg
Journal: Journal of Hepatology (2018)
Sudden death among Southeast Asian refugees. An unexplained nocturnal phenomenon
Authors: R. C. Baron
Journal: JAMA (1983)

In the period July 15, 1977, through March 30, 1982, there were at least 51 sudden, unexplained deaths in the United States among refugees from Southeast Asia. These deaths involved relatively young (median age, 33 years), previously healthy persons. All except one were male, and all died at night. Available data from studies among young adults in this country suggest that this specific phenomenon has an unusually high incidence among Laotian and Kampuchean refugees and, furthermore, has not previously been observed in the United States. Interviews with families of the decedents and a case-control study have failed to establish causal factors, but emotional stress cannot be ruled out as a contributing element. While reviews of the forensic investigations have confirmed the absence of important common pathological or toxicological findings, preliminary findings of special postmortem cardiac studies, now in progress, suggest that at least some of these deaths may be associated with developmentally abnormal conduction system pathways. Further studies are required to confirm this association, to define the apparently sleep-induced mechanism that triggers these deaths, and to explain the male preponderance in this disorder. (<i>JAMA</i>1983;250:2947-2951)
Error in Affiliation
Journal: JAMA Psychiatry (2016)

Britta Galling, MD; Alexandra Roldán, MD; René E. Nielsen, MD, PhD; Jimmi Nielsen, MD, PhD; Tobias Gerhard, PhD; Maren Carbon, MD; Brendon Stubbs, PhD; Davy Vancampfort, PhD; Marc De Hert, MD, PhD; Mark Olfson, MD, MPH; Kai G. Kahl, MD; Andres Martin, MD; Jeff J. Guo, MD; Hsien-Yuan Lane, MD, PhD; Fung-Chang Sung, PhD, MPH; Chun-Hui Liao, MD; Celso Arango, MD; Christoph U. Correll, MD
Nonsteroidal anti-inflammatory agents in rheumatoid arthritis and ankylosing spondylitis
Authors: C. Wasner
Journal: JAMA (1981)

The relative effectiveness of six nonsteroidal anti-inflammatory agents was studied in 33 patients with rheumatoid arthritis and 32 patients with ankylosing spondylitis in a double-blind, randomized, prospective study employing a six-way multiple crossover design with six-week trials of each agent. In ankylosing spondylitis, naproxen, indomethacin, and fenoprofen calcium were the most effective agents. In rheumatoid arthritis, relatively little mean difference between drugs was found. Most of this difference could be attributed to compliance factors, which favored drugs that required only a small number of pills daily. Despite the small differences in effect, patients had strong preferences. More than 85% of patients were still taking their preferred medication after a mean follow-up period of one year. (<i>JAMA</i>1981;246:2168-2172)
The 1940 Year Book of Radiology
Journal: JAMA (1941)

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 HomeNew OnlineCurrent IssueFor Authors Podcasts Clinical Reviews Editors' Summary Medical News Author Interviews More 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 journal
Retinoblastoma Obscured by Recent Trauma
Journal: JAMA (1963)

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 HomeNew OnlineCurrent IssueFor Authors 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) Podcasts Clinical Reviews Editors' Summary Medical News Author Interviews More 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 journal
Abstract 4677: Inhibition of 15-PGDH causes Kras-driven tumor expansion through prostaglandin E2-ALDH1 signaling in the pancreas
Authors: Takatsugu Ishimoto, Kota Arima, Tomoyuki Uchihara, Keisuke Miyake, Atsuko Yonemura, Tadahito Yasuda, Rumi Itoyama, Masaaki Iwatsuki, Yoshifumi Baba, Naoya Yoshida, Hideo Baba
Journal: Cancer Research (2019)

Abstract Chronic inflammation has a crucial role in cancer development and the progression of various tumors, including pancreatic ductal adenocarcinoma (PDAC). The arachidonate cascade is a major inflammatory pathway that produces several metabolites, such as prostaglandin E2 (PGE2). 15-hydroxyprostaglandin dehydrogenase (15-PGDH) participates in the degradation of PGE2 and has attracted attention in recent years. Although inhibition of 15-PGDH is reported to lead to PGE2 accumulation and expand tissue stem cell fraction, the role for pancreatic tumor progression is still unknown. The aim of this study is to elucidate the regulatory mechanism and functional role of 15-PGDH for cancer stem-like cell expansion during tumor progression in pancreas. We found that 15-PGDH expression is frequently down-regulated in PDAC cells and significantly correlated with the number of infiltrating macrophages in human PDAC tissues. Moreover, direct co-culture assay revealed that macrophage derived interleukin-1 beta down-regulates 15-PGDH expression in PDAC cells. Furthermore, pharmacological blockade of 15-PGDH led to PGE2 accumulation, and promoted growth and sphere formation through the expansion of ALDH1-positive cells. We also elucidated the molecular mechanism that PGE2 accumulation by 15-PGDH inhibition increases CYP26A1 expression and subsequently depletes all-trans retinoic acid, and results in ALDH1 up-regulation. Finally, genetic ablation of 15-Pgdh promoted tumorigenesis in KrasLSL-G12D; Ptf1aCre/+ mice through the expansion of ALDH1-positive cells. Our findings highlight the role and significance of PGE2 degradation pathway for PDAC tumor progression. Note: This abstract was not presented at the meeting. Citation Format: Takatsugu Ishimoto, Kota Arima, Tomoyuki Uchihara, Keisuke Miyake, Atsuko Yonemura, Tadahito Yasuda, Rumi Itoyama, Masaaki Iwatsuki, Yoshifumi Baba, Naoya Yoshida, Hideo Baba. Inhibition of 15-PGDH causes Kras-driven tumor expansion through prostaglandin E2-ALDH1 signaling in the pancreas [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2019; 2019 Mar 29-Apr 3; Atlanta, GA. Philadelphia (PA): AACR; Cancer Res 2019;79(13 Suppl):Abstract nr 4677.
Abstract 18085: Heparan Sulfate Proteoglycans Only Modestly Affect Postprandial Hepatic Remnant Clearance in Humans
Authors: Geesje M Dallinga-Thie, Han Levels, Alinda M Schimmel, Max Nieuwdorp
Journal: Circulation (2015)

Introduction and Methods: Elevated circulating levels of Triglyceride-rich Remnant Lipoproteins (TLR) are strongly associated with increased risk for CVD. The hepatic clearance of TRL involves lipoprotein receptors i.e. the low-density lipoprotein receptor (LDLr) and heparin sulfate proteoglycans (HSPG). The relevance of each pathway in humans remains to be established. To further dissect the relative contribution of each of these receptors, we studied postprandial TRL metabolism with an oral fat tolerance test using cream supplemented with retinyl palmitate (RP) in 1) patients with a heterozygous loss-of-function (LOF) variant in LDLR stratified for a low (n=10) or high (n=10) HSPG gene score; 2) patients with heterozygous LOF variants in EXT1 or EXT2 (n=13), characterised by decreased HSPG chains length but normal sulfation pattern, and compared to matched healthy controls (n=13) and 3) diabetic patients (n=29) stratified for a functional SNP in SULF2, that predisposes to lower SULF2 expression and increased 6-O-sulfation of HSPG chains. Results: Postprandial TRL clearance was significantly delayed in patients with FH compared to controls (AUC-RP FH: 1971±190 vs Con: 646±110 nmol/l/h;P&lt;0.0001 and iAUC-TG FH 6.9±1.0 vs Con 3.8±10 mmol/l/h, P&lt;0.05) supporting the important role of LDLr in TRL clearance. No additional effect was observed if the FH group was stratified for HSPG gene score. Also, in patients with LOF variants in EXT, resulting in shorter HSPG chains, no difference in TRL clearance versus controls could be observed. In contrast, improved 6-O-sulfation due to lower hepatic protein expression of SULF2 resulted in improved fasting and postprandial TG levels and significantly lower iAUC-RP (iAUC-TG AA 6.9±1.1 vs GG 4.1±1.2 mmol/l/h P&lt;0.05; AUC-RP AA 97±15 vs GG 15±2 mg/l/h; P&lt;0.001) Conclusion: Our findings clearly indicate an important role for the LDLr in postprandial TRL clearance in humans. In contrast to murine studies, HSPGs do only modestly contribute to hepatic TRL clearance in humans, and implicate that sulfation of HSPG’s is of more relevance for TRL clearance than HSPG chain length.
JAMA Internal Medicine
Authors: Phil Fontanarosa, Annette Flanagin, Paula Glitman, Lauren Fischer, John Mcfadden, Donald Smith, Karen Boyd, Rochelle Lodder, Diane Cannon, Connie Manno, Fred Marshall, Laura Copyreaders, Catherine Adamcyzk, Jan Alden, Mary Clavey, Gillian Coerver-Connolly, Laura Dickens, Juliann King, Donna Tarsney, Caroline Thordsen, Wanda Assistant, Larry Bryant, Monica Mungle, Elaine Managers, Staff Gardner- Gullens, Fanny Brown, Paul Frank, Rachell Lozano, Michaela Mark, Kathryn Muskovich, J Neff, Kate Pezalla, Gale Saulsberry, Michael Berkwits, Michelle Kurzynski, Eric Butkus, Christopher Hastings, Craig Mccaffrey, Reuben Rios, Ronna Henry, Staff Bird, Alison Burke, Cassio Lynm, David Song, Deputy Livingston, Thomas Cole, Marla Hall, Deanna Willis, Yolanda Davis, Joseph Thornton, Deanna Bellandi, Jim Periodical, Thomas Staff, Monica Smith, Jeffery Bonistalli Manager, Kathy Staff, Mirna Monal, Patricia Quilty, Thalia Moss, Tem Harrier, Susan Henning, Mark Thornbury, Tricia Castellano, Saskia Bolore, Gretchen Linder, Brian Mccartney, Rebecca Haas, Christine Hearne, Irma Lara, Audra Lawlor, Scott Marketing, Matt Herron, Elizabeth Solaro, Geoffrey Flick, Paul Gee Managers, Rick Bell, Michael Mcgraw, Sara Nagy, Stacy Staff, Millette Jackson-Bates, Karen Adams-Taylor, Karl Elvin, Sue Sherrill, Brian Shields Managers, David Antos, Chris Borden, Debbie Camp, Lydia Cruz, Diane Darnell, Colleen Kelly, Chris Meyer, Jaye Mize, Annemarie Neff, Sean O'donnell, Sean Ohlson, Phil O'leary, Ryan Patella, Susan Price, Denise Steinhauser, Production Services, Kim Boler, Rhonda Brown, Timothy Burke, Daniel Degroot, Jordynn Farrar, Euwanaha Joiner-Smith, Cindy Rendlen, Ellen Yoshihara, Circulation Services, Julie Burton, Dexter Golden, Erin Hinchcliff, Noemi Rivera, Geneine Van, Someren Composition, New Media, Gail Barrett, Regina Brownlee, Brenda Chandler-Grayer, Scott Curl, Amy Evers, Teri Hutchison, Mary Kuranda, Joshua Lampinen, Patricia Panek, La Son, Diggs Sledge, George Thomson Graphics, Joe Amft, Maria Duda, Fred Furtner, Carolyn Hall, Maria Kowalkowski, Proofreading Carol, Joy Farrell, Daniel James, Judith Literskis, Teresa Omiotek, James Madara, Bernard Hengesbaugh, Jeffrey Tabas, San Francisco, Paul Varosy, Gregory Marcus, Nora Goldschlager, California Cme, Rebecca Larson, California Less, Is More, Deborah Grady, Mitchell Katz, Los Angeles, Patrick California, David Glidden, John Neuhaus, Kenneth Covinsky, Joseph Ross, New Haven, Raman Khanna, Kathleen Mckibbin, Katherine Gilhooley, Mary Kohut, Howard Bauchner, Rita Redberg, Rosemary Gibson, Shannon Brownlee, Robert Steinbrook, Judyann Bigby, Ezekiel Emanuel, Mark Feinglos, Chi-Yuan Hsu, Kurt Kroenke, Grace Lin, Mary Mcdermott, C Noel, Bairey Merz, Samia California, Robert Phillips, Michael Pignone, Chapel Hill, North Carolina, Judith Prochaska, Bruce Psaty, Kathryn Rexrode, Mary Tinetti, Nicolas Rodondi, Iona Heath
Journal: JAMA Internal Medicine (2013)
BOOK REVIEWS
Authors: M. ROPER
Journal: JNCI Journal of the National Cancer Institute (1988)

Journal Article BOOK REVIEWS Get access 1987 Yearbook of Cancer. Robert C. Hickey, Grady F. Saunders, Randolph Lee Clark, Russell W. Cumley, Diane S. Rivera, eds. Chicago, IL: Yearbook Medical Publishers, Inc., 1987, 605 pp. $49.95 (Practitioner), $29.95 (Resident). MARYANN ROPER MARYANN ROPER Office of the Director, National Cancer InstituteBulding 31, Room 11A19, Bethesdu, MD 20892 Search for other works by this author on: Oxford Academic PubMed Google Scholar JNCI: Journal of the National Cancer Institute, Volume 80, Issue 13, 7 September 1988, Page 1073, https://doi.org/10.1093/jnci/80.13.1073 Published: 07 September 1988
Availability of NCI Monograph No. 51
Journal: JNCI Journal of the National Cancer Institute (1980)

Availability of NCI Monograph No. 51 Get access JNCI: Journal of the National Cancer Institute, Volume 64, Issue 6, June 1980, Page 1571, https://doi.org/10.1093/jnci/64.6.1571-a Published: 01 June 1980
The role of surgical resection following imatinib treatment in patients with metastatic or recurrent GIST.
Authors: Seong Joon Park, Min-Hee Ryu, Baek-Yeol Ryoo, Byeong Seok Sohn, Ki-Hun Kim, Sung Tae Oh, Chang Sik Yu, Jeong Hwan Yook, Byung Sik Kim, Yoon-Koo Kang
Journal: Journal of Clinical Oncology (2013)

62 Background: Several retrospective studies have suggested that surgical resection of residual lesions could be beneficial if resection was done after disease control (partial response or stable disease) with imatinib in patients with metastatic or recurrent GIST. However, the benefit of surgical resection has not been proven yet compared to imatinib alone. Therefore, we compared the outcomes of surgical resection of residual lesions after imatinib (SI group) with those of imatinib alone (IM group) in patients with metastatic or recurrent GIST controlled with imatinib treatment. Methods: A total of 134 patients with metastatic or recurrent GIST who showed at least 6 months of disease stabilization or response to imatinib were included in this retrospective analysis. Patients were categorized into SI (n=42) or IM (n=92) group. To reduce the selection bias, propensity scores and inverse-probability-weighting (IPTW) adjustment were used in the outcome analysis. Results: Patient and tumor characteristics were statistically similar between SI and IM groups, except median age (51 in SI, 58 in IM, p=0.002), and metastases to peritoneum (12 in SI, 56 in IM, p=0.001). The patients in SI group underwent surgery of residual disease after a median 19.1 months of imatinib treatment (range, 7.2-87.0). With a median follow-up of 58.9 months (range, 15.4-129.1), progression-free survival (PFS) and overall survival (OS) were significantly longer in SI group than in IM group (PFS, 87.7 vs 42.8 months, p=0,001; OS, not reached vs 88.8 months, p=0.001). Multivariate analysis revealed SI group as well as female sex, KIT exon 11 mutation, and low initial tumor burden were associated with longer PFS, and SI group and low initial tumor burden were associated with longer OS. Even after applying IPTW adjustment, SI group showed significantly better outcome in terms of PFS (hazard ratio [HR], 2.326; 95% CI, 1.034-5.236; p=0.0412) and OS (HR, 5.464; 95% CI, 1.460-20.408; p=0.0117). Conclusions: This study strongly suggests that surgical resection of the residual lesions after disease control with imatinib may be beneficial in patients with metastatic or recurrent GIST.
Gastrointestinal Bleeding
Authors: Robert L. Berger
Journal: JAMA (1971)

<h3>To the Editor.—</h3> A recent experience in the management of a patient with gastrointestinal tract bleeding should be brought to the attention of the medical profession. A 75-year-old man had had recurrent intestinal bleeding of varying amounts for more than two years. On admission to the Memorial Hospital in Hollywood, Fla, his hemoglobin level was 3.0 gm/100 ml. He never vomited blood nor was blood obtained from gastric aspiration. Many x-ray films, including selective angiography, did not demonstrate the source of the bleeding. He received more than 150 blood transfusions during more than 13 months of continuous hospitalization, and was explored surgically three times. The final procedure was prompted by a complication of the use of the "GI String." It had been suspected for some time that the source of the bleeding was in the distal ileum. Examination of the gastrointestinal tract at surgery from cardia to anus had been
Book Listings
Journal: Annals of Internal Medicine (2001)

Book Listings18 December 2001Book ListingsSearch for more papers by this authorhttps://doi.org/10.7326/0003-4819-135-12-200112180-00036 SectionsAboutFull Text ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail CardiologyCardioFaxDA Tighe, JR Cook, MJ Schweiger, and EK Chung. 239 pages. Cambridge, MA: Blackwell Science; 2001. $30.00. ISBN 0865425639. Order phone 800-759-6102.Coronary Artery Stenting: A Case-Oriented ApproachN Curzen and MT Rothman, eds. 194 pages. Philadelphia: Taylor&Francis; 2001. $120.00. ISBN 1853177180. Order phone 800-821-8312.Evidence-Based Manual of Coronary Care ManagementM Connaughton. 195 pages. Philadelphia: WB Saunders; 2001. $35.00. ISBN 0443064156. Order phone 215-238-7800.Fundamental Approaches to the Management of Cardiac ArrhythmiasRJ Sung and MR Lauer. 804 pages. Boston: Kluwer; 2000. $350.00. ISBN 0792365593. Order phone 781-871-1660.Heart Disease: Review and Assessment4th edition. LS Lilly. 268 pages. Philadelphia: WB Saunders; ... PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics 18 December 2001Volume 135, Issue 12 Page: B1 Keywords Behavior Cells Prevention, policy, and public health ePublished: 18 December 2001 Issue Published: 18 December 2001 Copyright & PermissionsCopyright © 2001 by American College of Physicians. All Rights Reserved.Loading ...
Glutaraldehyde colitis
Authors: H-H Yen, Y-Y Chen
Journal: Endoscopy (2006)

A previously healthy, 69-year-old woman underwent a screening colonoscopy in November 2005. A 1-cm polyp was found in the sigmoid colon and was resected by snare polypectomy. The patient developed mild lower abdominal pain and passed some bloody stools 6 hours after the procedure. She was subsequently admitted and a sigmoidscopy was performed without bowel preparation in order to evaluate the postpolypectomy bleeding. The sigmoidscopy revealed markedly inflamed mucosa, but no ulceration (Figure [1]). During the same period, a total of six patients developed similar symptoms, symptoms that have not been encountered in our endoscopy unit in the past 20 years. Abdominal computed tomography performed in another similar patient (aged 72 years) showed involvement of the rectum and sigmoid colon only (Figure [2]). All the microbiological studies in these patients were negative. The patient’s symptoms resolved 3 days after bowel rest and intravenous fluid supplementation.
Reliability and validity of the instrument for scoring clinical outcomes of research for epidermolysis bullosa (iscorEB)
Authors: A.L. Bruckner, D.L. Fairclough, J.A. Feinstein, I. Lara-Corrales, A.W. Lucky, J. Tolar, E. Pope
Journal: British Journal of Dermatology (2018)

Epidermolysis bullosa (EB) is a group of rare genetic blistering disorders that affects the skin and occasionally internal organs. Accurate estimates of disease severity of such complex disease in the context of research is difficult. We developed a tool called instrument for scoring clinical outcomes of research for Epidermolysis Bullosa (iscorEB) with the goal to assess the disease severity from both physician's and patient's perspective. In the current paper, we report the data from testing the iscorEB in 2 institutions from Canada and the USA. Thirty‐one patients and six physicians from various medical specialties took part in a study to demonstrate how consistent and good the score was at assessing the disease severity. We found that there was good agreement between physicians and between physicians and patients. We also demonstrated that iscorEB is able to differentiate between patients with mild, moderate and severe disease and between subtypes of EB.