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1,052 results on '"Mamula, Petar"'

110. Endoscopic Management of Achalasia: Time for Improvement

Author

Shah, Amit A., primary, Isserman, Rebecca, additional, Taylor, April, additional, Friedman, Marcey-Peyser, additional, Trautwein, Andrea, additional, Fuller, Christina D., additional, Benitez, Alain, additional, Shaw, Kathy, additional, Piccoli, David, additional, Flick, Jonathan, additional, and Mamula, Petar, additional

Published
2019
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111. Sa2042 TECHNICAL OUTCOMES IN PEDIATRIC ERCP: EXPERIENCE WITH THE MULTICENTER PEDIATRIC ERCP DATABASE INITIATIVE (PEDI)

Author

Troendle, David M., primary, Barth, Bradley, additional, Fishman, Douglas S., additional, Liu, Quin, additional, Giefer, Matthew J., additional, Kim, Kyung M., additional, Martinez, Mercedes, additional, Dall'Oglio, Luigi, additional, Angelino, Giulia, additional, De Angelis, Paola, additional, Faraci, Simona, additional, Bitton, Samuel, additional, Wilsey, Michael, additional, Khalaf, Racha T., additional, Werlin, Steven, additional, Dua, Kulwinder S., additional, Gugig, Roberto, additional, Huang, Clifton, additional, Mamula, Petar, additional, Quiros, Antonio, additional, Zheng, Yuhua, additional, Grover, Amit S., additional, and Fox, Victor L., additional

Published
2019
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118. Pediatric Inflammatory Bowel Disease

Author

Mamula, Petar., editor, Grossman, Andrew B., editor, Baldassano, Robert N., editor, Kelsen, Judith R., editor, Markowitz, Jonathan E., editor, Mamula, Petar., editor, Grossman, Andrew B., editor, Baldassano, Robert N., editor, Kelsen, Judith R., editor, and Markowitz, Jonathan E., editor

Published
2017

119. 52 Post-ERCP Pancreatitis in Children and a Report Form an International Multicenter Study Group (Pedi Database)

Author

Troendle, David M., primary, Barth, Bradley, additional, Fishman, Douglas S., additional, Liu, Quin, additional, Zheng, Yuhua, additional, Giefer, Matthew J., additional, Kim, Kyung M., additional, Dall'Oglio, Luigi, additional, Angelino, Giulia, additional, De Angelis, Paola, additional, Faraci, Simona, additional, Martinez, Mercedes, additional, Gugig, Roberto, additional, Mamula, Petar, additional, Bitton, Samuel, additional, Wilsey, Michael, additional, Khalaf, Racha T., additional, Werlin, Steven, additional, Dua, Kulwinder S., additional, Grover, Amit S., additional, and Fox, Victor L., additional

Published
2017
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122. A de novo whole gene deletion of XIAP detected by exome sequencing analysis in very early onset inflammatory bowel disease: a case report

Author

Kelsen, Judith R, Dawany, Noor, Martinez, Alejandro, Martinez, Alejuandro, Grochowski, Christopher M, Maurer, Kelly, Rappaport, Eric, Piccoli, David A, Baldassano, Robert N, Mamula, Petar, Sullivan, Kathleen E, and Devoto, Marcella

Subjects
Male, Pathology, medicine.medical_specialty, DNA Copy Number Variations, Population, Case Report, Disease, VEO-IBD (very early-onset IBD), XIAP (x-linked inhibitor of apoptosis), XLP2 (X-linked lymphoproliferative Disease 2), WES (whole exome sequencing), Age of Onset, Genetic Diseases, X-Linked, Humans, Infant, Newborn, Inflammatory Bowel Diseases, Lymphoproliferative Disorders, Exome, Gene Deletion, Bioinformatics, Inflammatory bowel disease, 03 medical and health sciences, 0302 clinical medicine, medicine, Copy-number variation, education, Exome sequencing, 030304 developmental biology, 0303 health sciences, education.field_of_study, business.industry, Gastroenterology, Infant, General Medicine, X-Linked, medicine.disease, Newborn, 3. Good health, Genetic Diseases, Primary immunodeficiency, 030211 gastroenterology & hepatology, Age of onset, business
Abstract

Background Children with very early-onset inflammatory bowel disease (VEO-IBD), those diagnosed at less than 5 years of age, are a unique population. A subset of these patients present with a distinct phenotype and more severe disease than older children and adults. Host genetics is thought to play a more prominent role in this young population, and monogenic defects in genes related to primary immunodeficiencies are responsible for the disease in a small subset of patients with VEO-IBD. Case Presentation We report a child who presented at 3 weeks of life with very early-onset inflammatory bowel disease (VEO-IBD). He had a complicated disease course and remained unresponsive to medical and surgical therapy. The refractory nature of his disease, together with his young age of presentation, prompted utilization of whole exome sequencing (WES) to detect an underlying monogenic primary immunodeficiency and potentially target therapy to the identified defect. Copy number variation analysis (CNV) was performed using the eXome-Hidden Markov Model. Whole exome sequencing revealed 1,380 nonsense and missense variants in the patient. Plausible candidate variants were not detected following analysis of filtered variants, therefore, we performed CNV analysis of the WES data, which led us to identify a de novo whole gene deletion in XIAP. Conclusion This is the first reported whole gene deletion in XIAP, the causal gene responsible for XLP2 (X-linked lymphoproliferative Disease 2). XLP2 is a syndrome resulting in VEO-IBD and can increase susceptibility to hemophagocytic lymphohistocytosis (HLH). This identification allowed the patient to be referred for bone marrow transplantation, potentially curative for his disease and critical to prevent the catastrophic sequela of HLH. This illustrates the unique etiology of VEO-IBD, and the subsequent effects on therapeutic options. This cohort requires careful and thorough evaluation for monogenic defects and primary immunodeficiencies.

Published
2015

123. Vedolizumab Therapy in Severe Pediatric Inflammatory Bowel Disease

Author

Conrad, Máire A., primary, Stein, Ronen E., additional, Maxwell, Elizabeth C., additional, Albenberg, Lindsey, additional, Baldassano, Robert N., additional, Dawany, Noor, additional, Grossman, Andrew B., additional, Mamula, Petar, additional, Piccoli, David A., additional, and Kelsen, Judith R., additional

Published
2016
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127. Sa2061 Above and Beyond Training: Colonoscopy Learning Curves and Expected Annual Procedural Volume of Credentialed Pediatric Gastroenterologists

Author

Lirio, Richard A., primary, Walsh, Catharine M., additional, Rosh, Joel R., additional, Fishman, Douglas S., additional, Kramer, Robert, additional, Lerner, Diana G., additional, Liu, Quin Y., additional, Mamula, Petar, additional, Pall, Harpreet, additional, Sahn, Benjamin, additional, Troendle, David M., additional, Tuchman, David N., additional, and Lightdale, Jenifer R., additional

Published
2016
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132. Erratum to: A de novo whole gene deletion of XIAP detected by exome sequencing analysis in very early onset inflammatory bowel disease: a case report

Author

Kelsen, Judith R., primary, Dawany, Noor, additional, Martinez, Alejandro, additional, Grochowski, Christopher M., additional, Maurer, Kelly, additional, Rappaport, Eric, additional, Piccoli, David A., additional, Baldassano, Robert N., additional, Mamula, Petar, additional, Sullivan, Kathleen E., additional, and Devoto, Marcella, additional

Published
2015
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133. O-017 Digenic Inheritance of Primary Immunodeficiency Gene Variants Identified by Whole Exome Sequence in Very Early Onset IBD Patients

Author

Baldassano Robert, Sonnenberg Gregory, Moran Christopher, Dawany Noor, Daly Mark, Winter Harland, Devoto Marcella, Piccoli David, Artis David, Rapport Eric, Pauly-Hubbard Helen, Kelsen Judith, and Mamula Petar

Subjects
Genetics, Gastroenterology, Primary immunodeficiency, medicine, Immunology and Allergy, Biology, medicine.disease, Gene, Very early onset, Exome, Digenic inheritance, Sequence (medicine)
Published
2014

134. Can Pediatric Endoscopists Accurately Assess Their Clinical Competency? A Comparison Across Skill Levels

Author

Scaffidi, Michael A., Khan, Rishad, Carnahan, Heather, Ling, Simon C., Lightdale, Jenifer R., Mamula, Petar, Yu, Jeffrey J., Grover, Samir C., and Walsh, Catharine M.

Abstract

Assessment is critical to support pediatric endoscopy training. Although trainee engagement in assessment is encouraged, the use of self-assessment and its accuracy among pediatric endoscopists is not well described. We aimed to determine the self-assessment accuracy of novice, intermediate, and experienced pediatric endoscopists. Novice (performed <50 previous colonoscopies), intermediate (50–500), and experienced (>1000) pediatric endoscopists from 3 North American academic teaching hospitals each performed a clinical colonoscopy. Endoscopists were assessed in real-time by 2 experienced endoscopists using the Gastrointestinal Endoscopy Competency Assessment Tool for Pediatric Colonoscopy (GiECATKIDS). In addition, participants self-assessed their performance using the same instrument. Self-assessment accuracy between the externally assessed and self-assessed scores was evaluated using absolute difference scores, intraclass correlation coefficients, and Bland-Altman analyses. Forty-seven endoscopists participated (21 novices, 16 intermediates, and 10 experienced). Overall, there was moderate agreement of externally assessed and self-assessed GiECATKIDStotal scores with an intraclass correlation coefficient of 0.72 (95% confidence interval, 0.55–0.83). The absolute difference scores among the 3 groups were significantly different (P= 0.005), with experienced endoscopists demonstrating a more accurate self-assessment compared to novices (P= 0.003). Bland-Altman plots revealed that novice endoscopists’ self-assessed scores tended to be higher than their externally assessed scores, indicating they overestimated their performance. We found that endoscopic experience was positively associated with self-assessment accuracy among pediatric endoscopists. Novices were inaccurate in assessing their endoscopic competence and were prone to overestimation of their performances. Our findings suggest novices may benefit from targeted interventions aimed at improving their insight and self-awareness.

Published
2019
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135. Biologics for the treatment of moderate- to-severe ulcerative colitis in pediatric patients

Author

Mamula, Petar and Kelsen

Subjects
Pediatric Health, Medicine and Therapeutics
Abstract

Lindsey G Albenberg, Petar Mamula, Judith R KelsenDivision of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia, Philadelphia, PA, USAAbstract: Ulcerative colitis (UC) is a chronic, inflammatory disease of the large intestine. In pediatric patients, UC is associated with significant morbidity including persistent symptoms affecting quality of life, hospitalizations, and surgery, and therapeutic strategies are limited. The advent of biologics, specifically anti-tumor necrosis factor- α medications, has been very beneficial for pediatric patients suffering from UC. Since the introduction of these therapies, there has been improvement in the rates of both remission as well as colectomy-free survival. However, there has been concern regarding the adverse events associated with these medications including the risk of infection and malignancy. The efficacy and safety of infliximab, the most frequently used biologic medication in pediatric patients with UC, will be the focus of this review.Keywords: ulcerative colitis, biologics, anti-TNF-α, infliximab, adalimumab

Published
2012

136. Exome Sequencing Analysis Reveals Variants in Primary Immunodeficiency Genes in Patients With Very Early Onset Inflammatory Bowel Disease

Author

Kelsen, Judith R., primary, Dawany, Noor, additional, Moran, Christopher J., additional, Petersen, Britt-Sabina, additional, Sarmady, Mahdi, additional, Sasson, Ariella, additional, Pauly-Hubbard, Helen, additional, Martinez, Alejandro, additional, Maurer, Kelly, additional, Soong, Joanne, additional, Rappaport, Eric, additional, Franke, Andre, additional, Keller, Andreas, additional, Winter, Harland S., additional, Mamula, Petar, additional, Piccoli, David, additional, Artis, David, additional, Sonnenberg, Gregory F., additional, Daly, Mark, additional, Sullivan, Kathleen E., additional, Baldassano, Robert N., additional, and Devoto, Marcella, additional

Published
2015
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141. Management of Ingested Foreign Bodies in Children

Author

Kramer, Robert E., primary, Lerner, Diana G., additional, Lin, Tom, additional, Manfredi, Michael, additional, Shah, Manoj, additional, Stephen, Thomas C., additional, Gibbons, Troy E., additional, Pall, Harpreet, additional, Sahn, Ben, additional, McOmber, Mark, additional, Zacur, George, additional, Friedlander, Joel, additional, Quiros, Antonio J., additional, Fishman, Douglas S., additional, and Mamula, Petar, additional

Published
2015
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143. Duodenal Hematoma Following EGD

Author

Sahn, Benjamin, primary, Anupindi, Sudha A., additional, Dadhania, Neha J., additional, Kelsen, Judith R., additional, Nance, Michael L., additional, and Mamula, Petar, additional

Published
2015
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146. Viskoznost tekućeg jogurta tijekom pohrane

Author

Zamberlin, Šimun, Samaržija, Dubravka, Mamula, Petar, Havranek, Jasmina, Pecina, Marija, and Pogačić, Tomislav

Subjects
tekući jogurt, viskoznost
Abstract

Cilj ovog rada bio je utvrditi promjene viskoziteta tekućeg jogurta (n=10) proizvođača A i proizvođača B (n=10) tijekom pohrane od 21 dana. Sadržaj masti, suhe tvari, bezmasne suhe tvari, količina mliječne kiseline, pH i viskozitet tekućeg jogurta utvrđeni su 0., 7., 14. i 21. dana pohrane proizvoda na temperaturi od 4° C. Značajna promjena viskoziteta tekućeg jogurta (p

Published
2007

147. Viskozitet tekućeg jogurta

Author

Mamula, Petar

Subjects
reološka svojstva, struktura, structura, viskozitet, jogurt
Abstract

Reološka svojstva prehrambenih proizvoda pripadaju čimbenicima koji najviše utječu na prihvačanje tih proizvoda od strane proizvođača.Viskozitet je jedno od reoloških svojstava koja stručnjacima najčešće služe pri određivanju kakvoće tekućih prehrambenih proizvoda. Iz tih razloga viskozitet je od najznačajnijih parametara koji utječu na senzorska svojstva finalnog proizvoda.Na viskozitet tekućeg jogurta imaju utjecaj: sastav i vrsta korištenog mlijeka ; homogenizacija ; toplinska obrada ; mljekarske mikrobne kulture ; temperatura i trajanje fermentacije ; dodaci mlijeku ; postupci s jogurtom nakon fermentacije. Zbog toga cilj ovog rada bio je utvrditi karakteristike viskoziteta tekućeg jogurta proizvedenog u dvije različite mljekare (A i B) do vremena održivosti od 21 dana. Tekući jogurti proizvođača A i B pokazuju tipično ne- Njutnovsko vremenski ovisno ponašanje . Signifikantna razlika (p 001) u viskozitetu između tekućih jogurta oba proizvođača utvrđena je 14 i 21 dana. Statistički značajno veća količina suhe tvari i kiselosti proizvođača B utjecala je na stabilniju strukturu tekućeg jogurta proizvođača B.

Published
2005

148. Variation in Preventive Care in Children Receiving Chronic Glucocorticoid Therapy.

Author

Basiaga, Matthew L., Burrows, Evanette K., Denburg, Michelle R., Meyers, Kevin E., Grossman, Andrew B., Mamula, Petar, Grundmeier, Robert W., and Burnham, Jon M.

Abstract

Objective: To assess preventive care measure prescribing in children exposed to glucocorticoids and identify prescribing variation according to subspecialty and patient characteristics.Study Design: Retrospective cohort study of children initiating chronic glucocorticoids in the gastroenterology, nephrology, and rheumatology divisions at a pediatric tertiary care center. Outcomes included 25-hydroxyvitamin D (25OHD) and lipid testing, pneumococcal polysaccharide (PPV) and influenza vaccination, and stress dose hydrocortisone prescriptions.Results: A total of 701 children were followed for a median of 589 days. 25OHD testing was performed in 73%, lipid screening in 29%, and PPV and influenza vaccination in 16% and 78%, respectively. Hydrocortisone was prescribed in 2%. Across specialties, 25OHD, lipid screening, and PPV prescribing varied significantly (all P < .001). Using logistic regression adjusting for specialty, 25OHD testing was associated with older age, female sex, non-Hispanic ethnicity, and lower baseline height and body mass index z-scores (all P < .03). Lipid screening was associated with older age, higher baseline body mass index z-score, and lower baseline height z-score (all P < .01). Vaccinations were associated with lower age (P < .02), and PPV completion was associated with non-White race (P = .04).Conclusions: Among children chronically exposed to glucocorticoids, 25OHD testing and influenza vaccination were common, but lipid screening, pneumococcal vaccination, and stress dose hydrocortisone prescribing were infrequent. Except for influenza vaccination, preventive care measure use varied significantly across specialties. Quality improvement efforts are needed to optimize preventive care in this high-risk population. [ABSTRACT FROM AUTHOR]

Published
2016
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