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4. Juvenile polyposis syndrome in children: The impact of SMAD4 and BMPR1A mutations on clinical phenotype and polyp burden

Author

Cohen, Shlomi, primary, Yerushalmy‐Feler, Anat, additional, Rojas, Isabel, additional, Phen, Claudia, additional, Rudnick, David A., additional, Flahive, Colleen B., additional, Erdman, Steven H., additional, Magen‐Rimon, Ramit, additional, Copova, Ivana, additional, Attard, Thomas, additional, Latchford, Andrew, additional, and Hyer, Warren, additional

Published
2024
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9. Baseline Analysis of a Young α‐1‐Antitrypsin Deficiency Liver Disease Cohort Reveals Frequent Portal Hypertension

Author

Teckman, Jeffrey H, Rosenthal, Philip, Abel, Robert, Bass, Lee M, Michail, Sonia, Murray, Karen F, Rudnick, David A, Thomas, Daniel W, Spino, Cathie, Arnon, Ronen, Hertel, Paula M, Heubi, James, Kamath, Binita M, Karnsakul, Wikrom, Loomes, Kathleen M, Magee, John C, Molleston, Jean P, Romero, Rene, Shneider, Benjamin L, Sherker, Averell H, Sokol, Ronald J, and Network, Childhood Liver Disease Research

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Clinical Research, Digestive Diseases, Liver Disease, Rare Diseases, Chronic Liver Disease and Cirrhosis, Pediatric, Aetiology, 2.1 Biological and endogenous factors, Oral and gastrointestinal, Adolescent, Adult, Child, Child, Preschool, Cohort Studies, Female, Humans, Hypertension, Portal, Infant, Infant, Newborn, Jaundice, Liver, Longitudinal Studies, Male, Prospective Studies, Young Adult, alpha 1-Antitrypsin, alpha 1-Antitrypsin Deficiency, cirrhosis, jaundice, liver enzymes, liver transplant, metabolic liver disease, Childhood Liver Disease Research Network, Medical and Health Sciences, Gastroenterology & Hepatology, Clinical sciences, Nutrition and dietetics, Paediatrics
Abstract

Objectivesα-1-Antitrypsin (A1AT) deficiency is a common genetic disease with an unpredictable and highly variable course. The Childhood Liver Disease Research and Education Network is a National Institutes of Health, multicenter, longitudinal consortium studying pediatric liver diseases, with the objective of prospectively defining natural history and identifying disease modifiers.MethodsLongitudinal, cohort study of A1AT patients' birth through 25 years diagnosed as having liver disease, type PIZZ or PISZ. Medical history, physical examination, laboratory, imaging, and standardized survey tool data were collected during the provision of standard of care.ResultsIn the present report of the cohort at baseline, 269 subjects were enrolled between November 2008 and October 2012 (208 with their native livers and 61 postliver transplant). Subjects with mild disease (native livers and no portal hypertension [PHT]) compared to severe disease (with PHT or postliver transplant) were not different in age at presentation. A total of 57% of subjects with mild disease and 76% with severe disease were jaundiced at presentation (P = 0.0024). A total of 29% of subjects with native livers had PHT, but age at diagnosis and growth were not different between the no-PHT and PHT groups (P > 0.05). Subjects with native livers and PHT were more likely to have elevated bilirubin, ALT, AST, INR, and GGTP than the no-PHT group (P <

Published
2015

10. Juvenile polyposis syndrome in children: The impact of SMAD4and BMPR1Amutations on clinical phenotype and polyp burden

Author

Cohen, Shlomi, Yerushalmy‐Feler, Anat, Rojas, Isabel, Phen, Claudia, Rudnick, David A., Flahive, Colleen B., Erdman, Steven H., Magen‐Rimon, Ramit, Copova, Ivana, Attard, Thomas, Latchford, Andrew, and Hyer, Warren

Abstract

A constitutional disease‐causing variant (DCV) in the SMAD4or BMPR1Agenes is present in 40%–60% of patients with juvenile polyposis syndrome (JPS). The aim of this study was to characterize the clinical course and polyp burden in children with DCV‐positive JPS compared to DCV‐negative JPS. Demographic, clinical, genetic, and endoscopic data of children with JPS were compiled from eight international centers in the ESPHGAN/NASPGHAN polyposis working group. A total of 124 children with JPS were included: 69 (56%) DCV‐negative and 55 (44%) DCV‐positive (53% SMAD4and 47% BMPR1A) with a median (interquartile range) follow‐up of 4 (2.8–6.4) years. DCV‐positive children were diagnosed at an older age compared to DCV‐negative children [12 (8–15.7) years vs. 5 (4–7) years, respectively, p< 0.001], had a higher frequency of family history of polyposis syndromes (50.9% vs. 1.4%, p< 0.001), experienced a greater frequency of extraintestinal manifestations (27.3% vs. 5.8%, p< 0.001), and underwent more gastrointestinal surgeries (16.4% vs. 1.4%, p= 0.002). The incidence rate ratio for the development of new colonic polyps was 6.15 (95% confidence interval 3.93–9.63, p< 0.001) in the DCV‐positive group compared to the DCV‐negative group, with an average of 12.2 versus 2 new polyps for every year of follow‐up. There was no difference in the burden of polyps between patients with SMAD4and BMPR1Amutations. This largest international cohort of pediatric JPS revealed that DCV‐positive and DCV‐negative children exhibit distinct clinical phenotype. These findings suggest a potential need of differentiated surveillance strategies based upon mutation status. Juvenile polyposis syndrome (JPS) can be diagnosed based upon identification of a disease‐causing variant (DCV) in the SMAD4or BMPR1Agenes.These variants are identified in up to 40%–60% of the patients and inherited in an autosomal dominant manner.There are limited data regarding phenotypic differences based on the presence or absence of a DCV.Surveillance guidelines for JPS in children fail to differentiate between DCV‐positive and DCV‐negative patients. Juvenile polyposis syndrome (JPS) can be diagnosed based upon identification of a disease‐causing variant (DCV) in the SMAD4or BMPR1Agenes. These variants are identified in up to 40%–60% of the patients and inherited in an autosomal dominant manner. There are limited data regarding phenotypic differences based on the presence or absence of a DCV. Surveillance guidelines for JPS in children fail to differentiate between DCV‐positive and DCV‐negative patients. DCV‐positive and DCV‐negative children exhibit distinct clinical phenotypes.DCV‐positive children have a higher burden of colonic polyps than DCV‐negative children, suggesting the need for surveillance strategies based upon mutation status. DCV‐positive and DCV‐negative children exhibit distinct clinical phenotypes. DCV‐positive children have a higher burden of colonic polyps than DCV‐negative children, suggesting the need for surveillance strategies based upon mutation status.

Published
2024
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12. A Learning Collaborative Approach Increases Specificity of Diagnosis of Acute Liver Failure in Pediatric Patients

Author

Squires, Robert H., Bukauskas, Kathryn, Schulte, Madeline, Narkewicz, Michael R., Hite, Michelle, Loomes, Kathleen M., Rand, Elizabeth B., Piccoli, David, Kawchak, Deborah, Seidman, Christa, Romero, Rene, Karpen, Saul, de la Cruz-Tracy, Liezl, Ng, Vicky, Hunt, Kelsey, Subbarao, Girish C., Klipsch, Ann, Munson, Sarah, Alonso, Estella M., Sorenson, Lisa, Kelly, Susan, Neighbors, Katie, Rosenthal, Philip, Fleck, Shannon, Leonis, Mike A., Bucuvalas, John, Horning, Tracie, Rodriguez Baez, Norberto, Montanye, Shirley, Cowie, Margaret, Horslen, Simon P., Murray, Karen, Young, Melissa, Nielson, Heather, Klein, Jani, Rudnick, David A., Shepherd, Ross W., Harris, Kathy, Karpen, Saul J., De La Torre, Alejandro, Dell Olio, Dominic, Kelly, Deirdre, Lloyd, Carla, Lobritto, Steven J., Bakhsh, Sumerah, Jonas, Maureen, Elifoson, Scott A., Raza, Roshan, Schwarz, Kathleen B., Karnsakul, Wikrom W., Alford, Mary Kay, Dhawan, Anil, Fitzpatrick, Emer, Shneider, Benjamin L., Kerkar, Nanda N., Haydel, Brandy, Narayanappa, Sreevidya, Lopez, M. James, Shieck, Victoria, Doo, Edward, Sherker, Averell H., Belle, Steven H., Horslen, Simon, Hardison, Regina M., Rodriguez-Baez, Norberto, Ng, Vicky L., and Li, Ruosha

Published
2018
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13. Health Related Quality of Life and Neurocognitive Outcomes in the First Year after Pediatric Acute Liver Failure

Author

Bukauskas, Kathryn, Schulte, Madeline, Narkewicz, Michael R., Hite, Michelle, Rand, Elizabeth B., Piccoli, David, Kawchak, Deborah, Seidman, Christa, Romero, Rene, Karpen, Saul, de la Cruz-Tracy, Liezl, Hunt, Kelsey, Subbarao, Girish C., Klipsch, Ann, Munson, Sarah, Kelly, Susan, Rosenthal, Philip J., Fleck, Shannon, Leonis, Mike A., Bucuvalas, John, Horning, Tracie, Baez, Norberto Rodriguez, Montanye, Shirley, Cowie, Margaret, Horslen, Simon P., Murray, Karen, Young, Melissa, Nielson, Heather, Klein, Jani, Rudnick, David A., Shepherd, Ross W., Harris, Kathy, Karpen, Saul J., De La Torre, Alejandro, Dell Olio, Dominic, Kelly, Deirdre, Lloyd, Carla, Lobritto, Steven J., Bakhsh, Sumerah, Jonas, Maureen, Elifoson, Scott A., Raza, Roshan, Schwarz, Kathleen B., Karnsakul, Wikrom W., Alford, Mary Kay, Dhawan, Anil, Fitzpatrick, Emer, Kerkar, Nanda N., Haydel, Brandy, Narayanappa, Sreevidya, Lopez, M. James, Shieck, Victoria, Sorensen, Lisa G., Neighbors, Katie, Hardison, Regina M., Loomes, Kathleen M., Varni, James W., Ng, Vicky L., Squires, Robert H., and Alonso, Estella M.

Published
2018
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14. Intravenous N‐acetylcysteine in pediatric patients with nonacetaminophen acute liver failure: A placebo‐controlled clinical trial

Author

Squires, Robert H, Dhawan, Anil, Alonso, Estella, Narkewicz, Michael R, Shneider, Benjamin L, Rodriguez‐Baez, Norberto, Olio, Dominic Dell, Karpen, Saul, Bucuvalas, John, Lobritto, Steven, Rand, Elizabeth, Rosenthal, Philip, Horslen, Simon, Ng, Vicky, Subbarao, Girish, Kerkar, Nanda, Rudnick, David, Lopez, M James, Schwarz, Kathleen, Romero, Rene, Elisofon, Scott, Doo, Edward, Robuck, Patricia R, Lawlor, Sharon, Belle, Steven H, and Group, for the Pediatric Acute Liver Failure Study

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Liver Disease, Digestive Diseases, Clinical Research, Pediatric, Clinical Trials and Supportive Activities, Evaluation of treatments and therapeutic interventions, 6.1 Pharmaceuticals, Oral and gastrointestinal, Acetylcysteine, Adolescent, Child, Child, Preschool, Double-Blind Method, Female, Free Radical Scavengers, Hepatic Encephalopathy, Humans, Infant, Infant, Newborn, Infusions, Intravenous, Liver Failure, Acute, Liver Transplantation, Male, Severity of Illness Index, Survival Rate, Treatment Outcome, Pediatric Acute Liver Failure Study Group, Medical Biochemistry and Metabolomics, Immunology, Gastroenterology & Hepatology, Clinical sciences
Abstract

UnlabelledN-acetylcysteine (NAC) was found to improve transplantation-free survival in only those adults with nonacetaminophen (non-APAP) acute liver failure (ALF) and grade 1-2 hepatic encephalopathy (HE). Because non-APAP ALF differs significantly between children and adults, the Pediatric Acute Liver Failure (PALF) Study Group evaluated NAC in non-APAP PALF. Children from birth through age 17 years with non-APAP ALF enrolled in the PALF registry were eligible to enter an adaptively allocated, doubly masked, placebo-controlled trial using a continuous intravenous infusion of NAC (150 mg/kg/day in 5% dextrose in water [D5W]) or placebo (D5W) for up to 7 days. The primary outcome was 1-year survival. Secondary outcomes included liver transplantation-free survival, liver transplantation (LTx), length of intensive care unit (ICU) and hospital stays, organ system failure, and maximum HE score. A total of 184 participants were enrolled in the trial with 92 in each arm. The 1-year survival did not differ significantly (P = 0.19) between the NAC (73%) and placebo (82%) treatment groups. The 1-year LTx-free survival was significantly lower (P = 0.03) in those who received NAC (35%) than those who received placebo (53%), particularly, but not significantly so, among those less than 2 years old with HE grade 0-1 (NAC 25%; placebo 60%; P = 0.0493). There were no significant differences between treatment arms for hospital or ICU length of stay, organ systems failing, or highest recorded grade of HE.ConclusionNAC did not improve 1-year survival in non-APAP PALF. One-year LTx-free survival was significantly lower with NAC, particularly among those

Published
2013

21. Clinical Course among Cases of Acute Liver Failure of Indeterminate Diagnosis

Author

Bukauskas, Kathryn, Narkewicz, Michael R., Hite, Michelle, Loomes, Kathleen M., Rand, Elizabeth B., Piccoli, David, Kawchak, Deborah, Romero, Rene, Karpen, Saul, de la Cruz-Tracy, Liezl, Ng, Vicky, Hunt, Kelsey, Subbarao, Girish C., Klipsch, Ann, Alonso, Estella M., Sorenson, Lisa, Kelly, Susan, Delute, Dhey, Neighbors, Katie, Rosenthal, Philip J., Fleck, Shannon, Leonis, Mike A., Bucuvalas, John, Horning, Tracie, Baez, Norberto Rodriguez, Montanye, Shirley, Cowie, Margaret, Murray, Karen, Young, Melissa, Vendettuoli, Heather, Rudnick, David A., Shepherd, Ross W., Harris, Kathy, Karpen, Saul J., De La Torre, Alejandro, Dell Olio, Dominic, Kelly, Deirdre, Lloyd, Carla, Lobritto, Steven J., Bakhsh, Sumerah, Jonas, Maureen, Elifoson, Scott A., Raza, Roshan, Schwarz, Kathleen B., Karnsakul, Wikrom W., Alford, Mary Kay, Dhawan, Anil, Fitzpatrick, Emer, Kerkar, Nanda N., Haydel, Brandy, Narayanappa, Sreevidya, Lopez, M. James, Shieck, Victoria, Li, Ruosha, Belle, Steven H., Horslen, Simon, Chen, Ling-wan, Zhang, Song, and Squires, Robert H.

Published
2016
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28. Regulation of PGC1α Downstream of the Insulin Signaling Pathway Plays a Role in the Hepatic Proteotoxicity of Mutant α1-Antitrypsin Deficiency Variant Z

Author

Rudnick, David A., primary, Huang, Jiansheng, additional, Hidvegi, Tunda, additional, Chu, Andrew S., additional, Hale, Pamela, additional, Munanairi, Admire, additional, Dietzen, Dennis J., additional, Cliften, Paul F., additional, Tycksen, Eric, additional, Lutkewitte, Andrew J., additional, Finck, Brian N., additional, Pak, Stephen C., additional, Silverman, Gary A., additional, and Perlmutter, David H., additional

Published
2022
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34. Flume

Author

Holland, P. G., Herschy, R. W., Archer, David, Green, Colin, Wright, Robert L., Shaw, Elizabeth M., Ervine, D. A., MacLeod, A. B., Rudnick, David T., Gibbard, David W., Herschy, Reginald W., editor, and Fairbridge, Rhodes W., editor

Published
1998
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38. LETTER

Author

Rudnick, David

Published
2010

42. Contributors

Author

Alpini, Gianfranco, primary, Apte, Udayan, additional, Bhave, Vishakha, additional, Brown, Alicia R., additional, Campbell, Jean S., additional, Cook, Daniel, additional, Diehl, Anna Mae, additional, Ding, Lili, additional, Duncan, Andrew W., additional, Fan, Mingjie, additional, Freeman, Christopher M., additional, Glaser, Shannon, additional, Goessling, Wolfram, additional, Han, Yuyan, additional, Huang, Jiansheng, additional, Huang, Wendong, additional, Lentsch, Alex B., additional, Li, Yanfeng, additional, Limaye, Pallavi B., additional, Locker, Joseph, additional, Machado, Mariana Verdelho, additional, Maroni, Luca, additional, Marzioni, Marco, additional, Meng, Fanyin, additional, Michalopoulos, George K., additional, Monga, Satdarshan Pal Singh, additional, Nejak-Bowen, Kari Nichole, additional, Nevzorova, Yulia A., additional, Nojima, Hiroyuki, additional, Petersen, Bryon E., additional, Pi, Liya, additional, Polgar, Zsuzsanna, additional, Pritchard, Michele T., additional, Ray, Debolina, additional, Riehle, Kimberly J., additional, Riggle, Kevin, additional, Robinson, Paulette M., additional, Roy-Chowdhury, Jayanta, additional, Roy-Chowdhury, Namita, additional, Rudnick, David A., additional, Shafritz, David A., additional, Sullivan, David C., additional, Trautwein, Christian, additional, Vadigepalli, Rajanikanth, additional, Walesky, Chad, additional, and Wilson, Gregory C., additional

Published
2015
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