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

Author

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

Published
2018
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104. Biliary atresia: a multidisciplinary approach to diagnosis and management

Author

Moreira, Roger Klein, Cabral, Rodrigo, Cowles, Robert A., and Lobritto, Steven J.

Subjects
Transplantation of organs, tissues, etc., Biliary atresia -- Development and progression, Algorithms, Algorithm, Health
Abstract

* Context.--Biliary atresia is an inflammatory cholangiopathy of infancy that results in progressive fibrosis and obliteration of bile ducts and represents the main indication for liver transplant in young children. In spite of extensive investigation, its etiology has remained poorly understood. Timely surgical intervention (Kasai procedure) may result in significant benefit to these patients and represents the final goal of an accurate diagnostic evaluation. Objective.--To present an overview of biliary atresia, including clinical and surgical approaches to this disease, with emphasis on the histopathologic evaluation. Data Sources.--Review of relevant literature indexed in PubMed (US National Library of Medicine). Conclusion.--A well-coordinated multidisciplinary approach is required in the assessment of suspected cases of biliary atresia. Pathologic examination of biopsy specimens is an integral part of the diagnostic algorithm and, therefore, plays a pivotal role in the diagnostic evaluation of this disease. (Arch Pathol Lab Med. 2012; 136:746-760; doi: 10.5858/arpa.2011-0623-RA), Biliary atresia is an inflammatory cholangiopathy of infancy that results in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts. The etiology of biliary atresia has been subject of [...]

Published
2012
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105. ZIRCON: Pharmacokinetics, Safety, and Efficacy of Ombitasvir/Paritaprevir/Ritonavir ± THU-251 Dasabuvir ± Ribavirin in Adolescents With Genotype 1 or 4 Hepatitis C Virus Infection

Author

UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, Leung, Daniel, Yao, Betty, Gonzalez-Peralta, Regino, Jonas, Maureen, Lobritto, Steven, Narkewicz, Michael, Sokal, Etienne, Fortuny, Claudia, Hsu, Evelyn, Wirth, Stefan, Del Valle-Segarra, Antonio, Zha, Jiuhong, Larsen, Loise, Liu, Li, Shuster, Diana, Cohen, Daniel, Rosenthal, Philip, The International Liver Congress (EASL), UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, Leung, Daniel, Yao, Betty, Gonzalez-Peralta, Regino, Jonas, Maureen, Lobritto, Steven, Narkewicz, Michael, Sokal, Etienne, Fortuny, Claudia, Hsu, Evelyn, Wirth, Stefan, Del Valle-Segarra, Antonio, Zha, Jiuhong, Larsen, Loise, Liu, Li, Shuster, Diana, Cohen, Daniel, Rosenthal, Philip, and The International Liver Congress (EASL)

Abstract

BACKGROUND In adults, treatment of hepatitis C virus (HCV) genotype 1 (GT1) and GT4 with ombitasvir, paritaprevir, ritonavir ± dasabuvir (OBV/PTV/r ± DSV) ± ribavirin (RBV) results in high rates of sustained virologic response (SVR)1–6. However, these direct-acting anti virals (DAAs) have not been studied in children or adolescents. ZIRCON is an ongoing, global, open-label, 3-part study to evaluate the pharmacokinetics (PK), safety, and efficacy of OBV/PTV/r ± DSV ± RBV for pediatric and adolescent patients infected with HCV GT1 or GT4 (NCT02486406) Data for patients aged ≥12–17 years enrolled in Parts 1 and 2 are presented here OBJECTIVES To asess the PK of OBV/PTV/r + DSV ± RBV in HCV GT1-infected adolescents in Part 1 • To assess the efficacy and safety of OBV/PTV/r ± DSV ± RBV for 12 or 24 weeks in HCV GT1- or GT4-infected adolescents in Parts 1 and 2 METHODS STUDY DESIGN • Part 1 is a Phase 2 study enrolling treatment-naïve, non-cirrhotic, HCV GT1-infected patients who received treatment for 12 weeks • Part 2 is a Phase 3 study enrolling treatment-naïve or -experienced HCV GT1- or GT4-infected patients with or without compensated cirrhosis who received treatment for 12 or 24 weeks depending on their GT subtype and cirrhosis status. Enrollment in Part 2 began as soon as dosing recommendations for the study drugs were available based on PK and clinical data from Part 1 • Treatment was OBV/PTV/r 25 mg/150 mg/100 mg once daily ± DSV 250 mg twice daily ± weight-based RBV 1000 mg or 1200 mg divided twice daily • Blood samples were collected for intensive PK sampling in Part 1 at treatment Week 2 (at 2, 4, 8, 12, and 24 hours post dose) • Blood samples were collected for measurement of HCV RNA in Parts 1 and 2 at treatment Day 1 and at Weeks 2, 4, 8, and 12 for patients who received the 12-week regimen, as well as Weeks 16, 20, and 24 for patients who received the 24-week regimen • SVR was assessed at post-treatment Week 12 (SVR12) PATIENT SELECTION Key Inclusion Crit

Published
2017

106. Production of proinflammatory cytokines by monocytes in liver transplanted recipients with de novo automimmune hepatitis is enhanced and induces TH1-like regulatory T cells

Author

Arterbery, Adam S., Asofo-Addo, Awo, Avitzur, Yaron, Ciarleglio, Maria, Deng, Yanhong, Lobritto, Steven J., Martinez, Mercedes, Hafler, David A., Kleinewietfeld, Markus, and Ekong, Udeme D.

Subjects
chemical and pharmacologic phenomena, hemic and immune systems
Abstract

A subset of human regulatory T cells (Tregs) can secrete IFN-γ or IL-17 and thus share features of TH1 or TH17 effector cells, and lose suppressive function. The main factors driving this differentiation of Tregs towards a pro-inflammatory phenotype include IL-12 for TH1-like and IL-6 for TH17-type Tregs. In this study we show that Tregs of patients with de novo autoimmune hepatitis (dAIH) display increased frequencies of pro-inflammatory IFN-γ and IL-17 cytokines. Irrespective of a fully demethylated FOXP3 locus, Tregs of subjects with de novo autoimmune hepatitis are functionally impaired. In line with the observed Treg phenotype, we detected the presence of two dominant cytokines (IL-12 and IL-6) clustering with CD68+ monocyte/macrophage cells in livers of subjects with de novo autoimmune hepatitis and isolated monocytes of subjects with de novo autoimmune hepatitis secrete high levels of pro-inflammatory IL-12 and IL-6 suggesting that this inflammatory milieu is key for functional impairment of Tregs. Importantly, the blockade of IFN-γ partially restores suppressive function of Tregs of subjects with de novo autoimmune hepatitis indicating that monocyte/macrophage derived triggers might play a central role in Treg dysfunction and pathogenesis of de novo autoimmune hepatitis. M.K. was supported by the European Research Council (ERC) under the European Union’s Horizon 2020 research and innovation programme (640116).

Published
2016

112. An Analysis of Viral Testing in non-Acetaminophen (non-APAP) Pediatric Acute Liver Failure (PALF)

Author

Schwarz, Kathleen B., Olio, Dominic Dell, Lobritto, Steven J., Lopez, M James, Rodriguez-Baez, Norberto, Yazigi, Nada A., Belle, Steven H., Zhang, Song, and Squires, Robert H.

Subjects
Adolescent, Child, Preschool, Infant, Newborn, Humans, Infant, Simplexvirus, Herpes Simplex, Liver Failure, Acute, Antibodies, Viral, Child, Polymerase Chain Reaction, Article
Abstract

Viral infections are often suspected to cause pediatric acute liver failure (PALF), but large-scale studies have not been performed. We analyzed the results of viral testing among nonacetaminophen PALF study participants.Participants were enrolled in the PALF registry. Diagnostic evaluation and final diagnosis were determined by the site investigator and methods for viral testing by local standard of care. Viruses were classified as either causative viruses (CVs) or associated viruses (AVs). Supplemental testing for CV was performed if not done clinically and serum was available. Final diagnoses included "viral," "indeterminate," and "other."Of 860 participants, 820 had at least 1 test result for a CV or AV. A positive viral test was found in 166/820 (20.2%) participants and distributed among "viral" (66/80 [82.5%]), "indeterminate" (52/420 [12.4%]), and "other" (48/320 [15.0%]) diagnoses. CVs accounted for 81/166 (48.8%) positive tests. Herpes simplex virus (HSV) was positive in 39/335 (11.6%) who were tested 26/103 (25.2%) and 13/232 (5.6%) among infants 0 to 6 and6 months, respectively. HSV was not tested in 61.0% and 53% of the overall cohort and those 0 to 6 months, respectively. Supplemental testing yielded 17 positive, including 5 HSV.Viral testing in PALF occurs frequently but is often incomplete. The evidence for acute viral infection was found in 20.2% of those tested for viruses. HSV is an important viral cause for PALF in all age groups. The etiopathogenic role of CV and AV in PALF requires further investigation.

Published
2014

113. Production of Proinflammatory Cytokines by Monocytes in Liver-Transplanted Recipients with De Novo Autoimmune Hepatitis Is Enhanced and Induces TH1-like Regulatory T Cells

Author

Arterbery, Adam S., primary, Osafo-Addo, Awo, additional, Avitzur, Yaron, additional, Ciarleglio, Maria, additional, Deng, Yanhong, additional, Lobritto, Steven J., additional, Martinez, Mercedes, additional, Hafler, David A., additional, Kleinewietfeld, Markus, additional, and Ekong, Udeme D., additional

Published
2016
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114. Clinical Course among Cases of Acute Liver Failure of Indeterminate Diagnosis

Author

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

Published
2016
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115. A Novel Pathogenic UGT1A1Variant in a Sudanese Child with Type 1 Crigler-Najjar Syndrome

Author

Elfar, Walaa, Ja¨rvinen, Erkka, Ji, Weizhen, Mosorin, Johanna, Sega, Annalisa G., Iuga, Alina C., Lobritto, Steven J., Konstantino, Monica, Chan, Albert, Finel, Moshe, and Lakhani, Saquib A.

Abstract

Uridine diphosphate glucuronosyltransferases (UGTs) are key enzymes responsible for the body’s ability to process a variety of endogenous and exogenous compounds. Significant gains in understanding UGT function have come from the analysis of variants seen in patients. We cared for a Sudanese child who showed clinical features of type 1 Crigler-Najjar syndrome (CN-1), namely severe unconjugated hyperbilirubinemia leading to liver transplantation. CN-1 is an autosomal recessive disorder caused by damaging mutations in the gene for UGT1A1, the hepatic enzyme responsible for bilirubin conjugation in humans. Clinical genetic testing was unable to identify a known pathogenic UGT1A1mutation in this child. Instead, a novel homozygous variant resulting in an in-frame deletion, p.Val275del, was noted. Sanger sequencing demonstrated that this variant segregated with the disease phenotype in this family. We further performed functional testing using recombinantly expressed UGT1A1 with and without the patient variant, demonstrating that p.Val275del results in a complete lack of glucuronidation activity, a hallmark of CN-1. Sequence analysis of this region shows a high degree of conservation across all known catalytically active human UGTs, further suggesting that it plays a key role in the enzymatic function of UGTs. Finally, we note that the patient’s ethnicity likely played a role in his variant being previously undescribed and advocate for greater diversity and inclusion in genomic medicine.

Published
2019
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118. Multiple cutaneous infantile hemangiomas associated with hepatic angiosarcoma: case report and review of the literature

Author

Nord, Kristin M., Kandel, Jessica, Lefkowitch, Jay H., Lobritto, Steven J., Morel, Kimberly D., North, Paula E., and Garzon, Maria C.

Subjects
Hemangioma -- Diagnosis, Hemangioma -- Drug therapy, Hemangioma -- Research, Infants (Premature) -- Health aspects, Liver -- Transplantation, Liver -- Evaluation
Abstract

Multiple cutaneous hemangiomas can be associated with internal hemangiomas, with the liver being the most common site. Here we report a case of a premature female neonate who presented with cardiac failure at birth and had typical-appearing infantile hemangiomas on the skin in association with vascular lesions in the liver. Her clinical presentation was felt to be consistent with cutaneous and hepatic infantile hemangiomas. After failure to respond to systemic steroids and chemotherapy, she underwent liver transplantation. Histopathologic evaluation of the liver revealed a diagnosis of type 2 infantile hepatic hemangioendothelioma (regarded as synonymous with angiosarcoma) rather than benign infantile hemangioma of the liver. Subsequent skin biopsies confirmed that her multiple cutaneous lesions were infantile hemangiomas and not metastatic angiosarcoma. We report this case and a review of the literature on pediatric angiosarcoma of the liver associated with cutaneous infantile hemangiomas. KEY WORDS. hemangioma, neoplasm, liver transplantation., Kristin M. Nord, MD, Jessica Kandel, MD, Jay H. Lefkowitch, MD, Steven J. Lobritto, MD, Kimberly D. Morel, MD, Paula E. North, MD, PhD, Maria C. Garzon, [...]

Published
2006

119. Improved tolerance for enteral nutrition after serial transverse enteroplasty (STEP) in infants and children with short bowel syndrome—A seven-year single-center experience

Author

Oh, Pilyung S., primary, Fingeret, Abbey L., additional, Shah, Manan Y., additional, Ventura, Kara A., additional, Brodlie, Susan, additional, Ovchinsky, Nadia, additional, Martinez, Mercedes, additional, Lobritto, Steven J., additional, and Cowles, Robert A., additional

Published
2014
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121. 649 Long-Term Durability of Sustained Viral Response (SVR) in Children Treated for Chronic Hepatitis C (Peds C Trial)

Author

Schwarz, Kathleen, primary, Molleston, Jean P., additional, Jonas, Maureen M., additional, Wen, Jessica, additional, Murray, Karen F., additional, Rosenthal, Philip, additional, Gonzalez-Peralta, Regino P., additional, Lobritto, Steven J., additional, Mogul, Douglas, additional, Hooper, Gregory, additional, Warne, Charles, additional, and Thompson, Bruce, additional

Published
2014
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122. Differences in presentation and progression between severe FIC1 and BSEP deficiencies.

Author

UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, Pawlikowska, Ludmila, Strautnieks, Sandra, Jankowska, Irena, Czubkowski, Piotr, Emerick, Karan, Antoniou, Anthony, Wanty, Catherine, Fischler, Bjorn, Jacquemin, Emmanuel, Wali, Sami, Blanchard, Samra, Nielsen, Inge-Merete, Bourke, Billy, McQuaid, Shirley, Lacaille, Florence, Byrne, Jane A, van Eerde, Albertien M, Kolho, Kaija-Leena, Klomp, Leo, Houwen, Roderick, Bacchetti, Peter, Lobritto, Steven, Hupertz, Vera, McClean, Patricia, Mieli-Vergani, Giorgina, Shneider, Benjamin, Nemeth, Antal, Sokal, Etienne, Freimer, Nelson B, Knisely, A S, Rosenthal, Philip, Whitington, Peter F, Pawlowska, Joanna, Thompson, Richard J, Bull, Laura N, UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, Pawlikowska, Ludmila, Strautnieks, Sandra, Jankowska, Irena, Czubkowski, Piotr, Emerick, Karan, Antoniou, Anthony, Wanty, Catherine, Fischler, Bjorn, Jacquemin, Emmanuel, Wali, Sami, Blanchard, Samra, Nielsen, Inge-Merete, Bourke, Billy, McQuaid, Shirley, Lacaille, Florence, Byrne, Jane A, van Eerde, Albertien M, Kolho, Kaija-Leena, Klomp, Leo, Houwen, Roderick, Bacchetti, Peter, Lobritto, Steven, Hupertz, Vera, McClean, Patricia, Mieli-Vergani, Giorgina, Shneider, Benjamin, Nemeth, Antal, Sokal, Etienne, Freimer, Nelson B, Knisely, A S, Rosenthal, Philip, Whitington, Peter F, Pawlowska, Joanna, Thompson, Richard J, and Bull, Laura N

Abstract

Progressive familial intrahepatic cholestasis (PFIC) with normal serum levels of gamma-glutamyltranspeptidase can result from mutations in ATP8B1 (encoding familial intrahepatic cholestasis 1 [FIC1]) or ABCB11 (encoding bile salt export pump [BSEP]). We evaluated clinical and laboratory features of disease in patients diagnosed with PFIC, who carried mutations in ATP8B1 (FIC1 deficiency) or ABCB11 (BSEP deficiency). Our goal was to identify features that distinguish presentation and course of these two disorders, thus facilitating diagnosis and elucidating the differing consequences of ATP8B1 and ABCB11 mutations. A retrospective multi-center study was conducted, using questionnaires and chart review. Available clinical and biochemical data from 145 PFIC patients with mutations in either ATP8B1 (61 "FIC1 patients") or ABCB11 (84 "BSEP patients") were evaluated. At presentation, serum aminotransferase and bile salt levels were higher in BSEP patients; serum alkaline phosphatase values were higher, and serum albumin values were lower, in FIC1 patients. Elevated white blood cell counts, and giant or multinucleate cells at liver biopsy, were more common in BSEP patients. BSEP patients more often had gallstones and portal hypertension. Diarrhea, pancreatic disease, rickets, pneumonia, abnormal sweat tests, hearing impairment, and poor growth were more common in FIC1 patients. Among BSEP patients, the course of disease was less rapidly progressive in patients bearing the D482G mutation. Severe forms of FIC1 and BSEP deficiency differed. BSEP patients manifested more severe hepatobiliary disease, while FIC1 patients showed greater evidence of extrahepatic disease.

Published
2010

123. Differences in presentation and progression between severe FIC1 and BSEP deficiencies

Author

Pawlikowska, Ludmila, Strautnieks, Sandra, Jankowska, Irena, Czubkowski, Piotr, Emerick, Karan, Antoniou, Anthony, Wanty, Catherine, Fischler, Bjorn, Jacquemin, Emmanuel, Wali, Sami, Blanchard, Samra, Nielsen, Inge Merete, Bourke, Billy, McQuaid, Shirley, Lacaille, Florence, Byrne, Jane A., van Eerde, Albertien M., Kolho, Kaija Leena, Klomp, Leo, Houwen, Roderick, Bacchetti, Peter, Lobritto, Steven, Hupertz, Vera, McClean, Patricia, Mieli-Vergani, Giorgina, Shneider, Benjamin, Nemeth, Antal, Sokal, Etienne, Freimer, Nelson B., Knisely, A. S., Rosenthal, Philip, Whitington, Peter F., Pawlowska, Joanna, Thompson, Richard J., Bull, Laura N., Pawlikowska, Ludmila, Strautnieks, Sandra, Jankowska, Irena, Czubkowski, Piotr, Emerick, Karan, Antoniou, Anthony, Wanty, Catherine, Fischler, Bjorn, Jacquemin, Emmanuel, Wali, Sami, Blanchard, Samra, Nielsen, Inge Merete, Bourke, Billy, McQuaid, Shirley, Lacaille, Florence, Byrne, Jane A., van Eerde, Albertien M., Kolho, Kaija Leena, Klomp, Leo, Houwen, Roderick, Bacchetti, Peter, Lobritto, Steven, Hupertz, Vera, McClean, Patricia, Mieli-Vergani, Giorgina, Shneider, Benjamin, Nemeth, Antal, Sokal, Etienne, Freimer, Nelson B., Knisely, A. S., Rosenthal, Philip, Whitington, Peter F., Pawlowska, Joanna, Thompson, Richard J., and Bull, Laura N.

Abstract

Background & Aims: Progressive familial intrahepatic cholestasis (PFIC) with normal serum levels of gamma-glutamyltranspeptidase can result from mutations in ATP8B1 (encoding familial intrahepatic cholestasis 1 [FIC1]) or ABCB11 (encoding bile salt export pump [BSEP]). We evaluated clinical and laboratory features of disease in patients diagnosed with PFIC, who carried mutations in ATP8B1 (FIC1 deficiency) or ABCB11 (BSEP deficiency). Our goal was to identify features that distinguish presentation and course of these two disorders, thus facilitating diagnosis and elucidating the differing consequences of ATP8B1 and ABCB11 mutations. Methods: A retrospective multi-center study was conducted, using questionnaires and chart review. Available clinical and biochemical data from 145 PFIC patients with mutations in either ATP8B1 (61 "FIC1 patients") or ABCB11 (84 "BSEP patients") were evaluated. Results: At presentation, serum aminotransferase and bile salt levels were higher in BSEP patients; serum alkaline phosphatase values were higher, and serum albumin values were lower, in FIC1 patients. Elevated white blood cell counts, and giant or multinucleate cells at liver biopsy, were more common in BSEP patients. BSEP patients more often had gallstones and portal hypertension. Diarrhea, pancreatic disease, rickets, pneumonia, abnormal sweat tests, hearing impairment, and poor growth were more common in FIC1 patients. Among BSEP patients, the course of disease was less rapidly progressive in patients bearing the D482G mutation. Conclusions: Severe forms of FIC1 and BSEP deficiency differed. BSEP patients manifested more severe hepatobiliary disease, while FIC1 patients showed greater evidence of extrahepatic disease.

Published
2010

125. The Risk Factors Associated with Liver Injury and the Impact of Liver Injury on Transplant Related Mortality in Pediatric Recipients of Allogeneic Hematopoietic Stem Cell Transplantation

Author

Radhakrishnan, Kavita, primary, Bishop, Jacquelyn, additional, Jin, Zhezhen, additional, Kothari, Komal, additional, Bhatia, Monica, additional, George, Diane, additional, Garvin, James, additional, Martinez, Mercedes, additional, Ovchinsky, Nadia, additional, Lobritto, Steven, additional, Elsayed, Yasmin, additional, and Satwani, Prakash, additional

Published
2013
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126. A Prospective Clinical Trial Shows That Intravenous N-Acetylcysteine (NAC) Does Not Improve Survival in Pediatric Patients With Non-Acetaminophen Acute Liver Failure

Author

Squires, Robert, primary, Dhawan, Anil, additional, Alonso, Estella M., additional, Narkewicz, Michael R., additional, Rodriguez-Baez, Norberto, additional, Kelly, Deirdre A., additional, Bucuvalas, John, additional, Karpen, Saul J., additional, Shneider, Benjamin L., additional, Rand, Elizabeth B., additional, Lobritto, Steven J., additional, Rosenthal, Philip, additional, Murray, Karen F., additional, Kerkar, Nanda, additional, Ng, Vicky L., additional, Subbarao, Girish, additional, Rudnick, David A., additional, Lopez, M. James, additional, Schwarz, Kathleen B., additional, Romero, Rene, additional, Elisofon, Scott, additional, Robuck, Patricia R., additional, Lawlor, Sharon M., additional, and Belle, Steven H., additional

Published
2011
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127. Contributors

Author

Abu-Elmagd, Kareem, Ahn, Chul-Soo, Allamezadeh, Reza, M. Alonso, Estella, Alonso, Maria H., Ascher, Nancy L., Bäckman, Lars, Baker, Talia B., Balistreri, William F., Bañares, Rafael, Baquerizo, Angeles, Bathla, Lokesh, Bennet, William, Berenguer, Marina, Berlakovich, Gabriela A., Bezerra, Jorge A., Bilhartz, Jacob L., Brown, Robert S., Jr, Burroughs, Andrew, Burroughs, Sherilyn Gordon, Busuttil, Ronald W., Caicedo, Juan Carlos, Cameron, Andrew M., Campsen, Jeffrey, Carey, Elizabeth J., Carmody, Ian C., Cecka, J. Michael, Chan, See-Ching, Charlton, Michael, Cheaito, Ali, Chen, Pauline W., Chinnakotla, Srinath, Ciria, Ruben, Clavien, Pierre-Alain, Coito, Ana J., Collins, Thomas, Crippin, Jeffrey S., Cronin, David C., II, Danovitch, Gabriel M., Davis, Gary L., Gloria de la Rosa, Demetris, Anthony J., DiNorcia, Joseph, Duffy, John P., Durazo, Francisco A., Eghtesad, Bijan, Emond, Jean C., Esquivel, Carlos O., Fan, Sheung Tat, Farmer, Douglas G., Fondevila, Constantino, Forsythe, John L.R., Fox, Alyson N., Fox, Ira J., Frader, Joel E., Fredericks, Emily M., Fulmer, James M., Fung, John J., Gallegos-Orozco, Juan F., García-Valdecasas, Juan Carlos, Gerling, Till, Ghobrial, R. Mark, Gomes, Antoinette S., Gonzalez, Stevan A., Gordon, Elisa J., Green, Michael D., Harrison, Rick, Hasse, Jeanette M., Heaton, Nigel D., Hessheimer, Amelia J., Hiatt, Jonathan R., Holt, Curtis D., Hong, Johnny C., Humar, Abhinav, Ibrahim, Samar H., Ikegami, Toru, Imam, Mohamad H., Inomata, Yukihiro, Jensen, Sally E., Jowsey, Sheila, Kaldas, Fady M., Kam, Igal, Kelly, Jr., Burnett “Beau” S., Khungar, Vandana, Khwaja, Khalid, King, Kevin, Kinkhabwala, Milan, Kirk, Allan D., Kittleson, Michelle M., Klintmalm, Göran B.G., Kunder, Gregory D., Kupiec-Weglinski, Jerzy W., Lake, John R., Langnas, Alan, Lassman, Charles R., Lee, Sung-Gyu, Lin, Henry C., Lo, Chung-Mau, Lobritto, Steven, Locke, Jayme E., Lucey, Michael R., MacConmara, Malcolm, Maehara, Yoshihiko, Mai, Martin L., Makuuchi, Masatoshi, Manley, Kathy, Marder, Victor J., Markmann, James F., Martinez, Mercedes, Matesanz, Rafael, McCoy, Tara, McDiarmid, Suzanne V., McKenna, Greg J., Michaels, Marian G., Minervini, Marta I., Mobley, Constance, Moon, Deok-Bog, Moreno, Elisa A., Mühlbacher, Ferdinand, Muiesan, Paolo, Murase, Noriko, Naini, Bita V., Nalesnik, Michael A., Nathan, Jaimie D., Neuhaus, Peter, Nieto, Jose M., Nwadei, Ifeoma, O’Grady, John, O’Leary, Jacqueline G., Olthoff, Kim M., Onaca, Nicholas, Parekh, Justin, Parke, Chong, Pascher, Andreas, Persijn, Guido G., Petrowsky, Henrik, Pham, Phuong-Chi T., Pham, Phuong-Thu T., Platt, Jeffrey L., Pomfret, Elizabeth A., Porrett, Paige M., Raja Rajalingam, Rakela, Jorge, Raman, Steven S., Ramsay, Michael A.E., Randhawa, Parmjeet, Redfield, Robert R., III, Reed, Alan, Reed, Elaine F., Reich, David J., Renz, John F., Restrepo, Lucas, Roberts, John P., Roche, Bruno, Rockenschaub, Susanne Rasoul, Ross, Lainie Friedman, Ruiz, Richard, Ryckman, Frederick C., Saab, Sammy, Sai, Victor, Saliba, Faouzi, Sampaio, Luiz C., Samuel, Didier, Sano, Keiji, Sasatomi, Eizaburo, Sassi, Kareem, Saunders, Milda R., Schnickel, Gabriel T., Seetharam, Anil, Setoyama, Kentaro, Shaik, Imtiazuddin, Shaked, Abraham, Shirabe, Ken, Singal, Ashwani K., Soejima, Yuji, Starzl, Thomas E., Steadman, Randolph H., Stewart, Zoe, Stone, Marvin J., Strouse, Thomas B., Sturdevant, Mark L., Sugawara, Yasuhiko, Superina, Riccardo A., Taketomi, Akinobu, Talwalkar, Jayant A., Tanaka, Koichi, Tap, William D., Taylor, Doris A., Tiao, Greg, Tong, Myron J., Trotter, James F., Uchiyama, Hideaki, Vagefi, Parsia, Vargas, Hugo E., Venick, Robert S., Vilca-Melendez, Hector, Vincenti, Flavio, Wadei, Hani M., Washburn, Kenneth, Whitington, Peter F., Winston, Drew J., Wojciechowski, David, Wong, Deborah J.L., Yeh, Heidi, Yersiz, Hasan, Yoshizumi, Tomoharu, Zarrinpar, Ali, Zhai, Yuan, Zhang, Qiuheng, and Zimmerman, Michael A.

Published
2015
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128. Differences in presentation and progression between severe FIC1 and BSEP deficiencies

Author

Pawlikowska, Ludmila, primary, Strautnieks, Sandra, additional, Jankowska, Irena, additional, Czubkowski, Piotr, additional, Emerick, Karan, additional, Antoniou, Anthony, additional, Wanty, Catherine, additional, Fischler, Bjorn, additional, Jacquemin, Emmanuel, additional, Wali, Sami, additional, Blanchard, Samra, additional, Nielsen, Inge-Merete, additional, Bourke, Billy, additional, McQuaid, Shirley, additional, Lacaille, Florence, additional, Byrne, Jane A., additional, van Eerde, Albertien M., additional, Kolho, Kaija-Leena, additional, Klomp, Leo, additional, Houwen, Roderick, additional, Bacchetti, Peter, additional, Lobritto, Steven, additional, Hupertz, Vera, additional, McClean, Patricia, additional, Mieli-Vergani, Giorgina, additional, Shneider, Benjamin, additional, Nemeth, Antal, additional, Sokal, Etienne, additional, Freimer, Nelson B., additional, Knisely, A.S., additional, Rosenthal, Philip, additional, Whitington, Peter F., additional, Pawlowska, Joanna, additional, Thompson, Richard J., additional, and Bull, Laura N., additional

Published
2010
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131. Expression of CD20 but Not EBV Is Associated with Significantly Improved Overall (OS) and Event Free Survival (EFS) in Children with Post Transplant Lymphoproliferative Disease (PTLD) Following Solid Organ Transplantation (SOT).

Author

Orjuela, Manuela A., primary, Alobeid, Bachir, primary, Liu, Xinhua, primary, Siebert, Aisha L., primary, Kott, Emily R., primary, Addonizio, Linda J., primary, Morris, Erin, primary, Garvin, James H., primary, Lobritto, Steven J., primary, and Cairo, Mitchell S., primary

Published
2008
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138. Durability of Response in Children Treated With Pegylated Interferon alfa-2a?±?Ribavirin for Chronic Hepatitis C

Author

Schwarz, Kathleen B., Molleston, Jean P., Jonas, Maureen M., Wen, Jessica, Murray, Karen F., Rosenthal, Philip, Gonzalez-Peralta, Regino P., Lobritto, Steven J., Mogul, Douglas, Pavlovic, Vedran, Warne, Charles, Wat, Cynthia, and Thompson, Bruce

Abstract

No long-term data have been published on the durability of response following pegylated interferon (PegIFN) treatment in children with chronic hepatitis C. This prospective, multicenter, long-term follow-up (LTFU) study aimed to assess long-term durability of sustained virological response (SVR), long-term safety and tolerability, and the association between IL28Bgenotype and treatment response, in children previously treated with PegIFN alfa-2a?±?ribavirin (RBV) in the PEDS-C trial. A total of 93 patients were assessed for enrollment, and 38 enrolled in the study. Patients attended 2 study visits: 5 (mean 5.6, range 4.1–6.6) and 6 (6.6, 5.1–7.7) years after treatment cessation. Standardized medical history, physical examination, and laboratory testing were performed at these visits. Reminder telephone calls were conducted at 4 and 8 months after the initial visit. The LTFU cohort was the representative of the original PEDS-C cohort because both baseline and treatment characteristics were comparable. Of the 38 participants, 21 achieved SVR (responders) during the PEDS-C trial and 17 had not (nonresponders). All 21 responders maintained undetectable hepatitis C virus RNA during the LTFU (4.4–7.0 years after achieving SVR) in contrast to the nonresponders who demonstrated persistent viremia. IL28BCC genotype was associated with SVR (67% vs 30% in non-CC, P?=?0.028). Long-term durability of SVR is excellent following PegIFN alfa-2a treatment in children with chronic hepatitis C; SVR is higher in those with IL28BCC versus non-CC.

Published
2016
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141. 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, and Romero, Rene

Published
2013
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143. Pegylated interferon for chronic hepatitis C in children affects growth and body composition: Results from the pediatric study of hepatitis C (PEDS-C) trial.

Author

Jonas, Maureen M., Balistreri, William, Gonzalez-Peralta, Regino P., Haber, Barbara, Lobritto, Steven, Mohan, Parvathi, Molleston, Jean P., Murray, Karen F., Narkewicz, Michael R., Rosenthal, Philip, Schwarz, Kathleen B., Barton, Bruce A., Shepherd, John A., Mitchell, Paul D., and Duggan, Christopher

Published
2012
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144. The use of once‐daily LCP‐Tacrolimus with adolescent and young adult solid organ transplant recipients.

Author

Householder, Sarah, Ramakrishnan, Adarsh, Chen, Justin K., Gorsch, Lindsey, Tsapepas, Demetra, Lobritto, Steven, Rundle, Anna, and Vittorio, Jennifer M.

Subjects
*YOUNG adults, *TRANSPLANTATION of organs, tissues, etc., *PATIENT compliance, *TRANSITIONAL care, *TEENAGERS, *KIDNEY transplantation, *PROCUREMENT of organs, tissues, etc., *ALLOCATION of organs, tissues, etc.
Abstract

Background: Adolescent and young adult (AYA) solid organ transplant (SOT) recipients experience increased rates of rejection and graft loss surrounding the time of health care transition, in part due to poor medication adherence. This study aims to examine the impact of a once‐daily formulation of tacrolimus, LCP‐tacrolimus (LCPT), on medication adherence for AYA SOT patients. Methods: A retrospective descriptive analysis was performed for all patients who underwent SOT and were prescribed LCPT after the age of 12 at our single‐center pediatric hospital. Medication adherence was assessed via provider documentation and the medication level variability index (MLVI). Results: Twenty‐nine patients were prescribed LCPT as part of their immunosuppression regimen. Twenty patients were converted to LCPT from immediate‐acting (IR) tacrolimus; six patients were initiated immediately following transplant, and three patients were unable to receive LCPT due to insurance denial. There was a numeric improvement in medication adherence for converted patients when measured by provider assessment (45.0% vs. 68.4%, p =.140) and MLVI (40.0% vs. 71.4%, p =.276), though these did not reach statistical significance. There were no differences in episodes of rejection or adverse effects. LCPT prescription was not associated with decreased medication burden, and two patients transitioned back to IR tacrolimus due to increased cost. Conclusions: LCPT use did not significantly improve patient adherence; however, it resulted in numerically higher perceived and measured adherence rates. LCPT appears to be safe and effective in the management of SOT recipients; however, it may not affect pill burden and may result in a higher financial burden. Use may be considered for a select group of AYA SOT recipients. [ABSTRACT FROM AUTHOR]

Published
2024
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145. Evidence of Chronic Allograft Injury in Liver Biopsies From Long-term Pediatric Recipients of Liver Transplants.

Author

Feng, Sandy, Bucuvalas, John C., Demetris, Anthony J., Burrell, Bryna E., Spain, Katherine M., Kanaparthi, Sai, Magee, John C., Ikle, David, Lesniak, Andrew, Lozano, Juan J., Alonso, Estella M., Bray, Robert A., Bridges, Nancy E., Doo, Edward, Gebel, Howard M., Gupta, Nitika A., Himes, Ryan W., Jackson, Annette M., Lobritto, Steven J., and Mazariegos, George V.

Abstract

Background & Aims A substantial proportion of pediatric liver transplant recipients develop subclinical chronic allograft injury. We studied whether there are distinct patterns of injury based on histopathologic features and identified associated immunologic profiles. Methods We conducted a cross-sectional study of 157 stable, long-term pediatric recipients of transplanted livers (70 boys; > 6 years old at time of transplantation; mean, 8.9 ± 3.46 years after liver transplantation) who underwent liver biopsy analysis from August 13, 2012, through May 1, 2014. Participants had received livers from a living or deceased donor and had consistently normal results from liver tests. Liver biopsy specimens were scored by a central pathologist; an unsupervised hierarchical cluster analysis of histologic features was used to sort biopsy samples into 3 clusters. We conducted transcriptional and cytometric analyses of liver tissue samples and performed a systems biology analysis that incorporated clinical, serologic, histologic, and transcriptional data. Results The mean level of alanine aminotransferase in participants was 27.6 ± 14.57 U/L, and the mean level of γ-glutamyl transferase was 17.4 ± 7.93 U/L. Cluster 1 was characterized by interface activity (n = 34), cluster 2 was characterized by periportal or perivenular fibrosis without interface activity (n = 45), and cluster 3 had neither feature (n = 78). We identified a module of genes whose expression correlated with levels of alanine aminotransferase, class II donor-specific antibody, portal inflammation, interface activity, perivenular inflammation, portal and perivenular fibrosis, and cluster assignment. The module was enriched in genes that regulate T-cell–mediated rejection (TCMR) of liver and other transplanted organs. Functional pathway analysis showed overrepresentation of TCMR gene sets for cluster 1 but not clusters 2 or 3. Conclusion In an analysis of biopsies from an apparently homogeneous group of stable, long-term pediatric liver transplant recipients with consistently normal liver test results, we found evidence of chronic graft injury (inflammation and/or fibrosis). Biopsy samples with interface activity had a gene expression pattern associated with TCMR. Graphical abstract [ABSTRACT FROM AUTHOR]

Published
2018
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146. PHARMACOKINETICS OF GLECAPREVIR/PIBRENTASVIR IN CHILDREN WITH CHRONIC HCV INFECTION: INTERIM ANALYSIS OF PART 2 OF THE DORA STUDY

Author

Jonas, Maureen M., Lon, Hoi Kei, Rhee, Susan, Gilmour, Susan M., Gonzalez-Peralta, Regino P., Leung, Daniel H., Ling, Simon C., Lobritto, Steven, Narkewicz, Michael R., Vishakha Sabharwal, Del Valle-Segarra, Antonio, Wen, Jessica W., Lovell, Sandra S., Porcalla, Ariel, Tripathi, Rakesh, Sokal, Etienne M., UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, and UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique

150. Risk Factors for 30-Day Unplanned Readmission After Hepatectomy: Analysis of 438 Pediatric Patients from the ACS-NSQIP-P Database.

Author

Kang, Elise, Shin, John Inho, Griesemer, Adam D., Lobritto, Steven, Goldner, Dana, Vittorio, Jennifer M., Stylianos, Steven, and Martinez, Mercedes

Subjects
*PATIENT readmissions, *CHILD patients, *SURGICAL site infections, *ESOPHAGEAL cancer, *HEPATECTOMY, *PARENTERAL feeding, *INTESTINAL diseases
Abstract

Background: Hepatic resections are uncommon in children. Most studies reporting complications of these procedures and risk factors associated with unplanned readmissions are limited to retrospective data from single centers. We investigated risk factors for 30-day unplanned readmission after hepatectomy in children using the American College of Surgeons National Surgical Quality Improvement-Pediatric database. Methods: The database was queried for patients aged 0–18 years who underwent hepatectomy for the treatment of liver lesions from 2012 to 2018. Chi-squared tests were performed to evaluate for potential risk factors for unplanned readmissions. A multivariate regression analysis was performed to identify independent predictors for unplanned 30-day readmissions. Results: Among 438 children undergoing hepatectomy, 64 (14.6%) had unplanned readmissions. The median age of the hepatectomy cohort was 1 year (0–17); 55.5% were male. Patients readmitted had significantly higher rates of esophageal/gastric/intestinal disease (26.56% vs. 14.97%; p=0.022), current cancer (85.94% vs. 75.67%; p=0.012), and enteral and parenteral nutritional support (31.25% vs. 17.65%; p=0.011). Readmitted patients had significantly higher rates of perioperative blood transfusion (67.19% vs. 52.41%; p=0.028), organ/space surgical site infection (10.94% vs. 1.07%; p<.001), sepsis (15.63% vs. 3.74%; p<.001), and total parenteral nutrition at discharge (9.09% vs. 2.66%; p=0.041). Organ/space surgical site infection was an independent risk factor for unplanned readmission (OR=9.598, CI [2.070–44.513], p=0.004) by multivariable analysis. Conclusion: Unplanned readmissions after liver resection are frequent in pediatric patients. Organ/space surgical site infections may identify patients at increased risk for unplanned readmission. Strategies to reduce these complications may decrease morbidity and costs associated with unplanned readmissions. [ABSTRACT FROM AUTHOR]

Published
2021
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