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Quaglia A, Roberts EA, Torbenson M. Developmental and Inherited Liver Disease. MACSWEEN'S PATHOLOGY OF THE LIVER 2024:122-294. [DOI: 10.1016/b978-0-7020-8228-3.00003-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/02/2025]
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Ishikawa Y, Saito R, Murakami K, Fujio A, Miyazawa K, Sasaki K, Matsumura M, Mitsugashira H, Degawa K, Kobayashi Y, Muto R, Tokodai K, Furukawa T, Unno M, Kamei T. Small Intestinal Adenocarcinoma Arising at the Anastomotic Site after Kasai Operation for Biliary Atresia: A Case Report and Literature Review. TOHOKU J EXP MED 2023; 261:267-272. [PMID: 37766552 DOI: 10.1620/tjem.2023.j080] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/29/2023]
Abstract
Biliary atresia is an obliterative cholangiopathy of unknown etiology. Hepatic portoenterostomy, in which obliterated extrahepatic bile ducts are resected and bile flow is restored, known as Kasai operation, is performed within 3 months after birth. While this operation enhances long-term survival of patients, the occurrence of primary malignant hepatic tumors has been increasing. We report a case of small intestinal adenocarcinoma arising at the anastomotic site after Kasai operation. A 49-year-old man, who underwent Kasai operation for biliary atresia when he was 2 months old, experienced rapidly progressive jaundice and liver dysfunction. Deceased-donor liver transplantation was performed for liver failure. Macroscopically, there was a white-yellow tumor located at the anastomotic site of hepatic portoenterostomy of the resected liver. Pathological examination revealed a well-differentiated adenocarcinoma with some Paneth cells in the neoplastic lesion. Immunohistochemically, the tumor cells were negative for cytokeratin 7 (CK7) but positive for cytokeratin 20 (CK20) and a homeobox domain-containing transcription factor (CDX2). Mucin expression in tumor cells was negative for mucin 1 (MUC1) and mucin 6 (MUC6) and positive for mucin 2 (MUC2) and mucin 5AC (MUC5AC). The pathological diagnosis was small intestinal adenocarcinoma originating from the jejunum. The patient was discharged 48 days after the operation. The patient had not experienced recurrence at 10 months after the operation. This is the first report of small intestinal adenocarcinoma arising at the anastomotic site after Kasai operation for biliary atresia. Special care should be taken for the patients after Kasai operation with acute progressive jaundice and liver dysfunction because there is a possibility of malignancy in their native liver.
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Affiliation(s)
- Yuki Ishikawa
- Department of Surgery, Tohoku University Graduate School of Medicine
| | - Ryusuke Saito
- Department of Surgery, Tohoku University Graduate School of Medicine
| | - Keigo Murakami
- Department of Investigative Pathology, Tohoku University Graduate School of Medicine
| | - Atsushi Fujio
- Department of Surgery, Tohoku University Graduate School of Medicine
| | - Koji Miyazawa
- Department of Surgery, Tohoku University Graduate School of Medicine
| | - Kengo Sasaki
- Department of Surgery, Tohoku University Graduate School of Medicine
| | | | | | - Kazuki Degawa
- Department of Surgery, Tohoku University Graduate School of Medicine
| | | | - Ryo Muto
- Department of Surgery, National Hospital Organization Mito Medical Center
| | - Kazuaki Tokodai
- Department of Surgery, Tohoku University Graduate School of Medicine
| | - Toru Furukawa
- Department of Investigative Pathology, Tohoku University Graduate School of Medicine
| | - Michiaki Unno
- Department of Surgery, Tohoku University Graduate School of Medicine
| | - Takashi Kamei
- Department of Surgery, Tohoku University Graduate School of Medicine
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Sadiq J, Lloyd C, Hodson J, Trapero Marugan M, Ferguson J, Sharif K, Mirza DF, Hirschfield G, Kelly D. Long-term clinical and socioeconomic outcomes of children with biliary atresia. JGH Open 2023; 7:841-847. [PMID: 38162865 PMCID: PMC10757476 DOI: 10.1002/jgh3.12980] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/27/2022] [Revised: 08/28/2023] [Accepted: 10/02/2023] [Indexed: 01/03/2024]
Abstract
Background Biliary atresia (BA) is rare liver disease of unknown etiology, and is a major indication for liver transplant (LT). Previous data indicate improved outcomes with early referral for Kasai portoenterostomy (KPE). Objective Evaluate the long-term outcomes in BA, with particular focus on those transitioned to adult care with native livers. Subjects and Methods Patients with BA treated between1980 and 2012 were identified. Data were collected from the time of referral, transition to adult care, and the most recent clinic notes, from which patient and native liver survival were calculated. Results Four hundred and fifty-four patients with BA were identified, who were followed up for median of 16.4 years from birth; 74 died (41 of whom had a LT), giving a 20-year survival rate of 83.6%. Two hundred and seventy-two patients received an LT, with the median native liver survival being 35 months. Of patients who transitioned to adult care, 54 of 180 (30.0%) retained their native liver. Of these, 72% (39 of 54) had evidence of chronic liver disease at transition, of whom 8 were subsequently lost to follow-up, 9 were transplanted, and 22 remained stable with compensated liver disease. Of the 15 of 54 patients (28%) with no evidence of chronic disease in their native liver disease at transition, 3 were subsequently lost to follow-up; none received transplants, although 3 patients developed new-onset liver disease. All patients transitioned to adult care completed secondary school education (N = 180), with 49% having attended college/university and 87% being in employment or education at the last follow-up. Of female patients, 34% had at least one pregnancy (27 children in 21 women), while 22% of males had fathered a child. Conclusion Long-term outcomes in BA are good, with patients surviving into adult life. Progression of chronic liver disease and associated morbidity is common in those who retained their native livers, suggesting that these patients require monitoring of liver disease throughout adult life, and early recognition of the need for LT.
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Affiliation(s)
- Javaid Sadiq
- Birmingham Women's & Children's Hospital& University Hospital BirminghamBirminghamUK
| | - Carla Lloyd
- Birmingham Women's & Children's Hospital& University Hospital BirminghamBirminghamUK
| | - James Hodson
- Institute of Translational MedicineUniversity Hospitals Birmingham NHS Foundation TrustBirminghamUK
- Research Development and InnovationUniversity Hospitals Birmingham NHS Foundation TrustBirminghamUK
| | - Maria Trapero Marugan
- Centre for Liver ResearchNIHR Biomedical Research Unit, University of BirminghamBirminghamUK
| | - James Ferguson
- Centre for Liver ResearchNIHR Biomedical Research Unit, University of BirminghamBirminghamUK
| | - Khalid Sharif
- Birmingham Women's & Children's Hospital& University Hospital BirminghamBirminghamUK
| | - Darius F Mirza
- Birmingham Women's & Children's Hospital& University Hospital BirminghamBirminghamUK
- Centre for Liver ResearchNIHR Biomedical Research Unit, University of BirminghamBirminghamUK
| | - Gideon Hirschfield
- Centre for Liver ResearchNIHR Biomedical Research Unit, University of BirminghamBirminghamUK
| | - Deirdre Kelly
- Birmingham Women's & Children's Hospital& University Hospital BirminghamBirminghamUK
- Centre for Liver ResearchNIHR Biomedical Research Unit, University of BirminghamBirminghamUK
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Herrmann J, Petit P, Grabhorn E, Lenz A, Jürgens J, Franchi-Albella S. Liver cirrhosis in children - the role of imaging in the diagnostic pathway. Pediatr Radiol 2023; 53:714-726. [PMID: 36040526 PMCID: PMC10027649 DOI: 10.1007/s00247-022-05480-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/16/2022] [Revised: 06/23/2022] [Accepted: 07/31/2022] [Indexed: 10/14/2022]
Abstract
Liver cirrhosis in children is a rare disease with multifactorial causes that are distinct from those in adults. Underlying reasons include cholestatic, viral, autoimmune, hereditary, metabolic and cardiac disorders. Early detection of fibrosis is important as clinical stabilization or even reversal of fibrosis can be achieved in some disorders with adequate treatment. This article focuses on the longitudinal evaluation of children with chronic liver disease with noninvasive imaging tools, which play an important role in detecting cirrhosis, defining underlying causes, grading fibrosis and monitoring patients during follow-up. Ultrasound is the primary imaging modality and it is used in a multiparametric fashion. Magnetic resonance imaging and computed tomography are usually applied second line for refined tissue characterization, clarification of nodular lesions and full delineation of abdominal vessels, including portosystemic communications.
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Affiliation(s)
- Jochen Herrmann
- Section of Pediatric Radiology, Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, 20251, Hamburg, Germany.
| | - Philippe Petit
- Aix Marseille Université, Hopital Timone-Enfants, Marseille, France
| | - Enke Grabhorn
- Department of Pediatric Gastroenterology and Hepatology, University Medical Center Hamburg, Hamburg, Germany
| | - Alexander Lenz
- Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center, Hamburg, Germany
| | - Julian Jürgens
- Section of Pediatric Radiology, Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, 20251, Hamburg, Germany
| | - Stéphanie Franchi-Albella
- Department of Pediatric Radiology, Hôpital Bicêtre, National Reference Centre for Rare Pediatric Liver Diseases, Paris, France
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Namgoong JM, Hwang S, Kim DY, Ahn CS, Kwon H, Ha S, Kim KM, Oh SH. Living donor liver transplantation in an infant patient with progressive familial intrahepatic cholestasis along with hepatocellular carcinoma: a case report. KOREAN JOURNAL OF TRANSPLANTATION 2022; 36:73-78. [PMID: 35769428 PMCID: PMC9235533 DOI: 10.4285/kjt.21.0007] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/23/2021] [Revised: 06/20/2021] [Accepted: 06/21/2021] [Indexed: 01/09/2023] Open
Abstract
Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive inherited disease requiring liver transplantation (LT). Hepatocellular carcinoma (HCC) is very rare in infants. We present a case of living donor LT using a left lateral section graft performed in a 7-month-old female infant diagnosed with PFIC type II and HCC. No mutation on ABCB11 gene was identified. Because of progressive deterioration of liver function, living donor LT with her mother's left lateral section graft was performed. Pretransplant serum alpha-fetoprotein (AFP) level was increased to 2,740 ng/mL, but HCC was not taken into account because of its rarity. The explant liver showed micronodular liver cirrhosis, multiple infantile hemangiomas and two HCCs of 0.7 cm and 0.3 cm in size. The patient recovered uneventfully from the LT operation. This patient has been regularly followed up with abdomen ultrasonography and AFP measurement every 6 months. The patient has been continually doing well for 8 years after the LT. In conclusion, LT is currently the only effective treatment for PFIC-associated end-stage liver diseases. HCC can develop at the cirrhotic liver of any cause, thus elevation of HCC tumor markers in pediatric patients is an important clue to perform further investigation before LT.
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Affiliation(s)
- Jung-Man Namgoong
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Shin Hwang
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea,Corresponding author: Shin Hwang Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea, Tel: +82-2-3010-3930, Fax: +82-2-3010-6701, E-mail:
| | - Dae-Yeon Kim
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Chul-Soo Ahn
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Hyunhee Kwon
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Suhyeon Ha
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Kyung Mo Kim
- Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Seak Hee Oh
- Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Calinescu AM, Rougemont AL, Anooshiravani M, Rock NM, McLin VA, Wildhaber BE. Features of Nodules in Explants of Children Undergoing Liver Transplantation for Biliary Atresia. J Clin Med 2022; 11:jcm11061578. [PMID: 35329903 PMCID: PMC8950310 DOI: 10.3390/jcm11061578] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/30/2022] [Revised: 03/10/2022] [Accepted: 03/11/2022] [Indexed: 12/07/2022] Open
Abstract
(1) Background: In patients with biliary atresia (BA) liver nodules can be identified either by pre-transplant imaging or on the explant. This study aimed to (i) analyze the histopathology of liver nodules, and (ii) to correlate histopathology with pretransplant radiological features. (2) Methods: Retrospective analysis of liver nodules in explants of BA patients transplanted in our center (2000−2021). Correlations with pretransplant radiological characteristics, patient age at liver transplantation (LT), time from Kasai hepatoportoenterostomy (KPE) to LT, age at KPE and draining KPE. (3) Results: Of the 63 BA-patients included in the analysis, 27/63 (43%) had nodules on explants. A majority were benign macroregenerative nodules. Premalignant (low-grade and high-grade dysplastic) and malignant (hepatocellular carcinoma) nodules were identified in 6/63 and 2/63 patients, respectively. On pretransplant imaging, only 13/63 (21%) patients had liver nodules, none meeting radiological criteria for malignancy. The occurrence of liver nodules correlated with patient age at LT (p < 0.001), time KPE-LT (p < 0.001) and draining KPE (p = 0.006). (4) Conclusion: In BA patients, pretransplant imaging did not correlate with the presence of liver nodules in explants. Liver nodules were frequent in explanted livers, whereby 25% of explants harboured malignant/pre-malignant nodules, emphasizing the need for careful surveillance in BA children whose clinical course may require LT.
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Affiliation(s)
- Ana M. Calinescu
- Swiss Pediatric Liver Center, Geneva University Hospitals, 1205 Geneva, Switzerland; (A.-L.R.); (M.A.); (N.M.R.); (V.A.M.); (B.E.W.)
- Division of Child and Adolescent Surgery, Department of Pediatrics, Gynecology, and Obstetrics, Geneva University Hospitals, University of Geneva, 1205 Geneva, Switzerland
- Correspondence: ; Tel.: +41-22-372-4662
| | - Anne-Laure Rougemont
- Swiss Pediatric Liver Center, Geneva University Hospitals, 1205 Geneva, Switzerland; (A.-L.R.); (M.A.); (N.M.R.); (V.A.M.); (B.E.W.)
- Division of Clinical Pathology, Diagnostic Department, Geneva University Hospitals, University of Geneva, 1205 Geneva, Switzerland
| | - Mehrak Anooshiravani
- Swiss Pediatric Liver Center, Geneva University Hospitals, 1205 Geneva, Switzerland; (A.-L.R.); (M.A.); (N.M.R.); (V.A.M.); (B.E.W.)
- Unit of Pediatric Radiology, Diagnostic Department, Geneva University Hospitals, University of Geneva, 1205 Geneva, Switzerland
| | - Nathalie M. Rock
- Swiss Pediatric Liver Center, Geneva University Hospitals, 1205 Geneva, Switzerland; (A.-L.R.); (M.A.); (N.M.R.); (V.A.M.); (B.E.W.)
- Gastroenterology, Hepatology and Nutrition Unit, Division of Pediatric Specialties, Department of Pediatrics, Gynecology, and Obstetrics, Geneva University Hospitals, University of Geneva, 1205 Geneva, Switzerland
| | - Valerie A. McLin
- Swiss Pediatric Liver Center, Geneva University Hospitals, 1205 Geneva, Switzerland; (A.-L.R.); (M.A.); (N.M.R.); (V.A.M.); (B.E.W.)
- Gastroenterology, Hepatology and Nutrition Unit, Division of Pediatric Specialties, Department of Pediatrics, Gynecology, and Obstetrics, Geneva University Hospitals, University of Geneva, 1205 Geneva, Switzerland
| | - Barbara E. Wildhaber
- Swiss Pediatric Liver Center, Geneva University Hospitals, 1205 Geneva, Switzerland; (A.-L.R.); (M.A.); (N.M.R.); (V.A.M.); (B.E.W.)
- Division of Child and Adolescent Surgery, Department of Pediatrics, Gynecology, and Obstetrics, Geneva University Hospitals, University of Geneva, 1205 Geneva, Switzerland
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Kakos CD, Ziogas IA, Demiri CD, Esagian SM, Economopoulos KP, Moris D, Tsoulfas G, Alexopoulos SP. Liver Transplantation for Pediatric Hepatocellular Carcinoma: A Systematic Review. Cancers (Basel) 2022; 14:1294. [PMID: 35267604 PMCID: PMC8908995 DOI: 10.3390/cancers14051294] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/23/2022] [Revised: 02/23/2022] [Accepted: 02/28/2022] [Indexed: 02/05/2023] Open
Abstract
Liver transplantation (LT) is the only potentially curative option for children with unresectable hepatocellular carcinoma (HCC). We performed a systematic review of the MEDLINE, Scopus, Cochrane Library, and Web of Science databases (end-of-search date: 31 July 2020). Our outcomes were overall survival (OS) and disease-free survival (DFS). We evaluated the effect of clinically relevant variables on outcomes using the Kaplan-Meier method and log-rank test. Sixty-seven studies reporting on 245 children undergoing LT for HCC were included. DFS data were available for 150 patients and the 1-, 3-, and 5-year DFS rates were 92.3%, 89.1%, and 84.5%, respectively. Sixty of the two hundred and thirty-eight patients (25.2%) died over a mean follow up of 46.8 ± 47.4 months. OS data were available for 222 patients and the 1-, 3-, and 5-year OS rates were 87.9%, 78.8%, and 74.3%, respectively. Although no difference was observed between children transplanted within vs. beyond Milan criteria (p = 0.15), superior OS was observed in children transplanted within vs. beyond UCSF criteria (p = 0.02). LT can yield favorable outcomes for pediatric HCC beyond Milan but not beyond UCSF criteria. Further research is required to determine appropriate LT selection criteria for pediatric HCC.
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Affiliation(s)
- Christos D. Kakos
- Surgery Working Group, Society of Junior Doctors, 15123 Athens, Greece; (C.D.K.); (I.A.Z.); (C.D.D.); (S.M.E.); (K.P.E.)
| | - Ioannis A. Ziogas
- Surgery Working Group, Society of Junior Doctors, 15123 Athens, Greece; (C.D.K.); (I.A.Z.); (C.D.D.); (S.M.E.); (K.P.E.)
- Department of Surgery, Division of Hepatobiliary Surgery and Liver Transplantation, Vanderbilt University Medical Center, Nashville, TN 37232, USA
| | - Charikleia D. Demiri
- Surgery Working Group, Society of Junior Doctors, 15123 Athens, Greece; (C.D.K.); (I.A.Z.); (C.D.D.); (S.M.E.); (K.P.E.)
- 2nd Department of Pediatric Surgery, “Papageorgiou” General Hospital, Aristotle University School of Medicine, 54124 Thessaloniki, Greece
| | - Stepan M. Esagian
- Surgery Working Group, Society of Junior Doctors, 15123 Athens, Greece; (C.D.K.); (I.A.Z.); (C.D.D.); (S.M.E.); (K.P.E.)
| | - Konstantinos P. Economopoulos
- Surgery Working Group, Society of Junior Doctors, 15123 Athens, Greece; (C.D.K.); (I.A.Z.); (C.D.D.); (S.M.E.); (K.P.E.)
- Department of Surgery, Duke University Medical Center, Durham, NC 27710, USA;
| | - Dimitrios Moris
- Department of Surgery, Duke University Medical Center, Durham, NC 27710, USA;
| | - Georgios Tsoulfas
- Department of Surgery, Aristotle University School of Medicine, 54124 Thessaloniki, Greece;
| | - Sophoclis P. Alexopoulos
- Department of Surgery, Division of Hepatobiliary Surgery and Liver Transplantation, Vanderbilt University Medical Center, Nashville, TN 37232, USA
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Namgoong JM, Hwang S, Kwon H, Ha S, Kim KM, Oh SH, Hong SM. Liver transplantation in pediatric patients with progressive familial intrahepatic cholestasis: Single center experience of seven cases. Ann Hepatobiliary Pancreat Surg 2021; 26:69-75. [PMID: 34916336 PMCID: PMC8901976 DOI: 10.14701/ahbps.21-114] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/02/2021] [Revised: 09/24/2021] [Accepted: 09/27/2021] [Indexed: 11/17/2022] Open
Abstract
Backgrounds/Aims Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive inherited disease requiring liver transplantation (LT). The objective of this study was to investigate the clinicopathological features and posttransplant courses of seven LT recipients with PFIC. Methods This was a retrospective single-center study of patients with PFIC who underwent LT from January 2013 to June 2020. Results Two and five patients were diagnosed with PFIC type 1 and type 2, respectively. For all seven patients, age of PFIC onset was at birth. Jaundice was present in all cases. Mean pretransplant total and direct bilirubin levels were 16.1 ± 8.1 mg/dL and 12.4 ± 6.2 mg/dL, respectively. Median patient age and body weight at LT were 10 months and 7 kg, respectively. Types of donors were mothers of patients in four and deceased donors in three. All five patients with PFIC type 2 recovered uneventfully. One patient each with PFIC type 1 underwent retransplantation due to graft failure or died due to multi-organ failure. Overall graft and patient survival rates at five years were 66.7% and 83.3%, respectively. Bile salt export pump immunohistochemical staining showed normal canalicular expression in two patients with PFIC type 1, focal loss in two patients with PFIC type 2, and total loss in three patients with PFIC type 2. Conclusions LT is currently the only effective treatment for PFIC-associated end-stage liver diseases. It is mandatory to perform regular follow-up due to the risk of complications including steatohepatitis, especially for patients with PFIC type 1.
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Affiliation(s)
- Jung-Man Namgoong
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Shin Hwang
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Hyunhee Kwon
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Suhyeon Ha
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Kyung Mo Kim
- Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Seak Hee Oh
- Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Seung-Mo Hong
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Predisposing Conditions to Pediatric Hepatocellular Carcinoma and Association With Outcomes: Single-center Experience. J Pediatr Gastroenterol Nutr 2019; 68:695-699. [PMID: 30676520 DOI: 10.1097/mpg.0000000000002285] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/27/2023]
Abstract
OBJECTIVES Hepatocellular carcinoma (HCC) has been linked to chronic viral or metabolic liver disease and other conditions. The characteristics of children with HCC have not been fully elucidated and outcomes in children with predisposing liver disease are not well defined. METHODS Patients ≤21 years old with HCC managed at our institution and through external consultation between 1996 and 2016 were included. Demographics, clinical history, and pathology were tabulated. Fisher exact test and Wilcoxon test were employed for subgroup comparison, and survival differences were evaluated by Kaplan-Meier method. RESULTS Sixty-one cases of HCC were identified. Seven of 16 patients (44%) at our institution and 18 of 45 consult patients (40%) had a predisposing condition: cryptogenic cirrhosis/steatosis (9), genetic (7), biliary pathology (4), viral hepatitis (1), and other (4). Thirteen of 27 patients with de novo HCC had fibrolamellar HCC. Clinical characteristics were grouped by presence or absence of predisposing conditions: age at diagnosis (7.2 vs 10.2 years, P < 0.05), metastatic disease at presentation (15% vs 44%, P = n.s), and tumor size >4 cm (20% vs 100%, P < 0.05). In patients treated at our institution, 5 of 7 with predisposing conditions received liver transplant and achieved complete remission, whereas only 3 of 9 patients with de novo HCC received curative surgery and this group had decreased median overall survival (P < 0.05). CONCLUSIONS The majority of children with HCC did not have predisposing liver or associated disease. These patients were diagnosed later with more advanced stage disease and had significantly decreased overall survival.
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Quaglia A, Roberts EA, Torbenson M. Developmental and Inherited Liver Disease. MACSWEEN'S PATHOLOGY OF THE LIVER 2018:111-274. [DOI: 10.1016/b978-0-7020-6697-9.00003-0] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/02/2025]
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Torres-Landa S, Muñoz-Abraham AS, Fortune BE, Gurung A, Pollak J, Emre SH, Rodriguez-Davalos MI, Schilsky ML. De-novo hepatocellular carcinoma after pediatric living donor liver transplantation. World J Hepatol 2017; 9:1361-1366. [PMID: 29359020 PMCID: PMC5756726 DOI: 10.4254/wjh.v9.i36.1361] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/25/2017] [Revised: 11/22/2017] [Accepted: 12/07/2017] [Indexed: 02/06/2023] Open
Abstract
De-novo malignancies carry an incidence ranging between 3%-26% after transplant and account for the second highest cause of post-transplant mortality behind cardiovascular disease. While the majority of de-novo malignancies after transplant usually consist of skin cancers, there has been an increasing rate of solid tumor cancers over the last 15 years. Although, recurrence of hepatocellular carcinoma (HCC) is well understood among patients transplanted for HCC, there are increasing reports of de-novo HCC in those transplanted for a non-HCC indication. The proposed pathophysiology for these cases has been mainly connected to the presence of advanced graft fibrosis or cirrhosis and always associated with the presence of hepatitis B or C virus. We report the first known case of de-novo HCC in a recipient, 14 years after a pediatric living related donor liver transplantation for end-stage liver disease due to biliary atresia without the presence of hepatitis B or C virus before and after transplant. We present this case report to increase the awareness of this phenomenon and address on the utility for screening and surveillance of hepatocellular carcinoma among these individuals. One recommendation is to use similar guidelines for screening, diagnosis, and treatment for HCC as those used for primary HCC in the pre-transplant patient, focusing on those recipients who have advanced fibrosis in the allograft, regardless of etiology.
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Affiliation(s)
- Samuel Torres-Landa
- Department of Gastrointestinal Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA 19104, United States
| | | | - Brett E Fortune
- Division of Gastroenterology and Hepatology, Weill Cornell Medicine, New York, NY 10021, United States
| | - Ananta Gurung
- Department of Pathology, Royal Columbian Hospital, New Westminster, British Columbia V3L 3W7, Canada
| | - Jeffrey Pollak
- Department of Radiology and Biomedical Imaging, Yale University School of Medicine, New Haven, CT 06510, United States
| | - Sukru H Emre
- Department of Surgery, Yale University School of Medicine, New Haven, CT 06510, United States
| | | | - Michael L Schilsky
- Division of Digestive Diseases and Transplant and Immunology, Department of Medicine and Surgery, Yale University School of Medicine, New Haven, CT 06510, United States
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Abstract
Biliary atresia (BA) is the major cause of cholestasis and the leading indication for liver transplantation (LT). However, the incidence of BA in Korea has not been reported. The aim of this study was to investigate the incidence and clinical outcomes of BA in Korea. We used the Korean universal health insurance database and extracted data regarding BA patients younger than 18 years of age admitted between 2011 and 2015. The incidence of BA was calculated by dividing the number of BA patients by the number of live births. Two hundred forty infants were newly diagnosed with BA. A total of 963 BA patients younger than 18 years of age were followed up for 5 years. The overall incidence of BA was 1.06 cases per 10,000 live births. The incidence of BA was 1.4 times higher for female patients than for male patients. Additionally, significant seasonal variation was observed; in particular, the incidence of BA was 2 times higher from June through August than from December through February. Congenital anomalies were found in 38 of 240 patients (15.8%). Congenital heart diseases were major associated congenital anomalies (6.3%). Several complications developed during the study period, including cholangitis (24.0%), varix (6.2%), and gastrointestinal bleeding (4.4%). Three hundred and one of the 963 BA patients under 18 years of age (31.3%) received LT for BA. The incidence of BA is higher in Korea than that in Western countries. We also report significant gender-associated differences and seasonal variation with respect to the incidence of BA.
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Affiliation(s)
- Kyung Jae Lee
- Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
| | - Ju Whi Kim
- Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
| | - Jin Soo Moon
- Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
| | - Jae Sung Ko
- Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.
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13
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Sundaram SS, Mack CL, Feldman AG, Sokol RJ. Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care. Liver Transpl 2017; 23:96-109. [PMID: 27650268 PMCID: PMC5177506 DOI: 10.1002/lt.24640] [Citation(s) in RCA: 160] [Impact Index Per Article: 20.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/09/2016] [Accepted: 09/10/2016] [Indexed: 12/12/2022]
Abstract
Biliary atresia (BA) is a progressive, fibro-obliterative disorder of the intrahepatic and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation (LT). Indications for LT in BA include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extrahepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for LT. Optimal pretransplant management of these potentially life-threatening complications and maximizing nutrition and growth require the expertise of a multidisciplinary team with experience caring for BA. The timing of transplant for BA requires careful consideration of the potential risk of transplant versus the survival benefit at any given stage of disease. Children with BA often experience long wait times for transplant unless exception points are granted to reflect severity of disease. Family preparedness for this arduous process is therefore critical. Liver Transplantation 23:96-109 2017 AASLD.
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Affiliation(s)
- Shikha S. Sundaram
- Digestive Health Institute and Pediatric Liver Center, Children’s Hospital Colorado, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO
| | - Cara L. Mack
- Digestive Health Institute and Pediatric Liver Center, Children’s Hospital Colorado, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO
| | - Amy G. Feldman
- Digestive Health Institute and Pediatric Liver Center, Children’s Hospital Colorado, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO
| | - Ronald J. Sokol
- Digestive Health Institute and Pediatric Liver Center, Children’s Hospital Colorado, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO
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14
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Samuk I, Tekin A, Tryphonopoulos P, Pinto IG, Garcia J, Weppler D, Levi DM, Nishida S, Selvaggi G, Ruiz P, Tzakis AG, Vianna R. Abdominal transplantation for unresectable tumors in children: the zooming out principle. Pediatr Surg Int 2016; 32:337-46. [PMID: 26711121 DOI: 10.1007/s00383-015-3852-3] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 12/15/2015] [Indexed: 12/15/2022]
Abstract
PURPOSE To present our experience in abdominal transplantations to manage unresectable abdominal neoplasms in children and to describe the role of extensive surgeries in such cases. METHODS This is a retrospective study of 22 abdominal transplantations in 21 patients for abdominal tumors over 16 years. Transplantation techniques included liver transplant (LT), multivisceral transplant (MVTx), and intestinal autotransplant (IA). Follow-up intervals ranged from 0.3 to 168 months (median 20 months). RESULTS LT alone was performed in 15 patients for primary malignant (11) and benign (4) liver tumors. Pathological classification included HB hepatoblastoma (6), HCC hepatocellular cancer (3), hepatic epithelioid hemangioendothelioma HEH (1), angiosarcoma (1), benign vascular tumors (3), and adenoma (1). IA was performed in four patients for lesions involving the root of the mesentery; tumors of the head of pancreas (3) and mesenteric hemangioma (1). MVTx was performed in 2 patients for malignancies; pancreaticoblastoma (1), recurrent hepatoblastoma (1), and in one patient as a rescue procedure after IA failure. Four of the eleven patients who underwent LT for malignant liver tumor had metastatic disease at presentation. Six of them died of recurrent neoplasm (3), transplant-related complications (2), and underlying disease (1). All LT patients who had benign tumors are alive with functioning grafts. All IA patients survived and are on an oral diet, with one patient requiring TPN supplementation. One of the three patients who underwent MVTx died of metastatic disease. CONCLUSIONS Allo/auto transplantation for abdominal tumors is a valuable modality when conventional treatments fail or are not feasible.
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Affiliation(s)
- Inbal Samuk
- Pediatric and Adolescent Surgery, Schneider Children's Medical Center, Sackler School of Medicine, Tel Aviv University, 14 kaplan Street, Petach-Tikvha, 49202, Israel. .,Surgery, University of Miami Miller School of Medicine, Miami, FL, USA.
| | - Akin Tekin
- Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
| | | | - Ignacio G Pinto
- Surgery, University of Miami Miller School of Medicine, Miami, FL, USA.,Universidad de Oviedo, Beca, Spain
| | | | - Debbie Weppler
- Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
| | - David M Levi
- Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
| | - Seigo Nishida
- Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
| | - Gennaro Selvaggi
- Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
| | - Phillip Ruiz
- Surgery, University of Miami Miller School of Medicine, Miami, FL, USA.,Pathology, University of Miami, Miami, USA
| | - Andreas G Tzakis
- Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
| | - Rodrigo Vianna
- Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
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15
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Bessho K. Complications and Quality of Life in Long-Term Survivors of Biliary Atresia with Their Native Livers. J Pediatr 2015; 167:1202-6. [PMID: 26382628 DOI: 10.1016/j.jpeds.2015.08.041] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/14/2015] [Revised: 07/23/2015] [Accepted: 08/20/2015] [Indexed: 12/12/2022]
Affiliation(s)
- Kazuhiko Bessho
- Department of Pediatrics, Graduate School of Medicine, Osaka University, Suita-shi, Osaka, Japan.
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16
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Hepatocellular carcinoma in the native liver of a 38-year-old female patient with biliary atresia. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2015. [DOI: 10.1016/j.epsc.2015.09.016] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
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17
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Two Rare Cases of Hepatocellular Carcinoma after Kasai Procedure for Biliary Atresia: A Recommendation for Close Follow-Up. Case Rep Pathol 2015; 2015:982679. [PMID: 26339518 PMCID: PMC4539070 DOI: 10.1155/2015/982679] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/27/2015] [Revised: 07/19/2015] [Accepted: 07/21/2015] [Indexed: 01/30/2023] Open
Abstract
The instigation of the Kasai procedure in infants who are born with biliary atresia has led to increased survival in this population for over half a century. The many complications that arise as a result of biliary atresia led to an early death for most patients. However, the Kasai procedure is not without its own impediments. Among them is the development of hepatocellular carcinoma. We present two cases of hepatocellular carcinoma, after Kasai procedure, from two different age groups, as a recommendation that these patients should be even more closely monitored. Furthermore, if they are in need of transplant, we recommend that the explanted livers be carefully examined, as the tumor may not have been diagnosed preoperatively.
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18
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Abstract
Biliary atresia is a rare disease of unclear etiology, in which obstruction of the biliary tree causes severe cholestasis leading to cirrhosis and ultimately death if left untreated. Biliary atresia is the leading cause of neonatal cholestasis and the most frequent indication for pediatric liver transplantation. Any infant with persistent jaundice beyond 2 weeks of life needs to be evaluated for biliary atresia with fractionation of the bilirubin into conjugated and unconjugated portions. Early performance of a hepatoportoenterostomy in the first 45 days of life to restore bile flow and lessen further damage to the liver is thought to optimize outcome. Despite surgery, progressive liver scarring occurs, and 80% of patients with biliary atresia will require liver transplantation during childhood.
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19
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Squires RH, Ng V, Romero R, Ekong U, Hardikar W, Emre S, Mazariegos GV. Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline by the American Association for the Study of Liver Diseases, American Society of Transplantation and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Hepatology 2014; 60:362-98. [PMID: 24782219 DOI: 10.1002/hep.27191] [Citation(s) in RCA: 142] [Impact Index Per Article: 12.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/22/2014] [Accepted: 04/22/2014] [Indexed: 12/16/2022]
Affiliation(s)
- Robert H Squires
- Department of Pediatrics, University of Pittsburgh School of Medicine; Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA
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20
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Yoon H, Jeon T, Yoo SY, Kim J, Eo H, Lee SK, Kim J. Hepatic tumours in children with biliary atresia: Single-centre experience in 13 cases and review of the literature. Clin Radiol 2014; 69:e113-9. [DOI: 10.1016/j.crad.2013.10.017] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/11/2013] [Revised: 10/22/2013] [Accepted: 10/23/2013] [Indexed: 01/09/2023]
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21
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Morotti RA, Jain D. Pediatric Cholestatic Disorders: Approach to Pathologic Diagnosis. Surg Pathol Clin 2013; 6:205-225. [PMID: 26838972 DOI: 10.1016/j.path.2013.03.001] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/05/2023]
Abstract
This article addresses select liver diseases that are commonly seen in the pediatric group and pose diagnostic challenges in practice. The key genetic/molecular abnormalities, clinical features, histopathologic findings, diagnostic modalities, differential diagnoses, and possible pitfalls in diagnosis are discussed in detail. Although recent advances in understanding the pathophysiology of bile synthesis and transport along with advances in molecular genetics have allowed a better characterization of many of these liver diseases, significant overlap in the histopathologic features of many of these disorders still leads to diagnostic challenges for the pathologist.
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Affiliation(s)
- Raffaella A Morotti
- Department of Pathology, Yale University School of Medicine, 310 Cedar Street, PO Box 208023, New Haven, CT 06520-8023, USA.
| | - Dhanpat Jain
- Department of Pathology, Yale University School of Medicine, 310 Cedar Street, PO Box 208023, New Haven, CT 06520-8023, USA
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22
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Fukuda A, Sakamoto S, Kanazawa H, Shigeta T, Karaki C, Hamano I, Uchida H, Kitagawa H, Okuse C, Miyazaki O, Nosaka S, Nakazawa A, Kasahara M. Incidentally detected cholangiocarcinoma in an explanted liver with biliary atresia after Kasai operation. Pediatr Transplant 2013; 17:E62-6. [PMID: 23279592 DOI: 10.1111/petr.12036] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 11/14/2012] [Indexed: 12/11/2022]
Abstract
This report presents the case of a 30-yr-old woman with BA who developed incidental cholangiocarcinoma following the Kasai operation. She showed progressive liver dysfunction and cirrhosis at the age of 30 yr and underwent LDLT. A 4-cm-diameter liver tumor in the anastomotic site of portoenterostomy was incidentally found as a result of a pathological examination of the explanted native liver. The tumor was pathologically diagnosed to be intrahepatic cholangiocarcinoma. Although cholangiocarcinoma in patients with BA has been previously reported in only three cases, it should be nevertheless always considered in the differential diagnosis of hepatic tumors during a long follow-up course in patients with BA.
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Affiliation(s)
- Akinari Fukuda
- Division of Transplant Surgery, National Center for Child Health and Development, Setagaya-ku, Tokyo, Japan.
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23
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Wildhaber BE. Biliary atresia: 50 years after the first kasai. ISRN SURGERY 2012; 2012:132089. [PMID: 23304557 PMCID: PMC3523408 DOI: 10.5402/2012/132089] [Citation(s) in RCA: 71] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 09/23/2012] [Accepted: 10/30/2012] [Indexed: 12/20/2022]
Abstract
Biliary atresia is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. Biliary atresia is the most frequent surgical cause of cholestatic jaundice in neonates and should be evoked whenever this clinical sign is associated with pale stools and hepatomegaly. The treatment of biliary atresia is surgical and currently recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (a "Kasai," modifications of which are discussed in this paper) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated, for which biliary atresia represents the most frequent pediatric indication. Of importance, the earlier the Kasai is performed, the later a liver transplantation is usually needed. This warrants a great degree of awareness of biliary atresia, and the implementation of systematic screening for this life-threatening pathology.
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Affiliation(s)
- Barbara E Wildhaber
- Division of Pediatric Surgery, Department of Pediatrics, University Hospital of Geneva, 1211 Geneva, Switzerland
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24
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Orthotopic liver transplantation for biliary atresia complicated by incidental cholangiocarcinoma. J Pediatr Gastroenterol Nutr 2012; 55:336-7. [PMID: 21873891 DOI: 10.1097/mpg.0b013e318233ff17] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
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25
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Kim JM, Lee SK, Kwon CHD, Joh JW, Choe YH, Park CK. Hepatocellular carcinoma in an infant with biliary atresia younger than 1 year. J Pediatr Surg 2012; 47:819-21. [PMID: 22498405 DOI: 10.1016/j.jpedsurg.2012.01.020] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2011] [Revised: 12/23/2011] [Accepted: 01/18/2012] [Indexed: 02/02/2023]
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26
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Kumagi T, Drenth JPH, Guttman O, Ng V, Lilly L, Therapondos G, Hiasa Y, Michitaka K, Onji M, Watanabe Y, Sen S, Griffiths W, Roberts E, Heathcote J, Hirschfield GM. Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review. Liver Int 2012; 32:510-8. [PMID: 22098694 DOI: 10.1111/j.1478-3231.2011.02668.x] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/30/2011] [Accepted: 09/17/2011] [Indexed: 12/12/2022]
Abstract
BACKGROUND Biliary atresia is a progressive biliary injury which occurs only in infants. AIMS To review the experience of patients surviving into adulthood without the need for liver transplantation in childhood. METHODS A multicentre review of patients with biliary atresia treated surgically who survived into adulthood without the need for transplantation. RESULTS Twenty-two patients were identified across four centres. Median age at the last follow-up was 25 years (range: 18-46), and 21 patients had clinical features of portal hypertension. At last follow-up values of liver enzymes varied from normal to 15 × the upper limit of normal (ULN) for ALT (median 2.11 × ULN) and 9 × the ULN for ALP (median 2.02 × ULN). Six patients had a serum bilirubin > 50 μmol/l. Pruritus and jaundice were noted in 8 of 20 patients (40%) and 11 of 22 patients (50%) respectively. Thirteen patients (59.1%) were shown to have imaging features of sclerosing cholangitis, with strictures of intrahepatic bile duct(s) (IHBD), dilatation of IHBD (n = 8), or stone(s) within the IHBD (n = 5). A history of presumed bacterial cholangitis was present in 11 patients (50%). Successful pregnancies were recorded in three of fourteen female patients. Four patients underwent transplant between the ages of 20-27 years. Twenty-one patients (95.5%) were alive, including 18 (81.8%) with their native liver at the time of last follow-up. CONCLUSIONS Some patients treated for biliary atresia will survive into adulthood with their native liver, but commonly with secondary biliary disease including cholangitis and portal hypertension.
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27
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Hadžić N, Quaglia A, Portmann B, Paramalingam S, Heaton ND, Rela M, Mieli-Vergani G, Davenport M. Hepatocellular carcinoma in biliary atresia: King's College Hospital experience. J Pediatr 2011; 159:617-22.e1. [PMID: 21489554 DOI: 10.1016/j.jpeds.2011.03.004] [Citation(s) in RCA: 43] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/24/2010] [Revised: 02/01/2011] [Accepted: 03/02/2011] [Indexed: 02/06/2023]
Abstract
OBJECTIVES To establish risks for development of hepatocellular carcinoma (HCC) in children with biliary atresia (BA), the most common chronic liver disease of childhood. STUDY DESIGN In our tertiary referral center database we have identified children with BA who had development of or have been incidentally found to have HCC. Their demographic, clinical, radiologic, and histologic features were analyzed. RESULTS Between 1990 and 2008, 387 infants were diagnosed with BA at our center. Of these, three (0.8 %) who underwent operation at a median age of 68 (range 66 to 71) days had development of a histologically proven HCC detected at a median age of 2.1 (range 1.8 to 4.9) years. Another two, referred later, were diagnosed with HCC on their liver explants at ages 1.1 and 17.75 years, respectively. Overall, two had elevated serum levels of alpha-fetoprotein. All five children underwent successful liver transplantation at a median age of 2.1 years (range 1.1 to 17.75) and remain well after a median of 2.5 (range 2 to 5.7) years. CONCLUSION HCC develops in a small percentage of children with BA. Serum alpha-fetoprotein levels and ultrasound screening are helpful but not absolute markers of the malignant change. In the absence of the extrahepatic involvement, liver transplantation represents an effective treatment.
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Affiliation(s)
- Nedim Hadžić
- Paediatric Liver Centre, King's College Hospital, Denmark Hill, London, United Kingdom.
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28
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Paediatric chronic liver diseases: how to investigate and follow up? Role of imaging in the diagnosis of fibrosis. Pediatr Radiol 2010; 40:906-19. [PMID: 20432008 DOI: 10.1007/s00247-010-1600-3] [Citation(s) in RCA: 42] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/25/2010] [Accepted: 01/28/2010] [Indexed: 12/23/2022]
Abstract
Chronic liver diseases are rare in children, but encompass a wide spectrum of disorders that may all be complicated by liver fibrosis and therefore by portal hypertension. They may be classified according to the level of portal flow obstruction: prehepatic, intrahepatic or suprahepatic. Most of them, except presinusoidal diseases, may progress to cirrhosis that carries additional risks of impaired liver function and development of hepatocellular carcinoma. Imaging plays an important role in guiding the diagnosis and biopsy and for follow-up during treatment. US, with high-frequency transducers and Doppler, is the first modality of choice, directs the rest of the investigations and guides interventional radiology. MDCT has made great progress and has replaced angiography for diagnostic purposes. MRI is indicated for parenchyma and nodule characterization and for biliary tract evaluation. To avoid liver biopsy, several elasticity imaging techniques have been developed and have to be evaluated for accuracy and convenience in children. The role of each modality with main imaging findings is described in extrahepatic portal vein obstruction, hepatoportal sclerosis, congenital hepatic fibrosis, cirrhosis and Budd-Chiari syndrome.
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29
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Abstract
During the last decade, important progress has been made in the surgical treatment of malignant liver tumors in children. For hepatoblastoma, there is a general consensus for combining surgical resection with neoadjuvant (and adjuvant) chemotherapy. Long-term disease-free survival of around 85-90% can be achieved for resectable HB involving no more than three sections of the liver (PRETEXT I-III). For unresectable HB without extrahepatic invasion (PRETEXT IV with involvement of all four sections and some cases of PRETEXT III with invasion of, or close contact with major venous structures), similar results can be obtained with total hepatectomy and liver transplantation. For hepatocellular carcinoma, most often without underlying liver disease in children of the western world, results of resection with partial hepatectomy remain dismal, due to a high rate of recurrence. In contrast, remarkable survival rates have been obtained during the last decade with liver transplantation. There is no argument, either biological or based on evidence, that the selection of pediatric candidates for transplantation should be based on the same criteria as in adult patients (the Milan criteria). Optimization of results require to concentrate children with a malignant liver tumors in specialized, multidisciplinary pediatric centers with expertise in chemotherapy and in both major liver resections and transplantation. Enrolling these children in prospective trials should be encouraged, as well as prospective registration of transplanted patients in PLUTO (Pediatric Liver Unresectable Tumor Observatory-http://Pluto.cineca.org) in order to clarify issues unresolved by retrospective studies.
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30
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Iida T, Zendejas IR, Kayler LK, Magliocca JF, Kim RD, Hemming AW, Gonzalez-Peralta RP, Fujita S. Hepatocellular carcinoma in a 10-month-old biliary atresia child. Pediatr Transplant 2009; 13:1048-9. [PMID: 19032418 DOI: 10.1111/j.1399-3046.2008.01094.x] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
We present a case of a 10-month-old boy with BA who developed HCC and was treated with liver transplantation. A four-month-old boy was referred to our institution because of persistent jaundice, hepatomegaly, and coagulopathy. He had been treated for the diagnosis of neonatal hepatitis at an outside hospital. He was evaluated and was accepted as a liver transplant candidate, and was subsequently transplanted with a deceased donor liver allograft at the age of 10 months. His native liver showed established cirrhosis because of BA with one focus of moderately differentiated HCC, measuring 0.7 cm in a diameter with microscopic vascular invasion in pathological study. The postoperative course was uneventful, and he is well without recurrence four months after liver transplantation. The occurrence of HCC in a child under one yr old is extremely rare, and only three cases are reported so far including our case.
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Affiliation(s)
- Taku Iida
- Division of Transplantation and Hepatobiliary Surgery, Department of Surgery, College of Medicine, University of Florida, Gainesville, FL 32610-0286, USA
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31
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Liang JL, Cheng YF, Concejero AM, Huang TL, Chen TY, Tsang LLC, Ou HY. Macro-regenerative nodules in biliary atresia: CT/MRI findings and their pathological relations. World J Gastroenterol 2008; 14:4529-34. [PMID: 18680234 PMCID: PMC2731281 DOI: 10.3748/wjg.14.4529] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIM: To describe the radiological findings of a macro-regenerative nodule (MRN) in the liver of pre-transplantation biliary atresia (BA) patients and to correlate it with histological findings.
METHODS: Between August 1990 and November 2007, 144 BA patients underwent liver transplantation (LT) at our institution. The pre-transplantation computer tomography (CT) and magnetic resonance imaging (MRI) findings were reviewed and correlated with the post-transplantation pathological findings.
RESULTS: Nine tumor lesions in 7 patients were diagnosed in explanted livers. The post-transplantation pathological findings showed that all the lesions were MRNs without malignant features. No small nodule was detected by either MRI or CT. Of the 8 detectable lesions, 6 (75%) were in the central part of the liver, 5 (63%) were larger than 5 cm, 5 (63%) had intra-tumor tubular structures, 3 (38%) showed enhancing fibrous septa, 3 (38%) had arterial enhancement in CT, one (13%) showed enhancement in MRI, and one (13%) had internal calcifications.
CONCLUSION: Although varied in radiological appearance, MRN can be differentiated from hepatocellular carcinoma (HCC) in most of BA patients awaiting LT. The presence of an arterial-enhancing nodule does not imply that LT is withheld solely on the basis of presumed malignancy by imaging studies. Liver biopsy may be required in aid of diagnostic imaging to exclude malignancy.
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Abstract
Extrahepatic biliary atresia (EHBA), an inflammatory sclerosing cholangiopathy, is the leading indication for liver transplantation in children. The cause is still unknown, although possible infectious, genetic, and immunologic etiologies have received much recent focus. These theories are often dependent on each other for secondary or coexisting mechanisms. Concern for EHBA is raised by a cholestatic infant, but the differential diagnosis is large and the path to diagnosis remains varied. Current treatment is surgical with an overall survival rate of approximately 90%. The goals of this article are to review the important clinical aspects of EHBA and to highlight some of the more recent scientific and clinical developments contributing to our understanding of this condition.
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Affiliation(s)
- Mikelle D. Bassett
- Department of Pediatrics, Seattle Children’s Hospital, University of Washington School of Medicine, Seattle, WA
| | - Karen F. Murray
- Division of Gastroenterology and Nutrition, Seattle Children’s Hospital, University of Washington School of Medicine, Seattle, WA
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33
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Abstract
It is becoming increasingly evident that children, like adults, with chronic viral or metabolic liver diseases are at risk for the development of hepatocarcinoma. The aims of this article are to review the risk factors for hepatocarcinoma in chronic viral or metabolic liver disease, outline potential pathogenic mechanisms of hepatocarcinoma, and describe surveillance strategies, clinical evaluation, and management of hepatocarcinoma in children.
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34
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Affiliation(s)
- Benjamin L Shneider
- Department of Pediatrics, Thomas E. Starzl Transplantation Institute, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA.
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