Effect of Kasai Procedure on Hepatic Outcome in Alagille Syndrome
ABSTRACT Objectives: Alagille syndrome (AGS) frequently presents with neonatal jaundice and can mimic other causes of high γ‐glutamyl transpeptidase (GGT) cholestasis, most notably biliary atresia. As a result infants with AGS may undergo intraoperative cholangiogram and even Kasai procedure. The ai...
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Published in | Journal of pediatric gastroenterology and nutrition Vol. 51; no. 3; pp. 319 - 321 |
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Main Authors | , , , , , |
Format | Journal Article |
Language | English |
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Copyright by ESPGHAN and NASPGHAN
01.09.2010
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Abstract | ABSTRACT
Objectives:
Alagille syndrome (AGS) frequently presents with neonatal jaundice and can mimic other causes of high γ‐glutamyl transpeptidase (GGT) cholestasis, most notably biliary atresia. As a result infants with AGS may undergo intraoperative cholangiogram and even Kasai procedure. The aim of the study was to assess the hepatic outcomes of children with AGS who underwent the Kasai procedure.
Patients and Methods:
A retrospective review of the AGS clinical database at the Children's Hospital of Philadelphia was performed to identify clinically defined patients with AGS who underwent a Kasai. A cohort of Alagille control subjects was selected with equivalent symptoms of neonatal jaundice and matched for age and presence of cardiac anomaly. JAGGED1‐mutation analysis was performed on available samples. Clinical courses were reviewed. Fisher exact and t tests were used for analysis.
Results:
Of the 430 patients with AGS, 19 underwent a Kasai procedure (K). The control cohort (C) consisted of 36 patients. Total bilirubin measured between 6 and 10 weeks of age in each cohort was equivalent (K: 9.6 mg/dL, C: 8.7 mg/dL); GGT levels were higher in the control group (K:493.4 U/L, C:574.4 U/L). Of note, the Kasai cohort had a significantly larger number of liver transplants (K: 9 [47.3%], C: 5 [13.9%], P = 0.01) and sustained higher mortality (K: 6 [31.6%], C: 1 [2.8%], P = 0.005). There was no genotype‐phenotype correlation between the mutations identified and patients who underwent Kasai.
Conclusions:
These data suggest that the Kasai procedure, although appropriate for children with biliary atresia, does not benefit children with AGS and actually appears to worsen outcome. The current data suggest that the Kasai is not a marker for underlying severe liver disease, but the procedure itself may have a detrimental effect on outcome. An appropriate medical evaluation and particular consideration of AGS is essential before surgical referral in infants with high GGT cholestasis. |
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AbstractList | OBJECTIVESAlagille syndrome (AGS) frequently presents with neonatal jaundice and can mimic other causes of high gamma-glutamyl transpeptidase (GGT) cholestasis, most notably biliary atresia. As a result infants with AGS may undergo intraoperative cholangiogram and even Kasai procedure. The aim of the study was to assess the hepatic outcomes of children with AGS who underwent the Kasai procedure.PATIENTS AND METHODSA retrospective review of the AGS clinical database at the Children's Hospital of Philadelphia was performed to identify clinically defined patients with AGS who underwent a Kasai. A cohort of Alagille control subjects was selected with equivalent symptoms of neonatal jaundice and matched for age and presence of cardiac anomaly. JAGGED1-mutation analysis was performed on available samples. Clinical courses were reviewed. Fisher exact and t tests were used for analysis.RESULTSOf the 430 patients with AGS, 19 underwent a Kasai procedure (K). The control cohort (C) consisted of 36 patients. Total bilirubin measured between 6 and 10 weeks of age in each cohort was equivalent (K: 9.6 mg/dL, C: 8.7 mg/dL); GGT levels were higher in the control group (K:493.4 U/L, C:574.4 U/L). Of note, the Kasai cohort had a significantly larger number of liver transplants (K: 9 [47.3%], C: 5 [13.9%], P = 0.01) and sustained higher mortality (K: 6 [31.6%], C: 1 [2.8%], P = 0.005). There was no genotype-phenotype correlation between the mutations identified and patients who underwent Kasai.CONCLUSIONSThese data suggest that the Kasai procedure, although appropriate for children with biliary atresia, does not benefit children with AGS and actually appears to worsen outcome. The current data suggest that the Kasai is not a marker for underlying severe liver disease, but the procedure itself may have a detrimental effect on outcome. An appropriate medical evaluation and particular consideration of AGS is essential before surgical referral in infants with high GGT cholestasis. Alagille syndrome (AGS) frequently presents with neonatal jaundice and can mimic other causes of high gamma-glutamyl transpeptidase (GGT) cholestasis, most notably biliary atresia. As a result infants with AGS may undergo intraoperative cholangiogram and even Kasai procedure. The aim of the study was to assess the hepatic outcomes of children with AGS who underwent the Kasai procedure. A retrospective review of the AGS clinical database at the Children's Hospital of Philadelphia was performed to identify clinically defined patients with AGS who underwent a Kasai. A cohort of Alagille control subjects was selected with equivalent symptoms of neonatal jaundice and matched for age and presence of cardiac anomaly. JAGGED1-mutation analysis was performed on available samples. Clinical courses were reviewed. Fisher exact and t tests were used for analysis. Of the 430 patients with AGS, 19 underwent a Kasai procedure (K). The control cohort (C) consisted of 36 patients. Total bilirubin measured between 6 and 10 weeks of age in each cohort was equivalent (K: 9.6 mg/dL, C: 8.7 mg/dL); GGT levels were higher in the control group (K:493.4 U/L, C:574.4 U/L). Of note, the Kasai cohort had a significantly larger number of liver transplants (K: 9 [47.3%], C: 5 [13.9%], P = 0.01) and sustained higher mortality (K: 6 [31.6%], C: 1 [2.8%], P = 0.005). There was no genotype-phenotype correlation between the mutations identified and patients who underwent Kasai. These data suggest that the Kasai procedure, although appropriate for children with biliary atresia, does not benefit children with AGS and actually appears to worsen outcome. The current data suggest that the Kasai is not a marker for underlying severe liver disease, but the procedure itself may have a detrimental effect on outcome. An appropriate medical evaluation and particular consideration of AGS is essential before surgical referral in infants with high GGT cholestasis. OBJECTIVES:Alagille syndrome (AGS) frequently presents with neonatal jaundice and can mimic other causes of high γ-glutamyl transpeptidase (GGT) cholestasis, most notably biliary atresia. As a result infants with AGS may undergo intraoperative cholangiogram and even Kasai procedure. The aim of the study was to assess the hepatic outcomes of children with AGS who underwent the Kasai procedure. PATIENTS AND METHODS:A retrospective review of the AGS clinical database at the Childrenʼs Hospital of Philadelphia was performed to identify clinically defined patients with AGS who underwent a Kasai. A cohort of Alagille control subjects was selected with equivalent symptoms of neonatal jaundice and matched for age and presence of cardiac anomaly. JAGGED1-mutation analysis was performed on available samples. Clinical courses were reviewed. Fisher exact and t tests were used for analysis. RESULTS:Of the 430 patients with AGS, 19 underwent a Kasai procedure (K). The control cohort (C) consisted of 36 patients. Total bilirubin measured between 6 and 10 weeks of age in each cohort was equivalent (K9.6 mg/dL, C8.7 mg/dL); GGT levels were higher in the control group (K:493.4 U/L, C:574.4 U/L). Of note, the Kasai cohort had a significantly larger number of liver transplants (K9 [47.3%], C5 [13.9%], P = 0.01) and sustained higher mortality (K6 [31.6%], C1 [2.8%], P = 0.005). There was no genotype-phenotype correlation between the mutations identified and patients who underwent Kasai. CONCLUSIONS:These data suggest that the Kasai procedure, although appropriate for children with biliary atresia, does not benefit children with AGS and actually appears to worsen outcome. The current data suggest that the Kasai is not a marker for underlying severe liver disease, but the procedure itself may have a detrimental effect on outcome. An appropriate medical evaluation and particular consideration of AGS is essential before surgical referral in infants with high GGT cholestasis. ABSTRACT Objectives: Alagille syndrome (AGS) frequently presents with neonatal jaundice and can mimic other causes of high γ‐glutamyl transpeptidase (GGT) cholestasis, most notably biliary atresia. As a result infants with AGS may undergo intraoperative cholangiogram and even Kasai procedure. The aim of the study was to assess the hepatic outcomes of children with AGS who underwent the Kasai procedure. Patients and Methods: A retrospective review of the AGS clinical database at the Children's Hospital of Philadelphia was performed to identify clinically defined patients with AGS who underwent a Kasai. A cohort of Alagille control subjects was selected with equivalent symptoms of neonatal jaundice and matched for age and presence of cardiac anomaly. JAGGED1‐mutation analysis was performed on available samples. Clinical courses were reviewed. Fisher exact and t tests were used for analysis. Results: Of the 430 patients with AGS, 19 underwent a Kasai procedure (K). The control cohort (C) consisted of 36 patients. Total bilirubin measured between 6 and 10 weeks of age in each cohort was equivalent (K: 9.6 mg/dL, C: 8.7 mg/dL); GGT levels were higher in the control group (K:493.4 U/L, C:574.4 U/L). Of note, the Kasai cohort had a significantly larger number of liver transplants (K: 9 [47.3%], C: 5 [13.9%], P = 0.01) and sustained higher mortality (K: 6 [31.6%], C: 1 [2.8%], P = 0.005). There was no genotype‐phenotype correlation between the mutations identified and patients who underwent Kasai. Conclusions: These data suggest that the Kasai procedure, although appropriate for children with biliary atresia, does not benefit children with AGS and actually appears to worsen outcome. The current data suggest that the Kasai is not a marker for underlying severe liver disease, but the procedure itself may have a detrimental effect on outcome. An appropriate medical evaluation and particular consideration of AGS is essential before surgical referral in infants with high GGT cholestasis. |
Author | Kaye, Adam J Rand, Elizabeth B Kamath, Binita M Munoz, Pedro S Spinner, Nancy B Flake, Alan W |
AuthorAffiliation | Hospital of the University of Pennsylvania, USA †Childrenʼs Hospital of Philadelphia, Philadelphia, PA, USA ‡Hospital for Sick Children, Toronto, Canada |
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BackLink | https://www.ncbi.nlm.nih.gov/pubmed/20601899$$D View this record in MEDLINE/PubMed |
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References | 1995; 127 2001; 49 1993; 128 1987; 110 2003; 40 1999; 29 2003; 75 e_1_2_5_9_1 e_1_2_5_8_1 e_1_2_5_7_1 e_1_2_5_6_1 e_1_2_5_5_1 e_1_2_5_4_1 e_1_2_5_3_1 e_1_2_5_2_1 |
References_xml | – volume: 29 start-page: 431 year: 1999 end-page: 437 article-title: Variable morbidity in alagille syndrome: a review of 43 cases publication-title: J Pediatr Gastroenterol Nutr – volume: 128 start-page: 337 year: 1993 end-page: 339 article-title: Liver transplantation for Alagille's syndrome publication-title: Arch Surg – volume: 127 start-page: 220 year: 1995 end-page: 224 article-title: Outcome of syndromic paucity of interlobular bile ducts (Alagille syndrome) with onset of cholestasis in infancy publication-title: J Pediatr – volume: 29 start-page: 822 year: 1999 end-page: 829 article-title: Features of Alagille syndrome in 92 patients: frequency and relation to prognosis publication-title: Hepatology – volume: 40 start-page: 891 year: 2003 end-page: 895 article-title: Consequences of JAG1 mutations publication-title: J Med Genet – volume: 75 start-page: 2147 year: 2003 end-page: 2150 article-title: Living‐related liver transplantation for Alagille syndrome publication-title: Transplantation – volume: 49 start-page: 431 year: 2001 end-page: 435 article-title: Outcome of liver disease in children with Alagille syndrome: a study of 163 patients publication-title: Gut – volume: 110 start-page: 195 year: 1987 end-page: 200 article-title: Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases publication-title: J Pediatr – ident: e_1_2_5_8_1 doi: 10.1097/00005176-199910000-00011 – ident: e_1_2_5_3_1 doi: 10.1016/S0022-3476(87)80153-1 – ident: e_1_2_5_2_1 doi: 10.1136/jmg.40.12.891 – ident: e_1_2_5_5_1 doi: 10.1016/S0022-3476(95)70298-9 – ident: e_1_2_5_6_1 doi: 10.1097/01.TP.0000066804.33006.17 – ident: e_1_2_5_7_1 doi: 10.1136/gut.49.3.431 – ident: e_1_2_5_4_1 doi: 10.1002/hep.510290331 – ident: e_1_2_5_9_1 doi: 10.1001/archsurg.1993.01420150093017 |
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Alagille syndrome (AGS) frequently presents with neonatal jaundice and can mimic other causes of high γ‐glutamyl transpeptidase (GGT)... OBJECTIVES:Alagille syndrome (AGS) frequently presents with neonatal jaundice and can mimic other causes of high γ-glutamyl transpeptidase (GGT) cholestasis,... Alagille syndrome (AGS) frequently presents with neonatal jaundice and can mimic other causes of high gamma-glutamyl transpeptidase (GGT) cholestasis, most... OBJECTIVESAlagille syndrome (AGS) frequently presents with neonatal jaundice and can mimic other causes of high gamma-glutamyl transpeptidase (GGT)... |
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SubjectTerms | Alagille syndrome Alagille Syndrome - complications Alagille Syndrome - mortality Alagille Syndrome - surgery Bilirubin - blood Calcium-Binding Proteins - genetics Case-Control Studies gamma-Glutamyltransferase - blood Genotype Heart Diseases Humans Infant Intercellular Signaling Peptides and Proteins - genetics Jagged-1 Protein Jaundice - etiology Kasai Liver - enzymology Liver - surgery Liver Transplantation - statistics & numerical data Membrane Proteins - genetics Mutation Phenotype Portoenterostomy, Hepatic - methods Portoenterostomy, Hepatic - mortality Postoperative Complications - mortality Retrospective Studies Serrate-Jagged Proteins transplant Treatment Outcome |
Title | Effect of Kasai Procedure on Hepatic Outcome in Alagille Syndrome |
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