Relationship between biopsy-proven parenteralnutrition-associated liver fibrosis and biochemical cholestasis in children with short bowel syndrome

Shimae C. Fitzgibbons, Brian A. Jones, Melissa A. Hull, David Zurakowski, Debora Duro, Christopher Duggan, Dana Boctor, David L. Sigalet, Tom Jaksic

Research output: Contribution to journalArticle

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Abstract

Purpose: The aim of the study was to determine the frequency of biochemical cholestasis (direct bilirubin [DB] ≥2 mg/dL) in children with short bowel syndrome and biopsy-proven parenteral nutrition (PN)-associated liver disease and to define predictive factors for the occurrence and degree of hepatic fibrosis. Methods: After institutional review board approval, a retrospective review was conducted of patients followed by 2 multidisciplinary intestinal rehabilitation programs between January 1, 2000, and September 30, 2008. Inclusion criteria were exposure to PN (>30 days) and having undergone a liver biopsy. Liver biopsy specimens were graded from 0 to 3 based upon degree of fibrosis in the pathology report. The most recent DB within 10 days before biopsy was recorded. Results: A total of 66 children underwent 83 liver biopsy procedures. The most common diagnoses included necrotizing enterocolitis (NEC) (36.4%), gastroschisis (22.7%), and intestinal atresia (15.1%). Median age at biopsy was 6.1 months with a median duration of PN of 4.7 months. Of the patients, 70.3% had a history of exposure to parenteral ω-3 lipid emulsion. Of the liver biopsy specimens, 89% (74/83) demonstrated some degree of fibrosis (fibrosis scale 1-3), including 9.6% (8/83) with evidence of cirrhosis. 83% of biopsies without fibrosis and 55% of biopsies with fibrosis were obtained in patients without evidence of biochemical cholestasis (P = .20). Three (37%) of the 8 patients with cirrhosis on liver biopsy had no evidence of biochemical cholestasis. Univariate analysis identified only gestational age (GA) at birth as significantly associated with the degree of liver fibrosis (P = .03). A multivariate logistic regression model accounting for multiple biopsy procedures in patients revealed that GA was a predictor of fibrosis only in patients with a diagnosis other than NEC (P < .01). Conclusions: In children with short bowel syndrome, biochemical cholestasis does not reflect the presence or degree of histologically confirmed PN-associated liver fibrosis. Careful follow-up, combined with further refinement of diagnostic and hepatoprotective strategies, may be warranted in this patient population.

Original languageEnglish (US)
Pages (from-to)95-99
Number of pages5
JournalJournal of pediatric surgery
Volume45
Issue number1
DOIs
StatePublished - Jan 1 2010

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Short Bowel Syndrome
Cholestasis
Liver Cirrhosis
Biopsy
Fibrosis
Parenteral Nutrition
Liver
Necrotizing Enterocolitis
Bilirubin
Gestational Age
Logistic Models
Intestinal Atresia
Gastroschisis
Research Ethics Committees
Emulsions
Liver Diseases
Rehabilitation

Keywords

  • Biochemical cholestasis
  • Liver fibrosis
  • Parenteral nutrition
  • Short bowel syndrome

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

Relationship between biopsy-proven parenteralnutrition-associated liver fibrosis and biochemical cholestasis in children with short bowel syndrome. / Fitzgibbons, Shimae C.; Jones, Brian A.; Hull, Melissa A.; Zurakowski, David; Duro, Debora; Duggan, Christopher; Boctor, Dana; Sigalet, David L.; Jaksic, Tom.

In: Journal of pediatric surgery, Vol. 45, No. 1, 01.01.2010, p. 95-99.

Research output: Contribution to journalArticle

Fitzgibbons, SC, Jones, BA, Hull, MA, Zurakowski, D, Duro, D, Duggan, C, Boctor, D, Sigalet, DL & Jaksic, T 2010, 'Relationship between biopsy-proven parenteralnutrition-associated liver fibrosis and biochemical cholestasis in children with short bowel syndrome', Journal of pediatric surgery, vol. 45, no. 1, pp. 95-99. https://doi.org/10.1016/j.jpedsurg.2009.10.020
Fitzgibbons, Shimae C. ; Jones, Brian A. ; Hull, Melissa A. ; Zurakowski, David ; Duro, Debora ; Duggan, Christopher ; Boctor, Dana ; Sigalet, David L. ; Jaksic, Tom. / Relationship between biopsy-proven parenteralnutrition-associated liver fibrosis and biochemical cholestasis in children with short bowel syndrome. In: Journal of pediatric surgery. 2010 ; Vol. 45, No. 1. pp. 95-99.
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abstract = "Purpose: The aim of the study was to determine the frequency of biochemical cholestasis (direct bilirubin [DB] ≥2 mg/dL) in children with short bowel syndrome and biopsy-proven parenteral nutrition (PN)-associated liver disease and to define predictive factors for the occurrence and degree of hepatic fibrosis. Methods: After institutional review board approval, a retrospective review was conducted of patients followed by 2 multidisciplinary intestinal rehabilitation programs between January 1, 2000, and September 30, 2008. Inclusion criteria were exposure to PN (>30 days) and having undergone a liver biopsy. Liver biopsy specimens were graded from 0 to 3 based upon degree of fibrosis in the pathology report. The most recent DB within 10 days before biopsy was recorded. Results: A total of 66 children underwent 83 liver biopsy procedures. The most common diagnoses included necrotizing enterocolitis (NEC) (36.4{\%}), gastroschisis (22.7{\%}), and intestinal atresia (15.1{\%}). Median age at biopsy was 6.1 months with a median duration of PN of 4.7 months. Of the patients, 70.3{\%} had a history of exposure to parenteral ω-3 lipid emulsion. Of the liver biopsy specimens, 89{\%} (74/83) demonstrated some degree of fibrosis (fibrosis scale 1-3), including 9.6{\%} (8/83) with evidence of cirrhosis. 83{\%} of biopsies without fibrosis and 55{\%} of biopsies with fibrosis were obtained in patients without evidence of biochemical cholestasis (P = .20). Three (37{\%}) of the 8 patients with cirrhosis on liver biopsy had no evidence of biochemical cholestasis. Univariate analysis identified only gestational age (GA) at birth as significantly associated with the degree of liver fibrosis (P = .03). A multivariate logistic regression model accounting for multiple biopsy procedures in patients revealed that GA was a predictor of fibrosis only in patients with a diagnosis other than NEC (P < .01). Conclusions: In children with short bowel syndrome, biochemical cholestasis does not reflect the presence or degree of histologically confirmed PN-associated liver fibrosis. Careful follow-up, combined with further refinement of diagnostic and hepatoprotective strategies, may be warranted in this patient population.",
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AU - Fitzgibbons, Shimae C.

AU - Jones, Brian A.

AU - Hull, Melissa A.

AU - Zurakowski, David

AU - Duro, Debora

AU - Duggan, Christopher

AU - Boctor, Dana

AU - Sigalet, David L.

AU - Jaksic, Tom

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N2 - Purpose: The aim of the study was to determine the frequency of biochemical cholestasis (direct bilirubin [DB] ≥2 mg/dL) in children with short bowel syndrome and biopsy-proven parenteral nutrition (PN)-associated liver disease and to define predictive factors for the occurrence and degree of hepatic fibrosis. Methods: After institutional review board approval, a retrospective review was conducted of patients followed by 2 multidisciplinary intestinal rehabilitation programs between January 1, 2000, and September 30, 2008. Inclusion criteria were exposure to PN (>30 days) and having undergone a liver biopsy. Liver biopsy specimens were graded from 0 to 3 based upon degree of fibrosis in the pathology report. The most recent DB within 10 days before biopsy was recorded. Results: A total of 66 children underwent 83 liver biopsy procedures. The most common diagnoses included necrotizing enterocolitis (NEC) (36.4%), gastroschisis (22.7%), and intestinal atresia (15.1%). Median age at biopsy was 6.1 months with a median duration of PN of 4.7 months. Of the patients, 70.3% had a history of exposure to parenteral ω-3 lipid emulsion. Of the liver biopsy specimens, 89% (74/83) demonstrated some degree of fibrosis (fibrosis scale 1-3), including 9.6% (8/83) with evidence of cirrhosis. 83% of biopsies without fibrosis and 55% of biopsies with fibrosis were obtained in patients without evidence of biochemical cholestasis (P = .20). Three (37%) of the 8 patients with cirrhosis on liver biopsy had no evidence of biochemical cholestasis. Univariate analysis identified only gestational age (GA) at birth as significantly associated with the degree of liver fibrosis (P = .03). A multivariate logistic regression model accounting for multiple biopsy procedures in patients revealed that GA was a predictor of fibrosis only in patients with a diagnosis other than NEC (P < .01). Conclusions: In children with short bowel syndrome, biochemical cholestasis does not reflect the presence or degree of histologically confirmed PN-associated liver fibrosis. Careful follow-up, combined with further refinement of diagnostic and hepatoprotective strategies, may be warranted in this patient population.

AB - Purpose: The aim of the study was to determine the frequency of biochemical cholestasis (direct bilirubin [DB] ≥2 mg/dL) in children with short bowel syndrome and biopsy-proven parenteral nutrition (PN)-associated liver disease and to define predictive factors for the occurrence and degree of hepatic fibrosis. Methods: After institutional review board approval, a retrospective review was conducted of patients followed by 2 multidisciplinary intestinal rehabilitation programs between January 1, 2000, and September 30, 2008. Inclusion criteria were exposure to PN (>30 days) and having undergone a liver biopsy. Liver biopsy specimens were graded from 0 to 3 based upon degree of fibrosis in the pathology report. The most recent DB within 10 days before biopsy was recorded. Results: A total of 66 children underwent 83 liver biopsy procedures. The most common diagnoses included necrotizing enterocolitis (NEC) (36.4%), gastroschisis (22.7%), and intestinal atresia (15.1%). Median age at biopsy was 6.1 months with a median duration of PN of 4.7 months. Of the patients, 70.3% had a history of exposure to parenteral ω-3 lipid emulsion. Of the liver biopsy specimens, 89% (74/83) demonstrated some degree of fibrosis (fibrosis scale 1-3), including 9.6% (8/83) with evidence of cirrhosis. 83% of biopsies without fibrosis and 55% of biopsies with fibrosis were obtained in patients without evidence of biochemical cholestasis (P = .20). Three (37%) of the 8 patients with cirrhosis on liver biopsy had no evidence of biochemical cholestasis. Univariate analysis identified only gestational age (GA) at birth as significantly associated with the degree of liver fibrosis (P = .03). A multivariate logistic regression model accounting for multiple biopsy procedures in patients revealed that GA was a predictor of fibrosis only in patients with a diagnosis other than NEC (P < .01). Conclusions: In children with short bowel syndrome, biochemical cholestasis does not reflect the presence or degree of histologically confirmed PN-associated liver fibrosis. Careful follow-up, combined with further refinement of diagnostic and hepatoprotective strategies, may be warranted in this patient population.

KW - Biochemical cholestasis

KW - Liver fibrosis

KW - Parenteral nutrition

KW - Short bowel syndrome

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