Level of γ-glutamyltransferase in 2-Year-old Children With Biliary Atresia Associates With Progression of Portal Hypertension

Freeman, A. Jay; Ng, Vicky L.; Harpavat, Sanjiv; Hrycko, Alexander; Apted, Zoe; Bulut, Pinar; Leong, Traci; Karpen, Saul J.

doi:10.1016/j.cgh.2017.02.025

Cited by 5 publications

(5 citation statements)

References 9 publications

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“…Recently, few studies have shown that GGT to platelet ratio had a higher predictive values compared to APRI especially in patients with chronic hepatitis B and non‐alcoholic fatty liver disease (NAFLD) . In children, serum GGT has been shown to be an important parameter estimating the severity of liver fibrosis and ultimately portal hypertension . In our study, GGT:platelet ratio was observed to be a significant predictor of liver transplantation or death.…”

Section: Discussionsupporting

confidence: 52%

“…23,24 In children, serum GGT has been shown to be an important parameter estimating the severity of liver fibrosis and ultimately portal hypertension. 25 In our study, GGT:platelet ratio was observed to be a significant Children's Healthcare of Atlanta is a tertiary referral centre; therefore, our paediatric AIH cohort may be sicker than has been reported in prior studies. 29 Hence, we cannot completely exclude selection bias and a potential contributor the current findings.…”

Section: Discussionmentioning

confidence: 50%

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Racial disparities in presentation and outcomes of paediatric autoimmune hepatitis

Palle

Naik

Kolachala

et al. 2019

Liver International

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Background & Aims Most studies on autoimmune hepatitis (AIH) in children are in predominantly Caucasian cohorts. Paediatric AIH in African Americans (AA) is understudied, with a dearth of clinical predictors of outcome, often leading to serious complications and even mortality. The aim of the study was to define disease presentation, progression, response to therapy and outcomes in paediatric AIH in a well‐defined, large, single centre, demographically diverse population. Methods We conducted a review of patients with AIH who were followed at this tertiary liver transplant centre. Clinical and laboratory covariates were assessed with regard to disease presentation, progression and outcomes in AA vs Non‐AA children. Results African Americans patients constituted 42% of this cohort. At 1‐year follow‐up, AA children were receiving significantly higher doses of steroids compared to non‐AA. More AA presented with end‐stage liver disease (ESLD) with high immunoglobulin G and GGT:platelet ratio. After adjusting for other risk factor variables like gender, age at presentation and ESLD, AA children were at 4.5 times higher risk for significant outcome liver transplant/death within the first 12 months of presentation. Post‐transplant, recurrent AIH was seen in 50% of AA vs 8% in non‐AA. Conclusions African American patients with AIH are more likely to present with ESLD and have an increased early risk for transplantation with high likelihood of disease recurrence post‐transplantation. Studies are needed to delineate factors such as inherent biology, genetics and access to care. Early referral and tailored immunosuppressive regimens are required for AA patients with AIH.

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Section: Discussionsupporting

confidence: 52%

Section: Discussionmentioning

confidence: 50%

Racial disparities in presentation and outcomes of paediatric autoimmune hepatitis

Palle

Naik

Kolachala

et al. 2019

Liver International

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“…Other factors evaluated, such as anatomic pattern of BA (15,16) and liver histology (10,20) have shown variable impacts on outcomes. Although postoperative GGT levels have been evaluated as a predictor of clinical outcome in BA (36,37), initial GGT level at diagnosis has not been scrutinized till date. In this study, we hypothesized and confirmed that low GGT levels at presentation in BA is associated with poor outcomes as evidenced by earlier and younger age at transplantation in the normal GGT group.…”

Section: Discussionmentioning

confidence: 99%

Normal Gamma Glutamyl Transferase Levels at Presentation Predict Poor Outcome in Biliary Atresia

Shankar

Bolia

Foo

et al. 2020

J. pediatr. gastroenterol. nutr.

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Objectives: Gamma-glutamyl transferase levels (GGT) are typically elevated in biliary atresia (BA), but normal GGT levels have been observed. This cohort of “normal GGT” BA has neither been described nor has the prognostic value of GGT level on outcomes in BA. We aimed to describe outcomes of a single-centre Australian cohort of infants with BA and assess the impact of GGT level at presentation on outcomes in BA. Methods: Infants diagnosed with BA between 1991 and 2017 were retrospectively analysed. Outcomes were defined as survival with native liver, liver transplantation (LT), and death. Patients were categorized into normal (<200I U/L) or high GGT groups based on a mean of 3 consecutive GGT values done before Kasai portoenterostomy (KPE). Baseline parameters, age at surgery, clearance of jaundice (COJ), and outcomes were compared between the 2 groups. Results: One hundred thirteen infants underwent KPE at median 61 (30–149) days. At a median follow-up of 14.2 (0.9–26.3) years, 35% (39/113) patients were surviving with native liver, 55% (62/113) underwent LT and 11% (12/113) died pretransplant. 12.3% (14/113) patients had normal GGT. Age at KPE and time to COJ were similar between normal and high GGT groups. Normal GGT group had shorter time from KPE to LT (11 vs 18 months, P = 0.02), underwent LT at a younger age (14 vs 20 months, P = 0.04), and had poorer transplant-free survival (P = 0.04) than high GGT group. Conclusions: 12.3% of infants with BA had normal GGT levels at diagnosis. Low GGT levels at presentation in BA was associated with a poorer outcome.

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“…(15)(16)(17) Because the serum ALP levels widely fluctuate in children due to bone growth, serum gamma glutamyltransferase (GGT) is used commonly in children as a marker for cholestasis. (18) Serum GGT levels have been found to be of prognostic importance in other pediatric cholestatic diseases, including biliary atresia, (19) total parenteral nutrition-related cholestasis, (20) idiopathic neonatal hepatitis, (21,22) and neonatal sepsis-related aRtiCle inFoRmation: cholestasis. (23) Elevated serum GGT is the most consistent biochemical abnormality in pediatric patients with PSC, and it appears to be a more sensitive indicator for bile duct injury in PSC compared with ALP and bilirubin.…”

mentioning

confidence: 99%

Gamma Glutamyltransferase Reduction Is Associated With Favorable Outcomes in Pediatric Primary Sclerosing Cholangitis

Deneau

Mack

Abdou

et al. 2018

Hepatology Communications

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Adverse clinical events in primary sclerosing cholangitis (PSC) happen too slowly to capture during clinical trials. Surrogate endpoints are needed, but no such validated endpoints exist for children with PSC. We evaluated the association between gamma glutamyltransferase (GGT) reduction and long‐term outcomes in pediatric PSC patients. We evaluated GGT normalization (< 50 IU/L) at 1 year among a multicenter cohort of children with PSC who did or did not receive treatment with ursodeoxycholic acid (UDCA). We compared rates of event‐free survival (no portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or liver‐related death) at 5 years. Of the 287 children, mean age of 11.4 years old, UDCA was used in 81% at a mean dose of 17 mg/kg/day. Treated and untreated groups had similar GGT at diagnosis (314 versus 300, P= not significant [NS]). The mean GGT was reduced at 1 year in both groups, with lower values seen in treated (versus untreated) patients (99 versus 175, P= 0.002), but 5‐year event‐free survival was similar (74% versus 77%, P= NS). In patients with GGT normalization (versus no normalization) by 1 year, regardless of UDCA treatment status, 5‐year event‐free survival was better (91% versus 67%, P< 0.001). Similarly, larger reduction in GGT over 1 year (> 75% versus < 25% reduction) was also associated with improved outcome (5‐year event‐free survival 88% versus 61%, P= 0.005). Conclusion:A GGT < 50 and/or GGT reduction of > 75% by 1 year after PSC diagnosis predicts favorable 5‐year outcomes in children. GGT has promise as a potential surrogate endpoint in future clinical trials for pediatric PSC.

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Level of γ-glutamyltransferase in 2-Year-old Children With Biliary Atresia Associates With Progression of Portal Hypertension

Cited by 5 publications

References 9 publications

Racial disparities in presentation and outcomes of paediatric autoimmune hepatitis

Racial disparities in presentation and outcomes of paediatric autoimmune hepatitis

Normal Gamma Glutamyl Transferase Levels at Presentation Predict Poor Outcome in Biliary Atresia

Gamma Glutamyltransferase Reduction Is Associated With Favorable Outcomes in Pediatric Primary Sclerosing Cholangitis

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