Biliary Atresia: Clinical Profiles, Risk Factors, and Outcomes of 755 Patients Listed for Liver Transplantation

Utterson, Elizabeth C.; Shepherd, Ross W.; Sokol, Ronald J.; Bucuvalas, John C.; Magee, John C.; McDiarmid, S. V.; Anand, Ravinder

doi:10.1016/j.jpeds.2005.04.073

Cited by 185 publications

(193 citation statements)

References 29 publications

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“…This outcome further confirms that the split-liver technique has improved and is now comparable to the other techniques (McDiarmid, Anand, & Lindblad, 2004;Utterson et al, 2005;Bilik, Greig, Langer, & Superina, 1993;Whitington & Balistreri, 1991). The first split-liver procedures performed on segments II and III use to imply a longer hepatic ischemia time, thus affecting the outcomes of the transplant itself, with a high difficulty of recovery of the grafted organ (Spada et al, 2009).…”

Section: Discussionsupporting

confidence: 58%

Therapeutic Effectiveness of Nutrition Therapy in Pediatric Patients with Chronic Liver Diseases Awaiting Liver Transplantation

Giammanco¹,

Bavetta²,

Greco³

et al. 2013

JFR

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It is important to prevent protein/calorie malnutrition in children with end stage liver diseases prior to transplantation. This study involved 34 patients between the ages of 10 and 156 months (mean value 25.69 months  32.2) (13 females and 21 males) on the liver transplant waiting list. Data collected as of three months before transplant and up to ten months after the procedure concerned gender, age, weight, height, Pediatric End Stage Liver Disease Score, baseline pathology, type of nutrition, type of transplant, immunosuppression, pulse steroid therapy, length of stay, and post transplant complications. Linear regression analysis showed that the length of hospital stay was 24.5 days more for females than for males, but also that intensive nutrition therapy shortens this stay for both female patients (P = 0.085) and younger patients (P = 0.023). The study population was divided into two groups according to the different nutritional therapies adopted. The Student's t-test and Mann-Whitney test evidenced that the group receiving intensive nutrition therapy grew taller compared with the group following an oral diet (mean -1.37 and Prob = 0.043); that females grew taller compared to males (mean -1.65 +/-0.56); and that there was an increase in height among the children in the group receiving intensive nutrition therapy despite the presence (-1.37 +/-0.56) or absence (-14.8 +/-5.44 and Prob = 0.035) of complications, and despite the administration (-1.03 +/-0.33) or non administration (-1.48 +/-0.55 and Prob = 0.019) of steroids. Intensive nutrition therapy enhances the velocity of growth in height and shortens the length of hospital stay, thus optimizing the final prognosis of the baseline pathology.

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Section: Discussionsupporting

confidence: 58%

Therapeutic Effectiveness of Nutrition Therapy in Pediatric Patients with Chronic Liver Diseases Awaiting Liver Transplantation

Giammanco¹,

Bavetta²,

Greco³

et al. 2013

JFR

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“…Unfortunately, no large-scale studies have concurrently evaluated outcomes and risk factors for both rejection and all types of infection. The imperative for such a study is emphasized by recent data, which suggest that morbidity and mortality from infections in children after liver transplantation may exceed those for rejection (3,4).…”

Section: Introductionmentioning

confidence: 99%

Risk Factors for Rejection and Infection in Pediatric Liver Transplantation

Shepherd

Turmelle

Nadler

et al. 2008

American Journal of Transplantation

135

105

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Rejection and infection are important adverse events after pediatric liver transplantation, not previously subject to concurrent risk analysis. Of 2291 children (<18 years), rejection occurred at least once in 46%, serious bacterial/fungal or viral infections in 52%. Infection caused more deaths than rejection (5.5% vs. 0.6% of patients, p < 0.001). Early rejection (<6 month) did not contribute to mortality or graft failure. Recurrent/chronic rejection was a risk in graft failure, but led to retransplant in only 1.6% of first grafts. Multivariate predictors of bacterial/fungal infection included recipient age (highest in infants), race, donor organ variants, bilirubin, anhepatic time, cyclosporin (vs. tacrolimus) and era of transplant (before 2002 vs. after 2002); serious viral infection predictors included donor organ variants, rejection, Epstein-Barr Virus (EBV) naivety and era; for rejection, predictors included age (lowest in infants), primary diagnosis, donor-recipient blood type mismatch, the use of cyclosporin (vs. tacrolimus), no induction and era. In pediatric liver transplantation, infection risk far exceeds that of rejection, which causes limited harm to the patient or graft, particularly in infants. Aggressive infection control, attention to modifiable factors such as pretransplant nutrition and donor organ options and rigorous age-specific review of the risk/benefit of choice and intensity of immunosuppressive regimes is warranted.

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“…Often, the Kasai procedure will only reduce the rate of progression, and the patient will receive a liver transplant a few years later. 7,8 Contrary to BA, patients with Alagille syndrome (AGS) suffer from deep jaundice and severe pruritus as a consequence of the cholestasis caused by congenital intrahepatic ductopenia. 9,10 Progression to liver cirrhosis, however, is slower than in BA, and these patients rarely develop severe manifestations of portal hypertension and transplantation is eventually indicated because of failure to thrive, itching, and hypercholesterolemia.…”

mentioning

confidence: 99%

Analysis of Liver Repair Mechanisms in Alagille Syndrome and Biliary Atresia Reveals a Role for Notch Signaling

Fabris

Cadamuro

Guido

et al. 2007

The American Journal of Pathology

124

120

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Patients with Alagille syndrome (AGS), a genetic disorder of Notch signaling, suffer from severe ductopenia and cholestasis, but progression to biliary cirrhosis is rare. Instead, in biliary atresia (BA) severe cholestasis is associated with a pronounced "ductular reaction" and rapid progression to biliary cirrhosis. Given the role of Notch in biliary development, we hypothesized that defective Notch signaling would influence the reparative mechanisms in cholestatic cholangiopathies. Thus we compared phenotype and relative abundance of the epithelial components of the hepatic reparative complex in AGS (n ‫؍‬ 10) and BA (n ‫؍‬ 30) using immunohistochemistry and computer-assisted morphometry.

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Biliary Atresia: Clinical Profiles, Risk Factors, and Outcomes of 755 Patients Listed for Liver Transplantation

Cited by 185 publications

References 29 publications

Therapeutic Effectiveness of Nutrition Therapy in Pediatric Patients with Chronic Liver Diseases Awaiting Liver Transplantation

Therapeutic Effectiveness of Nutrition Therapy in Pediatric Patients with Chronic Liver Diseases Awaiting Liver Transplantation

Risk Factors for Rejection and Infection in Pediatric Liver Transplantation

Analysis of Liver Repair Mechanisms in Alagille Syndrome and Biliary Atresia Reveals a Role for Notch Signaling

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